Neurology #3

Question 1

Although a Bell Palsy may be idiopathic, it may also be related to

Answer 1

A herpes simplex virus reactivation

Question 2

Symptoms of a Bell Palsy

Answer 2

-ipsilateral hyperacusis (ear pain)
-unilateral facial weakness or paralysis involving the forehead
-taste disturbance
(weakness and paralysis ONLY affects the face)

Question 3

Although no treatment is required for a Bell Palsy, what medication, if started in the first 72 hours of symptom onset, reduces the time to fully recovery and increases likelihood of complete recuperation?

Answer 3

Prednisone

Question 4

What is the pathophysiology Guillain Barre Syndrome?

Answer 4

Autoantibody attacks the myelin sheath of peripheral nerves after an infection

Question 5

Etiologies of Guillain Barre Syndrome

Answer 5

• Campylobacter Jejuni (MC)
• GI or respiratory infections
• EBV, HIV
• Immunizations

Question 6

Symptoms of Guillain Barre Syndrome

Answer 6

• Symmetric ascending weakness and sensory changes
• Weakness of respiratory muscles (breathing difficulties)
• Decreased DTRs

Question 7

Diagnostics for Guillain Barre Syndrome

Answer 7

• Electrophysiologic studies: nerve conduction and needle electromyography (most specific)
• CSF analysis: high protein with normal WBC count

Question 8

Treatment for Guillain Barre Syndrome

Answer 8

• Plasmapheresis or IVIG are first line

- Mechanical ventilation if needed

Question 9

Myasthenia gravis is an autoimmune peripheral nerve disorder due to autoantibodies against

Answer 9

the acetylcholine receptor at neuromuscular junction on muscles, leading to weakness

Question 10

Myasthenia Gravis is MC in young women and older men. It has a strong association with what genetic things?

Answer 10

• Abnormal thymus gland (thymoma)
• HLA-B8
• HLA-DR3

Question 11

Symptoms of Myasthenia Gravis

Answer 11

• Ocular weakness: diplopia and ptosis
• Generalized weakness worse with repeated muscle use
• Respiratory weakness = myasthenia crisis

Question 12

In an outpatient setting, what is the diagnostic of choice for myasthenia gravis?

Answer 12

• Acetylcholine receptor antibodies (initial)
• MuSK antibodies if ACR negative
• Electrophysiology testing: most accurate
• CXR, CT, MRI to determine if thymus gland abnormal in all patients

Question 13

Diagnostics for Myasthenia Gravis in an emergent setting

Answer 13

• Edrophonium (Tensilon) Test: brief improvement after administration
• Ice pack test: to improve ocular symptoms

Question 14

If the patient is having a myasthenic crisis, what is the treatment?

Answer 14

-Plasmapheresis or IVIG

Question 15

For long-term treatment of myasthenia gravis, use…

Answer 15

-Acetylcholinesterase inhibitors: Pyridostigmine or Neostigmine

Question 16

What are some medications that can exacerbate myasthenia gravis?

Answer 16

• Fluoroquinolones (-floxacin)
• Aminoglycosides (gentamicin, tobramycin, etc.)
• Beta Blockers

Question 17

Multiple sclerosis is an autoimmune, inflammatory demyelinating disease of the CNS that is associated with

Answer 17

axon degeneration of the white matter

Question 18

MC type of multiple sclerosis

Answer 18

-Relapsing-remitting: episodic exacerbations

Question 19

Symptoms of Multiple Sclerosis

Answer 19

• Sensory disturbances followed by weakness and visual disturbances
• Diplopia, optic neuritis
• Trigeminal neuralgia

Question 20

Physical exam findings of a patient with MS

Answer 20

-Upper motor neuron signs: spasticity, upward Babinski, hyperreflexia, rigidity

Question 21

Regarding MS, what is Lhermitte’s Sign?

Answer 21

Neck flexion causes lightning-shock type pain radiating from the spine down the leg

Question 22

What is Uhtoff’s Phenomenon in MS?

Answer 22

-Worsening of symptoms with heat (exercise, fever, hot tub, weather, etc.)

Question 23

What is a Marcus-Gunn Pupil in MS?

Answer 23

-during swinging flashlight test, from unaffected eye into the affected eye, the pupils dilate

Question 24

Best initial and most accurate diagnostic for Multiple Sclerosis? What does it show?

