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Biomedical subjects

M J Nester

Publications and source records attributed to M J Nester.

5 recordsLinked to original sources
PubMed1996

Use of a brief assessment examination in a study of subacute sclerosing panencephalitis.

Subacute sclerosing panencephalitis is a degenerative disease affecting children and young adults that remains a distinct and relatively frequent problem in developing countries worldwide. Recent advances in treatment prompted a study at King Faisal Specialist Hospital, Riyadh, Saudi Arabia, using oral isoprinosine and intraventricular alpha-interferon. Initially, the Neurological Disability Assessment and Index was used for tracking patient progress. However, an additional means of assessment was deemed necessary and the Brief Assessment Examination was devised. Largely cognitive based, the Brief Assessment Examination appeared to be more sensitive to mental status changes than the Neurological Disability Assessment and Index, though it correlated modestly with the Neurologic Disability Assessment and Index and more strongly with staging. In addition, the Brief Assessment Examination can be administered by technician-level staff with a minimum of training. Though more study is needed, preliminary findings suggest that the Brief Assessment Examination should be a useful tracking tool for subacute sclerosing panencephalitis, particularly in the developing world.

Adolescent↗
PubMed1994

Sanfilippo type D presenting with acquired language disorder but without features of mucopolysaccharidosis.

A 7-year-old girl presented with a language disorder reminiscent of verbal auditory agnosia. Later, she proved to have defective N-acetylglucosamine-6-sulfate sulfatase, the enzyme deficient in Sanfilippo D syndrome. She did not show clinical features of mucopolysaccharidosis. The language disorder had a fluctuating course, which eventually evolved into a progressive dementing encephalopathy.

Agnosia↗
PubMed1992

Unknown dysmorphic syndromes and development delay in Saudi Arabia.

Forty-four Saudi Arabian patients with unknown dysmorphic syndromes were studied with respect to their level of cognitive delay. The relationship between such delay and particular patterns of dysmorphic features, as well as various demographic and historical data, were also investigated. Prognostication and genetic counseling are difficult for this group because little is known about morbidity or other factors. These concerns, along with broader issues, stimulated this study. Significant associations were found between level of cognitive function and consanguinity, abnormal motor and language milestones, and abnormal electroencephalograms. No significant relationship was found between various dysmorphic feature clusters and measured cognition. Concerns were expressed about demands on family and community for these children, as well as possible larger issues in this heavily consanguineous society. Further research, including epidemiologic studies, was recommended.

Abnormalities, Multiple↗