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Corticobasal Degeneration

How is it diagnosed?

It is a difficult disease to diagnose with accuracy and so CBD is sometimes diagnosed as something else, most commonly Parkinson's disease, vascular dementia, Alzheimer's disease or Pick's disease.

As the first symptoms a person develops could be cognitive or motor, some patients are referred to neurologists or geriatricians with an interest in movement disorders, whilst other patients may be referred to neurologists or psychiatrists with an interest in dementia.

There is no medical test for CBD. The clinical diagnosis is differential in that it is made by excluding other conditions that have similar symptoms such Alzheimer's disease or Parkinson's disease, by observing the person carefully and by talking to them and to those who know them well.

A conclusive diagnosis requires an autopsy. This is carried out after death, when brain tissue can be examined under a microscope. The characteristic hallmarks of the disease in the brain of someone who has died of CBD include:

  • death of neurones (brain cells) in a particular area of the brain
  • degeneration of the substantia nigra ( a small part of the brain described as the 'gearbox of the brain'); if the substantia negra is not working properly there may be difficulty with leg and arm movements.
  • an absence of Lewy bodies (abnormal structures found in certain areas of the brain)
  • the presence of swollen, 'ballooned' cells in the substantia nigra.
  • gliosis in the brain (a type of brain scar tissue which can be seen under a microscope) caused by glial cells multiplying. This also occurs in Alzheimer's disease.

View What are the symptoms? What are the symptoms?  |  Inheritance patterns and prenatal diagnosis View Inheritance patterns and prenatal diagnosis

Medical text written November 2001 by the Alzheimer's Society. Last reviewed December 2004 by Dr N Fox, Dementia Research Society and Alzheimer's Society Medical & Scientific Advisory Committee.

 

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