GLOSSARY OF TERMS

PROTEIN: Proteins are essential to life. They are molecules made up of thousands of smaller chemical units called amino acids. (More information is on page 4 of the pamphlet.)

PRION: The name given to a protein that has become infectious.

CSF: Cerebrospinal fluid. This is the fluid which is collected in a spinal tap test. It is tested for a specific protein (14-3-3) which may indicate the presence of CJD.

14-3-3: The name of the protein being tested for in the spinal fluid which may indicate the presence of a prion disease. CSF tests can produce false positives or false negatives or can be ambiguous.

BRAIN BIOPSY: A surgical procedure to remove a small specimen of tissue from the brain to test for CJD.

AUTOPSY: In cases of suspected CJD this is a brain only procedure. The brain is tested and a report generated to the physician. The family is entitled to receive a written report from the physician. Autopsy is the only conclusive way to confirm a diagnosis of CJD at present.

MRI: Magnetic Resonance Imaging: A non-invasive radiology scan used as a possible diagnostic tool.

DIFFUSION WEIGHTED MRI: A specific type of MRI which has proven to be a helpful diagnostic tool.

EEG: Electroencephalography: A non-invasive test of brainwave patterns in which changes can often be seen in CJD cases.

CEREBELLAR ATAXIA: Shaky movements, unsteady gait and clumsiness caused by damage to the cerebellum, a part of the brain which controls movement.

MYOCLONUS: Jerking movements or stiffening of the limbs caused by sudden muscle spasms.

AKINETIC MUTISM: A state of complete physical unresponsiveness caused by damage to the base of the brain.

SPONGIFORM CHANGE: Brain damage characterized by a spongy appearance of brain tissue seen under a microscope.

ENCEPHALOPATHY: Any disease in which the functioning of the brain is affected.