Neurologist William Landau
Image courtesy of Dr. Landau
As with many other epileptic syndromes, LKS children often resume normal brain activity around age 15, when the brain cells are reaching toward maturation, perhaps spurred by hormonal change. Unlike similar conditions, children with LKS are often left with neurological impairments, even after the electrical abnormalities have returned to normal. The neurological effects of LKS on a child can range anywhere from minimal to severe. For their parents, it’s the hardest waiting game they’ll ever have to play.
“I don’t know if it’s mysterious, but it sure is obscure,” says Kleff-ner, who prefers to be called “Frank.” Retired since 1991, Kleffner, now 82, moved on from LKS years ago and now finds it “at times a nuisance.” Of his famed publication he says with humility, “I really didn’t think at the time it was any great accomplishment.” He credits Landau as being the foreseer of its impending significance, the real pioneer. “A few years after our paper, we got labeled a syndrome,” he says, emphasizing the last word of his sentence with slight disdain. “I got a call from Landau one day asking me if I’d heard of Landau-Kleffner syndrome, and I thought he was kidding. Apparently he’d found it in a medical journal!” Kleffner now considers LKS “a badge of eponymic ignorance [the title of a paper by Landau]. We don’t know much more now than we did then.” To even attempt to research and write an article of this kind just “opens up a can of worms . . . you’re only going to come up with loose ends.”
Decades of continuous speculation have caused many scientists to refrain from offering opinions on LKS’s cause and curability. One doctor who remains undeterred is Josiane LaJoie, a pediatric neurologist specializing in epilepsy surgery and treatment at New York University Medical Center. LaJoie has treated patients with LKS since 1999 and believes these children have an “inherent brain susceptibility. There is activation of abnormal circuits in the brain that prevent the normal function of the affected brain cells. The presence of these abnormal circuits may also inhibit the natural process of removal of unnecessary developing brain cells known as pruning. While we are asleep, the events and the experiences of the day are recorded as memory. The abnormal brain circuitry of LKS patients renders the brain incapable of imprinting memory, further impairing brain function. The areas affected are primarily responsible for speech.”
The past 50 years of study and research have brought few concrete answers. “Much has changed since that landmark paper was published in 1957,” LaJoie says. “We have subsequently learned that similar EEG findings can be found in a variety of patients with different clinical symptoms. Because of this, the margins of what defines a clear-cut LKS patient have been blurred.” Kleffner himself, in his interview for this article, agreed, expressing concern that many LKS children were actually misdiagnosed. “Some use the term LKS to describe any patient with either language regression and/or EEG findings similar to those in LKS,” LaJoie continued. “These patients may also have other symptoms not found in the typical patient with LKS. It is well known that children on the autistic spectrum can also undergo a similar regression and have EEG findings similar to those found in LKS patients. There is much debate about whether these patients should be classified as LKS or a variant of LKS. Just as with any medical condition, there is a spectrum of clinical symptoms among LKS. With increased awareness of vast possibilities of presentation of these patients, more patients are properly diagnosed and treated. However, not every patient will follow exact guidelines, making clinical decisions and classifications more difficult.”
LaJoie believes that steroids are the best treatment for a patient’s language issues. “If unsuccessful,” she adds, “other options include intravenous immunoglobulin therapy or possibly surgery. Multiple subpial transections [MSTs] are a type of disconnection procedure that allows the preservation of function of brain cells but prevents the spread of abnormal electrical impulses. MSTs have been found to be helpful in the recovery of language in LKS patients. Although MSTs are as invasive as they are controversial, it is sometimes a child’s best chance at a functional recovery.”
