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What Is Semantic Dementia? a Cohort Study of Diagnostic Features and Clinical Boundaries

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What Is Semantic Dementia? a Cohort Study of Diagnostic Features and Clinical Boundaries ORIGINAL CONTRIBUTION What Is Semantic Dementia? A Cohort Study of Diagnostic Features and Clinical Boundaries Andrew Kertesz, MD; Sarah Jesso, BA; Michal Harciarek, PhD; Mervin Blair, MA; Paul McMonagle, MD Objectives: To describe a large, clinically defined co- tions were frequent in SD (54.1%) but phonological er- hort of patients with semantic dementia (SD) that high- rors were absent, in contrast to progressive nonfluent lights important, sometimes overlooked features and to aphasia with the opposite pattern. All but 3 patients with compare it with similar entities. probable SD questioned the meaning of words. Patients with SD had significantly lower naming and comprehen- Design: Cohort study. sion scores, and their fluency was between progressive nonfluent aphasia and Alzheimer disease or behavioral Setting: A cognitive neurology clinic. frontotemporal dementia. Behavior was abnormal in 94.6% of patients with probable SD. Patients: A population of 48 patients clinically diag- nosed with SD was contrasted with 52 patients with pro- Conclusions: Semantic dementia is distinguishable from gressive nonfluent aphasia, 42 patients with a behav- other presentations of frontotemporal dementia and Alz- ioral variety of frontotemporal dementia, and 105 patients heimer disease, not only by fluent speech and impaired with Alzheimer disease on speech output characteris- tics, comprehension, naming, and repetition subtests of comprehension without loss of episodic memory, syn- the Western Aphasia Battery, the Frontal Behavioral In- tax, and phonology but also by empty, garrulous speech ventory, and other cognitive tests. Neuroimaging was vi- with thematic perseverations, semantic paraphasias, and sually analyzed, and 6 patients with SD had autopsy. poor category fluency. Questioning the meaning of words (eg, “What is steak?”) is an important diagnostic clue not Results: Of 37 patients with probable SD, 48.6% had se- seen in other groups, and behavior change is prevalent. mantic jargon; 21.6%, excessive garrulous output; and 75.7%, some pragmatic disturbance. Semantic substitu- Arch Neurol. 2010;67(4):483-489 EMANTIC DEMENTIA (SD) Snowden et al1 and has been adopted by designates a progressive cog- others,9 including the consensus criteria nitive and language deficit, of Neary et al,13 as a variety of frontotem- primarily involving compre- poral dementia (FTD). The incidence of hension of words and related SD is estimated by one clinic to be 25% in Ssemantic processing.1 These patients lose their patients with FTD.12 the meaning of words, usually nouns, but Semantic dementia has been equated retain fluency, phonology, and syntax. with fluent progressive aphasia.9,14 Flu- Author Affiliations: Pick2 described similar patients as hav- ent aphasia, however, is common in Alz- Department of Clinical 15 Neurological Sciences, ing “pure word deafness” in association heimer disease (AD) and, at the onset, University of Western Ontario with left temporal atrophy. Transcortical all patients with progressive aphasia are (Dr Kertesz), and Cognitive sensory aphasia was used for similar cases.3 fluent, even those who become nonflu- Neurology and Alzheimer Semantic aphasia was a term used by Head4 ent later.16,17 The fluency-nonfluency dis- Research Centre, St Joseph’s in war-injured patients for a 2-way dis- tinction is often arbitrary and rarely quan- Hospital, London, Ontario, turbance of comprehension and naming. titated. Primary progressive aphasia is Canada (Dr Kertesz and The condition was called gogi (meaning) subdivided variably and sometimes in- Ms Jesso); University of aphasia in Japan.5 Some patients were con- cludes SD. Here we used the term pro- Gdan´ sk, Gdan´ sk, Poland sidered to have loss of semantic memory6 gressive nonfluent aphasia (PNFA) for a (Dr Harciarek); Concordia and others a language impairment.7,8 Se- comparison group. Semantic deficits, con- University, Montreal, Canada 18 (Mr Blair); and the Department mantic dementia has been further elabo- sidered basic to SD, also appear in AD. of Neurology, Royal Victoria rated as the degradation of a single cen- Because of these overlapping features from Hospital, Belfast, Northern tral network of conceptual knowledge.9-12 different clinical and biological entities, the Ireland (Dr McMonagle). The definition of SD originates from diagnostic boundaries remain uncertain. (REPRINTED) ARCH NEUROL / VOL 67 (NO. 4), APR 2010 WWW.ARCHNEUROL.COM 483 ©2010 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/28/2021 Table 1. Clinical Features of Semantic Dementiaa Sex/Age at Onset, y/ Duration of Memory Questioning Visual Face Surface Speech Neurological Autopsy 1st Syndrome Illness, yb for Names Words Agnosia Agnosia Pragmatics Artc Dyslexia Output Image Findings F/60/SD 4 1 1 1 1 1 . 