UF researchers identify new form of dementia

April 24, 2003

GAINESVILLE, Fla. — Researchers at the University of Florida believe they have identified a new form of dementia, a disease that develops mostly in elderly people and is characterized by a progressive, generally irreversible loss of mental capabilities.

The most common cause of dementia is Alzheimer’s disease, but recently scientists have reported a rise in the number of patients with a different form, called frontotemporal dementia, which damages parts of the brain that influence memory, decision making and language.

Now, UF neurologists have discovered a case of extensive degeneration in regions of the brain not typically linked to either type of dementia.

During the annual American Academy of Neurology meeting in April in Honolulu, scientists affiliated with UF’s Evelyn F. and William L. McKnight Brain Institute and Gainesville’s Malcom Randall Veterans Affairs Medical Center described a patient who was not paralyzed but had progressive problems speaking because he could not coordinate facial movements. The patient – an elderly man – also had difficulty moving his eyes and making decisions, but did not have the memory deficits associated with Alzheimer’s disease.

“Examination of this patient’s brain after his death found degeneration in the brain in regions not typically associated with Alzheimer’s disease or the speech-loss syndromes seen with frontotemporal dementia,” said Dr. Kenneth Heilman, the James D. Rooks Jr. distinguished professor of neurology and health psychology at UF. Researchers found extensive damage to the region of the brain known as the basal ganglia.

As for the patient’s symptoms, speech and eye-movement problems can be associated

with Parkinson’s-like syndromes, but this man had no tremor and no severe rigidity, Heilman said. He had problems coordinating movement of his mouth and eyes, but he could walk and move his arms and legs normally.

“This appears to be a new form of dementia,” Heilman said.

Researchers have called it progressive ocular-oral-verbal apraxia with basal ganglia degeneration.

The disorder was described at the neurology conference by Dr. Heidi L. Roth, a former visiting assistant professor of neurology at UF, now with the University of North Carolina, who worked in association with Heilman, Dr. Thomas A. Eskin, a professor of pathology at UF’s College of Medicine, and Diane L. Kendall, a professor of neurology at the College of Medicine.

Dementia develops when the parts of the brain that are involved with learning, memory, decision making and language are affected by one or more infections or diseases, according to the National Organization of Rare Disorders Inc. The most common cause of dementia is Alzheimer’s disease, but there are as many as 50 other known causes.

Typically, once a new form of a disorder is confirmed, it becomes recognized more frequently as doctors see patients who have similar symptoms and recognize the pattern.

“If this is a distinct entity, with sufficient evidence to distinguish it from other forms of dementia, then people who collect cases can identify other patients who manifest the same signs and symptoms,” said Darryl Wieland, a professor of geriatrics with the School of Medicine at the University of South Carolina. “Rare cases are interesting, and you never know what they might mean. If it turns out the condition is more prevalent than previously recognized, researchers can look for causes.”