Answer 24

MRI with gadolinium

-Hyperintense white matter plaques

Question 25

However, what does an LP show in MS? This is only performed if the MRI is negative

Answer 25

-Increased IgG and oligoclonal bands

Question 26

First line treatment for MS

Answer 26

``` IV glucocorticoids (high dose) -Plasmapheresis if no response to IV glucocorticoids ```

Question 27

What are the first line medications given to prevent relapse and progression of MS

Answer 27

-Beta-interferon or Glatiramer

Question 28

Most benign type of all astrocytomas and are most common in children

Answer 28

-Pilocytic Astrocytoma

Question 29

MC primary CNS tumor in adults

Answer 29

Glioblastoma Multiforme

Question 30

Symptoms of an astrocytoma

Answer 30

- Headaches that wake patients up at night | - Increased intracranial pressure: headache, nausea, vomiting, stupor, ataxia

Question 31

Diagnostics for astrocytoma

Answer 31

- CT or MRI with contrast | - Brain biopsy: most diagnostic

Question 32

Treatment for astrocytoma

Answer 32

-Surgical excision (chemo and radiation as well for Grade IV)

Question 33

What is the study of choice for a glioblastoma multiforme (the most common and most aggressive primary malignant CNS tumor in adults)?

Answer 33

MRI brain with contrast

Question 34

What does the brain MRI with contrast show?

Answer 34

Variable ring of enhancement with central necrosis and irregular margins

Question 35

Treatment for glioblastoma multiforme?

Answer 35

Surgical excision when possible, radiotherapy and chemotherapy

Question 36

Meningiomas are benign, slow growing tumors that most commonly arise from

Answer 36

The dura

Question 37

Although meningiomas are often asymptomatic, what are some symptoms that can occur due to the compression and displacement of the brain?

Answer 37

seizures or focal neurological signs | Fixed dilated pupil (CN III) common

Question 38

Diagnostics for meningioma

Answer 38

- MRI with contrast of brain: ring enhancing lesion often attached to dura - Histology: spindle-cells in a whorled pattern. Psammoma bodies (round calcifications)

Question 39

Treatment for meningiomas

Answer 39

- Asymptomatic: observation | - Symptomatic: surgical excision when possible

Question 40

An ependymoma is a tumor of the ependymal cells in the ventricles and parts of the spinal cord. Who is it most common in and where is it most common?

Answer 40

Most common in children -In the 4th ventricle, spinal cord, and medulla

Question 41

What is delirium?

Answer 41

Acute, abrupt transient confused state due to an identifiable cause (due to medications, infections, electrolyte abnormalities, CNS injury, uremia, illicit drugs)

Question 42

Symptoms of Alzheimer Dementia (MC type of dementia)

Answer 42

- Short-term memory loss (first symptom) - Progression to long-term memory loss and cognitive deficits - Behavioral and personality changes - Loss of motor skills, gradual in nature

Question 43

Although Alzheimer Dementia is a clinical diagnosis, there are two diagnostics that can be done to diagnose. What are they and what do you see?

Answer 43

MRI (preferred): cortex atrophy, white matter lesions | Histology: amyloid-beta protein deposition (senile plaques) in he brain. Neurofibrillary tangles of tau protein.

Question 44

Treatment for Alzheimer Dementia

Answer 44

- Acetylcholinesterase Inhibitors: Donepezil, Tacrine, Rivastigmine, Galantamine. - NMDA Antagonist: Memantine: may be used as monotherapy or adjunctive

Question 45

Brain disease due to chronic ischemia and multiple infarctions (lacunar infarcts)

Answer 45

Vascular dementia

Question 46

What is the most important risk factor for vascular dementia?

Answer 46

Hypertension -Others: DM, history of CVA, A-fib

Question 47

Symptoms of vascular dementia

Answer 47

-Stepwise progression of symptoms: random infarct, then decline --> stable, then another infarct, then decline, etc.

Question 48

Main prevention of vascular dementia?

Answer 48

Strict blood pressure control!

Question 49

localized brain degeneration of the frontotemporal lobes

Answer 49

Frontotemporal Dementia (Pick's Disease)

Question 50

Symptoms of Pick's Disease

Answer 50

marked changes in social behavior, personality, and language (aphasia) with eventual exercise and memory dysfunction -Behavioral changes: socially inappropriate behaviors, apathy, hyperorality (binge eating, putting large amounts of food in their mouth, changes in taste preferences)

Question 51

On histology for Pick's Disease, what is seen?

Answer 51

Pick bodies: round or oral aggregates of Tau protein seen on silver-staining of cortex