Dominick P. Purpura

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Golgi studies reveal abnormally long, thin spines and the absence of short, thick spines on dendrites of cortical neurons in retarded children with normal karyotypes. The degree of dendritic spine loss and abnormality appears to be related to age and the severity of developmental retardation. Dendritic spine "dysgenesis" is a common feature of the(More)
Four persons who exhibited prominent autistic features throughout life died when 4, 14, 27, and 33 years old. All were mentally retarded. One had documented phenylketonuria, but the cause of mental retardation and autistic behavior was undefined in three. At the time of autopsy, brain weights were within 2 SDs of the norm for age. Complete neuropathologic(More)
Intracellular recording and intracellular HRP staining were employed to trace the recurrent terminal plexus of cat substantia nigra pars reticulata neurons. Autaptic neurons were labeled. The axon of an autaptic neuron was found to emit a recurrent collateral which distributed 'en passage' and terminal boutons contacting dendrites of the parent cell.(More)
Cortical biopsies obtained from 5 young children with severe neurobehavioral retardation of unknown etiology have been analyzed using Golgi and EM techniques. The normally cylindrical geometry of individual dendritic processes of pyramidal and non-pyramidal neurons is interrupted by the formation of distinct varicosities. While over 90% of observed cells(More)
Golgi and electron microscope studies of cortical neurons in several lysosomal storage diseases were carried out to elucidate structural features of the large neural processes (meganeurites) that develop as storage sites for accumulated undigestible substrates. Meganeurites occur preferentially in pyramidal neurons wherein they develop between the base of(More)
Clinical and pathological studies are reported from investigation of a 27-year-old man with GM1 gangliosidosis who experienced a slowly progressive dystonia that began about age 4, primarily affected the face and limbs, and eventually became almost totally incapacitating. There was only mild intellectual deterioration; myoclonus, seizures, and macular(More)