Clinicopathologic analysis of progressive non-fluent aphasia and corticobasal degeneration: Case report and review
نویسندگان
چکیده
Objective To investigate progressive non-fluent aphasia and histopathologically-proven corticobasal degeneration. Methods 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. Results 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. Conclusion 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.
منابع مشابه
Primary progressive aphasia as the initial manifestation of corticobasal degeneration. A "three in one " syndrome?
In 1994, the term "Pick complex" was proposed to indicate significant clinical and pathological overlapping between primary progressive aphasia, frontal lobe dementia and corticobasal degeneration. We report the case of a 60-year-old man, who initially presented progressive non-fluent aphasia with orofacial apraxia, and subsequently, over a period of 3 years, developed mutism, pathological laug...
متن کاملClinicopathologic analysis of frontotemporal and corticobasal degenerations and PSP.
OBJECTIVE To examine the relationship between early clinical features, pathologies, and biochemistry of the frontotemporal lobar degenerations (FTLDs), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD). METHODS The authors conducted pathologic reexamination with the most recent immunohistochemistry of all cases diagnosed with FTLD, PSP, and CBD between 1970 and 2004. T...
متن کاملDiffusion tensor magnetic resonance imaging for single subject diagnosis in neurodegenerative diseases.
Although magnetic resonance imaging is a standard investigation in neurodegenerative disease, sensitive and specific markers for the underlying histopathological diagnosis are largely lacking. This report presents evidence to indicate that corticobasal degeneration and progressive supranuclear palsy, in particular, might be identifiable at a single subject level with diffusion tensor imaging. P...
متن کاملCorticobasal degeneration as cause of progressive non-fluent aphasia: clinical, radiological and pathological study of an autopsy case.
A Japanese male developed gradual loss of spontaneous speech at age 60. Three years later meaningful speech had deteriorated to the point that it had become restricted to monotonous utterances. Neuropsychological examination at age 62 showed that he had severe non-fluent aphasia. A brain MRI demonstrated mild cortical atrophy with ischemic lesions in the cerebral white matter. He was diagnosed ...
متن کاملCorticobasal degeneration -- clinico-pathological considerations.
Corticobasal degeneration (CBD) is a rare sporadic 4-repeat tauopathy. We report here the first Polish case of pathologically proven CBD. Our patient developed clumsiness of the right hand at age 63 years. During the course of his illness he suffered from progressive asymmetric parkinsonism unresponsive to dopaminergic therapy. Focal dystonia affecting right upper extremity, non-fluent aphasia,...
متن کامل