Case report:
A 53-year-old Iranian woman was visited in neurology clinic with a history of acute onset dizziness and gait disturbance for 1 week. There was no history of ear discharge, fullness, tinnitus or hearing loss. She gave a history of long-standing rheumatoid arthritis and was on low dose maintenance corticosteroid therapy. Physical examination revealed normal higher mental functions, speech and cranial nerves. There was no nystagmus, and saccades and pursuit were normal. Head impulse test was normal. Gait examination showed wide base, ataxic gait with difficulty in doing tandem walk. No dysmetria was seen in upper limbs. Rest of the neurological exam was normal. Brain MRI showed periventricular linear and radial enhancement pattern. CSF analysis including protein, glucose, WBC, ADA, ACE, VDRL, and cytology were normal. She was treated with an increase in the dose of oral steroids and all her symptoms improved in few days.
After a year she once again presented with a subacute onset, moderate to severe holocephalic headache. There was no papilledema and her neurological examination was normal. This time again she responded to an increase in the dose of oral steroids. A year later, for the third time she presented with rapid cognitive decline and gait unsteadiness. This time she had gaze evoked nystagmus and ataxic wide base gait on physical exam. Brain MRI with contrast was repeated which showed bilateral diffuse, pachymeningeal and leptomeningeal enhancement (Figure 1). CSF examination was once again normal. Her work up for infective causes including tuberculosis was negative. According to her Brain MRI imaging with relapsing remitting course steroid responsive ataxia and aseptic meningitis, a clinical decision was made to send her serum for GFAP IgG antibodies, which came back as positive.