Vasculitis with Involvement of the Central and Peripheral Nervous System and Anti-Ro Antibodies

Vasculitis of the nervous system is a rare cause of multifocal neurological symptoms and may involve both the central and peripheral nervous system. Typical symptoms include headache, encephalopathy with cognitive impairment, and psychotic symptoms, epileptic seizures, as well as peripheral neuropathies. Here, we report the case of 71-year-old female who presented with Raynaud syndrome and paresthesia of both feet. Some weeks later, she was admitted to our hospital with a status epilepticus with complex partial seizures. On admission she had mild aphasia and distal paresis of the arms without sensory deficits. MRI of the skull revealed small, multifocal infarctions in several arterial territories. Multiple cerebral artery stenoses were detected by ultrasound and MR angiography. CSF studies were unremarkable. Serological tests for autoimmune disorders detected ENA and Ro antibodies, compatible with systemic lupus erythematodes or Sjögren's syndrome. A sural nerve biopsy revealed ischemic axonal neuropathy. During administration of i.v. methylprednisolone, 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 only by combined immunosuppressive treatment with cyclophosphamide, prednisolone, and immunoadsorption over 8 weeks, and was paralleled by a reduction of anti-Ro titers and normalization of cerebral blood flow velocities as detected by repeated transcranial Doppler sonography. Systemic vasculitis may present with multiple neurological and psychiatric symptoms due to an involvement of the central and peripheral nervous system. After exclusion of a 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.