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IC‐P‐022: Reversible dementia as first symptom of multiple myeloma: A case report
Author(s) -
Markou Sofia,
Markoula Sofia,
Kapsali Eleni,
Kyritsis Athanasios,
Pelidou SygklitiHenrietta
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.2011.05.039
Subject(s) - medicine , multiple myeloma , dementia , peripheral neuropathy , neurological examination , pediatrics , surgery , diabetes mellitus , disease , endocrinology
language-led dementias presenting canonically as Semantic Dementia (SemD), Progressive Non-Fluent Aphasia (PNFA) or Logopenic Aphasia (LPA). A further subtype of progressive aphasia associated with progranulin mutations (GAA) has been described. The PPA spectrum is neurobiologically heterogeneous and may be underpinned by Alzheimer or non-Alzheimer pathologies; in particular, LPA is closely associated with Alzheimer’s disease. There is a need both for improved understanding of syndrome pathophysiology and accurate diagnostic biomarkers in PPA. Here we assessed changes in white matter pathways in relation to clinical syndrome and grey matter loss in a cohort of patients with PPA. Methods: 26 consecutive patients with a clinical diagnosis of PPA (8 SemD; 8 PNFA; 7 LPA; 3GAA) and 20 healthy age-matched individuals had volumetric brain MRI and DTI. Tractographic and VBM changes in PPA syndromes compared with healthy controls and between syndromes were assessed using Tract Based Spatial Statistics and SPM8 software. Results: PPA syndromic groups showed well-defined fractional anisotropy (FA), axial (DA) and radial diffusivity (DR) changes affecting white matter pathways implicated in language processing (Figure 1). Compared with healthy individuals, all PPA groups showed reduced FA and increased DR and DA in the anterior superior longitudinal fasciculus (SLF), and additional tracts were involved in particular syndromes (in SemD, inferior longitudinal fasciculus (ILF), uncinate fasciculus (UF), and commissural pathways; in PNFA, more posterior SLF; in LPA and GAA, ILF). Inter-group comparisons showed significantly (Threshold free cluster enhancement corrected p <0.05) greater involvement of ILF and UF in SD than in PNFA or GAA; and greater involvement of left ILF in GAA than PNFA. Tract alterations were largely restricted to the left hemisphere in GAA but bihemispheric in other syndromes. There was evidence of correlation between white matter changes and regional grey matter loss. Conclusions: DTI delineates specific profiles of language network breakdown in PPA and may generate useful biomarkers for distinguishing PPA syndromes and pathologies.