Rasch progressive Demenzen

Zusammenfassung

Demenz als klinisches Syndrom hat viele Ursachen. Während die Alzheimer-Erkrankung die häufigste Ursache neurodegenerativer Demenzen darstellt, kommen insbesondere bei rasch progressiven Demenzen differenzialdiagnostisch eine Reihe von potentiell reversiblen Erkrankungen infrage. Dabei müssen autoimmunvermittelte, entzündliche und metabolische Ursachen abgegrenzt werden. Daneben gehören die Creutzfeldt-Jakob-Krankheit und die rasch progressive Demenz vom Alzheimer-Typ neben einigen genetischen Erkrankungen zu dem differenzialdiagnostischen Spektrum. Die Arbeit gibt eine Übersicht über die häufigsten Ursachen und potentielle diagnostische Möglichkeiten wider.

Abstract

Dementia has a broad range of underlying diseases. Alzheimer’s disease is the most frequent one in neurodegenerative dementia. Nevertheless, in rapid progressive dementia, a variety of potential reversible conditions has to be considered. Autoimmune mediated, inflammatory and metabolic conditions have to be excluded. Creutzfeldt-Jakob disease, rapid progressive Alzheimer disease and some genetic disorders are part of the differential diagnostic spectrum. This manuscript gives an overview about the most frequent conditions and diagnostic potential.

