Corticobasal Degeneration by
Dr Basil Rodha & Dr. Jonathan Rohrer, Dementia Research Centre
Corticobasal degeneration is the name given to a progressive degenerative disorder causing problems with movement and thinking. The disease is named after the parts of the brain affected – cortico refers to the cortex, which is the outer layer of the brain, and basal refers to the basal ganglia which are structures deep in the brain that control motor skills. In CBD there is progressive loss of brain cells in these areas. It usually affects people in their 60’s or 70’s. Males and females are equally affected. It is only very rarely genetic.
At post-mortem, most patients with CBD will have abnormal deposition of a protein called tau in their brain cells although this is not the case for every patient.
Symptoms
There are normally problems with both movement and thinking in CBD syndrome. There may also be behavioural symptoms similar to frontotemporal dementia.
Motor problems (normally much worse on one side)
- Limb clumsiness.
- Slowing down of movements.
- Stiffness.
- Tremor or jerkiness.
- Inability to use hands to do complex actions.
- Holding the arm in an odd posture (which is given the name “alien limb”).
Thinking problems
- Language difficulties e.g. non-fluent aphasia (See Progressive Non-Fluent Aphasia).
- Problems with planning, problem-solving, calculation.
Medical tests
Language ability, memory and other aspects of thinking (cognitive functions) will be assessed, initially by a doctor, and often followed by a more detailed assessment by a psychologist. A neurological examination looking in particular for problems with movement will be performed. Brain scans can show a loss of brain cells in the frontal lobes and also at the back of the brain (the parietal lobes) but the pattern of brain changes seen may vary from patient to patient. There is no single test that can specifically diagnose CBD with complete reliability during a person’s lifetime. Your doctor will often arrange blood tests or other tests (a brain scan and an EEG or electroencephalogram, and sometimes a lumbar puncture) to help rule out diseases that can produce similar symptoms to CBD.
Treatment
There is currently no medication that can cure the disease or slow its progression. Treatment therefore focuses on helping people manage their symptoms, and treating problems such as mood changes that may contribute to the difficulties that people experience.
Prognosis
The physical and thinking problems deteriorate over time and behavioural problems may become worse. Later in the illness people are likely to have increasing problems carrying out their normal daily living activities and managing their affairs, and they will need extra care and support.
back to list
|
