The Finseth Review
Brief Cases in Clinical Neurology
Sections (click on link to jump to section):
Headache (pg. 1-6)
Traumatic Head Injury (pg. 6-8)
Vascular Neurology (pg. 8- 10)
Seizure/Syncope (pg. 10-15)
Cognitive Disorders (pg. 15-20)
Infectious Diseases (pg. 20-24)
Neuroophthalmology (pg. 24-28)
Movement Disorders (pg. 28-33)
Neuromuscular Disorders (pg. 33-43)
Neuroimmunology (pg. 43-45)
Spinal Cord (pg. 45-48)
Sleep Disorders (pg. 48-49)
Neurootology (pg. 50-53)
Neurooncology & Neurocutaneous Disorders (pg. 53-57)
Pediatric Neurology (pg. 57-60)
Neuropharmacology (pg. 60-61)
Index (pg. 62-65)
Note: Index includes rudimentary ratings of the relative importance of topics.
Statement of Purpose
This review is intended to supplement your medical education in neurology with
the primary purpose of preparing you for the Clinical Neurology “Shelf” Exam and the
Neurology portion of USMLE Step 2 Exam. It is also our hope that you come away with
knowledge that will help you take care of patients with neurological problems in your
future career. We have focused on the topics most likely to be tested on your “Shelf”:
1)common problems, their pathophysiology, diagnosis and treatment 2)emergent
problems and the next step and 3) clinical syndromes that have pathognomonic
characteristics, making them easily testable. We hope that seeing these clinical
syndromes in vignette format will help you recognize them on your shelf and accurately
diagnose them in your future patients. While the diagnostic workup and treatment plans
referred to in the review should suffice for your test and are up to date to the best of our
knowledge (as of 1/2011), you should refer to medical references when diagnosing and
treating patients. Best of luck to you in your clerkship and beyond.
Headache
Back to top
H1. A 23 yo M comes to the ER after he suddenly develops the worst headache of his
life. He states he had a brief but terrible headache about a week ago while doing some
yard work.
What is it? Subarachnoid Hemorrhage secondary to aneurysmal bleed. The prior self-
limited headache was a sentinel bleed of the aneurysm. The hyperacute rapid onset
1
,(thunderclap headache) is a clinical red flag for SAH rather than a migraine that evolves
more slowly.
How is it diagnosed? CT scan without contrast. Contrast is held so that blood will show
up bright white on the scan (be able to recognize blood in the subarachnoid space).
Treatment? Your next step would be angiography to locate the aneurysm and a
neurosurgical consult. This is done because the aneurysm can continue to bleed
leading to coma/death or if the bleeding has stopped, there is a significant chance of
rebleeding for the next 2 weeks, and in particular in the next 24 hours. Nimodipine is
given prophylactically to prevent the complication of cerebral vasospasm 4-14 days later
(which can cause ischemic injury).
H2. A 23 yo M comes to the ER after he suddenly developed the worst headache of his
life. He states he had a terrible headache about a week ago while doing some yard
work. CT scan of the head is negative.
What is it? It’s still a SAH due to aneurysmal bleed until proven otherwise. CT misses
roughly 10% of SAHs.
Next step? Perform a lumbar puncture. A positive lumbar puncture would show
xanthochromia (yellow color to fluid from RBC breakdown) or RBCs that do not clear
from tube 1-4.
Treatment? As described above.
H3. A 75 yo female presents to the ER with pain in her right temple. She complains of
some arthritis in her shoulders and hips as well for the past year. Her scalp is tender to
the touch. Lab studies show a mild microcytic anemia and markedly elevated ESR.
What is it? Temporal arteritis in a patient with polymyalgia rheumatica.
Next step? High dose steroids. Steroids should be given immediately to prevent
vascular occlusion of the temporal artery and permanent loss of vision to the eye. If
vision loss has already occurred, steroids are still given to prevent vision loss on the
contralateral side.
Diagnosis? Elevated ESR and temporal artery biopsy.
H4. A 27 yo M comes in with unilateral right neck and facial pain. The patient has
trouble talking to you and has trouble finding words. His friend relates that they were
playing tennis earlier in the day, and he was hit in the neck with a tennis ball. He had
finished the game without much complaining but later was complaining of pain. On
exam, you notice the patient has ptosis and a constricted pupil on the right.
What is it? Carotid Dissection resulting in Horner’s syndrome and ischemic stroke.
Horner’s and stroke need not be present but can be sequelae.
Diagnosis? MR or CT Angiography or conventional angiogram.
