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CAN’T MISS CASES IN NEUROLOGY October 24, 2019 – Bernard S. Chang, M.D., M.M.Sc. Primary Care Internal Medicine course

DISCLOSURES

None

2 Can’t Miss Cases in Neurology | October 2019

CASES

1 Episodic spells


Case 1: Episodic Spells

A 33-year-old woman has “spells” involving a feeling of déjà vu followed by a loss of awareness. During this time she stares and repetitively smacks her lips for 30 seconds, followed by a few minutes of confusion. These have occurred about once a month for the past few months.

Which of the following is an appropriate next step?

A. Watchful waiting

B. Order a brain MRI and EEG

C. Begin ethosuximide therapy

D. Begin valproic acid therapy

E. Restrict driving to daytime only

Can’t Miss Cases in Neurology | October 2019 4

Differential Diagnosis of Episodic Spells

Episodic Spells: Focal seizures with impaired awareness

Type of seizure that can be characterized by unresponsiveness, automatisms, and postictal confusion Originate in a localized seizure focus within the brain Déjà vu and oral automatisms are suggestive of mesial temporal lobe onset


Episodic Spells: Evaluation for Possible Seizures

EEG → normal EEG does not rule out seizure Laboratory → CBC, electrolyte and glucose levels, toxicology screen Brain MRI → head CT if MRI contraindicated • Mesial temporal lobe sclerosis not well visualized on

CT CSF examination → if fever, altered mental status, severe headache


Diagnostic Tests in the Evaluation of Possible Seizures

Can’t Miss Cases in Neurology | October 2019

Seizure Therapy General Rules

Risk of recurrence • After first seizure → 25% to 40% • After second seizure → 80% to 90% • Risk of recurrence ↑ with abnormal EEG, abnormal MRI,

or history of potential cause (head trauma, stroke) Chronic therapy typically started after second unprovoked seizure


Seizure Therapy General Rules

Single-agent therapy • ↑ Dosage until seizures are controlled or side

effects occur • If unsuccessful, initiate a second drug as

adjunctive therapy and then try tapering first agent Drug levels

− Let clinical response guide therapy − Drug levels mostly help assess possible drug

toxicity


Case 1: Episodic Spells

A 33-year-old woman has “spells” involving a feeling of déjà vu followed by a loss of awareness. During this time she stares and repetitively smacks her lips for 30 seconds, followed by a few minutes of confusion. These have occurred about once a month for the past few months.


A. Watchful waiting

B. Order a brain MRI and EEG

C. Begin ethosuximide therapy

D. Begin valproic acid therapy

E. Restrict driving to daytime only

Can’t Miss Cases in Neurology | October 2019 11

CASES

1 Episodic spells 2 Facial asymmetry


Case 2: Facial Asymmetry

A 35-year-old man is evaluated 3 hours after he woke up with a droopy right face, noticed by himself in the mirror.


A. Send him to the ED for acute stroke workup B. Nonurgent MRI to evaluate for left intracranial lesion C. EMG to evaluate facial nerve function D. Begin prednisone and valacyclovir E. Begin anti-platelet therapy with aspirin or clopidogrel

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http://physicaltherapyprogramshq.blogspot.com


Neuroanatomy of Facial Asymmetry


Berkowitz, Clinical Neurology and Neuroanatomy: A Localization-Based Approach, 2017

Acute Ischemic Stroke Evaluation

TIA associated with ↑ risk of subsequent stroke within 48 h Hospitalize all acute TIA/stroke patients for urgent evaluation −Emergent CT scan for stroke or TIA → rule out intracranial

hemorrhage −Baseline PT and aPTT −Cardiac monitoring (evaluate for atrial fibrillation) −Echocardiography (if cardiac etiology suspected) −Carotid artery ultrasonography, head/neck MRA or CTA within

