Neuromuscular Disease

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Cerebrovascular disease
A 68-year-old woman with a one-month history of nondescript aching and stiffness in
her shoulders and intermittent fever, both responsive to Extra Strength Anacin, comes
to your office concerned that she has developed a severe, right frontal throbbing
headache and while reading this morning’s newspaper became aware that something
was wrong with the vision in her right eye. On examination, she is blind in her right eye.
1. The most likely diagnosis is:
2. How would you confirm your diagnosis?
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A 65-year-old previously healthy housekeeper awakens from sleep unable to stand or
walk. On exam, she has moderate-to-severe left leg and foot weakness, mild left arm
weakness, is unable to identify numbers drawn over the dorsum of her left foot, but can
identify them when drawn over the dorsum of her right foot or palm of either hand.
There is no language deficit or visual field loss. The left plantar response is extensor.
1. What is your diagnosis?
2. Where is the lesion?
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A 65–year-old man drove himself to the emergency room after experiencing a suddenonset ten-minute episode of right hand weakness and word-finding difficulty. He had
experienced similar episodes over the last month but this one had lasted considerably
longer. In the ER he tells you he has had a long history of hypertension and diabetes for
which he takes medications regularly; he takes no other medications. He had a cardiac
stent implanted a year ago following an evaluation for unstable angina. His neurological
examination is normal except for a mid-calf stocking neuropathy with diminished deep
tendon reflexes at the ankles.
1) Where would you localize the brain lesion that caused this man’s symptoms?
What vascular territory?
2) What are the risk factors for stroke?
3) What studies would you suggest are appropriate in evaluating this individual’s
problem?
4) How (or) would you treat this man’s presenting problem?
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Case Study #1: Patient BF
A 52-year-old, right-handed woman with medical history of hypertension awakened
from sleep at 2330 hours with the urge to urinate. She arose from bed and walked to the
bathroom adjoining the bedroom in her home. As she was walking, patient BF spoke to
her husband who remained in the bed that she left. At 12 midnight (0000 hours), her
husband was startled to hear the sound of the patient collapsing behind the closed door
of the bathroom. He found his wife lying on the floor of the bathroom, unable to move
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her left side but still capable of speaking. Patient BF was transported by a paramedic
ambulance unit to the Emergency Department of a local community hospital and arrived
at 0030 hours. Upon arrival, the patient weighed 100 kg and had blood pressure of
190/110. The pulse rate was regular at 98 beats per minute. She was afebrile. Patient
BF breathed room air comfortably with oxygen saturation measured at 98% from a
cutaneous monitor. In addition to hypertension and moderate tachycardia, the physical
examination showed absence of cervical bruits or cardiac murmurs, rightward gaze
preference, a left homonymous hemianopsia, left hemiplegia, left hypesthesia to
pinprick, and an extensor plantar sign in the left foot. The patient was able to correctly
identify her left hand when it was placed by the examiner within her intact right visual
field. Computerized tomography (CT) of the brain was performed without infusion of
iodinated contrast and is shown in the Figure displayed below:
Figure: CT scan of the brain performed without contrast
enhancement in a 52-year-old woman with acute onset of
left hemiplegia. This scan was performed approximately 75
minutes after the onset of symptoms.
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Questions
1. Which of the following statements regarding the clinical presentation is correct?
A. The neurological deficit sustained by patient BF began at 2330 hours.
B. The neurological deficit sustained by patient BF began at 12 midnight.
C. Because of the patient’s body weight of 100 kg, she is not a candidate for
administration of recombinant tissue plasminogen activator (rt-PA) to treat acute
ischemic stroke.
D. Because the blood pressure is elevated to 190/110, the intravenous dose of rtPA should be reduced by 50%.
2. Which of the following interpretations of the CT scan is correct?
A. Subarachnoid hemorrhage
B. Normal calcification of an intracranial artery
C. “False falx sign”
D. “Hyperdense MCA sign”
3. Which of the following is the most appropriate choice for treatment of hypertension
experienced by this patient?
A. The blood pressure of 190/110 should not be treated with any anti-hypertensive
agent to avoid hypoperfusion of ischemic brain and further expansion of stroke.
B. The blood pressure of 190/110 is supportive of the clinical diagnosis of
hypertensive encephalopathy and should be treated aggressively to achieve
systolic blood pressure of 160 mm Hg.
C. The blood pressure of 190/110 should be treated with application of one inch of
nitroglycerin paste to the skin surface and with serial boluses of labetalol given
intravenously to lower the systolic blood pressure below 185 mm Hg.
D. None of the preceding choices A, B, and C
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Case Study #2: Patient AG
Patient AG, a 21-year-old, right-handed man with Marfan’s syndrome, underwent
replacement of the aortic and mitral valves and surgical repair of an aneurysm of the
aortic root at age 19 years. He was placed on warfarin for anticoagulation after the
surgery. Three days before admission to a local community hospital, the patient
developed headache, fever to 102oF, and left-sided weakness. He was then transferred
to an academic medical center. Physical examination upon admission revealed
Marfanoid body habitus, normal mental status, no cervical bruits, mechanical “clicks”
representing opening or closure of the aortic valvular prosthesis, left visual neglect, and
a spastic left hemiparesis of modest severity. Direct funduscopy was unremarkable. A
CT scan of the brain obtained upon admission to the local community hospital is shown
in Figure 1. Selected views from a cerebral angiogram performed after transfer to the
academic medical center are shown in Figure 2.
Figure 1: CT scan of the brain performed
without contrast enhancement in a 21year-old man with an infected aortic
valvular prosthesis who was taking
warfarin at the time of onset of left 533571450
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Questions
1. Select the only correct statement from the following options:
A. Examination of the fingers will show white, linear discolorations oriented
transversely across the nail bed.
B. Examination of the blood smear will show a microcytic, hypochromic anemia with
fragmented red cells.
C. Examination of the anterior chamber of the eye by slit lamp will reveal goldenbrown discoloration of the peripheral cornea.
D. None of the preceding choices A, B, and C
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Figure 2: Views of selective right carotid
injection (upper panel: lateral projection;
lower panel: anterior-posterior projection)
during cerebral angiogram performed in a
21-year-old man who experienced the
acute onset of left hemiparesis and
headache in association with the lesion
observed on CT of the head in Figure 1.
2. Select the most accurate statement from the following options:
A. This patient sustained intracerebral hemorrhage (ICH) within a metastatic
melanoma deposited in the right cerebral hemisphere.
B. This patient sustained ICH in the right cerebral hemisphere due to spontaneous
rupture of a congenital aneurysm at the bifurcation of the right middle cerebral
artery.
