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Peripheral nerve polyglucosan inclusion bodies

MedGen UID:
1841731
•Concept ID:
C5826693
•
Finding
HPO: HP:0034834

Definition

Accumulation of a poorly branched and poorly spherical amylopectin-like glycogen (polyglucosan) in peripheral nerve fibers. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPeripheral nerve polyglucosan inclusion bodies

Recent clinical studies

Etiology

Corpus amylaceum (polyglucosan body) in the peripheral olfactory system.
Leel-Ossy L, Gáti I
Pathol Oncol Res 1998;4(3):212-6. doi: 10.1007/BF02905251. PMID: 9761940

Diagnosis

Congenital type IV glycogenosis: the spectrum of pleomorphic polyglucosan bodies in muscle, nerve, and spinal cord with two novel mutations in the GBE1 gene.
Nolte KW, Janecke AR, Vorgerd M, Weis J, Schröder JM
Acta Neuropathol 2008 Nov;116(5):491-506. Epub 2008 Jul 26 doi: 10.1007/s00401-008-0417-8. PMID: 18661138
Adult polyglucosan body disease associated with lewy bodies and tremor.
Trivedi JR, Wolfe GI, Nations SP, Burns DK, Bryan WW, Dewey RB Jr
Arch Neurol 2003 May;60(5):764-6. doi: 10.1001/archneur.60.5.764. PMID: 12756142
Adult polyglucosan body disease.
Milde P, Guccion JG, Kelly J, Locatelli E, Jones RV
Arch Pathol Lab Med 2001 Apr;125(4):519-22. doi: 10.5858/2001-125-0519-APBD. PMID: 11260627
Childhood-onset spinocerebellar syndrome associated with massive polyglucosan body deposition.
Felice KJ, Grunnet ML, Rao KR, Wolfson LI
Acta Neurol Scand 1997 Jan;95(1):60-4. doi: 10.1111/j.1600-0404.1997.tb00070.x. PMID: 9048988
Adult polyglucosan body disease (APBD).
Gray F, Gherardi R, Marshall A, Janota I, Poirier J
J Neuropathol Exp Neurol 1988 Jul;47(4):459-74. doi: 10.1097/00005072-198807000-00007. PMID: 2838589

Therapy

A 63-year-old woman with urinary incontinence and progressive gait disorder.
Lossos A, Klein CJ, McEvoy KM, Keegan BM
Neurology 2009 May 5;72(18):1607-13. doi: 10.1212/WNL.0b013e3181a413fe. PMID: 19414729

Prognosis

Adult polyglucosan body disease associated with lewy bodies and tremor.
Trivedi JR, Wolfe GI, Nations SP, Burns DK, Bryan WW, Dewey RB Jr
Arch Neurol 2003 May;60(5):764-6. doi: 10.1001/archneur.60.5.764. PMID: 12756142
Probable adult polyglucosan body disease.
Klein CM, Bosch EP, Dyck PJ
Mayo Clin Proc 2000 Dec;75(12):1327-31. doi: 10.4065/75.12.1327. PMID: 11126844
Corpus amylaceum (polyglucosan body) in the peripheral olfactory system.
Leel-Ossy L, Gáti I
Pathol Oncol Res 1998;4(3):212-6. doi: 10.1007/BF02905251. PMID: 9761940
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.
Robitaille Y, Carpenter S, Karpati G, DiMauro SD
Brain 1980 Jun;103(2):315-36. doi: 10.1093/brain/103.2.315. PMID: 6249438

Clinical prediction guides

Adult polyglucosan body disease associated with lewy bodies and tremor.
Trivedi JR, Wolfe GI, Nations SP, Burns DK, Bryan WW, Dewey RB Jr
Arch Neurol 2003 May;60(5):764-6. doi: 10.1001/archneur.60.5.764. PMID: 12756142
Intraneuronal polyglucosan bodies and peculiar inclusions in the midbrain-tegmentum of the aged.
Ikeda K, Kosaka K, Hori A
Jpn J Psychiatry Neurol 1987 Dec;41(4):719-23. doi: 10.1111/j.1440-1819.1987.tb00430.x. PMID: 2839726
Polyglucosan bodies in the sural nerve of a diabetic patient with polyneuropathy.
Mancardi GL, Schenone A, Tabaton M, Tassinari T, Mainardi P
Acta Neuropathol 1985;66(1):83-6. doi: 10.1007/BF00698301. PMID: 2986406

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