This is an interesting French study correlating clinical symptoms related to language and speech with the pathology seen in autopsied brain tissue. Eighteen patients were monitored over a 15-year period. Four patients developed right-predominant corticobasal syndrome. One patient was given a clinical diagnosis of PSP.
“Of the 18 cases, 8 had FTLD-TDP, 3 had AD, 2 had PSP, 2 had CBD, 2 had PiD, and 1 had AGD,” upon brain autopsy. Of the two who had confirmed PSP diagnoses, one was diagnosed with the behavioral variant of FTD during life though the diagnosis was later changed to PSP when supranuclear palsy appeared. The other was diagnosed with corticobasal syndrome during life.
Of the four patients diagnosed with corticobasal syndrome during life, one had PSP upon brain autopsy, one had CBD, one had Pick’s Disease, and one had FTLD-TDP.
Of the two cases who had confirmed CBD diagnoses, one was diagnosed with FTDbv during life and the other with CBS during life.
The five patients who stopped speaking (“progressive anarthria”) all had tau pathology — either PSP, CBD, or Pick disease. (“[All] progressed to mutism, swallowing difficulties, and orofacial apraxia.”)
Findings of atrophy (on a CT or MRI) and findings of hypometabolism (on a SPECT) in nearly all of the the cases are provided along with info such as disease duration, MMSE score, Frontotemporal Behavior Scale rating, and Dementia Rating Scale score.
Eighteen patients is a very small study. We’ll have to see if the results can be replicated.
Neurology. 2009 Nov 25. [Epub ahead of print]
Prediction of pathology in primary progressive language and speech disorders.
Deramecourt V, Lebert F, Debachy B, Mackowiak-Cordoliani MA, Bombois S, Kerdraon O, Buée L, Maurage CA, Pasquier F.
From the Memory Clinic (V.D., F.L., B.D., M.A.M.-C., S.B., F.P.) and Department of Neuropathology (O.K., C.-A.M.), CHU-Lille, Lille; University Lille Nord de France (V.D., F.L., B.D., M.A.M.-C., S.B., O.K., L.B., C.-A.M., F.P.), Lille; and INSERM (O.K., L.B., C.-A.M.), JP Aubert Research Centre, Lille, France.
OBJECTIVE: Frontotemporal lobar degeneration (FTLD) encompasses a variety of clinicopathologic entities. The antemortem prediction of the underlying pathologic lesions is reputed to be difficult.
This study sought to characterize correlations between 1) the different clinical variants of primary progressive language and speech disorders and 2) the pathologic diagnosis.
METHODS: The latter was available for 18 patients having been prospectively monitored in the Lille Memory Clinic (France) between 1993 and 2008.
RESULTS: The patients were diagnosed with progressive anarthria (n = 5), agrammatic progressive aphasia (n = 6), logopenic progressive aphasia (n = 1), progressive jargon aphasia (n = 2), typical semantic dementia (n = 2), and atypical semantic dementia (n = 2).
All patients with progressive anarthria had a tau pathology at postmortem evaluation: progressive supranuclear palsy (n = 2), Pick disease (n = 2), and corticobasal degeneration (n = 1).
All patients with agrammatic primary progressive aphasia had TDP-43-positive FTLD (FTLD-TDP).
The patients with logopenic progressive aphasia and progressive jargon aphasia had Alzheimer disease.
Both cases of typical semantic dementia had FTLD-TDP.
The patients with atypical semantic dementia had tau pathologies: argyrophilic grain disease and corticobasal degeneration.
CONCLUSIONS: The different anatomic distribution of the pathologic lesions could explain these results: opercular and subcortical regions in tau pathologies with progressive anarthria, the left frontotemporal cortex in TDP-43-positive frontotemporal lobar degeneration (FTLD-TDP) with agrammatic progressive aphasia, the bilateral lateral and anterior temporal cortex in FTLD-TDP or argyrophilic grain disease with semantic dementia, and the left parietotemporal cortex in Alzheimer disease with logopenic progressive aphasia or jargon aphasia. These correlations have to be confirmed in larger series.
PubMed ID#: 19940270 (see pubmed.gov for abstract only)
Robin’s note: I suggest looking up terms in wikipedia.