Asymmetrical temporal lobe atrophy with massive neuronal inclusions in multiple system atrophy

This report concerns a rare association of asymmetrical temporal lobe atrop hy with multiple system atrophy (MSA). A 53-year-old Japanese woman develop ed cerebellar ataxia and parkinsonism and was diagnosed as olivopontocerebe llar atrophy (OPCA). This patient showed forgetfulness and subsequent disor ientation even in the early stage of the disease. She fell into a decortica te state at the age of 64, and died a year later. The autopsy showed MSA wi th asymmetrical atrophy of temporal lobes, intraneuronal globular inclusion s mostly confined to the hippocampus, amygdaloid nucleus, and most abundant in the granule cells in the dentate fascia. These inclusions were intensel y argyrophilic and expressed marked immunoreactivity to ubiquitin, but not to neurofilament (NF), tau and paired helical filaments (PHF). Ultrastructu rally, they were composed of scattered short filamentous structures of 15 t o 30 nm in diameter, ribosome-like granules, mitochondria and lipofuscin. T he lack of immunoreactivity against tau, NF and PHF suggests that the inclu sions are distinct from Pick bodies. To our knowledge, MSA in association w ith asymmetrical temporal lobe atrophy with the present neuronal inclusions has not been reported. This case is distinct from MSA combined with atypic al Pick's disease in the distribution and immunohistochemical properties of neuronal inclusions, and may present a new variant of MSA since the neuron al inclusions are similar, in many respects, to those of neuronal inclusion s reported in MSA. Globular inclusions are also discussed in variants of Pi ck's disease, amyotrophic lateral sclerosis and Alzheimer's disease. (C) 20 00 Published by Elsevier Science BN.