Primary progressive aphasia Export

@article{citeulike:4078337, abstract = {Primary progressive aphasia (PPA) is a focal dementia characterized by an isolated and gradual dissolution of language function. The disease starts with word-finding disturbances (anomia) and frequently proceeds to impair the grammatical structure (syntax) and comprehension (semantics) of language. The speech output in PPA can be fluent or nonfluent. Memory, visual processing, and personality remain relatively well-preserved until the advanced stages and help to distiguish PPA from frontal lobe dementia and the typical forms of Alzheimer's disease. The term "semantic dementia" was originally introduced to designate a different group of patients with a combination of verbal and visual processing deficits. In practice, however, this diagnosis is also being used in a variant sense to denote a subtype of PPA with fluent speech and impaired comprehension, even in the absence of visual processing deficits. Insofar as the diagnosis of semantic dementia can have these two different meanings, it is important to specify whether it is being used in the original sense or to denote a subtype of PPA. Structural and physiological neuroimaging confirms the selective predilection of PPA for the left hemisphere, especially for its language-related cortices. A few patients with PPA display the neuropathological markers of Alzheimer's disease, but in an unusual distribution. The majority of the autopsies in PPA have shown either Pick's disease or lobar atrophy without distinctive histopathology. The suggestion has been made that PPA and frontal lobe dementia constitute phenotypical variations of a unitary disease process within the "Pick-lobar atrophy" spectrum. Recent advances in chromosome 17-linked dementias justify a rigorous search for tau polymorphisms and tauopathy in sporadic PPA. An informed approach to this syndrome will increase the effectiveness with which clinicians can address the unique challenges associated with the diagnosis and care of PPA.}, address = {Cognitive Neurology and Alzheimer's Disease Center, Northwestern Cognitive Brain Mapping Group, Northwestern University, Chicago, IL, United States}, author = {Mesulam, M. M.}, citeulike-article-id = {4078337}, citeulike-linkout-0 = {http://www.scopus.com/scopus/inward/record.url?eid=2-s2.0-0035071053&partnerID=40}, comment = {References: Mesulam, M.-M., Weintraub, S., Spectrum of primary progressive aphasia (1992), pp. 583-609. , Rossor MN, editor. Unusual dementias. London: Bailli\`{e}re TindallWeintraub, S., Rubin, N.P., Mesulam, M.M., Primary progressive aphasia. Longitudinal course, neuropsychological profile, and language features (1990) Arch Neurol, 47, pp. 1329-1335; Weintraub, S., Mesulam, M.-M., Four neuropsychological profiles in dementia (1993) Handbook of neuropsychology, 8, pp. 253-281. , Boller F, Grafman J, editors. Amsterdam: Elsevier; Weintraub, S., Mesulam, M.-M., From neuronal networks to dementia: Four clinical profiles (1996), pp. 75-97. , F\^{o}ret F, Christen Y, Boller F, editors. La demence: pourquoi? Paris: Foundation Nationale de GerontologieBroussolle, E., Bakchine, S., Tommasi, M., Slowly progressive anarthria with late anterior opercular syndrome: A variant form of frontal cortical atrophy syndromes (1996) J Neurol Sci, 144, pp. 44-58; Mesulam, M.