Skip to main content
Top
Published in: Acta Neuropathologica 5/2004

01-11-2004 | Regular Paper

Frontotemporal lobar degeneration with motor neuron disease-type inclusions predominates in 76 cases of frontotemporal degeneration

Authors: Anne M. Lipton, Charles L. White III, Eileen H. Bigio

Published in: Acta Neuropathologica | Issue 5/2004

Login to get access

Abstract

This report presents the largest series of consecutive, neuropathologically confirmed cases of frontotemporal degeneration (FTD). Prior studies have found dementia lacking distinctive histology (DLDH) to be the most common pathology underlying the clinical diagnosis of FTD. In this series of 76 cases, 29 (38%) were found to have frontotemporal lobar degeneration with motor neuron disease-type inclusions (FTLD-MND-type) or FTLD-MND (with ALS), the most common neuropathological classification in our series. Only eight (11%) were classified as Pick’s disease. Several cases originally designated as DLDH could be reclassified as FTLD-MND-type based on current recommendations for classification of FTD.
Literature
1.
go back to reference Arnold SE, Han LY, Clark CM, Grossman M, Trojanowski JQ (2000) Quantitative neurohistological features of frontotemporal degeneration. Neurobiol Aging 21:913–919CrossRefPubMed Arnold SE, Han LY, Clark CM, Grossman M, Trojanowski JQ (2000) Quantitative neurohistological features of frontotemporal degeneration. Neurobiol Aging 21:913–919CrossRefPubMed
2.
go back to reference Bigio EH, Brown DF, White CL III (1999) Progressive supranuclear palsy presenting with dementia: cortical pathology. J Neuropathol Exp Neurol 58:359–364PubMed Bigio EH, Brown DF, White CL III (1999) Progressive supranuclear palsy presenting with dementia: cortical pathology. J Neuropathol Exp Neurol 58:359–364PubMed
3.
go back to reference Bigio EH, Lipton AM, Yen SH, Hutton ML, Baker M, Nacharaju P, White CL III, Davies P, Lin W, Dickson DW (2001) Frontal lobe dementia with novel tauopathy: sporadic multiple system tauopathy with dementia. J Neuropathol Exp Neurol 60:328–341PubMed Bigio EH, Lipton AM, Yen SH, Hutton ML, Baker M, Nacharaju P, White CL III, Davies P, Lin W, Dickson DW (2001) Frontal lobe dementia with novel tauopathy: sporadic multiple system tauopathy with dementia. J Neuropathol Exp Neurol 60:328–341PubMed
4.
go back to reference Bigio EH, Lipton AM, White CL III, Dickson DW, Hirano A (2003) Frontotemporal and motor neuron degeneration with neurofilament inclusion bodies: additional evidence for overlap between FTD and ALS. Neuropathol Appl Neurobiol 29:239–253CrossRefPubMed Bigio EH, Lipton AM, White CL III, Dickson DW, Hirano A (2003) Frontotemporal and motor neuron degeneration with neurofilament inclusion bodies: additional evidence for overlap between FTD and ALS. Neuropathol Appl Neurobiol 29:239–253CrossRefPubMed
5.
go back to reference Boeve BF, Maraganore DM, Parisi JE, Ahlskog JE, Graff-Radford N, Caselli RJ, Dickson DW, Kokmen E, Petersen R (1999) Pathologic heterogeneity in clinically diagnosed corticobasal degeneration. Neurology 53:795–800PubMed Boeve BF, Maraganore DM, Parisi JE, Ahlskog JE, Graff-Radford N, Caselli RJ, Dickson DW, Kokmen E, Petersen R (1999) Pathologic heterogeneity in clinically diagnosed corticobasal degeneration. Neurology 53:795–800PubMed
6.
go back to reference Brun A (1987) Frontal lobe degeneration of non-Alzheimer type. I. Neuropathology. Arch Gerontol Geriatr 6:193–208CrossRefPubMed Brun A (1987) Frontal lobe degeneration of non-Alzheimer type. I. Neuropathology. Arch Gerontol Geriatr 6:193–208CrossRefPubMed
7.
go back to reference Brun A, Englund E, Gustafson L, Passant U, Mann DMA, Neary D, Snowden JS (1994) Clinical and neuropathological criteria for frontotemporal dementia: the Lund and Manchester groups. J Neurol Neurosurg Psychiatry 57:416–418PubMed Brun A, Englund E, Gustafson L, Passant U, Mann DMA, Neary D, Snowden JS (1994) Clinical and neuropathological criteria for frontotemporal dementia: the Lund and Manchester groups. J Neurol Neurosurg Psychiatry 57:416–418PubMed
8.
