Frontotemporal Dementia: It's Not Knuckleheadedness

From the archives of the Johns Hopkins BrainWise (The Newsletter of the Johns Hopkins Department of Psychiatry and Behavioral Sciences)

Psychiatrist Chiadi Onyike remembers it vividly: A middle-aged woman sat quietly at a dining table and picked up her glass several times for a sip. This went on for some time, says Onyike, yet not a scrap of food had been served. Her plate was bare, her glass empty. “It’s the small scenerios,” he explains, “that give you a fine sense of frontotemporal dementia (FTD).”

Onyike, who heads Hopkins’ FTD clinic, aims to increase awareness of this type of dementia—often wrongly diagnosed as Alzheimer’s—as well as spread the word about clinic benefits. “Up to 20 percent of middle-aged patients who seek memory or dementia help have some form of FTD,” he says, “but few physicians other than neuropsychiatrists or behavioral neurologists are aware of it.”

An umbrella term, FTD covers up to seven fatal diseases—like Pick’s disease—all marked by dementia and atrophy of the brain’s frontal and/or anterior temporal lobes. The illnesses can affect behavior or language. The former brings what Onyike calls “a history of social miscues and misdemeanors that patients’ families may misinterpret as something psychiatric or as just knuckleheadedness.” Uncle George’s wanting to wear sweatpants to work seems odd, for example, but it’s his rude remarks, his repeated hair-combing and overblown jolliness that cross the line. In the language variants of FTD, patients’ speech deteriorates gradually; they become mute. Alternately, those with semantic dementia lose ability to comprehend language.

While the FTD diseases share the anatomy that’s under siege, sharp differences exist in pathology. At a cell level, FTD still has no definitive signature, Onyike says. In some of the diseases, brain cells carry telltale tau or beta-amyloid proteins; others may display different inclusions or none at all, apparently. Even when FTD results from a known gene—some 40 percent is familial—there’s puzzling variety, he adds. “Members of one family can have different types of FTD.” 

“The complexity gets in the way of research and of educating people,” Onyike explains. It can also slow diagnosis, though at Hopkins, clinical expertise usually wins out. Patients, with their caregivers, initially spend two to three hours at the clinic, giving a detailed history and undergoing cognitive and neurological testing. Brain imaging and EEGs may also be scheduled.

Clinic staff additionally assess home safety and driving competence. “Driving problems, by the way, don’t typically involve forgetting where you’re going,” says Onyike. But with advancing FTD, attentional skills may change for things like timing oncoming traffic. “When judgment lapses, patients can resemble inexperienced, reckless teenagers behind the wheel.” 

The clinic provides a prognosis, aims for a safe environment, educates families and helps them anticipate future care, legal and financial issues. 

Because there’s no cure, a certain mindset defines the place. “One needs calmness and ability to accept things as they are, not as you’d have them,” says Onyike. Perhaps this comes from his growing up in Nigeria or attending medical school there years before he came to Hopkins. “I never knew the main role of medicine as providing a cure. I came to know it as providing clarity, comfort and guidance and, if you’re fortunate, a cure. If a cure’s available, you only walk a short way before you and patients shake hands. With FTD, you walk a longer distance, sometimes the whole way, with the family.”