Non-Alzheimer Dementias

 

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ABSTRACT

The past decade has seen a considerable resurgence of interest in non-Alzheimer forms of neurodegenerative dementia. Advances in our understanding and classification of these conditions have taken place over a diverse range of disciplines: from genetics and immunohistochemistry to neuropsychology and psychiatry. The aim of this article is to review, from a clinician's perspective, our current understanding of the major degenerative dementias that fall into the differential diagnosis of Alzheimer's disease. The clinical variants of frontotemporal dementia (semantic dementia, progressive nonfluent aphasia, and dementia of a frontal type), amyotrophic lateral sclerosis associated dementia, corticobasal degeneration, and dementia with Lewy bodies are considered.

REFERENCES

• 1 Foster N L, Wilhelmsen K, Sima A A. Frontotemporal dementia and parkinsonism linked to chromosome 17: a consensus conference. Conference Participants.  Ann Neurol . 1997;  41 706-715
  CrossrefPubMedGoogle Scholar
• 2 Hutton M, Lendon C L, Rizzu P. Association of missense and 5′-splice-site mutations in tau with the inherited dementia FTDP-17.  Nature . 1998;  393 702-705
  CrossrefPubMedGoogle Scholar
• 3 Goedert M, Spillantini M G, Davies S W. Filamentous nerve cell inclusions in neurodegenerative diseases.  Curr Opin Neurobiol . 1998;  8 619-632
  CrossrefPubMedGoogle Scholar
• 4 Brown J, Ashworth A, Gydesen S. Familial non-specific dementia maps to chromosome 3.  Hum Mol Genet . 1995;  4 1625-1628
  PubMedGoogle Scholar
• 5 Harvey R. Epidemiology of presenile dementia. In: Hodges JR, ed. Early Onset Dementia. In press. New York: Oxford University Press
  Google Scholar
• 6 Garrard P, Perry R, Hodges J R. Disorders of semantic memory (Editorial).  J Neurol Neurosurg Psychiatry . 1997;  62 431-435
  PubMedGoogle Scholar
• 7 Hodges J R, Patterson K, Oxbury S, Funnell E. Semantic dementia: progressive fluent aphasia with temporal lobe atrophy.  Brain . 1992;  115 1783-1806
  CrossrefPubMedGoogle Scholar
• 8 Nelson H E. The National Adult Reading Test.  Windsor: NFER-Nelson; 1982
  Google Scholar
• 9 Graham N L, Patterson K, Hodges J R. The impact of semantic memory impairment on spelling: evidence from semantic dementia.  Neuropsychologia . 2000;  38 143-163
  CrossrefPubMedGoogle Scholar
• 10 Hodges J R, Spatt J, Patterson K. What and how: evidence for the dissociation of object knowledge and mechanical problem solving skills in the human brain.  Proc Natl Acad Sci U S A . 1999;  96 9444-9448
  CrossrefPubMedGoogle Scholar
• 11 Graham K S, Simons J S, Pratt K H, Patterson K, Hodges J R. Insights from semantic dementia on the relationship between episodic and semantic memory.  Neuropsychologia . 2000;  38 313-324
  CrossrefPubMedGoogle Scholar
• 12 Graham K S, Hodges J R. Differentiating the roles of the hippocampal complex and the neocortex in long-term memory storage: evidence from the study of semantic dementia and Alzheimer's disease.  Neuropsychology . 1997;  11 77-89
  CrossrefPubMedGoogle Scholar
• 13 Evans J J, Heggs A J, Antoun N, Hodges J R. Progressive prosopagnosia associated with selective right temporal lobe atrophy: a new syndrome?.  Brain . 1995;  118 1-13
  CrossrefPubMedGoogle Scholar
• 14 Edwards-Lee T, Miller B L, Benson D F. The temporal variant of fronto-temporal dementia.  Brain . 1997;  120 1027-1040
  CrossrefPubMedGoogle Scholar
• 15 Hodges J R, Patterson K. Nonfluent progressive aphasia and semantic dementia: a comparative neuropsychological study.  J Int Neuropsychol Soc . 1996;  2 511-524
  PubMedGoogle Scholar
• 16 Bishop D VM. Test for the Reception of Grammar.  2nd ed. Cambridge, UK: Medical Research Council; 1989
  Google Scholar
• 17 Weintraub S, Rubin N P, Mesulam M M. Primary progressive aphasia: longitudinal course, neuropsychological profile, and language features.  Arch Neurol . 1990;  47 1329-1335
  PubMedGoogle Scholar
• 18 Tyrrell P J, Kartsounis L D, Frackowiak R S, Findley L J, Rossor M N. Progressive loss of speech output and orofacial dyspraxia associated with frontal lobe hypometabolism.  J Neurol Neurosurg Psychiatry . 1991;  54 351-357
