Skip to main content
SpringerLink
Log in

Primär progressive Aphasie

Primary progressive aphasia

  • Übersichten
  • Published:
Der Nervenarzt Aims and scope Submit manuscript

Zusammenfassung

Das seltene Krankheitsbild der primär progressiven Aphasie ist durch eine progrediente Sprachstörung gekennzeichnet, bei der recht häufig Wortfindungsstörungen das initiale Symptom darstellen. Die Sprachstörung bleibt über mehrere Jahre das führende und oft auch einzige Symptom. Andere kognitive Funktionen wie Gedächtnis, Orientierung, Urteilsvermögen oder visuell-räumliche Fähigkeiten sind über längere Zeit intakt und können gelegentlich nach einer Krankheitsdauer von 5–7 Jahren beeinträchtigt sein. In der morphologischen Bildgebung ist meist eine links temporale Atrophie zu sehen, sie kann aber auch die frontale Region betreffen oder beidseits vorhanden sein. Die funktionelle Bildgebung weist strukturelle Auffälligkeiten links temporal oder frontotemporal nach. Neuropathologische Untersuchungen zeigen in den Regionen der Atrophie einen neuronalen Zellverlust und eine Gliose, die gelegentlich von spongiformen Veränderungen begleitet sind. In seltenen Fällen finden sich τ-positive Neurone oder Plaques und Fibrillen. Eine spezifische Therapie ist nicht bekannt, symptomorientierte logopädische Behandlung scheint eine gewisse Stabilisierung zu bewirken.

Summary

Primary progressive aphasia is a rare disease characterised by slow deterioration of language, which remains the leading symptom whereas other cognitive functions such as memory, orientation, judgement, and visual-spatial skills are relatively spared and mostly not affected until 5 to 7 years after disease onset. Structural imaging displays atrophy of the left temporal region which can also affect the frontal region or both hemispheres. Reduced neuronal activity in the left temporal lobe can be seen using functional imaging even before structural anomalies are detected. Neuropathological examination reveals neuronal cell loss and gliosis in the regions of atrophy which are sometimes accompanied by spongiform changes. T-positive neurons or plaques and neurofibrillary tangles are present in rare cases. A specific therapy is not known. Logopaedic therapy focussed on the main symptoms seems to stabilise linguistic abilities.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Abb. 1a,b

Literatur

  1. Abe K, Ukita H, Yanagihara (1997) Imaging in primary progressive aphasia. Neuroradiology 39:556–559

    Article  CAS  PubMed  Google Scholar 

  2. Alberca R, Montes E, Russell E et al. (2004) Left hemicranial hypoplasia in 2 patients with primary progressive aphasia. Arch Neurol 61:265–268

    Article  PubMed  Google Scholar 

  3. Catani M, Piccirilli M, Cherubini A et al. (2003) Axonal injury within language network in primary progressive aphasia. Ann Neurol 53:242–247

    Article  PubMed  Google Scholar 

  4. Chawluk JB, Mesulam MM, Hurtig H et al. (1986) Slowly progressive aphasia without generalized dementia: studies with positron emission tomography. Ann Neurol 19:68–74

    CAS  PubMed  Google Scholar 

  5. Cummings JL, Benson JF, Hill MA et al. (1985) Aphasia in dementia of the Alzheimer’s type. Neurology 35:394–397

    CAS  PubMed  Google Scholar 

  6. Drzezga A, Grimmer T, Siebner H et al. (2002) Prominent hypometabolism of the right temporoparietal and frontal cortex in two left-handed patients with primary progressive aphasia. J Neurol 249:1263–1267

    Article  PubMed  Google Scholar 

  7. Galton CJ, Patterson K, Xuereb JH et al. (2000) Atypical and typical presentation of Alzheimer’s disease: a clinical, neuropsychological, neuroimaging and pathological study of 13 cases. Brain 123:484–498

    Article  PubMed  Google Scholar 

  8. Graff-Radford NR, Damasio AR, Hyman et al. (1990) Progressive aphasia in a patient with Pick’s disease: a neuropsychological, radiologic, and anatomic study. Neurology 40:620–626

    CAS  PubMed  Google Scholar 

  9. Heath PD, Kennedy P, Kapur N (1983) Slowly progressive aphasia without generalized dementia. Ann Neurol 13:687–688

    CAS  Google Scholar 

  10. Holland AL, McBurney DH, Moossy J et al. (1985) The dissolution of language in Pick’s disease with neurofibrillary tangles: a case study. Brain Lang 24:36–58

    CAS  PubMed  Google Scholar 

  11. Karbe H, Kersetz A, Polk M (1993) Profiles of language impairment in primary progressive aphasia. Arch Neurol 50:193-201

    CAS  PubMed  Google Scholar 

  12. Kempler D, Metter EJ, Riege WH et al. (1990) Slowly progressive aphasia: three cases with memory, language, CT and PET data. J Neurol Neurosurg Psychiatry 53:987–993

    CAS  PubMed  Google Scholar 

  13. Kertesz A (2003) Pick complex: an integrative approach to frontotemporal dementia. Neurologist 9:311–317

    Article  PubMed  Google Scholar 

  14. Kertesz A, Hudson L, Mackenzie IR et al. (1994) The pathology and nosology of primary progressive aphasia. Neurology 44:2065–2072

    CAS  PubMed  Google Scholar 

  15. Kertesz A, Martinez-Lage P, Davidson W et al. (2000) The corticobasal degeneration syndrome overlaps progressive aphasia and frontotemporal dementia. Neurology 55:1368–1375