go back to reference Cairns NJ, Perry R, Jaros E, Lowe J, Skellerud K, Duyckaerts C, Cruz-Sanchez F, Lantos P (2002) A new dementia: neurofilament inclusion body dementia. J Neuropathol Exp Neurol 61:451 Cairns NJ, Perry R, Jaros E, Lowe J, Skellerud K, Duyckaerts C, Cruz-Sanchez F, Lantos P (2002) A new dementia: neurofilament inclusion body dementia. J Neuropathol Exp Neurol 61:451
9.
go back to reference Cooper PN, Jackson M, Lennox G, Lowe J, Mann DMA (1995) τ, ubiquitin, and αB-crystallin immunohistochemistry define the principal causes of degenerative frontotemporal dementia. Arch Neurol 52:1011–1015PubMed Cooper PN, Jackson M, Lennox G, Lowe J, Mann DMA (1995) τ, ubiquitin, and αB-crystallin immunohistochemistry define the principal causes of degenerative frontotemporal dementia. Arch Neurol 52:1011–1015PubMed
10.
go back to reference Delisle M-B, Murrell JR, Richardson R, Trofatter JA, Rascol O, Soulages X, Mohr M, Calvas P, Ghetti B (1999) A mutation at codon 279 (N279K) in exon 10 of the tau gene causes a tauopathy with dementia and supranuclear palsy. Acta Neuropathol 98:62–77CrossRefPubMed Delisle M-B, Murrell JR, Richardson R, Trofatter JA, Rascol O, Soulages X, Mohr M, Calvas P, Ghetti B (1999) A mutation at codon 279 (N279K) in exon 10 of the tau gene causes a tauopathy with dementia and supranuclear palsy. Acta Neuropathol 98:62–77CrossRefPubMed
11.
go back to reference Dickson DW (1998) Pick’s disease: a modern approach. Brain Pathol 8:339–354PubMed Dickson DW (1998) Pick’s disease: a modern approach. Brain Pathol 8:339–354PubMed
12.
go back to reference Dickson DW, Yen S-H, Suzuki KI, Davies P, Garcia JH, Hirano A (1986) Ballooned neurons in select neurodegenerative diseases contain phosphorylated neurofilament epitopes. Acta Neuropathol 71:216–223PubMed Dickson DW, Yen S-H, Suzuki KI, Davies P, Garcia JH, Hirano A (1986) Ballooned neurons in select neurodegenerative diseases contain phosphorylated neurofilament epitopes. Acta Neuropathol 71:216–223PubMed
13.
go back to reference Feany MB, Dickson DW (1996) Neurodegenerative disorders with extensive tau pathology: a comparative study and review. Ann Neurol 40:139–148PubMed Feany MB, Dickson DW (1996) Neurodegenerative disorders with extensive tau pathology: a comparative study and review. Ann Neurol 40:139–148PubMed
14.
go back to reference Feany MB, Mattiace LA, Dickson DW (1996) Neuropathologic overlap of progressive supranuclear palsy, Pick’s disease and corticobasal degeneration. J Neuropathol Exp Neurol 55:53–67PubMed Feany MB, Mattiace LA, Dickson DW (1996) Neuropathologic overlap of progressive supranuclear palsy, Pick’s disease and corticobasal degeneration. J Neuropathol Exp Neurol 55:53–67PubMed
15.
go back to reference Hasegawa M, Smith MJ, Iijima M, Tabira T, Goedert M (1999) FTDP-17 mutations N279K and S305N in tau produce increase splicing of exon 10. FEBS Lett 443:93–96CrossRefPubMed Hasegawa M, Smith MJ, Iijima M, Tabira T, Goedert M (1999) FTDP-17 mutations N279K and S305N in tau produce increase splicing of exon 10. FEBS Lett 443:93–96CrossRefPubMed
16.
go back to reference Jackson M, Lowe J (1996) The new neuropathology of degenerative frontotemporal dementias. Acta Neuropathol 91:127–134CrossRefPubMed Jackson M, Lowe J (1996) The new neuropathology of degenerative frontotemporal dementias. Acta Neuropathol 91:127–134CrossRefPubMed
17.
go back to reference Kertesz A, Martinez-Lage P, Davidson W, Munoz DG (2000) The corticobasal degeneration syndrome overlaps progressive aphasia and frontotemporal dementia. Neurology 55:1368–1375PubMed Kertesz A, Martinez-Lage P, Davidson W, Munoz DG (2000) The corticobasal degeneration syndrome overlaps progressive aphasia and frontotemporal dementia. Neurology 55:1368–1375PubMed
18.
go back to reference Knopman DS (1993) Overview of dementia lacking distinctive histology: pathological designation of a progressive dementia. Dementia 4:132–136PubMed Knopman DS (1993) Overview of dementia lacking distinctive histology: pathological designation of a progressive dementia. Dementia 4:132–136PubMed
19.
go back to reference Knopman D, Mastri A, Frey W, Sung J, Rustan T (1990) Dementia lacking distinctive histologic features: a common non-Alzheimer degenerative dementia. Neurology 40:251–256PubMed Knopman D, Mastri A, Frey W, Sung J, Rustan T (1990) Dementia lacking distinctive histologic features: a common non-Alzheimer degenerative dementia. Neurology 40:251–256PubMed
