Summary
Polyglucosan bodies in the nervous system and other viscera are the main findings at autopsy of a 64-year-old woman who had a chronic neurologic disorder of 20 years' duration. The clinical features included muscle weakness, sensory disturbances, neurogenic bladder, dementia, and cataracts. Light and electron microscopy of the nervous system showed numerous polyglucosan bodies in the processes of neurons and astrocytes but not in neuronal perikarya. A similar clinico-pathologic presentation has been described in six previous cases. It is suggested that this is a type of adult polyglucosan body disease which probably forms a variant of Lafora's disease.
References
Austin JH, Sakai M (1972) Corpora amylacea. In: Minckler J (ed) Pathology of the nervous system, vol 3, McGraw-Hill, New York, pp 2961–2968
Averback P, Langevin H (1978) Corpora amylacea of the lumbar spinal cord and peripheral nervous system. Arch Neurol 35: 95–96
Barz H, Kemmer Ch, Kunze D, Sachs B (1976) Amyotrophe Lateralsklerose mit Myoklonuskörpern. Zentralbl Allg Pathol 120:333–342
Cajal SR y, Blanes A, Martinez A, Sáenz E, Gutierrez M (1974) Lafora's disease. An ultrastructural and histochemical study. Acta Neuropathol (Berl) 30:189–196
Carpenter S, Karpati G, Robitaille Y, Melmed C (1978) Adult polyglucosan body axonopathy. A distinct chronic neurological disease. J Neuropathol Exp Neurol 37:598 (Abstr)
Hirano A (1974) Some fine structural alterations of the central nervous system in aging. In: Környey St, Tariska St, Gosztonyi G (eds) Proceedings of the 7th International Congress of Neuropathology, vol 2. Excerpta Medica, Amsterdam, pp 83–90
Jakob H (1969) Ablagerungen im Zentralnervensystem bei der protrahierten Verlaufsform (Typ Lundborg) der Myoklonus-körperkrankheit. Acta Neuropathol (Berl) 12:260–275
Kraus-Ruppert R, Ostertag B, Häfner H (1970) A study of the late form (type Lundborg) of progressive myoclonic epilepsy. J Neurol Sci 11:1–15
McMaster KR, Powers JM, Henningar GR Jr, Wohltmann HJ, Farr GH Jr (1979) Nervous system involvement in type IV glycogenosis. Arch Pathol Lab Med 103:105–111
Orthner H, Becker PE, Müller D (1973) Recessiv erbliche amyotrophische Lateralsklerose mit ‘Lafora-Körpern’. Arch Psychiat Nervenkrankh 217:387–412
Peress NS, DiMauro S, Roxburgh VA (1979) Adult polysaccharidosis. Clinicopathological, ultrastructural, and biochemical features. Arch Neurol 36:840–845
Probst A, Sandoz P, Vanoni Ch, Baumann JU (1980) Intraneuronal polyglucosan storage restructed to the lateral pallidum (Bielschowsky bodies). A Golgi, light, and electron microscopic study. Acta Neuropathol (Berl) 51:119–126
Ramsey HJ (1965) Ultrastructure of corpora amylacea. J Neuropathol Exp Neurol 24:25–39
Robitaille Y, Carpenter S, Karpati G, DiMauro S (1980) A distinct form of adult polyglucosan body disease with massive involvement of central and peripheral neuronal processes and astrocytes. A report of four cases and a review of the occurrence of polyglucosan bodies in other conditions, such as Lafora's disease and normal ageing. Brain 103:315–336
Schwarz GA, Yanoff M (1965) Lafora's disease. Distinct clinico-pathologic form of Unverricht's syndrome. Arch Neurol 12:172–188
Seitelberger F (1968) Myoclonus body disease. In: Minckler J (ed) Pathology of the nervous system, vol 1. McGraw-Hill, New York, pp 1121–1134
Stam FC, Wigboldus JM, Bots GThAM (1980) Presenile dementia. A form of Lafora disease. J Am Geriatr Soc 28:237–240
Suzuki K, David E, Kutschman B (1971) Presenile dementia with “Lafora-like” intraneuronal inclusions. Arch Neurol 25:69–80
Vogel F, Häfner H, Diebold K (1965) Zur Genetik der progressiven Myoklonusepilepsien (Unverricht-Lundborg). Humangenetik 1:437–475
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Okamoto, K., Llena, J.F. & Hirano, A. A type of adult polyglucosan body disease. Acta Neuropathol 58, 73–77 (1982). https://doi.org/10.1007/BF00692701
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00692701