The
Bobbsey Twins Take Neurology (multiple sclerosis)
Craig R. Schaul
Source: Klawans, Harold (1990). The Bobbsey Twins Take Neurology Newton's
Madness, 115-123.
Patient Background: Mary Rickert was a medical student who had decided
to become a neurologist along with her husband at the time. She was devastated
when, in 1972, she developed retrobulbar neuritis in her right eye, which
is an inflammation of the retrobulbar optic nerve. The inflammation is
the known precursor of Multiple Sclerosis, which she was later diagnosed
with. She was then put on steroids, which was the only available treatment
for controlling Multiple Sclerosis in 1972, The diagnosis of MS caused
the breakup of her and her husband's marriage. She decided to become a
dermatologist instead of a neurologist. In 1976 she was remarried, and
had a child by 1980. Behavior Change: Mary's first symptom was retrobulbar
neuritis, a complication in which 1/3 of it's sufferers develop MS. Characteristics
of this inflammation are blurry vision, with what seemed like a "black
hole" in front of her eye. This "hole" is actually behind
the eye in the optic nerve. Mary also showed other early symptoms characteristic
of MS before the actual diagnosis was made. These included her right leg
and foot ceased to work properly for about 2 weeks. These two symptoms
were separated by "time and space", a distinction crucial to
make the diagnosis of MS. This means that the two lesions causing the symptoms
had to be in completely different locations within the brain to be classified
as separated. Mary possessed the needed separate lesions, which determined
the diagnosis of Multiple Sclerosis.
Nervous System Changes: Mary also had another symptom characteristic
of MS, increased reflexes in her right leg. Mary had three serious attacks
in the next two years, all three involved the brainstem, the symptoms of
the attacks were the same, slurred speech, drunken gait, and inability
to control her urine. Physician and Family Reflection: Mary's mentor
in medical school was the neurologist who made the diagnosis. He has maintained
friendship with her since she was diagnosed with MS. He has supported her
throughout the ordeal. Mary's husband at the time of both of their admission
into medical school couldn't deal with her disorder and he left her between
her first and second attacks.
My Reaction to the Case: My reaction to this article comes from
experience. My dad was diagnosed with Multiple Sclerosis in 1984. I can
understand her husband's reaction, but to walk away when your loved one
needs you the most isn't the answer. There has been many times that I wanted
to escape from my dad's situation, but I have stuck it out and have given
him as much support as I can. Right now, this is the time in which he needs
it most.
The Wandering Man with
Epilepsy
Jaime Rogers
Source: Klawans, Harold. (1990). The man about town. Newton's Madness,
73-79.
Patient Background: William (Willey) Miranda was fifty-six years
old when first admitted to the E.R. around 10:30 am. The police admitted
him after observing him sitting in his car, staring straight ahead in an
apparent daze, for about two hours. Mr. Miranda did not know where he was
or how he'd gotten there. Nor did Mrs. Miranda whom was used to such incidences.
Mr. Miranda was now retired. However, in his early twenties he received
a medical discharge from the armed forces after sustaining a cerebral concussion.
Behavioral Change: According to Mrs. Miranda, Willie's first episode
had taken place some thirty or more years earlier. During his shorter episodes,
which she usually observed, he seemed to be indifferent to what was going
on around him. He would neither speak nor answer questions. Instead, he
would repeatedly perform meaningless tasks: opening and shutting a window,
washing his hands over and over again, or taking off his shoes and putting
them back on. If Mrs. Miranda spoke to him, he would often reply but always
with one- or two- word answers. Never any more. Willie's trips were all
similar in that he never remembered any detail of his travels. The didn't
occur often, only once every few years. They usually lasted well over a
day. He apparently drove reasonably well: he had no accidents or tickets.
And he never ran out of gas, always remembering to refill the tank as he
drove around. Once he even changed a tire. Or at least they thought he
did, since when he got home, the spare had replaced the left rear wheel,
which was now in the trunk and in need of repair.
Nervous System Changes: Mr. Miranda's neurologic exam was normal,
as was his psychiatric assessment. His CAT scan showed no abnormal shapes
of brain tissue or ventricles, and also no gross abnormalities (tumors,
hematoma, compression of tissue etc.). However, an EEG showed a seizure
like activity from the left temporal lobe. Damage to the left temporal
lobe may have occured after sustaining a cerebral concussion.
Family or Physician Reflections: Mrs. Miranda was ready to take
Willie home after his last "Little Trip". Apparently, she had
always linked Willie's little trips to stress as she had done in this case.
Willie's brother had just died in an automobile accident. Thus, she linked
Willie's little trip to his "shock" over his brother's death.
Once Willie had had his first three or four episodes and always came back
alive and well, the Mirandas stopped worrying about him. They had become
just Willie's little trips to relieve stress and forget about everything.
Other people had worse problems so they didn't make a big deal out of it.
They even made a game out of his longer journeys. As the neurology resident
on call examined Mr. Miranda, he was not so certain. He thought Willie
might well have just had a seizure and that his entire trip might have
been a part of that seizure. He felt strongly that medications that help
to control seizures might help him. He was aware that such trips had been
described before in other patients with "epilepsy" and that in
such patients they had nothing to do with any psychiatric problems. Mr.
Miranda was put on anticonvulsants and, as far as the resident knew, he
had not taken another little trip since.
Your Reaction to the Case: I thoroughly enjoyed the chapter, "The
Man about Town". It contained just the right amount of information
and was not the least bit sterile. I have always been interested in epilepsy
because my brother's good friend has it. Mr. Miranda provided a lot of
valuable and interesting information on the subject of epilepsy.
Tourette's Syndrome
Jeff Wooton
Source: Sacks, Oliver (1995). An Anthropologist on Mars,
New York.
Online resource: Karch, Joshua P. (1997) An Encounter with Tourette's
Syndrome. Http://cec.wustl.edu/~ jpkl1/tourettes.html.
Behavioral Changes: Dr. Carl Bennett is a skilled surgeon and
general practioner living and working in Branford, British Columbia. His
symptoms of Tourette's Syndrome began at the age of seven, but he was not
clinically diagnosed until age thirty-seven. He was an adopted child, therefore
his family history is unknown. When the disease first manifested in his
youth the symptoms were modest, but they soon became progressively more
elaborate and consuming of his daily life. He maintains an active interest
in hiking, mountain climbing, cross-country skiing and piloting a single-engine
jet plane. The author focused on his multi-day visit with Dr. Bennett,
therefore much of his early repurcussions of the disease were undocumented.
Dr. Bennett has experienced what could be called a full-fledged alteration
of personality as a result of his infliction of Tourette's Syndrome. "Funny
disease, I don't think of it as a disease but as just me," said Dr.
Bennett on the topic of his lifelong neurological disorder. Tourette's
involves many attributes of obsessive-compulsive disorder. His biggest
obsessions are touching his glasses to make sure they are on perfectly
straight and rubbing his mustache so that it is groomed perfectly, down
to each individual hair. His touching of objects or people must be done
symetrically. He will constantly re-adjust his legs in relation to the
steering wheel (car or plane) until his knees are symetrical with the wheel.
They must be exactly centered at all times. He also has a rapid walk with
a skip on each fifth step. Another plaguing action is his sudden reachings
to the ground as if to pick up something. No documentation of screaming
obsenities was given, but he instead has several catch-phrases he will
illicit in a high-pitched vocalization. His current favorite is "hideous."
He is attracted to the word by its sound. He also enjoys "Hooty-hoo,"
"Hi, there" and "whoo, whoo." He obsesses on one or
two words for about three months at a time. When one is released from his
current vocabulary it is immediately replaced with a new word or phrase.
He is attracted to the "melody" of a word and than implants into
his mind for future repetitive usage. In refrence to his "Tourette
Vocabulary" he said "Tourette's is a disease of inhibition. It
releases primitive behaviors." His children adore him and even offer
him lists of potentially new vocabulary words. His wife says that she wouldn't
want him any other way, she loves him just the way he is. Unfortunately,
the disease also strikes him with sudden rage. He is capable of supressing
this instict until he is at home where he will fling inanimate objects
across the room, making sure no one is in the pathway. If his motor and
cognitive functioning is simultaneously activated, his symptoms are kept
to a minimum, often seemingly disease free.
Nervous System Changes: Tourette's Syndrome is a hereditary
neurological disorder which affects mostly males, it usually strikes during
childhood or adolescence. It alters the individual's motor functions and
produces involuntary movements (tics). The cause of the disorder is still
unknown. The current research is speculating that an overabundance of the
neurotransmitter Dopamine produces the tics. Tourette's is characterized
by compulsive tics, involuntary mimicry or repetition of others' words
or actions and by involuntary or compulsive utterances of obscenities.
Tourette's is semi-controllable by medications known as Neuroleptics. These
medications work at reducing the release of Dopamine, which in turn is
to reduce the tics of the patient. It's results and side-effects vary from
patient to patient.
Physician's Reflections: Oliver Sacks, author of Bennett's account,
offered much reflection the daily life of Dr. Bennett. He was struck by
how Tourette's Syndrome has a highly creative aspect as well as its negative
attributes. He was often impressed by Dr. Bennett's observations of situations
and scenery. What seemed like drab scenery came to life when described
by Dr. Bennett. He also sees him as a deep thinker with a restless and
questioning mind. Before this meeting he believed many professions would
be necessarily closed to people with Tourette's. He thought this especially
true of the "intricate, precise and steady work of a surgeon."
