Abstract
The pathological process and lesion topography in patients with the syndrome of progressive aphasia are heterogeneous and few necropsy examination cases have been investigated. This is a case report of a 53 year old right handed man with progressive anarthria and secondary Parkinsonism over a period of six years. Positron emission tomography (PET) showed a decreased cerebral blood flow and metabolism in the frontal cortex, which was more pronounced on the left. Neuropathology disclosed a spongiform vacuolation in layer II of the frontal cortex, mostly in the Broca area, and neuronal loss in the substantia nigra. This original case reinforces the view that there are different entities of the syndrome of progressive aphasia which can be identified on the basis of clinical, neuroimaging and anatomical data.
Full text
PDFImages in this article
Selected References
These references are in PubMed. This may not be the complete list of references from this article.
- Chawluk J. B., Mesulam M. M., Hurtig H., Kushner M., Weintraub S., Saykin A., Rubin N., Alavi A., Reivich M. Slowly progressive aphasia without generalized dementia: studies with positron emission tomography. Ann Neurol. 1986 Jan;19(1):68–74. doi: 10.1002/ana.410190112. [DOI] [PubMed] [Google Scholar]
- Delecluse F., Andersen A. R., Waldemar G., Thomsen A. M., Kjaer L., Lassen N. A., Postiglione A. Cerebral blood flow in progressive aphasia without dementia. Case report, using 133xenon inhalation, technetium 99m hexamethylpropyleneamine oxime and single photon emission computerized tomography. Brain. 1990 Oct;113(Pt 5):1395–1404. doi: 10.1093/brain/113.5.1395. [DOI] [PubMed] [Google Scholar]
- Frackowiak R. S., Lenzi G. L., Jones T., Heather J. D. Quantitative measurement of regional cerebral blood flow and oxygen metabolism in man using 15O and positron emission tomography: theory, procedure, and normal values. J Comput Assist Tomogr. 1980 Dec;4(6):727–736. doi: 10.1097/00004728-198012000-00001. [DOI] [PubMed] [Google Scholar]
- Gibb W. R., Luthert P. J., Marsden C. D. Corticobasal degeneration. Brain. 1989 Oct;112(Pt 5):1171–1192. doi: 10.1093/brain/112.5.1171. [DOI] [PubMed] [Google Scholar]
- Goulding P. J., Northen B., Snowden J. S., Macdermott N., Neary D. Progressive aphasia with right-sided extrapyramidal signs: another manifestation of localised cerebral atrophy. J Neurol Neurosurg Psychiatry. 1989 Jan;52(1):128–130. doi: 10.1136/jnnp.52.1.128. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Green J., Morris J. C., Sandson J., McKeel D. W., Jr, Miller J. W. Progressive aphasia: a precursor of global dementia? Neurology. 1990 Mar;40(3 Pt 1):423–429. doi: 10.1212/wnl.40.3_part_1.423. [DOI] [PubMed] [Google Scholar]
- Horoupian D. S., Thal L., Katzman R., Terry R. D., Davies P., Hirano A., DeTeresa R., Fuld P. A., Petito C., Blass J. Dementia and motor neuron disease: morphometric, biochemical, and Golgi studies. Ann Neurol. 1984 Sep;16(3):305–313. doi: 10.1002/ana.410160306. [DOI] [PubMed] [Google Scholar]
- Kempler D., Metter E. J., Riege W. H., Jackson C. A., Benson D. F., Hanson W. R. Slowly progressive aphasia: three cases with language, memory, CT and PET data. J Neurol Neurosurg Psychiatry. 1990 Nov;53(11):987–993. doi: 10.1136/jnnp.53.11.987. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Kirshner H. S., Tanridag O., Thurman L., Whetsell W. O., Jr Progressive aphasia without dementia: two cases with focal spongiform degeneration. Ann Neurol. 1987 Oct;22(4):527–532. doi: 10.1002/ana.410220413. [DOI] [PubMed] [Google Scholar]
- Knopman D. S., Mastri A. R., Frey W. H., 2nd, Sung J. H., Rustan T. Dementia lacking distinctive histologic features: a common non-Alzheimer degenerative dementia. Neurology. 1990 Feb;40(2):251–256. doi: 10.1212/wnl.40.2.251. [DOI] [PubMed] [Google Scholar]
- Lecours A. R. The "Pure Form" of the phonetic disintegration syndrome (pure anarthria); anatomo-clinical report of a historical case. Brain Lang. 1976 Jan;3(1):88–113. doi: 10.1016/0093-934x(76)90008-0. [DOI] [PubMed] [Google Scholar]
- Leenders K. L., Perani D., Lammertsma A. A., Heather J. D., Buckingham P., Healy M. J., Gibbs J. M., Wise R. J., Hatazawa J., Herold S. Cerebral blood flow, blood volume and oxygen utilization. Normal values and effect of age. Brain. 1990 Feb;113(Pt 1):27–47. doi: 10.1093/brain/113.1.27. [DOI] [PubMed] [Google Scholar]
- Mesulam M. M. Slowly progressive aphasia without generalized dementia. Ann Neurol. 1982 Jun;11(6):592–598. doi: 10.1002/ana.410110607. [DOI] [PubMed] [Google Scholar]
- Tyrrell P. J., Warrington E. K., Frackowiak R. S., Rossor M. N. Heterogeneity in progressive aphasia due to focal cortical atrophy. A clinical and PET study. Brain. 1990 Oct;113(Pt 5):1321–1336. doi: 10.1093/brain/113.5.1321. [DOI] [PubMed] [Google Scholar]