RESUMEN
BACKGROUND AND PURPOSE: Voxel-based analysis has suggested that deep gray matter rather than cortical regions is initially affected in adult Niemann-Pick type C. We sought to examine a range of deep gray matter structures in adults with NPC and relate these to clinical variables. MATERIALS AND METHODS: Ten adult patients with NPC (18-49 years of age) were compared with 27 age- and sex-matched controls, and subcortical structures were automatically segmented from normalized T1-weighted MR images. Absolute volumes (in cubic millimeters) were generated for a range of deep gray matter structures and were compared between groups and correlated with illness variables. RESULTS: Most structures were smaller in patients with NPC compared with controls. The thalamus, hippocampus, and striatum showed the greatest and most significant reductions, and left hippocampal volume correlated with symptom score and cognition. Vertex analysis of the thalamus, hippocampus, and caudate implicated regions involved in memory, executive function, and motor control. CONCLUSIONS: Thalamic and hippocampal reductions may underpin the memory and executive deficits seen in adult NPC. Volume losses in other subcortical regions may also be involved in the characteristic range of motor, psychiatric, and cognitive deficits seen in the disease.
Asunto(s)
Encéfalo/patología , Imagen por Resonancia Magnética/métodos , Enfermedad de Niemann-Pick Tipo C/patología , Adolescente , Adulto , Amígdala del Cerebelo/patología , Estudios de Casos y Controles , Núcleo Caudado/patología , Cognición/fisiología , Estudios de Cohortes , Cuerpo Estriado/patología , Estudios Transversales , Función Ejecutiva/fisiología , Femenino , Hipocampo/patología , Humanos , Aumento de la Imagen/métodos , Procesamiento de Imagen Asistido por Computador/métodos , Imagenología Tridimensional/métodos , Masculino , Memoria/fisiología , Persona de Mediana Edad , Destreza Motora/fisiología , Tamaño de los Órganos , Putamen/patología , Tálamo/patología , Adulto JovenRESUMEN
A battery of tests was administered to 17 patients with congenital adrenal hyperplasia (CAH) and 17 normal controls to investigate the effect of prenatal androgen exposure on cerebral lateralization and cognitive performance. Individuals were compared on measures of hand preference, verbal and performance IQ, and temporal processing asymmetry. A higher incidence of left-handedness was found among CAH participants. CAH individuals exhibited higher performance IQs as opposed to verbal IQs. Temporal processing asymmetries were investigated using an auditory gap detection task. Measures of reaction time and response error revealed a right-ear, therefore left-hemisphere, advantage for gap detection. This right-ear advantage did not differ between CAH individuals and controls. Results partially support the hypothesis that prenatal androgen exposure causes a shift in cerebral lateralization toward right-hemisphere dominance.
