Tumefactive myelinoclastic diffuse sclerosis--case report.
A 6-year-old boy presented with mental disturbance and progressive left hemiparesis. Magnetic resonance imaging demonstrated large intracranial mass lesions with ring-like enhancement. His neurological condition deteriorated rapidly. Open biopsy via craniotomy was performed under the suspicion of tumor. Histological examination showed massive demyelination and axon preservation, but no tumor cells. The diagnosis was myelinoclastic diffuse sclerosis (MDS). He was treated with high-dose methylprednisolone and improved dramatically. MDS is a rare demyelinating disorder of the central nervous system that affects mainly children and may mimic a brain tumor. MDS must be included in the differential diagnosis in young patients with a brain tumor with atypical radiological appearance.
Obara
Soichi
S
Department of Neurosurgery, Faculty of Medicine, Kagoshima University, Kagoshima. sochian@m.kufm.kagoshima-u.ac.jp
Takeshima
Hideo
H
Awa
Ryuji
R
Yonezawa
Hajime
H
Oyoshi
Tatsuki
T
Nagayama
Tetsuya
T
Hirano
Hirofumi
H
Niiro
Masaki
M
Kuratsu
Jun-ichi
J
D001932:Brain Neoplasms / Q000175:diagnosis; D002648:Child; D003937:Diagnosis, Differential; D002549:Diffuse Cerebral Sclerosis of Schilder / Q000175:diagnosis*; D005684:Gait; D006429:Hemiplegia / Q000209:etiology; D006801:Humans; D008297:Male; D001523:Mental Disorders / Q000209:etiology