Schmidt C, Haïk S, Satoh K, Rábano A, Martinez-Martin P, Roeber S, Brandel JP, Calero-Lara M, de Pedro-Cuesta J, Laplanche JL, Hauw JJ, Kretzschmar H, Zerr I. Rapidly progressive Alzheimer's disease: a multicenter update. J Alzheimers Dis. 2012;30(4):751-6. PubMed.
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Institute of Clinical Neurobiology
This, and the previous article by Schmidt et al. (Schmidt et al., 2010), are very interesting. They discern the possibility that there is a subgroup of AD patients that progresses more rapidly, mimicking Creutzfeldt-Jakob disease (CJD) and displaying genetic profiles (absence of ApoE4) and biomarkers distinct from classical AD. Although, as the authors state, the neuropathologic data justify the diagnosis of AD. Unfortunately, no detailed morphological data were presented. This would be important, since there are several distinct clinicopathologic subtypes of AD (Murray et al., 2011; Jellinger, 2012), although they show similar disease duration.
According to the U.S. National Prion Disease Pathology Surveillance Center, rapidly progressing dementia is frequently mistaken for CJD. Among 1,106 brains autopsied, 32 percent were negative for prion disease. AD was the most frequent diagnosis (50 percent), vascular dementia being less common (12 percent), while 23 percent were potentially treatable disorders, including immune-mediated disease such as infections, etc. (Chitravas et al., 2011). The roles of prions and inflammation in rapidly progressive AD are unclear, although links between prions and AD are under investigation, and the presence of amyloid plaques in prion diseases like CJD is well documented.
In conclusion, more detailed clinicopathologic studies are warranted in order to better elucidate rare rapidly progressive AD.
References:
Schmidt C, Redyk K, Meissner B, Krack L, von Ahsen N, Roeber S, Kretzschmar H, Zerr I. Clinical features of rapidly progressive Alzheimer's disease. Dement Geriatr Cogn Disord. 2010;29(4):371-8. PubMed.
Murray ME, Graff-Radford NR, Ross OA, Petersen RC, Duara R, Dickson DW. Neuropathologically defined subtypes of Alzheimer's disease with distinct clinical characteristics: a retrospective study. Lancet Neurol. 2011 Sep;10(9):785-96. PubMed.
Jellinger KA. Neuropathological subtypes of Alzheimer's disease. Acta Neuropathol. 2012 Jan;123(1):153-4. PubMed.
Chitravas N, Jung RS, Kofskey DM, Blevins JE, Gambetti P, Leigh RJ, Cohen ML. Treatable neurological disorders misdiagnosed as Creutzfeldt-Jakob disease. Ann Neurol. 2011 Sep;70(3):437-44. PubMed.
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