Globoid Cell Leukodystrophy is a degenerative disease of the white matter of the brain and spinal cord, and also tied in with this is the loss of myelin, which is a fatty substance that forms the outer sheath around many nerve cells.  What causes this loss is a genetic deficiency of an enzyme that is involved in breaking down certain lipids (fats) in the brain and spinal cord.
     GCL has been seen in puppies as young as four weeks of age, and as late as five months.  The disease progresses very rapidly, resulting in death usually within two to three months after the onset of the clinical signs of the disease.  There is currently no treatment for GCL.
     The clinical signs for GCL are: loss of coordination, failure to recognize familiar individuals (people or other pets), stumbling, blindness, weakness, tail tremors, total limb paralysis, and lack of response to pain.  GCL has been found primarily in the bloodlines of West Highland White Terriers and Cairn Terriers, although it has been detected among other breeds as well.  It is imperative that breeding stock be checked for this condition due to its being autosomal recessive (genetically passed on from one generation to another).  In order for any autosomal recessive characteristic to appear in an offspring, both parents must either be carriers, or have the recessive characteristic. A blood test can determine the carrier (s) of this disease.