Basophilic inclusion body disease and neuronal intermediate filament inclusion disease: A comparative clinicopathological study

Osamu Yokota, Kuniaki Tsuchiya, Seishi Terada, Hideki Ishizu, Hirotake Uchikado, Manabu Ikeda, Kiyomitsu Oyanagi, Imaharu Nakano, Shigeo Murayama, Shigetoshi Kuroda, Haruhiko Akiyama

Research output: Contribution to journalArticle

53 Citations (Scopus)

Abstract

While both neuronal intermediate filament inclusion disease (NIFID) and basophilic inclusion body disease (BIBD) show frontotemporal lobar degeneration and/or motor neuron disease, it remains unclear whether, and how, these diseases differ from each other. Here, we compared the clinicopathological characteristics of four BIBD and two NIFID cases. Atypical initial symptoms included weakness, dysarthria, and memory impairment in BIBD, and dysarthria in NIFID. Dementia developed more than 1 year after the onset in some BIBD and NIFID cases. Upper and lower motor neuron signs, parkinsonism, and parietal symptoms were noted in both diseases, and involuntary movements in BIBD. Pathologically, severe caudate atrophy was consistently found in both diseases. Cerebral atrophy was distributed in the convexity of the fronto-parietal region in NIFID cases. In both BIBD and NIFID, the frontotemporal cortex including the precentral gyrus, caudate nucleus, putamen, globus pallidus, thalamus, amygdala, hippocampus including the dentate gyrus, substantia nigra, and pyramidal tract were severely affected, whereas lower motor neuron degeneration was minimal. While α-internexin-positive inclusions without cores were found in both NIFID cases, one NIFID case also had α-internexin- and neurofilament-negative, but p62-positive, cytoplasmic spherical inclusions with eosinophilic p62-negative cores. These two types of inclusions frequently coexisted in the same neuron. In three BIBD cases, inclusions were tau-, α-synuclein-, α-internexin-, and neurofilament-negative, but occasionally p62-positive. These findings suggest that: (1) the clinical features and distribution of neuronal loss are similar in BIBD and NIFID, and (2) an unknown protein besides α-internexin and neurofilament may play a pivotal pathogenetic role in at least some NIFID cases.

Original languageEnglish
Pages (from-to)561-575
Number of pages15
JournalActa Neuropathologica
Volume115
Issue number5
DOIs
Publication statusPublished - May 2008

Fingerprint

Intermediate Filaments
Inclusion Bodies
Cytomegalovirus Infections
Dysarthria
Motor Neurons
Atrophy
Synucleins
Frontotemporal Lobar Degeneration
Hospital Distribution Systems
Nerve Degeneration
Motor Neuron Disease
Parietal Lobe
Pyramidal Tracts
Globus Pallidus
Caudate Nucleus
Putamen
Dentate Gyrus
Dyskinesias
Parkinsonian Disorders
Frontal Lobe

Keywords

  • α-Internexin
  • Caudate nucleus
  • Frontotemporal dementia
  • Motor neuron disease
  • TDP-43

ASJC Scopus subject areas

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

Cite this

Basophilic inclusion body disease and neuronal intermediate filament inclusion disease : A comparative clinicopathological study. / Yokota, Osamu; Tsuchiya, Kuniaki; Terada, Seishi; Ishizu, Hideki; Uchikado, Hirotake; Ikeda, Manabu; Oyanagi, Kiyomitsu; Nakano, Imaharu; Murayama, Shigeo; Kuroda, Shigetoshi; Akiyama, Haruhiko.

In: Acta Neuropathologica, Vol. 115, No. 5, 05.2008, p. 561-575.