2-3 days


Ischemic Stroke Acute Therapy

Intubation and mechanical ventilation if ↓ level of consciousness

Recombinant tissue plasminogen activator (rt-PA) → ischemic stroke within 3 h of onset −If unknown onset → 3 hours of the last time the

patient was seen to be well

−rt-PA may be administered up to 4.5 h if no high risk factors for hemorrhage

−Age >80 y, severe (large territory) stroke, DM with previous stroke, and any anticoagulant use


Secondary Prophylaxis for Ischemic Stroke

Antiplatelets • Aspirin plus dipyridamole, clopidogrel Anticoagulation • Atrial fibrillation, left atrial thrombus, dilated

cardiomyopathy Carotid endarterectomy • Ipsilateral stenosis >70% if patient likely to live 5 years Statins • All patients regardless of cholesterol level Long-term control of hypertension • <140/90 mm Hg after acute event 17 Can’t Miss Cases in Neurology | October 2019

Imaging of acute ischemic stroke and intracranial hemorrhage


Intracranial Hemorrhage

Most common risk factor is hypertension

Presentation − Often similar to ischemic stroke

− May not be able to be distinguished clinically

Diagnosis • Study of choice → CT without contrast

• Cerebral angiography for patients <45 years old and cocaine use

− ↑ Risk of vascular abnormalities


Therapy for intracranial hemorrhage

• Reverse anticoagulation

− Use appropriate reversal agent → IV vitamin K, fresh frozen plasma, or prothrombin complex concentrates

• ↓ Intracranial pressure

− Mannitol, barbiturate coma, hyperventilation

• Maintain SBP 140-160 mm Hg (or MAP 70-130 mm Hg)

− Labetalol or nicardipine

• Ventricular drainage or decompression for increased ICP

• Surgical evacuation if needed


Bell’s Palsy

Unexplained episode of facial muscle weakness on one side, localizable to the facial (VII) nerve Unknown cause, many associations, likely related to inflammation Acute onset, worse over a few days, better starting within two weeks but could take months Steroids, antiviral agents, supportive therapy for face and eye


Case 2: Facial Asymmetry

A 35-year-old man is evaluated 3 hours after he woke up with a droopy right face, noticed by himself in the mirror.


A. Send him to the ED for acute stroke workup B. Nonurgent MRI to evaluate for left intracranial lesion C. EMG to evaluate facial nerve function D. Begin prednisone and valacyclovir E. Begin anti-platelet therapy with aspirin or clopidogrel

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http://physicaltherapyprogramshq.blogspot.com


CASES

1 Episodic spells 2 Facial asymmetry 3 Rapidly progressive weakness


Case 3: Rapidly Progressive Weakness

A 43-year-old woman has noticed progressive leg weakness over the past week. First she began having trouble getting out of automobiles, standing up from a chair, and climbing or descending stairs. The weakness progressed and by two days ago she began having difficulty raising her arms to wash her hair and turning a key in the lock of her front door. She has also noted bilateral upper and lower extremity numbness during this time period.

Which of the following is the most likely diagnosis?

A. A disorder of peripheral nerves

B. A disorder of muscles

C. A disorder of motor neurons

D. A disorder of the cerebral hemispheres

E. A disorder of the spinal cord


Pattern Recognition in Rapidly Progressive Weakness


Peripheral Neuropathy Overview

• Typical symptoms → pain, paresthesias, weakness, or autonomic dysfunction • Small fiber axonal neuropathy → pain and paresthesias of hands and feet

without weakness • Mononeuropathies → isolated disorders of a single peripheral nerve • Mononeuropathy multiplex → multiple noncontiguous peripheral nerves

− Systemic disease (vasculitis, sarcoidosis)

• Polyneuropathy → diffuse, generalized, usually symmetric peripheral neuropathy − Systemic disease (diabetes), toxin (alcohol), medication


Peripheral Neuropathy Syndromes

Diabetic amyotrophy • Severe leg pain, proximal weakness, atrophy Guillain-Barré syndrome • Acute ascending, areflexic paralysis, paresthesias • Therapy → plasma exchange, IV immune globulin Chronic inflammatory demyelinating polyneuropathy • Chronic progressive motor and sensory neuropathy • Therapy → prednisone, plasma exchange, IV immune

globulin


Case 3: Rapidly Progressive Weakness

A 43-year-old woman has noticed progressive leg weakness over the past week. First she began having trouble getting out of automobiles, standing up from a chair, and climbing or descending stairs. The weakness progressed and by two days ago she began having difficulty raising her arms to wash her hair and turning a key in the lock of her front door. She has also noted bilateral upper and lower extremity numbness during this time period.

