Nonsystemic vasculitic neuropathy NSVN

Diagnosis:

This is based on clinical features +nerve/muscle biopsy findings of vasculitis +exclusion of systemic causes

Clinical features:

Clinical features: usually subacute, painful, distal, asymmetric,

Findings on investigations:

+Nerve conduction studies +EMG:

  • Axonal neuropathy

+CSF analysis: increased protein
+ESR: might be increased
+ANCA negative

Pathology, nerve biopsy:

Sural nerve or superficial peroneal nerve biopsy +/-muscle biopsy (peroneus brevis)

  • Arterioles of the epineurium:
    • Inflammation: Infiltrate of lymphocytes
  • Necrosis of intima and media
  • Endothelial cell changes
  • Fibrinoid necrosis: a definite sign
  • Axons: loss of axons in the periphery of the fascicle i.e. subfascicular ischemic change
  • Clusters of demyelination/remyelination is consistent
  • Immunohistochemistry: CD3 for T cells

Treatment:

Immunosuppression:

  • Prednisolone P.O. +cyclophosphamide I.V.

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