What SANDHOFF DISEASE means: Sandhoff Disease - is a rare, genetic, lipid storage disorder resulting in the progressive deterioration of the central nervous system. It is caused by a deficiency of the enzyme beta- hexosaminidase, which results in the accumulation of certain fats (lipids) in the brain and other organs of the body. Sandhoff disease is a severe form of Tay-Sachs disease--which is prevalent primarily in people of Eastern European and Ashkenazi Jewish descent--but it is not limited to any ethnic group. Onset of the disorder usually occurs at 6 months of age. Neurological symptoms may include motor weakness, startle reaction to sound, early blindness, progressive mental and motor deterioration, macrocephaly (an abnormally enlarged head), cherry-red spots in the eyes, seizures, and myoclonus (shock-like contractions of a muscle).
- Definition Schizencephaly:
- Dictionary an extremely rare developmental birth defect characterized by abnormal slits, or clefts, in the cerebral hemispheres of the brain. Babies with clefts in both hemispheres (called bilateral clefts sandhoff disease.
- Definition Sjogren'S Syndrome:
- Dictionary Syndrome - is an autoimmune disorder in which immune cells attack and destroy the glands that produce tears and saliva. Sjogren's syndrome is also associated with rheumatic disorders such as sandhoff disease.
- Definition Shingles - (Herpes Zoster):
- Dictionary zoster) is an outbreak of rash or blisters on the skin that is caused by the same virus that causes chickenpox - the varicella-zoster virus. The first sign of shingles is often burning or tingling sandhoff disease.
- Definition Syncope:
- Dictionary temporary loss of consciousness due to a sudden decline in blood flow to the brain. It may be caused by an irregular cardiac rate or rhythm or by changes of blood volume or distribution. Syncope can sandhoff disease.