Acute disseminated encephalomyelitis-like manifestations in a patient with neuro-Behçet disease.

OBJECTIVE

To describe a patient with a rare presentation of neuro-Behçet disease.

METHODS AND

RESULTS

A 42-year-old patient with definite Behçet disease was referred to Nemazee Hospital, affiliated to Shiraz University of Medical Sciences, Shiraz, South of Iran, with headache, biparesis, left central facial palsy, and urinary dysfunction. Brain magnetic resonance imaging revealed a hypo/hypersignal lesion in T1-/T2-weighted images in the right cerebral peduncle extending to the right basal ganglia. Cervical magnetic resonance imaging showed a lesion in the parenchyma of the spinal cord extending from C2 to T1 which was iso-hyperintense in T1-/T2-weighted images. Initial cerebrospinal fluid analysis revealed significant pleocytosis, hypoglycorrhachia and increased protein content. The patient had an incomplete response to treatment with corticosteroids and cyclophosphamide.

CONCLUSIONS

An acute disseminated encephalomyelitis (ADEM)-like presentation is a rare manifestation of neuro-Behçet disease.