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CorticoBasal Degeneration by Dr Basil Ridha, Dementia Research Group What is corticobasal degeneration?This is a rare progressive neurological disease that causes disturbance of mental processes and motor skills. The disease is named after the areas of the brain that are principally affected. Cortico refers to the cortex, which is the outer layer of the brain, and basal refers to the basal ganglia, which are deep structures in the brain that control motor skills. In these brain regions, there is more significant loss of brain cells than in normal aging. ~The cause of brain cell loss is not known, but there is excessive accumulation of a certain substance called tau protein. This also occurs in other similar neurological diseases such a progressive supranuclear palsy or PSP.Who may get affected by corticobasal degeneration?The disease usually affects people in their 60’s or 70’s. Males and females are equally affected. There is no risk factor that makes a person more likely to develop corticobasal degeneration. The disease is quite rarely inherited.What are the symptoms of corticobasal degeneration?Corticobasal degeneration can present in many ways. It can affect mental processes such a memory, vision perception, speech, organizational skills, personality and behaviour. It can also affect motor skills causing rigidity, tremor, clumsiness, limitation of eye movements, involuntary movements, odd posturing of limbs and difficulty walking. A specific symptom that may be present in corticobasal degeneration patients is the ‘alien limb’, where a limb seems to move without control in a mind of its own. In all these movement abnormalities, one side of the body is affected more than the other.How is corticobasal degeneration diagnosed?The diagnosis of corticobasal degeneration is based on clinical judgement. Other conditions such as progressive supranuclear palsy, Parkinson’s disease and Pick’s disease can cause a similar picture. There is no definite blood test or brain scan that confirms the diagnosis. However, these investigations are necessary to exclude other causes of cognitive impairment and abnormal motor skills. Definite diagnosis can only be reached by examining brain tissue taken during brain biopsy or when examining the brain after death.What treatment and help are available for patients with corticobasal degeneration.There is no effective drug to treat corticobasal degeneration. However, a lot can be done to support the patient and his/her family. This would include the provision of appropriate appliances such as tailor-made cutlery, handles, Zimmer frame, wheelchair and stair-lift at home. Additional help at home and respite care may be necessary. Counselling and advice to carers about arranging power of attorney, benefits available from Social Services and managing difficult situations are vital.Dr. Basil Ridha, | Home | Newsletters | Events | Contacts | Internet Resources | Email Directory | Phone Directory | |
