Premium
P4‐033: Triple neurodegeneration in a dementia/parkinsonism patient ‐ case report
Author(s) -
Trošt Maja,
Pirtošek Zvezdan,
Popovic Mara
Publication year - 2011
Publication title -
alzheimer's and dementia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 6.713
H-Index - 118
eISSN - 1552-5279
pISSN - 1552-5260
DOI - 10.1016/j.jalz.2011.05.2053
Subject(s) - parkinsonism , psychology , basal ganglia , atrophy , dementia with lewy bodies , pathology , gliosis , medicine , dementia , neuroscience , disease , central nervous system
Background: A 70 years old female developed gradual loss of interest for every day activities, memory problems and anhedonia . She lost 13 kg in a few months, experienced urinary frequency and constipation. At six months of the disease her MMSE was 26/30. A mild akinetc-rigid (AR) syndrome without tremor or gaze palsy, occasional myoclonus and slow, shuffling gait in flexed posture with severe postural instability were noticed without improvement on levodopa. Three months later she deteriorated severely withdementia (MMSE 16/30), hallucinations, severe AR syndrome, hyper-reflexia, facial dystonia and inability to walk. Brain MRI revealed mild, general atrophy. EEG was diffusely slow without any periodic rhythm, CSF was normal,with negative 14-3-3 protein. Dopamine transporter SPECT was normal. Patient died 11 months after first symptoms developed. Methods: At authopsy several brain samples were obtained and fresh frozen for DNA analysis on PRNP. Whole brain fixation sampling was performed according to Alzheimer’s disease (AD) protocol. The tissue samples were immersed in 95% formic acid for one hour before paraffin embedding. 5 Âmm thick sections were tested immunohistochemically on PrP, alfa-synuclein, A-beta, and hiperphosphorilated tau protein. Results: Spongiform neuropil degeneration, neuronal loss and gliosis were present in basal ganglia, neocortex and subcortical gray matter. Synaptic type of immunohistochemical reaction (IHR) on PrP, performed on the sections of basal ganglia and neocortex, was present. Gene analysis of PRNP showed no mutation with the codon 129MM status. Distribution and quantity of tau positive neuro fibrilary changes, beside A-beta positive senile plaques, corresponded to the stage III of AD according to the Braakstaging. Pigmented nuclei in brain stem did not show obvious neuronal loss, butsome neuronal Lewy bodies. IHR on alfa-synuclein revealed some positive neurons and Lewy neurites in the region of the three nuclei. Few cortical Lewy bodies were present in cingular cortex. Conclusions: In a patien twith rapid progression of park insonism and dementia, and suspicion of sporadic Creutzfeldt-Jakob disease (sCJD) but without CSF and EEG markers for sCJD apost-mortem brain examination showed a rare coincidence of three neurodegenerative disease: sCJD, AD and synucleinopathy of Parkinson’s disease type, assuming sCJD to be clinically relevant. P4-034 PATHOLOGICALLY CONFIRMED FRONTAL VARIANTALZHEIMERÂ S DISEASE: TWO CASES AND REVIEW OF THE LITERATURE Alberto Villarejo, Alejandro Herrero, Alberto Rabano, Jes us Porta-Etessam, Carmen Guerrero-M arquez, Alvaro S anchez-Ferro, Felix Bermejo-Pareja, Hospital Universitario Doce de Octubre, Madrid, Spain; 2 Hospital Doce de Octubre, Madrid, Spain; 3 Banco de tejidos de la Fundaci on CIEN (BT-CIEN), Madrid, Spain; Hospital Cl ınico San Carlos, Madrid, Spain; Fundaci on Hospital de Alcorc on, Alcorc on (Madrid), Spain; 6 Hospital Doce de Octubre. Universidad Complutense de Madrid. CIBERNED, Madrid, Spain.