Transcript Neurology Board Review

Neurology
Board Review
A 38-year-old woman is evaluated in the office for a 10-month history of increasingly
frequent headache. The headache is often worse in the morning on awakening. She has
recently started keeping a headache diary, which reveals episodes on approximately 25 days
of each month. The headache varies from a near-daily bilateral frontal dull throbbing to a
severe left hemicranial throbbing associated with nausea, photophobia, and phonophobia.
The patient has a 20-year history of migraine without aura and a history of depression. Her
mother also has a history of migraine and depression, and her sister has a history of
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migraine.
The patient has been taking propranolol for 3 months; a mixed analgesic containing
butalbital, caffeine, and acetaminophen for mild or moderate headache at least 3 days per
week for 9 months; rizatriptan for severe headache at least 2 days per week for 4 months;
and citalopram for 1 year. Rizatriptan has become increasingly ineffective over the past
month.
Physical examination findings, including neurologic examination findings, are normal.
Which of the following is the most likely diagnosis for her current symptoms?
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2.
3.
4.
Chronic migraine
Chronic tension-type headache
Idiopathic intracranial hypertension
Medication overuse headache
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Headache
Symptoms
Treatment
Prophylactic
Only image pt with headache if patient has a “red flag” for
Migraine
Unilateral, pulsating, Acetaminophen,
Beta-blockers,
secondary headache:
last 4-72hrs + aura
Aspirin, NSAIDs and TCAs,
triptans
anticonvulsants,
1. first or worst headache
CCB
2. abrupt onset or thunderclap
Cluster 3. progression
Unilateral,
severe
Oxygen
and
Verapamil
or fundamental
change
in headache
pattern
pain
with vasomotor
triptans
4. abnormal
physical
exam
phenomenon
5. neurologic
symtoms lasting longer than an hour
6. new headache in pt <5 >50 years old
Tension7. new heache
Squeezing,
bilateral,
Aspirin,
Amitriptyline,
in pt with
cancer/pregnant/immunosuppressed
chronic,
constant,
Acetominophen,
gabapentin
8. headache
with LOC
non-pulsating
9. headache
triggered by exertion,NSAIDs
sexual activity or valsalva
Idiopathic
In obese womenRepeat LP’s,
Weight reduction
MRI is study
of choice
unless there acetazolamide,
is a skull fracture, acute SAH or
Intracranial
morning
headaches
ICH and paranasal
sinus
Hypertension
worse with
incdisease! loop diuretics
(Pseudotumor) pressure, papilledema
Trigeminal
Neuralgia
Brief unilateral
lancinating pain
Carbamazepine,
oxcarbazepine
A 32-year-old woman is seen for a follow-up evaluation. She had a witnessed
generalized tonic-clonic seizure 1 week ago and was evaluated in the emergency
department, where results of physical examination, complete blood count,
measurement of serum electrolyte levels, and urine toxicology screen were all
normal. She is otherwise healthy, has no significant personal or family medical
history, and takes no medications.
Results of a repeat physical examination are also normal.
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In addition to electroencephalography, which of the following diagnostic tests
should be performed next?
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2.
3.
4.
Ct of the head
Lumbar puncture
MRI of the brain
Positron emission tomography
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A 70-year-old man is seen in the office for routine follow-up of partial seizures
that began 2 years ago after a stroke. At that time, he was started on
phenytoin, 300 mg/d, and has had no subsequent seizures; he tolerates the
medication well, with no reported adverse effects. His current medications are
phenytoin, an angiotensin-converting enzyme inhibitor, a statin, and aspirin.
Results of physical examination, including a neurologic examination, are
normal.
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Laboratory studies show a total serum phenytoin level of 9 mg/L (35.6 µmol/L)
(therapeutic range, 10-20 mg /L [39.6-79.2 µmol/L]). Results of a complete
blood count and liver chemistry tests are normal.
