Autoimmune axonal neuropathies Flashcards
What are primary systemic vasculitides?
Forms of vasculitis not caused by another medical condition or toxic exposure.
What is secondary vasculitis associated with?
Classical connective tissue disease, sarcoidosis, Behçet disease, infection, drugs, malignancy, IBD.
What are the key clinical features of vasculitic neuropathy?
Acute onset, motor/sensory features, neuropathic pain, profound sensory loss, multifocal presentation.
What can cloud the diagnosis of vasculitic neuropathy later in the disease course?
Multiple overlapping mononeuropathies leading to a symmetric length-dependent examination.
What symptoms are important constitutional symptoms to assess in cases of suspected vasculitis?
Fevers, chills, night sweats, and associated weight loss.
What condition is significant weight loss associated with?
Radiculoplexus neuropathy, a type of nonsystemic microvasculitis.
What symptoms should be assessed for rheumatologic disease?
Rash, arthritis, arthralgia, and sicca symptoms.
Which organ systems should be checked for involvement in rheumatologic disease?
Lung, renal, skin, gastrointestinal systems.
What is the target of cytoplasmic ANCA?
Proteinase 3.
What is the target of perinuclear ANCA?
Myeloperoxidase.
What condition is myeloperoxidase-perinuclear ANCA commonly associated with?
Microscopic polyangiitis.
What condition is proteinase 3 ANCA commonly associated with?
GPA (Granulomatosis with polyangiitis).
What is the benefit of performing an additional muscle biopsy along with a nerve biopsy?
Improves the yield of definite vasculitis diagnosis by 27%.
What are the main Primary vasculitides associated with PN?
Microscopic polyangiitis, GPA, EGPA, and PAN.
What medications are recommended for remission induction in active severe GPA or microscopic polyangiitis?
Glucocorticoids and rituximab.
What is cryoglobulinemic neuropathy?
Vasculitis due to circulating immunoglobulins precipitating at low temperatures, causing vessel damage.
What percentage of Hepatitis C cases have mixed cryoglobulins?
56%.
What factors suggest an inflammatory cause for postsurgical neuropathy?
Delay in symptom onset and progressive symptoms post-surgery indicate an inflammatory cause.
What is the most common clinical post-surgical inflammatory neuropathy presentation according to one study?
Lumbosacral radiculoplexus neuropathy (in 10 of 21 patients).
What is the prognosis of post-surgical inflammatory neuropathy?
Long-term follow-up showed improvement in neuropathy scores regardless of whether a patient received immunotherapy.
Are constitutional symptoms typically present in neuralgic amyotrophy?
Patients typically have no associated constitutional symptoms.
Most commonly involved regions in Neuralgic Amyotrophy?
Upper trunk of brachial plexus
Suprascapular
Long thoracic
Axillary
AIN
What should be considered in cases of recurrent neuralgic amyotrophy?
Hereditary brachial plexus neuropathy with SEPTIN9 genetic testing.
What percentage of patients showed full functional recovery from neuralgic amyotrophy at 3 years?
89% of patients showed full functional recovery at 3 years.
What are the typical symptoms of Wartenberg’s migratory sensory neuritis?
Rapid-onset sensory symptoms, pain or numbness, in one or multiple nerve distributions.
Percentage of Sjögren syndrome patients with peripheral nervous system complications?
5% to 20%.
What MRI finding is noted in some patients with nonataxic painful sensory Sjögren syndrome neuropathy?
Minimal T2 hyperintensities in the posterior columns.
What is the typical response of Sjogren’s sensory neuronopathy to immunotherapy?
It tends not to respond well.
What is the treatment window for stabilizing Sjogren’s related sensory neuronopathy?
Very brief, approximately 8 months.
What type of neuropathies are common in rheumatoid arthritis (RA)?
Entrapment mononeuropathies are common in RA.
How should entrapment mononeuropathies in RA be treated?
Treated the same as in patients without RA.
What additional spinal cord conditions can occur in patients with RA?
Cervical spondylosis, synovitis, and atlantoaxial disease.
What percentage of electrophysiologically detected RA related neuropathies were subclinical in one study?
75% were subclinical with no clinical signs.
What side effect can tumor necrosis factor a inhibitor therapy cause in RA patients?
Peripheral neuropathy, often demyelinating.
What part of the nervous system (CNS/PNS) is more commonly affected by neurologic symptoms in SLE?
Central nervous system.
What was the most common peripheral presentation in lupus patients?
Peripheral polyneuropathy (36.7% sensory, 18.8% sensorimotor symptoms).
What percentage of lupus patients had mononeuritis multiplex?
9.2% of the patients had mononeuritis multiplex.
What is an important clue for diagnosing paraneoplastic neuropathy?
Acute to subacute onset is an important clue.
Most commonly noted antibodies in paraneoplastic neuropathy?
ANNA-1 (anti-Hu), CRMP5 (anti-CV2), amphiphysin antibodies.
What are less common antibodies associated with paraneoplastic neuropathy?
MAP1B-IgG
LGI1 (VGKC)
CASPR2 (VGKC)
NIF
LUZP4.
What central nervous system findings could be present in some patients with ANNA 1 paraneoplastic syndrome?
Cerebellar ataxia and limbic encephalitis.
What gastrointestinal issue was seen in some patients with ANNA-1 PNP syndrome?
Gastrointestinal dysmotility.
What neurologic paraneoplastic syndromes are associated with CRMP-5 antibodies?
PN, Encephalitis, cerebellar ataxia, myelopathy, optic neuritis.
What to test for SPS patients negative for glutamic acid decarboxylase 65 (GAD65)?
Anti-amphiphysin ab.
What were the common CSF findings in most evaluated patients with anti-amphysin related paraneoplastic syndrome?
Abnormal findings, lymphocytic pleocytosis, and elevated protein levels.
