Asymmetrical temporal lobe atrophy with massive neuronal inclusions in multiple system atrophy
Introduction
Multiple system atrophy (MSA) includes olivopontocerebellar atrophy (OPCA), striatonigral degeneration (SND) and Shy–Drager syndrome [1]. The clinicopathological unity of these syndromes is supported by the presence of characteristic argyrophilic cytoplasmic inclusions in the oligodendroglia and neurons [2], [3], [4], [5], [6], [7], [8], [9]. Rarely, this disease may be associated with Alzheimer’s disease [10], progressive supranuclear palsy [11], or atypical Pick’s disease [12]. This study documents the case of a patient with MSA who displays asymmetrical temporal lobe atrophy with neuronal intracytoplasmic inclusions having some features of Pick bodies.
Section snippets
Case report
A 53-year-old woman, the proprietress of ‘ryokan’ (Japanese inn) developed an unsteady gait, dysarthria, and impairment of memory. The dysmnesia gradually became worse. She often forgot the telephone number of booking visitors and was unable to continue her work as proprietress. At age 55 years, she was diagnosed as having OPCA by one of the authors (K.I.). The patient developed depressive state and neurogenic bladder that gradually progressed. She was admitted to Kanagawa Rehabilitation Center
Subject and methods
The brain was fixed in 20% formalin solution for a week and the representative sections including large hemispheric sections of the cerebrum and cerebellum were embedded in paraffin wax. The routine stains employed were hematoxylin-eosin, Klüver–Barrera for myelin, Holzer for glial fiber, Bodian and Bielschowsky silver impregnation, and Gallyas silver development.
For electron microscopy, a small fragment of Ammon’s horn was taken from the formalin-fixed brain (postmortem delay of 6 h). The
Neuropathologic findings
The brain weight was 920 g. Marked atrophy of the cerebellum and pons was present (Fig. 3a). On coronal sections, the putamen was atrophic and showed brown discoloration. The substantia nigra was totally depigmented. In addition, the right anterior temporal lobe and hippocampus appeared considerably atrophic, and in contrast, the left one was spared, being asymmetrical (Fig. 3b).
Microscopically, the cerebellar hemispheres showed considerable loss of Purkinje cells and thinning of the granular
Discussion
In the present case, there was severe neuronal loss and gliosis in the olivopontocerebellar system involving the cerebellar cortex, and in the striatonigral system. In addition, oligodendroglial argyrophilic inclusions [2], [3], [4], [8], [9], [13] were widely distributed throughout the brain. So, this case was easily diagnosed as MSA [1], [14].
Interestingly, the patient developed dysmnesia and subsequent disorientation even in the early stage of the disease. The cerebrum showed asymmetrical
References (27)
- et al.
Glial cytoplasmic inclusions in the CNS of patients with multiple system atrophy (striatonigral degeneration, olivopontocerebellar atrophy and Shy–Drager syndrome)
J Neurol Sci
(1989) - et al.
Orthostatic hypotension and nicotine sensitivity in a case of multiple system atrophy
J Neurol Neurosurg Psychiatry
(1969) - et al.
Oligodendroglial microtubular tangles in olivopontocerebellar atrophy
J Neuropathol Exp Neurol
(1990) - et al.
Immunohistochemical study of glial cytoplasmic inclusion in multiple system atrophy
Brain Nerve
(1991) - et al.
Argyrophilic glial intracytoplasmic inclusions in multiple system atrophy: immunocytochemical and ultrastructural study
Acta Neuropathol
(1992) - et al.
Cytoskeletal protein abnormalities in patients with olivopontocerebellar atrophy — an immunocytochemical and Gallyas silver impregnation study
J Neuropathol Appl Neurobiol
(1992) - et al.
Argentophilic intracytoplasmic inclusions in multiple system atrophy
J Neurol
(1992) - et al.
The distribution of oligodendroglial inclusions in multiple system atrophy and its relevance to clinical symptomatology
Brain
(1994) - et al.
Oligodendroglial microtubular tangles in multiple system atrophy
Neuropathology
(1996) - et al.
Striatonigral degeneration combined with Alzheimer’s disease
Acta Neuropathol
(1981)