  PubMedGoogle Scholar
• 19 Brun A, Englund B, Gustafson L. Clinical and neuropathological criteria for frontotemporal dementia.  J Neurol Neurosurg Psychiatry . 1994;  57 416-418
  CrossrefPubMedGoogle Scholar
• 20 Gregory C A, Orrell M, Sahakian B, Hodges J R. Can frontotemporal dementia and Alzheimer's disease be differentiated using a brief battery of tests?.  Int J Geriatr Psychiatry . 1997;  12 375-383
  PubMedGoogle Scholar
• 21 Levy M L, Miller B L, Cummings J L, Fairbanks L A, Craig A. Alzheimer disease and frontotemporal dementias: behavioral distinctions.  Arch Neurol . 1996;  53 687-690
  PubMedGoogle Scholar
• 22 Gregory C A, Serra-Mestres J, Hodges J R. Early diagnosis of the frontal variant of frontotemporal dementia: how sensitive are standard neuroimaging and neuropsychologic tests?.  Neuropsychiatry Neuropsychol Behav Neurol . 1999;  12 128-135
  PubMedGoogle Scholar
• 23 Rahman S, Sahakian B J, Hodges J R, Rogers R D, Robbins T W. Specific cognitive deficits in early frontal variant frontotemporal dementia.  Brain . 1999;  122 1469-1493
  CrossrefPubMedGoogle Scholar
• 24 Talbot P R, Snowden J S, Lloyd J J, Neary D, Testa H J. The contribution of single photon emission tomography to the clinical differentiation of degenerative cortical brain disorders.  J Neurol . 1995;  242 579-586
  PubMedGoogle Scholar
• 25 Mummery C J, Patterson K, Wise R J, Vandenbergh R, Price C J, Hodges J R. Disrupted temporal lobe connections in semantic dementia.  Brain . 1999;  122 61-73
  CrossrefPubMedGoogle Scholar
• 26 Mitsuyama Y. Presenile dementia with motor neuron disease.  Dementia . 1993;  4 137-142
  PubMedGoogle Scholar
• 27 Lowe J. New pathological findings in amyotrophic lateral sclerosis.  J Neurol Sci . 1994;  124(suppl) 38-51
  PubMedGoogle Scholar
• 28 Vercelletto M, Ronin M, Huvet M, Magne C, Feve J R. Frontal type dementia preceding amyotrophic lateral sclerosis: a neuropsychological and SPECT study of five clinical cases.  Eur J Neurol . 1999;  6 295-299
  CrossrefPubMedGoogle Scholar
• 29 Rakowicz W P, Hodges J R. Dementia and aphasia in motor neuron disease: an underrecognised association?.  J Neurol Neurosurg Psychiatry . 1998;  65 881-889
  CrossrefPubMedGoogle Scholar
• 30 Caselli R J, Windebank A J, Petersen R C. Rapidly progressive aphasic dementia and motor neuron disease.  Ann Neurol . 1993;  33 200-207
  CrossrefPubMedGoogle Scholar
• 31 Rinne J O, Lee M S, Thompson P D, Marsden C D. Corticobasal degeneration: a clinical study of 36 cases.  Brain . 1994;  117 1183-1196
  CrossrefPubMedGoogle Scholar
• 32 Litvan I, Agid Y, Goetz C. Accuracy of the clinical diagnosis of corticobasal degeneration: a clinicopathologic study.  Neurology . 1997;  48 119-125
  CrossrefPubMedGoogle Scholar
• 33 Feany M B, Dickson D W. Neurodegenerative disorders with extensive tau pathology: a comparative study and review.  Ann Neurol . 1996;  40 139-148
  CrossrefPubMedGoogle Scholar
• 34 Pillon B, Blin J, Vidailhet M. The neuropsychological pattern of corticobasal degeneration: comparison with progressive supranuclear palsy and Alzheimer's disease.  Neurology . 1995;  45 1477-1483
  CrossrefPubMedGoogle Scholar
• 35 Graham N L, Zeman A, Young A W, Patterson K, Hodges J R. Dyspraxia in a patient with corticobasal degeneration: the role of visual and tactile inputs to action.  J Neurol Neurosurg Psychiatry . 1999;  66 334-344
  PubMedGoogle Scholar
• 36 Bergeron C, Pollanen M S, Weyer L, Black S E, Lang A E. Unusual clinical presentations of cortical-basal ganglionic degeneration.  Ann Neurol . 1996;  40 893-900
  CrossrefPubMedGoogle Scholar
• 37 Lippa C F, Cohen R, Smith T W, Drachman D A. Primary progressive aphasia with focal neuronal achromasia.  Neurology . 1991;  41 882-886
  PubMedGoogle Scholar
• 38 Ikeda K, Akiyama H, Iritani S. Corticobasal degeneration with primary progressive aphasia and accentuated cortical lesion in superior temporal gyrus: case report and review.  Acta Neuropathol (Berl) . 1996;  92 534-539
  CrossrefPubMedGoogle Scholar
• 39 McKeith I G, Galasko D, Kosaka K. Consensus guidelines for the clinical and pathologic diagnosis of dementia with Lewy bodies (DLB): report of the consortium on DLB international workshop.  Neurology . 1996;  47 1113-1124
