Intranuclear inclusion bodies in an elderly demented woman: A form of intranuclear inclusion body disease

K. M. Weidenheim, Dennis W Dickson

Research output: Contribution to journalArticle

36 Citations (Scopus)

Abstract

Intranuclear inclusion body disease (NIBD) is a rare neuropathological entity characterized by eosinophilic intranuclear bodies in neurons and/for glia. While this disease generally occurs in children, in whom it presents as a multiple systems degeneration, a few adult cases are also described. Only 4 previously reported adult cases have had an associated dementia and all of these patients had additional significant neurological abnormalities. We report a 72-year-old woman with primary degenerative dementia in whom intranuclear inclusion bodies (INIB) were a major neuropathologic finding. The INIB were most easily found in astrocytes of Alzheimer II type, which had proliferated in the cortex and white matter. Occasional neurons were affected. The inclusions consisted of 13 nm diameter filaments associated with amorphous electron-dense material, arranged in a random pattern without lattice formation. They did not stain with antibodies against all 3 neurofilament subunits, glial fibrillary acidic protein, tau-1 protein, vimentin, keratin or actin. We conclude that INIBD is a rare substrate of primary degenerative dementia in elderly patients.

Original languageEnglish (US)
Pages (from-to)93-99
Number of pages7
JournalClinical Neuropathology
Volume14
Issue number2
StatePublished - 1995
Externally publishedYes

Fingerprint

Intranuclear Inclusion Bodies
Cytomegalovirus Infections
Dementia
Neurons
tau Proteins
Intermediate Filaments
Glial Fibrillary Acidic Protein
Vimentin
Keratins
Neuroglia
Astrocytes
Actins
Coloring Agents
Electrons
Antibodies

Keywords

  • Dementia
  • Nuclear inclusions
  • Nucleus

ASJC Scopus subject areas

  • Clinical Neurology
  • Pathology and Forensic Medicine
  • Neuroscience(all)

Cite this

Intranuclear inclusion bodies in an elderly demented woman : A form of intranuclear inclusion body disease. / Weidenheim, K. M.; Dickson, Dennis W.

In: Clinical Neuropathology, Vol. 14, No. 2, 1995, p. 93-99.

Research output: Contribution to journalArticle

@article{cdc9991b49d94780ad2ef7176ec59047,
title = "Intranuclear inclusion bodies in an elderly demented woman: A form of intranuclear inclusion body disease",
abstract = "Intranuclear inclusion body disease (NIBD) is a rare neuropathological entity characterized by eosinophilic intranuclear bodies in neurons and/for glia. While this disease generally occurs in children, in whom it presents as a multiple systems degeneration, a few adult cases are also described. Only 4 previously reported adult cases have had an associated dementia and all of these patients had additional significant neurological abnormalities. We report a 72-year-old woman with primary degenerative dementia in whom intranuclear inclusion bodies (INIB) were a major neuropathologic finding. The INIB were most easily found in astrocytes of Alzheimer II type, which had proliferated in the cortex and white matter. Occasional neurons were affected. The inclusions consisted of 13 nm diameter filaments associated with amorphous electron-dense material, arranged in a random pattern without lattice formation. They did not stain with antibodies against all 3 neurofilament subunits, glial fibrillary acidic protein, tau-1 protein, vimentin, keratin or actin. We conclude that INIBD is a rare substrate of primary degenerative dementia in elderly patients.",
keywords = "Dementia, Nuclear inclusions, Nucleus",
author = "Weidenheim, {K. M.} and Dickson, {Dennis W}",
year = "1995",
language = "English (US)",
volume = "14",
pages = "93--99",
journal = "Clinical Neuropathology",
issn = "0722-5091",
publisher = "Dustri-Verlag Dr. Karl Feistle",
number = "2",

}

TY - JOUR

T1 - Intranuclear inclusion bodies in an elderly demented woman

T2 - A form of intranuclear inclusion body disease

AU - Weidenheim, K. M.

AU - Dickson, Dennis W

PY - 1995

Y1 - 1995

N2 - Intranuclear inclusion body disease (NIBD) is a rare neuropathological entity characterized by eosinophilic intranuclear bodies in neurons and/for glia. While this disease generally occurs in children, in whom it presents as a multiple systems degeneration, a few adult cases are also described. Only 4 previously reported adult cases have had an associated dementia and all of these patients had additional significant neurological abnormalities. We report a 72-year-old woman with primary degenerative dementia in whom intranuclear inclusion bodies (INIB) were a major neuropathologic finding. The INIB were most easily found in astrocytes of Alzheimer II type, which had proliferated in the cortex and white matter. Occasional neurons were affected. The inclusions consisted of 13 nm diameter filaments associated with amorphous electron-dense material, arranged in a random pattern without lattice formation. They did not stain with antibodies against all 3 neurofilament subunits, glial fibrillary acidic protein, tau-1 protein, vimentin, keratin or actin. We conclude that INIBD is a rare substrate of primary degenerative dementia in elderly patients.

AB - Intranuclear inclusion body disease (NIBD) is a rare neuropathological entity characterized by eosinophilic intranuclear bodies in neurons and/for glia. While this disease generally occurs in children, in whom it presents as a multiple systems degeneration, a few adult cases are also described. Only 4 previously reported adult cases have had an associated dementia and all of these patients had additional significant neurological abnormalities. We report a 72-year-old woman with primary degenerative dementia in whom intranuclear inclusion bodies (INIB) were a major neuropathologic finding. The INIB were most easily found in astrocytes of Alzheimer II type, which had proliferated in the cortex and white matter. Occasional neurons were affected. The inclusions consisted of 13 nm diameter filaments associated with amorphous electron-dense material, arranged in a random pattern without lattice formation. They did not stain with antibodies against all 3 neurofilament subunits, glial fibrillary acidic protein, tau-1 protein, vimentin, keratin or actin. We conclude that INIBD is a rare substrate of primary degenerative dementia in elderly patients.

KW - Dementia

KW - Nuclear inclusions

KW - Nucleus

UR - http://www.scopus.com/inward/record.url?scp=0028919786&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0028919786&partnerID=8YFLogxK

M3 - Article

C2 - 7606903

AN - SCOPUS:0028919786

VL - 14

SP - 93

EP - 99

JO - Clinical Neuropathology

JF - Clinical Neuropathology

SN - 0722-5091

IS - 2

ER -