Research output: Contribution to journalArticle

Yokota, O, Tsuchiya, K, Terada, S, Ishizu, H, Uchikado, H, Ikeda, M, Oyanagi, K, Nakano, I, Murayama, S, Kuroda, S & Akiyama, H 2008, 'Basophilic inclusion body disease and neuronal intermediate filament inclusion disease: A comparative clinicopathological study', Acta Neuropathologica, vol. 115, no. 5, pp. 561-575. https://doi.org/10.1007/s00401-007-0329-z
Yokota, Osamu ; Tsuchiya, Kuniaki ; Terada, Seishi ; Ishizu, Hideki ; Uchikado, Hirotake ; Ikeda, Manabu ; Oyanagi, Kiyomitsu ; Nakano, Imaharu ; Murayama, Shigeo ; Kuroda, Shigetoshi ; Akiyama, Haruhiko. / Basophilic inclusion body disease and neuronal intermediate filament inclusion disease : A comparative clinicopathological study. In: Acta Neuropathologica. 2008 ; Vol. 115, No. 5. pp. 561-575.
@article{76a50dc395c046f2ab187ec30d3439a3,
title = "Basophilic inclusion body disease and neuronal intermediate filament inclusion disease: A comparative clinicopathological study",
abstract = "While both neuronal intermediate filament inclusion disease (NIFID) and basophilic inclusion body disease (BIBD) show frontotemporal lobar degeneration and/or motor neuron disease, it remains unclear whether, and how, these diseases differ from each other. Here, we compared the clinicopathological characteristics of four BIBD and two NIFID cases. Atypical initial symptoms included weakness, dysarthria, and memory impairment in BIBD, and dysarthria in NIFID. Dementia developed more than 1 year after the onset in some BIBD and NIFID cases. Upper and lower motor neuron signs, parkinsonism, and parietal symptoms were noted in both diseases, and involuntary movements in BIBD. Pathologically, severe caudate atrophy was consistently found in both diseases. Cerebral atrophy was distributed in the convexity of the fronto-parietal region in NIFID cases. In both BIBD and NIFID, the frontotemporal cortex including the precentral gyrus, caudate nucleus, putamen, globus pallidus, thalamus, amygdala, hippocampus including the dentate gyrus, substantia nigra, and pyramidal tract were severely affected, whereas lower motor neuron degeneration was minimal. While α-internexin-positive inclusions without cores were found in both NIFID cases, one NIFID case also had α-internexin- and neurofilament-negative, but p62-positive, cytoplasmic spherical inclusions with eosinophilic p62-negative cores. These two types of inclusions frequently coexisted in the same neuron. In three BIBD cases, inclusions were tau-, α-synuclein-, α-internexin-, and neurofilament-negative, but occasionally p62-positive. These findings suggest that: (1) the clinical features and distribution of neuronal loss are similar in BIBD and NIFID, and (2) an unknown protein besides α-internexin and neurofilament may play a pivotal pathogenetic role in at least some NIFID cases.",
keywords = "α-Internexin, Caudate nucleus, Frontotemporal dementia, Motor neuron disease, TDP-43",
author = "Osamu Yokota and Kuniaki Tsuchiya and Seishi Terada and Hideki Ishizu and Hirotake Uchikado and Manabu Ikeda and Kiyomitsu Oyanagi and Imaharu Nakano and Shigeo Murayama and Shigetoshi Kuroda and Haruhiko Akiyama",
year = "2008",
month = "5",
doi = "10.1007/s00401-007-0329-z",
language = "English",
volume = "115",
pages = "561--575",
journal = "Acta Neuropathologica",
issn = "0001-6322",
publisher = "Springer Verlag",
number = "5",

}

TY - JOUR

T1 - Basophilic inclusion body disease and neuronal intermediate filament inclusion disease

