Autoimmune axonal neuropathies Flashcards
What are primary systemic vasculitides?
Forms of vasculitis not caused by another medical condition or toxic exposure.
What can cause a secondary Vasculitis?
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 plexus 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.
According to my research NA can recur in 1/4 patients. Can also consider HNPP, CTD (eg SLE), vasculitis.
What percentage of patients showed full functional recovery from neuralgic amyotrophy at 3 years?
89% of patients showed full functional recovery at 3 years.
