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NEUROLOGY 1986;36:785
© 1986 American Academy of Neurology

Adult-onset neuronal intra nuclear hyaline inclusion disease

D. Munoz-Garcia, MD, FRCP(C) and S. K. Ludwin, MBBCh, FRCP(C)

Department of Pathology (Neuropathology), Queen's University, Kingston, Ontario, Canada.

We studied the clinical and pathologic features of two cases of neuronal intra nuclear hyaline inclusion disease. The cases were unique in late onset, presentation with dementia, possible autosomal dominant pattern of inheritance (in one patient), predominance of inclusions in glial cells, and mineral deposits within some inclusions. Differences from other reported cases indicate that this is probably not a homogeneous entity.

Address correspondence and reprint requests to Dr. Munoz-Garcia, Department of Pathology (Neuropathology), University Hospital, University of Saskatchewan, Saskatoon, Saskatchewan, Canada.

Presented in part at the thirty-seventh annual meeting of the American Academy of Neurology, Dallas, TX, April 1985.

Accepted for publication October 14, 1985.




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