Semantische Demenz: Klinisches Profil und kognitiv-neuropsychologische Bedeutung

Zusammenfassung

Die Semantische Demenz (SD) stellt eine seltene Variante der im Präsenium auftretenden primär degenerativen Erkrankungen der „frontotemporal lobar degeneration” (FTLD) mit anterior-temporaler Atrophie und einer selektiven Störung des semantischen Wissens dar. Das neuropsychologische Störungsprofil der SD ist durch eine flüssige Aphasie mit ausgeprägten Wortfindungs- und Sprachverständnisschwierigkeiten oder bzw. zusätzlich durch eine Störung der Objekterkennung gekennzeichnet. Im Krankheitsverlauf auftretende Verhaltensauffälligkeiten mit stereotypem Verhalten und Änderung der Esspräferenz werden als Korrelat zur Ausweitung der Pathologie auf frontale Strukturen interpretiert. Da die SD ein anderes Management als die Alzheimer-Demenz (AD) erfordert, jedoch häufig als diese missklassifiziert wird, werden die diagnostischen Konsensuskriterien von Neary u. Mitarb. [3] ausführlich dargestellt und das neuropsychologische Störungsprofil u. a. anhand einer Falldarstellung erörtert. Die sich aus der Dissoziation der gestörten Gedächtnissysteme bei SD (semantisch) und AD (episodisch) ergebenden Implikationen für Modelle der Langzeitspeicherung im Hinblick auf die Konnektivität im medialen Temporallappen werden diskutiert. SD kann außerdem einen Beitrag zum besseren Verständnis der Struktur und Organisation des semantischen Systems leisten.

Abstract

Semantic Dementia (SD) represents a rare variant of presenile primary degenerative diseases of “frontotemporal lobar degenerations” (FTLD). SD is characterized by anterior-temporal atrophy and a selective semantic deficit. Neuropsychological impairment either bears on fluent aphasia with marked naming and comprehension difficulties or on a disorder of object recognition or on both. Behavioural alterations such as stereotyped behaviour and change in food preference become prominent as the disease progresses and are interpreted as a correlative for the pathological spreading to frontal structures. Since SD is often misclassified as Alzheimer's disease (AD), but calls for a different management, diagnostic consensus criteria by Neary and colleagues [3] are depicted. The neuropsychological profile is described in detail and complemented by a case report. Implications from the dissociation of disturbed memory systems of SD (semantic) and AD (episodic) for models of long-term memory are discussed in terms of connectivity in the medial temporal lobe. SD can contribute to a better understanding of the structure and organisation of the semantic system.

Literatur

• 1 Warrington E K. The selective impairment of semantic memory.  The quarterly Journal of Experimental Psychology. 1975;  27 635-657
• 2 Snowden J S, Goulding P J, Neary D. Semantic dementia: a form of circumscribed atrophy.  Behavioural Neurology. 1989;  2 167-182
• 3 Neary D, Snowden J S, Gustafson L, Passant U, Stuss D, Black S, Freedman M, Kertesz A, Robert P H, Albert M, Boone K, Miller B L, Cummings J L, Benson D F. Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria.  Neurology. 1998;  51 1546-1554
• 4 Snowden J S, Neary D, Mann D MA. Fronto-temporal lobar degeneration: fronto-temporal dementia, progressive aphasia, semantic dementia. New York: Churchill Livingstone 1996
• 5 Hodges J R, Patterson K, Oxbury S, Funnell E. Semantic dementia. Progressive fluent aphasia with temporal lobe atrophy.  Brain. 1992;  115 1783-1806
