GLOSSARY OF TERMS

GLOSSARY OF TERMS

14-3-3: The name of one of the biomarkers which, if present, in the spinal fluid may indicate the presence of a prion disease.

TAU: One of the biomarkers found in the cerebrospinal fluid. An elevated level can indicate the presence of a prion disease.

AKINETIC MUTISM: A state in which patients cannot speak (mutism) or move (akinesia) due to damage of the brain.

AUTOPSY: In cases of suspected CJD, this is a post-mortem, brain only procedure.  At present, it is the only conclusive method to confirm a diagnosis of CJD.

AUTOSOMAL DOMINANT: One of several ways a trait or disorder can be passed down through families.  If a disease is autosomal dominant, it means a child requires an abnormal gene from one parent to inherit the disease.

BOVINE SPONGIFORM ENCEPHALOPATHY (BSE): A prion disease in cattle. Also known as “Mad Cow Disease.”

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

CEREBROSPINAL FLUID (CSF): A bodily fluid composed mostly of water, glucose, salt and proteins that surrounds cushions and provides nutrients to the brain and spinal cord.  CSF is the fluid collected in a spinal tap test.

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

CHRONIC WASTING DISEASE (CWD): A prion disease in deer and elk.

ELECTROENCEPHALOGRAPHY (EEG): A non-invasive test of brain wave patterns in which changes can often be seen in CJD cases. 

ENCEPHALOPATHY: A disorder or disease of the brain.

FAMILIAL: Occurring in or affecting blood relatives

IATROGENIC: Acquired from outside sources, e.g. infected transplant or contaminated surgical instruments.  It literally means unintentionally induced by a physician, surgeon, medical treatment or diagnostic procedure.

INTRACEREBRAL: Occurring or situated within the brain.

KURU: A prion disease caused by the consumption of human tissue infected by a prion disease by members of the Fore Tribe in Papua, New Guinea. 

MAGNETIC RESONANCE IMAGING (MRI): A non-invasive radiology scan which provides good contrast between soft tissue and is generally a useful diagnostic test for CJD. 

DIFFUSION WEIGHTED MRI: An MRI imaging technique capable of showing connections between brain regions through water diffusion.

MYOCLONUS: Jerking movements of a muscle or groups of muscles that are caused by sudden spasms.

PRION: The name given to a protein that has become infectious.  It stands for a proteinaceous infectious agent.

PROTEIN: A large molecule made up of amino acids.  Proteins perform many different functions in the body.

RUMINANT: Any variety of hoofed, even footed, and usually horned mammals that characteristically have their stomachs divided into four sections, including cows, sheep, goats and deer.

SCRAPIE: A prion disease in sheep and goats characterized by chronic itching, loss of muscular control and progressive degeneration of the central nervous system.

SPINAL TAP or LUMBAR PUNCTURE: A diagnostic procedure that uses a hollow needle to enter the spinal canal in the lower (lumbar) spine to remove cerebrospinal fluid (CSF) for analysis.

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

SPORADIC: If means that this happens occasional, infrequent or random manner.  Sporadic CJD, presently has no known cause

TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY (TSE): Any fatal, incurable degenerative disease of the human or animal brain caused by prions.