Paraneoplastic Vasculitic Neuropathy PVN

Synonyms:

or paraneoplastic neuromyopathy

Diagnosis:

A subtype of nonsystemic vasculitic neuropathy, requires features of neuropathy on clinical exam or electrodiagnostic studies plus evidence of vasculitis and underlying neoplastic disorder

Clinical features:

Weakness +/-sensory abnornalities, often with features of mononeuropathy multiplex

Findings on investigations:

+Nerve conduction studies +EMG:

  • Polyneuropathy or mononeuritis multiplex, axonal neuropathy
  • +/-evidence of myopathy

+CSF analysis: increased protein
+ESR: increased

Pathology, nerve biopsy:

Sural nerve or superficial peroneal nerve biopsy

  • 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

+/-muscle biopsy (peroneus brevis): perivascular infiltrate +/-perifasicular infiltrates
+evidence of an underlying cancer

Treatment:

Treat the underlying cancer
Immunosuppresion:

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

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