When he was invited to attend a surgery, his position changed. For when
he operates, he is indeed Tourette free, as long as he is void of interruption.
He contended that Dr.Bennett was such a good surgeon as a result of the
disease. His compulsive nature would not allow him to overlook any aspect
of the operation, and would require multiple checks on the work completed.
No detail could possibly be overlooked. This observation also made him
relaxed enough to be flown by Bennett to Calgary via his plane. As he observed
his rechecking of every pre-flight detail, he knew the plane would be in
top-notch condition. He was also impressed, perhaps shocked, when a patient
came in with a grotesque skin growth disorder that covered large portions
of his body for examination and removal that there were no shrills of "hideous."
These are just a handful of the many observations that Sacks made. He did
have a tendency to question every aspect of Bennett's life and wondered
if it was a result of Tourette's Syndrome or not. Once you go past the
obvious it is difficult to tell. It's nearly impossible to draw the line
between normal personality and symptoms of Tourette's poking through. This
is under the assumption that there is a line to be drawn.
Personal Reflections: I would be lying if I didn't admit I
was hoping for some humerous operating room tales of obscenity rants and
raves. In fact, Dr. Bennett apparently does not suffer from this typical
trait of Tourette's Syndrome. Sad but true. Before this report, I was highly
ignorant of the disease. All I knew, in regard to the syptoms, was the
uninhibited usage of profanity. In fact, I thought my elderly life would
be enhanced by the onset of Tourette's Syndrome. I will have to find a
new hobby for my retirement as I learned it is genetic (no family background
here) and onsets by adolescence. With more knowledge of the disease I no
longer believe it would be a fun experience. It is dysfunctional to one's
lifestyle, but not disabling. Not only are the thoughts uninhibited, but
so are unneccesary motor activity. So instead of jealousy towards victims
of Tourette's Syndrome, I now carry compassion, empathy- but most of all
an understanding. Your Title: Rolando P. Your Name: Jennifer Jewell Source:
Sack, Oliver. Awakenings. New York: HarperCollins Publishing, 1990. 116-128.
Patient Background: Rolando P.'s infancy and early childhood was very normal.
He showed no signs of any dysfunctions or incapabilities. In fact, Rolando
P. was excelling in motor and speech. At 30 months of age he suffered from
encephali- tis lethargica. This illness lasted for about 18 week and forever
changed Rolando's life. Behavioral Change: After Mr. P's bout with encephalitis
lethargica, he suffered from severe motor dys- function, the inability
to communicate through speech or facial expressions, as well as excessive
salivating. Many felt his mental ability had also been diminished, but
his mother argued that he was still as bright as he was before he became
ill, now he just couldn't express it to others. In early childhood, Rolando
attended classes for the mentally challenged, but this became too difficult
for Mr. P because of his inability to sustain his balance. He was helpless
without the support of someone else. From then he was at home with his
parent through his adolescent years. His parents then admitted him to a
hospital where Mr. P's limb became more rigid and his speech was more inaudible.
The rigidity was slightly decreased in a left-sided chemopallidectomy operation.
Even after the opera- tion, Mr. P's speech remained predominately inaudible
and the rigidity in the right side of the body persisted. Mr. P was started
on L-DOPA in an effort to reduce some of these symptoms. By the first week,
the results were evident. His motor capability increased, he was now able
to walk with very little assistance. The rigidity in his limbs, trunk,
and neck were almost non-existent, the drooling had stopped, and he was
able to communicate audibly. Mr. P also developed sexual urges. These positive
results were short lived. By the second week on the medica- tion, there
was an onset of akathisia. Thus his L-DOPA dosage was reduced. His akathisia
was now specific which affected his right leg and a forced chewing movement.
His libido continued to be strongly present. Mr. P increasingly became
more hostile, irritable, and anxious. He found his eye to become fixed
by moving object and he could not stop staring at the object until it was
removed from his field of vision. Once again his L-DOPA was reduced. The
excitomotor syndrome continued and haloperidal was added to his medication
list. Mr. P's Parkinson's symptoms began to overshadow his excitomotor
states. He continued to fall into sleepy states whether L-DOPA was increased
or decreased. These states were only suppressed when in the presence of
his family and while he was away from the facility. When he was returned
to the facility the sleepy/depressed states resumed. Nervous System Changes:
Mr. P's nervous system was very unstable. All of his behavior had an oscillatory,
all-or-none quality that the doctors could not correct with medication.
They tried to correct his inability to be mobile and communicate with an
operation and the use of L-DOPA. L-DOPA did help these symptoms to a certain
extent only to onset excitomotor syndrome. Family or Physician Reflection:
Mr. P's mother played a very important role in his life. From the beginning
she stood by him, not labeling him an invalid. She visited him weekly while
she was well and it was during these visits when Rolando showed better
mental and physical signs. When his mother passed away, Rolando became
very depressed, having no one there to nurture and love him. To fill the
shoes of his mother was a physiotherapist. She took Rolando under her wing
and gave him the nurturance that he needed. It was only after she was dismissed
from her position at the hospital and was no longer able to see Rolando
that he gave up the will to live. He gave up all hope, knowing that there
was no one left to care about him and love him the way his mother and the
physiotherapist could. Your Reaction to the Case: With Rolando's situation,
it is sad to see how easy it would be to give up on someone because of
their lack of ability to communicate. Rolando was lucky enough to have
a loving and caring family and doctor that never gave up on him. Despite
some of the side effects of the L-DOPA, it did make dramatic changes in
his life. It allowed him to be mobile and allowed his speech to be audible.
Medicine can only do so much for a person, the power of love can be the
cure for all. In the presence of his family, Rolando's symptoms seemed
to prac- tically disappear. Feeling loved gave him the will to go on and
con- tinue fighting for his life. When those people that cared for him
were gone was when Rolando gave up hope. I believe that without hope there
was little that science could do for Rolando.
- Those Two Rays
Jeffrey A. Hulne
Source: Sacks, Oliver W. (1985). Witty Ticcy Ray. The Man Who Mistook
His Wife For a Hat and Other Clinical Tales. 87-96.
Patient Background: Patient Ray, presented to Dr. Sacks, a nearly
incapacitated condition consisting of multiple tics of extreme violence
that occurred every few seconds. Ray had been suffering from this condition
since he was four years old and had attracted much attention to himself
throughout his lifetime.
Behavior Change: After college and prior to treatment, Ray had been
fired from a dozen jobs ¾ always due to his tics and never for poor
work performance. He was very impatient, combative, and had problems with
relationships involving his wife and friends. The problems in his marriage
came from his crying out curse words when he got sexually excited. The
problems with his friends came from his combative and overly competitive
personality. He was, before treatment, a remarkably passionate musician
and weekend jobs as a Jazz drummer gave him a place to vent his built-up
emotions and earn a bit of money. Due to his fast reaction reflexes he
was also a very good Ping-Pong player. He also had a very quick wit. Ray
was very sensitive to Sack's prescribed medication, haldol. Following his
first treatment, Ray showed up at Sack's office with a black eye from trying
to beat it through a fast revolving door. Prior to medication Ray could
dart in and out of revolving doors with no problems. The haldol had slowed
his reaction time down and he got caught in the door. Once the proper dosage
was determined Ray was able to function quite normally. Today Ray has a
good job and a healthy familial and social life. Nervous System Changes:
Tourette's Syndrome is an organic neurological disorder. Tourette's
increase in emotions and passions is due to changes that lie in the thalamus,
hypothalamus, limbic system, and ambygdala. Touretter's have an excess
of exciter neurotransmitters, one of which is dopamine. This increase in
dopamine is believed to also contribute to the tics and quick reaction
reflexes. Haldol is a dopamine antagonist and is used to relieve the patients
of their symptoms. Family and Physician Reactions: As mentioned
above, Ray's marriage was threatened due to his symptoms. He had few close
friends and had trouble keeping them. He could not hold down a job. After
treatment with haldol his marriage was improved and he became a father.
He has many close friends and a good, stable job. His quick wit also returned.
He did, however, miss his pre-treatment drumming and Ping-Pong playing
abilities and felt that the haldol made him dull. To remedy this, Ray decided
to take haldol during the week but go haldol free on the weekends. Now
he has the best of both worlds. There are two Rays, one on haldol and the
other off haldol. Dr. Sacks stood behind this decision without regret and
felt Ray was handling things very well.
Personal Reactions: This case study satisfied a need in me to learn
more about Tourette's Syndrome. The study revealed how Tourette's was first
discovered and how the knowledge of it has progressed. In this case study
Ray only knew himself as a Touretter and was unready, at first, to suppress
that person. I found it interesting that Ray was able to keep that Tourette
person for the weekends.
A Case of Tourette
Syndrome
Sara Nelson
Source: Sacks, Oliver. (1995). A surgeon's life. An Anthropologist
from Mars, 77-107.
