Frontotemporal and motor neurone degeneration with neurofilament inclusion bodies

Additional evidence for overlap between FTD and ALS

Eileen H. Bigio, A. M. Lipton, C. L. White, D. W. Dickson, A. Hirano

Research output: Contribution to journalArticle

91 Citations (Scopus)

Abstract

We present the case of a patient who had clinical frontal lobe dementia without apparent motor neurone disease (MND), with pathologic findings not typical of any single currently classified frontotemporal degeneration (FTD). At autopsy, the brain had frontal and temporal atrophy with neuronal loss, gliosis, and superficial spongiosis, typical of all FTDs. There were at least three different morphologic types of intracytoplasmic neuronal inclusions in a variety of brain and brainstem regions, including the hippocampal dentate gyrus and pyramidal neurones, the neocortex (in particular, the motor cortex), basal ganglia, thalamus, subthalamic nucleus, basis pontis, and inferior olivary nuclei. Inclusions had the morphologies of Pick-like bodies, pleomorphic inclusions, and hyaline conglomerate (HC)-like inclusions. None of these were positive with tau immunostains. Pick-like bodies in the dentate gyrus were labelled with ubiquitin. The pleomorphic inclusions in the neocortex and dentate gyrus and the HC-like inclusions in the motor and parietal cortex were strongly positive with immunostains for neurofilament. We discuss the differential diagnosis and compare this case with those disorders to which it is most similar. In particular, we compare the unique neurofilament-positive inclusions to the inclusions of FTD-MND, to Pick bodies, and to the basophilic and HC inclusions that are occasionally seen in amytrophic lateral sclerosis (ALS). Although FTD-MND may be found in ALS, the findings in this case may have additional implications for a link between FTD and ALS.

Original languageEnglish (US)
Pages (from-to)239-253
Number of pages15
JournalNeuropathology and Applied Neurobiology
Volume29
Issue number3
DOIs
StatePublished - Jun 2003

Fingerprint

Nerve Degeneration
Motor Neuron Disease
Intermediate Filaments
Inclusion Bodies
Motor Neurons
Hyalin
Dentate Gyrus
Neocortex
Motor Cortex
Olivary Nucleus
Subthalamic Nucleus
Parahippocampal Gyrus
Parietal Lobe
Gliosis
Pyramidal Cells
Brain
Frontal Lobe
Ubiquitin
Basal Ganglia
Thalamus

Keywords

  • Amyotrophic lateral sclerosis
  • Frontotemporal dementia
  • Hyaline conglomerate inclusions
  • Motor neurone disease
  • Neurofilament
  • Pick disease
  • Ribosomes

ASJC Scopus subject areas

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

Cite this

Frontotemporal and motor neurone degeneration with neurofilament inclusion bodies : Additional evidence for overlap between FTD and ALS. / Bigio, Eileen H.; Lipton, A. M.; White, C. L.; Dickson, D. W.; Hirano, A.

In: Neuropathology and Applied Neurobiology, Vol. 29, No. 3, 06.2003, p. 239-253.

Research output: Contribution to journalArticle

Bigio, Eileen H. ; Lipton, A. M. ; White, C. L. ; Dickson, D. W. ; Hirano, A. / Frontotemporal and motor neurone degeneration with neurofilament inclusion bodies : Additional evidence for overlap between FTD and ALS. In: Neuropathology and Applied Neurobiology. 2003 ; Vol. 29, No. 3. pp. 239-253.
@article{d662b2ccf417449aadbb6ce8e1eea183,
title = "Frontotemporal and motor neurone degeneration with neurofilament inclusion bodies: Additional evidence for overlap between FTD and ALS",
abstract = "We present the case of a patient who had clinical frontal lobe dementia without apparent motor neurone disease (MND), with pathologic findings not typical of any single currently classified frontotemporal degeneration (FTD). At autopsy, the brain had frontal and temporal atrophy with neuronal loss, gliosis, and superficial spongiosis, typical of all FTDs. There were at least three different morphologic types of intracytoplasmic neuronal inclusions in a variety of brain and brainstem regions, including the hippocampal dentate gyrus and pyramidal neurones, the neocortex (in particular, the motor cortex), basal ganglia, thalamus, subthalamic nucleus, basis pontis, and inferior olivary nuclei. Inclusions had the morphologies of Pick-like bodies, pleomorphic inclusions, and hyaline conglomerate (HC)-like inclusions. None of these were positive with tau immunostains. Pick-like bodies in the dentate gyrus were labelled with ubiquitin. The pleomorphic inclusions in the neocortex and dentate gyrus and the HC-like inclusions in the motor and parietal cortex were strongly positive with immunostains for neurofilament. We discuss the differential diagnosis and compare this case with those disorders to which it is most similar. In particular, we compare the unique neurofilament-positive inclusions to the inclusions of FTD-MND, to Pick bodies, and to the basophilic and HC inclusions that are occasionally seen in amytrophic lateral sclerosis (ALS). Although FTD-MND may be found in ALS, the findings in this case may have additional implications for a link between FTD and ALS.",
keywords = "Amyotrophic lateral sclerosis, Frontotemporal dementia, Hyaline conglomerate inclusions, Motor neurone disease, Neurofilament, Pick disease, Ribosomes",
author = "Bigio, {Eileen H.} and Lipton, {A. M.} and White, {C. L.} and Dickson, {D. W.} and A. Hirano",
year = "2003",
month = "6",
doi = "10.1046/j.1365-2990.2003.00466.x",
language = "English (US)",
volume = "29",
pages = "239--253",
journal = "Neuropathology and Applied Neurobiology",
issn = "0305-1846",
publisher = "Wiley-Blackwell",
number = "3",

