MKSAP Neuro III

Question 1

Idiopathic brachial plexopathy (also known as neuralgic amyotrophy and Parsonage-Turner syndrome) is characterized by:

Answer 1

Subacute severe pain followed by resolution of pain and progressive weakness and atrophy involving the shoulder girdle and upper extremity muscles. Can also have mixed presentation with motor, sensory an pain symptoms. This syndrome often is triggered by a preceding event, such an infection or surgery.

Question 2

First-line agents used to treat Tourette syndrome when the associated tics interfere with education, daily function, or work are …. (there are 4)

Answer 2

clonidine, guanfacine, topiramate, and tetrabenazine (NOT just CBT)

Question 3

First line agents for relapsing-remitting MS (there are two)

Answer 3

1. Interferon beta

2. Glatiramer acetate (better in patients with liver disease)

Question 4

Subacute ataxia, areflexia, and ophthalmoplegia, with or without diffuse weakness. Antibodies to GQ1b (a ganglioside component of nerve) are present.

Answer 4

Miller Fisher varian of GBS

Question 5

Numbness, distal extremity weakness, unsteady gait, areflexia, high arches, hammer toes, and atrophy of distal extremity muscles and foreleg muscles (“stork leg” deformity).

Answer 5

Charcot-Marie-Tooth (CMT) disease (inherited neuropathy)

Question 6

Painful ophthalmoplegia affecting multiple nerves involved with extraocular movement indicates a pathologic process in the _____ or ____.

Answer 6

superior orbital fissure or cavernous sinus

Question 7

Thrombosis of the cavernous sinus most commonly arises from _____.

Answer 7

Contiguous spread of infection, most likely from nasal, sinus, and dental sites. Staphylococcus aureus is the most common organism and is found in 70% of affected patients.

Question 8

Occipital and neck pain and posterior fossa symptoms, such as dysarthria, dysphagia, ataxia, and hemifield visual loss.

Answer 8

Vertebral artery dissection

Question 9

Orbital pain, partial Horner syndrome (ptosis and miosis only), and ipsilateral signs of cerebral or retinal ischemia

Answer 9

Dissection of the carotid artery

Question 10

What is autoimmune limbic encephalitis

Answer 10

new-onset status epilepticus and progressive confusion in a previously healthy patient. Antibodies (LGI1) directed against the voltage-gated potassium channel receptor complex. This condition is marked by the presence of hyponatremia, myoclonus, and limbic encephalitis (amnesia, temporal lobe seizures, and confusion).

Question 11

New-onset status epilepticus in a previously healthy person suggests the presence of a ____.

Answer 11

Neurologic autoimmune disorder. Often paraneoplastic.

Question 12

In a patient with treatment-refractory depression who also has cognitive and functional decline, ____ can be useful in identifying a pattern or degree of cognitive impairment that is not typical of depression-related cognitive impairment.

Answer 12

Neuropsychological testing

Question 13

What is the next step in medically intractable epilepsy?

Answer 13

Video EEG to see if pt is candidate for surgery

Question 14

High-risk features of intracranial aneurysm

Answer 14

1. size equal to or greater than 7 millimeters
2. rapid growth
3. cranial nerve deficit,

Question 15

What is pimavanserin?

Answer 15

Nondopaminergic atypical antipsychotic agent and only FDA-approved medication for Parkinson psychosis

Question 16

1st step in treated Parkinson disease psychosis?

Answer 16

Discontinue dopaminergic agonists (like pramipexole)

Question 17

What do you test in patients with biopsy-inaccessible lesions who have suspected primary central nervous system lymphoma?

Answer 17

Slit lamp exam and vitreous fluid collection with cytology

Question 18

What is McArdle disease

Answer 18

metabolic myopathy associated with exercise intolerance, cramping, myalgia, mild weakness, “second-wind” phenomenon, and myoglobinuria.

Question 19

What is myotonia?

Answer 19

Delayed muscle relaxation

Question 20

Classic feature of polymyositis

Answer 20

Symmetric painless proximal weakness of the arms and legs

Question 21

___________ commonly occurs in dementia with Lewy bodies (DLB) and can help distinguish DLB from Alzheimer disease and other cognitive disorders.

Answer 21

Rapid eye movement sleep behavior disorder

Note that all synucleinopathies (Parkinson’s, etc) have this issue.

Question 22

Patients who have intracerebral hemorrhage without elevated intracranial pressure whose systolic blood pressure is greater than 180 mm Hg should be treated with an intravenous antihypertensive agent, such as nicardipine, to a target blood pressure of _____.

Answer 22

140 mm Hg (systolic)