title: Frontotemporal dementia with severe thalamic involvement: a clinical and neuropathological study.
abstract: Frontotemporal dementia (FTD) is the third-leading cause of cortical dementia after Alzheimer's disease and Lewy body dementia, and is characterized by a dementia where behavioral disturbances are prominent and appear early in the course of the disease. We report the case of a 58 year-old man affected by dementia with behavioral disturbances, in addition to rigid-hypokinetic and a lower motor neuron syndrome that were present at later stages of the illness. Neuroimaging studies showed frontotemporal atrophy. Neuropathological studies revealed intense thalamic neuronal loss and astrocytic gliosis, as well as moderate frontotemporal neuronal loss, astrocytosis and spongiform degeneration. Thalamic degeneration has previously been described among the wide group of neuropathological features of FTD. The aim of the present study is to show the clinical and neuropathological aspects of thalamic degeneration in FTD, along with its role in behavioral disturbances, a common finding in this condition.
authors.affiliation: Department of Pathology, School of Medicine, University of São Paulo, São Paulo, SP, Brazil. radano@tecway.com.br
authors.forename: Márcia
authors.initials: M
authors.lastname: Radanovic
authors.forename: Sérgio
authors.initials: S
authors.lastname: Rosemberg
authors.forename: Rogério
authors.initials: R
authors.lastname: Adas
authors.forename: Stênio C
authors.initials: SC
authors.lastname: Miranda
authors.forename: Paulo
authors.initials: P
authors.lastname: Caramelli
authors.forename: Leonardo
authors.initials: L
authors.lastname: Caixeta
authors.forename: Ricardo
authors.initials: R
authors.lastname: Nitrini
mesh_terms: D001284:Atrophy; D003704:Dementia / Q000175:diagnosis / Q000473:pathology*; D017809:Fatal Outcome; D005625:Frontal Lobe / Q000473:pathology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009474:Neurons / Q000473:pathology; D013702:Temporal Lobe / Q000473:pathology*; D013788:Thalamus / Q000473:pathology*; D015899:Tomography, Emission-Computed, Single-Photon