Schlüsselwörter

Demenz - Progression - M. Alzheimer - Risikofaktoren

Keywords

dementia - rate of progression - Alzheimer‘s disease - risk factors

• Literatur

• 1 Starkstein SE, Jorge R, Mizrahi R et al. A prospective longitudinal study of apathy in Alzheimer’s disease. J Neurol Neurosurg Psychiatry 2006; 77: 8-11
• 2 Smith MZ, Esiri MM, Barnetson L et al. Constructional apraxia in Alzheimer’s disease: association with occipital lobe pathology and accelerated cognitive decline. Dement Geriatr Cogn Disord 2001; 12: 281-288
• 3 Laukka EJ, Fratiglioni L, Bäckman L. The influence of vascular disease on cognitive performance in the preclinical and early phases of Alzheimer’s disease. Dement Geriatr Cogn Disord 2010; 29: 498-503
• 4 Mielke MM, Rosenberg PB, Tschanz J et al. Vascular factors predict rate of progression in Alzheimer disease. Neurology 2007; 69: 1850-1858
• 5 Roselli F, Tartaglione B, Federico F et al. Rate of MMSE score change in Alzheimer’s disease: influence of education and vascular risk factors. Clin Neurol Neurosurg 2009; 111: 327-330
• 6 Silvestrini M, Pasqualetti P, Baruffaldi R et al. Cerebrovascular reactivity and cognitive decline in patients with Alzheimer disease. Stroke 2006; 37: 1010-1015
• 7 Abellan van Kann G, Rolland Y, Nourhashemi F et al. Cardiovascular disease risk factors and progression of Alzheimer’s disease. Dement Geriatr Cogn Disord 2009; 27: 240-246
• 8 Holmes C, Cunningham C, Zotova E et al. Systemic inflammation and disease progression in Alzheimer disease. Neurology 2009; 73: 768-774
• 9 Sanz C, Andrieu S, Sinclair A et al. Diabetes is associated with a slower rate of cognitive decline in Alzheimer disease. Neurology 2009; 73: 1359-1366
• 10 Mangone CA. Clinical heterogeneity of Alzheimer’s disease. Different clinical profiles can predict the progression rate. Rev Neurol 2004; 38: 675-681
• 11 Wilkosz PA, Seltman HJ, Devlin B et al. Trajectories of cognitive decline in Alzheimer’s disease. Int Psychogeriatr 2010; 22: 281-290
• 12 Josephs KA, Ahlskog JE, Parisi JE et al. Rapidly progressive neurodegenerative dementias. Arch Neurol 2009; 66: 201-207
• 13 Schmidt C, Redyk K, Meissner B et al. Clinical features of rapidly progressive Alzheimer’s disease. Dement Geriatr Cogn Disord 2010; 29: 371-378
• 14 Tschampa HJ, Neumann M, Zerr I et al. Patients with Alzheimer’s disease and dementia with Lewy bodies mistaken for Creutzfeldt-Jakob disease. J Neurol Neurosurg Psychiatry 2001; 71: 33-39
• 15 Van Everbroeck B, Dobbeleir I, De Waele M et al. Differential diagnosis of 201 possible Creutzfeldt-Jakob disease patients. J Neurol 2004; 251: 298-304
• 16 Pavlik VN, Doody RS, Massman PJ et al. Influence of premorbid IQ and education on progression of Alzheimer’s disease. Dement Geriatr Cogn Disord 2006; 22: 367-377
• 17 Portet F, Scarmeas N, Cosentino S et al. Extrapyramidal signs before and after diagnosis of incident Alzheimer disease in a prospective population study. Arch Neurol 2009; 66: 1120-1126
• 18 Scarmeas N, Albert M, Brandt J et al. Motor signs predict poor outcomes in Alzheimer disease. Neurology 2005; 64: 1696-1703
• 19 Soto ME, Andrieu S, Cantet C et al. Predictive value of rapid decline in mini mental state examination in clinical practice for prognosis in Alzheimer’s disease. Dement Geriatr Cogn Disord 2008; 26: 109-116
• 20 Volicer L, Smith S, Volicer BJ. Effect of seizures on progression of dementia of the Alzheimer type. Dementia 1995; 6: 258-263
• 21 Atchison TB, Massman PJ, Doody RS. Baseline cognitive function predicts rate of decline in basic-care abilities of individuals with dementia of the Alzheimer’s type. Arch Clin Neuropsychol 2007; 22: 99-107
• 22 Ito K, Ahadieh S, Corrigan B et al. Disease progression meta-analysis model in Alzheimer’s disease. Alzheimers Dement 2010; 6: 39-53
• 23 Marra C, Silveri MC, Gainotti G. Predictors of cognitive decline in the early stage of probable Alzheimer’s disease. Dement Geriatr Cogn Disord 2000; 11: 212-218
• 24 Hui JS, Wilson RS, Bennett DA et al. Rate of cognitive decline and mortality in Alzheimer’s disease. Neurology 2003; 61: 1356-1361
• 25 Kester MI, van der Vlies AE, Blankenstein MA et al. CSF biomarkers predict rate of cognitive decline in Alzheimer disease. Neurology 2009; 73: 1353-1358
• 26 Cosentino S, Scarmeas N, Helzner E et al. APOE epsilon 4 allele predicts faster cognitive decline in mild Alzheimer disease. Neurology 2008; 70: 1842-1849
• 27 van der Vlies AE, Koedam EL, Pijnenburg YA et al. Most rapid cognitive decline in APOE epsilon4 negative Alzheimer’s disease with early onset. Psychol Med 2009; 39: 1907-1911
• 28 Holmes C, Ballard C, Lehmann D et al. Rate of progression of cognitive decline in Alzheimer’s disease: effect of butyrylcholinesterase K gene variation. J Neurol Neurosurg Psychiatry 2005; 76: 640-643
• 29 Tumini E, Porcellini E, Chiappelli M et al. The G51S purine nucleoside phosphorylase polymorphism is associated with cognitive decline in Alzheimer’s disease patients. Hum Psychopharmacol 2007; 22: 75-80