Patient Background: Patient A suffers from Tourette Syndrome, a
disease which affects sufferers by inducing unpredictable tics, twitches,
strange gestures and vocalizations, and a variety of other seemingly strange
behaviors. In earlier years, Tourette's was viewed as a moral disease,
not one of organic origin. Later, it was seen as a psychological disorder,
and even later in the 1960s as a chemical disturbance involving an imbalance
of the neurotransmitter dopamine in the brain. All of these views need
to be combined to attempt to fully explain this disease. Patient A leads
a fairly normal life outside of the Tourette's, a surgeon from British
Columbia with a wife and two sons. His work as well as personal life seem
to be manageable and pleasant to him despite the disorder.
Behavioral Change: Patient A's disorder has inflicted him with a
variety of behaviors that can be assessed as both negative and positive.
In the company of those A is aquatinted with, his Tourette behaviors are
frequent and quite visible. Patient A has a compulsion to touch things,
often repetitively and in groups of threes, five's, fours, etc. The touching
must also be symmetrical, for example the patient will repeatedly touch
his glasses, top, bottom, left, right, continually and always in this specific
order. The patient reports the touching is often a matter of something
being in A's personal space, therefore he feels compelled to touch it.
This issue of space seems to be markedly altered in many individuals with
Tourette Syndrome. Patient A will often reach out and touch or tap a familiar
colleague during a meeting or a press conference if they are sitting in
a close viscinity. Another symptom visible in this patient is the sudden
shrill voiced comments that seem to come out of nowhere. A reports being
fascinated with strange sounds, such as any name, title, or anything sounding
unusual to a speaker of the English language. To the patient, the comments
seem to have no explanation. For example, one phrase used often by A is
"Hi Patti!" with Patti being the name of an old girlfriend from
years ago. A will often be unable to remember these spontaneous recitations
if asked about them minutes later. Quite remarkably, this patient is able
to complete surgical procedures without a single tic or other Tourette
related syndrome, as long as nothing interrupts the procedure, such as
other people entering the OR, etc. Since many patients suffering form Tourette
Syndrome are symptom free when in situations that involve a stream like
flow of consistence, for example driving down an open road or listening
to soft flowing music, it is assumed in this case report that while A is
involved in a surgical procedure, A is involved in a take over of the skills,
feeling, and entire neural engrams in the brain, redefining the person
as long as this activity or performance lasts. Patient A reports "forgetting"
he has Tourette's while involved in a surgical procedure. This patient
has learned to deal effectively with the above listed behaviors. A reports
that he does feel impaired by the more darker sides of this disorder, such
as the feeling of rage and violence that will occasionally wave over him.
The patient describes the experience as "an epilepsy of the subcortex,...a
thin line of control between the person and the raging storm..." A
has experienced outbursts of rage and violence, his home and office are
revealing of this with evidence of punches, kicks, etc. on the walls. Despite
this behavior, A reports it has not led to any real disturbance in his
daily routine.
Nervous System Change: As stated above, Tourette Syndrome must be
examined from an integrative viewpoint, considering psychological and chemical
factors. However, it has been found that Tourette Syndrome does have a
relation to an imbalance of the neurotransmitter dopamine in the brain.
Family or Physician Reflections: This case report was originally
written by a neurologist who had the opportunity to observe patient A in
A's professional and personal life. The physician noted that A's symptoms
in many cases did have a positive side, making A more meticulous, exact,
and investigative-useful attributes for a surgeon. The physician also noted
the patients adaptability to the disorder, as well as the acceptance A
received from colleagues and friends. A's family is well accustomed to
the patient's symptoms. They do not report any disturbance in the families
function due to A's condition.
Reactions to the Case: This case report was informative about Tourette
Syndrome and gave a good illustration of patient A and how having the disease
affected A's life in all aspects. I expected more of a clinical or physiological
description of Tourette's, but the lack of this may be due to the integrative
definition needed to describe this disorder.
Anton's Syndrome in Transient Cortical
Blindness
Jennifer A. Willey
Source: Goldenberg, G., Mullbacher, W., & Nowak, A. (1995). Imagery
without perception- A case study of anosognosia for cortical blindness.
Neuropsychologia, 33(11) 1373-1382.
Patient Background: H.S., a 46-year-old woman with eight years of
schooling, suffered a bilateral posterior cerebral infarction (stroke)
due to the blockage of the tip of the basilar artery and a cardiac embolism
was suspected.
Behavioral Change: H.S.'s stroke has left her blind with limited
mobility due to tetraspasticity (spastic responses in all four limbs) and
ataxia, most evident in the lift limbs and trunk. She also developed Anton's
syndrome, in which the patient doesn't simply deny their blindness but
positively report visual perceptions. H.S., contradicting the medical evidence
of her blindness would often report that sometimes she could see, though
that the sight was unreliable. She never saw things that she didn't believe
were unreal or imaginary but everyday things like cups and tables, which
she would try to grasp but it would not be where she expected it to be.
She felt that the greatest problems in her daily life were her immobility
and her bad sight' and that she had little hope of ever regaining normal
vision. While her mobility improved over the next three months, her blindness
remained unchanged, however within the two months following her husband
reported that she now actually saw things. H.S. reported that her vision
had improved, that "she could describe [objects] and reach them with
her hands." Upon examination it was found that she had recovered a
narrow band of sight. Never once after her accident did H.S. doubt that
she could see and this may be due to actual visual memories. Now that she
has recovered a small proportion of her sight she denies sight of any object
outside her visual field.
Nervous System Changes: MRI scans reveal that the stroke caused
extensive and severe brain damage and atrophy in H.S. The destruction of
the calcarine cortex is nearly complete with fragments left on the left
side of the brain in the occipital tip. The right side, however, is also
atrophied in the parahippocampal and the temporo-occipital gyrus (Brodman's
areas), and there is a lesion on the lateral thalamus on the same side.
Family or Physician Reflections: My case report is from a professional
journal so that there is no real personal connection to the person. However,
there are reports from family members on H.S.'s progress in adapting her
visual behavior and her husband was the first to notice that she could
indeed see after five months.
My Reactions to the Case: It is very hard to empathize with a person's
condition with the cold clinical description, however H.S.'s case is appealing
because of her tenacity of belief in the fact that she could see.
Symptoms of Stroke
Chuck Bortscheller
Source: May, Sarton. After the Stroke. W.W. Norton
and Company, New York and London. (1988)
Patient Background: Mary Sarton was an author and a poet
before she had her stroke. She had authored over a dozen poetry books,
eighteen novels, nine nonfiction works, and two children's books. On the
20th of February, at the age of 73, she awoke in the middle
of the night complaining of numbness in her left arm. She called for an
ambulance and was admitted to the hospital that night. After six days of
tests, a CAT scan showed a small hemorrhage of the brain. Two months earlier
she had been diagnosed with congestive heart failure.
BEHAVIORAL CHANGE: Most of her psychological changes came
about due to the fact that her left side was weakened and she could not
perform all of the tasks she was used to doing. She would become depressed
very easily due to the fact that light household chores exhausted her.
She would at times cry due to this. At this point, she also felt not whole
for she had no poetry lines flowing through her as she had been accustomed
to all her life. Added to everything else that she was going through, was
the fact that her heart medication made her feel ill and uncomfortable.
Ms. Sarton had many people that supported her throughout this time in her
life, which helped to keep her from becoming overly depressed.
NERVOUS SYSTEM CHANGES: The case report that I am using
is the patient's own story, and does not go into detail about the actual
physiological changes that took place. All that is really revealed is that
she suffered from a minor hemorrhage on the right side of the brain. There
did not seem to be any major damage for Ms. Sarton was able to use her
left arm and leg again after recuperating from the stroke.
FAMILY OR PHYSICIAN REFLECTIONS: Once again, this study
is actually a journal kept by the patient, so it does not include any thoughts
by the physicians that treated her. It does talk about the kindness of
her friends that helped her with her recovery by doing simple household
chores and supplying meals at times. Besides May's own feelings about what
happened to her though, there are no other reflections in this book.
MY REACTION TO THE CASE: While this case lacked in neurological
and physiological information, it more than made up for it with the personal
feelings of the patient. This journal allows us to see more than just "what
happened" to her. It allows us to understand the emotions and feelings
of someone who has gone through something that many people go through,
yet can be hard for someone else to comprehend the effects of. This will
allow us to be able to better understand and treat patients who suffer
from mild strokes, for we will be more familiar with some of the emotional
symptoms that can be associated with this disorder. I feel this is a great
example of a woman who never gives up and uses the tragedies that she encounters
to make her stronger.
Encephalitis Lethargica
Michelle Laird
Source: Sacks, Oliver. (1995). Rose R. Awakenings. Patient Background:
Rose R. was born in New York City in 1905. She was the youngest child in
a large, wealthy and talented family. Rose was full of spirit and had an
intense love and affection for her family. She did not experience any neurotic
problems or identity crisis while growing up. However, one night, at the
age of 21, Rose became trapped inside her own body, never to fully return.
Behavioral Changes: The night the change occurred, rose was plagued
with nightmares of being trapped. The next morning her family had trouble
waking her and once they did, she showed little response. For the rest
of her life, Rose's symptoms were variable. At first, Rose was only able
to speak short sentences and make sudden movements before she would freeze
up again. She seemed alert to what was going on about her and always kept
her affection for her family. After three or four years, Rose became rigid
on her left side and developed many other signs of Parkinsonism. She had
more and more trouble with balance and walking. Rose would sit for hours
on end without expression on her face. She drooled and sweat profusely,
and her skin became very oily. Rose could only whisper a little and became
unable to move her tongue much. Her legs became hypertonic with the left
foot bent inward. Tremors would sometimes develop in her right arm, head,
lips, and tongue. Rose was most likely to talk in times of severe crisis
where she would cry out, "Help me...I'm in terrible pain...I'm frightened...I'm
going to die!" At the age of 64 she began to be treated with L-dopa.
