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Publication
Enfermedad de Schilder: dos nuevos casos
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Advisor(s)
Abstract(s)
Summary. Introduction. 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. Case reports. 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
(astrocytoma) 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 astrocytoma, radiotherapy and
corticotherapy were 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
with 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.
Description
Keywords
Child Demyelinating diseases of the CNS Multiple sclerosis Pseudotumoural lesion Schilder’s disease
Citation
REV NEUROL 2004; 39: 734-8
Publisher
Revista de Neurologia