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Very Early Semantic Dementia with Progressive Temporal Lobe Atrophy an 8-Year Longitudinal Study

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Very Early Semantic Dementia with Progressive Temporal Lobe Atrophy an 8-Year Longitudinal Study OBSERVATION Very Early Semantic Dementia With Progressive Temporal Lobe Atrophy An 8-Year Longitudinal Study Kathrin Czarnecki, MD; Joseph R. Duffy, PhD; Carissa R. Nehl, PhD; Shelley A. Cross, MD; Jennifer R. Molano, MD; Clifford R. Jack Jr, MD; Maria M. Shiung, BA; Keith A. Josephs, MD, MST; Bradley F. Boeve, MD Background: Semantic dementia is a syndrome within mal but subtle left anterior temporal lobe atrophy was the spectrum of frontotemporal lobar degenerations char- present. During the follow-up period of 8 years, she de- acterized by fluent progressive aphasia (particularly ano- veloped profound anomia and loss of word meaning as- mia) and loss of word meaning. sociated with progressive left anterior temporal lobe at- rophy, consistent with semantic dementia. In more recent Objective: To report a unique case of very early seman- years, anterograde memory impairment and mild prosop- tic dementia with a slowly progressive course, allowing agnosia evolved in association with left hippocampal at- insights into the early natural history of this disorder. rophy and subtle atrophy in the homologous gyri of the right anterior temporal lobe. She remains functionally in- Design: Case report. dependent despite her current deficits. Setting: A tertiary care center. Conclusions: Early identification of patients who will develop semantic dementia is difficult and might be missed Patient: A 62-year-old woman who presented with with standard clinical, neuropsychological, and struc- “memory loss” complaints. tural neuroimaging evaluations. Recognition of this rela- Main Outcome Measures: Clinical course, neuropsy- tively rare syndrome is important for early diagnosis and chological data, and magnetic resonance imaging results. prognostication and particularly for therapeutic inter- ventions in the future. Results: The patient was first evaluated when the re- sults of standard neuropsychological measures were nor- Arch Neurol. 2008;65(12):1659-1663 N 1982, MESULAM1 FIRST DE- semantic dementia have ubiquitin- scribed a series of patients who positive, tau-negative inclusions as the cor- had aphasia without dementia; he relate of their disease.5 More recent analy- later named this syndrome pri- ses6 have shown that such inclusions mary progressive aphasia. Such immunostain with TAR-DNA binding pro- Ipatients can be distinguished on the ba- tein 43 (TDP-43). There also have been re- sis of fluency of speech, having either pro- ports of tauopathies and Alzheimer dis- gressive nonfluent or fluent aphasia. The ease in patients with semantic dementia,7 term semantic dementia refers to fluent although differentiating features might be Author Affiliations: aphasia with additional loss of word mean- found on neuropsychological testing and 2 8 Departments of Neurology ing. Clinically, patients have impair- brain imaging. The early natural course (Drs Czarnecki, Duffy, Cross, ment in language function and object rep- of semantic dementia is largely un- Josephs, and Boeve), Psychiatry resentation with preservation of episodic known, and many patients only come to and Psychology (Dr Nehl), and memory and absence of deficits in other medical attention when either profound Diagnostic Radiology (Dr Jack cognitive domains during the first 2 years anomia is already established or addi- and Ms Shiung), Mayo Clinic, of the illness.3 Imaging studies4 reveal dis- tional behavioral symptoms have devel- and Robert H. and Clarice tinct focal atrophy involving the infero- oped. We describe a unique patient who Smith and Abigail Van Buren Alzheimer’s Disease Research lateral temporal lobes, usually in an asym- presented to our clinic early in her illness Program of the Mayo metric distribution. There have been and developed slowly progressive seman- Foundation (Drs Jack, Josephs, advances in determining the underlying tic dementia with typical clinical and ra- and Boeve), Rochester, neuropathologic features of frontotempo- diologic features, allowing insights into the Minnesota. ral dementia, and most patients with early natural history of the disorder. (REPRINTED) ARCH NEUROL / VOL 65 (NO. 12), DEC 2008 WWW.ARCHNEUROL.COM 1659 ©2008 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/26/2021 METHODS plaints, her husband reported that she was substituting words, speaking in a circumlocutory manner, and mak- The patient was evaluated once yearly for 8 years by compre- ing semantic and frequent spelling errors. Formal speech hensive neurologic examination,9 neuropsychological testing, evaluation by an experienced speech pathologist (J.R.D.) and magnetic resonance imaging (MRI) of the brain as a