This drug would help for a few weeks, but in a short time, the disadvantages
and reactions it caused outweighed the benefits. During times of lucidity
she had an outstanding memory of the year of encephalitis onset and would
often pretend she was still living in that time period, although she knew
how much time had actually gone by. Rose didn't age much. At 64 she still
looked 30 years younger. In the end, she could only be roused out of her
trance-like state by external stimuli. For the most part, she reported
that usually there was absolutely nothing going on in her head.
Nervous System Changes: At the time, there were no brain scans performed
on Rose. However, it is obvious that some of the motor pathways were interrupted
through her tremors, rigidity, hypertension, etc.. Her memory did not seem
to be affected.
Physician Response: The physician pointed out that during her last
years, Rose was best when visited by her family. When one would see her
at these times, he/she could see that Rose was really a normal, charming
person who was imprisoned by the disease. She didn't actually live most
of her life. In fact, he reports that in conversation with Rose, she remarked
that she still felt like she was 21 years old because she had never experienced
anything else. My Reactions: This was a very scary story to read.
I can't imagine what torture Rose must have gone through. Although for
the most part she thought absolutely nothing, it is apparent that during
some of the bad times she was aware that she was pretty much trapped inside
of her own body and could not express herself. She missed out on the majority
of her life and could do nothing to help herself.
Rolando P.
Jennifer Jewell
Source: Sack, Oliver. Awakenings. New York: HarperCollins Publishing,
1990. 116-128.
Patient Background: Rolando P.'s infancy and early childhood was
very normal. He showed no signs of any dysfunctions or incapabilities.
In fact, Rolando P. was excelling in motor and speech. At 30 months of
age he suffered from encephali- tis lethargica. This illness lasted for
about 18 week and forever changed Rolando's life.
Behavioral Change: After Mr. P's bout with encephalitis lethargica,
he suffered from severe motor dys- function, the inability to communicate
through speech or facial expressions, as well as excessive salivating.
Many felt his mental ability had also been diminished, but his mother argued
that he was still as bright as he was before he became ill, now he just
couldn't express it to others. In early childhood, Rolando attended classes
for the mentally challenged, but this became too difficult for Mr. P because
of his inability to sustain his balance. He was helpless without the support
of someone else. From then he was at home with his parent through his adolescent
years. His parents then admitted him to a hospital where Mr. P's limb became
more rigid and his speech was more inaudible. The rigidity was slightly
decreased in a left-sided chemopallidectomy operation. Even after the opera-
tion, Mr. P's speech remained predominately inaudible and the rigidity
in the right side of the body persisted. Mr. P was started on L-DOPA in
an effort to reduce some of these symptoms. By the first week, the results
were evident. His motor capability increased, he was now able to walk with
very little assistance. The rigidity in his limbs, trunk, and neck were
almost non-existent, the drooling had stopped, and he was able to communicate
audibly. Mr. P also developed sexual urges. These positive results were
short lived. By the second week on the medica- tion, there was an onset
of akathisia. Thus his L-DOPA dosage was reduced. His akathisia was now
specific which affected his right leg and a forced chewing movement. His
libido continued to be strongly present. Mr. P increasingly became more
hostile, irritable, and anxious. He found his eye to become fixed by moving
object and he could not stop staring at the object until it was removed
from his field of vision. Once again his L-DOPA was reduced. The excitomotor
syndrome continued and haloperidal was added to his medication list. Mr.
P's Parkinson's symptoms began to overshadow his excitomotor states. He
continued to fall into sleepy states whether L-DOPA was increased or decreased.
These states were only suppressed when in the presence of his family and
while he was away from the facility. When he was returned to the facility
the sleepy/depressed states resumed.
Nervous System Changes: Mr. P's nervous system was very unstable.
All of his behavior had an oscillatory, all-or-none quality that the doctors
could not correct with medication. They tried to correct his inability
to be mobile and communicate with an operation and the use of L-DOPA. L-DOPA
did help these symptoms to a certain extent only to onset excitomotor syndrome.
Family or Physician Reflection: Mr. P's mother played a very important
role in his life. From the beginning she stood by him, not labeling him
an invalid. She visited him weekly while she was well and it was during
these visits when Rolando showed better mental and physical signs. When
his mother passed away, Rolando became very depressed, having no one there
to nurture and love him. To fill the shoes of his mother was a physiotherapist.
She took Rolando under her wing and gave him the nurturance that he needed.
It was only after she was dismissed from her position at the hospital and
was no longer able to see Rolando that he gave up the will to live. He
gave up all hope, knowing that there was no one left to care about him
and love him the way his mother and the physiotherapist could. Your Reaction
to the Case: With Rolando's situation, it is sad to see how easy it would
be to give up on someone because of their lack of ability to communicate.
Rolando was lucky enough to have a loving and caring family and doctor
that never gave up on him. Despite some of the side effects of the L-DOPA,
it did make dramatic changes in his life. It allowed him to be mobile and
allowed his speech to be audible. Medicine can only do so much for a person,
the power of love can be the cure for all. In the presence of his family,
Rolando's symptoms seemed to prac- tically disappear. Feeling loved gave
him the will to go on and con- tinue fighting for his life. When those
people that cared for him were gone was when Rolando gave up hope. I believe
that without hope there was little that science could do for Rolando.
Reminiscence
David Sivesind
Source: Sacks, Oliver (1995). The Man Who Mistook His Wife For a
Hat. 125-142. Patient Background: Mrs. O'C, besides some deafness,
was a relatively healthy woman in her eighties living in an old people's
home. One night she had been dreaming of her childhood in Ireland. When
she woke, she continued hearing the Irish music, although no radio was
playing. She thought the music might be collecting like radio signals in
her dental fillings. This was not the case. She consulted a nurse, an otologist,
and a psychiatrist, none of whom could find an explanation. Finally, the
author, a neurologist, was consulted in the case. Mrs. O'M, also a woman
in her eighties at the same home, later came to the author with similar
concerns. The woman complained of hearing several songs. Sometimes, she
heard several songs at once. Her songs began one day when she was grating
parsnips in the kitchen. She heard several church hymns in rapid succession.
Mrs. O'M expressed considerably greater distress from hearing the music
than Mrs. O'C.
Behavioral Change: Continuing from the night described above, Mrs.
O'C continued to experience an "ocean of sound" both during sleep
and waking hours. The songs were all Irish songs from her childhood. As
the days progressed, the music continued to become louder and more prominent.
Tests showed that she was not experiencing any deafness or loss of hearing.
She was bright and alert, but was described as appearing remote and self
absorbed. All indications were that Mrs. O'C was a completely normal 88
year old woman the day before she began hearing the music. After four months,
the songs were gone. Mrs. O'C was relieved that the songs were gone, but
expressed missing the songs. She felt that the experience had brought back
a piece of her childhood that was lost before the incident. She felt that
the experience, although unnerving, had been a sort of healing process
for her. Mrs. O'M was a similar case with slight differences. She had heard
sound in her head for four years before telling anyone of her symptoms.
She was somewhat deaf. She heard music in her head, sometimes with ringing,
hissing or rumbling sounds. She sometimes heard distant voices, occasionally
many at once. Mrs. O'M's experience was significantly more rattling. The
songs she heard did not hold any special meaning for her. Thinking of the
songs would bring them into her mind. Avoidance of thinking of the songs
would bring them into her mind. She came to hate the songs. She could hear
several songs or sounds at the same time. Her condition progressed and
worsened over the next year. At times, the music became so loud that she
was unable to hear conversation. Mrs. O'M was finally treated successfully
by the use of anticonvulsants. She did not miss the songs. Nervous System
Changes: The concern expressed by both patients was dubbed a sort of
'Musical Epilepsy'. It is believed that the epileptic seizures occurred
in the "reminiscence" portion of the brain. EEG's confirmed that
both patients had experienced temporal lobe seizures during the time that
they experienced the music in their heads. The songs experienced by both
women did appear to have some psychological significance to them. While
it is obvious that Mrs. O'C was experiencing songs she had recalled from
a pleasant childhood, it was only late in treatment that Mrs. O'M revealed
that she was apt to hum the songs she later heard in her head before she
began to hear them in her head. Mrs. O'C's symptoms disappeared without
physiological treatment. Her experience appears to have had the effect
of a psychological healing, what the author dubbed "nostalgia".
Mrs. O'M was successfully treated by anticonvulsants which halted her temporal
lobe seizures.
Family or Physician Reflections: There was no mention of reactions
from the families of either of the women. However, the author/neurologist
expressed personal insights into the cases. He commented that the cases
could be considered at the same time both from a physiological and psychoanalytic
framework. He saw both women's experiences as "reminiscence".
That is, an experience that was both meaningful physically and nostalgically,
the workings of experiential memory.
