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October 1995, Vol 52, No. 10 >
Article | October 1995

τ Ubiquitin, and αB-Crystallin Immunohistochemistry Define the Principal Causes of Degenerative Frontotemporal Dementia

Paul N. Cooper, MA; Matthew Jackson, MB,BS; Graham Lennox, MA; James Lowe, DM; David M. A. Mann, PhD
Arch Neurol. 1995;52(10):1011-1015. doi:10.1001/archneur.1995.00540340103019
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Objective:  We investigated the use of immunostaining with antibodies to τ, ubiquitin, and αB-crystallin in defining a protocol for the staged neuropathologic examination of brains from patients with a progressive frontotemporal dementia.

Design:  Brains obtained from 50 patients dying with the clinical diagnosis of frontotemporal dementia were examined histopathologically to define pathologic distinctions.

Setting:  Two university hospital neuropathology departments.

Results:  Anti-τ immunostaining defined corticobasal degeneration, Alzheimer's disease, and Pick's disease; antiubiquitin defined motor neuron disease with dementia. The remaining brains have frontal lobe degeneration: the use of αB-crystallin immunostaining, on these, to detect ballooned neurons may help to define two groups of patients, one of which we believe may represent a variant of Pick's disease.

Conclusion:  These findings indicate that immunostaining with these antibodies is essential for the evaluation of frontal dementia.

REFERENCES

1 +
Gustafson L.  Frontal lobe degeneration of non-Alzheimer type, II: clinical picture and differential diagnosis . Arch Gerontol Geriatr . 1987;;6:209-223.
2 +
Neary D, Snowden JS, Northen B, Goulding P.  Dementia of frontal lobe type . J Neurol Neurosurg Psychiatry . 1988;;51:353-361.
3 +
Neary D.  Dementia of frontal lobe type . J Am Geriatr Soc . 1990;;38:71-78.
4 +
Neary D, Snowden JS, Mann DMA, Northen B, Goulding PJ, Macdermott N.  Frontal lobe dementia and motor neuron disease . J Neurol Neurosurg Psychiatry . 1990;;53:23-32.
5 +
Caselli RJ, Windebank AJ, Petersen RC, et al.  Rapidly progressive aphasic dementia and motor neuron disease . Ann Neurol . 1993;;33:200-207.
6 +
Hodges JR, Patterson K, Oxbury S, Funnell E.  Semantic dementia: progressive fluent aphasia with temporal lobe atrophy . Brain . 1992;;115:1783-1806.
7 +
Lippa CF, Cohen R, Smith TW, Drachman DA.  Primary progressive aphasia with focal neuronal achromasia . Neurology . 1991;;41:882-886.
8 +
Sinforiani E, Mauri M, Sances G, Martelli A.  Slowly progressive aphasia: report of a case . Acta Neurol (Napoli) . 1992;;14:51-55.
9 +
Caselli RJ, Jack CR Jr.  Asymmetric cortical degeneration syndromes: a proposed clinical classification . Arch Neurol . 1992;;49:770-780.
10 +
Caselli RJ, Jack CR Jr, Petersen RC, Wahner HW, Yanagihara T.  Asymmetric cortical degenerative syndromes: clinical and radiologic correlations . Neurology . 1992;;42:1462-1468.
11 +
Neary D, Snowden JS, Mann DMA.  The clinical pathological correlates of lobar atrophy . Dementia . 1993;;4:154-159.
12 +
Brun A, Englund B, Gustafson L, et al.  Clinical and neuropathological criteria for frontotemporal dementia . J Neurol Neurosurg Psychiatry . 1994;;57:416-418.
13 +
Snowden JS, Neary D, Mann DM, Goulding PJ, Testa HJ.  Progressive language disorder due to lobar atrophy . Ann Neurol . 1992;;31:174-183.
14 +
Mann DMA, South PW, Snowden JS, Neary D.  Dementia of frontal lobe type: neuropathology and immunohistochemistry . J Neurol Neurosurg Psychiatry . 1993;;56:605-614.