• 6 Mesulam M-M. Primary progressive aphasia.  Ann Neurol. 2001;  49 425-432
• 7 Mesulam M-M. Slowly progressive aphasia without generalized dementia.  Ann Neurol. 1982;  11 592-598
• 8 Brun A, Englund B, Gustafson L. et al . Consensus statement. Clinical and neuropathological criteria for frontotemporal dementia.  Journal of Neurology, Neurosurgery and Psychiatry. 1994;  57 416-418
• 9 Levy M L, Miller B L, Cummings J L. Frontal and frontotemporal dementia. In: Growdon JH, Rossor MN (Hrsg). The Dementias. Boston: Butterworth-Heinemann 1998: 45-66
• 10 Garrard P, Hodges J R. Semantic dementia: clinical, radiological and pathological perspectives.  J Neurol. 2000;  247 409-422
• 11 Tanabe H, Nakagawa Y, Ikeda M, Hashimoto M, Yamada N, Kazui H, Nishikawa T, Okuda J. Selective loss of semantic memory for words. In: Ishikawa K, McGaugh JL, Sakata H (Hrsg). Brain Processes and Memory. Amsterdam: Elsevier Science B.V 1996: 141-152
• 12 Patterson K, Hodges J R. Disorders of semantic memory. In: Baddeley AD, Wilson BA, Watts FN (Hrsg). Handbook of memory disorders. Chichester: John Wiley & Sons Ltd 1995: 167-186
• 13 Kertesz A, Davidson W, Munoz D G. Clinical and pathological overlap between frontotemporal dementia, primary progressive aphasia and corticobasal degeneration: the pick complex.  Dement Geriatr Cogn Disord. 1999;  10 (suppl 1) 46-49
• 14 Kertesz A, Martinez-Lage P, Davidson W, Munoz D G. The corticobasal degeneration syndrome overlaps progressive aphasia and frontotemporal dementia.  Neurology. 2000;  55 1368-1375
• 15 Neary D. Frontotemporal degeneration, Pick disease, and corticobasal degeneration. One entity or 3? 3.  Arch Neurol. 1997;  54 1425-1427
• 16 Kertesz A. Frontotemporal degeneration, Pick disease, and corticobasal degeneration. One entity or 3? 1.  Arch Neurol. 1997;  54 1427-1429
• 17 Grossman M. Frontotemporal dementia: a review.  J Int Neuropsychol Soc. 2002;  8 566-583
• 18 Knopman D S, Mastri A R, Frey W H, Sung J H, Rustan T. Dementia lacking distinctive histologic features: a common non-Alzheimer degenerative dementia.  Neurology. 1990;  40 251-256
• 19 Hodges J R, Patterson K. Nonfluent progressive aphasia and semantic dementia: a comparative neuropsychological study.  J Int Neuropsychol Soc. 1996;  2 511-524
• 20 Tolnay M, Probst A. Frontotemporal lobar degeneration.  Gerontology. 2001;  47 1-8
• 21 Mummery C J, Patterson K, Price C J, Ashburner J, Frackowiak R SJ, Hodges J R. A voxel-based morphometry study of semantic dementia: relationship between temporal lobe atrophy and semantic memory.  Ann Neurol. 2000;  47 36-45
• 22 Rosen H J, Gorno Tempini M L, Goldman W P, Perry R J, Schuff N, Weiner M, Feiwell R, Kramer J H, Miller B L. Patterns of brain atrophy in frontotemporal dementia and semantic dementia.  Neurology. 2002;  58 198-208
• 23 Chan D, Fox N C, Scahill R I, Crum W R, Whitwell J L, Leschziner G, Rossor A M, Stevens J M, Cipolotti L, Rossor M N. Patterns of temporal lobe atrophy in semantic dementia and Alzheimer's disease.  Ann Neurol. 2001;  49 433-442
• 24 Galton C J, Patterson K, Graham K S, Lambon-Ralph M A, Williams G, Antoun N, Sahakian B J, Hodges J R. Differing patterns of temporal atrophy in Alzheimer's disease and semantic dementia.  Neurology. 2001;  57 216-225