Which of the following is the most appropriate next step in management?
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2.
3.
4.
Continue the phenytoin at the current dosage
Increase the phenytoin dosage
Measure the free serum phenytoin level
Substitute gabapentin for the phenytoin
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Partial Seizures
• Simple partial- focal seizure that does not impair
awareness
• Complex partial- involves an alteration of
consciousness that typically occurs when the seizure
spreads to involve one or both temporal lobes
• Secondarily generalized- when a partial seizure
spreads to involve both hemispheres diffusely and
results in a generalized convulsion
Generalized Seizures
• Generalized tonic-clonic
• Absence- momentary lapse of consciousness
(typically less than 5 seconds)
• Myoclonic- brief shocklike muscular contractions
that can occur in rapid succession and usually are
not associated with an impairment of
consciousness
Diagnostic Evaluation of Seizures and
Epilepsy
• Head CT in the acute setting
• MRI brain
• EEG
• Inpatient video EEG monitoring should be considered
in any patient whose seizures have not responded to
two or more antiepileptic drugs or require further
characterization
Increased Risk of Seizure Recurrence
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Age > 65 years
Abnormal EEG
History of prior neurologic injury
Postictal Todd paralysis
Family history
Partial seizure as first seizure
MRI with abnormality
• The presence of one or more of these risk factors
justifies the institution of AED treatment, even
after a single seizure.
Treatment Pearls
• Monotherapy preferred over polytherapy
• Uptitrate medication until seizure free or develop adverse side
effects
• Make dose adjustments based on clinical seizure control and
drug side effects moreso than based on therapeutic levels.
• Consider slowly tapering AEDs off after being seizure free for 2
years.
• Juvenile myoclonic epilepsy requires lifelong treatment.
• Continue therapy: abnormal neuro exam or underlying structural
abnormality.
• IV lorazepam followed by phenytoin (fosphenytoin if
available) is first-line management of status epilepticus
A 75-year-old woman is evaluated 1 day after experiency a 60-minute
episode of right arm weakness and dysarthria. The symptoms have not
recurred. She has hypertension and type 2 diabetes mellitus. Medications
are aspirin, metoprolol, enalapril, and metformin.
On physical examination, blood pressure is 156/94 mm Hg, pulse rate is
62/min and regular, and respiration rate is 16/min. No carotid bruits are
noted. Neurologic examination findings are normal.
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Results of laboratory studies obtained 3 weeks ago show a hemoglobin A1c
value of 7.1% and a serum LDL cholesterol level of 68 mg/dL (1.76 mmol/L).
Which of the following is the most appropriate next step in management?
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2.
3.
4.
Addition of clopidogrel
Immediate hospital admission
Outpatient MRI of the brain
24-Hour ECG monitoring
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TIA
• Transient neurologic
deficit without acute
infarction
– Risk of stroke at 90
days is 10%
– Most stroke risk within
first 48 hours, 5.3%
patients at risk
– Identify patients at
highest risk of stroke
with ABCD2 score.
Hospitalize patients seen within 72hours of
initial symptoms and whose score is at least 3.
A 62-year-old woman is evaluated in the stroke unit for a 2-day history of difficulty
speaking and right arm weakness. She has a history of hypertension and dyslipidemia and a
35-pack-year smoking history. Medications are lisinopril, atenolol, simvastatin, and aspirin.
On physical examination, blood pressure is 148/78 mm Hg, pulse rate is 84/min and regular,
and respiration rate is 12/min. Other general medical examination findings are normal.
Neurologic examination shows mild sensory aphasia and right arm drift.
An MRI of the brain shows an acute infarct in the left middle cerebral artery distribution
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that appears embolic. A magnetic resonance angiogram of the head and neck is normal. An
electrocardiogram shows sinus rhythm and is normal. Telemetry performed over the next 3
days shows occasional premature ventricular complexes. A transesophageal
echocardiogram shows no intracardiac thrombus, normal left atrial appendage velocities,
and a patent foramen ovale with an atrial septal aneurysm. No evidence of deep venous
thrombosis is found.