Treatment? Probably best to just recognize this on your shelf. Anticoagulation vs
antiplatelet therapy would be initiated to prevent formation of thrombus in the dissection.
H5. A 45 yo F migraine patient presents with a chronic daily headache for the past 3
months. The headache is diffuse and dull and occurs everyday. She states she was
taking frequent analgesic pain meds for migraines and then slowly developed a daily,
dull headache that won’t go away. She is currently taking oxycodone every 4-6 hours,
which only partially relieves her headache. Her pain has prevented her from achieving
good sleep but does not wake her from sleep. Fundus exam shows no papilledema. No
2
,nocturnal worsening or symptoms. No worsening of symptoms with straining. No focal
neurological deficits or visual changes.
What is it? Analgesic rebound headache.
Diagnosis? Made clinically based on chronic, frequent use (3 days a week or more) of
abortant or analgesic pain medications transforming intermittent headaches into a
chronic daily headache. There also should not be any red flag symptoms for
tumor/increased ICP or other secondary cause of headache.
Treatment? Cessation of frequent use of acute analgesic medications and transitioning
to medications without analgesic rebound potential.
H6. A 45 yo M presents with a headache that has gradually worsened for the past two
months. He describes a pressure like pain that is worse in the morning but persists
throughout the day. Fundoscopic exam shows papilledema.
What is it? Intracranial mass leading to increased intracranial pressure. Any subacute
progressive headache should raise suspicion for a brain tumor. Pain that is worse in the
morning/middle of the night after being supine and papilledema are signs of increased
ICP.
Diagnosis? MRI with contrast to look for tumor.
Treatment? If MRI +, therapy will potentially involve chemotherapy, radiation, surgery or
hospice care. Dexamethasone would be indicated if symptomatic mass effect was
present. If multifocal pattern on MRI suggesting metastasis, search for a primary cancer
should follow with lung, melanoma, and breast as the most likely culprits.
H7. A 65 yo M presents to the hospital with headache for the past 12 hours and
confusion. He has had a fever up to 103. On exam, patient complains of pain with neck
flexion and flexes his hips when you perform this maneuver. No focal signs are present.
Multiple ecchymotic/petechial patches are present over trunk and extremities.
What is it? Bacterial meningitis. Any patient with fever, headache, and neck stiffness
should be suspected of having meningitis. His skin findings make N. Meningitidis as the
likely culprit for his infection.
Diagnosis? Lumbar puncture with glucose, protein, WBC, RBC and gram stain, and
cultures. Blood cultures would be drawn as well. If focal signs were present, an
abscess might be present leading to increased ICP, and therefore a head CT should be
performed prior to LP to avoid herniation, though empirically treating with antibiotics
should not be delayed while waiting to be able to obtain LP with clinical suspicion of
bacterial meningitis. Bacterial meningitis shows depressed glucose with elevated white
count and a neutrophil predominance. Viral would have a near normal glucose with
elevated white count and a lymphocytic predominance.
Treatment? Empiric IV antibiotics - specifically ceftriaxone for bacterial meningitis, with
vancomycin initially to cover penicillin-resistant strains while csf and blood cultures are
pending. Ampicillin added for certain populations, such as the elderly,
immunocompromised, etc.
H8. A 33 year old male presents to the ED with a severe headache and confusion. The
headache is severe and constant and has been progressing for the past 24 hours. It
was submaximal at onset. He denies fever or neck stiffness. Vital signs reveal he is
afebrile with pulse of 120, BP 210/130. Exam reveals papilledema. Lab studies are
remarkable for creatinine of 2.3. CT and LP are negative.
3
, What is the most likely diagnosis? Hypertensive encephalopathy. This is a diagnosis of
exclusion but suspect in a patient with elevated blood pressure, papilledema and without
signs of ICH, stroke or meningitis.
Treatment? Acute reduction in blood pressure by 25% and diastolic to 100-110.
Labetolol and nicardipine are preferred agents.
Of note, acutely elevated BP/hypertensive encephalopathy may cause posterior signal
on an MRI of the brain and is called PRES (posterior reversible encephalopathy
syndrome). In addition to headache and confusion, cortical visual disturbance and
seizures can be seen. The treatment is similar to above. This can be seen in
eclampsia, as well, and even in normotensive patients on cytotoxic agents such as
cyclosporine.
H9. A 25 yo obese F presents with a constant headache for the past few months. She
reports that headaches are worst in the morning. She also reports sudden loss of vision
for 1 to 2 seconds that occurs intermittently as well as ringing in her ears that coincides
with her heartbeat. Fundus shows papilledema. An emergent head CT shows no
abnormalities.