• 25 Galton C J, Gomez-Anson B, Antoun N, Scheltens P, Patterson K, Graves M, Sahakian B J, Hodges J R. Temporal lobe rating scale: application to Alzheimer's disease and frontotemporal dementia.  Journal of Neurology, Neurosurgery, and Psychiatry. 2001;  70 165-173
• 26 Francis P T, Holmes C, Webster M T, Stratmann G C, Procter A W, Bowen D M. Preliminary neurochemical findings in non-Alzheimer dementia due to lobar atrophy.  Dementia. 1993;  4 172-177
• 27 Perry E, Goodchild R, Piggott M. Neurochemical pathology in degenerative dementias. In: Hodges JR (Hrsg). Early-Onset Dementia: a multidisciplinary approach. Oxford: University Press 2001: 190-226
• 28 Hodges J R. Frontotemporal dementia (Pick's disease): clinical features and assessment.  Neurology. 2001;  56 (suppl 4) 6-10
• 29 Graham K S, Lambon-Ralph M A, Hodges J R. Determining the impact of autobiographical experience on “meaning”: new insights from investigating sports-related vocabulary and knowledge in two cases with semantic dementia.  Cognitive Neuropsychology. 1997;  14 801-837
• 30 Hodges J R, Graham K S. Episodic memory: insights from semantic dementia.  Philos Trans R Soc Lond B Biol Sci. 2001;  356 1423-1434
• 31 Simons J S, Graham K S, Galton C J, Patterson K, Hodges J R. Semantic knowledge and episodic memory for faces in semantic dementia.  Neuropsychology. 2001;  15 101-114
• 32 McCarthy R A, Warrington E K. Repeating without semantics: surface dysphasia?.  Neurocase. 2001;  7 77-87
• 33 Graham N L, Patterson K, Hodges J R. The impact of semantic memory impairment on spelling: evidence from semantic dementia.  Neuropsychologia. 2000;  38 143-163
• 34 Macoir J, Bernier J. Is surface dysgraphia tied to semantic impairment? Evidence from a case of semantic dementia.  Brain Cogn. 2002;  48 452-457
• 35 Hodges J R, Patterson K, Ward R, Garrard P, Bak T, Perry R, Gregory C. The differentiation of semantic dementia and frontal lobe dementia (temporal and frontal variants of frontotemporal dementia) from early Alzheimer's disease: a comparative neuropsychological study.  Neuropsychology. 1999;  13 31-40
• 36 Hodges J R, Bozeat S, Lambon Ralph M A, Patterson K, Spatt J. The role of conceptual knowledge in object use evidence from semantic dementia.  Brain. 2000;  123 (Pt 9) 1913-1925
• 37 Hodges J R, Spatt J, Patterson K. “What” and “how”: evidence for the dissociation of object knowledge and mechanical problem-solving skills in the human brain.  Proc Natl Acad Sci USA. 1999;  96 9444-9448
• 38 Snowden J S, Griffiths H, Neary D. Semantic dementia: autobiographical contribution to preservation of meaning.  Cognitive Neuropsychology. 1994;  11 265-288
• 39 Bozeat S, Ralph M A, Patterson K, Hodges J R. The influence of personal familiarity and context on object use in semantic dementia.  Neurocase. 2002;  8 127-134
• 40 Hodges J R, Graham N, Patterson K. Charting the progression in semantic dementia: implications for the organisation of semantic memory.  Memory. 1995;  3 463-495
• 41 Ratnavalli E, Brayne C, Dawson K, Hodges J R. The prevalence of frontotemporal dementia.  Neurology. 2002;  58 1615-1621
• 42 Snowden J S, Neary D, Mann D MA. Frontotemporal dementia.  Br J Psychiatry. 2002;  180 140-143
• 43 Snowden J S. Semantic dysfunction in frontotemporal lobar degeneration.  Dement Geriatr Cogn Disord. 1999;  10 (suppl 1) 33-36
• 44 Mendez M F, Selwood A, Mastri A R, Frey W H. Pick's disease versus Alzheimer's disease: a comparison of clinical characteristics.  Neurology. 1993;  43 289-292