Which of the following is the most appropriate next step in management?
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2.
3.
4.
Percutaneous patent foramen closure
Prolonged cardiac rhythm monitoring
Surgical closure of patient foramen ovale
Warfarin
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A 57-year-old man has a follow-up evaluation 3 months after discharge from the hospital,
where he was treated for an ischemic stroke. He has a history of hypertension, dyslipidemia,
and peripheral arterial disease for which he has required no revascularization procedures.
Medications are enalapril, hydrochlorothiazide, rosuvastatin, and aspirin.
On physical examination, blood pressure is 138/68 mm Hg, pulse rate is 68/min and regular,
and respiration rate is 16/min. Cardiac examination reveals no carotid bruits. Neurologic
examination shows only a right pronator drift.
Results of laboratory studies show a platelet count of 340,000/µL (340 × 109/L), a serum
creatinine level of 1.1 mg/dL (97.2 µmol/L), and an LDL cholesterol level of 68 mg/dL (1.76
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A head CT scan and a brain MRI show a left pontine infarct. An electrocardiogram shows
normal sinus rhythm with no ischemic changes. A transthoracic echocardiogram and a
magnetic resonance angiogram of the head and neck are normal.
Which of the following is the most appropriate treatment?
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2.
3.
4.
5.
Add clopidogrel
Add ticlopidine
Add warfarin
Substitute clopidogrel for aspirin
Substitute warfarin for aspririn
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Ischemic Stroke
• Cardioembolism
– Most common etiology is a. fib
– Multiple infarcts or infarcts involving the surface of the brain
• Large artery atherosclerosis
– Risk of recurrence is highest with >70% occlusion
• Subcortical infarcts (lacunae)
– Commonly occur in setting of HTN
– Cause pure motor or pure sensory strokes
• Cryptogenic
Stroke
Location
Symptoms
Notes
Anterior Cerebral Artery
Weakness and sensory
loss in contralateral leg
Middle Cerebral Artery
Contralateral weakness
(hemiplegia), sensory
loss and homonymous
hemianopsia
If dominant hemisphere
affected  aphasia
If nondominant 
changes in spatial
perception
Posterior Cerebral Artery
Contralateral
homonymous
hemianopsia; mild
contralateral sensory
loss; may have color
blindness
NO motor deficits
Lacunar infarcts
Either pure motor or
sensory loss
Treatment
TPA
BP
TPA: goal BP
<180/105
Anti-thrombotic
Goal BP
<140/80
No TPA goal BP
<220/120
Secondary
Prevention
Goal LDL <100
Antithrombotic
Aspirin
Warfarin
Clopidogrel
Aspirin and
Dipyridamole
*If this is the only contraindication, try to reduce BP and then give tpa
Hemorrhagic Stroke
• Intracerebral hemorrhage
– Deep location hemorrhage 
most likely due to HTN and
originates in basal ganglia or
cerebellum
– Lobar hemorrhage  more likely
related to cerebral amyloid
angiopathy
– Differential:
• Vascular malformation
• Infection
• Brain tumor with hemorrhage
– Higher mortality rate than
ischemic stroke
• Subarachnoid hemorrhage
– Typically caused by rupturued
cerebral saccular aneurysm
– Rarer causes include AVMs and
mycotic aneurysms
– “Worst headache ever”
Hemorrhagic Stroke
• 17% strokes, with 50% intracerebral hemorrhage and 50% subarachnoid hemorrhage
• Diagnosis: non-contrast CT; if negative and high suspicion for SAH  LP (erythrocytes
and xanthrocromia)
ICH
• Complications
– Hematoma expansion
– Elevated ICP
• Lower BP with IV labetalol or
nicardipine if sBP >200 or
MAP > 150
• Target BP 160/90 or MAP
110
– Hyperventilation, mannitol
and Surgical evacuation for
elevated ICP
SAH
• “Worst headache ever”
• Complications:
• Rebleeding
• Vasospams
• Hydrocephalus
Dural Sinus Venous Thrombosis
• Risk factors include venous hypercoagulable
disorders, systemic malignancy, trauma, systemic
inflammatory disorders (i.e. ulcerative colitis),
severe dehydration, infection, pregnancy
• Head CT with contrast may show “empty delta
sign”
• MRV is the diagnostic exam of choice
• Treat with anticoagulation
A 64-year-old woman is evaluated for a 1-year history of increasing difficulty finding the right word in
conversation and completing sentences; she sometimes says the wrong word accidentally. Her family
now has difficulty understanding her, and she no longer has any interest in speaking on the telephone.