-M., Johnson, N., Grujic, Z., Weintraub, S., Apolipoprotein E genotypes in primary progressive aphasia (1997) Neurology, 49, pp. 51-55; Rosenfeld, M., Die partielle grosshirnatrophie (1909) J Psychol Neurol, 14, pp. 115-130; Pick, A., Ueber die beziehungen der senilen hirnatrophie zur aphasie (1892) Prager Med Wochenschr, 17, pp. 165-167; Pick, A., Zur symptomatologie der linksseitigen schlaffenlappenatrophie (1904) Monatsschr Psychiatr Neurol, 16, pp. 378-388; Franceschi, F., Gliosi perivasculare in un caso de demenza afasica (1908) Ann Neurol, 26, pp. 281-290; ?rieux, P., Sur un cas de surdit\'{e} verbale pure (1893) Rev Med, 13, pp. 733-750; Dejerine, J., ?rieux, P., Un cas de surdit\'{e} verbale pure termin\'{e}e par aphasie sensorielle, suivie d'autopsie (1897) CR Acad Sci, 49, pp. 1074-1077; Mesulam, M.M., Primary progressive aphasia - Differentiation from Alzheimer's disease (1987) Ann Neurol, 22, pp. 533-534; Mesulam, M.M., Slowly progressive aphasia without generalized dementia (1982) Ann Neurol, 11, pp. 592-598; Westbury, C., Bub, D., Primary progressive aphasia: A review of 112 cases (1997) Brain Lang, 60, pp. 381-406; Habib, M., Pelletier, J., Khalil, R., Aphasie progressive primaire (syndrome de Mesulam) (1993) Presse M\'{e}d, 22, pp. 757-764; Mesulam, M.-M., From sensation to cognition (1998) Brain, 121, pp. 1013-1052; Mesulam, M.-M., Aphasias and other focal cerebral disoders (1998), pp. 134-142. , Fauci AS, Braunwald E, Isselbacher KJ, editors. In Harrison's principles of internal medicine, 14th ed. New York: McGraw HillThompson, C.K., Ballard, K.J., Tait, M.E., Patterns of language decline in non-fluent primary progressive aphasia (1997) Aphasiology, 11, pp. 297-321; Snowden, J.S., Neary, D., Mann, D.M.A., Progressive language disorder due to lobar atrophy (1992) Ann Neurol, 31, pp. 174-183; Mehler, M.F., Mixed transcortical aphasia in nonfamilial dysphasic dementia (1988) Cortex, 24, pp. 545-554; Otsuki, M., Soma, Y., Sato, M., Slowly progressive pure word deafness (1998) Eur Neurol, 39, pp. 135-140; Price, B.H., Gurvit, H., Weintraub, S., Neuropsychological patterns and language deficits in 20 consecutive cases of autopsy-confirmed Alzheimer's disease (1993) Arch Neurol, 50, pp. 931-937; B\'{e}land, R., Ska, B., Interaction between verbal and gestural language in progressive aphasia: A longitudinal case study (1992) Brain Lang, 43, pp. 355-385; Cappa, S.F., DeFanti, C.A., DeMarco, R., Progressive dysphasic dementia with bucco-facial apraxia: A case report (1993) Behav Neurol, 6, pp. 159-163; McCarthy, R.A., Warrington, E.K., Evidence for modality-specific meaning systems in the brain (1988) Nature, 334, pp. 428-430; Basso, A., Capitani, E., Laiacona, M., Progressive language impairment without dementia: A case with isolated category specific semantic defect (1988) J Neurol Neurosurg Psychiatr, 51, pp. 1201-1207; Neary, D., Snowden, J.S., Gustafson, L., Frontotemporal lobar degeneration. A consensus on clinical diagnostic criteria (1998) Neurology, 51, pp. 1546-1554; Rossor, M.N., Revesz, T., Lantos, P.L., Warrington, E.K., Semantic dementia with ubiquitin-positive tau-negative inclusion bodies (2000) Brain, 123, pp. 267-276; Hodges, J.R., Patterson, K., Oxbury, S., Funnell, E., Semantic dementia. Progressive fluent aphasia with temporal lobe atrophy (1992) Brain, 115, pp. 1783-1806; Snowden, J.S., Goulding, P.J., Neary, D., Semantic dementia: A form of circumscribed atrophy (1989) Behav Neurol, 2, pp. 167-182; Tyrrell, P.J., Warrington, E.K., Frackowiak, R.S.J., Rossor, M.N., Progressive degeneration of the right temporal lobe studied with positron emission tomography (1990) J Neurol Neurosurg Psychiatr, 53, pp. 1046-1050; DeRenzi, E., Slowly progressive visual agnosia or apraxia without dementia (1986) Cortex, 22, pp. 171-180; Freedman, L., Costa, L., Pure alexia and right hemiachromatopsia in posterior dementia (1992) J Neurol Neurosurg Psychiatr, 55, pp. 500-502; Benson, D.F., Davis, J.R., Snyder, B.D., Posterior cortical atrophy (1988) Arch Neurol, 45, pp. 789-793; Peavy, G.M., Herzog, A.G., Rubin, N.P., Mesulam, M.M., Neuropsychological aspects of dementia of motor neuron disease: A report of two cases (1992) Neurology, 