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NEURO CLERKSHIP > Peripheral Neuropathies > Flashcards

Peripheral Neuropathies Flashcards

1
Q

Most common inherited polyneuropathy?

A

Charcot-Marie-Tooth spectrum disorders.

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2
Q

What are the types of CMT spectrum disorders?

A 
CMT 1 (Demyelinating) 
CMT 2 (Axonal) 
CMT X (X-Linked)
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3
Q

What is the inheritance pattern and genetic cause of Demyelinating CMT?

A

Type 1 (Demyelinating)- AD disorder from mutation in peripheral myelin protein 22 (PMP22) gene.

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4
Q

Clinical manifestations: distal muscle wasting of the legs with high arches and hammer toes with lifelong numbness that the patient doesn’t complain about.

A

Demyelinating CMT (Type 1)

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5
Q

What is the inheritance pattern of Axonal CMT?

A

AD. CMT 2 is less severe than CMT 1 and many patients can ambulate.

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6
Q

What is the inheritance pattern of CMT X

A

X-Linked with carrier females less severely affected.

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7
Q

Clinical manifestation: patient presents in their 20s with frequent falls, exercise intolerance, and cramping.

A

CMT X

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8
Q

Nerve conduction study: decreased amplitude.

A

Axonal Disease (CMT 2)

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9
Q

Nerve conduction study: decreased conduction velocity

A

Demyelinating Disease (CMT 1)

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10
Q

How can vasculitis cause neuropathy?

A

Lack of oxygen from decreased blood flow or inflammation that damages vessels can cause hypoxic neuronal injury.

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11
Q

Patient presents with painful, asymmetrical, asynchronus sensory and motor peripheral neuropathy.

A

Mononeuritis multiplex: isolated damage to at least two separate nerve areas.

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12
Q

Size of arteries affected by polyarteritis nodosa?

A

A systemc vasculitis affecting small and medium sized muscular arteries.

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13
Q

Necrotizing inflammatory lesions that affect muscular arteries and can cause mononeuritis multiplex, myalgia, abdominal pain, nephropathy, and skin ulceration?

A

Polyarteritis nodosa

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14
Q

What abnormal lab is found in 30% of patients with polyarteritis nodosa?

A

Hypergammaglobulinemia.

*Patients can also have a low C3/C4 complement level.

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15
Q

What studies should be ordered for patients with suspected polyarteritis nodosa?

A

CBC, CCP, ESR, hepatitis panel.

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16
Q

What is the histological characterization of polyarteritis nodosa?

A

Fibrinoid necrosis of the arterial wall with a leukocytic infiltrate.

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17
Q

What skin condition is associated with Livedo reticularis?

A

Polyarteritis Nodosa

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18
Q

What is the pathology of Guillain-Barre Syndrome?

A

Auto-immune demyelinating disease with or without secondary axonal loss. Up to 75% are preceded by an infection, immunization, or surgery, making it a likely parainfectious disease.

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19
Q

What infections have been linked to Guillain-Barre Syndrome?

A

Campylobacter jejuni, EBV, CMV, lyme disease, hepatitis, and HIV

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20
Q

A patient presents with rapidly ascending weakness beginning at their feet and deep absence of deep tendon reflexes.

A

Guillain-Barre Syndrome

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21
Q

In addition to ascending weakness, what other findings may be associated with Guillain-Barre Syndrome?

A

Pain and parasthesia, autonomic instability, and respiratory failure

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22
Q

What is the most likely clinical course from Guillain-Barre Syndrome?

A

Symptoms progress for 2-4 weeks then plateau. 5-10% mortality linked to respiratory failure.

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23
Q

What is a Cytoalbumic Dissociation?

A

Elevation in CSF protein without an associated increase in WBCs.

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24
Q

Guillian-Barre Patients will have what finding in their CSF on lumbar puncture?

A

Cytoalbumic Dissociation

25
Q

What is the treatment for Guillian-Barre Syndrome?

A

IVIG for five days or PLEX for five treatments.

26
Q

What antibodies are present in the Miller Fisher Variant of Guillian-Barre Syndrome?

A

Anti-GQ1B antibodies.

27
Q

Patient presents with ophthalmoplegia, ataxia, facial weakness, dysarthria, and areflexia.

A

Miller Fisher Variant of Guillian-Barre Syndrome.

28
Q

What condition is characterized as an antibody-mediated reaction along with interstitial and perivascular infiltration of the endoneurium with inflammatory T cells and macrophages causing segmental demyelination of peripheral nerves?