What is it? Pseudotumor cerebri (idiopathic intracranial hypertension). This condition
tends to occur in young, obese females who will complain of chronic headaches and
may have associated symptoms of transient visual obscurations (loss of vision lasting
seconds) or pulsatile tinnitus or gradual visual field loss if severe. Look for Isotretinoin
use in the vignette, as the drug can cause this condition as a side effect.
Diagnosis? Head CT is performed to rule out a mass lesion or hydrocephalus (dilated
ventricles). LP with elevated opening pressure (greater than 20-25 for diagnosis and
potentially much higher). Formal visual field evaluation could confirm visual field loss.
Treatment? LP can be temporarily therapeutic by withdrawing fluid to normalize
pressure. Acetazolamide is a first line medical therapy that can decrease CSF
production. Lasix may also be used. Weight loss may help. In cases with visual field
loss, optic nerve sheath fenestrations should be considered to relieve pressure on the
optic nerve and prevent further vision loss. Ventriculoperitoneal shunts may be
considered in refractory cases.
H10. A 30 yo F patient is admitted for left sided paresthesias. Yesterday, while in the
hospital, she had an MRI, which showed some demyelination and lumbar puncture
which showed oligoclonal bands. While discussing with her the possibility of multiple
sclerosis, she complains to you that she has been having a severe headache every time
she gets up to use the restroom since yesterday. Once she returns to her bed and calls
the nurse, her headache is always better when the nurse arrives.
What is it? Post-lumbar puncture headache (intracranial hypotension headache). The
loss of fluid from the lumbar puncture and continued dural leak will cause a low pressure
in the CSF that can lead to headache while sitting/standing that is relieved by lying flat.
Diagnosis? Usually clinical. An MRI could show diffuse meningeal enhancement +/-
sagging of cerebellar tonsils (similar to Chiari malformation) on sagittal views.
Treatment? The process is typically self-limited and will generally resolve within 2-5
days. Fluids and bedrest will often be enough. Caffeine (cerebral vasoconstrictor) may
provide some relief. A blood patch placed in the epidural space could be utilized for
closing off refractory dural leaks.
4
Brief Cases in Clinical Neurology
Sections (click on link to jump to section):
Headache (pg. 1-6)
Traumatic Head Injury (pg. 6-8)
Vascular Neurology (pg. 8- 10)
Seizure/Syncope (pg. 10-15)
Cognitive Disorders (pg. 15-20)
Infectious Diseases (pg. 20-24)
Neuroophthalmology (pg. 24-28)
Movement Disorders (pg. 28-33)
Neuromuscular Disorders (pg. 33-43)
Neuroimmunology (pg. 43-45)
Spinal Cord (pg. 45-48)
Sleep Disorders (pg. 48-49)
Neurootology (pg. 50-53)
Neurooncology & Neurocutaneous Disorders (pg. 53-57)
Pediatric Neurology (pg. 57-60)
Neuropharmacology (pg. 60-61)
Index (pg. 62-65)
Note: Index includes rudimentary ratings of the relative importance of topics.
Statement of Purpose
This review is intended to supplement your medical education in neurology with
the primary purpose of preparing you for the Clinical Neurology “Shelf” Exam and the
Neurology portion of USMLE Step 2 Exam. It is also our hope that you come away with
knowledge that will help you take care of patients with neurological problems in your
future career. We have focused on the topics most likely to be tested on your “Shelf”:
1)common problems, their pathophysiology, diagnosis and treatment 2)emergent
problems and the next step and 3) clinical syndromes that have pathognomonic
characteristics, making them easily testable. We hope that seeing these clinical
syndromes in vignette format will help you recognize them on your shelf and accurately
diagnose them in your future patients. While the diagnostic workup and treatment plans
referred to in the review should suffice for your test and are up to date to the best of our
knowledge (as of 1/2011), you should refer to medical references when diagnosing and
treating patients. Best of luck to you in your clerkship and beyond.
Headache
Back to top
H1. A 23 yo M comes to the ER after he suddenly develops the worst headache of his
life. He states he had a brief but terrible headache about a week ago while doing some
yard work.
What is it? Subarachnoid Hemorrhage secondary to aneurysmal bleed. The prior self-
limited headache was a sentinel bleed of the aneurysm. The hyperacute rapid onset
1
,(thunderclap headache) is a clinical red flag for SAH rather than a migraine that evolves
more slowly.