42, pp. 1004-1008; Rakowicz, W., Hodges, J.R., Dementia and aphasia in motor neuron disease: An underrecognized association? (1998) J Neurol Neurosurg Psychiatr, 65, pp. 881-889; Strong, M.J., Grace, G.M., Orange, J.B., A prospective study of cognitive impairment in ALS (1999) Neurology, 53, pp. 1665-1670; Caselli, R.J., Windebank, A.J., Petersen, R.J., Rapidly progressive aphasic dementia and motor neuron disease (1993) Ann Neurol, 33, pp. 200-207; Frattali, C.M., Grafman, J., Patronas, N., Language disturbances in corticobasal degeneration (2000) Neurology, 54, pp. 990-992; Foster, N.L., Frontotemporal dementia with parkinsonism linked to chromosome 17 (FTDP-17): A clinician's guide (1999) Neurologist, 5, pp. 213-221; Snowden, J.S., Neary, D., Progressive language dysfunction and lobar atrophy (1993) Dementia, 4, pp. 226-231; Lendon, C.L., Lynch, T., Norten, J., Hereditary dysphasia disinhibition dementia. A frontotemporal dementia linked to 17q 21-22 (1998) Neurology, 50, pp. 1546-1555; Basun, H., Almkvist, O., Axelman, K., Clinical characteristics of a chromosome 17-linked rapidly progressive familial frototemporal dementia (1997) Arch Neurol, 54, pp. 539-544; Bird, T.D., Wijsman, E.M., Nochlin, D., Chromosome 17 and hereditary dementia: Linkage studies in three non-Alzheimer families and kindreds with late onset FAD (1997) Neurology, 48, pp. 949-954; Murrell, J.R., Spillantini, M.G., Zolo, P., Tau gene mutation G398R causes a tauopathy with abundant Pick body-like inclusions and axonal deposits (1999) J Neuropathol Exp Neurol, 58, pp. 1207-1226; Ghorayeb, I., Series, C., Parchi, P., Creutzfeldt-Jakob disease with long duration and panencephalopathic lesions: Molecular analysis of one case (1998) Neurology, 51, pp. 271-274; Chawluk, J.B., Mesulam, M.M., Hurtig, H., Slowly progressive aphasia without generalized dementia: Studies with positron emission tomography (1986) Ann Neurol, 19, pp. 68-74; 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 (1990) Brain, 113, pp. 1321-1336; Kempler, D., Metter, E.J., Riege, W.H., Slowly progressive aphasia: Three cases with language, memory, CT and PET data (1990) J Neurol Neurosurg Psychiatr, 53, pp. 987-993; Mummery, C.J., Patterson, K., Wise, R.J.S., Disrupted temporal lobe connections in semantic dementia (1999) Brain, 122, pp. 61-73; Abe, K., Ukita, H., Yanagihara, T., Imaging in primary progressive aphasia (1997) Neuroradiology, 39, pp. 556-559; Gitelman, D.R., Nobre, A., Sonty, S., Phonological and semantic activations in primary progressive aphasia (2000) Neuroimage, 11 S, p. 122; Harasty, J.A., Halliday, G.M., Code, C., Brooks, W.S., Quantification of cortical atrophy in a case of progressive fluent aphasia (1996) Brain, 119, pp. 181-190; Turner, R.S., Kenyon, L.C., Trojanowski, J.Q., Clinical, neuroimaging, and pathologic features of progressive nonfluent aphasia (1996) Ann Neurol, 39, pp. 166-173; Galton, C.J., Patterson, K., Xuereb, J.H., Hodges, J.R., Atypical and typical presentations of Alzheimer's disease: A clinical, neuropsychological, neuroimaging and pathological study of 13 cases (2000) Brain, 123, pp. 484-498; Chin, S.S.-M., Goldman, J.E., Devenand, D.R., Thalamic degeneration presenting as primary progressive aphasia (1994) Brain Pathol, 4, p. 515; Kinoshita, A., Tomimoto, H., Tachibana, N., A case of primary progressive aphasia with abnormally ubiquinated neurites in the cerebral cortex (1996) Acta Neuropathol, 92, pp. 520-524; Lippa, C.F., Cohen, R., Smith, T.W., Drachman, D.A., Primary progressive aphasia with focal neuronal achromasia (1991) Neurology, 41, pp. 882-886; Ikeda, K., Akiyama, H., Iritani, S., Corticobasal degeneration with primary progressive aphasia and accentuated cortical lesion in superior temporal gyrus: Case report and review (1996) Acta