Which of the following is the most likely diagnosis?

A. A disorder of peripheral nerves

B. A disorder of muscles

C. A disorder of motor neurons

D. A disorder of the cerebral hemispheres

E. A disorder of the spinal cord


CASES

1 Episodic spells 2 Facial asymmetry 3 Rapidly progressive weakness 4 Memory loss


Case 4: Memory Loss

An 81-year-old man presents with cognitive decline over several years, according to his family. (He himself believes they are overreacting.) He has been repeating himself frequently and loses things around the house. He was formerly an active volunteer and golfer but can no longer participate in those activities. Once he got lost while driving a familiar route home.

Which of the following might be an appropriate treatment?

A. Carbidopa-levodopa

B. Gabapentin C. Riluzole D. Tetrabenazine E. Memantine 30 Can’t Miss Cases in Neurology | October 2019


Evaluation for Memory Loss

Neuroimaging to look for: mass, subdural hematoma, stroke(s), pattern of atrophy suggestive of dementia Laboratory evaluation to look for potentially treatable causes of cognitive decline: Vitamin B12, TSH, RPR, HIV Formal neuropsychological testing to evaluate for particular pattern of cognitive deficits Other types of evaluation to consider: sleep study for sleep apnea, evaluation for depression (“pseudodementia”)


Statistics on Alzheimer’s, the most common form of dementia • Approximately 5 million individuals over age 65 in the US have Alzheimer’s (1

in 9 individuals > age 65). • Average lifespan after diagnosis of Alzheimer’s is 4-8 years (but can be up to

20 depending on how early diagnosis is made and social support structures) • Mini-mental state exam (MMSE) in Alzheimer’s score drops by 2-4 points per

year • At time of advanced dementia (inability to recognize family, limited speech,

total dependence), median survival ~1.3 years (death often due to infections and eating problems)


Treatment for Alzheimer’s Dementia

Cholinesterase inhibitors are modestly effective: • donepezil • galantamine • rivastigmine

Memantine (NMDA receptor antagonist) can be used for moderate AD


66-year-old woman brought in by her family for progressive changes in her personality over 5 years. Had become increasingly short-tempered, irritable, and disheveled. She would also complain of headaches upon awakening sometimes.

35

The Need for Full Evaluation of Cognitive Changes


Case 4: Memory Loss

An 81-year-old man presents with cognitive decline over several years, according to his family. (He himself believes they are overreacting.) He has been repeating himself frequently and loses things around the house. He was formerly an active volunteer and golfer but can no longer participate in those activities. Once he got lost while driving a familiar route home.

Which of the following might be an appropriate treatment?

A. Carbidopa-levodopa

B. Gabapentin C. Riluzole D. Tetrabenazine E. Memantine 36 Can’t Miss Cases in Neurology | October 2019

KEY POINTS


There are two pieces of information that contribute to making a neurological diagnosis: 1) Identifying the location of the problem in the nervous system

From the pattern of signs and symptoms From knowledge of very basic anatomy 2) Understanding the time course and progression of the illness

NEXT BEST STEPS


Always keep in mind for any neurological complaint: 1) What part of the nervous system might be involved?

Brain, spinal cord, peripheral nerves, muscles, etc.

2) What kind of disease process might be at work? Is it a process that can be imaged, or subject to neurophysiological testing, or further evaluated clinically?

Documents

Can’t Miss Cases in Neurology - Blank Titleprimarycareinternalmedicine2018.com/uploads/1/2/2/...3 Rapidly progressive weakness . Can’t Miss Cases in Neurology | October 2019 23