ALS Alert Newsletter | July

On the Clinical Side

Signs and symptoms of ALS effects in the brain

Patients with ALS who also develop cognitive or behavioral problems vary widely in their symptoms. Some may have only the mildest of brain effects, such as slowness to call up words — verbs especially, one study says — or meet appointments. A smaller number have significant problems that fit the criteria of dementia.

The latter “is not Alzheimer’s,” says psychiatrist Chaidi Onyiki. “That’s a global brain disease.” The frontotemporal dementia (FTD) that affects a small number of patients is restricted to frontal and temporal areas of the brain, as the name implies. “Memory, fortunately, isn’t much affected,” he says.

Onyike heads the FTD clinic at Johns Hopkins Hospital and works closely with neurologists there who are also Packard Center researchers. Onyike sees patients referred from Hopkins’ Multidisciplinary ALS Clinic.

For ALS patients whose symptoms are mild, he says, trouble keeping track of appointments or planning for what lies ahead isn’t unusual. “And you also may not see the emotional downturn that would be understandable in having a disease like ALS,” he adds. “Patients can show an indifference to the fact that they have a serious illness or need treatment.” That’s not the same as being resigned to what’s happening, he says. Indifference and a cheerfulness that doesn’t fit are often signs.

“The earliest problems seem to be attentional,” Onyike adds. So patients may have a problem staying alert to what’s going on around them, he explains. “They don’t seem as lively or sharp.”

As for the frank dementia that few patients go on to develop: “It’s the small details,” he explains, “that give you a fine sense of frontotemporal dementia” as opposed to other types. Patients may not appreciate irony or be able to show surprise; they may turn callous or less sensitive to peoples’ emotions or become truly obstinate — something especially hurtful if caregivers don’t realize the cause. Behavior can become inappropriate or uninhibited, even promiscuous in the type of FTD most common to a small number of ALS patients.

Is there help? “Yes,” says Onyike. It comes in the form of developing tactics to counter symptoms and on medications that can ease—but not cure—some of them. “Getting a diagnosis and support and knowing what to expect,” he explains, “make a real difference for patients and families.”