• 45 Snowden J S, Neary D. Progressive language dysfunction and lobar atrophy.  Dementia. 1993;  4 226-231
• 46 Neary D, Snowden J S, Mann D M. The clinical pathological correlates of lobar atrophy.  Dementia. 1993;  4 154-159
• 47 Edwards-Lee T, Miller B L, Benson D F, Cummings J L, Russel G L, Boone K, Mena I. The temporal variant of frontotemporal dementia.  Brain. 1997;  120 1027-1040
• 48 Swartz J R, Miller B L, Lesser I M, Darby A L. Frontotemporal dementia: treatment response to serotonin selective reuptake inhibitors.  J Clin Psychiatry. 1997;  58 212-216
• 49 Hodges J R, Miller B. Frontotemporal dementia (Pick's disease). In: Hodges JR (Hrsg). Early-Onset Dementia: a multidisciplinary approach. Oxford: University Press 2001: 285-303
• 50 Bozeat S, Gregory C A, Lambon-Ralph M A, Hodges J R. Which neuropsychiatric and behavioural features distinguish frontal and temporal variants of frontotemporal dementia from Alzheimer's disease?.  Journal of Neurology, Neurosurgery and Psychiatry. 2000;  69 178-186
• 51 Snowden J S, Bathgate D, Varma A, Blackshaw A, Gibbons Z C, Neary D. Distinct behavioural profiles in frontotemporal dementia and semantic dementia.  Journal of Neurology, Neurosurgery and Psychiatry. 2001;  70 323-332
• 52 Tulving E. Episodic and semantic memory. In: Tulving E, Donaldson W (Hrsg). Organization of Memory. New York and London: Academic Press 1972: 381-403
• 53 Howard D, Patterson K. The pyramids and palm trees test. Bury St Edmunds: Thames Valley Test Company 1992
• 54 Bozeat S, Lambon-Ralph M A, Patterson K, Garrard P, Hodges J R. Non-verbal semantic impairment in semantic dementia.  Neuropsychologia. 2000;  38 1207-1215
• 55 Gorno Tempini M L, Price C J, Josephs O, Vandenberghe R, Cappa S F, Kapur N, Frackowiak R SJ, Tempini M L. The neural systems sustaining face and proper-name processing.  Brain. 1998;  121 2103-2118
• 56 Wiggs C L, Weisberg J, Martin A. Neural correlates of semantic and episodic memory retrieval.  Neuropsychologia. 1999;  37 103-118
• 57 Mummery C J, Patterson K, Wise R JS, Vandenbergh R, Price C J, Hodges J R. Disrupted temporal lobe connections in semantic dementia.  Brain. 1999;  122 61-73
• 58 Warrington E K, Shallice T. Category specific semantic impairments.  Brain. 1984;  107 829-854
• 59 Mummery C J, Patterson K, Hodges J R, Price C J. Functional neuroanatomy of the semantic system: divisible by what?.  J Cogn Neurosci. 1998;  10 766-777
• 60 Caramazza A, Shelton J R. Domain-specific knowledge systems in the brain: the animate-inanimate distinction.  J Cogn Neurosci. 1998;  10 1-34
• 61 Luckhurst L, Lloyd-Jones T J. A selective deficit for living things after temporal lobectomy for relief of epileptic seizures.  Brain Lang. 2001;  79 266-296
• 62 Devlin J T, Russell R P, Davis M H, Price C J, Moss H E, Fadili M J, Tyler L K. Is there an anatomical basis for category-specificity? Semantic memory studies in PET and fMRI.  Neuropsychologia. 2002;  40 54-75
• 63 Lambon-Ralph M A, Graham K S, Ellis A W, Hodges J R. Naming in semantic dementia - what matters?.  Neuropsychologia. 1998;  36 775-784
• 64 Hodges J R, Graham K S. A reversal of the temporal gradient for famous person knowledge in semantic dementia: implications for the neural organisation of long-term memory.  Neuropsychologia. 1998;  36 803-825
• 65 Graham K S, Hodges J R. Differentiating the roles of the hippocampal complex and the neocortex in long-term memory storage: evidence from the study of semantic dementia and Alzheimer's disease.  Neuropsychology. 1997;  11 77-89