Her ability to drive, shop, pay bills, and cook seems unimpaired. She has no other relevant personal or
family medical history. Her only medication is aspirin, 81 mg/d.
On physical examination, temperature is 36.6 °C (97.9 °F), blood pressure is 122/78 mm Hg, pulse rate is
80/min, respiration rate is 14/min, and BMI is 23. The patient is right-handed. Her level of alertness is
normal, and her comprehension appears to be intact, with her correctly executing the commands to
show the right thumb and two fingers on the left hand. Spontaneous speech is effortful, and she talks in
short, telegraphic sentences filled with many mispronunciations (such as “posital” for “hospital”). She
makes similar errors when trying to write words rather than speak them, can repeat no more than two
words or four numbers at a time, and can repeat essentially no sentences.
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Results of a complete blood count, a basic metabolic panel, a serum vitamin B12 measurement, and
thyroid function tests are normal.
An MRI of the brain without contrast shows mild atrophy but is otherwise unremarkable.
This patient’s impairment in speech and writing is most likely due to which of the following disorders?
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5.
Alzheimer dementia
Creutzfeldt-Jakob disease
Dementia with Lewy bodies
Frontotemporal lobar degeneration
Vascular dementia
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An 81-year-old man is evaluated for the gradual onset and progression of memory loss over the
past year. He says he has difficulty recalling the names of familiar people, has misplaced his wallet
on numerous occasions, and is slower to find his car in large, crowded parking lots. He continues to
manage his finances, travel with his wife, and perform the activities of daily living without
difficulty. He has borderline hyperlipidemia that is managed by diet alone. A paternal uncle
developed Alzheimer dementia at age 74 years. His only medications are aspirin and a daily
multivitamin.
On physical examination, temperature is 36.7 °C (98.1 °F), blood pressure is 126/82 mm Hg, pulse
rate is 68/min, respiration rate is 14/min, and BMI is 26. His level of alertness, speech, and gait are
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normal. He scores 26/30 on the Folstein Mini–Mental State Examination,
the recall portion and one point on the orientation section for incorrectly stating today’s date.
Results of a complete blood count, serum vitamin B12 measurement, thyroid function tests, and a
basic metabolic panel are normal.
An MRI of the brain without contrast shows no abnormalities.
Which of the following is the most likely diagnosis at this time?
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2.
3.
4.
5.
Alzheimer dementia
Dementia with Lewy bodies
Frontotemporal dementia
Mild Cognitive impairment
Vascular dementia
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Dementia
• MMSE < 22 indicates dementia
• Alzheimer Disease: Insidious, progressive disease with significant impairment of normal
daily functioning. Persistent forgetfulness is hallmark.
