Peripheral Neuropathies

Question 1

What is the most common cause of peripheral neuroopathy?

Answer 1

Diabetes Mellitus

Question 2

What is the type of neuropathy where axonal degeneration predominates?

Answer 2

Axonal neuropathy

Question 3

What is the type of neuropathy in which segmental degeneration predominates?

Answer 3

Demyelinating Neuropathy

Question 4

What type is the majority of neuropathies?

Answer 4

Axonal/Dying-back type (Distal axonal neuropathy)

Question 5

What findings on a nerve conduction study would differentiate between an axonal and demyelinating neuropathy?

Answer 5

NCV is typically near normal in axonal neuropathies but conspicuously decreased in demyelinating neuropathies

Question 6

Which type of peripheral neuropathy has a limited number of causes? Which neuropathy type is most likely hereditary, immune mediated and inflammatory/IgM associated?

Answer 6

Demyelinating; Demyelinating

Question 7

What are typical findings of peripheral neuropathy?

Answer 7

Muscle weakness, muscle atrophy, sensory loss, paresthesia, pain, autonomic dysfunction

Question 8

What type of neuropathy is localized to dorsal root ganglia?

Answer 8

Sensory neuropathy

Question 9

What type of neuropathy is localized to nerve roots? One nerve?

Answer 9

Radiuclopathy; Mononeuropathy

Question 10

What type of neuropathy is localized to several nerves?

Answer 10

Mononeuropathy Multiplex

Question 11

What type of neuropathy is diffuse and symmetric involving peripheral nerves?

Answer 11

Polyneuropathy

Question 12

What type of neuropathy involes nerve roots and peripheral nerves?

Answer 12

Polyradiculoneuropathy

Question 13

What type of neuropathy predominates in diabetes?

Answer 13

Distal, sensory, polyneuropathy

Question 14

What type of degeneration predominates in diabetic neuropathy?

Answer 14

Both axonal degen and segmental demyelination are observed, but axonal predominates

Question 15

What is uremic neuropathy? How can it be improved?

Answer 15

Distal sensorimotor axonal polyneuropathy associated with renal failure; Can improve with dialysis and renal transplant

Question 16

What do critical illness, alcoholism, and nutritional deficit have in common?

Answer 16

They can all cause peripheral neuropathy

Question 17

What is AIDP? What events does it typically follow? What is it the most common cause of?

Answer 17

Acute inflammatory demyelinating polyradiculoneuropathy; Bacterial (C. jejuni), viral (CMV, EBV) or mycoplasmal (M. pneumoniae) infection; Guillain-Barre Syndrome

Question 18

What is Guillain-Barre Syndrome? How long does it take for neuropathy to resolve? Findings on CSF?

Answer 18

Acute symmetric neuromuscular paralysis that begins distally and ascends proximally; 2 to 4 weeks; Increased protein w/o pleocytosis

Question 19

What is Fisher syndrome?

Answer 19

Guillain-Barre Syndrome + Opthalmoplegia, Ataxia, Areflexia

Question 20

What infection and Abs are associated with the axonal form of GBS?

Answer 20

C. jejuni and atiganglioside Abs (anti-GM1)

Question 21

What is CIDP? Clinical Syx?

Answer 21

Chronic Inflammatory Demyelinating Polyradiculoneuropathy; Symmetric demyelinating neuropathy, involving both proximal and distal sensorimotor neurons

Question 22

A patient with subactue symptoms of polyneuropathy with sensory ataxia. The patient has Sjoegren Syndrome. What is your Dx? What other conditions are associated? Dscribe the findings of the dorsal root ganglia

Answer 22

Sensory Neuropathy; Paraneoplastic syndromes; Infiltrates by lymphocytes

Question 23

What type of neuropathy is caused by various types of vasculitis?

Answer 23

Mononeuropathy Multiplex

Question 24

What type of neuropathies can monoclonal gammopathy lead to?

Answer 24

Amyloid neuropathy, Cryoglobulinemia-associated vasculitic neuropathy, or chronic demyelinating polyneuropathy

Question 25

A patient has a point mutation in the transthyretin gene and has a history of familial amyloidosis. What type of neuropathy is he likely to have? What is the mechanism leading to neuropathy? What syndrome is associated causing numbness and tingling in the fingers?

Answer 25

Amyloid neuropathy; Mechanical insult and amyloid obstruction of the vasovasorum; Carpal Tunnel Syndrome

Question 26

A patient has paraneoplastic sensory neuronopathy. What is the likely cause? What Abs are associated?

Answer 26

Small cell lung cancer; Anti-Hu Abs

Question 27

What is the most frequent cause of toxic neuropathy?

Answer 27

Drugs (iatrogenic)

Question 28

What is the most commonly inherited neuropathy? What are the two subtypes? What is the clinical presentation?

Answer 28

Charcot-Marie-Tooth Disease; CMT1 Demyelinating and CMT2 Axonal; Slowly progressive distal sensorimotor polyneuropathies manifesting in childhood/early adulthood

Question 29

What type of inheritance occurs in CMT1, CMT2, CMTX, and CMT4

Answer 29

Autosomal Dominant, Autosomal Dominant, X-linked, Autosomal Recessive

Question 30

What is Dejerine-Sottas syndrome?

Answer 30

CMT3 resembling CMT1 but much more severe with an onset in early infancy (severe demyelination)

Question 31

In what neuropathy does myelin show sausage-shaped thickenings (tomacula) and axonal loss (tomaculous neuropathy)?

Answer 31

Hereditary neuropathy with liability to pressure palsies (HNPP)

Question 32

What is the most common type of neuropathy associated with HIV infection?

Answer 32

Distal Symmetric Polyneuropathy