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First symptom guides diagnosis and prognosis in neurodegenerative diseases—a retrospective study of autopsy proven cases
Author(s) -
Vöglein Jonathan,
Kostova Irena,
Arzberger Thomas,
Roeber Sigrun,
Schmitz Peer,
Simons Mikael,
Ruf Viktoria,
Windl Otto,
Herms Jochen,
Dieterich Marianne,
Danek Adrian,
Höglinger Günter U.,
Giese Armin,
Levin Johannes
Publication year - 2021
Publication title -
european journal of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.881
H-Index - 124
eISSN - 1468-1331
pISSN - 1351-5101
DOI - 10.1111/ene.14800
Subject(s) - progressive supranuclear palsy , frontotemporal lobar degeneration , corticobasal degeneration , medicine , dementia with lewy bodies , dementia , parkinsonism , aphasia , lewy body , frontotemporal dementia , population , amyotrophic lateral sclerosis , atrophy , gait disturbance , primary progressive aphasia , disease , pathology , psychiatry , physical medicine and rehabilitation , environmental health
Background and purpose Clinical diagnostic criteria for neurodegenerative diseases have been framed based on clinical phenomenology. However, systematic knowledge about the first reported clinical symptoms in neurodegenerative diseases is lacking. Therefore, the aim was to determine the prevalence and clinical implications of the first clinical symptom (FS) as assessed by medical history in neuropathologically proven neurodegenerative diseases. Methods Neuropathological diagnoses from the Neurobiobank Munich, Germany, were matched with clinical records for analyses of the diagnostic and prognostic values of FSs. Results In all, 301 patients with the neuropathological diagnoses Alzheimer disease (AD), progressive supranuclear palsy (PSP), frontotemporal lobar degeneration (FTLD), Lewy body disease (LBD) including the neuropathologically indistinguishable clinical phenotypes Parkinson disease and dementia with Lewy bodies, multiple system atrophy (MSA) and corticobasal degeneration (CBD) were studied. Memory disturbance was the most common FS in AD (34%), FTLD (19%) and LBD (26%), gait disturbance in PSP (35%) and MSA (27%) and aphasia and personality changes in CBD (20%, respectively). In a model adjusting for prevalence in the general population, AD was predicted by memory disturbance in 79.0%, aphasia in 97.2%, personality changes in 96.0% and by cognitive disturbance in 99.0%. Gait disturbance and tremor predicted LBD in 54.6% and 97.3%, coordination disturbance MSA in 59.4% and dysarthria FTLD in 73.0%. Cognitive FSs were associated with longer survival in AD (12.0 vs. 5.3 years; p < 0.001) and FTLD (8.2 vs. 4.1 years; p = 0.005) and motor FSs with shorter survival in PSP (7.2 vs. 9.7; p = 0.048). Conclusions Assessing FSs in neurodegenerative diseases may be beneficial for accuracy of diagnosis and prognosis and thereby may improve clinical care and precision of study recruitment.