20.
go back to reference Lomen-Hoerth C, Anderson T, Miller B (2002) The overlap of amyotrophic lateral sclerosis and frontotemporal dementia. Neurology 59:1077–1079PubMed Lomen-Hoerth C, Anderson T, Miller B (2002) The overlap of amyotrophic lateral sclerosis and frontotemporal dementia. Neurology 59:1077–1079PubMed
21.
go back to reference Love S, Saitoh T, Quijada S, Cole GM, Terry RD (1988) Alz-50, ubiquitin and tau immunoreactivity of neurofibrillary tangles, Pick bodies, and Lewy bodies. J Neuropathol Exp Neurol 47:393–405PubMed Love S, Saitoh T, Quijada S, Cole GM, Terry RD (1988) Alz-50, ubiquitin and tau immunoreactivity of neurofibrillary tangles, Pick bodies, and Lewy bodies. J Neuropathol Exp Neurol 47:393–405PubMed
22.
go back to reference Mann DM, South P, Snowden JS, Neary D (1993) Dementia of frontal lobe type: neuropathology and immunohistochemistry. J Neurol Neurosurg Psychiatry 56:605–614PubMed Mann DM, South P, Snowden JS, Neary D (1993) Dementia of frontal lobe type: neuropathology and immunohistochemistry. J Neurol Neurosurg Psychiatry 56:605–614PubMed
23.
go back to reference McKhann GM, Albert MS, Grossman M, Miller B, Dickson D, Trojanowski JQ (2001) Clinical and pathological diagnosis of frontotemporal dementia: Report of the Work Group on Frontotemporal Dementia and Pick’s disease. Arch Neurol 58:18–3-1809 McKhann GM, Albert MS, Grossman M, Miller B, Dickson D, Trojanowski JQ (2001) Clinical and pathological diagnosis of frontotemporal dementia: Report of the Work Group on Frontotemporal Dementia and Pick’s disease. Arch Neurol 58:18–3-1809
24.
go back to reference Murayam S, Mori H, Ihara Y, Tomonaga M (1990) Immunocytochemical and ultrastructural studies of Pick’s disease. Ann Neurol 27:394–405PubMed Murayam S, Mori H, Ihara Y, Tomonaga M (1990) Immunocytochemical and ultrastructural studies of Pick’s disease. Ann Neurol 27:394–405PubMed
25.
go back to reference Murrell JR, Koller D, Foroud T, Goedert M, Spillantini MG, Edenberg HJ, Farlow MR, Ghetti B (1997) Familial multiple-system tauopathy with presenile dementia is localized to chromosome 17. Am J Hum Gen 61:1131–1138CrossRef Murrell JR, Koller D, Foroud T, Goedert M, Spillantini MG, Edenberg HJ, Farlow MR, Ghetti B (1997) Familial multiple-system tauopathy with presenile dementia is localized to chromosome 17. Am J Hum Gen 61:1131–1138CrossRef
26.
go back to reference Spillantini MG, Goedert M, Crowther RA, Murrell JR, Farlow MR, Ghetti B (1997) Familial multiple system tauopathy with presenile dementia: a disease with abundant neuronal and glial tau filaments. Proc Nat Acad Sci USA 94:4113–4118CrossRefPubMed Spillantini MG, Goedert M, Crowther RA, Murrell JR, Farlow MR, Ghetti B (1997) Familial multiple system tauopathy with presenile dementia: a disease with abundant neuronal and glial tau filaments. Proc Nat Acad Sci USA 94:4113–4118CrossRefPubMed
27.
go back to reference Spillantini MG, Murrell JR, Goedert M, Farlow MR, Klug A, Ghetti B (1998) Mutation in the tau gene in familial multiple system tauopathy with presenile dementia. Proc Nat Acad Sci USA 95:7737–7741CrossRefPubMed Spillantini MG, Murrell JR, Goedert M, Farlow MR, Klug A, Ghetti B (1998) Mutation in the tau gene in familial multiple system tauopathy with presenile dementia. Proc Nat Acad Sci USA 95:7737–7741CrossRefPubMed
28.
go back to reference Trojanowski JQ, Dickson D (2001) Update on the neuropathological diagnosis of frontotemporal dementias. J Neuropathol Exp Neurol 60:1123–1126PubMed Trojanowski JQ, Dickson D (2001) Update on the neuropathological diagnosis of frontotemporal dementias. J Neuropathol Exp Neurol 60:1123–1126PubMed
Metadata
Title
Frontotemporal lobar degeneration with motor neuron disease-type inclusions predominates in 76 cases of frontotemporal degeneration
Authors
Anne M. Lipton
Charles L. White III
Eileen H. Bigio
Publication date
01-11-2004
Publisher
Springer-Verlag
Published in
Acta Neuropathologica / Issue 5/2004
Print ISSN: 0001-6322
Electronic ISSN: 1432-0533
DOI
https://doi.org/10.1007/s00401-004-0900-9

Other articles of this Issue 5/2004

Acta Neuropathologica 5/2004 Go to the issue