• 66 Snowden J S, Griffiths H L, Neary D. Semantic-episodic memory interactions in semantic dementia: implications for retrograde memory function.  Cognitive Neuropsychology. 1996;  13 1101-1139
• 67 Westmacott R, Leach L, Freedman M, Moscovitch M. Different patterns of autobiographical memory loss in semantic dementia and medial temporal lobe amnesia: a challenge to consolidation theory.  Neurocase. 2001;  7 37-55
• 68 Murre J M, Graham K S, Hodges J R. Semantic dementia: relevance to connectionist models of long-term memory.  Brain. 2001;  124 647-675
• 69 Murre J M. Implicit and explicit memory in amnesia: some explanations and predictions by the TraceLink model.  Memory. 1997;  5 213-232
• 70 Collins A M, Quillian M R. Retrieval time from semantic memory.  Journal of Verbal Learning and Verbal Behavior. 1969;  8 240-247
• 71 Collins A M, Loftus E F. A spreading-activation theory of semantic processing.  Psychol Rev. 1975;  82 407-428
• 72 Anderson J R. Retrieval of information from long-term memory.  Science. 1983;  220 25-30
• 73 McClelland J L, Rumelhart D E. Distributed memory and the representation of general and specific information.  J Exp Psychol. 1985;  114 159-188
• 74 Hinton G E, Shallice T. Lesioning an attractor network: investigations of acquired dyslexia.  Psychol Rev. 1991;  98 74-95
• 75 Hodges J R. Memory in the dementias. In: Tulving E, Craik FIM (Hrsg). The Oxford handbook of memory. London: Oxford University Press 2000: 441-459
• 76 McClelland J L, McNaughton B L, O'Reilly R C. Why there are complementary learning systems in the hippocampus and neocortex: insights from the successes and failures of connectionist models of learning and memory.  Psychol Rev. 1995;  102 419-457
• 77 Garrard P, Perry R, Hodges J R. Disorders of semantic memory.  Journal of Neurology, Neurosurgery, and Psychiatry. 1997;  62 431-435
• 78 Petersen R C, Smith G E, Ivnik R J, Kokmen E, Tangalos E G. Memory function in very early Alzheimer's disease.  Neurology. 1994;  44 867-872
• 79 Welsh K, Butters N, Hughes J, Mohs R, Heyman A. Detection of abnormal memory decline in mild cases of Alzheimer's disease using CERAD neuropsychological measures.  Arch Neurol. 1991;  48 278-281
• 80 Welsh K, Butters N, Hughes J P, Mohs R C, Heyman A. Detection and staging of dementia in Alzheimer's disease.  Arch Neurol. 1992;  49 448-452
• 81 Morris J C, Edland S, Clark C, Galasko D, Koss E, Mohs R, Belle G van, Fillenbaum G, Heyman A. The consortium to establish a registry for Alzheimer's disease (CERAD). Part IV. Rates of cognitive change in the longitudinal assessment of probable Alzheimer's disease.  Neurology. 1993;  43 2457-2465
• 82 Hodges J R, Salmon D P, Butters N. Differential impairment of semantic and episodic memory in Alzheimer's and Huntington's diseases: a controlled prospective study.  J Neurol Neurosurg Psychiatry. 1990;  53 1089-1095
• 83 Perry R J, Watson P, Hodges J R. The nature and staging of attention dysfunction in early (minimal and mild) Alzheimer's disease: relationship to episodic and semantic memory impairment.  Neuropsychologia. 2000;  38 252-271
• 84 Perry R J, Hodges J R. Attention and executive deficits in Alzheimer's disease. A critical review.  Brain. 1999;  122 383-404
• 85 Perry R J, Hodges J R. Differentiating frontal and temporal variant frontotemporal dementia from Alzheimer's disease.  Neurology. 2000;  54 2277-2284
