Vasculitis of the nervous system is a rare cause of multifocal
neurologic symptoms and may involve both the central and peripheral nervous systems. Typical symptoms include
headache,
encephalopathy with
cognitive impairment and psychotic symptoms, epileptic
seizures, and
peripheral neuropathies. Here we report the case of a 71-year-old female presenting with Raynaud's syndrome and
paresthesia of the feet. Several weeks later she was admitted to our hospital with a
status epilepticus and
complex partial seizures. On admission she had mild
aphasia, distal
paresis of the arms without sensory deficits, and disorientation with
hallucinations. Cerebral MRI revealed small, multifocal
infarctions in several arterial territories. Multiple cerebral artery
stenoses were detected by ultrasound. Examination of the CSF was unremarkable. Serologic tests for autoimmune disorders detected
Ro antibodies compatible with
systemic lupus erythematosus or Sjögren's syndrome. A sural nerve biopsy revealed ischemic axonal neuropathy. During administration of i.v.
methylprednisolone, the symptom progression stopped but dosages could not be tapered due to severe CNS symptoms (mental decline, disorientation,
aphasia,
hallucinations). Slow but sustained clinical improvement was achieved by immunoadsorption over 3 weeks followed by a combined high-dose immunosuppressive treatment with
cyclophosphamide and
prednisolone that paralleled a reduction in anti-Ro titers and normalization of cerebral blood flow velocities as detected by repeated transcranial Doppler sonography.
Systemic vasculitis may present with multiple neurologic and psychiatric symptoms due to involvement of the central and peripheral nervous systems. After excluding systemic
infection, immunosuppressive therapy should be started early. In our case a combination of high-dose
methylprednisolone, immunoadsorption with elimination of
Ro antibodies, and
cyclophosphamide led to the patient's recovery.