Acquired dementia with retinitis pigmentosa.
The coexistence of mental retardation and retinitis pigmentosa has been well described in a number of congenital syndromes such as the Lawrence-Moon and Bardet-Biedl disorders. In contrast to this, little information is available regarding the association of acquired dementia with retinitis pigmentosa. This report summarizes the clinical features of a developmentally normal adult having the unusual symptom complex of retinitis pigmentosa, early-onset dementia, sensorineural hearing loss, and sensorimotor neuropathy. Though the coexistence of retinitis pigmentosa and dementia in this particular patient may be completely coincidental, it could be representative of a previously unrecognized subset of dementing illness. The literature pertaining to related symptom complexes is reviewed and discussed.
Grollman
E M
EM
Department of Neurology, University of Rochester School of Medicine and Dentistry, NY.
Papacostas
S S
SS
D001284:Atrophy; D002540:Cerebral Cortex / Q000473:pathology; D003704:Dementia / Q000150:complications* / Q000175:diagnosis / Q000235:genetics; D005654:Fundus Oculi; D006319:Hearing Loss, Sensorineural / Q000150:complications* / Q000175:diagnosis / Q000235:genetics; D006801:Humans; D008297:Male; D008609:Mental Status Schedule; D008875:Middle Aged; D009460:Neurologic Examination; D009896:Optic Atrophy / Q000150:complications / Q000175:diagnosis / Q000235:genetics; D012174:Retinitis Pigmentosa / Q000150:complications* / Q000175:diagnosis / Q000235:genetics; D014057:Tomography, X-Ray Computed; D014888:Wechsler Scales