• 30 Porcellini E, Calabrese E, Guerini F et al. The hydroxy-methyl-glutaryl CoA reductase promoter polymorphism is associated with Alzheimer’s risk and cognitive deterioration. Neurosci Lett 2007; 416: 66-70
• 31 Belbin O, Beaumont H, Warden D et al. PSEN1 polymorphisms alter the rate of cognitive decline in sporadic Alzheimer’s disease patients. Neurobiol Aging 2009; 30: 1992-1999
• 32 Belbin O, Dunn JL, Chappell S et al. A SNP in the ACT gene associated with astrocytosis and rapid cognitive decline in AD. Neurobiol Aging 2008; 29: 1167-1176
• 33 Kamboh MI, Minster RL, Kenney M et al. Alpha-1-antichymotrypsin (ACT or SERPINA3) polymorphism may affect age-at-onset and disease duration of Alzheimer’s disease. Neurobiol Aging 2006; 27: 1435-1439
• 34 Licastro F, Chiappelli M, Grimaldi LM et al. A new promoter polymorphism in the alpha-1-antichymotrypsin gene is a disease modifier of Alzheimer’s disease. Neurobiol Aging 2005; 26: 449-453
• 35 Murphy Jr GM, Claassen JD, DeVoss JJ et al. Rate of cognitive decline in AD is accelerated by the interleukin-1 alpha -889 *1 allele. Neurology 2001; 56: 1595-1597
• 36 Bossù P, Ciaramella A, Moro ML et al. Interleukin 18 gene polymorphisms predict risk and outcome of Alzheimer’s disease. J Neurol Neurosurg Psychiatry 2007; 78: 807-811
• 37 Chiappelli M, Nasi M, Cossarizza A et al. Polymorphisms of fas gene: relationship with Alzheimer’s disease and cognitive decline. Dement Geriatr Cogn Disord 2006; 22: 296-300
• 38 Li K, Dai D, Zhao B et al. Association between the RAGE G82S polymorphism and Alzheimer’s disease. J Neural Transm 2010; 117: 97-104
• 39 Aksamit Jr AJ, Preissner CM, Homburger HA. Quantitation of 14-3-3 and neuron-specific enolase proteins in CSF in Creutzfeldt-Jakob disease. Neurology 2001; 57: 728-730
• 40 Collins S, Boyd A, Fletcher A et al. Creutzfeldt-Jakob disease: diagnostic utility of 14-3-3 protein immunodetection in cerebrospinal fluid. J Clin Neurosci 2000; 7: 203-208
• 41 Gelpi E, Heinzl H, Hoftberger R et al. Creutzfeldt-Jakob disease in Austria: an autopsy-controlled study. Neuroepidemiology 2008; 30: 215-221
• 42 Haïk S, Brandel JP, Sazdovitch V et al. Dementia with Lewy bodies in a neuropathologic series of suspected Creutzfeldt-Jakob disease. Neurology 2000; 55: 1401-1404
• 43 Huang N, Marie SK, Livramento JA et al. 14-3-3 protein in the CSF of patients with rapidly progressive dementia. Neurology 2003; 61: 354-357
• 44 Jansen C, Schuur M, Spliet WG et al. Eleven years of autopsy on account of Creutzfeldt-Jakob disease in the Netherlands. Ned Tijdschr Geneeskd 2009; 153: A172
• 45 Jayaratnam S, Khoo AK, Basic D. Rapidly progressive Alzheimer’s disease and elevated 14-3-3 proteins in cerebrospinal fluid. Age Ageing 2008; 37: 467-469
• 46 Mahmoudi R, Manckoundia P, Morrone I et al. Atypical case of Alzheimer’s disease mimicking Creutzfeldt-Jakob disease: interest of cerebrospinal fluid biomarkers in the differential diagnosis. J Am Geriatr Soc 2010; 58: 1821-1823
• 47 Reinwald S, Westner IM, Niedermaier N. Rapidly progressive Alzheimer’s disease mimicking Creutzfeldt-Jakob disease. J Neurol 2004; 251: 1020-1022
• 48 Kelley BJ, Boeve BF, Josephs KA. Young-onset dementia: demographic and etiologic characteristics of 235 patients. Arch Neurol 2008; 65: 1502-1508
• 49 McMurtray A, Clark DG, Christine D et al. Early-onset dementia: frequency and causes compared to late-onset dementia. Dement Geriatr Cogn Disord 2006; 21: 59-64
• 50 Stefanova N, Bucke P, Duerr S et al. Multiple system atrophy: an update. Lancet Neurol 2009; 8: 1172-1178
• 51 Brouwers N, Sleegers K, Van Broeckhoven C. Molecular genetics of Alzheimer’s disease: an update. Ann Med 2008; 40: 562-583
• 52 Sampson EL, Warren JD, Rossor MN. Young onset dementia. Postgrad Med J 2004; 80: 125-139
• 53 Lippa CF, Duda JE, Grossman M et al. DLB and PDD boundary issues: diagnosis, treatment, molecular pathology, and biomarkers. Neurology 2007; 68: 812-819
• 54 Selikhova M, Williams DR, Kempster PA et al. A clinico-pathological study of subtypes in Parkinson’s disease. Brain 2009; 132: 2947-2957
• 55 Walker FO. Huntington’s disease. Lancet 2007; 369: 218-228
• 56 Zerr I, Poser S. Clinical diagnosis and differential diagnosis of CJD and vCJD. With special emphasis on laboratory tests. APMIS 2002; 110: 88-98
• 57 Shibuya-Tayoshi S, Tsuchiya K, Seki Y et al. Presenile dementia mimicking Pick’s disease: an autopsy case of localized amygdala degeneration with character change and emotional disorder. Neuropathology 2005; 25: 235-240
• 58 Schmidt C, Wolff M, Weitz M et al. Rapidly progressive Alzheimer disease. Arch Neurol 2011; 68: 1124-1130
• 59 Gaig C, Valldeoriola F, Gelpi E et al. Rapidly progressive diffuse Lewy body disease. Mov Disord 2011; 26: 1316-1323
• 60 Kelley BJ, Boeve BF, Josephs KA. Rapidly progressive young-onset dementia. Cogn Behav Neurol 2009; 22: 22-27