C. This patient sustained ICH in the right cerebral hemisphere due to spontaneous
rupture of an arteriovenous malformation.
D. This patient sustained ICH in the right cerebral hemisphere due to spontaneous
rupture of a mycotic aneurysm.
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A 65-year-old male went to sleep on a Thursday night in his usual state of health. He is
taking medication for high blood pressure and elevated cholesterol. He has smoked
more than 2 packs per day for 40 years. Upon awakening Friday morning he cannot
move his right side. He is alert and without headache or visual complaints.
1. What happened?
2. Where is the lesion?
3. Your diagnosis can be confirmed by…?
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Cord/root disease
A 55-year-old male with a 3-week history of back pain radiating into the right leg comes
to your office for evaluation. Abnormalities on exam include decreased sensation to
pain over the dorsum of the lateral aspect of the right foot.
1. What muscles would you expect to be weak?
2. What deep tendon reflexes would you expect to find diminished or absent?
3. The 5th lumbar nerve root exits between what vertebrae?
4. What are the indications for a lumbar discectomy?
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A 36-year-old construction worker complains of neck pain radiating into the left arm after
stacking lumber on a flatbed truck. On examination, there is mild weakness of elbow
extension, a depressed triceps deep tendon reflex, and loss of sensation to pinprick
over the 3rd (middle) finger – all involving the left arm and hand.
1. What muscles comprise the C-7 myotome?
2. Where is the C-7 dermatome?
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Dementia
A 40-year-old man with AIDS presents with memory problems. His problems date to six
months earlier when he found he had to re-read everything several times as he was
unable to recall what he had just read. His family states that he had become socially
withdrawn. Examination showed him to be very slow in both response to questions and
in movement. He had a masked face. His postural balance to threat was impaired and
his gait slow and unsteady. An MRI showed diffuse brain atrophy. No focal weakness
or sensory loss was evident.
1. List causes of dementia:
2. How do you distinguish one type of dementia versus another on clinical and
radiologic grounds?
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A 78 year-old woman visited her doctor because her husband "thought she needed to
be checked." She reported that nothing was wrong, that her husband had always been
overly protective. He reminded her that, while driving, she had become lost on several
familiar streets and that, at a recent reunion, she had been unable to recall friends.
About a year ago she had quit helping him with the bills, whereas they used to sit down
once a month and go over finances together. She takes no medications, does not use
tobacco, and rarely drinks alcohol. Her general exam is normal. She is very outgoing
and speaks fluently, but makes frequent paraphasic errors. She cannot recall any of
three simple objects, has significant difficulty drawing a simple clock, and cannot think
of the name of the current president. When told the President's name she says, "I knew
that. I thought you asked if I had voted for him."
1. Where would you localize a lesion that leads to:
a. Paraphasic errors?
b. Problems with short-term memory?
c. Difficulty constructing simple figures?
2. What are the 3 most common causes of dementia in the US?
3. What is the difference between a cortical and a subcortical dementia?
4. One should screen for what treatable diseases in a person who presents with
signs/symptoms of dementia?
5. What is the recommended therapy for Alzheimer’s disease? What is the
prognosis?
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NEUROPSYCHOLOGICAL CASE #1
Reason for Referral: A 61-year-old, right-handed, Caucasian male is referred for a
neurocognitive evaluation by a neurologist secondary to a two-year progression of
slowed cognition, memory difficulty, nocturnal disorientation, difficulty maintaining train
of thought, increased distractibility, word-finding difficulty, usage of incorrect words and
relatively intact activities of daily living independently. Patient spends his day working on
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his farm or completing projects at his home. Patient’s medical history is significant for a
REM-sleep behavior disorder, vivid dreams, chronic headaches, head injuries with brief
LOC, back pain, ulcers and orthostatic hypotension with a possible neurological
diagnosis. Neuroimaging and EEG have been normal.
Collateral Information: Patient’s wife indicates patient is occasionally unable to drive
secondary to dizziness but is otherwise able to manage activities of daily living. Wife
reports patient has fallen often in the last year with no known cause. Wife indicates
patient is disoriented and sluggish after these episodes.
Background Information: Patient completed high school and three years of college. He
denies a history of special education or learning disability. Patient worked as an
electromechanical engineer until retiring in 1995. He has been married for 37 years and
has two adult children. His adult son lives at home with he and his wife secondary to a
seizure disorder. Current medications include Lipitor, Tricor, Inderal, amitriptyline,
Nexium, Paxil, and Klonopin. Family medical history is significant for myocardial
infarction, cancer, lung disease, bipolar disorder, and unknown dementing illnesses
(grandfather and father). Psychiatric history is unremarkable. Patient describes his
mood as easygoing, and denies depression and anxiety. His wife’s description was
similar. Patient reports social consumption of alcohol and denies current use of tobacco
products. He reports a history of heavy alcohol consumption when he was in the Navy.
He describes his sleep quality as consistent. Patient indicates his appetite has
increased.
Procedures: Clinical Interview, Collateral Interview, Functional Assessments Inventory,
Memory Assessment Questionnaire, Mini-Mental Status Examination (MMSE),
Wechsler Adult Intelligence Scale-III (WAIS-III, select subtests), Boston Naming Test
(BNT), National Adult Reading Test (NART), Sentence Repetition, Token Test, Verbal
Fluency (FAS and Animal Naming), California Verbal Learning Test-II (CVLT-II),
Wechsler Memory Scales-III (WMS-III, select subtests), Wisconsin Card Sorting Test
(WCST), Ruff 2 & 7 Selective Attention Test, Trails A & B, Finger Tapping Test, Test of
Memory Malingering (TOMM), Geriatric Depression Scale (GDS).
Behavioral Observations: Patient was awake and alert and demonstrated an
appropriate level of arousal throughout the examination process. Affect was
appropriate. No thought disturbance or bizarre mentation was evident. Gross motor
functioning appeared intact. Speech was of normal rate, volume, and prosody; however,
several mispronunciations were noted. Patient’s performance on a task of effort was
good; therefore, the current evaluation is believed to accurately reflect his
neurocognitive functioning.
Summary of Test Data: Patient’s effort on testing was good; therefore, test results are
believed to provide an accurate indication of his cognition. Global mental status
indicated disorientation to time (3/5) and place (4/5), mild memory impairment with 2/3
objects recalled, and mildly impaired repetition (MMSE = 25). His pattern of
neurocognitive testing is characterized by slowed upper motor speed, slowed
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processing speed, inefficient working memory, dysfluency, fluctuating divided attention,
impaired retrieval of visual and auditory material (relative to recall) superimposed on
average intellectual abilities (FSIQ = 94). Testing was not suggestive of global
intellectual deterioration. Visuospatial skills, abstraction, problem solving, simple
attention, naming, retention and receptive language were within normal limits. Patient is
not acknowledging considerable affective distress, but mild worry about his physical
symptoms.