15 +
Owen F, Cooper PN, Pickering-Brown S, McAndrew C, Mann DMA, Neary D.  The lobar atrophies are not prior encephalopathies . Neurodegeneration . 1993;; 2:195-199.
16 +
Collinge J, Palmer MS, Sidle KCL, et al.  Familial Pick's disease and dementia in frontal lobe degeneration of non-Alzheimer type are not variants of prior disease . J Neurol Neurosurg Psychiatry . 1994;;57:762.
17 +
Quinlan RA, Nicholl ID.  Chaperone activity of alpha-crystallins modulates intermediate filament assembly . EMBO J . 1994;;13:945-953.
18 +
Lowe J, McDermott H, Pike I, Spendlove I, Landon M, Mayer RJ.  αB-crystallin expression in non-lenticular tissues and selective presence in ubiquitinated inclusion bodies in human disease . J Pathol . 1992;;166:61-68.
19 +
Kato S, Hirano A, Umahara T, Kato M, Herz F, Ohama E.  Comparative immunohistochemical study on the expression of αB-crystallin, ubiquitin and stressresponse protein 27 in ballooned neurons in various disorders . Neuropathol Appl Neurobiol . 1992;;18:335-340.
20 +
Rebeiz JJ, Kolodny EH, Richardson EP.  Corticodentatonigral degeneration with neuronal achromasia . Arch Neurol . 1968;:18:21-33.
21 +
Okamoto K, Murakami N, Kusaka H, et al.  Ubiquitin-positive intraneuronal inclusions in the extramotor cortices of presenile dementia patients with motor neuron disease . J Neurol . 1992;;239:426-430.
22 +
Leigh N, Anderton BH, Dodson A, Gallo J-M, Swash M, Power DM.  Ubiquitin deposits in anterior horn cells in motor neuron disease . Neurosci Lett . 1988;; 93:197-203.
23 +
Neumann MA, Cohn R.  Progressive subcortical gliosis, a rare form of presenile dementia . Brain . 1967;;90:405-418.
24 +
Brun A.  Frontal lobe degeneration of non-Alzheimer type, I: neuropathology . Arch Gerontol Geriatr . 1987;;6:193-208.
25 +
Knopman DS, Mastri AR, Frey WH II, Sung JH, Rustan T.  Dementia lacking distinctive histologic features: a common non-Alzheimer degenerative dementia . Neurology . 1990;;40:251-256.
26 +
Gibb WR, Luther PJ, Marsden CD.  Corticobasal degeneration . Brain . 1989;; 112:1171-1192.
27 +
Riley DE, Lang AE, Lewis A, et al.  Cortico-basal ganglionic degeneration . Neurology . 1990;;40:1203-1212.
28 +
Lennox G, Jackson M, Lowe J. Corticobasal degeneration manifesting as a frontal lobe syndrome without movement disorder. Neuropathol Appl Neurobiol. In press.
29 +
Brown J, Lantos PL, Collinge J, Rossor MN.  Familial Pick's syndrome with corticobasal inclusion bodies: a clinicopathological study . Neuropathol Appl Neurobiol . 1993;;19:448.
30 +
Clark AW, Manz HJ, White CL III, Lehmann J, Miller D, Coyle JT.  Cortical degeneration with swollen chromatolytic neurones: its relationship to Pick's disease . J Neuropathol Exp Neurol . 1986;;45:268-284.
31 +
Lowe J.  New pathological findings in amyotrophic lateral sclerosis . J Neurol Sci . 1994;;124( (suppl) ):38-51.
32 +
Lennox G, Lowe J, Morrell K, Landon M, Mayer RJ.  Anti-ubiquitin immunocytochemistry is more sensitive than conventional techniques in the detection of diffuse Lewy body disease . J Neurol Neurosurg Psychiatry . 1989;:52: 67-71.
33 +
Gunnarsson L-G, Dahlbom K, Strandman E.  Motor neuron disease and dementia reported among 13 members of a single family . Acta Neurol Scand . 1991;;84:429-433.
34 +
Lynch T, Sano M, Marder KS, et al.  Clinical characteristics of a family with chromosome-17 linked disinhibition-dementia-parkinsonism complex . Neurology . 1994;;44:1878-1884.

