Neuropsychological and Neuroimaging Findings of Semantic Dementia

Transcription

1 Neuropsychological and Neuroimaging Findings of Semantic Dementia Jay C. Kwon, M.D., Sue J. Kang, M.S., Juhee Chin, M.A., Yeonwook Kang, Ph.D.*, Young Mi Lee, M.S., Hyanghee Kim, Ph.D., Jung Mi Park, M.D., Sang Eun Kim, M.D., Duk L. Na, M.D. Department of Neurology and Nuclear Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine Department of Psychology, Hallym University* Background : Semantic dementia (SD) is a temporal variant of frontotemporal lobar degeneration (FTLD), which is characterized by naming difficulty, decreased comprehension of words, prosopagnosia and object visual agnosia. We report clinical features, neuropsychological and neuroimaging findings of 4 patients with SD. Methods : Of 55 patients diagnosed as having FTLD between Jan 1995 and May 2001 at Samsung Medical Center, four patients fulfilled the diagnostic criteria of SD proposed by consensus on FTLD diagnostic criteria. We investigated their clinical features such as presenting symptoms and abnormal behaviors, neuropsychological and neuroimaging findings. Neuropsychological tests included the Seoul Neuropsychological Screening Battery, the Korean-version of Western Aphasia Battery and Hanja reading and writing. All patients underwent brain MRI and FDG-PET. Results : All of the patients showed naming difficulty as a presenting symptom. Language assessments showed severe naming and comprehension difficulties with preserved fluency and repetition, which were compatible with transcortical sensory aphasia. Whereas Hangul reading aloud and writing were intact, three patients were impaired at Hanja reading and writing. Other neuropsychological tests were remarkable for prosopagnosia, object visual agnosia and memory loss. Brain MRI showed asymmetric temporal atrophies, mainly left antero-inferior temporal cortices. FDG-PET also showed hypometabolism in bilateral anterior temporal lobes, more severe on the left. Conclusions : Our SD patients had characteristic neuropsychological and neuroimaging findings, which can be differentiated from other neurodegenerative diseases. We also found Hanja alexia and agraphia in SD patients, which has not been reported yet. J Korean Neurol Assoc 19(6):598~607, 2001 Key Words : Semantic dementia, Neuropsychological test, MRI, FDG-PET, Hanja, Frontotemporal dementia. Duk L. Na, M.D. 598 Copyright 2001 by the Korean Neurological Association

2 Table 1. Chinese letters used for Hanja reading and writing *,, *, *, * *, *, *, * *, *, *, *, *, *,, *, *,, *,, *,, *, *, *, *, *, *, *, *, *, *, *,, *, *, *, *, *,, *, *, *, *,, *, * * * Hanja stimuli used in Case 1 (41/79), Hanja stimuli used in Case 2 (65/79), Hanja stimuli used in Case 3 (18/79) J Korean Neurol Assoc / Volume 19 / November,

3 Table 2. Results of neuropsychological tests in the patients with semantic dementia. Neuropsychologic Tests Case 1 Case 2 Case 3 Case 4 (Oct97) (Aug00) (Jan95) (Dec96) (Feb00) Attention Digit span Forward/backward ND 5/4 ND 3/0 Language & related functions K-WAB* Fluency 7/10 10/10 9/10 9/10 Fluent Auditory comprehension 5.6/10 6.4/10 8.8/10 7.3/10 AB Repetition 7.8/10 7.2/10 8.2/10 7.2/10 8/10 K-BNT 6/60 2/60 3/10 3/60 0/60 (<1%ile) (<1%ile) (<1%ile) (<1%ile) Reading/Writing (Hangul) NL/NL NL/NL NL/NL NL/NL NL/AB Reading/Writing (Hanja) AB/AB AB/AB ND AB/AB ND Calculation NL NL NL AB Finger naming NL NL NA AB Right-left orientation NL NL AB AB Praxis: pantomiming gesture NA AB NL AB Visuospatial function Copy of Rey-CFT 34/36 II 34/36 II 34/36 II 26.5/36 II Memory Seoul Verbal Learning Test Free recall(1st, 2nd, 3rd trial) 0/2/4 1/3/5 <1%ile 0/0/1 20-minute delayed recall 0 0 <1%ile 0 Frontal/executive function Fist-edge-palm NL NL NL NL Luria loop NL NL AB NL Alternating square & triangle NL NL NL NL Word fluency (for one minute) Letter items (,, ) NA 0/0/0 AB NA Category items (animal, supermarket) NA 1/2 AB NA Stroop ND 112/0, 30/11 ND NA MMSE 20/30 22/30 20/30 5/30 CDR Barthel index 20/20 20/20 20/20 19/20 *Korean version of the Western Aphasia Battery, Korean version of Boston Naming Test, Naming subtest of K-WAB, Raw data were missing. Results from neuropsychological test reports were presented, Normal range, adjusted to age and education level. NL ; within normal limit, AB ; abnormal, NA ; Test was not possible because of comprehension deficit, ND ; Test was not done, %tile ; percentile, MMSE ; minimental state eraminction, CDR ; clinical dementia nating 600 J Korean Neurol Assoc / Volume 19 / November, 2001

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6 Figure 1. Coronal, sagittal or axial brain MR images of the 4 patients with semantic dementia. Note severe atrophy in left temporal lobe, especially anteroinferior portion. Compared to general atrophy of temporal lobe, hippocampus seems to be relatively spared in case 4 (arrow). J Korean Neurol Assoc / Volume 19 / November,

7 Figure 2. Coronal, axial and sagittal brain FDG-PET images of the 4 patients with semantic dementia. Glucose hypometabolism is seen in both anterior temporal lobe, with left side being much more prominent than the right side. 604 J Korean Neurol Assoc / Volume 19 / November, 2001

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