1 Empty LTA FTLD-U F/56/bvFTD 4 1 1 1 1 1 . 1 Stereotypy RTAϾLF FTLD-U M/61/bvFTD 6 1 . 1 . 1 Semantic jargon LTA FTLD-U M/46/SD 1 1 1 1 . 1 1 1 Empty LTA FTLD-U M/38/bvFTD 2 1 . 1 . Semantic jargon, perseverative LTA FTLD-U F/64/bvFTD 1 1 1 . Perseverative FTA DLDH F/51/bvFTD 7 1 1 1 0 1 . 1 Semantic jargon Diffuse . F/68/bvFTD 3 . 1 1 1 1 . Semantic jargon RϾLTA ... F/56/SD 3 . 1 . 1 1 1 Semantic jargon LTA . F/65/SD 2 1 1 1 . 0 Tangential LϾRTA ... M/51/SD 6 1 1 1 1 . 1 Empty LTA . M/63/SD 1 1 1 1 . 1 . Semantic jargon, perseverative Diffuse . F/46/SD 4 1 1 1 1 . 1 Semantic jargon LϾRTA ... F/48/SD 3 . 1 1 1 . Garrulous, semantic jargon LFTA . F/55/SD 1 1 1 . 1 . 1 Semantic jargon LTA . M/48/SD 2 1 1 0 1 1 . 1 Dysarthric LϾRTFA ... F/47/bvFTD 4 1 1 1 1 1 . Garrulous LTPA . M/66/bvFTD 6 . 1 . 1 . Garrulous, stereotypy FTA . F/57/SD 3 1 1 0 . 1 Empty LTPA . F/72/bvFTD 5 . 1 1 0 1 . Garrulous, abusive LTA . M/67/SD 3 1 1 . 1 . Semantic jargon LTA . M/60/bvFTD 1 0 1 1 0 1 . Semantic jargon FTA LϾR ... M/72/SD 5 1 1 . 1 . Stereotypy FTA . M/58/bvFTD 5 . 1 . 1 . Semantic jargon LTPA . F/64/bvFTD 5 1 1 . 1 Semantic jargon RϾLFTA . M/69/SD 5 1 1 . 1 1 1 Semantic jargon LϾRTA ... F/62/bvFTD 6 1 1 1 1 1 . Garrulous, perseverative LϾRTA ... M/59/SD 4 1 1 . 1 . 1 Semantic jargon LϾRTA ... F/63/SD 4 1 1 . 1 . 1 Perseverative RFTA . F/54/SD 6 1 1 . 1 1 . 1 Garrulous LTA . F/58/SD 3 . 1 . 1 . 1 . Semantic jargon LTA . M/57/SD 5 1 1 . 1 1 . Garrulous LϾRTA ... M/64/SD 3 1 1 . 0 1 1 1 Perseverative LϾRTA ... F/66/SD 4 1 1 . 1 . Normal, fluent LϾRTA ... M/59/bvFTD 10 1 1 . 1 1 . Semantic jargon LϾRTFA ... M/52/bvFTD 9 . 1 0 1 1 1 1 Stereotypy, semantic jargon LTFA . F/41/bvFTD 2 1 1 1 1 1 . Perseverative, garrulous RTFA . Totald 28 34 13 15 28 9 18 Abbreviations: A, atrophy; ellipses, not tested for; bvFTD, behavioral frontotemporal dementia; DLDH, dementia lacking distinctive histology; F, frontal; FTLD-U, frontotemporal lobar degeneration with ubiquitin-positive inclusions; L, left; P, parietal; R, right; SD, semantic dementia; T, temporal. a 0 indicates that a symptom does not exist; 1, symptom exists. b Years from onset of illness to first clinical visit. c Indicates an obsession with painting or jigsaw puzzles. d For men, n=17; women, n=20; mean (SD) age, 58 (8.4) years; a total of 21 patients had SD; 16, bvFTD; the mean (SD) duration of illness was 4 (2.1) years. Some features of SD such as distinctive speech output 361 patients with FTD or Pick complex. Patients with SD had characteristics, impaired pragmatics (the study of the give- progressive loss of naming and comprehension, with preserved and-take and efficiency of communication), “What is...” syntax, phonology, fluency, and relatively preserved episodic 20 questioning of meaning, and behavioral abnormality are memory. They were followed up at yearly intervals, but only relatively unexplored. We aimed to study SD in a cog- the results of the first examination were used for the statistical nitive neurology clinic population of patients in an at- analyses in this study. Thirty-seven patients were considered to have probable SD (Table 1). This group had prominent com- tempt to delineate the syndrome from the behavioral pre- prehension and word-finding difficulty, either from the begin- sentation of patients with FTD (bvFTD), PNFA, and AD. ning of the illness or by the first time they were seen. Patients In addition to comparing neurocognitive features with with possible SD (n=11) were cases with atypical features and related conditions, we characterized the pragmatics of were not included in the statistical analysis. One patient had epi- speech and quantitated language, including fluency and sodic memory loss and 2 had confused close relatives as strang- the behavioral abnormality. In view of a recent sugges- ers (the Capgras delusion), which raised the suspicion of pos- tion associating a specific pathology with SD,19 the study sible AD.21 One patient had significant vascular disease and of clinical boundaries of SD is even more relevant. We another panic attacks. Five possible cases had predominant be- report autopsy confirmation in 6 of 48 patients. havioral disturbance and only incipient SD by the first clinic visit, and 1 had mixed nonfluent and SD features. Behavioral FTD was diagnosed clinically when a patient pre- METHODS sented with mainly behavioral or personality disturbance, ful- filling Neary and colleagues’ criteria,13 and no significant lan- The target population was 48 patients with SD who were diag- guage impairment was evident on the first examination.
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