    CAS  PubMed  Google Scholar 

  16. Kinoshita A, Tomimoto H, Tachibana N et al. (1996) A case of primary progressive aphasia with abnormally ubiquinated neurites in the cerebral cortex. Acta Neuropathol 92:520–524

    Article  CAS  PubMed  Google Scholar 

  17. Kirshner HS, Tanridag O, Thurman L et al. (1987) Progressive aphasia without dementia: two cases with focal spongiform degeneration. Ann Neurol 22:527–532

    CAS  PubMed  Google Scholar 

  18. Louis M, Espesser R, Rey V et al. (2001) Intensive training of phonological skills in progressive aphasia: a model of brain plasticity in neurodegenerative disease. Brain Cogn 46:197–201

    CAS  PubMed  Google Scholar 

  19. Mendez MF, Clark DG, Shapira JS et al. (2003) Speech and language in progressive nonfluent aphasia compared with early Alzheimer’s disease. Neurology 61:1108–1113

    PubMed  Google Scholar 

  20. Mesulam M-M (1982) Slowly progressive aphasia without generalized dementia. Ann Neurol 11:592–598

    CAS  PubMed  Google Scholar 

  21. Mesulam M-M (1987) Primary progressive aphasia—differentiation from Alzheimer’s disease. Ann Neurol 22:533–534

    CAS  PubMed  Google Scholar 

  22. Mesulam M-M (2001) Primary progressive aphasia. Ann Neurol 49:425–432

    Article  CAS  PubMed  Google Scholar 

  23. Mesulam MM (2003) Primary progressive aphasia—a language-based dementia. N Engl J Med 349:1535–1542

    Article  CAS  PubMed  Google Scholar 

  24. Mesulam MM, Grossman M, Hillis A et al. (2003) The core and halo of primary progressive aphasia and semantic dementia. Ann Neurol 54(Suppl 5):S11–S14

    Google Scholar 

  25. Nestor PJ, Graham NL, Fryer TD et al. (2003) Progressive non-fluent aphasia is associated with hypometabolism centred on the left anterior insula. Brain 126:2406–2418

    Article  PubMed  Google Scholar 

  26. Pick A (1892) Über die Beziehung der senilen Hirnatrophie zur Aphasie. Prager Med Wochenschr 17:165–167

    Google Scholar 

  27. Pick A (1905) Zur Symptomatologie der linksseitigen Schläfenlappenatrophie. Monatsschr Psychiatr Neurol 16:378–388

    Google Scholar 

  28. Poeck K, Luzzatti C (1988) Slowly progressive aphasia in three patients. The problem of accompanying neuropsychological deficit. Brain 111:151–168

    PubMed  Google Scholar 

  29. Pogacar S, Williams RS (1984) Alzheimer’s disease presenting as slowly progressive aphasia. RI Med J 67:181–185

    CAS  Google Scholar 

  30. Rossor MN, Revesz T, Lantos PL et al. (2000) Semantic dementia with ubiquitin-positive τ-negative inclusion bodies. Brain 123:267–276

    Article  PubMed  Google Scholar 

  31. Schwarz M, DeBleser R, Poeck K et al. (1998) A case of primary progressive aphasia. A 14-year follow-up study with neuropathological findings. Brain 121:115–126

    Article  PubMed  Google Scholar 

  32. Sobrido M-J, Abu-Khalil A, Weintraub S et al. (2003) Possible association of the τ H1/H1 genotype with primary progressive aphasia. Neurology 60:862–864

    PubMed  Google Scholar 

  33. Sonty SP, Mesulam MM, Thompson CK et al. (2003) Primary progressive aphasia: PPA and the language network. Ann Neurol 53:35–49

    Article  PubMed  Google Scholar 

  34. Soriani-Lefevre M-H, Hannequin D, Bakchine S et al. (2003) Evidence of bilateral temporal lobe involvement in primary progressive aphasia: a SPECT study. J Nucl Med 44:1013–1022

    PubMed  Google Scholar 

  35. The Lund and Manchester Groups (1994) Clinical and neuropathological criteria for frontotemporal dementia. J Neurol Neurosurg Psychiatry 57:416–418

    PubMed  Google Scholar 

  36. Thompson CK, Ballard KJ, Tait ME et al. (1997) Patterns of language decline in non-fluent primary progressive aphasia. Aphasiology 11:297–321

    Google Scholar 

  37. Turner RS, Kenyon LC, Trojanowski JQ et al. (1996) Clinical, neuroimaging, and pathologic features of progressive nonfluent aphasia. Ann Neurol 39:166–173

    CAS  PubMed  Google Scholar 

  38. Weintraub S, Rubin NP, Mesulam M-M (1990) Primary progressive aphasia. Longitudinal course, neuropsychological profile, and language features. Arch Neurol 47:1329–1335

    CAS  PubMed  Google Scholar 

  39. Westbury C, Bub D (1997) Primary progressive aphasia: a review of 112 cases. Brain Lang 60:381–406

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Neurologische Klinik UK Aachen

    F. Block & F. Kastrau

  2. Neurologische Klinik UK Aachen, Pauwelsstraße 30, 52057, Aachen, Deutschland

    F. Block

Authors
  1. F. Block
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. F. Kastrau
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to F. Block.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Block, F., Kastrau, F. Primär progressive Aphasie. Nervenarzt 75, 1167–1171 (2004). https://doi.org/10.1007/s00115-004-1770-z

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00115-004-1770-z

Schlüsselwörter

Keywords

Search

Navigation