How is it diagnosed? CT scan without contrast. Contrast is held so that blood will show
up bright white on the scan (be able to recognize blood in the subarachnoid space).
Treatment? Your next step would be angiography to locate the aneurysm and a
neurosurgical consult. This is done because the aneurysm can continue to bleed
leading to coma/death or if the bleeding has stopped, there is a significant chance of
rebleeding for the next 2 weeks, and in particular in the next 24 hours. Nimodipine is
given prophylactically to prevent the complication of cerebral vasospasm 4-14 days later
(which can cause ischemic injury).
H2. A 23 yo M comes to the ER after he suddenly developed the worst headache of his
life. He states he had a terrible headache about a week ago while doing some yard
work. CT scan of the head is negative.
What is it? It’s still a SAH due to aneurysmal bleed until proven otherwise. CT misses
roughly 10% of SAHs.
Next step? Perform a lumbar puncture. A positive lumbar puncture would show
xanthochromia (yellow color to fluid from RBC breakdown) or RBCs that do not clear
from tube 1-4.
Treatment? As described above.
H3. A 75 yo female presents to the ER with pain in her right temple. She complains of
some arthritis in her shoulders and hips as well for the past year. Her scalp is tender to
the touch. Lab studies show a mild microcytic anemia and markedly elevated ESR.
What is it? Temporal arteritis in a patient with polymyalgia rheumatica.
Next step? High dose steroids. Steroids should be given immediately to prevent
vascular occlusion of the temporal artery and permanent loss of vision to the eye. If
vision loss has already occurred, steroids are still given to prevent vision loss on the
contralateral side.
Diagnosis? Elevated ESR and temporal artery biopsy.
H4. A 27 yo M comes in with unilateral right neck and facial pain. The patient has
trouble talking to you and has trouble finding words. His friend relates that they were
playing tennis earlier in the day, and he was hit in the neck with a tennis ball. He had
finished the game without much complaining but later was complaining of pain. On
exam, you notice the patient has ptosis and a constricted pupil on the right.
What is it? Carotid Dissection resulting in Horner’s syndrome and ischemic stroke.
Horner’s and stroke need not be present but can be sequelae.
Diagnosis? MR or CT Angiography or conventional angiogram.
Treatment? Probably best to just recognize this on your shelf. Anticoagulation vs
antiplatelet therapy would be initiated to prevent formation of thrombus in the dissection.
H5. A 45 yo F migraine patient presents with a chronic daily headache for the past 3
months. The headache is diffuse and dull and occurs everyday. She states she was
taking frequent analgesic pain meds for migraines and then slowly developed a daily,
dull headache that won’t go away. She is currently taking oxycodone every 4-6 hours,
which only partially relieves her headache. Her pain has prevented her from achieving
good sleep but does not wake her from sleep. Fundus exam shows no papilledema. No
2
,nocturnal worsening or symptoms. No worsening of symptoms with straining. No focal
neurological deficits or visual changes.
What is it? Analgesic rebound headache.
Diagnosis? Made clinically based on chronic, frequent use (3 days a week or more) of
abortant or analgesic pain medications transforming intermittent headaches into a
chronic daily headache. There also should not be any red flag symptoms for
tumor/increased ICP or other secondary cause of headache.
Treatment? Cessation of frequent use of acute analgesic medications and transitioning
to medications without analgesic rebound potential.
H6. A 45 yo M presents with a headache that has gradually worsened for the past two
months. He describes a pressure like pain that is worse in the morning but persists
throughout the day. Fundoscopic exam shows papilledema.
What is it? Intracranial mass leading to increased intracranial pressure. Any subacute
progressive headache should raise suspicion for a brain tumor. Pain that is worse in the
morning/middle of the night after being supine and papilledema are signs of increased
ICP.
Diagnosis? MRI with contrast to look for tumor.
Treatment? If MRI +, therapy will potentially involve chemotherapy, radiation, surgery or
hospice care. Dexamethasone would be indicated if symptomatic mass effect was
present. If multifocal pattern on MRI suggesting metastasis, search for a primary cancer
should follow with lung, melanoma, and breast as the most likely culprits.
H7. A 65 yo M presents to the hospital with headache for the past 12 hours and
confusion. He has had a fever up to 103. On exam, patient complains of pain with neck
flexion and flexes his hips when you perform this maneuver. No focal signs are present.
Multiple ecchymotic/petechial patches are present over trunk and extremities.
