Corticobasal degeneration

doi:10.1016/S1474-4422(04)00936-6

The Lancet Neurology

Volume 3, Issue 12, December 2004, Pages 736-743

https://doi.org/10.1016/S1474-4422(04)00936-6 Get rights and content

Summary

Corticobasal degeneration is a progressive neuro-degenerative 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 that look like it but are caused by other neurodegenerative diseases. Although definitive diagnosis of Corticobasal degeneration can only be made at post-mortem examination, recent advances in imaging can assist the clinician with diagnosis. Treatment options remain limited and mostly address symptoms.

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

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.

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