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Vol. 27, No. 6, 2009
Issue release date: July 2009
Section title: Original Research Article
Free Access
Dement Geriatr Cogn Disord 2009;27:564–571
(DOI:10.1159/000228258)

Cognitive and Noncognitive Neurological Features of Young-Onset Dementia

Kelley B.J. · Boeve B.F. · Josephs K.A.
Department of Neurology, Mayo Clinic, Rochester, Minn., USA
email Corresponding Author

Abstract

Background: The rarity of young-onset dementia (YOD), the broad differential diagnosis and unusual clinical presentations present unique challenges to correctly recognize the condition and establish an accurate diagnosis. Limited data exist regarding clinical features associated with dementia prior to the age of 45. Methods: We retrospectively assessed cognitive and noncognitive neurological characteristics of 235 patients who presented for evaluation of YOD to investigate the clinical characteristics of YOD compared to later-onset dementias and to identify clinical features associated with specific etiologies that may aid in the evaluation of YOD. Results: Multiple cognitive domains were affected in most patients, and no significant differences in affected domains existed between groups. Early psychiatric and behavioral features occurred at very high frequencies. Nearly 80% of this YOD cohort had additional noncognitive symptoms or signs as a feature of their disease. Chorea was strongly associated with Huntington disease. Parkinsonism was not seen in patients having an autoimmune/inflammatory etiology. Conclusions: The rarity of YOD and the high frequency of early psychiatric features led to frequent misdiagnosis early in the clinical course. The high frequency of noncognitive symptoms and signs may aid clinicians in distinguishing patients requiring a more extensive evaluation for YOD.

© 2009 S. Karger AG, Basel


  

Key Words

  • Young-onset dementia
  • Cognitive decline, age of onset
  • Presenile dementia, clinical features

References

  1. Kelley BJ, Boeve BF, Josephs KA: Young-onset dementia: demographic and etiologic characteristics of 235 patients. Arch Neurol 2008;65:1502–1508.
  2. Licht EA, McMurtray AM, Saul RE, Mendez MF: Cognitive differences between early- and late-onset Alzheimer’s disease. Am J Alzheimers Dis Other Demen 2007;22: 218–222.
  3. Appleby BS, Appleby KK, Rabins PV: Does the presentation of Creutzfeldt-Jakob disease vary by age or presumed etiology? A meta-analysis of the past 10 years. J Neuropsychiatry Clin Neurosci 2007;19:428–435.
  4. Corato M, Cereda C, Cova E, Ferrarese C, Ceroni M: Young-onset CJD: age and disease phenotype in variant and sporadic forms. Funct Neurol 2006;21:211–215.
  5. Members of the Department of Neurology MC: Clinical Examinations in Neurology, ed 6. St. Louis, Mosby Year Book, 1998.
  6. Kelley BJ, Boeve BF, Josephs KA: Rapidly progressive young-onset dementia. Cogn Behav Neurol 2009;22:22–27.
  7. Woodburn KJ, Johnstone EC: Early-onset dementia in Lothian, Scotland: an analysis of clinical features and patterns of decline. Health Bull (Edinb) 1999;57:384–392.
  8. Harvey RJ: Young Onset Dementia: Epidemiology, Clinical Symptoms, Family Burden, Support and Outcome. London, Dementia Research Group, Imperial College School of Medicine, 1998, pp 1–145.
  9. Lapid MI, Rummans TA: Evaluation and management of geriatric depression in primary care. Mayo Clin Proc 2003;78:1423–1429.
  10. Fujihara S, Brucki SM, Rocha MS, Carvalho AA, Piccolo AC: Prevalence of presenile dementia in a tertiary outpatient clinic. Arq Neuropsiquiatr 2004;62:592–595.
  11. Harvey RJ, Skelton-Robinson M, Rossor MN: The prevalence and causes of dementia in people under the age of 65 years. J Neurol Neurosurg Psychiatry 2003;74:1206–1209.
  12. McMurtray A, Clark DG, Christine D, Mendez MF: Early-onset dementia: frequency and causes compared to late-onset dementia. Dement Geriatr Cogn Disord 2006;21:59–64.
  13. Newens AJ, Forster DP, Kay DW: Referral patterns and diagnosis in presenile Alzheimer’s disease: implications for general practice. Br J Gen Pract 1994;44:405–407.
  14. Panegyres PK, Frencham K: Course and causes of suspected dementia in young adults: a longitudinal study. Am J Alzheimers Dis Other Demen 2007;22:48–56.
  15. Treves T, Korczyn AD, Zilber N, Kahana E, Leibowitz Y, Alter M, Schoenberg BS: Presenile dementia in Israel. Arch Neurol 1986;43:26–29.
  16. Filley CM, Rollins YD, Alan Anderson C, Arciniegas DB, Howard KL, Murrell JR, Boyer PJ, Kleinschmidt-Demasters BK, Ghetti B: The genetics of very early onset Alzheimer disease. Cogn Behav Neurol 2007;20:149–156.
  17. Filley CM: White matter and behavioral neurology. Ann NY Acad Sci 2005;1064:162–183.
  18. Guo Y, Guo Z, Chen L, Zhang J, Wang W, Liu X, Ren H, Gao S: Clinical, pathologic and genetic studies on mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes. Chin Med J 1997;110:851–855.
  19. Hodges JR, Davies RR, Xuereb JH, Casey B, Broe M, Bak TH, Kril JJ, Halliday GM: Clinicopathological correlates in frontotemporal dementia. Ann Neurol 2004;56:399–406.
  20. Mendez MF: The accurate diagnosis of early-onset dementia. Int J Psychiatr Med 2006;36:401–412.
  21. Shinagawa S, Ikeda M, Toyota Y, Matsumoto T, Matsumoto N, Mori T, Ishikawa T, Fukuhara R, Komori K, Hokoishi K, Tanabe H: Frequency and clinical characteristics of early-onset dementia in consecutive patients in a memory clinic. Dement Geriatr Cogn Disord 2007;24:42–47.