What is it? Bacterial meningitis. Any patient with fever, headache, and neck stiffness
should be suspected of having meningitis. His skin findings make N. Meningitidis as the
likely culprit for his infection.
Diagnosis? Lumbar puncture with glucose, protein, WBC, RBC and gram stain, and
cultures. Blood cultures would be drawn as well. If focal signs were present, an
abscess might be present leading to increased ICP, and therefore a head CT should be
performed prior to LP to avoid herniation, though empirically treating with antibiotics
should not be delayed while waiting to be able to obtain LP with clinical suspicion of
bacterial meningitis. Bacterial meningitis shows depressed glucose with elevated white
count and a neutrophil predominance. Viral would have a near normal glucose with
elevated white count and a lymphocytic predominance.
Treatment? Empiric IV antibiotics - specifically ceftriaxone for bacterial meningitis, with
vancomycin initially to cover penicillin-resistant strains while csf and blood cultures are
pending. Ampicillin added for certain populations, such as the elderly,
immunocompromised, etc.
H8. A 33 year old male presents to the ED with a severe headache and confusion. The
headache is severe and constant and has been progressing for the past 24 hours. It
was submaximal at onset. He denies fever or neck stiffness. Vital signs reveal he is
afebrile with pulse of 120, BP 210/130. Exam reveals papilledema. Lab studies are
remarkable for creatinine of 2.3. CT and LP are negative.
3
, What is the most likely diagnosis? Hypertensive encephalopathy. This is a diagnosis of
exclusion but suspect in a patient with elevated blood pressure, papilledema and without
signs of ICH, stroke or meningitis.
Treatment? Acute reduction in blood pressure by 25% and diastolic to 100-110.
Labetolol and nicardipine are preferred agents.
Of note, acutely elevated BP/hypertensive encephalopathy may cause posterior signal
on an MRI of the brain and is called PRES (posterior reversible encephalopathy
syndrome). In addition to headache and confusion, cortical visual disturbance and
seizures can be seen. The treatment is similar to above. This can be seen in
eclampsia, as well, and even in normotensive patients on cytotoxic agents such as
cyclosporine.
H9. A 25 yo obese F presents with a constant headache for the past few months. She
reports that headaches are worst in the morning. She also reports sudden loss of vision
for 1 to 2 seconds that occurs intermittently as well as ringing in her ears that coincides
with her heartbeat. Fundus shows papilledema. An emergent head CT shows no
abnormalities.
What is it? Pseudotumor cerebri (idiopathic intracranial hypertension). This condition
tends to occur in young, obese females who will complain of chronic headaches and
may have associated symptoms of transient visual obscurations (loss of vision lasting
seconds) or pulsatile tinnitus or gradual visual field loss if severe. Look for Isotretinoin
use in the vignette, as the drug can cause this condition as a side effect.
Diagnosis? Head CT is performed to rule out a mass lesion or hydrocephalus (dilated
ventricles). LP with elevated opening pressure (greater than 20-25 for diagnosis and
potentially much higher). Formal visual field evaluation could confirm visual field loss.
Treatment? LP can be temporarily therapeutic by withdrawing fluid to normalize
pressure. Acetazolamide is a first line medical therapy that can decrease CSF
production. Lasix may also be used. Weight loss may help. In cases with visual field
loss, optic nerve sheath fenestrations should be considered to relieve pressure on the
optic nerve and prevent further vision loss. Ventriculoperitoneal shunts may be
considered in refractory cases.
H10. A 30 yo F patient is admitted for left sided paresthesias. Yesterday, while in the
hospital, she had an MRI, which showed some demyelination and lumbar puncture
which showed oligoclonal bands. While discussing with her the possibility of multiple
sclerosis, she complains to you that she has been having a severe headache every time
she gets up to use the restroom since yesterday. Once she returns to her bed and calls
the nurse, her headache is always better when the nurse arrives.
What is it? Post-lumbar puncture headache (intracranial hypotension headache). The
loss of fluid from the lumbar puncture and continued dural leak will cause a low pressure
in the CSF that can lead to headache while sitting/standing that is relieved by lying flat.
Diagnosis? Usually clinical. An MRI could show diffuse meningeal enhancement +/-
sagging of cerebellar tonsils (similar to Chiari malformation) on sagittal views.
Treatment? The process is typically self-limited and will generally resolve within 2-5
days. Fluids and bedrest will often be enough. Caffeine (cerebral vasoconstrictor) may
provide some relief. A blood patch placed in the epidural space could be utilized for
closing off refractory dural leaks.
4