• 86 Hodges J R, Salmon D P, Butters N. Semantic memory impairment in Alzheimer's disease: failure of access or degraded knowledge?.  Neuropsychologia. 1992;  30 301-314
• 87 Hodges J R, Patterson K. Is semantic memory consistently impaired early in the course of Alzheimer's disease? Neuroanatomical and diagnostic implications.  Neuropsychologia. 1995;  33 441-459
• 88 Ikeda M, Brown J, Holland A J, Fukuhara R, Hodges J R. Changes in appetite, food preference, and eating habits in frontotemporal dementia and Alzheimer's disease.  J Neurol Neurosurg Psychiatry. 2002;  73 371-376
• 89 Kertesz A, Munoz D. Clinical and pathological characteristics of primary progressive aphasia and frontal dementia.  J Neural Transm. 1996;  (Suppl) 47 133-141
• 90 Kertesz A, Hudson L, Mackenzie I R, Munoz D G. The pathology and nosology of primary progressive aphasia.  Neurology. 1994;  44 2065-2072
• 91 Huber W, Poeck K, Weniger D, Willmes K. Aachener Aphasie Test (AAT). Göttingen: Verlag für Psychologie Dr. C. J. Hogrefe 1983
• 92 Zimmermann P, Fimm B. Testbatterie zur Aufmerksamkeitsprüfung (TAP) Version 1.02c. Psytest 1994
• 93 Lee G P, Strauss E, Loring D W, McCloskey L, Haworth J M. Sensitivity of figural fluency on the Five-Point Test to focal neurological disease.  The Clinical Neuropsychologist. 1997;  11 59-68
• 94 Glindemann R, Klintwort D, Ziegler W, Goldenberg G. Bogenhausener Semantik-Untersuchung BOSU. München: Urban & Fischer 2002
• 95 Warrington E K. Recognition memory test. Windsor, UK: NFER-Nelson 1984
• 96 Folstein M F, Folstein S E, McHugh P R. “Mini-mental state”: a practical method for grading the cognitive state of patients for the clinician.  Journal of psychiatric research. 1975;  12 189-198
• 97 Taylor L B. Localization of cerebral lesions by psychological testing.  Clinical Neurosurgery. 1969;  16 269-287
• 98 Pick A. Über die Beziehungen der senilen Hirnatrophie zur Aphasie.  Prager Medizinische Wochenschrift. 1892;  17 165-167
• 99 Pick A. Zur Symptomatologie der linksseitigen Schläfenlappenatrophie.  Monatsschrift für Psychiatrie und Neurologie. 1904;  16 378-388
• 100 Rosenfeld M. Die partielle Großhirnatrophie.  Journal für Psychologie und Neurologie. 1909;  14 115-130
• 101 Poeck K, Luzzatti C. Slowly progressive aphasia in three patients. The problem of accompanying neuropsychological deficit.  Brain. 1988;  111 151-168
• 102 Meins W. Die Semantische Demenz - eine Variante der Frontotemporalen Demenz.  Fortschr Neurol Psychiatr. 2003;  71 1-3
• 103 Greck J, Lautenschlager N, Kurz A. Klinische Aspekte der frontotemporalen Demenz.  Fortschr Neurol Psychiatr. 2000;  68 447-457
• 104 Benke T, Donnemiller E. Die Diagnose der Frontotemporalen Demenz.  Fortschr Neurol Psychiatr. 2002;  70 243-251
• 105 Spiessl H, Marienhagen J, Stohr H, Klein H E. Aphasie und Demenz.  Psychiatr Prax. 2001;  28 246-248
• 106 Watson I J, Arfken C L, Birge S J. Clock completion: an objective screening test for dementia.  Journal of the American Geriatrics Society. 1993;  41 1235-1240
• 107 Thalmann B, Monsch A U, Schneitter M, Ermini-Fünfschilling D, Spiegel R, Stähelin H B. Die CERAD Neuropsychologische Testbatterie. Ein gemeinsames minimales Instrumentarium zur Demenzabklärung. http://www.memoryclinic.ch/tests/tests.php. CH-4031 Basel, Hebelstraße 10. Memory Clinic, Geriatrische Universitätsklinik 1998