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1 +
Gustafson L.  Frontal lobe degeneration of non-Alzheimer type, II: clinical picture and differential diagnosis . Arch Gerontol Geriatr . 1987;;6:209-223.
2 +
Neary D, Snowden JS, Northen B, Goulding P.  Dementia of frontal lobe type . J Neurol Neurosurg Psychiatry . 1988;;51:353-361.
3 +
Neary D.  Dementia of frontal lobe type . J Am Geriatr Soc . 1990;;38:71-78.
4 +
Neary D, Snowden JS, Mann DMA, Northen B, Goulding PJ, Macdermott N.  Frontal lobe dementia and motor neuron disease . J Neurol Neurosurg Psychiatry . 1990;;53:23-32.
5 +
Caselli RJ, Windebank AJ, Petersen RC, et al.  Rapidly progressive aphasic dementia and motor neuron disease . Ann Neurol . 1993;;33:200-207.
6 +
Hodges JR, Patterson K, Oxbury S, Funnell E.  Semantic dementia: progressive fluent aphasia with temporal lobe atrophy . Brain . 1992;;115:1783-1806.
7 +
Lippa CF, Cohen R, Smith TW, Drachman DA.  Primary progressive aphasia with focal neuronal achromasia . Neurology . 1991;;41:882-886.
8 +
Sinforiani E, Mauri M, Sances G, Martelli A.  Slowly progressive aphasia: report of a case . Acta Neurol (Napoli) . 1992;;14:51-55.
9 +
Caselli RJ, Jack CR Jr.  Asymmetric cortical degeneration syndromes: a proposed clinical classification . Arch Neurol . 1992;;49:770-780.
10 +
Caselli RJ, Jack CR Jr, Petersen RC, Wahner HW, Yanagihara T.  Asymmetric cortical degenerative syndromes: clinical and radiologic correlations . Neurology . 1992;;42:1462-1468.
11 +
Neary D, Snowden JS, Mann DMA.  The clinical pathological correlates of lobar atrophy . Dementia . 1993;;4:154-159.
12 +
Brun A, Englund B, Gustafson L, et al.  Clinical and neuropathological criteria for frontotemporal dementia . J Neurol Neurosurg Psychiatry . 1994;;57:416-418.
13 +
Snowden JS, Neary D, Mann DM, Goulding PJ, Testa HJ.  Progressive language disorder due to lobar atrophy . Ann Neurol . 1992;;31:174-183.
14 +
Mann DMA, South PW, Snowden JS, Neary D.  Dementia of frontal lobe type: neuropathology and immunohistochemistry . J Neurol Neurosurg Psychiatry . 1993;;56:605-614.
15 +
Owen F, Cooper PN, Pickering-Brown S, McAndrew C, Mann DMA, Neary D.  The lobar atrophies are not prior encephalopathies . Neurodegeneration . 1993;; 2:195-199.
16 +
Collinge J, Palmer MS, Sidle KCL, et al.  Familial Pick's disease and dementia in frontal lobe degeneration of non-Alzheimer type are not variants of prior disease . J Neurol Neurosurg Psychiatry . 1994;;57:762.
17 +
Quinlan RA, Nicholl ID.  Chaperone activity of alpha-crystallins modulates intermediate filament assembly . EMBO J . 1994;;13:945-953.
18 +
Lowe J, McDermott H, Pike I, Spendlove I, Landon M, Mayer RJ.  αB-crystallin expression in non-lenticular tissues and selective presence in ubiquitinated inclusion bodies in human disease . J Pathol . 1992;;166:61-68.
19 +
Kato S, Hirano A, Umahara T, Kato M, Herz F, Ohama E.  Comparative immunohistochemical study on the expression of αB-crystallin, ubiquitin and stressresponse protein 27 in ballooned neurons in various disorders . Neuropathol Appl Neurobiol . 1992;;18:335-340.
20 +
Rebeiz JJ, Kolodny EH, Richardson EP.  Corticodentatonigral degeneration with neuronal achromasia . Arch Neurol . 1968;:18:21-33.
21 +
Okamoto K, Murakami N, Kusaka H, et al.  Ubiquitin-positive intraneuronal inclusions in the extramotor cortices of presenile dementia patients with motor neuron disease . J Neurol . 1992;;239:426-430.
22 +
Leigh N, Anderton BH, Dodson A, Gallo J-M, Swash M, Power DM.  Ubiquitin deposits in anterior horn cells in motor neuron disease . Neurosci Lett . 1988;; 93:197-203.
23 +
Neumann MA, Cohn R.  Progressive subcortical gliosis, a rare form of presenile dementia . Brain . 1967;;90:405-418.
24 +
Brun A.  Frontal lobe degeneration of non-Alzheimer type, I: neuropathology . Arch Gerontol Geriatr . 1987;;6:193-208.
25 +
Knopman DS, Mastri AR, Frey WH II, Sung JH, Rustan T.  Dementia lacking distinctive histologic features: a common non-Alzheimer degenerative dementia . Neurology . 1990;;40:251-256.
26 +
Gibb WR, Luther PJ, Marsden CD.  Corticobasal degeneration . Brain . 1989;; 112:1171-1192.
27 +
Riley DE, Lang AE, Lewis A, et al.  Cortico-basal ganglionic degeneration . Neurology . 1990;;40:1203-1212.
28 +
Lennox G, Jackson M, Lowe J. Corticobasal degeneration manifesting as a frontal lobe syndrome without movement disorder. Neuropathol Appl Neurobiol. In press.
29 +
Brown J, Lantos PL, Collinge J, Rossor MN.  Familial Pick's syndrome with corticobasal inclusion bodies: a clinicopathological study . Neuropathol Appl Neurobiol . 1993;;19:448.
30 +
Clark AW, Manz HJ, White CL III, Lehmann J, Miller D, Coyle JT.  Cortical degeneration with swollen chromatolytic neurones: its relationship to Pick's disease . J Neuropathol Exp Neurol . 1986;;45:268-284.
31 +
Lowe J.  New pathological findings in amyotrophic lateral sclerosis . J Neurol Sci . 1994;;124( (suppl) ):38-51.
32 +
Lennox G, Lowe J, Morrell K, Landon M, Mayer RJ.  Anti-ubiquitin immunocytochemistry is more sensitive than conventional techniques in the detection of diffuse Lewy body disease . J Neurol Neurosurg Psychiatry . 1989;:52: 67-71.
33 +
Gunnarsson L-G, Dahlbom K, Strandman E.  Motor neuron disease and dementia reported among 13 members of a single family . Acta Neurol Scand . 1991;;84:429-433.
34 +
Lynch T, Sano M, Marder KS, et al.  Clinical characteristics of a family with chromosome-17 linked disinhibition-dementia-parkinsonism complex . Neurology . 1994;;44:1878-1884.

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