• Cholinesterase inhibitors: donepezil, rivastigmine, galantamine
• N-methyl-d asparte R antagonist: memantine
Dementia
Dementia with Lewy bodies
Dementia, hallucinations, REM sleep disorder,
parkinsonism
Vascular
Abrupt onset with stepwise decline
Frontotemporal
Rapid onset dementia with change in
personality- disinhibition, impulsivity, loss of
verbal fluency
CJD
Rapid dementia with startle myoclonus
NPH
Dementia, gait ataxia, incontinence
Additional Dementia Treatments
A 62-year-old woman is evaluated for a 1-year history of tremor that affects
both upper extremities. She says that her handwriting has become sloppier
since she first noticed the tremor and that she occasionally spills her morning
coffee because of it. Although she feels otherwise healthy, she is concerned
that she may have Parkinson disease. The patient has a history of
hyperlipidemia controlled by diet and exercise but is otherwise healthy. Her
mother, who died at age 79 years, had a similar tremor. Her only medication is
a daily multivitamin.
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On examination, she has a mild tremor in the upper extremities that is present
with the arms extended and during finger-to-nose testing. No resting tremor is
apparent. Muscle tone and gait and limb coordination are normal.
Administration of which of the following drugs is the most appropriate
treatment of this patient?
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2.
3.
4.
Carbidopa-Levodopa
Clonazepam
Propranolol
Ropinirole
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A 53-year-old woman is evaluated in the office for a 4-month history of tremor. The
tremor affects both upper extremities and is present “most of the time.” She has a 15year history of type 2 diabetes mellitus; she also has a history of hypertension,
gastroparesis, and chronic kidney disease. Medications are insulin glargine, insulin
lispro, lisinopril, hydrochlorothiazide, and metoclopramide.
On examination, she has diminished pedal pulses. Speech, language, and mental status
are normal. Cranial nerve function is normal, although a paucity of facial expression is
noted. Movements are slow, and there is mild bilateral upper and lower extremity
rigidity. Deep tendon reflexes are normal, as are results of manual muscle strength
testing. Sensory examination reveals distal sensory loss. She had
a mildly
stooped
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posture but no postural instability. A 4-Hz resting tremor in both upper extremities is
noted, as is a prominent postural tremor.
Which of the following is the most likely diagnosis?
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2.
3.
4.
Dementia with Lewy bodies
Drug-induced parkinsonism
Multiple system atrophy
Parkinson disease
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Parkinson’s Disease
Symptoms: (the 4 R’s) initially unilateral disease
1. Resting tremor and cogwheeling
2. Rigidity and flexed posture
3. Retarded movement (brady and hypokinesia)
4. Loss of postural Reflexes
Drug
Mechanism
Adverse effect
Levodopa-carbidopa
Dopamine
replacement
DOC for older
patients
1. Motor fluctuations:
dopamine agonist or
amantadine
2. Wearing off: inc dose or
frequency
Ropinirole, Pramipexole
Dopamine
receptor agonist
DOC for <65yo
Impulse control disordershypersexual and gambling
Amantadine
NMDA receptor
antagonist
Mild symptoms,
tremor
AMS and psychosis
Selegiline, Rasagiline
MAO-B inhibitor
May delay need
for L-dopa
Seratonin syndrome if with
SSRIs or tricyclics
**Acutely discontinuing dopa therapy may cause NMS. Treat with a dopamine agonist:
bromocriptine, amantadine or dantrolene
Movement Disorders
Disorder
Key Points
Essential Tremor
Fine tremor that impairs manual tasks and is present with action.
Treat with propranolol or primidone.
Dystonia
Torsional, twisting movement. Focal dystonia should be treated with
injections of botulinum toxin A or B.
Huntington disease Neurodegenerative choric disorder. Generalized chorea, dysarthria,
parkinsonism, psychiatric disease, dementia, and death
Tardive Dyskinesia
Choreiform movement of the face. Caused by D2 blocking agents. May
persist when offending agent is withdrawn.
Myoclonus
Single, rapid, shocklike muscle jerk. Seen in CJD, anoxic brain injury,
stroke. Treat with anticonvulsant.
