We retrospectively analyzed the clinical features of two cases of neurodegenerative disease, whose initial symptoms were motor speech disorder and dementia, brought to autopsy. We compared the distributions of pathological findings with the clinical features. The main symptom of speech disorder was dysarthria, involving low pitch, slow rate, hypernasality and hoarseness. Other than these findings, effortful speech, sound prolongation and initial difficulty were observed. Moreover, repetition of multisyllables was severely impaired compared to monosyllables. Repetition and comprehension of words and sentences were not impaired. Neither atrophy nor fasciculation of the tongue was observed. Both cases showed rapid progression to mutism within a few years. Neuropathologically, frontal lobe degeneration including the precentral gyrus was observed. The bilateral pyramidal tracts also showed severe degeneration. However, the nucleus of the hypoglossal nerve showed only mild degeneration. These findings suggest upper motor neuron dominant motor neuron disease with dementia. We believe the results indicate a subgroup of motor neuron disease with dementia whose initial symptoms involve pseudobulbar palsy and dementia, and which shows rapid progression to mutism.

Ogar JM, Dronkers NF, Brambati SM, Miller BL, Gorno-Tempini M: Progressive nonfluent aphasia and its characteristic motor speech deficits. Alzheimer Dis Assoc Disord 2007;21:S23-S30.
Gorno-Tempini ML, Hillis AE, Weintraub S, Kertesz A, Mendez M, Cappa SF, Ogar JM, Rohrer JD, Black S, Boeve BF, Manes F, Dronkers NF, Vandenberghe R, Rascovsky K, Patterson K, Miller BL, Knopman DS, Hodges JR, Mesulam MM, Grossman M: Classification of primary progressive aphasia and its variants. Neurology 2011;76:1006-1014.
Rohrer JD, Warren JD, Modat M, Ridgway GR, Douiri A, Rossor MN, Ourselin S, Fox NC: Syndromes of nonfluent primary progressive aphasia: a clinical and neurolinguistic analysis. Neurology 2010;75:603-610.
Gallagher GP: Pathologic laughter and crying in ALS: a search for their origin. Acta Neurol Scand 1989;80:114-117.
Olney NT, Goodkind MS, Lomen-Hoerth C, Whalen PK, Williamson CA, Holley DE, Verstaen A, Brown LM, Miller BL, Kornak J, Levenson RW, Rosen HJ: Behaviour, physiology and experience of pathologic laughter and crying in amyotrophic lateral sclerosis. Brain 2011;134:3458-3469.
Bak TH: Overlap syndromes; in Hodges JR (ed): Frontotemporal Dementia Syndromes. New York, Cambridge University Press, 2007, pp 80-101.
Mackenzie IRA, Neumann M, Barborie A, Sampathu DM, Plessis DD, Jaros E, Perry RH, Trojanowski JQ, Mann DM, Lee VM: A harmonized classification system for FTLD-TDP pathology. Acta Neuropathol 2011;122:111-113.
Seilhean D, Le Ber I, Sarazin M, Lacomblez L, Millecamps S, Salachas F, Pradat PF, Le Forestier N, LeGuern E, Dubois B, Meininger V, Brice A, Hauw JJ, Duyckaerts C: Fronto-temporal lobar degeneration: neuropathology in 60 cases. J Neural Transm 2011;118:753-764.
Hsiung GY, DeJesus-Hernandez M, Feldman HH, Sengdy P, Bouchard-Kerr P, Dwosh E, Butler R, Leung B, Fok A, Rutherford NJ, Baker M, Rademakers R, Mackenzie IR: Clinical and pathological features of familial frontotemporal dementia caused by C9ORF72 mutation on chromosome 9p. Brain 2012;135:709-722.
Cooper-Knock J, Hewitt C, Highley JR, Brockington A, Milano A, Man S, Martindale J, Hartley J, Walsh T, Gelsthorpe C, Baxter L, Forster G, Fox M, Bury J, Mok K, McDermott CJ, Traynor BJ, Kirby J, Wharton SB, Ince PG, Hardy J, Shaw PJ: Clinico-pathological features in amyotrophic lateral sclerosis with expansions in C9ORF72. Brain 2012;135:751-764.
de Koning I, van Doorn PA, van Dongen HR: Slowly progressive isolated dysarthria: longitudinal course, speech features, and neuropsychological deficits. J Neurol 1997;244:664-666.
Becker A, Hardmeiser M, Steck AJ, Czaplinski A: Primary lateral sclerosis presenting with isolated progressive pseudobulbar syndrome. Eur J Neurol 2007;14:e3.
Santens P, Van Borsel J, Foncke E, Meire V, Merkx H, De Bleecker J, De Reuck J: Progressive dysarthria. Case reports and review of the literature. Dement Geriatr Cogn Disord 1999;10:231-236.
Soliveri P, Piacentini S, Carella F, Testa D, Ciano C, Girotti F: Progressive dysarthria: definition and clinical follow-up. Neurol Sci 2003;24:211-212.
Broussolle E, Bakchine S, Tommasi M, Laurent B, Bazin B, Cinotti L, Cohen L, Chazot G: Slowly progressive anarthria with late anterior opercular syndrome: a variant form of frontal cortical atrophy syndrome. J Neurol Sci 1996;144:44-58.
Caselli RJ, Windebank AJ, Petersen RC, Komori T, Parisi JE, Okazaki H, Kokmen E, Iverson R, Dinapoli RP, Graff-Radford NR, Stein SD: Rapidly progressive aphasic dementia and motor neuron disease. Ann Neurol 1993;33:200-207.
Doran M, Xuereb J, Hodges JR: Rapidly progressive aphasia with bulbar motor neurone disease: a clinical and neuropsychological study. Behav Neurol 1995;8:169-180.
Bak TH, O'Donovan DG, Xuereb JH, Boniface S, Hodges JR: Selective impairment of verb processing associated with pathological changes in Brodmann areas 44 and 45 in the motor neurone disease-dementia-aphasia syndrome. Brain 2001;124:103-120.
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.