ABSTRACT

Schilder’s disease (SD), which is characterised with large asymmetric demyelination areas in cerebral hemispheres, is a Multiple Sclerosis (MS) variant viewed clinically by increasing mental retardation, loss in sight and hearing, hemiplegia or tetraplegia.

26 year-old woman patient, married, has a child. Complaints have started at the age of 11. Had an operation because of an intracranial tumor at that time. However, no malignant cells were found in the patology. The lesion seen with Cranial Magnetic Resonans Imaging were evaluated as SD in 1998. Patient has come with complaints that are loss in hearing, deterioration in speaking, mental retardation and epileptic convulsions by means of generalised tonic clonic. In the neurological inspection, patient wal walking with disphasic small steps and was supported when walking. Achill clonus was taken and cerebellar tests were deteriorated on the right. Fundoscopic inspecton showed temporal paleness. The lesions in the Cranial Magnetic Resonans Imaging matched with the last MRI values. 7 days 1000 mg/ day methylprednisolone treatment was applied. Clinical follow up has shown that the complaints were decreasing.

19 year-old women patient. Compained of weakness in the right arm and leg for the last week. In the neurological inspection of the patient has shown that the patients was concious, cooperating, had sole epider reflexy extansor on the right and satisfactory cerebellar tests. Funduscopic inspection has show slight temporal paleness on left and bilateral concantric in sight area. Lesions in the Cranial MRI and MR spectroscopies and findings matching with 5 cm. diamtered tumoric demyelinisan plaque which forms a minimal signal increase, reaching from left frontoparietal subcortical white element to the corpus collosum have led to think of SD in the first place which is a variant of MS. 10 days 1000 mg/day methylprenisolone treatment was applied. After the treatment complaints decreased significantly. We have found useful to present these cases because of their rareness.

Keywords: Schilder’s disease, Multiple Sclerosis, Myelinoclastic diffuse sclerozis