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.