A UNIQUE AUTOSOMAL-DOMINANT DISORDER WITH INDIFFERENCE TO PAIN - CLINICOPATHOLOGICAL CORRELATION OF INDIFFERENCE TO PAIN AND THALAMIC GLIOSIS

We present updated information on a previously reported kindred with a n autosomal dominant disorder variably expressed as indifference to pa in, dementia, and ataxia. Additional clinical and radiological informa tion is presented, as are autopsy results form the index case. In addi tion to evidence of Alzheimer's disease, the autopsy revealed bilatera l thalamic gliosis, which may be a neuroanatomic substrate for the ind ifference to pain seen in this patient. To our knowledge, this is the first reported association of thalamic gliosis and indifference to pai n.