  CrossrefPubMedGoogle Scholar
• 40 Imamura T, Hirono N, Hashimoto M. Clinical diagnosis of dementia with Lewy bodies in a Japanese dementia registry.  Dement Geriatr Cogn Disord . 1999;  10 210-216
  CrossrefPubMedGoogle Scholar
• 41 Olichney J M, Galasko D, Salmon D P. Cognitive decline is faster in Lewy body variant than in Alzheimer's disease.  Neurology . 1998;  51 351-357
  PubMedGoogle Scholar
• 42 Ballard C, McKeith I, Harrison R. A detailed phenomenological comparison of complex visual hallucinations in dementia with Lewy bodies and Alzheimer's disease.  Int Psychogeriatr . 1997;  9 381-388
  CrossrefPubMedGoogle Scholar
• 43 Ala T A, Yang K H, Sung J H, Frey 2nd H W. Hallucinations and signs of parkinsonism help distinguish patients with dementia and cortical Lewy bodies from patients with Alzheimer's disease at presentation: a clinicopathological study.  J Neurol Neurosurg Psychiatry . 1997;  62 16-21
  PubMedGoogle Scholar
• 44 Louis E D, Klatka L A, Liu Y, Fahn S. Comparison of extrapyramidal features in 31 pathologically confirmed cases of diffuse Lewy body disease and 34 pathologically confirmed cases of Parkinson's disease.  Neurology . 1997;  48 376-380
  PubMedGoogle Scholar
• 45 Gnanalingham K K, Byrne E J, Thornton A, Sambrook M A, Bannister P. Motor and cognitive function in Lewy body dementia: comparison with Alzheimer's and Parkinson's diseases.  J Neurol Neurosurg Psychiatry . 1997;  62 243-252
  CrossrefPubMedGoogle Scholar
• 46 Ballard C, Grace J, McKeith I, Holmes C. Neuroleptic sensitivity in dementia with Lewy bodies and Alzheimer's disease (Letter).  Lancet . 1998;  351 1032-1033
  CrossrefPubMedGoogle Scholar
• 47 Perry E K, Perry R H. Acetylcholine and hallucinations: disease-related compared to drug-induced alterations in human consciousness.  Brain Cogn . 1995;  28 240-258
  CrossrefPubMedGoogle Scholar
• 48 Shea C, MacKnight C, Rockwood K. Donepezil for treatment of dementia with Lewy bodies: a case series of nine patients.  Int Psychogeriatr . 1998;  10 229-238
  CrossrefPubMedGoogle Scholar
• 49 Ballard C G, Shaw F, Lowery K, McKeith I, Kenny R. The prevalence, assessment and associations of falls in dementia with Lewy bodies and Alzheimer's disease.  Dement Geriatr Cogn Disord . 1999;  10 97-103
  CrossrefPubMedGoogle Scholar
• 50 Boeve B F, Silber M H, Ferman T J. REM sleep behavior disorder and degenerative dementia: an association likely reflecting Lewy body disease.  Neurology . 1998;  51 363-370
  PubMedGoogle Scholar
• 51 Ferman T J, Boeve B F, Smith G E. REM sleep behavior disorder and dementia: cognitive differences when compared with AD.  Neurology . 1999;  52 951-957
  PubMedGoogle Scholar
• 52 Ballard C, McKeith I G. Psychiatric features in diffuse Lewy body disease (Letter; comment).  Neurology . 1998;  50 573
  PubMedGoogle Scholar
• 53 Hansen L, Salmon D, Galasko D. The Lewy body variant of Alzheimer's disease: a clinical and pathologic entity.  Neurology . 1990;  40 1-8
  PubMedGoogle Scholar
• 54 Shimomura T, Mori E, Yamashita H. Cognitive loss in dementia with Lewy bodies and Alzheimer disease.  Arch Neurol . 1998;  55 1547-1552
  CrossrefPubMedGoogle Scholar
• 55 Calderon J, Perry R J, Erzinçliog˘lu S W, Berrios G E, Dening T, Hodges J R. Perception, attention, and working memory are disproportionately impaired in dementia with Lewy Body (DLB) compared to Alzheimer's disease (AD).  J Neurol Neurosurg Psychiatry. In press.
  PubMedGoogle Scholar
• 56 Warrington E K, James M. The Visual Object and Space Perception Battery.  Bury St. Edmunds, UK: Thames Valley Test Company; 1991
  Google Scholar
• 57 Connor D J, Salmon D P, Sandy T J, Galasko D, Hansen L A, Thal L J. Cognitive profiles of autopsy-confirmed Lewy body variant vs. pure Alzheimer disease [published erratum appears in Arch Neurol 1998;55:1352].  Arch Neurol . 1998;  55 994-1000
  CrossrefPubMedGoogle Scholar
• 58 Barber R, Gholkar A, Scheltens P, Ballard C, McKeith I G, O'Brien J T. Medial temporal lobe atrophy on MRI in dementia with Lewy bodies.  Neurology . 1999;  52 1153-11581
  CrossrefPubMedGoogle Scholar