Reaction to Case: It is very interesting that very similar neurological
cases can be experienced so differently by individual patients. While the
neurologist was able to determine the physiological cause of the songs
experienced by both women, it is intriguing that the psychological importance
of the songs was directly related to how the women experienced their symptoms.
The Man who Fell out of Bed
Magen Swarbrick
Sources: Sack, Oliver (1985). The Man who Mistook His Wife For a
Hat. P.53-55. Kolb, B. & Whishaw, Ian Q. (1990). Fundamentals
of Human Neuropsychology. p.424-427.
Patient Background: Patient C was admitted to the hospital for tests.
This young man had no complaints, but the neurologists, feeling that he
has a "lazy" left leg should come in for some tests.
Behavioral Change: All day Patient C felt fine, no complaints. He
seemed very nice and normal until he awoke from a nap in the evening. Immediately
after awakening he felt fine until he moved in his bed. Recalling that
there was another leg in bed with him, a severed human leg. As he went
to throw the leg out of his bed, he came with it. He now lay on the floor
refusing to get back into bed. In confusion, he could not recognize that
it was his own leg that he had felt in bed nor could he not understand
where his left leg had gone.
Nervous System Changes: Patient C is suffering from sensory neglect.
According to Kolb's & Whishaw's text and Dr. Walsh, further testing
on Patient C would indicate that he is suffering from a lesion on his right
parietal lobe. Patient C is portraying a classic symptom of contralateral
neglect. This involves the neglect of part of the body or space contralateral
to the lesion. Family or Physician Reflections: My case report comes
out of a book written by Oliver Sack. There is no information included
about the patient's family, where he lives, or any other personal background
information.
Reaction to the Case: There was very little information given about
this patient and no diagnosis, which made it difficult to understand the
case. However, looking to other sources I was able to come up with a prognosis,
and realizing that this would be a difficult injury/disease to live with.
A follow-up of Patient C and coping with this disorder would definitely
make for and interesting read.
Embolitic and Thrombolitic Cerebrovascular
Accidents in Two Individuals
Janelle K. Chaney
Source: Klawans, Harold (1990). My Mother's Best Friend. Newton's
Madness, 7-18.
Patient Background: Patient e was discovered by his wife lying on
the bathroom floor unable to speak or move his right side. At the age of
72, he had suffered a cerebrovascular accident (CVA), commonly referred
to as a stroke. This resulted due to a thrombosis on the left side of his
brain. Patient B had a history of rheumatic heart disease with abnormal
heart rhythms. Upon waking in the morning, her left leg had "given
out" when trying to stand. An embolus caused by a blood clot in the
heart had induced a CVA in B as well.
Behavioral Change: Patient E experienced partial paralysis of his
right side (hemiparesis) and the inability to speak or understand language(aphasia).
His eyes tended to look to the left as well. The cerebral thrombosis had
blocked an artery in the brain causing the stroke resulting in these symptoms.
Only three days after the stroke, E was speaking well enough to be understood
and was walking with a cane. Patient B's symptoms were more severe. B suffered
from a total left hemiplegia with no muscle tone. Although B's speech and
memory were unaffected, she did exhibit severe agnosia involving all forms
of sensation including touch, pressure and vision. Agnosia means "without
knowledge" and more specifically being unaware of her body parts.
When asked to identify her hand, B was unable to recognize it. B also exhibited
neglect, meaning that the left half of the world did not exist for her.
Patient B's condition never improved significantly. She was unable to walk,
dress herself, or even read because of her unawareness of the left side
of herself and other objects. Nervous System Changes: Patient E's CAT scan
did not reveal a large stroke and only the neurons in the center of the
stroke were unable to recover. As the other cells regained their function,
E's condition improved. Patient B's CAT scan showed that she had experienced
a moderate-sized stroke involving the motor strip and much of the parietal
lobe. Although the stroke was not massive in size, it was massive by location.
Family and Physician Reflections: In this case there is a close
personal relationship between Patient B and the physician. Behavioral changes
in B were much easier to see because of this relationship prior to the
stroke. Although it was hard to accept the severity of this stroke, the
physician knew that little could be done to help B's condition. Since Patient
B was the focus of this case, little extra attention was given to Patient
E.
My Reaction: This case illustrates two different types of CVA's
and the very different consequences that arise. Working with stroke victims
is a large part of what I do in physical therapy. I felt that this case
did a good job of presenting both the physical and emotional aspects associated
with strokes.
Dystonia, Ben Hogan's Downfall
Holly S. Barberra
Source: Klawans, H. L. (1996). The bantam: Ben Hogan. In Why Michael
couldn't hit; and other tales of the neurology of sports. (pp. 83-108).
New York: Freeman.
Paitent Background: Ben Hogan was the greatest golfer in the history
of golf. He was recognized as one of the people who put the sport of golf
"on the map". At the age of 37, Mr. Hogan and his wife were victims
of a car crash that left Mr. Hogan with a fractured pelvis, collarbone,
ankle and several broken ribs. He was able to recover remarkably well,
and was back on the tour by 1950. Though the accident was not the cause
of his dystonia, his will to overcome any impairment took its toll on his
nervous system. His constant, seeminly never-ending drive to perfect his
posture and to control his movement taxed his extrapyramidal system. His
first symptoms were seen at the U. S. Open in Rochester, his finger pulled
away from the club, disrupting his controlled posture, causing him to miss
the putt and lose the open. This loss of control has been termed the "yips"
and has been associated with "oldtimers" in the game. Mr. Hogan's
yips, like many others, does have a neurological basis.
Behavioral Changes: Mr. Hogan's golf game deteriorated from that
point forward. His spasams increased until he was no longerable to play
golf. Mr. Hogan's memory and cognitive abilities were not affected. He
was suffering from a severe case of the yips. His ability to hold and maintain
his posture and fine movement continued to occur after he had been playing
for some time usually resulting in blowing the winning shot. As a result,
he gave up golf. Nervous System Changes: The body is controlled
by three systems. The pyramidal system which directs movement, the cerebellar
system which provides synergy and coordination, and the extrapyramidal
system which creates and maintains postures. It was this third system that
failed for Ben Hogan. He lost the ability to control his posture in order
to make the swing. His extrapyramidal system short circuts when he needs
it most. There were no tests to be run on Mr. Hogan to make a medical diagnosis
at that time. The yips have been viewed as a natural occurance in aging
golfers. Dr. Klawans research discovered the connection and breakdown of
the systems years after Mr. Hogan had retired.
Family or Physician Reflections: There are no reports about Mr.
Hogan's family or his physicians reflections regarding his condition. The
author shares his own views about Mr. Hogan's conditions as more of an
annoyance than a disease. Dystonia musculorum deformans is the most severe
form and can result in disability and death. Mr. Hogan's occupational dystonia
only interfered with the types of movement and postures associated with
his occupation, golf. Reaction to the Case: Mr. Hogan's disorder,
dystonia is a common problem for many athletes. I believe that Dr. Klawans
findings will help put these athletes minds at ease. They do suffer from
a real problem. They aren't just getting old, or losing it. Their nervous
systems have been worked hard and are reacting to their years of hard work.
Although for many people, dystonia is only an annoyance, it can have a
major impact on their psychological and emotional well being. Losing ones
career is no small inconvience. Professional athletes like Mr. Hogan have
a great deal to lose. As for the amateur, struggling to make the putt will
not mean the difference between feeding their family or not this month,
it can mean ridicule and loss of self-esteem. Golf, like any sport is competitive.
But golf is a competition for the individual. He or she strives to be better
against him/herself, not just the other players.
Four Cases of Epilepsy
Elizabeth D. Williams
Source: Martin, Russell (1986). Fire At Brief Moments. Matters Gray
& White, 39-61. Wendy Stetham:
Patient Background: Wendy is a twenty year old woman. She is 33
weeks pregnant with her first child and unwed. She has a long seizure history
extending back to her childhood. Prior to her pregnancy, her seizures were
controlled with Dilantin, but the drug has recently caused a severe rash
and her seizures are becoming frequent.
Behavioral Change: During her seizures, Wendy's eyes grow white
and stare blankly, saliva pours from her mouth, and her arms and legs shake
wildly. Each of the most recent seizures have brought about contractions,
but each time they have ended shortly after the seizure. The child is still
growing and the heartbeat appears strong.
Nervous System Change: Wendy is suffering from grand mal seizures.
They start in a small, localized area but quickly move throughout the cortex.
Physician Reflections: Her physician, Ferrier, is quite worried
about the child and is considering inducing labor. While he does believe
that there is a physiological reason for her epilepsy, Ferrier thinks that
some of Wendy's seizures are "pseudoseizures" caused by a psychological
disorder.
Elliot:
Patient Background: Elliot is a four year old boy recently diagnosed
with epilepsy. There is no family history of epilepsy and no major head
traumas. He had two grand mal seizures, but since beginning treatment with
Phenobarbital he has been relatively seizure free.
Behavioral Change: His first seizure occurred while riding home
from a baseball game. He became drowsy and he slumped back in his seat.
His eyes rolled back, he became pale, and his body quivered. All the time
he held an ice cream cone upright. Upon regaining consciousness, he was
drowsy and had slurred speech.
Nervous System Changes: Elliot's EEG showed normal activity until
he fell asleep, then there was unusual activity in the right frontal region.
He also showed weak ankle reflexes and had difficulty jumping off his left
foot.