Question 1: Is neurocognitive testing globally consistent with a cortical or subcortical
process? What are characteristics of each?
Question 2: Is testing suggestive of a possible Alzheimer’s disease, Seizure disorder,
Parkinson’s disease (or Parkinson’s plus dementia), or dementia with Lewy bodies?
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NEUROPSYCHOLOGICAL CASE #2
Reason for Referral and Background Information: Patient is a 75-year-old Caucasian
female referred for a neurocognitive evaluation as a component of the Memory Disorder
Clinic at Sanders-Brown Center on Aging secondary to cognitive dysfunction. Patient
reports a six-month history of forgetfulness and decreased attention when driving. She
reports she has been involved in several motor vehicle accidents in the last few months.
She denies other change in ability to manage activities of daily living independently. She
currently lives alone; however, one son frequently visits overnight.
Her daughter reports a three-year history of gradual memory decline, decreased
comprehension, disorientation to time and place, and difficulty managing daily activities
including remembering dates, managing finances, and driving. She indicates her mother
frequently repeats herself and still drives locally despite directional confusion and her
involvement in several accidents.
Patient has an eighth grade education and denies a history of special education or
learning disability. She is a retired nurse’s assistant. She is divorced and has five living
adult children. Medical history is significant for hysterectomy, aortic insufficiency,
hypertension, asthma, and hypothyroidism. Current medications include Accupril,
Synthroid, Theolair, calcium supplement, multivitamin, and vitamin E. Family medical
history is significant for cancer and Alzheimer's disease. Psychiatric history is significant
for treatment of anxiety and depression. She denies current use of alcohol, tobacco,
and illicit drugs. Patient describes herself as mostly happy; however, she reports
occasional sadness secondary to financial limitations on her activities. Her daughter
indicates patient has always been very emotional. Patient reports consistent appetite
and sleep quality.
Behavioral Observations: Patient was awake and alert and demonstrated an
appropriate level of arousal throughout the examination process. Affect was
occasionally tearful and patient expressed concern regarding her performance. She
wore corrective lenses. No thought disturbance or bizarre mentation was evident. Gross
motor functioning appeared intact. Speech was of normal rate, volume, and prosody.
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Patient’s effort appeared consistent; therefore, the current evaluation is believed to be
an accurate reflection of her neurocognitive functioning.
Test Results: Testing is characterized by mild to moderate cognitive impairment with
prominent dysnomia, receptive language dysfunction, disorientation, memory
impairment, constructional dyspraxia, executive dysfunction, and moderate IADL
impairment. Global mental status on the MMSE (21) suggests mild to moderate
impairment based on disorientation to time (3/5) and place (3/5), and episodic memory
changes (3/3 immediate recall, 1/3 delayed recall). She was unable to spell “world”
backwards (4/5), could not repeat “no ifs, ands, or buts” (0/1), and was unable to
complete an oral three-step command (2/3). Testing is suggestive of a mild to moderate
dementing disorder such as possible Alzheimer’s disease with co-morbid depression
and anxiety; however, an underlying vascular component cannot be ruled out.
Question 1: a) Can the patient’s eighth grade education account for her cognitive
impairment? b) Does patient’s limited eighth grade education increase her risk for
dementia?
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A 52 year-old male develops expressive dysphasia over several months. He becomes
unkempt, disinterested and loses his temper easily. He has previously been healthy.
There is no history of neurologic disease in the family. On exam he has several frontal
lobe release signs, poor recent memory and a nonfluent dysplasia. His MR scan shows
frontal and temporal lobe atrophy.
1. Diagnosis?
2. What are frontal lobe release signs?
3. What are the common causes of dementia?
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Demyelinating Disease
A previously healthy 28 year-old female develops sudden loss of vision in the left
eye. Her visual acuity is 20/400 in the left eye. The left pupil is 2mm larger than the
right. Shining a bright light in the right eye causes a brisk, equal constriction of both
pupils. Shining the same light in the left eye causes a brisk constriction of the right pupil
but a sluggish response on the left. VER (visual evoked response) for the right eye is
normal but the VER for the left eye is delayed (a prolonged P100). The fundi are
normal.
This lady has which one of the following:
a. optic neuritis
b. papillitis
c. papilledema
d. increased intracranial pressure
e. retrobulbar neuritis
1. What is likely to be her underlying disease?
2. What might her CSF exam show?
3. What abnormalities might her MRI of the brain show?
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A 26-year-old woman presents to the ER following the sudden onset of bilateral
lower extremity weakness and numbness. She denies prior neurological symptoms, but
examination shows a right Marcus Gunn pupil and inability to read color plates with the
right eye. Temporal pallor was observed in both eyes. Her lower extremities were
profoundly weak and bilateral Babinskis were elicited. There was sensory loss to
pinprick at T10.
1. What is the difference in retrobulbar neuritis and optic neuritis?
2. What are CSF findings in MS?
3. What is your diagnosis?
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A 37 year-old bank teller came to your office complaining of a right arm tremor that
worsened with movement. She was also having difficulty counting change with that
hand and stated she "couldn't make it do" what she wanted. These symptoms had
insidiously progressed over several days and had now been about the same for a week.
She had been healthy all of her life and denied any prior neurological problems. She did
seem to remember that, as a teenager, she had experienced an episode of decreased
visual acuity OD which lasted about a month and then spontaneously cleared over
about a week. Additionally, her husband reminded her that after the birth of their first
child, she had had difficulty with micturition and had to use a catheter for about 2 - 3
weeks.
1. How well can you neurologically localize each of the highlighted symptoms?
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2. What is the most likely diagnosis for this woman? Why?
3. What additional studies might be helpful at this time?
4. Should this woman be treated? If so, what would you consider appropriate
treatment? Why?
5. If I were to tell you this was a case of multiple sclerosis, what could you tell me
about underlying disease mechanism?
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Epilepsy
A 16-year-old female with a history of simple partial seizures is brought to the ER
because of stupor. Examination reveals slow responses to simple questions, normal
vital signs and no focal neurologic findings. A urine drug screen is positive for tricyclic
antidepressants.