T2 - A comparative clinicopathological study

AU - Yokota, Osamu

AU - Tsuchiya, Kuniaki

AU - Terada, Seishi

AU - Ishizu, Hideki

AU - Uchikado, Hirotake

AU - Ikeda, Manabu

AU - Oyanagi, Kiyomitsu

AU - Nakano, Imaharu

AU - Murayama, Shigeo

AU - Kuroda, Shigetoshi

AU - Akiyama, Haruhiko

PY - 2008/5

Y1 - 2008/5

N2 - While both neuronal intermediate filament inclusion disease (NIFID) and basophilic inclusion body disease (BIBD) show frontotemporal lobar degeneration and/or motor neuron disease, it remains unclear whether, and how, these diseases differ from each other. Here, we compared the clinicopathological characteristics of four BIBD and two NIFID cases. Atypical initial symptoms included weakness, dysarthria, and memory impairment in BIBD, and dysarthria in NIFID. Dementia developed more than 1 year after the onset in some BIBD and NIFID cases. Upper and lower motor neuron signs, parkinsonism, and parietal symptoms were noted in both diseases, and involuntary movements in BIBD. Pathologically, severe caudate atrophy was consistently found in both diseases. Cerebral atrophy was distributed in the convexity of the fronto-parietal region in NIFID cases. In both BIBD and NIFID, the frontotemporal cortex including the precentral gyrus, caudate nucleus, putamen, globus pallidus, thalamus, amygdala, hippocampus including the dentate gyrus, substantia nigra, and pyramidal tract were severely affected, whereas lower motor neuron degeneration was minimal. While α-internexin-positive inclusions without cores were found in both NIFID cases, one NIFID case also had α-internexin- and neurofilament-negative, but p62-positive, cytoplasmic spherical inclusions with eosinophilic p62-negative cores. These two types of inclusions frequently coexisted in the same neuron. In three BIBD cases, inclusions were tau-, α-synuclein-, α-internexin-, and neurofilament-negative, but occasionally p62-positive. These findings suggest that: (1) the clinical features and distribution of neuronal loss are similar in BIBD and NIFID, and (2) an unknown protein besides α-internexin and neurofilament may play a pivotal pathogenetic role in at least some NIFID cases.

AB - While both neuronal intermediate filament inclusion disease (NIFID) and basophilic inclusion body disease (BIBD) show frontotemporal lobar degeneration and/or motor neuron disease, it remains unclear whether, and how, these diseases differ from each other. Here, we compared the clinicopathological characteristics of four BIBD and two NIFID cases. Atypical initial symptoms included weakness, dysarthria, and memory impairment in BIBD, and dysarthria in NIFID. Dementia developed more than 1 year after the onset in some BIBD and NIFID cases. Upper and lower motor neuron signs, parkinsonism, and parietal symptoms were noted in both diseases, and involuntary movements in BIBD. Pathologically, severe caudate atrophy was consistently found in both diseases. Cerebral atrophy was distributed in the convexity of the fronto-parietal region in NIFID cases. In both BIBD and NIFID, the frontotemporal cortex including the precentral gyrus, caudate nucleus, putamen, globus pallidus, thalamus, amygdala, hippocampus including the dentate gyrus, substantia nigra, and pyramidal tract were severely affected, whereas lower motor neuron degeneration was minimal. While α-internexin-positive inclusions without cores were found in both NIFID cases, one NIFID case also had α-internexin- and neurofilament-negative, but p62-positive, cytoplasmic spherical inclusions with eosinophilic p62-negative cores. These two types of inclusions frequently coexisted in the same neuron. In three BIBD cases, inclusions were tau-, α-synuclein-, α-internexin-, and neurofilament-negative, but occasionally p62-positive. These findings suggest that: (1) the clinical features and distribution of neuronal loss are similar in BIBD and NIFID, and (2) an unknown protein besides α-internexin and neurofilament may play a pivotal pathogenetic role in at least some NIFID cases.

KW - α-Internexin

KW - Caudate nucleus

KW - Frontotemporal dementia

KW - Motor neuron disease

KW - TDP-43

UR - http://www.scopus.com/inward/record.url?scp=41949120633&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=41949120633&partnerID=8YFLogxK

U2 - 10.1007/s00401-007-0329-z

DO - 10.1007/s00401-007-0329-z

M3 - Article

C2 - 18080129

AN - SCOPUS:41949120633

VL - 115

SP - 561

EP - 575

JO - Acta Neuropathologica

JF - Acta Neuropathologica

SN - 0001-6322

IS - 5

ER -