Neurology
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Perception of facial expression and facial identity in subjects with social developmental disorders.
It has been hypothesized that the social dysfunction in social developmental disorders (SDDs), such as autism, Asperger disorder, and the socioemotional processing disorder, impairs the acquisition of normal face-processing skills. The authors investigated whether this purported perceptual deficit was generalized to both facial expression and facial identity or whether these different types of facial perception were dissociated in SDDs. ⋯ The processing of facial identity and that of facial expression are dissociable in social developmental disorders. Deficits in perceiving facial expression may be related to emotional processing more than face processing. Dissociations between the perception of facial identity and facial emotion are consistent with current cognitive models of face processing. The results argue against hypotheses that the social dysfunction in social developmental disorder causes a generalized failure to acquire face-processing skills.
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Case Reports
Neuronal intranuclear hyaline inclusion disease showing motor-sensory and autonomic neuropathy.
Neuronal intranuclear hyaline inclusion disease (NIHID), a rare neurodegenerative disease in which eosinophilic intranuclear inclusions develop mainly in neurons, has not yet been described to present as hereditary motor-sensory and autonomic neuropathy. ⋯ Patients with neuronal intranuclear hyaline inclusion disease (NIHID) can manifest symptoms limited to those of peripheral neuropathy. NIHID therefore is part of the differential diagnosis of hereditary motor-sensory neuropathy associated with autonomic symptoms. Intranuclear hyaline inclusions in Schwann cells and in the myenteric plexus may permit antemortem diagnosis of NIHID.