[Metachromatic leukodystrophy: an exceptional cause of dementia in the adult]. / Leucodistrofia metacromática: una causa excepcional de demencia en el adulto.

Hereditary metabolic diseases are an exceptional cause of neurological disorders in adults. Metachromatic leukodystrophy is a hereditary alteration of the metabolism of myelin which may be manifested in adults as intellectual deterioration. A case of metachromatic leukodystrophy presented in adulthood is presented with cognitive deterioration and behavioral alterations as the only clinical manifestation. The patient was a 28 year old male studied for dementia of one year of evolution. Computerized tomography and cranial magnetic resonance demonstrated diffuse and symmetric involvement of the periventricular white matter. The visual evoked potentials were involved while the brain stem auditory potentials were normal. Study of the speed of nerve conductions was compatible with demyelinating neuropathy. The diagnosis of metachromatic leukodystrophy was confirmed by enzyme study revealing very diminished levels of aryl-sulfatase A. Although it is exceptional the adult form of metachromatic leukodystrophy should be included in the differential diagnosis of dementia. Computerized tomography and cranial magnetic resonance together with neurophysiologic studies are the principle procedures orienting diagnosis to this disease.

[Nontraumatic cerebral hematoma in young adults: a study of 42 cases]. / Hematoma intracerebral no traumático en adultos jóvenes: estudio de 42 casos.

BACKGROUND: Cerebrovascular disease has particular features in young adults (15-45 years). In this context, non-traumatic intracranial hematoma (NTICH) has received little attention. Therefore, its analysis has been attempted focusing on etiology, localization and short term prognosis. METHODS: 42 patients aged 15-45 years who were admitted because of NTICH were evaluated. 41 variables were analyzed with chi-square method and Fischer's exact test. RESULTS: The localization of hematoma was as follows: basal ganglia/thalamus in 59%, lobar in 19%, posterior fossa in 12%, pure intraventricular in 2 cases and multiple in one case. The most common etiology was hypertension (HT) (32%), followed by arteriovenous malformations (12%), oral anticoagulants (10%), chronic alcohol abuse (10%), coagulation disorders and one central nervous system arteriopathy; 12 cases were idiopathic. On the basis of etiology two groups were distinguished: 15-30 years (no case with hypertension) and 30-45 years (HT as the leading cause). Survival was 89%. CONCLUSIONS: NTICH in young adults has a heterogeneous etiology. On the basis of the most common cause two groups can be considered: from 15 to 30 years (arteriovenous malformation) and from 30 to 45 years (HT). High blood pressure at the time of stroke is correlated with previous HT. The short term life prognosis is better than that of NTICH in general series. Deterioration of consciousness in the acute phase and oral anticoagulation are poor prognostic factors.