  

Author Contacts

Brendan J. Kelley, MD
Department of Neurology, University of Cincinnati
260 Stetson Street, Suite 2300
Cincinnati, OH 45267 (USA)
Tel. +1 513 558 5501, Fax +1 513 558 4305, E-Mail kelleyb3@uc.edu

  

Article Information

Accepted: March 16, 2009
Published online: July 11, 2009
Number of Print Pages : 8
Number of Figures : 0, Number of Tables : 5, Number of References : 21

  

Publication Details

Dementia and Geriatric Cognitive Disorders

Vol. 27, No. 6, Year 2009 (Cover Date: July 2009)

Journal Editor: Chan-Palay V. (New York, N.Y.)
ISSN: 1420-8008 (Print), eISSN: 1421-9824 (Online)

For additional information: http://www.karger.com/DEM


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 or, in the case of photocopying, direct payment of a specified fee to the Copyright Clearance Center.
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 goverment 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.

Abstract

Background: The rarity of young-onset dementia (YOD), the broad differential diagnosis and unusual clinical presentations present unique challenges to correctly recognize the condition and establish an accurate diagnosis. Limited data exist regarding clinical features associated with dementia prior to the age of 45. Methods: We retrospectively assessed cognitive and noncognitive neurological characteristics of 235 patients who presented for evaluation of YOD to investigate the clinical characteristics of YOD compared to later-onset dementias and to identify clinical features associated with specific etiologies that may aid in the evaluation of YOD. Results: Multiple cognitive domains were affected in most patients, and no significant differences in affected domains existed between groups. Early psychiatric and behavioral features occurred at very high frequencies. Nearly 80% of this YOD cohort had additional noncognitive symptoms or signs as a feature of their disease. Chorea was strongly associated with Huntington disease. Parkinsonism was not seen in patients having an autoimmune/inflammatory etiology. Conclusions: The rarity of YOD and the high frequency of early psychiatric features led to frequent misdiagnosis early in the clinical course. The high frequency of noncognitive symptoms and signs may aid clinicians in distinguishing patients requiring a more extensive evaluation for YOD.

© 2009 S. Karger AG, Basel


  

Author Contacts

Brendan J. Kelley, MD
Department of Neurology, University of Cincinnati
260 Stetson Street, Suite 2300
Cincinnati, OH 45267 (USA)
Tel. +1 513 558 5501, Fax +1 513 558 4305, E-Mail kelleyb3@uc.edu

  

Article Information

Accepted: March 16, 2009
Published online: July 11, 2009
Number of Print Pages : 8
Number of Figures : 0, Number of Tables : 5, Number of References : 21

  

Publication Details

Dementia and Geriatric Cognitive Disorders

Vol. 27, No. 6, Year 2009 (Cover Date: July 2009)

Journal Editor: Chan-Palay V. (New York, N.Y.)
ISSN: 1420-8008 (Print), eISSN: 1421-9824 (Online)

For additional information: http://www.karger.com/DEM


Article / Publication Details

First-Page Preview
Abstract of Original Research Article

Accepted: 3/16/2009
Published online: 7/11/2009
Issue release date: July 2009

Number of Print Pages: 8
Number of Figures: 0
Number of Tables: 5

ISSN: 1420-8008 (Print)
eISSN: 1421-9824 (Online)

For additional information: http://www.karger.com/DEM


Copyright / Drug Dosage

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 or, in the case of photocopying, direct payment of a specified fee to the Copyright Clearance Center.
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 goverment 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.

