Phenotypic Variation of Autosomal-Dominant Corticobasal Degeneration
نویسندگان
چکیده
منابع مشابه
Corticobasal degeneration.
Corticobasal degeneration is a progressive neurodegenerative disease that typically presents with asymmetrical parkinsonism and cognitive dysfunction. Recent molecular advances have given some clues to the pathogenesis of the disease. Clinical diagnosis is complicated by both the variability of presentation of true corticobasal degeneration, for example as a dementing illness, and the syndromes...
متن کاملCorticobasal degeneration
75 Introduction and history While historical review suggests earlier reports of the entity now known as corticobasal degeneration (CBD), the disease was fi rst clearly described in a series of three cases in 1968, when it was termed corticodentatonigral degeneration with neuronal achromasia [ 1 ]. Subsequent terminology has included corticonigral degeneration with neuronal achromasia [ 2 ], cor...
متن کاملNeuropsychiatric features of corticobasal degeneration.
OBJECTIVE To characterise the neuropsychiatric symptoms of patients with corticobasal degeneration (CBD). METHODS The neuropsychiatric inventory (NPI), a tool with established validity and reliability, was administered to 15 patients with CBD (mean (SEM), age 67.9 (2) years); 34 patients with progressive supranuclear palsy (PSP) (66.6 (1.2) years); and 25 controls (70 (0.8) years), matched fo...
متن کاملCognitive dysfunction in corticobasal degeneration.
Corticobasal degeneration (CBD) was originally described as a distinct clinicopathological entity in 1967. Since then, different phenotypic presentations have emerged as possible manifestations of CBD histopathological findings. In addition, pathophysiological findings and the molecular basis have been delineated and several aspects of its cognitive manifestations have been clarified. Thus, not...
متن کاملRapidly Progressive Corticobasal Degeneration Syndrome
INTRODUCTION Corticobasal syndrome (CBS) has a heterogeneous clinical presentation with no specific pathologic substratum. Its accurate diagnosis is a challenge for neurologists; in order to establish CBS definitively, postmortem confirmation is required. Some clinical and radiological features can help to distinguish it from other neurodegenerative conditions, such as Creutzfeldt-Jakob disease...
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ژورنال
عنوان ژورنال: European Neurology
سال: 2012
ISSN: 1421-9913,0014-3022
DOI: 10.1159/000334731