[Schilder's disease: two new cases and a review of the literature].
BACKGROUND
Schilder's disease, or diffuse myelinoclastic sclerosis, is an infrequent disease that presents clinically as a pseudotumoural demyelinating lesion, which makes its diagnosis more complicated as it can be mistaken for a tumour or an abscess.


METHODS
We examine the case of a male who was healthy up to the age of 8 years, when symptoms of a left hemiparesis appeared with a subacute onset and which were associated to symptoms of intracranial hypertension. A brain CAT scan showed a hypodense lesion in the right temporoparietal region, and the hypothesis of a tumoural lesion (astrocyte) was suggested. Treatment was started with dexamethasone and furosemide, and a complete regression of the symptoms and a considerable decrease in the cerebral lesion were observed. The second case is that of a female adolescent who, at the age of 11, developed a clinical picture of subacute onset of left hemiplegia. A brain CAT scan revealed hypodense lesions with ring-shaped contrast enhancement. In view of the histological diagnosis of an astrocyte, radiotherapy and corticotherapy are started. After two months' treatment, a sharp involution of the lesions was observed, which led to the acceptance of the diagnostic hypothesis of Schilder's disease. Both children presented recurrence of the lesions three years and nine months, in the first and second case respectively, after the first episode. Treatment with corticoid therapy was started and gave good clinical and radiological responses.


CONCLUSIONS
In the presence of a neurological deficit with a subacute onset, associated to a brain image showing a 'tumoural' lesion containing an important amount of oedema and little mass effect, diagnoses other than that of a brain tumour must be taken into account. It thus becomes possible to avoid invasive forms of treatment, such as surgical resection, which entail a number of sequelae.
Garrido
C
C
Servicio de Pediatría, Hospital Geral de Santo António, Porto, Portugal.
Levy-Gomes
A
A
Teixeira
J
J
Temudo
T
T
D000293:Adolescent; D002540:Cerebral Cortex / Q000000981:diagnostic imaging / Q000473:pathology; D002648:Child; D003907:Dexamethasone / Q000627:therapeutic use; D002549:Diffuse Cerebral Sclerosis of Schilder / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology* / Q000503:physiopathology; D004232:Diuretics / Q000627:therapeutic use; D005260:Female; D005665:Furosemide / Q000627:therapeutic use; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D008297:Male; D011859:Radiography; D012008:Recurrence
D004232:Diuretics; D005938:Glucocorticoids; D005665:Furosemide; D003907:Dexamethasone