‘He Doesn’t Get Angry’ : Rare Nerve Disease, Seizures Plague Boy

PITTSBURGH —  Every week, sometimes several times a week, Doris Sitler trudges to the calendar hanging on her bathroom wall, picks up the pencil dangling from a string and scratches an X on the date. Once again, her 11-year-old son has suffered a stroke or seizure.

She has marked the X’s 194 times. Each time, she has come a little closer to losing her son, Chris, to a rare neurological disease known as moyamoya.

“He doesn’t get angry. He just gets depressed,” Sitler said on a recent trip to Children’s Hospital of Pittsburgh for tests for her son.

More Prevalent in Orient

Because moyamoya is so rare in the United States--it is far more prevalent in the Orient--few American doctors are acquainted with the disease.

Japanese neurologists believe a progressive constriction of the two carotid arteries causes abnormal vessels to form at the base of the brain, restricting cerebral blood flow and causing strokes. In X-rays, the vessels resemble puffs of smoke drifting in the air, expressed in Japanese as moyamoya .

The Handbook of Clinical Neurology, one of the few sources of statistical information on moyamoya, reports that about 1,000 people worldwide suffer from the disease. Two-thirds of them are children. More than half of the victims, for reasons unknown, are Japanese. Some improve as they grow older, while others gradually weaken because of the seizures and die of a major stroke.

Disease Still a Mystery

Dr. James Mellinger, a pediatric neurologist at the Mayo Clinic, believes that many victims are not even counted in the statistics.

“In a large number of areas of the world, Third World areas, they just die and never even are discovered to have the problem,” he said.

How Chris got moyamoya is a mystery, although Dr. Howard Yonas, a neurosurgeon at Children’s Hospital, suspects that an infection may have caused the major arteries at the base of Chris’ brain to constrict.

In artery bypasses, Yonas has twice built new vessels to Chris’ brain in hopes of increasing cerebral blood flow.

“It’s hard to know why it’s progressed,” he said. “Every time there’s a flurry (of spells), he’s not as good as before.”

Trouble Walking, Speaking

The chubby, bespectacled boy has difficulty walking and speaking. He has little use of his left hand, and a fluid buildup has enlarged his left leg and foot.

“At the moment, about the only thing available is something far from superior and that is the bypass,” Mellinger said.

Although hyperactive and asthmatic since birth, Chris led a fairly normal life, attending school and collecting animals at his home in Foundryville, Pa., until age 8.

Without warning, he suffered a stroke Aug. 23, 1984, and was left speechless and partially paralyzed on the left side.

‘Bottom Fell Out’

“When they told me he had a stroke, it hit real hard. . . . The bottom fell out of the world,” recalled Sitler, 32, who lives with Chris at her parents’ home.

After nearly a week of hospital tests, Chris was diagnosed as having moyamoya, she said.

By mid-October of that year, Chris had suffered four more strokes and seizures. On Oct. 29, neurosurgeons at Geisinger Medical Center in Danville took vessels from his temple and laid them on the surface of his brain in hope that new channels would form. None did, and the spells continued.

Seizures Increased

In March, 1986, after 115 strokes and seizures, Chris underwent his first artery bypass in which Yonas took vessels from the boy’s scalp and sewed them onto the arteries on the left side of the brain. The procedure was repeated last October on the right side.

The seizures began occurring again in December. Before the bypasses, Chris had at most two spells a week. He is now averaging two to six a week.

Doctors tell her Chris might continue to have mini-strokes or could someday suffer a fatal one.