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Dementia & Neuropsychologia

Print version ISSN 1980-5764

Dement. neuropsychol. vol.5 no.2 São Paulo Apr./June 2011 

Case Reports

Clinicopathologic analysis of progressive non-fluent aphasia and corticobasal degeneration:Case report and review

Estudo clínicopatologico de afasia progressiva não-fluente e degeneração córtico-basal: relato de caso e revisão

Paulo Roberto de Brito-Marques 1  

Roberto José Vieira-Mello 2  

Luciano Montenegro 2  

Maria de Fátima Vasco Aragão 3  

1Behavioral Neurology Unit, Department of Neurology, Faculty of Medical Sciences, University of Pernambuco, Recife PE, Brazil;

2Department of Pathology, Health Sciences Center, Federal University of Pernambuco, Receife PE Brazil;

3Centro de Diagnóstico Multimagem, Recife PE, Brazil.



To investigate progressive non-fluent aphasia and histopathologically-proven corticobasal degene­ration.


We evaluated symptoms, signs, neuropsychological deficits, and radiology data longitudinally, in a patient with autopsy-proven corticobasal degeneration and correlated these observations directly to the neuroanatomic distribution of the disease.


At presentation, a specific pattern of cognitive impairment was evident with an extreme extrapyramidal motor abnormality. Follow-up examination revealed persistent impairment of praxis and executive functioning, progressive worsening of language performance, and moderately preserved memory. The motor disorder manifested and worsened as the condition progressed. Many of the residual nerve cells were ballooned and achromatic with eccentric nuclei. Tau-immunoreactive pathology was significantly more prominent in neurons in the frontal and parietal cortices and dentate nuclei than in temporal neocortex, hippocampi and brainstem.


The clinical diagnosis of progressive non-fluent aphasia secondary to corticobasal degeneration hinged on a specific pattern of impaired cognition as well as an extrapyramidal motor disorder, reflecting the neuroanatomic distribution of the disease in frontal and anterior temporal cortices and the dentate nuclei.

Key words: progressive non-fluent aphasia; neuropsychology; neuroimaging; neuropathology; and corticobasal degeneration.



Investigar o quadro clínico de afasia progressiva não-fluente e confirmação histopatológica de degeneração córtico-basal.


Foram avaliadas as alterações clínicas, neuropsicológicas e de neuroimagem, durante todo o curso clínico da doença. O diagnóstico de degeneração córtico-basal foi confirmado por estudo histopatológico. Essas observações foram diretamente relacionadas com a distribuição anatômica da doença.


Foi observada uma forma específica de prejuízo cognitivo associada com importante alteração extrapiramidal. Durante o curso clínico, surgiram apraxia e disfunção executiva, piora progressiva da linguagem e memória moderadamente preservada. As alterações extrapiramidais pioraram progressivamente à rigidez universal e postura distônica. As reações de imunohistoquímica para a proteína tau foram significativamente mais proeminentes nos neurônios residuais de aspecto baloniformes e acromáticos do córtex frontal e parietal e núcleo denteado do cerebelo do que nos do neocórtex temporal, hipocampo e tronco cerebral.


O diagnóstico clínico de afasia progressiva não-fluente quando evolui para degeneração córtico-basal deve apresentar uma forma específica de prejuízo cognitivo, incluindo as alterações motoras, refletindo uma distribuição neuroanatômica da doença no córtex frontal e temporal anterior e no núcleo denteado do cerebelo.

Palavras-chave: afasia progressiva não-fluente; neuropsicologia; neuroimagem; neuropatologia e degeneração córtico-basal.

Texto completo disponível apenas em PDF.

Full text available only in PDF format.


Neary D, Snowden JS, Gustafson L, et al. Frontotemporal lobar degeneration. A consensus on clinical diagnostic criteria. Neurology 1998;51:1546-1554. [ Links ]

Gorno-Tempini ML, Dronkers NF, Rankin KP, et al. Cognition and anatomy in three variants of primary progressive aphasia. Ann Neurol 2004;55:335-346. [ Links ]

Josephs KA, Petersen RC, Knopman DS, et al. Clinicopathologic analysis of frontotemporal, corticobasal degenerations and PSP. Neurology 2006;66:41-48. [ Links ]

Stover NP, Watts RL. Corticobasal degeneration. Sem Neurol 2001;21:49-58. [ Links ]

Rebeiz JJ, Kolodny EH, Richardson EPJr. Corticodentatonigral degeneration with neuronal achromasia: a progressive disorder of late adult life. Trans Am Neurol Assoc 1968;92:23-26. [ Links ]

Folstein MF, Folstein SE, McHugh PR. Mini-mental state: a practical method for grading the cognitive state of patients for the clinician. J Psychiat Res 1975;12:189-198. [ Links ]

Joanette Y, Arlette P, Bernadette S, Brito-Marques PR. Avaliação neuro-psicológica adequada às demências. Arq Neuropsiquiatr 1995;53:147-152. [ Links ]

DeRenzi E, Faglioni P. Normative data and screening power of a shortened version of the Token Test. Cortex 1978,14:41. [ Links ]

Reitain R. Validity of the trail making test as an indicator of organic brain damage. Percept Mot Skills 1958;5:152. [ Links ]

