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.