1. What are some diagnostic possibilities?
2. When mom arrives in the ER what additional information would you like to have?
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An 8-year-old male is taking phenytoin 100 mg BID for partial complex seizures. He
has been seizure-free for 10 months. His general health is good. He is referred to you
for temper tantrums, mood swings and outbursts of destructive, combative behavior.
You place this child on valproic acid 250 mg BID. One week later the child’s mother
calls you because the child is stumbling, clumsy, and sleepy. In addition, he had one
90-second generalized tonic-clonic seizure.
1. What are some diagnostic possibilities?
2. What is the physiologic basis for your diagnosis?
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A 27-year-old woman in the second trimester of her first pregnancy was referred to the
Epilepsy Clinic from the High-Risk Obstetric Clinic. As a college student, at the age of
19, she had her first generalized tonic-clonic seizure. She had three more seizures
during her early twenties, each beginning by staring and picking at her blouse buttons
before progressing into a generalized tonic-clonic seizure. She was the product of a
normal pregnancy and delivery with normal developmental milestones. At age 15
months she had experienced two prolonged seizures with high fever, both lasing
approximately 15 minutes. She had continued to develop normally and had been
successful both athletically and scholastically.
1. What type of seizure does she have?
2. What drug would you use and why?
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A 26 y.o. woman in the second trimester of her first pregnancy was referred to the
Epilepsy Specialty Clinic. In her early 20’s she had had the first of three generalized
seizures that were ultimately controlled with carbamazepine, which she continues to
take. Each of these episodes began with a vague and “scary feeling,” followed by loss
of awareness. On one occasion she was also observed to stare and pick at her blouse
before progressing into a tonic-clonic seizure. She had been the product of a normal
birth and delivery, but at age 10 months she had had two prolonged febrile seizures,
both lasting about 15 minutes and associated with transient paralysis of her left arm.
She had otherwise developed normally and had been successful both athletically and
scholastically.
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1. Does this woman have epilepsy? Based on her symptoms, how would you
classify it?
2. How well can you localize the ictus, or does it begin as a generalized
phenomenon?
3. What is the significance of complicated febrile seizures?
4. What is the mechanism of action of carbamazepine in the treatment of epilepsy?
Are there other anticonvulsants that share this mechanism?
5. How is the potential teratogenicity of anticonvulsants mitigated? Should this
woman’s anticonvulsant be changed at this time?
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Sitting in the classroom, 9 year-old Meriem felt "funny" and after several minutes she
had the strange feeling that she had never seen the classroom, her class mates or the
teacher before. During this time the teacher noticed that Meriem was staring and
unresponsive. Meriem then looked confused and fumbled with her papers for another
60 seconds after which she was back to normal.
She has had this type of spell once or twice a month for the entire school year. No one
in the family has similar episodes. Except for a 30 minute seizure with a febrile illness at
age 18 months she has been well. Meriem is an average student.
1. What is the differential diagnosis?
2. What is the most likely diagnosis?
3. What tests would be appropriate and what would they be expected to show?
4. How would you manage this child?
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Four-year-old Joe has had four spells, all shortly after going to bed at night. With each
one he has jerking of his left lower face that rapidly becomes generalized and lasts
about one minute. He is developmentally normal and has a normal examination. Family
history is negative for anyone having similar episodes. Pregnancy, labor and delivery
were unremarkable and he has never had a serious illness.
1. What is the differential diagnosis?
2. What is the most likely diagnosis?
3. What tests would be appropriate and what would they be expected to show?
4. How would you manage this child?
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As she starts to answer the teacher's question, 8 year old Allison stops in mid-sentence,
stares without moving for 30 seconds and then resumes speaking. She is apparently
unaware that she did anything unusual and resumes her previous activity. Mother
reports that pregnancy, labor and delivery were unremarkable. Allison did well in school
until September when these spells began. Mother has noticed 10 or 12 spells a day.
Sometimes Allison will stop in the middle of a sentence, have a spell and then resume
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talking. There is a strong family history of epilepsy on father’s side of the family.
1. What is the differential diagnosis?
2. What is the most likely diagnosis?
3. What tests would be appropriate and what would they be expected to show?
4. How would you manage this child?
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Running through the house, 2 year old Bill trips, falls, cries, suddenly stops crying,
seems to stop breathing, and then arches his back for about 10 seconds. He then
seems to catch his breath, resumes a normal posture, and falls asleep. He is the
product of an uncomplicated pregnancy, labor and delivery. His development has been
normal and he has not had any surgery or serious illness. Family history is negative for
epilepsy.
1. What is the differential diagnosis?
2. What is the most likely diagnosis?
3. What tests would be appropriate and what would they be expected to show?
4. How would you manage this child?
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Three-year-old George W. has repeated episodes where he wakes up at night
disoriented and confused. These spells last 15 to 45 minutes and during them he cries,
looks fearful, and is out of contact with his environment. After the episode ends he goes
back to sleep. He has never had any sort of spell during the day. These episodes began
6 months ago and occur once or twice a month. He is the product of an uncomplicated
pregnancy, labor and delivery. His development has been normal and he has not had
any surgery or serious illness. Family history is negative for epilepsy.
1. What is the differential diagnosis?
2. What is the most likely diagnosis
3. What tests would be appropriate and what would they be expected to show?
4. How would you manage this child?
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Five year old Arman was well until this morning when he had a low grade fever and ‘just
lay around’. This evening he stopped talking and then began to have jerking movements
of his right hand that progressed to a generalized seizure. After a large dose of IV
lorazepam followed by phosphenytoin his seizures stopped but he is sleepy, not
speaking, and febrile to 101. On examination he has a mild right hemiparesis. His spinal
fluid has 400 WBC, 86 RBC, glucose = 52, and protein = 89. He was previously well. He
is the product of an uncomplicated pregnancy, labor and delivery. His development has
been normal and he has not had any surgery or serious illness. Family history is
negative for epilepsy.
1. What is the differential diagnosis?
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2. What is the most likely diagnosis
3. What tests would be appropriate and what would they be expected to show?
4. How would you manage this child?
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Bob was well until the 1st grade when he developed a series of rapid eye-blinks. They
resolved but that summer he began to do a rapid & repetitive nose twitch. That also
resolved but his second grade teacher complained that he made repetitive sniffing
noises. Now at the beginning of 3rd grade, his mother reports that Bob also makes an
unusual, brief, rapid head turning movement to the right. Bob seems unaware of any of
this. He was previously well. He is the product of an uncomplicated pregnancy, labor
and delivery. His development has been normal and he has not had any surgery or
serious illness. Family history is negative for epilepsy.
1.
2.
3.
4.
What is the differential diagnosis?