Neuropathol, 92, pp. 534-539; Molina, J.A., Probst, A., Villanueva, C., Primary progressive aphasia with glial cytoplasmic inclusions (1998) Eur Neurol, 40, pp. 71-77; Kertesz, A., Hudson, L., Mackenzie, I.R.A., Munoz, D.G., The pathology and nosology of primary progressive aphasia (1994) Neurology, 44, pp. 2065-2072; Miller, B.L., Boone, K., Geschwind, D., Wilhelmsen, B.L., Pick's disease and frontotemporal dementias: Emerging clinical and molecular concepts (1999) Neurologist, 5, pp. 205-212; Bird, T.D., Genotypes, phenotypes, and frontotemporal dementia. Take your pick (1998) Neurology, 50, pp. 1526-1527; Wilhelmsen, K.C., Frontotemporal dementia is on the MAPt (1997) Ann Neurol, 41, pp. 139-140; Nasreddine, Z.S., Loginov, M., Clark, L.N., From genotype to phenotype: A clinical, pathological, and biochemical investigation of frontotemporal dementia and parkinsonism (FTDP-17) caused by the P301L tau mutation (1999) Ann Neurol, p. 45; Brown, J., Ashworth, A., Gydesen, S., Familial non-specific dementia maps to chromosome 3 (1995) Hum Mol Genet, 4, pp. 1625-1628 ---=note-separator=--- Correspondence Address: Mesulam, M.-M.; Northwestern Univ. Medical School; 320 East Superior Street Chicago, IL 60611, United States; email: mmesulam@nwu.edu ---=note-separator=--- Language of Original Document: English ---=note-separator=--- doi: 10.1002/ana.91 ---=note-separator=--- CODEN: ANNED ---=note-separator=--- Source: Scopus ---=note-separator=--- Export Date: 21 February 2009 ---=note-separator=--- Cited By (since 1996): 176}, isbn = {03645134 (ISSN)}, journal = {Annals of Neurology}, keywords = {17, alzheimer, anomia, aphasia, autopsy, chromosome, dementia, disease, dna, human, humans, journal, neuropathology, pick, polymorphism, presenile, primary, priority, progressive, protein, review, tau}, number = {4}, pages = {425--432}, posted-at = {2009-02-21 20:07:06}, priority = {2}, title = {Primary progressive aphasia}, url = {http://www.scopus.com/scopus/inward/record.url?eid=2-s2.0-0035071053&partnerID=40}, volume = {49}, year = {2001} }

TY - JOUR ID - citeulike:4078337 L3 - citeulike-article-id:4078337 N2 - Primary progressive aphasia (PPA) is a focal dementia characterized by an isolated and gradual dissolution of language function. The disease starts with word-finding disturbances (anomia) and frequently proceeds to impair the grammatical structure (syntax) and comprehension (semantics) of language. The speech output in PPA can be fluent or nonfluent. Memory, visual processing, and personality remain relatively well-preserved until the advanced stages and help to distiguish PPA from frontal lobe dementia and the typical forms of Alzheimer's disease. The term "semantic dementia" was originally introduced to designate a different group of patients with a combination of verbal and visual processing deficits. In practice, however, this diagnosis is also being used in a variant sense to denote a subtype of PPA with fluent speech and impaired comprehension, even in the absence of visual processing deficits. Insofar as the diagnosis of semantic dementia can have these two different meanings, it is important to specify whether it is being used in the original sense or to denote a subtype of PPA. Structural and physiological neuroimaging confirms the selective predilection of PPA for the left hemisphere, especially for its language-related cortices. A few patients with PPA display the neuropathological markers of Alzheimer's disease, but in an unusual distribution. The majority of the autopsies in PPA have shown either Pick's disease or lobar atrophy without distinctive histopathology. The suggestion has been made that PPA and frontal lobe dementia constitute phenotypical variations of a unitary disease process within the "Pick-lobar