Family Reflection: His parents were extremely worried over his diagnosis
and frightened by his seizures. They felt that Elliot was taking it quite
well. In fact, he would even tell his baby-sitters what to do if he had
a seizure.
Melanie Brubaker:
Patient Background: Melanie has been on medication since childhood
for absence seizures. She also has been diagnosed with tension seizures,
which she has had since childhood also. These tension seizures typically
occurred when someone would get upset with her or when her parents fought.
Behavioral Changes: During the absence seizures, Melanie would go
into a sort of trace. She would stare vacantly and would sometimes undergo
a sudden loss of consciousness but would just as suddenly return to normal
functioning. As a child her tension seizures consisted of her rocking back
and forth screaming. In adulthood, she gets lightheaded and feels ready
to faint but she can control when (or if) she does faint. She also has
petit mal seizures that last 15-45 minutes. Her vision becomes blurry,
she has a metallic taste in her mouth, she gets weak and feels queasy,
and she feels hazy and unfocused.
Nervous System Changes: There was no discussion of CAT scan or EEG
results for Melanie. It was noted that the petit mal seizures were located
in the temporal lobe. The tension seizures are a psychological manifestation
of stress. Physician Reflection: Melanie was placed on Phenurone, Diamox,
and Tegretol. Ferrier also advised her to avoid stress, lack of sleep.
and alcohol.
Sandra Fischer:
Patient Background: Nineteen year old female college student. No history
of seizures. Her friends recognized that she had been having staring spells
for the previous week.
Behavioral Changes: Sandra started with absence seizures, but was
brought into the hospital when they had progressed. She started to loose
focus and her left leg would quiver. Each episode lasted for about 15 seconds
but they were appearing every 5 minutes. Nervous System Change: Sandra
had a normal CAT scan but had unusual EEG activity in her temporal lobes.
Physician Reflections: Ferrier started her on Tegretol. There was
a statement made about the unusual occurrence of absence seizures in an
adult. Absence seizures usually only occur in young children, but there
was no mention as to why Sandra may be having this type of seizure.
My Reactions: I felt this chapter was extremely informative. I would
have enjoyed a little more detail about the patients with perhaps less
accounts so that I could get more background and treatment information.
The most interesting though was some of the history and research of seizures
that was discussed.
Memory
loss following brain tumor
Melissa L. Staker
Source: Sacks, Oliver (1995). Amnesia
in a young man following a brain tumor. An Anthropologist on
Mars, 42-77.
Patient Background: Patient F was an intelligent boy, especially
in the area of music. However, he quickly tired of school and dropped out;
turning to rock-n-roll, and drug use, which quickly became an immense problem.
Now tiring of life, he decided to join the International Society for Krishna
Consciousness in a Brooklyn temple. He was thought to be gifted, so he
was sent to New Orleans. While in New Orleans, patient F began complaining
of impaired vision; the temple ignored his problem, stating it was because
he was merely connecting with life at another level. When his parents visited
him at the temple, they sent him to the doctor; concerned about his apparent
altered state of consciousness. It was at this point that the brain tumor
was discovered.
Behavioral Changes: Patient F’s mid-line tumor left him with severe
memory problems, not only with present day memory but also with past memories.
If told a story and asked to repeat it five minutes later, the story he
would tell would have no correlation with that which he was told. His vision
has become totally lost, however, he does not seem to realize he is blind;
but his attitude would suggest that he believes everyone sees the world
as he does. He is happy when his parents come to visit him, but does not
seem upset if they do not come for a number of days. Many years later,
his father passes away. Upon being told the news, he appears to be very
upset, but after a time forgets that his father is dead. He did not seem
to mourn until after he had a nightmare, complaining that he lost something
very important to him. While left alone he is placid, but becomes quite
lively while in other's company. He has problems remembering information
about people, so he has to break one person into two separate people. He
has problems identifying with time, yet can remember geometric truths and
procedural memory. He is slow with familiarization, but will retain it.
He has problems transferring information into his long-term memory. He
still posses a love for music and still has the ability to play instruments
and remember the lyrics to his favorite songs, but has problems remembering
new songs.
Nervous System Changes: Brain imaging showed an enormous mid-line
tumor, destroying the pituitary gland, optic chiasm, and both sides of
the frontal lobe. The tumor reached backwards toward the temporal lobe
and downward toward the diencephalon. An EEG shows an incoherent pattern;
however, it is calm and even becomes almost rhythmic while he is listening
to music.
Family or Physician Reflections: His physician feels that most of
the brain damage could have been prevented if only he had seen a doctor
as soon as he started having vision problems. His parents saw him almost
daily, yet they did not have many comments. His father does give the impression
that he is not upset about his son’s condition, mainly because his son
seems to enjoy their company.
My Reaction: I felt that there was a few things thrown in that had
nothing to do with the patient directly. This leads to the article being
choppy. There was an abundance of facts, but it did touch on some of Patient
F’s interests.
An Anthropologist On Mars, Temple Grandin,
Autistic Ph.D.
Leslie Jorgensen
Source: Sacks, Oliver (1995), An Anthropologist On Mars: Seven Paradoxical
Tales.
Patient Background: Temple Grandin Ph.D. a now successful assistant
professor in the Animal Science Department at Colorado State University,
engineer, and successful author suffers from Asperger's type autism.
Behavioral Changes: At age six months Temple started to stiffen
in her mother's arms, and at age ten months she started to claw "like
a trapped animal", which made normal contact with her almost impossible.
Temple suffered from heightened sensations sometimes at an excruciating
degree, she said "her ears were like microphones transmitting everything
at full volume. At age three she became destructive and violent and was
taken to a neurologist where her diagnosis of autism was made. Temple's
family was then told that in would be in her best interest to be institutionalized
and that she may never speak. With help from special therapists Temple
not only learned to speak, she went on to junior high and high school,
and even graduated college with a Ph. D. Miss Grandin is now an assistant
professor at Colorado State University, and with her powerful visualization
skills is the designer of the Grandin Livestock Handling Systems Inc. Although,
Temple has little interaction socially with other people due to her autistic
condition she has dedicated most of her life to her work developing and
improving cattle slaughtering and processing plants. Miss Grandin also
travels the U.S. giving lectures about autism, and her autobiography about
her life as an autistic Ph.D.
Nervous System Changes: Temple has a slightly clumsy or uncouth
gait attributed to a simple ataxia associated with impaired development
of her vestibular system and part of her cerebellum associated with autism.
She also sometimes has episodes of short spasms or fixed posture in her
arms.
Family Or Physician Reflections: The paradoxical tale I read from
was written from the author's perspective and not much was mentioned about
Temple Grandin's family or physicians. The author was amazed with Temple's
ability to understand feelings even though she has little feelings (such
as live, joy, wonder, etc.)herself. Temple also used jokes and metaphors,
which is something autistic people do not comprehend. The author was also
intrigued with the idea that on the surface Temple did not seem autistic
at all, and found her accomplishments overwhelming.
My Reaction To The Case; In conclusion I thought this was an exceptional
article. Not only did the article include information about autism, it
described how one person, Temple Grandin, overcame it to become a very
successful person. This was a very informative as well as interesting article
and I am glad I was able to read and broaden my knowledge of autism.
Korsakoff's Syndrome
Jennifer Michur
Source: Sacks, Oliver. (1985). The Lost Mariner. The Man Who Mistook
his Wife for a Hat, 22-41.
Patient Background: Jimmie was a friendly 49 year-old man born in
Connecticut who finds himself in an office with a nuerologist. The problem
is that Jimmie does not remember going there or why he is even there. Earlier,
Jimmie found himself forgetting things, especially recent events. Jimmie
graduated from high school in 1943 and then was drafted into the navy.
In the navy he was an assistant radio operator on a submarine. He remained
in the navy until 1965. In 1970 Jimmie began to drink more heavily. Around
Christmas he exploded and was taken to the Bellevue hospital. A letter
from 1971 was found from the hospital saying that he was disoriented with
a advanced organic brain-syndrome caused from alcohol. A month later he
calmed down, but still had some memory loss. He was later admitted to a
home for the aged near New York City in 1975.
Behavioral Change: When Jimmie was admitted to the home for the
aged, he talked about high school and the navy believing that the year
was 1945 when it was really 1975. During his conversation with the nuerologist
he talked in the present tense like his experiences were recent. The doctor
did a test on him by getting up and leaving the room for a few minutes.
When the doctor returned Jimie did not recall ever meeting him before.
The doctor then did some ecaminations of Jimmie and on the intelligence
testing he did very well. He was able to solve complex problems quickly
without any difficulty. Although, if the tests took too long he would forget
what he was doing. His recent memory was at great loss because he could
not even remember anything that was said or done a few seconds before.
He sometimes would remember what he had done, like playing a game with
the doctor, but could not remember if it happened a few minutes ago or
months ago. When he found out that the game was played with the doctor
he became amused, which is very characteristic of Korsakoff's Syndrome.
Jimmie was very good at math and science, just like a bright student who
had graduated from high school. In other words, he acted like he was in
the past through his feelings and by his words.