What is the most likely diagnosis?
What tests would be appropriate and what would they be expected to show?
How would you manage this child?
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Headache
A 50-year-old woman with a history of migraine headache comes to the ER with the
chief complaint of headache, which came on very suddenly about two hours earlier.
The pain is described as severe and began without the aura she usually experiences
with her migraines. She had a similar headache about 3 weeks ago, but it was not as
severe, her neck seemed stiff for a few days after the headache. The remainder of her
history is unremarkable. Her family history is positive for migraines and two family
members have polycystic kidney disease. On examination, she acts sleepy, has a
complete right CN III palsy, diffusely but mildly brisk tendon reflexes, and positive
Kernig’s and Brudzinski’s signs. A CT scan of the head, done without contrast was
ordered before you were told of the patient; you agree with the radiologist that it is
normal.
1. What test would confirm your diagnosis?
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A 16 year-old female comes to your office with a chief complaint of progressive
headaches for 6 months. She is otherwise healthy with an unremarkable past history.
Her exam is normal except for obscure disc margins (optic nerve).
1. What would you include in your differential?
2. What is “pseudopapilledema”?
3. What diagnostic tests would you perform?
4. What are some known causes of pseudotumor?
5. If this patient’s opining pressure was 190 mm of H2O, what would the
diagnosis be?
6. Why is Diamox effective in treating pseudotumor cerebri?
______________________________________________________________________
“Brenda” is a 32 year old second grade school teacher who has had bilateral periorbital
headaches since the age of 14. She remembers having to leave school early in high
school, feeling sick to her stomach and wishing to lie down in a quiet room. The
headaches are often worse during her menstrual period, but can occur anytime,
particularly after times of stress or with abrupt weather changes. Brenda recalls her
mother having had “sick headaches,” when she and her sisters needed to keep quiet
and turn off all the lights in the house.
1. What are the primary causes (not due to structural lesions, or metabolic
dysfunction) of headache in young women?
2. If you had one question to ask Brenda, to help differentiate this headache from an
“ordinary,” or mild headache, what would it be?
3. Brenda would like to avoid taking preventative medicines for headache. What can
you tell her about her diet that may minimize or avoid headache; i.e. what are
common headache triggers?”
4. Does the location of Brenda’s headache help you identify its cause?
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5. Brenda would like to become pregnant. What will happen to her headaches in
pregnancy, and which medications are safe for the fetus?
______________________________________________________________________
“Charles” is 42 years old and has had headaches that occur in a peculiar pattern,
for more than twenty years. They have a circannual and circadian rhythm, occurring
most commonly at the summer and winter solstice, and awakening him from sleep at 1
AM like clockwork. Charles’ pain is like an “auger” in his eye; he paces in circles
holding his head on one side, and ends up thrashing on the floor for nearly an hour.
Finally he can sleep.
1. Why should Charles not watch Super Bowl football commercials on TV?
2. What type of primary headache does he characterize?
3. What acute and preventative treatments are available for him?
4. Where do the “rhythm” centers that control sleep/wake cycles and circadian
rhythms reside in the brain?
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Inflammatory Disease/Infection
A 40-year-old male had fever and headache for 3 days. Over the past 24 hours he
has had difficulty getting his words out. His family brought him to the ER following a
generalized tonic–clonic seizure. On exam he was lethargic and appeared confused.
His neck was supple. The optic discs were sharp. There were no focal findings on
neurologic examination. His CT scan of the head was unremarkable. CSF studies
showed 46 WBC’s (90% lymphs). 356 RBC’s (nontraumatic tap), and a protein of 66.
1. Probable diagnosis?
2. What tests would confirm your diagnosis
3. Discuss management
______________________________________________________________________
A 62 year old college professor is brought to the ER by fire rescue after suffering a
grand mal seizure at home. His wife reports that he has been ill for the past 3 days
complaining of increasing headache and malaise. She noted that he acted “strange”
the day before the seizure and that his memory seemed to be poor, however, she
ascribed it to his systemic illness. He remained in bed through the day until the time of
the seizure.
On examination, he is lethargic and appears incoherent. His temperature is 102.3 and
the other vital signs are normal. His general examination is unremarkable. There is mild
nuchal rigidity. No focal weakness is detected. His reflexes are quite brisk but no
pathological reflexes can be demonstrated.
An MRI is done and shows the following:
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1. What is the likely diagnosis?
2. What will you do next to confirm that diagnosis?
3. How will you treat this patient?
______________________________________________________________________
A 35 year-old, previously healthy male, presents to ER with headache, vomiting, and
a temperature of 101○. He has not felt well for 3 days. His exam is unremarkable
except for fever and resistance to anterior flexion of his neck.
I. For each of the following CSF findings give a probable diagnosis
1. protein 65; glucose 48; 75 WBC’s (75% lymphs)
2. protein 120; glucose 22; 1,220 WBC’s (90% polys)
3. protein 155; glucose 35; 120 WBC’s (90% mononuclear cells)
4. protein 48; glucose 70; 65 WBC’s (90% lymphs)
5. protein 75; glucose 68; 85 WBC’s, 850 RBC’s (90% lymphs)
6. protein 200; glucose 15; 65 “bizarre” mononuclear cells
II. What is the most common cause of non epidemic encephalitis?
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Movement Disorders
A previously healthy 7-year-old male developed quick, jerky movements of both
arms over several days. He gradually became irritable and his parents reported he
would cry for no apparent reason. His speech became difficult to understand.
Examination revealed quick, random movements of both upper extremities that were
more pronounced on the right. The movements were worse distally. Speech was
slurred and he would occasionally “fling” his right arm. Mild generalized hypotonia was
present, and the child could not sustain a fixed posture.
1. What is your diagnosis?
2. What is the mechanism of action of dopamine?
3. What are the signs and symptoms of dopamine toxicity?
______________________________________________________________________
A 7-year-old girl is taken to the pediatrician by her mother. She notes that her daughter
has been “moody” and acting “spacey” for the past few days. She also reveals that she
has been “fidgety” and had some unusual, seemingly uncontrollable “jerking”
movements for the past week. The child is the product of a normal pregnancy and birth.
She has achieved all of her developmental milestones on time. She has been healthy
although mom recalls her daughter having a sore throat several weeks ago.
1. What test would you perform to help confirm the diagnosis? What else is would
you include in the differential diagnosis?