atrophy" spectrum. Recent advances in chromosome 17-linked dementias justify a rigorous search for tau polymorphisms and tauopathy in sporadic PPA. An informed approach to this syndrome will increase the effectiveness with which clinicians can address the unique challenges associated with the diagnosis and care of PPA. IS - 4 JF - Annals of Neurology SN - 03645134 (ISSN) AD - Cognitive Neurology and Alzheimer's Disease Center, Northwestern Cognitive Brain Mapping Group, Northwestern University, Chicago, IL, United States EP - 432 TI - Primary progressive aphasia VL - 49 SP - 425 KW - 17 KW - alzheimer KW - anomia KW - aphasia KW - autopsy KW - chromosome KW - dementia KW - disease KW - dna KW - human KW - humans KW - journal KW - neuropathology KW - pick KW - polymorphism KW - presenile KW - primary KW - priority KW - progressive KW - protein KW - review KW - tau N1 - References: Mesulam, M.-M., Weintraub, S., Spectrum of primary progressive aphasia (1992), pp. 583-609. , Rossor MN, editor. Unusual dementias. London: Baillière TindallWeintraub, S., Rubin, N.P., Mesulam, M.M., Primary progressive aphasia. Longitudinal course, neuropsychological profile, and language features (1990) Arch Neurol, 47, pp. 1329-1335; Weintraub, S., Mesulam, M.-M., Four neuropsychological profiles in dementia (1993) Handbook of neuropsychology, 8, pp. 253-281. , Boller F, Grafman J, editors. Amsterdam: Elsevier; Weintraub, S., Mesulam, M.-M., From neuronal networks to dementia: Four clinical profiles (1996), pp. 75-97. , Fôret F, Christen Y, Boller F, editors. La demence: pourquoi? Paris: Foundation Nationale de GerontologieBroussolle, E., Bakchine, S., Tommasi, M., Slowly progressive anarthria with late anterior opercular syndrome: A variant form of frontal cortical atrophy syndromes (1996) J Neurol Sci, 144, pp. 44-58; Mesulam, M.-M., Johnson, N., Grujic, Z., Weintraub, S., Apolipoprotein E genotypes in primary progressive aphasia (1997) Neurology, 49, pp. 51-55; Rosenfeld, M., Die partielle grosshirnatrophie (1909) J Psychol Neurol, 14, pp. 115-130; Pick, A., Ueber die beziehungen der senilen hirnatrophie zur aphasie (1892) Prager Med Wochenschr, 17, pp. 165-167; Pick, A., Zur symptomatologie der linksseitigen schlaffenlappenatrophie (1904) Monatsschr Psychiatr Neurol, 16, pp. 378-388; Franceschi, F., Gliosi perivasculare in un caso de demenza afasica (1908) Ann Neurol, 26, pp. 281-290; ?rieux, P., Sur un cas de surdité verbale pure (1893) Rev Med, 13, pp. 733-750; Dejerine, J., ?rieux, P., Un cas de surdité verbale pure terminée par aphasie sensorielle, suivie d'autopsie (1897) CR Acad Sci, 49, pp. 1074-1077; Mesulam, M.M., Primary progressive aphasia - Differentiation from Alzheimer's disease (1987) Ann Neurol, 22, pp. 533-534; Mesulam, M.M., Slowly progressive aphasia without generalized dementia (1982) Ann Neurol, 11, pp. 592-598; Westbury, C., Bub, D., Primary progressive aphasia: A review of 112 cases (1997) Brain Lang, 60, pp. 381-406; Habib, M., Pelletier, J., Khalil, R., Aphasie progressive primaire (syndrome de Mesulam) (1993) Presse Méd, 22, pp. 757-764; Mesulam, M.-M., From sensation to cognition (1998) Brain, 121, pp. 1013-1052; Mesulam, M.-M., Aphasias and other focal cerebral disoders (1998), pp. 134-142. , Fauci AS, Braunwald E, Isselbacher KJ, editors. In Harrison's principles of internal medicine, 14th ed. New York: McGraw HillThompson, C.K., Ballard, K.J., Tait, M.E., Patterns of language decline in non-fluent primary progressive aphasia (1997) Aphasiology, 11, pp. 297-321; Snowden, J.S., Neary, D., Mann, D.M.A., Progressive language disorder due to lobar atrophy (1992) Ann Neurol, 31, pp. 174-183; Mehler, M.F., Mixed transcortical aphasia in nonfamilial dysphasic dementia (1988) Cortex, 24, pp. 545-554; Otsuki, M., Soma, Y., Sato, M., Slowly