Wilson Disease
Curable metabolic disease due to irreversible copper deposition in the
basal ganglia and cerebellum. Kayser-Fleischer rings, “wing-beating”
tremor, young adults. Confirm diagnosis with low ceruloplasmin level.
Treat with penicillamine or liver transplantation.
NMS
Hyperthermia + extrapyramidal signs + delirium. D2 receptor blockers
can induce syndrome. Bromocriptine and dantrolene to reverse.
Restless Leg
Syndrome
Associated with IDA, uremia, DM, RA, fibromyalgia. Treat with
bromocriptine or pramipexole.
A 33-year-old man is evaluated for a 3-day history of worsening
weakness and numbness of the right arm and leg. He has a 5-year
history of multiple sclerosis. His only current medication is
glatiramer acetate.
On physical examination, temperature is 36.5 °C (97.7 °F), blood
pressure is 105/75 mm Hg, pulse rate is 68/min, and respiration rate
is 14/min. Moderate right arm and leg weakness, hyperreflexia, an
extensor plantar response, and vibratory sense impairment are
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noted.
Which of the following should this patient receive to treat his acute
relapse?
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2.
3.
4.
5.
Empiric antibiotic therapy
Immune globulin, intravenously
Methylprenisolone, intravenously
Plasmapheresis
Prednisone only
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Multiple Sclerosis Presenting Signs and
Symptoms
• Optic neuritis- acute loss of vision and pain with eye
movement
• Myelitis- inflammation of the spinal cord that causes
sensory or motor symptoms below the involved
spinal cord level
• Brainstem involvement- diplopia, sensations of
oscillopsia, internuclear ophthalmoplegia
• In contrast to cerebrovascular disease, cortical
syndromes, such as aphasia and neglect, do not
often occur in MS.
Multiple Sclerosis Diagnosis
• Demyelinating disease of CNS beginning at 20-30
yo; CNS lesions disseminated over time and space
• Dx with brain MRI ovoid white matter lesions in
periventricular and posterior fossa regions and
perpendicular to lateral ventricles
• Do LP if MRI inconclusive CSF with lymphocytic
pleocytosis; oligoclonal bands and elevated IgG
Multiple Sclerosis Treatment
Acute Relapse: distinguish
between pseudo (infection) or
true
• Observe if minimal symptoms
• IV methylprednisolone or
plasmapheresis
Disease Modifying Therapies
(relapsing remitting disease):
• Interferon beta 1a/b
• Glatiramer
• Natalizumab (assoc with PML)
• Fingolimod (only oral agent)
• Mitoxantrone
No therapies for primary
progressive
A 54-year-old man is evaluated for a 9-month history of progressively worsening paresthesia
in the legs that has recently spread to the hands and a 6-month history of an ataxic gait. The
patient developed a malabsorption syndrome 3 years ago after gastric bypass surgery. He
takes a daily multivitamin, iron, ascorbic acid, calcium, and vitamins D, B6, and B12.
On physical examination, temperature is 37.2 °C (99.0 °F), blood pressure is 110/60 mm Hg,
and pulse rate is 70/min; BMI is 31. Neurologic examination shows decreased vibratory
sensation in the hands and feet and decreased position sense in the feet. Hip flexion strength
is 4/5 bilaterally, and reflexes are 3+ throughout with upgoing toes.
Laboratory studies:
Hemoglobin 12.9 g/dL (129 g/L)
Mean corpuscular volume 102 fL
Vitamin B12 590 pg/mL (435 pmol/L)
Rapid plasma reagin Nonreactive
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T2-weighted MRIs of the cervical and thoracic spines show slight hyperintensity in the
posterior columns from approximately C4 through T9 with no contrast enhancement.
Which of the following serum levels should be measured next?
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2.
3.
4.
5.
Copper
Folate
25-hydroxyvitamin D
Thiamine
Vitamin A
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Diseases of the Spinal Cord
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Subacute Combined
degeneration of the spinal cord
Viral infection, Autoimmune dz or MS
Leg weakness, numbness, sensory level at trunk.