2. Do the movements require treatment? If so, how would you accomplish this?
3. Is any other treatment required? What about long-term treatment?
4. If untreated, what is the most significant long-term complication?
______________________________________________________________________
A 13-year-old female presents to your office with a one-week history of quick,
random jerks of her left shoulder. At age 10 years she had been taken to an
ophthalmologist for repetitive eye blinking. She was diagnosed as having allergic
conjunctivitis and placed on topical steroid drops. The eye blinking responded to
treatment over the next several weeks. The following year the child was taken to an
allergist for repetitive “sniffing”. She was diagnosed with allergic rhinitis and treated with
an antihistamine plus a nasal spray. She improved over the next few months with
resolution of her symptoms. During the several months prior to her visit to your office
there had been a decline in school performance. Teachers reported poor attention to
task and problems concentrating.
1. The child most likely has?
2. Name 3 movement disorders seen in children:
3. What movement disorders begin in childhood?
______________________________________________________________________
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A 65-year-old retired lawyer was brought in by his wife because of “shaking”. His
wife reported that not only did his hands shake, but there had also been a recent
“personality” change: the patient had become very “slow” in his movements and often
sat motionless with an expressionless face. Examination showed a 4 Hz resting tremor
that improved with movement. There was cogwheel rigidity of the limbs and a shuffling
gait. The patient was alert and intelligent, and the remainder of the exam was normal.
1. An appropriate differential diagnosis of this case would include:
2. What are the basal ganglia?
3. What is the striatum?
4. What is the lenticular nucleus?
______________________________________________________________________
A 70-year-old retired rehabilitation professor is referred to a neurologist for a right
hand tremor that began about 6 months earlier. He complains about “getting older” and
losing a lot of his previous energy and motivation. He no longer attends conferences
and has laid down a book that he had been actively writing up until a few months ago.
He has noticed that his writing is “shrinking” and that his right hand feels clumsy. He has
noticed difficulty arising from deep chairs and feels unsteady but has not fallen. He
takes no medications, does not abuse tobacco and rarely drinks wine with dinner. He
has been healthy throughout life and has no family history of neurological illness. His
general exam is normal but he has a slight stoop with no postural instability. He has a ↓
right arm swing while walking, a resting tremor of the right hand that improves with
volitional movement, and cogwheel rigidity on the right.
1. Can you anatomically and pharmacologically localize this man’s lesion?
2. What is the most likely diagnosis? What should be considered in the differential?
3. What medications can typically cause symptoms such as this man has?
4. What is the biochemical deficit that causes the majority of this man’s symptoms?
How would you treat this man’s symptoms? What can you tell him about the
prognosis?
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Neuromuscular Disease
A 30 year-old female comes to your office complaining of not feeling well for 6
months. Recently she has had difficulty rising from a chair and climbing stairs. At times
she thought she might have had “a little fever”.
1) Your diagnosis?
2) What blood test will support your diagnosis?
3) What will her EMG show (needle electromyography)?
4) How would you treat this lady?
______________________________________________________________________________
A 73 year old man had enjoyed excellent health until the last 2 months. He first
noticed difficulties in his golf game, with his fairway drives loosing approximately 25
yards. Within weeks he experienced difficulty arising from the floor, requiring the use of
his hands. Recently, he has had to pull himself up from the seat of his car.
On neurologic examination he is an excellent historian with good orientation and
recall. His cranial nerve examination is normal. He has no discernible sensory deficits.
On motor examination his muscle bulk and tone are normal. He has hesitation of
hip and back extension when arising from a chair with his arms folded. He is unable to
arise from a complete squat, nor is he able to jump off the ground. When arising from
the floor, he leads with his buttocks while pushing off with his hands to extend his back
and hips. Forced contraction of the biceps is easily broken. Intrinsic hand muscle
strength is normal. His tendon reflexes are easily obtained except for relatively
depressed ankle reflexes. He has no ataxia.
Dx-Polymyositis
Questions
1. 1What laboratory tests will directly aid in the diagnosis of his condition?
i. CK
ii. ANA
iii. EMG/NCS
iv. Muscle Biopsy
2. Discuss the expected findings for each of these tests.
3. What portion of the general physical exam is particularly important for his
diagnosis?
i. Skin for extensor surface and periungual rash
4. What criteria are used in the diagnosis of his condition
5. –List potential appropriate initial treatment options
Steroids
Methotrexate
IvIg
______________________________________________________________________
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A 60 year-old overweight female complains of numbness and tingling in her toes.
Her general health is good except for mild hypertension. Her exam reveals a PB of
135/85, mild obesity, and a question of unsteadiness when asked to stand within a
narrow base and her eyes closed. The dorsalis pedis and posterior tibial pulses are
difficult to palpate.
What readily available laboratory test will confirm your diagnosis?
What other abnormalities on neurologic examination does this lady have?
_______________________________________________________________________
A 13-year-old girl describes a three-month history of difficulty rising to her feet without
using her arms, initially for low chairs, and now from any chair. More recently, she
started having difficulty holding up her arms to set her hair. Her weakness is symmetric
and has not fluctuated. She has no shortness of breath or weakness of head and neck
muscles. She does not have pain or sensory disturbance. There is no family history of
neurologic or neuromuscular disorders. She has no relevant past medical history, and
is taking no medication. On examination, she has normal mental status and cranial
nerve function. Muscle bulk and tone are normal. Neck flexor strength is grade 4 over
5. Shoulder abduction is grade 4+ and hip flexion is grade 3+, in a symmetric
distribution. She must be helped to a standing position and cannot perform a deep
knee bend. Tendon reflexes, plantar responses, and sensory examination are normal.
1. Compare and contrast the signs and symptoms of muscle versus peripheral nerve
disease.
2. What is your diagnosis?
______________________________________________________________________
A 55-year-old man suddenly experienced an excruciating, violent, shooting pain
along the left side of his face. The pain was so severe that he was forced to stop work.
The attack lasted 15-20 seconds, but recurred several times. That evening while you
examined him, he again had another attack. You observed lacrimation of the
conjunctiva, excess flow of saliva, and palpated a trigger point along the inferior edge of
the zygoma. He describes the pain as radiating to the forehead, the eye, and the root of
the nose. During the next month these attacks appeared paroxysmally.
1) Discuss your diagnosis
2) Why would Tegretol help this man?
______________________________________________________________________
A 60-year-old male complains of generalized, symmetrical weakness of 3 months
duration. On examination, he demonstrates mild generalized weakness that appears
more prominent proximally than distally. He seems to become stronger with repeat
testing of strength. There is no facial or bulbar weakness. Tone and coordination are
normal. Sensation to the primary modalities is normal. Deep tendon reflexes are
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normal and plantar stimulation elicits a flexor response. Nerve conduction studies show
a low amplitude compound motor action potential that with repetitive stimulation at 50
Hz shows an incremental response. EMG needle exam of the right leg and paraspinous
muscles is normal.