A

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

29
Q

What are the clinical features of CIDP?

A

Sensory and motor symptoms with associated autonomic system dysfunction (orthostatic dizziness, bowel and bladder dysfunction, cardiac problems)

30
Q

Findings of CIDP:

A

CSF- increased protein

EMG/NCS- demyelination

31
Q

Treatment of CIDP:

A

Immunosuppression and immunomodulation: steroids, azathioprine, mycophenolate, cytoxan, PLEX, IVIG

32
Q

What are common causes of metabolic neuropathies?

A

Diabetes mellitus, hyopthyroidism, uremia, and critical illness polyneuropathy

33
Q

Characteristic of neuropathy in diabetic neuropathy:

A

Stocking-glove sensory loss, burning and weakness.

34
Q

Treatment of diabetic neuropathy:

A

Glycemic control, TCAs, gabapentin, topical capscaisin, and other anti-epileptic drugs.

35
Q

What vitamin deficiencies can cause neuropathy?

A

B-12 (cobalamin) and B-1 (thiamine)

36
Q

What drug of abuse can lead to cobalamin deficiency?

A

Nitrous oxide.

37
Q

What is the typical presentation of cobalamin deficiency?

A

Numbness, neuropathic pain, gait disturbance, and possible posterior column myelopathy (fine touch and conscious proprioception).

38
Q

If serum B12 is low, what will be elevated, indicating B12 deficiency?

A

methyl malonic acid (MMA)

39
Q

Which vitamin deficiency is associated with the following? Muscle cramps and distal sensory loss. Can progress to ascending weakness of the legs and sensorimotor neuropathy of the hands.

A

Thiamine

40
Q

Which vitamin deficiency is associated with the following? Numbness and paresthesia in a stocking glove distribution?

A

Pyridoxine

41
Q

Which vitamin deficiency is associated with the following? Painful neuropathy with sensory ataxia, areflexia, and numbness?

A

Pyridoxine toxicity.

42
Q

Which vitamin deficiency is associated with the following? Numbness and tingling which can progress to weakness and gait impairment. Can cause myelopathy creating myeloneuropathy syndrome (ataxia, hyperreflexia, weakness, and sensory loss)

A

Cobalamin

43
Q

What physical examination finding is consistent with arsenic toxicity?

A

Mees lines (underneath the fingernails)

44
Q

What causes peripheral neuropathy with persistent nausea and vomiting.

A

Arsenic toxicity

45
Q

How is arsenic toxicity diagnosed?

A

24-hour urinalysis and hair sample.

46
Q

What finding is unique to lead poisoning?

A

Basophilic stipling.

47
Q

What causes spotty alopecia?

A

Thalium

48
Q

What medications have been implicated in peripheral neuropathy?

A

Paclitaxel, vincristine, metronidazole, nitrofurantoin, isoniazie, cholicine, hydralazine, nitrous oxide, amiodarone

49
Q

Which neurons are affected in ALS?

A

Anterior motor neurons.

50
Q

When is ALS onset typically?

A

65-70

51
Q

What is the gene commonly affected in familial ALS?

A

Super oxide dismutase.

52
Q

What are the signs of ALS?

A

Upper and lower motor neuron signs (spasticity, atrophy, fasiculations, asymmetry, bulbar involvement)

53
Q

A patient presents with dysfunction of on body part that progressively spreads, eventually resulting in ventilatory failure.

A

Clinical course of ALS

54
Q

What is the FDA approved drug for ALS?

A

Rilutek (Riluzole). Prolongs life by 3 months. It is a glutamate blocker.

55
Q

What is stiff person syndrome?

A

A motor neuron hyperexcitability disorder that arises from an autoimmune or paraneoplastic syndrome where antibodies disrupt spinal inhibitory cells.

56
Q

What are the clinical findings of stiff person syndrome?

A

Spine and leg rigidity, hyperreflexia, lordosis, paraspinal and abdominal hypertonicity, and back and leg spasms induced by startle.

57
Q

What antibody is associated with non-paraneoplastic stiff person syndrome?

A

Anti-GAD-65

58
Q

What antibody is associated with paraneoplastic stiff person syndrome (breast cancer, small cell lung, colon, hodgkin lymphoma)

A

Amphyphysin antibodies.

59
Q

How is stiff person syndrome treated?

A

Benzodiazepines for symptoms, IVIG for acute treatment followed by long prednisone taper.

NEURO CLERKSHIP (10 decks)

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