Adult polyglucosan body disease
Springer Online Journal Archives 1860-2000
Abstract The significance of the development of polyglucosan bodies (PBs) in the CNS is incompletely understood. We present the clinicopathological features of three autopsy cases with numerous PBs other than the common corpora amylacea or Lafora bodies. The first patient had pleomorphic PBs in the neuronal processes of pallidum and substantia nigra which, thus, are consistent with Bielschowsky bodies. Bielschowsky bodies involved also the hypothalamus and tegmentum of midbrain and medulla. The present case was the first not associated with any clinical symptoms. The second patient also had incidental Bielschowsky bodies in the external pallidum, substantia nigra, and pallidothalamic, pallidonigral and nigrostriatal tracts. Additionally, unique clusters of small PBs appeared in the cerebral cortex, putamen, pallidum, and caudate nucleus. Immunostaining suggested that these small clustered PBs were located in the cytoplasm and processes of astrocytes. Ultrastructurally, these clustered PBs were in agreement with previous descriptions of PBs. The third patient had adult polyglucosan body disease. Most PBs in the white matter were corpora amylacea situated in astrocytic processes or axons. In the gray matter, many pleomorphic PBs resembling Bielschowsky bodies occurred in neuronal processes. In the peripheral nervous system, a few PBs were seen in myelinated axons. The following conclusions may be drawn from this study: (1) each type of PBs develops in distinct cell types of the human brain in variable distribution; (2) Bielschowsky bodies may not manifest clinically; (3) PBs other than corpora amylacea or Lafora bodies may be distributed in localized or diffuse patterns; (4) in the localized pattern (patients 1 and 2), PBs occur as Bielschowsky bodies or clustered PBs, and tend to involve certain systems (pallidum, striatum, and substantia nigra); and (5) in the diffuse pattern (patient 3), PBs develop as corpora amylacea and Bielschowsky-like bodies of adult polyglucosan body disease.
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