1) What is the difference in botulism and the Lambert-Eaton syndrome at the
neuromuscular junction?
2) What is a paraneoplastic syndrome?
______________________________________________________________________
A 40-year-old female with no significant past medical history and who was an
adopted child, complains of numbness and tingling sensations in her feet that have
been present for 6 months. On exam, she has decreased pin and light touch sensation
in a stocking distribution. Motor strength is normal but deep tendon reflexes are
depressed with absent ankle jerks. The response to plantar stimulation was weakly
flexor. She is on no medications and does not use alcohol.
1) What are the two most common causes of neuropathy in this country?
2) What causes muscle atrophy in neuropathy?
______________________________________________________________________
A 65-year-old male is referred to you by his primary care physician for a few months
history of burning feet. It initially bothered him at night bur more recently, it is present
almost constantly. The pain involves the entire feet up to the ankles. It is aggravated
by physical activity. He also describes decreased sensation in his feet and finger tips.
He has no weakness. He occasionally has back pain with no radiation. There is no
difficulty controlling his bowel or bladder. He has experienced some lightheadedness
with abrupt change of position (mostly from supine to standing). He has a history of
coronary artery disease, diabetes and tobacco abuse. There is family history of
diabetes and HTN. His exam is remarkable for trace of weakness in both tibialis
anteriors, decreased sensation in his feet up to ankles and absent ankle jerks.
1) Localize the site of lesion.
2) Provide a differential diagnosis for this problem.
3) What is your evaluation and treatment plan?
______________________________________________________________________
A 15-year-old female who has been in good health comes to the UK Emergency
Room on a Friday evening with complaints of a three-day history of tingling in her legs.
Over the last two days, she has become aware of weakness in her legs, which began
with frequent tripping and progressed to difficulty getting out of a chair. Today, she
feels weak in her hands, complains of aching in her hips and thighs and feels short of
breath. She and her mother deny any history of nausea, vomiting, changes in mental
status, or cranial nerve symptoms. Her family doctor diagnosed her with a mild viral
upper respiratory infection about two weeks ago. On examination, she is symmetrically
weak, lower > upper extremities and distal > proximal muscles. Her reflexes are all
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diminished; absent in the knees and ankles. Her sensory exam is normal to all
modalities.
1) The most likely diagnosis is?
2) How would you confirm this diagnosis?
______________________________________________________________________
A 7 year-old boy complains of falling and his legs feeling funny. His mother
reports his symptoms began about 5 days earlier and seem to be getting worse. On
examination he is irritable and uncooperative. Motor and sensory examinations are
unreliable. His Achilles reflexes are absent.
Which of the following statements concerning this child could be true?
1.
2.
3.
4.
He has elevated CSF protein
He had gastroenteritis 1 month ago
He will develop significant muscle atrophy
He could develop respiratory failure
Explain why patients with some forms of peripheral neuropathy have slow nerve
conduction velocity measurements.
______________________________________________________________________
A 40-year-old man comes to the ER with a complaint of progressive weakness. He
had awakened in the morning and on his way to the bathroom, “stumbled”. He also
noted some pain in his lower back. Later that morning, he felt clumsy while typing at his
computer and subsequently noted some difficulty getting up from his desk chair. A coworker told him he should see a doctor. In the ER, he recalls that besides a recent
gastroenteritis, he had felt well and even played basketball and gone out for pizza and
beer with his friends.
1) What, if any, is the significance of the previous gastroenteritis?
2) What, if any, is the significance of eating pizza and drinking beer?
3) While examining the patient, he complains of feeling “weaker”. Is there anything
in particular that you need to be worried about?
4) What do expect to find on Neurological exam?
5) The patient is admitted to the Neurology Service. What test(s) do you want to
perform to confirm your clinical suspicions? What do you expect them to reveal?
6) You make the diagnosis. How would you treat the patient?
______________________________________________________________________
A 30-year-old male complains of easy fatigability and generalized weakness A 48year old man describes double vision, mild arm weakness and rapid fatigability with
routine activities. Double vision was noted transiently several weeks ago, but has
recently reappeared along with difficulty climbing stairs. His endurance has decreased
markedly: he finds it tiring to even chew his food and he thinks he has become more
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liable to choke. He has no pain or sensory loss, takes no medications, and has no
family history of neurological illness. His neurological examination is normal except for
right eyelid droop and bilateral eye closure weakness. He has normal strength in his
shoulders at first, but prominent fatigue after several repetitions of muscle activation.
1) Where would you localize a lesion that leads to fatigable weakness?
2) What electrophysiologic tests are useful in making the diagnosis of this patient’s
illness?
3) What laboratory test, if positive, allows for a definitive diagnosis?
4) Describe the pathophysiology of this autoimmune disease.
5) What are current recommended symptomatic and therapeutic treatments.
______________________________________________________________________
A 30-year-old male complains of easy fatigability and generalized weakness, worse
in the afternoon. On examination, he demonstrates mild proximal muscle weakness
involving the arms and legs. There is mild bifacial muscle weakness and a nasal quality
to his voice. Sensory exam and deep tendon reflexes are normal; plantar response is
flexor.
1) What serologic studies are potentially abnormal in myasthenia gravis?
2) What are the “classic” electrodiagnostic findings in myasthenia?
3) What is your differential diagnosis in this case?
4) What is your differential diagnosis in this case?
______________________________________________________________________
A 32 year old woman is seen because of weakness and fatigue. She reports feeling so
exhausted that she can “barely get through the day”.
______________________________________________________________________
A previously healthy 35 year-old female comes to your office because of
intermittent horizontal diplopia of 6 weeks duration. On examination you find only mild
ptosis of the right upper eyelid.
1) What would you do next?
2) How does botulinum toxin cause paralysis?
3) How does cobra venom cause paralysis?
______________________________________________________________________
A 62 year-old male comes to your office with a chief complaint of muscle cramps.
For the past several months he has had cramping of his calf muscles, thigh and upper
arms. He has also noticed occasional “twitching” of muscles in the same areas as the
cramping. His general health is good but he reports that he tires easily. On exam both
great toes extend with plantar stimulation. His DTRs are brisk but there are no other
definite abnormalities.
1) Diagnosis?
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2) What is McArdle’s disease?
3) Who was the “Iron Horse”?
4) What is a fasciculation?
5) What is a fibrillation?
______________________________________________________________________
A 38-year-old female presents to your office with 5 days history of double vision.
