A boy with gliomatosis cerebri initially treated for acute disseminated encephalomyelitis

Abstract

  We report a case of a 10-year-old boy with gliomatosis cerebri (GC) who was initially treated for suspected acute disseminated encephalomyelitis (ADEM). He presented to a nearby hospital's neurology department with a≧1-week history of headache and nausea. A neurologic examination revealed no abnormality, and the results of cerebrospinal fluid (CSF) analysis were normal. MRI showed a diffuse white matter abnormality, and ADEM was suspected. He received three courses of methylprednisolone pulse therapy (1 g/day for 3 days). His symptoms improved and then relapsed withn 1 month. A repeat MRI showed residual white matter abnormality and enlargement of the ventricle indicating hydrocephalus. One month after the start of the steroid therapy, he was referred to our hospital's neurosurgery department and we performed a venticuloperitoneal shunt surgery. His symptoms transiently disappeared. One month later, his symptoms reappeared. He was referred to our department. He showed somnolence, horizontal nystagmus with left gazing, and truncal ataxia. The results of a CSF analysis was normal. MRI showed belt-shaped hyperintense lesions lining ventricle walls from the lateral ventricles to the 4th ventricle in a T2-weighted image and FLAIR image, with poor gadolinium-enhancement. We performed an endscopic biopsy through the lateral ventricle. Pathology revealed a gradeⅢ astrocytoma. Taken together, the imaging findings and pathologic analysis led to the diagnosis of GC. The differential diagnosis for diffuse white matter lesions should include GC.