Nervous System changes: Korsakov's Syndrome is described as nueron
distruction produced by alcohol in the tiny mammillary bodies with the
rest of the brain perfectly viewed. All of the tests taken on Jimmie indicated
that there was not any massive brain damage. He was able to remember and
recognize the nurse, but thinks that he knows her from high school. His
brother is the only person that he truely knows, but cannot understand
why he looks so old. Jimmie was encouraged to keep notes everyday, but
it was hard for him to remember to take notes. Later, instead of keeping
notes, he got involved in recreation programs. By playing the games he
was able to get involved and not be so bored. Soon he perfected everything
and had nothing left to do. He liked to type so the home found him an easy
typing job. He found it challenging and to his satisfaction. Jimmie also
found continuity and unity spiritually at church, in nature, through art,
and listening to music.
Physcian Reflections: Korsakov's Syndrome is due to a alcoholic
degeneration of the mammillary bodies. Jimmie is a man without a past or
a future, and is stuck in a period of time. He was lost and how would he
ever establish roots? Jimmie always looked sad even though he never expressed
any sadness. He really did not feel anything. It was also thought that
Jimmie might of had hysterical or fugal amnesia because he only remembered
up to the year of 1945 and not to 1970.
My Reaction to the Case: I found this case to be very interesting
because I have never truely experienced what really happens to a person
with amnesia. Alcohol is used so often and by so many people that more
people need to realize how destructive it can be. Especially with the effects
that it can have on the nervous system like in Korsakov's Syndrome. It
makes me think that having a drink is not worth destroying my neurons which
could destroy my life.
Phantom Limbs
Courtney Determan
Source: Lacroix, R., Melzack, R., Smith, D., and Mitchell, N. (1992).
Multiple phantom limbs in a child. Cortex, 28, 503-507.
Patient Background: Patient K.G. was born in 1975 with a shortened
leg. The right leg was 10 cm shorter than the left leg. The foot of the
right leg was normal in size and had five toes, but the fourth was held
in flexion overlying the third toe. When K.G. was 6 years old, went to
the Shriners Hospital for a consultation. At this point, she was wearing
a knee brace that provided a 4 cm elevation to the right foot. She was
diagnosed with having a right congenital fibular hemimelia and a Symes
amputation was performed. After the amputation at age 11, K.G. was provided
with a prosthetic foot.
Behavioral Change: Patient K.G. was interviewed at 16. She was attending
high school and living with her parents and her two brothers. She was very
active at school and made use of her prosthetic leg. K.G. mentioned that
shortly after her leg had been amputated, she had felt a phantom foot where
her amputated foot had once been. This phantom foot extended forward like
her original foot and it had five toes. This was phantom #1. Shortly after
K.G. noticed phantom #1, she noticed another set of phantom toes which
appeared at the top of the stump. This was referred to as phantom #2. About
three months after the amputation, K.G. developed a third phantom foot.
This phantom included a calf which extended from the stump and lead to
a foot with a set of five toes that was coordinate with the prosthetic
limb. This phantom was referred to as phantom #3. Each phantom was very
distinct in size, length, and position in relation to the others. K.G.
related also that the phantom foot (#3) seemed to be stuck in a flattened
position which was quite unpleasant for her. K.G. experienced a wide range
of sensations such as tickling, heat, formication, and fatigue. When interviewed,
K.G.'s memories of her amputated foot were inaccurate. She recalled that
her foot was normal with five toes, when in fact she had had a right flatfoot
deformity and the foot was locked into an equinovalgus position with no
active or passive movement. This suggests that she experienced somatosensory
memories of the actual deformities without having conscious recall of them.
Nervous System Changes: Patient K.G.'s experiences with her phantoms
had persisted for ten years after her lower leg was amputated. It is proposed
that phantom limbs are generated by a widespread network of neurons (neuromatrix)
which consists of loops that are integrated in the somatosensory thalamus
and cortex, the limbic system and the association cortex. It is assumed
that this neural network is genetically determined and is responsible for
these experiences in K.G.'s body. Also, Changes in innervation due to neuroplasticity
may conceivably play a role in the multiple phantoms that K.G. experienced,
and may contributed to the fragmentation, fading and telescoping that are
observed in the natural changes of the phantom limb in adults and children.
Family or Physician Reflections: This case report was taken from
a professional journal so it does not reflect the emotions of K.G. or her
family. It does report on K.G.'s sensory experiences and the unpleasantness
of the feeling in phantom #3 which seemed to be flat and stuck in a forward
position. This report also contains a picture of a clay figure that K.G.
made which depicts her three phantoms.
Reaction to the Case: This case is remarkable because it was the
first case of a child who had a limb amputated at a young age and developed
multiple phantoms. Although there was much detail of K.G.'s physical experiences
with her phantoms, it was difficult to empathize with her situation because
there was not much account of her daily life and how she coped with her
disorder in her disorder in her everyday activities. From the vague detail
that was given, however, it appears that K.G. copes fairly well. She has
been active with her prosthetic foot, done well in school, and is a very
serious intelligent girl.
Lou Gehrig's
Disease
Jescica Lage
Source: Klawans, Harold L.. Why Michael Couldn't Hit: And Other
Tales of the Neurology of Sports. New York: W.H. Freeman, 1996,(pgs.245-261).
Patient Background: Lou Gehrig was the greatest first baseman in
the history of major league baseball and the most famous person during
his time to develop amyotrophic lateral sclerosis, a slowly progressive
neurological disease. In 1938, at the age of 35, his performance began
to decline and he began experiencing weakness in his lower limbs (especially)
followed by atrophy of the muscles of his hands. In 1939, he permanately
removed himself from the Yankee lineup, was evaluated at the Mayo Clinic
and diagnosed with amyotrophic lateral sclerosis. Twenty-five months later,
on June 2, 1942, Lou Gehrig died at the age of 38.
Behavioral Changes: My case report focused mainly on Lou Gehrig's
career and performance as a professional baseball player and how his performance
changed based on the symptoms of amyotrophic lateral sclerosis. It also
focused on defining the disease and its effects. Lou's behavioral changes
due to ALS were looked at in terms of changes in his performance and his
batting statistics on a weekly basis during the final four years of his
career. It wasn't until the 1938 season that his performance declined and
continued to deteriorate from there. His batting average declined and the
predominant weakness early in his illness was in his legs which altered
his speed. He began using his hands to push down on his legs in order to
straighten his hip joints when standing from a seated position. This is
known as Gower's sign and showed evidence of weakness in his thigh muscles.
Another factor which altered his batting performance was atrophy of his
hand muscles.
Nervous System Changes: The neurological damage of ALS on Lou Gehrig
could be seen by the progressive weakness and degeneration of his muscles
as well changes in his motor funcion (voluntary motor function). Microscopic
examinations attempted to correlate sclerosis of the lateral columns of
his spinal cord and changes in his motor function, especially that of his
lower limbs. ALS prevents the command messages for voluntary movements
from getting from the upper motor neurons of the cerebreal cortex to the
lower motor neurons that innervate the skeletal muscles and cause them
to contract. Therefore these lower motor neurons die which causes atrophy
(shrinking) of the muscles and a lack of voluntary movement-paralysis.
Family or Physician Reflections: Lou Gehrig was an inspiration to
others not only as an incredible baseball player, but as an individual
facing an incurable disease that wouldn't let it get him down. My case
report did not give much detailed information about the people close to
him, but it does illustrate how much he meant to his teamates and his fans-especially
by holding a day for him in 1939 after he permanately left the Yankees,
where thousands of fans flocked to Yankee Stadium to honor him and all
of his hard work.
My Reaction to the Case: Case reports are especially important when
trying to understand both the physical and psychological effects of disorders
and diseases. In order to make a proper diagnosis, it is important to understand
both aspects of the human being. I enjoyed reading this case report because
it was not as dry and impersonal, and filled with medical jargon I couldn't
understand as many case reports are. I could actually understand the definition
of the disease, the symptoms and its effects, which made it that much more
interesting.
The Colorblind Painter
Sheila Parks
Source: Sacks, Oliver. (1995). An anthropologist on mars: Seven
paradoxical tales. New York: Alfred A. Knoff, Inc.
Patient Background: Jonathan I., Mr. I., was a successful artist.
A painter whose colors play a major role in life. At the prime age of sixty-five,
he was involved in a car accident and suffered a concussion. Through an
eye examination, Mr. I could not distinguish letter or colors. The letters
appeared to him in Greek although they were written in English. Within
days he could distinguish letters, but he could not see any colors only
black, white, and shades of gray. He also suffered transient amnesia. He
completely forgot he was in an accident.
Behavioral Changes: Mr. I. changed socially, emotionally, and behaviorally.
He could no longer stand social or sexual encounters. People were no longer
flesh-colored but rat colored. He also could not distinguish the colors
in traffic lights and ran right through them while driving. He closed his
eyes to eat foods, because they seemed abnormal. He would stare at an orange
wanting to force it to show its color. Finally, his diet only included
naturally black and white foods like black olives, white rice, yogurt,
and black coffee. He could no longer dress himself. His wife would set
out his clothes for the following day, but he became annoyed. Finally,
they categorized his belts, ties, and other articles of clothing with labels.
Since he was a successful painter, he was hurting emotionally. His way
of life was forever changed. He knew the exactness of color, but could
no longer imagine the sight of colors. His visual imagery was colorless.