Dx: MRI, CSF with inc protein and lymphocytosis
Tx: plasmapheresis or cyclophosphamide
• B12 deficiency (even without anemia), copper deficiency,
nitrous oxide exposure
• Gradual weakness, paresthesias, spastic paresis, loss of
proprioception and ataxia.
• Dx: MRI, check MMA and HC (both inc with B12 def)
• Tx: replacement therapy will usually halt progression of but
may not improve symptoms
• Back Pain, fever, local tenderness, radicular pain + neuro
deficits
• Dx: MRI, blood cultures
• Tx: laminectomy, drainage and antibiotics
Epidural Abscess
A 34-year-old woman is evaluated in the office for right-sided facial paralysis that she
noticed on awakening 1 hour ago. She has a 10-pack-year smoking history. Personal
and family medical history is noncontributory. Her only medication is a daily oral
contraceptive.
On physical examination, temperature is 36.5 °C (97.7 °F), blood pressure is 110/70
mm Hg, pulse rate is 82/min, respiration rate is 14/min, and BMI is 26. Limb strength,
reflexes, and tone are normal bilaterally. Findings from a sensory examination, which
included her face, are also normal. When asked to raise her eyebrows, the patient
does not elevate the right side. When asked to shut her25%
eyes, she
the
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right one, but the globe rotates upward, partially covering the iris. When asked to
smile, the patient does not move the right side of her face.
Which of the following is the most likely diagnosis?
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2.
3.
4.
Graves opthalmopathy
Left cerebral infarction
Right facial nerve (Bell) palsy
Right trigeminal neuralgia
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Mononeuropathies
Radial neuropathy:
acute wrist drop.
Compression,
diabetes, lead.
Wrist splints, PT
Bell’s Palsy: ipsilateral facial paralysis
• Treat with prednisone if within 7 days of symptom
onset to shorten course and improve function..
• Cortical lesions spare forehead and eyelid
Median nerve
entrapment: CTS.
Entrapment at wrist
Splints, steroids,
surgery
Ulnar neuropathy:
Lesion at elbow or
cubital tunnel in
wrist
elbow pads, splints,
Peroneal nerve
compression: Foot
drop
Lesion at fibula
head
**L5 radiculopathy
also causes foot
drop, but cannot
invert OR evert foot.
Also weak thigh
muscles.
Polyneuropathies
Guillain Barre Syndrome
Chronic Inflammatory
Demyelinating
Polyneuropathy (CIDP)
Critical Illness Neuropathy
Ascending paralysis of
muscles with areflexia;
usually with preceding
Campylobacter infection
Symmetric sensorimotor
loss for > 8 weeks
Unable to wean from vent
and generalized weakness
with flacdid paralysis.
Sparing of cranial nerves.
CSF with normal cell count
and high protein; monitor
inspiratory force and
expiratory vital capacity
CSF with normal cell count
and high protein
Serum CK and CSF
protein levels are
normal
Plasmapheresis and IVIG
Steroids, plasmapheresis,
IVIG
Supportive treatment with
PT/OT; AVOID steroids
A 56-year-old man is evaluated in the office for a 1-month history of intermittent weakness of the left foot, a
6-month history of progressive right arm weakness, and 1-year history of muscle cramps. He says he feels
lately as if he is “catching” his foot on things when ambulating. He has noticed no shortness of breath,
dysphagia, or other bulbar symptoms and reports no other pain, sensory loss, or bowel or bladder
impairment. The patient is otherwise healthy, has no history of disease, and is unaware of any family history
of neurologic disorders. He takes no medications.
