References for this review were identified from searches of MEDLINE from 1980 to July 2004 with the terms “CBD”, “corticobasal degeneration”, “corticobasal ganglionic degeneration”, and “corticodentatonigral degeneration”. References were also identified from relevant articles and from the authors' files.
ReviewCorticobasal degeneration
Section snippets
Epidemiology
Corticobasal degeneration typically presents in the sixth to eighth decades of life, with onset of symptoms at mean age 63 years (SD 7·7).7 The youngest case, according to pathological confirmation, had onset at age 45 years.7 A patient who meets the clinical criteria for this disease with onset at age 28 years has been reported, but confirmation of this diagnosis awaits post-mortem analysis.8 Both men and women are affected, with some investigators reporting a predominance of women.9, 10, 11,
Clinical presentation
Several clinicopathological case series have examined clinical semiology, particularly the typical features at presentation. The data bias the motor presentation to that of atypical parkinsonism, because most of the series have come from movement disorder clinics (case report 1). The diagnostic criteria for Corticobasal degeneration were mainly developed from these studies. The disorder may present as a cognitive disorder more than is recognised: such patients are more likely to be referred to
Differential diagnosis: the “corticobasal degeneration-lookalike” syndromes
Clinical diagnosis can be difficult because of overlap with other neurodegenerative disorders. Despite a high specificity of clinical diagnosis (nearly 100%), sensitivity is only about 35% at presentation, rising to about 48% at the last visit.9 Of all the disorders that may be confused with corticobasal degeneration, perhaps the most difficult to differentiate is PSP. Although patients with PSP typically present with prominent axial rigidity, postural instability, and abnormal vertical eye
Imaging and electrophysiology
Results of routine laboratory studies of blood, urine, and CSF are normal in patients with corticobasal degeneration. CT and MRI scans of the brain tend to be normal in early stages of the disease. As the disease progresses, a pattern of asymmetric posterior, frontal, and parietal cortical atrophy becomes evident with dilatation of the lateral ventricle.51, 52
Electroencephalograms may be normal at first, but may later show asymmetric slowing that is maximum over the hemisphere contralateral to
Clinical diagnostic criteria
Given the clinical heterogeneity of corticobasal degeneration and the overlap of symptoms with other neurodegenerative diseases, there have been various attempts to create diagnostic criteria for clinical and research use. The first such criteria, outlined by Riley and colleagues5 comprised unilateral onset and asymmetric course, insidious onset with gradual progression, and clinical signs reflecting dysfunction in both cerebral cortex and basal ganglia. These criteria are broad, and would be
Treatment
There is no curative treatment for corticobasal degeneration.16 Management of symptoms and support can help patients with this disease.
Conclusion
There have been incremental advances in the understanding of the genetics, cellular pathology, and clinical features of corticobasal degeneration. However, clinical diagnosis of this heterogeneous disorder is difficult, and despite the use of specialised functional imaging methods in differential diagnosis, post-mortem neuropathology is the only definitive method of diagnosis. Current diagnostic criteria are likely to be biased towards “atypical parkinsonism” and may exclude patients with a
Search strategy and selection criteria
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Cortical degeneration with swollen chromatolytic neurons: its relationship to Pick's disease
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Neurology
Cortical-basal ganglionic degeneration
Neurology
Corticobasal degeneration
Brain
Natural history and survival of 14 patients with corticobasal degeneration confirmed at postmortem examination
J Neurol Neurosurg Psychiatry
Corticobasal degeneration: a clinical study of 36 cases
Brain
Corticobasal ganglionic degeneration
Corticobasal degeneration: neuropathologic and clinical heterogeneity
Neurology
Corticobasal degeneration
Epidemiologic aspects
Adv Neurol
Clinical presentation and pharmacological therapy in corticobasal degeneration
Arch Neurol
Phenotypes and prognosis: clinicopathologic studies of corticobasal degeneration
Adv Neurol
Dystonia in corticobasal degeneration
Adv Neurol
Accuracy of the clinical diagnosis of corticobasal degeneration: a clinicopathologic study
Neurology
Apraxia, mechanical problem solving and semantic knowledge: contributions to object usage in corticobasal degeneration
J Neurol
Dysarthria and orofacial apraxia in corticobasal degeneration
Mov Disord
The alien hand and related signs
J Neurol Neurosurg Psychiatry
Arm levitation in progressive supranuclear palsy
Neurology
The alien limb
Pract Neurol
Corticobasal degeneration as a cognitive disorder
Mov Disord
Corticobasal degeneration: selected developments
Mov Disord
Cortical-basal ganglionic degeneration
Corticobasal degeneration and its relationship to progressive supranuclear palsy and frontotemporal dementia
Ann Neurol
Corticobasal degeneration
Semin Neurol
The FAB: a Frontal Assessment Battery at bedside
Neurology
Corticobasal ganglionic degeneration and progressive supranuclear palsy presenting with cognitive decline
Brain Pathol
Dementia as the most common presentation of cortical-basal ganglionic degeneration
Neurology
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