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Washington University Experience | NEURODEGENERATION | Hippocampal Sclerosis of Aging | 3A0 Case 3 History
Case 3 History ---- This 68 year old male was a member of the Hereditary Dysphasic Disinhibition Dementia Family 2 (HDDD2), a frontotemporal dementia with prominent changes in behavior and language deficits. A previous study found significant linkage to chromosome 17 and a missense mutation in the signal peptide of the progranulin (GRN) gene. He was seen in 2002 by members of the ADRC Genetics Core. His mother, aunts and uncles, as well as cousins, had the family disease with onset ranging from age 52 - 77. His short term memory after a stroke 4 years prior to his death was “slim to none” but he could still recall long term information. His daughter stated that he had a seizure one time about 2.5 years prior to death (her brother witnessed the seizure). He stopped walking at about the same time “just gave up” because his left leg was dragging. It was harder for him to talk after the stroke and he became even less talkative about 2 years prior to death. He had been hospitalized for one week in late November 2012 and diagnosed with Clostridium difficile and dehydration. Hospice was recommended and he was released back to the nursing home. He was in kidney failure and died in December 2012.