His colorful paintings of flowers were now black, white, and shades of
gray. He even dreamed in black and white. He longed to see color anyway
he could. He tried pressing on his eyeballs, like staring into the light,
to see any blotches of color. His career took a downfall. He tried painting
again with color, because he knew the exactness of mixing paint. But only
when he was shown a black and white photograph of his new paintings, could
he see the confusion others seen. It completely frustrated him. His was
depressed when he visited galleries. His favorite paintings seemed washed
out. He created his own room in black, white, and grays to portray to others
what he was experiencing. It included a elaborate dinner with tables and
chairs. However, people could never understand the degree to which Mr.
I. was living. His world wasn't like a black and white television although
he wishes it was that simple. It was a different tonal and contrast scale,
and completely three dimensional. He was forced to construct a new life.
He began painting in black and white almost eighteen hours a day. He started
to become a "night person" later in life. At night, it seemed
normal for things to be dark. He could also do more than others at night
like read a license plate from far distances.
Nervous System Changes: The doctors revealed he suffered cerebral
achromatopsia, colorless vision that was uncontaminated by additional defects
of perception of form, motion, or depth. He was administered many tests
like the MRI and Cat scans, but technology in 1986 did not show any dysfunctions.
They tested him for color anomia, failure to recall the colors of familiar
object like a banana is yellow, but the results were negative. Mr. I. was
also a smoker which can cause dimming in vision and sometimes achromatopsia,
but that was due to effects on the cells of the retina. Mr. I's problem
was cerebral from the concussion or a small stroke before or after the
accident. Intriguingly, mechanisms of neural construction of how the brain
transforms atoms into colors has been researched. Neurologist believe the
damage was around the V4 area of the secondary visual cortex. It may have
been reductions of oxygen to this area or carbon monoxide poisoning to
this area which are known for changes in color that caused his deficit.
However, because of his loss of memory, imagery of color, and dreaming
in color, other places in the brain must play a role. The hippocampus plays
a role in memories, so some damage may have occurred there.
Family and Physician Reflections: The Physicians in charge of this
case were intrigued by its magnitude. Achromatopsia occurring to anyone
can be devastating. Moreover, their client was a painter whose life revolved
around many shades of color. Some cases of acquired cerebral achromatopsia
improve although this was not the case for Mr. I. His wife was not really
included in the discussion. However, Mr. I. commented how others could
never experience the wholeness and strength achromatopsia affected his
life.
My Reaction to the Case: I can not imagine how devastating life
would be without color. I am affected enough when the sun doesn't shine
for a week, and the winter grays set in. As the title of the book explains,
it is a paradoxical tale.
Myasthenia Gravis in
a NFL player
Teresa Parks
Source: Klawans, Harold L. (1996) Why Michael Couldn't Hit: And
Other Tales of the Neurology of Sports. Washington D.C.: First Printing.
Patient Background: Dwight Jameson (not his real name) is a six
foot, one inch, 195 pound NFL football player. In his senior year the onset
of myasthenia gravis began with the droopy eyes.
Behavioral Background: Myasthenia gravis had many behavioral effects
on Dwight Jameson. One effect was the weakening of his eye muscles. This
weakness caused him to have two problems. One problem being ptosis, drooping
eyes caused by the weakening of the muscle that lifts up the upper eyelid.
This made Dwight Jameson look sleepy. The second problem with his eye muscle
caused to have double vision (diplopia). So when he went out for the long
touchdown pass he saw two footballs, so he couldn't catch it. This inability
to see the long bomb had detrimental effects on his playing ability. This
in turn, caused him to play only on the special teams, although he referred
to himself as being a safety. Another behavioral change the doctor noticed
was that he slurred his words after exercising.
Nervous System Changes: Myasthenia gravis means "grave weakness
of the muscles". It is a disease of the neuromuscular junction. The
neurotransmitter, acetylcholine (ACh) which initiates the muscles to contract
and has a "growth stimulating function". Acetylcholinesterase
is an enzyme that destroys ACh. Acetylcholinesterase inhibitors prolong
the action of ACh to reverse the weakness. One such inhibitor is Tensilon.
When the physician had Dwight Jameson exercise he became fatigued and began
to get droopy eyes. When the doctor gave him Tensilon the symptoms disappeared.
However, too much ACh can have the same effects as too little. Myasthenia
gravis is also an auto immune disease. The patient produces antibodies
in the thymus gland that reacts with the acetylcholine receptor. The doctor
measured Dwight Jameson's level of acetylcholine receptor antibodies and
found that it was above normal. Likewise, through a MRI scan they found
the thymus gland was enlarged, which is common in patients with myasthenia
gravis. A chest surgeon removed his thymus gland. Dwight was also put on
immunosuppressant therapy to decrease the auto immune response. After having
treatment, Dwight returned to being a free safety and he no longer was
called "First Half Jameson".
Peer Reflections: Dwight Jameson had a reputation of letting the
"big play" slip through his fingers in the fourth quarter. He
acquired a nickname "First Half Jameson" because he played terrific
in the first half, but not in the second. In college, his nickname was
"sleepy Jameson" because his eyes were droopy.
My reaction to the case: I found this case on myasthenia gravis
to be very interesting. It explained this auto immune disease very well.
"First-Half Jameson" is an excellent example of a person with
this disease. His nickname even gives a clue to how myasthenia gravis affects
a person. Being a football fan, I understood this more on a personal level.
I could see the impact of this disease clearer, compared to the basic text
in a neurology book.
Parkinsonlike Disease
Rachel Fazio
Source: Klawans, Harold (1992). Such is Fame. Life, Death &
In-Between, 12, 121-131.
Patient Background: Roberta Rose Richards lived in Muncie, Indiana
and often had to travel to the Mayo Clinic in Rochester, Minnesota for
treatments for what physicians there had diagnosed as Parkinson's disease.
Fifteen years earlier, she had a thyroid gland removed due to a hyperthyroid
condition. When she was being treated for Parkinson's disease at the Mayo
Clinic, she had taken three grams of levodopa (transforms into dopamine)
a day for four months and had no improvement or side effects. After having
received medical care at the Mayo Clinic for fifteen years, Roberta decided
it was becoming too difficult for her to commute that far, so she was referred
to a doctor in Chicago. The doctor in Chicago had her go to an endocrinologist
to rule out other possible causes of her symptoms; the results showed no
signs of hypothyroidism. She began to take increased doses of levodopa
(up to twelve grams a day) and still showed no improvements and her symptoms
were getting worse. Her doctor took her off levodopa and put her on an
experimental drug, bromocriptine (acts as a dopamine replacement). Her
dosage started at two and a half milligrams a day, and slowly increased
to one hundred sixty milligrams a day, and still she showed no improvements
or side effects. Roberta was taken off bromocriptine and given another
experimental drug, lergotrile (a second cousin of bromocriptine). She showed
no response to lergotrile and her symptoms were still worsening. Roberta
was taken off her medications and had more tests run including another
examination by an endocrinologist, blood tests, and skull X-rays. They
all came back normal. A spinal tap was performed on her and the results
indicated her HVA level was 164-168,which was within the normal range and
helped rule out Parkinson's disease because Parkinson's patients' HVA levels
are well below normal. The doctor was able to diagnose Roberta with hypoparathyroidism
after he ran a CAT scan (the CAT scanner had just been invented at the
time). Roberta was given treatment for hypoparathyroidism which slowed
the progression of the disease, but could not fix what had been damaged.
Behavioral Change: Roberta had Parkinsonlike symptoms, which included
slow and slurred speech, a shuffling walk, tremors, unbalanced posture,
and an expressionless face. As her symptoms worsened, she tried to use
a cane to aid in walking, but it did not help, so she began to use a walker
instead. Roberta was no longer able to do many self-care or daily activities
on her own. For instance, she was unable to get out of an easy chair herself,she
needed assistance with getting dressed, and it took her more than ten minutes
to cut her own meat. She felt she no longer had a sense of well-being.
She missed her sense of well-being and very much wanted to try everything
possible, such as having tests run or trying new medications, to try to
get it back.
Nervous System Changes: Roberta's HVA level was 164-168,which was
within the normal range of 60-180, unlike that of Parkinson's patients
whose HVA level is usually well below 40. The normal HVA level indicated
that her dopamine level was also normal. Results from a CAT scan showed
massive calcium deposits within each half of her brain around the area
of each striatum (a major motor area that is acted upon by dopamine). Her
striatum had been invaded by calcified tissue which had destroyed the dopamine
receptors. Since her striatum could no longer respond to dopamine, she
had developed Parkinsonlike symptoms. The striatum disorder was diagnosed
as hypoparathyroidism, which possibly developed due to a complication from
her thyroid surgery.
Family or Physician Reflections: The chapter that this case report
is based on does not discuss any reflections from Roberta's family or friends.
The author of the chapter was her doctor in Chicago and reflects his viewpoint
very well. He was very determined to find a treatment that would help Roberta's
condition and when nothing was working he did everything possible until
he found the true diagnosis.
Your Reaction to the Case: This case presentation gave a well defined
description of the steps taken and the determination that was involved
by both Roberta and her doctor as they worked to find the true diagnosis
for Roberta that would allow her to receive more affective treatment. This
case also reflects the importance of the CAT scan. The CAT scan allowed
Roberta's doctor to confidently diagnose her with hypoparathyroidism.
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