par- demonstrated normal motor speech, verbal comprehen- ticipant in the Mayo Alzheimer Disease Research Center pro- sion and retention, verbal expression, and reading and writ- gram, a Mayo Foundation institutional review board– ing skills but a marked deficit in word retrieval (less than approved research program. the fifth percentile on the Boston Naming Test). Further- more, she often did not recognize missed target words when NEUROPSYCHOLOGICAL TESTING they were provided to her. During the following years, the patient had progression of anomia as documented by de- The patient underwent serial neuropsychological testing, in- cline in scores on the Boston Naming Test and category cluding assessment of global functioning (Folstein Mini- fluency. In contrast, scores remained average to above av- Mental State Examination),10 Kokmen Short Test of Mental erage for attention/executive functioning and visuospa- Status,11 learning and memory (Logical Memory and Visual Re- tial tasks. She performed near or above generally ac- productions subtests of the Wechsler Memory Scale–Revised, Per- cepted cutoff scores on screening measures (eg, Ն24 on cent Retention on the Auditory Verbal Learning Test), execu- the Folstein Mini-Mental State Examination and Ն29 on tive functioning (Trail Making Test parts A and B), Digit Symbol subtest of the Wechsler Adult Intelligence Scale–Revised, lan- the Kokmen Short Test of Mental Status). Anterograde guage functioning (Boston Naming Test, Controlled Oral Word memory performance was variable across follow-up with Association Test, category fluency), and visuospatial function- generally impaired visual reproductions of the Wechsler ing (Block Design subtest of the Wechsler Adult Intelligence Scale– Memory Scale–Revised and percent retention on the Rey Revised, Rey-Osterrieth Complex Figure Test, and Judgment of Auditory Verbal Learning Test performance for the last 3 Line Orientation). Mayo Older American Normative Studies to 5 years. In the most recent evaluation, all measures of norms were used to determine scaled scores for these tests, in anterograde memory were impaired (Figure 1). On serial which 10 represents the mean and 3 the SD.12-15 language examinations, she developed surface dyslexia, loss of word meaning, and difficulties in recognizing famous BRAIN IMAGING faces; she clearly met criteria for semantic dementia.3 Se- rial MRIs document progressive left anterior inferolateral The MRIs were performed using a Signa MRI scanner (General temporal lobe atrophy with relative sparing of the hippo- Electric Medical Systems, Waukesha, Wisconsin) at 1.5 Tesla, and campus until age 68 (Figure 2). On the most recent MRIs, images of the brain were obtained in sagittal (T1-weighted), axial there is evidence of right temporal lobe atrophy in a strik- (proton-density, T2-weighted, fluid-attenuated inversion recov- ingly similar pattern of topography compared with the left ery), and coronal (T1-weighted) planes. side. From a behavioral standpoint, the patient has be- come slightly more rigid mentally but remains indepen- REPORT OF A CASE dent in her simple and complex activities of daily living. A 62-year-old woman was referred to our behavioral neu- rology clinic with a 6-month history of “memory loss,” COMMENT specified as problems recalling names of people and ob- jects. She had mild difficulties in organizational skills and We report a case of very early semantic dementia with complex decision making, such as cooking a meal follow- longitudinal neuropsychological and radiologic follow- ing a detailed recipe, but was highly functional in her ac- up, allowing insights into the early natural course of this tivities of daily living and engaged in various social activi- syndrome. Our patient exemplifies the way that subjec- ties. Her medical history was significant for treated tive “memory loss” complaints precede objective evi- pernicious anemia and hypothyroidism. The patient had dence of cognitive impairment by standard testing meth- recently retired from teaching and led a health-conscious ods. Her course during 8 years was remarkably slow in lifestyle. She reported that several second-degree family its progression, compared with previously published data, members had been diagnosed as having late-onset Alzhei- which gave an estimated mean survival time of 8 years mer disease. The results of general neurologic examina- after diagnosis.16 On initial evaluation, both her neuro- tion and standard laboratory workup were unremark- psychological assessment and imaging findings were be- able. Her mental status examination revealed a score of lieved to be normal, although in hindsight subtle atro- 33/38 on the Kokmen Short Test of Mental Status, recall- phy of the left collateral sulcus and superior temporal gyrus ing 3 of 4 items correctly on delay. On detailed neuropsy- was present on MRI. The striking
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