References

  1. Kelley BJ, Boeve BF, Josephs KA: Young-onset dementia: demographic and etiologic characteristics of 235 patients. Arch Neurol 2008;65:1502–1508.
  2. Licht EA, McMurtray AM, Saul RE, Mendez MF: Cognitive differences between early- and late-onset Alzheimer’s disease. Am J Alzheimers Dis Other Demen 2007;22: 218–222.
  3. Appleby BS, Appleby KK, Rabins PV: Does the presentation of Creutzfeldt-Jakob disease vary by age or presumed etiology? A meta-analysis of the past 10 years. J Neuropsychiatry Clin Neurosci 2007;19:428–435.
  4. Corato M, Cereda C, Cova E, Ferrarese C, Ceroni M: Young-onset CJD: age and disease phenotype in variant and sporadic forms. Funct Neurol 2006;21:211–215.
  5. Members of the Department of Neurology MC: Clinical Examinations in Neurology, ed 6. St. Louis, Mosby Year Book, 1998.
  6. Kelley BJ, Boeve BF, Josephs KA: Rapidly progressive young-onset dementia. Cogn Behav Neurol 2009;22:22–27.
  7. Woodburn KJ, Johnstone EC: Early-onset dementia in Lothian, Scotland: an analysis of clinical features and patterns of decline. Health Bull (Edinb) 1999;57:384–392.
  8. Harvey RJ: Young Onset Dementia: Epidemiology, Clinical Symptoms, Family Burden, Support and Outcome. London, Dementia Research Group, Imperial College School of Medicine, 1998, pp 1–145.
  9. Lapid MI, Rummans TA: Evaluation and management of geriatric depression in primary care. Mayo Clin Proc 2003;78:1423–1429.
  10. Fujihara S, Brucki SM, Rocha MS, Carvalho AA, Piccolo AC: Prevalence of presenile dementia in a tertiary outpatient clinic. Arq Neuropsiquiatr 2004;62:592–595.
  11. Harvey RJ, Skelton-Robinson M, Rossor MN: The prevalence and causes of dementia in people under the age of 65 years. J Neurol Neurosurg Psychiatry 2003;74:1206–1209.
  12. McMurtray A, Clark DG, Christine D, Mendez MF: Early-onset dementia: frequency and causes compared to late-onset dementia. Dement Geriatr Cogn Disord 2006;21:59–64.
  13. Newens AJ, Forster DP, Kay DW: Referral patterns and diagnosis in presenile Alzheimer’s disease: implications for general practice. Br J Gen Pract 1994;44:405–407.
  14. Panegyres PK, Frencham K: Course and causes of suspected dementia in young adults: a longitudinal study. Am J Alzheimers Dis Other Demen 2007;22:48–56.
  15. Treves T, Korczyn AD, Zilber N, Kahana E, Leibowitz Y, Alter M, Schoenberg BS: Presenile dementia in Israel. Arch Neurol 1986;43:26–29.
  16. Filley CM, Rollins YD, Alan Anderson C, Arciniegas DB, Howard KL, Murrell JR, Boyer PJ, Kleinschmidt-Demasters BK, Ghetti B: The genetics of very early onset Alzheimer disease. Cogn Behav Neurol 2007;20:149–156.
  17. Filley CM: White matter and behavioral neurology. Ann NY Acad Sci 2005;1064:162–183.
  18. Guo Y, Guo Z, Chen L, Zhang J, Wang W, Liu X, Ren H, Gao S: Clinical, pathologic and genetic studies on mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes. Chin Med J 1997;110:851–855.
  19. Hodges JR, Davies RR, Xuereb JH, Casey B, Broe M, Bak TH, Kril JJ, Halliday GM: Clinicopathological correlates in frontotemporal dementia. Ann Neurol 2004;56:399–406.
  20. Mendez MF: The accurate diagnosis of early-onset dementia. Int J Psychiatr Med 2006;36:401–412.
  21. Shinagawa S, Ikeda M, Toyota Y, Matsumoto T, Matsumoto N, Mori T, Ishikawa T, Fukuhara R, Komori K, Hokoishi K, Tanabe H: Frequency and clinical characteristics of early-onset dementia in consecutive patients in a memory clinic. Dement Geriatr Cogn Disord 2007;24:42–47.