Affiliations: [a] Department of Cognitive Neurology, Neuropsychology and Neuropsychiatry, Institute for Neurological Research “Raul Carrea”, FLENI, Buenos Aires, Argentina | [b] Laboratory for Memory Research, Hospital Abel Zubizarreta, GCBA. Buenos Aires, Argentina | [c] Department of Neuroimaging, Institute for Neurological Research “Raul Carrea”, FLENI, Buenos Aires, Argentina | [d] Department of Neuropathology, Institute for Neurological Research “Raul Carrea”, FLENI, Buenos Aires, Argentina
Correspondence:
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Correspondence to: Prof. Dr. Ricardo F. Allegri, Department of Cognitive Neurology, Neuropsychology and Neuropsychiatry, Institute for Neurological Research “Raul Carrea”, FLENI, Montañeses 2325, (C1428AQK) Buenos Aires, Argentina. Tel.: +54 11 5777 3200/Ext: 2818; Fax: +54 11 5777 3209; E-mail: [email protected].
Abstract: We report a 77-year-old man, presenting with progressive aphasia as an initial symptom, who developed severe dementia over the course of 20 months. Frontal cortex PrPSc western blot was type 2 and codon 129 was MM; brain neuropathology showed cortical vacuoles with perivacuolar PrP immunostaining characteristic of MM2C. Cerebellum showed focal coarse, patchy staining in different sections of the molecular layer, diffuse fine punctuate and coarse PrP immunopositive deposits in the granule cell layer, and focal synaptic immunostaining in the molecular layer, suggestive of MM1+2C by histotyping. This clinical presentation has not yet been described in an MM1+2C subtype by histotyping.