She first noticed this while driving. She had to close one eye to be able to continue
drive home. The symptom has persisted although it may wax and wane at times. She
also has difficulty staying awake and “keeping her eyes open” at night. She has felt a
“tension” around her eyes but denies having headache, numbness or focal weakness.
She has a history of type I diabetes and hypothyroidism. Family history and social
history are unremarkable. On exam, she has a mildly convergent gaze with worsening
diplopia when looking to the right.
1) Given the finding on the neurological examination, localize the potential sites of
the lesion.
2) What is the differential diagnosis for this patient?
3) What is your evaluation and treatment plan?
______________________________________________________________________
A 43-year-old gentleman has been evaluated extensively for two years due to
increased ALT and AST, including two liver biopsies, numerous scans and innumerable
blood tests – all normal. His doctor checks a CPK, which is found to be 2198 (normal
40-240).
In retrospect, he now recalls that at age 8, his father had to drag him several
blocks after he became tired walking to Rupp Arena. In high school, though he was a
good athlete, he could never run very far before becoming tired, and had to be removed
from basketball games frequently from fatigue. As an adult, he recalls three occasions,
after strong exercise, his back hurt for a day and his urine “looked like Coca-Cola®”. He
runs the treadmill most days. He notices that he feels very fatigued for a few minutes
while running at first and then he feels stronger and faster.
PE:
–General Exam - Normal
–Cranial Nerves - Normal
–Motor – Normal bulk, tone, strength
–Sensation – normal
–Cerebellar – normal
–DTR 2 and symmetrical, plantar responses are downgoing.
–Gait normal. He is easily able to do repeated squats and arise from a chair with his
arms crossed.
Questions
1. Where would you localize the neurological lesion?
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2. Does this affect your differential diagnosis?
3. What is your differential diagnosis?
Labs
NCS - Normal
EMG - Normal
______________________________________________________________________________
This 47 year old woman awoke with a mild dull ache behind her right ear. When
applying make up to her face shortly afterwards, she noted that her face was
asymmetric and the right side seemed to be “ironed out”. Facial sensation was normal
and there was no limb weakness or other neurological disturbance. She was very
concerned that she had suffered a stroke and was taken immediately to the emergency
room by her husband. She had no prior history of significant medical illness. Her only
hospitalizations were for childbirth.
1. What is the most likely diagnosis?
2. What diagnostic studies, if any, are warranted?
3. How would you treat this lady?
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Spinal Stenosis
This 60 year-old gentleman complains of progressive back pain which occasionally
radiates to the bilateral hip areas over several years. This is worsened by walking or
prolonged sitting. He notices intermittent numbness in the feet for one year. He denies
any trauma to the back or change in bladder or bowel habits.
Exam
•
•
Neck: supple, nontender
Back
– Mild midline lumbar tenderness
– Bilateral sacroiliac joint tenderness
– Slightly limited anterior and posterior ROM
– Nontender paraspinous muscles
Exam
•
•
•
•
•
Cranial nerves - normal
Motor - Moderate weakness right foot dorsiflexion
DTR - 2 symmetrical except decreased left ankle jerk. Plantar responses are flexor.
Sensation - decreased pinprick medial right foot
Gait - leans forward slightly at the waist.
Exam 2
•
•
•
•
•
Laségue’s sign negative
Fajerstein test negative
Hip rotation test negative
Patrick’s test negative
Stoop test positive
Stoop Test
• The "stoop test" has been devised to assess the relationship between claudicationlike symptoms (pain, paresthesia in the lower extremity) elicited while standing versus
walking. The test consists of having the patient walk "briskly", while maintaining an
upright posture. When the symptoms of claudication become intense, the patient
assumes a stooped posture while continuing to walk. The patient is then asked to
stop walking and stand upright, at which time the symptoms usually return. The stoop
test is considered positive for neurogenic claudication when flexion while walking or
stooping relieves the symptoms in the limb.
Testing
• NCS - Right leg • EMG - Right leg -
Normal
Normal
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•
•
•
•
•
•
L Ant tib
Fibs
L Gastroc
Normal
L Post Tib
Fibs
L Peron Long
Normal
L Vastus Lat
Normal
L L-S Paraspinous Fibs
MRI
Spinal Stenosis
•
•
“narrowing of the spinal canal sufficient to produce transient vascular compromise of
the the neural elements”
Frequency*
– 13-14% of LBP patients referred to back specialist
– 3-4% of LBP patients seen by PCP
Spinal Stenosis
•
•
Congenital
– Appears usually 30’s - 50’s
Acquired
– Appears usually 50s - 60’s
– Diabetics at 16x risk
Spinal Stenosis
Anatomic Features
•
•
•
Disc protrusion
– Canal narrows, nerve compression
– Osteophyte growth
– Facet arthrosis
Ligamentum flavum hypertrophy
Spondylolisthesis
Symptoms
•
•
•
•
Back pain 95%
Leg pain 71%
Weakness 33%
Pseudoclaudication 94%
Exam
•
Laségue’s sign usually negative
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•
•
Sensory changes in the L5(91%), S1(63%) or L1-4(28%)
Stoop test
– Walk until symptomatic, bending at waist relieves pain
Testing
•
•
•
•
•
•
EMG/NCV
CT/MRI/CT myelography
– Normal AP diameter of spinal canal 15 mm
– 10-12 mm relative stenosis
– <10 mm absolute stenosis
Natural History70% remained stable over 4 years
15% improved
15% worsened
Progression more likely if
– Smaller AP diameter
– Bilateral symptoms initially
Treatments
•
•
•
•
•
•
Regular exercise – pref nonweightbearing
Weight loss
Management of osteoporosis
PT
Pain control
Surgery
• Laminectomy
• Spinal fusion
• Diskectomy
Treatments
•
•
Conservative therapy (nonsurgical) appropriate for mild and moderate symptoms (no
diff in early or late surgical treatment groups*)
Surgical therapy for
– Intractable pain
– Significant neurological deficits
– Bladder or bowel dysfunction
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Sydenham’s Chorea
In the Age of MRI: A Case Report and Review
William C. Robertson, Jr, MD and Charles D. Smith, MD
A 9-year-old female with acute chorea was found to have multiple areas of abnormal
signal on magnetic resonance imaging of the brain consistent with vasculitis. The
child’s serology and clinical course were indicative of Sydenham’s corea, and other
causes of vasculopathy were excluded. Although magnetic resonance imaging findings
similar to those observed in this patient have been rarely reported in Sydenham’s
chorea, their presence alone should not prompt a search for an alternative diagnosis.
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