Mechanisms of neurologic involvement:
(1) vasculitis which may be complicated by thromobosis (arterial, dural sinus, other) and/or arterial aneurysms
(2) parenchymal inflammation, especially in the basal ganglia and brainstem
Course of neurologic involvement:
(1) relapsing-remitting
(2) chronic progressive
(3) secondary progressive
Clinical findings:
(1) headache
(2) intracranial hypertension
(3) gait ataxia with unsteady gait
(4) cranial nerve palsy, including ophthalmoplegia
(5) hemiplegia or hemiparesis
(6) cognitive dysfunction, including confusion
(7) attention deficits
(8) frontal lobe dysfunction
(9) psychiatric, behavioral or personality change, including psychosis
(10) subarchnoid hemorrhage (from ruptured aneurysm)
(11) seizures
(12) meningeal signs
(13) decreased hearing
(14) paresthesias and/or sensory deficits
(15) muscle weakness
(16) sphincter disturbances
(17) impotence in males
Laboratory findings:
(1) elevated ESR and C-reactive protein (CRP)
(2) elevated cytokines (interleukin-6, interleukin-8, TNF-alpha)
(3) elevated serum complement
(4) elevated CSF pressure
(5) CSF neutrophilic pleocytosis
(6) elevated CSF protein with or without oligoclonal bands
Differential diagnosis includes:
(1) multiple sclerosis
(2) stroke
(3) psychiatric disease
(4) meningoencephalitis
(5) dementias
