Systemic Vasculitic Neuropathy

Diagnosis:

The diagnosis is made based on clinical features +Peripheral neuropathy, +evidence of vasculitis, +evidence of other organ involvement

Clinical features:

acute, mononuritis multiplex or less commonly mononeuritis simplex or symetric polyneuropathy, sensorimotor
+Peripheral neuropathy
+evidence of vasculitis
+evidence of other organ involvement

Findings on investigations:

+NCS/EMG:
NCS:

  • Axonal neuropathy
  • Conduction block: often transient
  • CMAP: reduced
  • Motor conduction velocity: normal or slightly reduced
  • SNAP: reduced

EMG: fibrillation potentials occasionally,

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

Immunohistochemistry: CD3 for T cells

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