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Neurology Articles > Autoimmune axonal neuropathies > Flashcards

Autoimmune axonal neuropathies Flashcards

1
Q

What are primary systemic vasculitides?

A

Forms of vasculitis not caused by another medical condition or toxic exposure.

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2
Q

What is secondary vasculitis associated with?

A

Classical connective tissue disease, sarcoidosis, Behçet disease, infection, drugs, malignancy, IBD.

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3
Q

What are the key clinical features of vasculitic neuropathy?

A

Acute onset, motor/sensory features, neuropathic pain, profound sensory loss, multifocal presentation.

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4
Q

What can cloud the diagnosis of vasculitic neuropathy later in the disease course?

A

Multiple overlapping mononeuropathies leading to a symmetric length-dependent examination.

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5
Q

What symptoms are important constitutional symptoms to assess in cases of suspected vasculitis?

A

Fevers, chills, night sweats, and associated weight loss.

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6
Q

What condition is significant weight loss associated with?

A

Radiculoplexus neuropathy, a type of nonsystemic microvasculitis.

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7
Q

What symptoms should be assessed for rheumatologic disease?

A

Rash, arthritis, arthralgia, and sicca symptoms.

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8
Q

Which organ systems should be checked for involvement in rheumatologic disease?

A

Lung, renal, skin, gastrointestinal systems.

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9
Q

What is the target of cytoplasmic ANCA?

A

Proteinase 3.

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10
Q

What is the target of perinuclear ANCA?

A

Myeloperoxidase.

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11
Q

What condition is myeloperoxidase-perinuclear ANCA commonly associated with?

A

Microscopic polyangiitis.

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12
Q

What condition is proteinase 3 ANCA commonly associated with?

A

GPA (Granulomatosis with polyangiitis).

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13
Q

What is the benefit of performing an additional muscle biopsy along with a nerve biopsy?

A

Improves the yield of definite vasculitis diagnosis by 27%.

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14
Q

What are the main Primary vasculitides associated with PN?

A

Microscopic polyangiitis, GPA, EGPA, and PAN.

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15
Q

What medications are recommended for remission induction in active severe GPA or microscopic polyangiitis?

A

Glucocorticoids and rituximab.

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16
Q

What is cryoglobulinemic neuropathy?

A

Vasculitis due to circulating immunoglobulins precipitating at low temperatures, causing vessel damage.

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17
Q

What percentage of Hepatitis C cases have mixed cryoglobulins?

A

56%.

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18
Q

What factors suggest an inflammatory cause for postsurgical neuropathy?

A

Delay in symptom onset and progressive symptoms post-surgery indicate an inflammatory cause.

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19
Q

What is the most common clinical post-surgical inflammatory neuropathy presentation according to one study?

A

Lumbosacral radiculoplexus neuropathy (in 10 of 21 patients).

20
Q

What is the prognosis of post-surgical inflammatory neuropathy?

A

Long-term follow-up showed improvement in neuropathy scores regardless of whether a patient received immunotherapy.

21
Q

What symptoms are typically absent in neuralgic amyotrophy?

A

Patients typically have no associated constitutional symptoms.

22
Q

Most commonly involved regions in Neuralgic Myopathy?

A

Upper trunk of brachial plexus, suprascapular, long thoracic, axillary, AIN nerves.

23
Q

What should be considered in cases of recurrent neuralgic amyotrophy?

A

Hereditary brachial plexus neuropathy with SEPTIN9 genetic testing.

24
Q

What percentage of patients showed full functional recovery from neuralgic amyotrophy at 3 years?

A

89% of patients showed full functional recovery at 3 years.

25
Q

What are the typical symptoms of Wartenberg’s migratory sensory neuritis?

A

Rapid-onset sensory symptoms, pain or numbness, in one or multiple nerve distributions.

26
Q

Percentage of Sjögren syndrome patients with peripheral nervous system complications?

A

5% to 20%.

27
Q

What MRI finding is noted in some patients with nonataxic painful sensory Sjögren syndrome neuropathy?

A

Minimal T2 hyperintensities in the posterior columns.

28
Q

What is the typical response of Sjogren’s sensory neuronopathy to immunotherapy?

A

It tends not to respond well.

29
Q

What is the treatment window for stabilizing Sjogren’s related sensory neuronopathy?

A

Very brief, approximately 8 months.

30
Q

What type of neuropathies are common in rheumatoid arthritis (RA)?

A

Entrapment mononeuropathies are common in RA.

31
Q

How should entrapment mononeuropathies in RA be treated?

A

Treated the same as in patients without RA.

32
Q

What additional spinal cord conditions can occur in patients with RA?

A

Cervical spondylosis, synovitis, and atlantoaxial disease.

33
Q

What percentage of electrophysiologically detected RA related neuropathies were subclinical in one study?

A

75% were subclinical with no clinical signs.

34
Q

What side effect can tumor necrosis factor a inhibitor therapy cause in RA patients?

A

Peripheral neuropathy, often demyelinating.

35
Q

What system is more commonly affected by neurologic symptoms in SLE?

A

Central nervous system.

36
Q

What was the most common peripheral presentation in lupus patients?

A

Peripheral polyneuropathy (36.7% sensory, 18.8% sensorimotor symptoms).

37
Q

What percentage of lupus patients had mononeuritis multiplex?

A

9.2% of the patients had mononeuritis multiplex.

38
Q

What is an important clue for diagnosing paraneoplastic neuropathy?

A

Acute to subacute onset is an important clue.

39
Q

Most commonly noted antibodies in paraneoplastic neuropathy?

A

ANNA-1 (anti-Hu), CRMP5 (anti-CV2), amphiphysin antibodies.

40
Q

What are the antibodies associated with paraneoplastic neuropathy?

A

MAP1B-IgG, LGI1, CASPR2, NIF, LUZP4.

41
Q

What central nervous system findings could be present in some patients with ANNA 1 paraneoplastic syndrome?

A

Cerebellar ataxia and limbic encephalitis.

42
Q

What gastrointestinal issue was seen in some patients with ANNA-1 PNP syndrome?

A

Gastrointestinal dysmotility.

43
Q

What neurologic paraneoplastic syndromes are associated with CRMP-5 antibodies?

A

PN, Encephalitis, cerebellar ataxia, myelopathy, optic neuritis.

44
Q

What to test for SPS patients negative for glutamic acid decarboxylase 65 (GAD65)?

A

Anti-amphiphysin ab.

45
Q

What were the common CSF findings in most evaluated patients with anti-amphysin related paraneoplastic syndrome?

A

Abnormal findings, lymphocytic pleocytosis, and elevated protein levels.

Neurology Articles (6 decks)

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