Corticobasal Degeneration (CBD) is a degenerative brain disease, affecting people from the age of 40 onwards. There are biological similarities to PSP, but the clinical picture is quite distinct.
- Cortico – affecting the brain cortex
- Basal – affecting other parts of the brain such as the basal ganglia
- Degeneration – death of nerve cells in the brain
Signs and Symptoms
Corticobasal Degeneration (CBD) often starts with progressive numbness and loss of the use of one hand/leg. The muscle strength is normal but the problems of tone, slowness, and tremor occur. Other symptoms include awkwardness, rigidity, jerking of the fingers, and ‘alien limb’ syndrome. These complex unintentional movements of one limb cause problems with normal motor tasks. Everyday tasks such as dressing, tying shoelaces using the remote control, or writing become increasingly difficult. Gradually the condition spreads to the other side of the body.
Eye movements can be disturbed and there may be difficulty orientating an object in space. Eye issues are normally less debilitating than in PSP. Simple calculations such as addition and subtraction can be difficult. Speech expression can be hesitant and halting. As CDB progresses there is increased muscle spasm, difficulties with speech and swallow and a decline in mental function. However it is important to know that CBD does not affect everybody in the same way.
Diagnosis and Treatment
Diagnosis in CBD is by clinical observation of symptoms and by excluding other conditions such as Parkinson’s disease, Stroke, Motor Neuron Disease and common forms of dementia. It is important to see a neurologist with experience of PSP to get a definite diagnosis. On referral to hospital numerous tests and assessments similar to that described under PSP will be carried out and you will be seen by a multidisciplinary team who will focus on managing symptoms and making it easier to cope with daily life.
Treatment is aimed at relieving symptoms while maintaining best quality of life. Medications are prescribed to improve balance, relieve stiffness and rigidity, to address muscle spasm and other aspects of the condition. Regular exercise and a good diet is important.While CBD is not life threatening in itself swallowing difficulties increases the risk of choking and aspiration pneumonia. Sepsis and pulmonary embolism are also a risk in the later stages of CBD.
Having a diagnosis of CDB is emotionally upsetting so plenty of family support is important. PSPA Ireland provides information advice and support group meetings for patients, carers and families whose life is affected by Corticobasal Degeneration.