progressive pure word deafness (1998) Eur Neurol, 39, pp. 135-140; Price, B.H., Gurvit, H., Weintraub, S., Neuropsychological patterns and language deficits in 20 consecutive cases of autopsy-confirmed Alzheimer's disease (1993) Arch Neurol, 50, pp. 931-937; Béland, R., Ska, B., Interaction between verbal and gestural language in progressive aphasia: A longitudinal case study (1992) Brain Lang, 43, pp. 355-385; Cappa, S.F., DeFanti, C.A., DeMarco, R., Progressive dysphasic dementia with bucco-facial apraxia: A case report (1993) Behav Neurol, 6, pp. 159-163; McCarthy, R.A., Warrington, E.K., Evidence for modality-specific meaning systems in the brain (1988) Nature, 334, pp. 428-430; Basso, A., Capitani, E., Laiacona, M., Progressive language impairment without dementia: A case with isolated category specific semantic defect (1988) J Neurol Neurosurg Psychiatr, 51, pp. 1201-1207; Neary, D., Snowden, J.S., Gustafson, L., Frontotemporal lobar degeneration. A consensus on clinical diagnostic criteria (1998) Neurology, 51, pp. 1546-1554; Rossor, M.N., Revesz, T., Lantos, P.L., Warrington, E.K., Semantic dementia with ubiquitin-positive tau-negative inclusion bodies (2000) Brain, 123, pp. 267-276; Hodges, J.R., Patterson, K., Oxbury, S., Funnell, E., Semantic dementia. Progressive fluent aphasia with temporal lobe atrophy (1992) Brain, 115, pp. 1783-1806; Snowden, J.S., Goulding, P.J., Neary, D., Semantic dementia: A form of circumscribed atrophy (1989) Behav Neurol, 2, pp. 167-182; Tyrrell, P.J., Warrington, E.K., Frackowiak, R.S.J., Rossor, M.N., Progressive degeneration of the right temporal lobe studied with positron emission tomography (1990) J Neurol Neurosurg Psychiatr, 53, pp. 1046-1050; DeRenzi, E., Slowly progressive visual agnosia or apraxia without dementia (1986) Cortex, 22, pp. 171-180; Freedman, L., Costa, L., Pure alexia and right hemiachromatopsia in posterior dementia (1992) J Neurol Neurosurg Psychiatr, 55, pp. 500-502; Benson, D.F., Davis, J.R., Snyder, B.D., Posterior cortical atrophy (1988) Arch Neurol, 45, pp. 789-793; Peavy, G.M., Herzog, A.G., Rubin, N.P., Mesulam, M.M., Neuropsychological aspects of dementia of motor neuron disease: A report of two cases (1992) Neurology, 42, pp. 1004-1008; Rakowicz, W., Hodges, J.R., Dementia and aphasia in motor neuron disease: An underrecognized association? (1998) J Neurol Neurosurg Psychiatr, 65, pp. 881-889; Strong, M.J., Grace, G.M., Orange, J.B., A prospective study of cognitive impairment in ALS (1999) Neurology, 53, pp. 1665-1670; Caselli, R.J., Windebank, A.J., Petersen, R.J., Rapidly progressive aphasic dementia and motor neuron disease (1993) Ann Neurol, 33, pp. 200-207; Frattali, C.M., Grafman, J., Patronas, N., Language disturbances in corticobasal degeneration (2000) Neurology, 54, pp. 990-992; Foster, N.L., Frontotemporal dementia with parkinsonism linked to chromosome 17 (FTDP-17): A clinician's guide (1999) Neurologist, 5, pp. 213-221; Snowden, J.S., Neary, D., Progressive language dysfunction and lobar atrophy (1993) Dementia, 4, pp. 226-231; Lendon, C.L., Lynch, T., Norten, J., Hereditary dysphasia disinhibition dementia. A frontotemporal dementia linked to 17q 21-22 (1998) Neurology, 50, pp. 1546-1555; Basun, H., Almkvist, O., Axelman, K., Clinical characteristics of a chromosome 17-linked rapidly progressive familial frototemporal dementia (1997) Arch Neurol, 54, pp. 539-544; Bird, T.D., Wijsman, E.M., Nochlin, D., Chromosome 17 and hereditary dementia: Linkage studies in three non-Alzheimer families and kindreds with late onset FAD (1997) Neurology, 48, pp. 949-954; Murrell, J.R., Spillantini, M.G., Zolo, P., Tau gene mutation G398R causes a tauopathy with abundant Pick body-like inclusions and axonal deposits (1999) J Neuropathol