European Concerted Action on Pick.s Disease (ECAPD) Consortium: provisional clinical and neuropathological criteria for the diagnosis of Pick's disease. Eur Neurol 1998;5:519-520. [ Links ]

Rinne JO, Lee MS, Thompson PD, Marsden CD. Corticobasal degeneration: a clinical study of 36 cases. Brain 1994;117: 1183-1196. [ Links ]

Wenning GK, Litvan I, Jankovic J. Natural history and survival of 14 patients with corticobasal degeneration confirmed at postmortem examination. J Neurol Neurosurg Psychiatry 1998;64:184-189. [ Links ]

Gibb WRG, Luthert PJ, Marsden CD. Corticobasal degeneration. Brain 1989;112:1171-1193. [ Links ]

Boeve BF, Maraganore DM, Parisi JE, et al. Pathologic heterogeneity in clinically diagnosed corticobasal degeneration. Neurology 1999;53:795-800. [ Links ]

Litvan I, Bhatia KP, Burn DJ, et al. SIC task force appraisal of clinical diagnostic criteria for parkinsonian disorders. Mov Disord 2003;18:467-486. [ Links ]

Grimes DA, Lang AE, Bergeron CB. Dementia as the most common presentation of corticobasal ganglionic degeneration. Neurology 1999;53:1969-1974. [ Links ]

Murray R, Neumann M, Forman MS, et al. Cognitive and motor assessment in autopsy-proven corticobasal degeneration. Neurology 2007;68:1274-1283. [ Links ]

Yamauch H, Fukuyama H, Nagahama Y, et al. Atrophy of the corpus callosum, cortical hypometabolism, and cognitive impairment in corticobasal degeneration. Arch Neurol 1998; 55:609-614. [ Links ]

Brito-Marques PR, Vieira de Melo R, Montenegro L. Classic Pick's disease type with ubiquitina-positive and tau-negative inclusions: report of a case. Arq Neuropsiquiatr 2000;59;128-133 [ Links ]

Brito-Marques PR, Vieira de Mello R, Montenegro L. Frontoparietal cortical atrophy with gliosis in the gray matter of cerebral cortex. Arq Neuropsiquiatr 2002;60:462-468. [ Links ]

Massman PJ, Kreiter KT, Jankovic J, Doody RS. Neuropsychological functioning in cortical-basal ganglionic degeneration: differentiation from Alzheimer's disease. Neurology 1996;46:720-726. [ Links ]

Pillon B, Blin J, Vidailhet M, et al. The neuropsychological pattern of corticobasal degeneration: comparison with progressive supranuclear palsy and Alzheimer's disease. Neurology 1995;45:1477-1483. [ Links ]

Graham NL, Bak T, Hodges JR. Corticobasal degeneration as a cognitive disorder. Mov Disord 2003;18:1224-1232. [ Links ]

Kertesz A, Hudson L, Mackenzie IR, Munoz DG. The pathology and nosology of primary progressive aphasia. Neurology 1994;44:2065-2072. [ Links ]

Kertesz A, Martinez-Lage P, Davidson W, Munoz DG. The corticobasal degeneration syndrome overlaps progressive aphasia and frontotemporal dementia. Neurology 2000;55: 1368-1375. [ Links ]

Mimura M, White RF, Albert ML. Corticobasal degeneration: neuropsychological and clinical correlates. J Neuropsychiatry Clin Neurosci 1997;9:94-98. [ Links ]

Riley DE, Lang AE, Lewis A, et al. Cortico-basal ganglionic degeneration. Neurology 1990;40:1203-1212. [ Links ]

Tang-Wai DF, Josephs KA, Boeve BF, Dickson DW, Parisi JE, Petersen RC. Pathologically confirmed corticobasal degeneration presenting with visuospatial dysfunction. Neurology 2003;61:1134-1135. [ Links ]

Josephs KA, Duffy JR. Apraxia of speech and nonfluent aphasia: a new clinical marker for corticobasal degeneration and progressive supranuclear palsy. Curr Opin Neurol 2008;21: 688-692. [ Links ]

Josephs KA, Duffy JR, Strand EA, et al. Clinicopathological and imaging correlates of progressive aphasia and apraxia of speech. Brain 2006;129:1385-1398. [ Links ]

Freeman RQ, Giovannetti T, Lamar M, et al. Visuoconstructional problems in dementia: contribution of executive systems functions. Neuropsychology 2000;14:415-426. [ Links ]

Mochizukia A, Ueda Y, Komatsuzaki Y, Tsuchiya K, Arai T, Shoji S. Progressive supranuclear palsy presenting with primary progressive aphasia: clinicopathological report of an autopsy case. Acta Neuropathol 2003:105;610-614. [ Links ]

Forman MS, Zhukareva V, Bergeron CB, et al. Signature tau neuropathology in gray and white matter of corticobasal degeneration. Am J Pathol 2002;160:2045-2053. [ Links ]

Ling H, O'Sullivan SS, Holton JL, et al. Does corticobasal degeneration exist? A clinicopathological re-evaluation. Brain 2010;133:2045-2057. [ Links ]

Received: March 18, 2011; Accepted: May 20, 2011

Paulo Roberto de Brito Marques - Rua Ana Angélica, 63 - 52010-230 Recife PE - Brazil. E-mail:

Disclosure: The authors reports no conflicts of interest.

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