Results of a general medical examination are normal. Neurologic examination reveals normal speech,
language, and mental status. His tongue appears atrophic with fasciculations. He has diffuse weakness and
atrophy of the proximal muscles in the right arm; fasciculations are noted. Left arm strength and muscle bulk
are normal. Moderate weakness of the distal muscles in the left leg is noted, with fasciculations present in
both lower extremities. Deep tendon reflexes are brisk in the upper and
lower limbs,
the plantar
25%
25% and 25%
25%
response is extensor bilaterally. Sensory examination reveals no abnormalities, and there is no appendicular
ataxia.
Laboratory studies show a serum creatine kinase level of 602 U/L. Results of a complete blood count;
measurement of serum creatinine, electrolyte, and vitamin B12 levels; and liver chemistry studies are
normal.
A radiograph of the chest shows no abnormalities.
Which of the following is the most likely diagnosis?
1.
2.
3.
4.
Amyotrophic Lateral Sclerosis
Cervical Myelopathy
Chronic Inflammatory Demyeliting Polyradiculoneuropathy
1
Primary Lateral Sclerosis
2
3
4
A 56-year-old man is evaluated for a 6-month history of generalized fatigue, slowly progressive
weakness in the legs, difficulty climbing stairs and raising his arms above his head, and a 2month history of dry mouth and impotence. He reports that on two occasions in the past month
he almost lost consciousness when rising quickly from a sitting position. The patient has a 38pack-year smoking history.
On physical examination, blood pressure in the right arm is 130/80 sitting and 100/60 standing
and pulse rate is 80/min and regular sitting and 84/min and regular standing. Muscle strength
testing reveals moderate weakness in the arms and proximal legs, especially when the patient
rises from a low chair. Repeated testing slightly improves strength. Stretch reflexes are diffusely
reduced or absent but increase to normal after brief exercise. Results of cranial nerve testing,
cerebellar function testing, and sensory testing are normal.
Results of laboratory studies show an erythrocyte sedimentation rate of 75 mm/h and a serum
creatine kinase level of 120 units/L.
A chest radiograph is suspicious for left hilar lymphadenopathy.
Which of the following diagnostic tests should be performed next?
1. Acetylcholine receptor antibody assay
2. Lumbar puncture
3. Muscle biopsy
4. Nerve conduction studies
Neuromuscular junction disorders
Myasthenia Gravis
Lambert Eaton
Autoantibodies
Post synaptic AchReceptor
MuSK Receptor
Voltage gated calcium
channels
DTRs
Normal
Absent
Symptoms
Improve with rest
Improve with movement
Associated with
Thymoma
Small Cell lung cancer
Weakness
Descending
Proximal lower ext
Bulbar
Present
Less common
Treatment
Pyridostigmine
Pyridostigmine and treat
underlying malignancy
A 71-year-old man is evaluated in the intensive care unit 11 days after undergoing surgery to
relieve a bowel obstruction. His postoperative course has been complicated by septic shock and
multiorgan failure, for which he has received intravenous fluids, broad-spectrum antibiotics,
vasopressors, corticosteroids, and insulin. He has been on mechanical ventilation for 10 days.
For the past 72 hours, he has been hemodynamically stable, but attempts at weaning him from
the ventilator have been unsuccessful. The patient previously was given muscle relaxants and
neuromuscular junction–blocking agents, but these have been withheld for the past 4 days.
On physical examination, the patient is alert, follows commands, and cooperates with the
examiner. Vital signs are stable. Cranial nerves are intact. Flaccid quadriparesis of the upper and
25%
25%is present.
25%
25%
lower extremities is noted that is greater proximally than distally.
Areflexia
Results of laboratory studies show a serum creatine kinase level of 850 units/L and a plasma
glucose level of 200 mg/dL (11.1 mmol/L).
Results of electromyography show absent sensory responses in the legs and low amplitudes in
the hands. Short duration, low-amplitude motor units consistent with myopathy are noted.
Which of the following is the most likely diagnosis?
1.
2.
3.
4.
Corticosteroid myopathy
Critical illness myopathy
Guillain-Barre syndrome
Myasthenia gravis
1
2
3
4