Exp Neurol, 58, pp. 1207-1226; Ghorayeb, I., Series, C., Parchi, P., Creutzfeldt-Jakob disease with long duration and panencephalopathic lesions: Molecular analysis of one case (1998) Neurology, 51, pp. 271-274; Chawluk, J.B., Mesulam, M.M., Hurtig, H., Slowly progressive aphasia without generalized dementia: Studies with positron emission tomography (1986) Ann Neurol, 19, pp. 68-74; 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 (1990) Brain, 113, pp. 1321-1336; Kempler, D., Metter, E.J., Riege, W.H., Slowly progressive aphasia: Three cases with language, memory, CT and PET data (1990) J Neurol Neurosurg Psychiatr, 53, pp. 987-993; Mummery, C.J., Patterson, K., Wise, R.J.S., Disrupted temporal lobe connections in semantic dementia (1999) Brain, 122, pp. 61-73; Abe, K., Ukita, H., Yanagihara, T., Imaging in primary progressive aphasia (1997) Neuroradiology, 39, pp. 556-559; Gitelman, D.R., Nobre, A., Sonty, S., Phonological and semantic activations in primary progressive aphasia (2000) Neuroimage, 11 S, p. 122; Harasty, J.A., Halliday, G.M., Code, C., Brooks, W.S., Quantification of cortical atrophy in a case of progressive fluent aphasia (1996) Brain, 119, pp. 181-190; Turner, R.S., Kenyon, L.C., Trojanowski, J.Q., Clinical, neuroimaging, and pathologic features of progressive nonfluent aphasia (1996) Ann Neurol, 39, pp. 166-173; Galton, C.J., Patterson, K., Xuereb, J.H., Hodges, J.R., Atypical and typical presentations of Alzheimer's disease: A clinical, neuropsychological, neuroimaging and pathological study of 13 cases (2000) Brain, 123, pp. 484-498; Chin, S.S.-M., Goldman, J.E., Devenand, D.R., Thalamic degeneration presenting as primary progressive aphasia (1994) Brain Pathol, 4, p. 515; Kinoshita, A., Tomimoto, H., Tachibana, N., A case of primary progressive aphasia with abnormally ubiquinated neurites in the cerebral cortex (1996) Acta Neuropathol, 92, pp. 520-524; Lippa, C.F., Cohen, R., Smith, T.W., Drachman, D.A., Primary progressive aphasia with focal neuronal achromasia (1991) Neurology, 41, pp. 882-886; Ikeda, K., Akiyama, H., Iritani, S., Corticobasal degeneration with primary progressive aphasia and accentuated cortical lesion in superior temporal gyrus: Case report and review (1996) Acta Neuropathol, 92, pp. 534-539; Molina, J.A., Probst, A., Villanueva, C., Primary progressive aphasia with glial cytoplasmic inclusions (1998) Eur Neurol, 40, pp. 71-77; Kertesz, A., Hudson, L., Mackenzie, I.R.A., Munoz, D.G., The pathology and nosology of primary progressive aphasia (1994) Neurology, 44, pp. 2065-2072; Miller, B.L., Boone, K., Geschwind, D., Wilhelmsen, B.L., Pick's disease and frontotemporal dementias: Emerging clinical and molecular concepts (1999) Neurologist, 5, pp. 205-212; Bird, T.D., Genotypes, phenotypes, and frontotemporal dementia. Take your pick (1998) Neurology, 50, pp. 1526-1527; Wilhelmsen, K.C., Frontotemporal dementia is on the MAPt (1997) Ann Neurol, 41, pp. 139-140; Nasreddine, Z.S., Loginov, M., Clark, L.N., From genotype to phenotype: A clinical, pathological, and biochemical investigation of frontotemporal dementia and parkinsonism (FTDP-17) caused by the P301L tau mutation (1999) Ann Neurol, p. 45; Brown, J., Ashworth, A., Gydesen, S., Familial non-specific dementia maps to chromosome 3 (1995) Hum Mol Genet, 4, pp. 1625-1628 N1 - Correspondence Address: Mesulam, M.-M.; Northwestern Univ. Medical School; 320 East Superior Street Chicago, IL 60611, United States; email: mmesulam@nwu.edu N1 - Language of Original Document: English N1 - doi: 10.1002/ana.91 N1 - CODEN: ANNED N1 - Source: Scopus N1 - Export Date: 21 February 2009 N1 - Cited By (since 1996): 176 AU - Mesulam, MM PY - 2001/// UR - http://www.scopus.com/scopus/inward/record.url?eid=2-s2.0-0035071053&partnerID=40 ER -