A case of aseptic
meningoencephalitis with recurrent abnormal behavior,
status epilepticus and
aphthous stomatitis was described. A 39-year-old man, who had a history of low grade
fever and abnormal behavior over 2 years without mucocutaneo-ocular symptoms, was admitted to Shinshu University Hospital because of
status epilepticus. On admission, neurological examination revealed
comatose state with neck stiffness,
anisocoria (right greater than left), conjugate deviation to right and bilateral pyramidal tract signs. His cerebrospinal fluid examination revealed a total
protein content of 206 mg/dl with 22 lymphocytes and 134 polymorphonuclear leukocytes/mm3. EEG recorded on the 3rd hospital day showed periodic sharp waves and polyspikes, occurring every 1 seconds, predominantly arise from the right hemisphere, which corresponds to periodic lateralized epileptiform discharges (PLEDs).
Herpes simplex encephalitis was suspected and
acyclovir was administered. Neck stiffness and cerebrospinal fluid findings were improved and PLEDs disappeared. But
aphthous stomatitis appeared and
meningoencephalitis was relapsed. Laboratory examination revealed erythrocyte sedimentation rate 40 mm/hr, positive
C-reactive protein, elevated CH50 and
sialic acid, enhanced migration of neutrophils and
HLA-B 51. Neuro-Behçet's syndrome was strongly suspected with laboratory data but the
clinical course was atypical. His clinical signs were markedly improved after administration of
prednisolone. The present case suggests that neuro-Behçet's syndrome with only a part of major symptoms could be diagnosed as
viral encephalitis and
acyclovir may be effective for the treatment of neuro-Behçet's syndrome.