Peripheral Neuropathy

Question 1

Despite extensive evaluation, no etiology is found in… of cases

Answer 1

Approximately half of patients. These patients typically have a predominantly sensory polyneuropathy

Question 2

If pain and temperature perception are lost, with preserved vibratory and position sense along with muscle strength and deep tendon reflexes, which type of neuropathy is most likely ?

Answer 2

A small-fiber neuropathy. (Most likely causes are diabetes melitus or glucose intolerance)

Question 3

The most common causes of sensory ganglionopathies are… ?

Answer 3

Sjögren’s syndrome and a paraneoplastic neuropathy

Question 4

Two features of CMT 1 ?

Answer 4

There is often atrophy of the muscles below the knee (particularly the anterior compartment), leading to so-called inverted champagne bottle legs. Nerve biopsies reveal a predilection for loss of large-diameter fibers and Schwann cell proliferation around thinly or demyelinated fibers, forming so-called onion bulbs

Question 5

Some features of Hereditary Sensory and Autonomic Neuropathy (HSAN) ?

Answer 5

Most people with HSAN1 do not complain of numbness, they often describe burning, aching, or lancinating pains. Bladder dysfunction and reduced sweating in the feet may occur.

Question 6

Classic tetrad of Refsum’s disease ?

Answer 6

Peripheral neuropathy, retinitis pigmentosa, cerebellar ataxia, elevated CSF protein concentration.

Question 7

Which forms of porphyria are associated with peripheral neuropathy ?

Answer 7

Acute intermittent porphyria (porphobilinogen deaminase)
Hereditary coproporphyria (coproporphyrin oxidase)
Variegate porphyria (protoporphyrinogen oxidase)

Question 8

Some features of an acute attack of porphyria ?

Answer 8

Abdominal pain, agitation, hallucination, seizures, several days later back and extremity pain followed by weakness, dysautonomia and signs of sympathetic overactivity is common (tachycardia, hypertension etc.)

Question 9

Which is the presenting manifestation of DM in appr. one-third of patients ?

Answer 9

Diabetic radiculoplexus neuropathy. Patients typically present with severe pain in the low back, hip and thigh in one leg.

Question 10

Common cranial neuropathies in diabetics ?

Answer 10

Occulomotor (characteristically pupil-sparing),abducens and trochlear

Question 11

Which types of neuropathies have been reported in association with celiac disease or antigliadin/antiendomysial antibodies ?

Answer 11

Generalized sensorimotor neuropathy, pure motor, multiple mononeuropathies, autonomic neuropathy small-fiber neuropathy, and neuromyotonia.

Question 12

Most common HIV-related form of peripheral neuropathy ?

Answer 12

Distal Symmetric Polyneuropathy

Question 13

Some characteristics of thallium poisoning ?

Answer 13

Increased thirst, sleep disturbances and psychotic behavior may be noted. Hair pigmentation, acne-like rash in the malar area of the face and hyperreflexia.

Question 14

Which pattern is most common in patients with Immune-Mediated Brachial Plexus Neuropathy

Answer 14

The most common pattern involves the upper trunk or a single or multiple neuropathies primarily involving the suprascapular, long thoarcic, or axillary nerves.