Progressive language disorder due to lobar atrophy

Abstract

Sixteen patients with progressive language disorder have been studied longitudinally. Anomia was a prominent presenting characteristic and mutism ultimately occurred. Patients, however, were clinically heterogeneous. Some exhibited nonfluent, agrammatic features, whereas others demonstrated a fluent aphasia, with profound loss of word meaning. Although language disorder remained the sole symptom in a minority of patients, in others an associative agnosia or personality and behavioral changes, or both, emerged. Findings on computed tomography and single photon emission tomography mirrored the areas of dysfunction suggested by the neuropsychological profiles and demonstrated abnormalities restricted to the left hemisphere or involving bilateral frontotemporal cortices. Brains of 3 patients, with distinctive clinical pictures, have been examined at autopsy. Each revealed a focal distribution of atrophy, gliosis and spongiform change, and an absence of senile plaques and neurofibrillary tangles. There was clinical and pathological overlap with frontal lobe dementia. We argue that progressive language disorder is clinically heterogeneous and forms part of a spectrum of clinical presentations of non-Alzheimer lobar atrophy.

Discovering EEG resting state alterations of semantic dementia

“…Moreover, the current results correspond with the clinical observation of a distinct symptomatology in semantic dementia rather than a general cognitive deficit (Snowden et al, 1992, Hodges, 2001. In other words, semantic dementia is characterized by an isolated semantic memory deficit (best reflected in the MMSE language score of the available data) while other cognitive functions remain intact.…”

Syntactic and thematic components of sentence processing in progressive nonfluent aphasia and nonaphasic frontotemporal dementia

“…It is characterized by effortful and agrammatic speech which is often dysarthric and contains phonemic paraphasic errors (Grossman, 2002). PNFA patients often have difficulty understanding grammatical aspects of sentences (Grossman & Ash, 2004;Grossman et al, 1996;Snowden, Neary, Mann, Goulding, & Testa, 1992;Thompson, Ballard, Tait, Weintraub, & Mesulam, 1997).…”

The anatomy of semantic knowledge: Medial vs. lateral temporal lobe

“…Additional important information about semantic knowledge comes from the study of patients with semantic dementia (SD), also known as the temporal variant of fronto-temporal dementia (8)(9)(10). These patients have progressive atrophy, prominently involving the anterolateral temporal lobes, and they have severe loss of conceptual knowledge about objects, facts, and word meanings.…”