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Corticobasal Degeneration

This article provides an in-depth look at Corticobasal Degeneration (CBD), a neurodegenerative disorder characterized by motor and gait disorders, cognitive decline, and behavioral abnormalities. It discusses the median survival rate for patients with CBD and notes the absence of effective treatments for the disease. The article outlines various non-pharmacological and pharmacological management strategies to alleviate symptoms, including physical and occupational therapy, dietary modifications, and a range of medications targeting different symptoms of CBD.

Symptoms and Characteristics of Corticobasal Degeneration

Corticobasal degeneration (CBD) is a neurodegenerative disorder which causes progressive asymmetrical motor and gait disorders including limb rigidity, bradykinesia, focal myoclonus, dystonia, and/or apraxia. Also, it is associated with cognitive decline and behavioural abnormalities. Behavioural manifestations include compulsive behaviour, social withdrawal, depression, hyperorality, agitation, irritability, and apathy. The median survival of patients with CBD is 5.5-6.5 years.

Lack of Effective Treatment for CBD

There is no effective treatment for CBD and no medications provide significant symptomatic benefits. Treatment remains targeted at symptom amelioration and is not wholly effective as per trial results.

Non-Pharmacological Measures in CBD Management

Non-pharmacological measures include physical therapy which helps some patients with balance and gait problems. Occupational therapy can be beneficial in assisting with devices for eating and grooming and other adaptive measures. Measures, such as, wheelchairs could help prevent falls. Orthotic splinting may also reduce contractures and relieve pressure from tightly clenched fingers pressing into the palm. Dietician involvement is additionally important because dysphagia is a common symptom in the course of disease.

Pharmacological Treatments in CBD

Levodopa therapy may provide some transient benefits for parkinsonism, which is commonly present in the disease. Other medications include baclofen, clonazepam, and botulinum toxin. For patients with cognitive dysfunction, a cholinesterase inhibitor is reasonable to prescribe.

References

1- Dickson D.W. , Bergeron C. , Chin S.S. , et al.Office of Rare Diseases neuropathologic criteria for corticobasal degeneration. J. Neuropathol. Exp. Neurol., 61 (11) (2002), pp. 935-946 2- Schneider J.A. , Watts R.L. , Gearing M. , Brewer R.P: Corticobasal degeneration: neuropathologic and clinical heterogeneity. Neurology., 48 (4) (1997), pp. 959-969 3-Karakaya T, Fußer F, Prvulovic D, Hampel H. Treatment options for tauopathies. Curr Treat Options Neurol 2012; 14:126. 4-Fusco FR, Iosa M, Fusco A, et al. Bilateral upper limb rehabilitation with videogame-based feedback in corticobasal degeneration: a case reports study. Neurocase 2018; 24:156. 5- Kompoliti K. , Goetz C.G. , Boeve B.F. , et al.Clinical presentation and pharmacological therapy in corticobasal degeneration. Arch. Neurol., 55 (7) (1998), pp. 957-961 6-Constantinescu R, Richard I, Kurlan R. Levodopa responsiveness in disorders with parkinsonism: a review of the literature. Mov Disord 2007; 22:2141. 7- Watts R, Mirra SS, Richardson EP. Corticobasal ganglionic degeneration. In: Movement Disorders, Marsden CD, Fahn S (Eds), Butterworth-Heinemann, Oxford 1994. p.282. 8- Cordivari C. , Misra V.P. , Catania S. , Lees A.J. : Treatment of dystonic clenched fist with botulinum toxin. Mov. Disord., 16 (5) (2001), pp. 907-913 9- Kumar R, Bergeron C, Pollanen M, Lang AE. Corticobasal degeneration. In: Parkinson's Disease and Movement Disorders, 3rd ed, Jankovic J, Tolosa E (Eds), Lippincott Williams & Wilkins, Baltimore 1998. p.185.

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