}

TY - JOUR

T1 - Frontotemporal and motor neurone degeneration with neurofilament inclusion bodies

T2 - Additional evidence for overlap between FTD and ALS

AU - Bigio, Eileen H.

AU - Lipton, A. M.

AU - White, C. L.

AU - Dickson, D. W.

AU - Hirano, A.

PY - 2003/6

Y1 - 2003/6

N2 - We present the case of a patient who had clinical frontal lobe dementia without apparent motor neurone disease (MND), with pathologic findings not typical of any single currently classified frontotemporal degeneration (FTD). At autopsy, the brain had frontal and temporal atrophy with neuronal loss, gliosis, and superficial spongiosis, typical of all FTDs. There were at least three different morphologic types of intracytoplasmic neuronal inclusions in a variety of brain and brainstem regions, including the hippocampal dentate gyrus and pyramidal neurones, the neocortex (in particular, the motor cortex), basal ganglia, thalamus, subthalamic nucleus, basis pontis, and inferior olivary nuclei. Inclusions had the morphologies of Pick-like bodies, pleomorphic inclusions, and hyaline conglomerate (HC)-like inclusions. None of these were positive with tau immunostains. Pick-like bodies in the dentate gyrus were labelled with ubiquitin. The pleomorphic inclusions in the neocortex and dentate gyrus and the HC-like inclusions in the motor and parietal cortex were strongly positive with immunostains for neurofilament. We discuss the differential diagnosis and compare this case with those disorders to which it is most similar. In particular, we compare the unique neurofilament-positive inclusions to the inclusions of FTD-MND, to Pick bodies, and to the basophilic and HC inclusions that are occasionally seen in amytrophic lateral sclerosis (ALS). Although FTD-MND may be found in ALS, the findings in this case may have additional implications for a link between FTD and ALS.

AB - We present the case of a patient who had clinical frontal lobe dementia without apparent motor neurone disease (MND), with pathologic findings not typical of any single currently classified frontotemporal degeneration (FTD). At autopsy, the brain had frontal and temporal atrophy with neuronal loss, gliosis, and superficial spongiosis, typical of all FTDs. There were at least three different morphologic types of intracytoplasmic neuronal inclusions in a variety of brain and brainstem regions, including the hippocampal dentate gyrus and pyramidal neurones, the neocortex (in particular, the motor cortex), basal ganglia, thalamus, subthalamic nucleus, basis pontis, and inferior olivary nuclei. Inclusions had the morphologies of Pick-like bodies, pleomorphic inclusions, and hyaline conglomerate (HC)-like inclusions. None of these were positive with tau immunostains. Pick-like bodies in the dentate gyrus were labelled with ubiquitin. The pleomorphic inclusions in the neocortex and dentate gyrus and the HC-like inclusions in the motor and parietal cortex were strongly positive with immunostains for neurofilament. We discuss the differential diagnosis and compare this case with those disorders to which it is most similar. In particular, we compare the unique neurofilament-positive inclusions to the inclusions of FTD-MND, to Pick bodies, and to the basophilic and HC inclusions that are occasionally seen in amytrophic lateral sclerosis (ALS). Although FTD-MND may be found in ALS, the findings in this case may have additional implications for a link between FTD and ALS.

KW - Amyotrophic lateral sclerosis

KW - Frontotemporal dementia

KW - Hyaline conglomerate inclusions

KW - Motor neurone disease

KW - Neurofilament

KW - Pick disease

KW - Ribosomes

UR - http://www.scopus.com/inward/record.url?scp=0038509194&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0038509194&partnerID=8YFLogxK

U2 - 10.1046/j.1365-2990.2003.00466.x

DO - 10.1046/j.1365-2990.2003.00466.x

M3 - Article

VL - 29

SP - 239

EP - 253

JO - Neuropathology and Applied Neurobiology

JF - Neuropathology and Applied Neurobiology

SN - 0305-1846

IS - 3

ER -