In September of 2000, Sara had just entered nursing school, and the combination of classes and Cody’s illness brought her to the point of exhaustion. There seemed to be no preventing his rages. At one point, Sara and Beth considered handing Cody over to the state of Kansas so that his medical bills would be taken care of, but in the end Sara just couldn’t bring herself to give up her only child.
On the morning of September 11, 2001, Cody bit a nurse’s finger so hard he broke the bone, and he wouldn’t stop screaming and beating himself, even through the early afternoon. Beth arrived at school to pick him up and said he was like a “crazed animal.” Sara and Beth held Cody down and tried to calm him all evening, but by 9:30 that night they were out of options. Beth, still working for the pediatric neurologist Samira el-Zind, called the doctor in hysterics, asking to be seen right away: “Something has to be done for this poor child! He’s going mad right in front of us!” When they met the doctor at Wesley Medical Center, Cody was immediately put on the sedative Ativan, without any results. Doctors then tried a heavier sedative, Nembutal. There seemed to be no stopping the biting, hitting, and screaming.
We know nothing about cause yet, Landau says. I think research has not been done adequately
The next morning happened to be Sara’s final exam in nursing, and so Beth sent her home just after midnight, as the doctors were administering a heavy dose of Thorazine. The Thorazine, without effect, was followed up by lithium, which also proved futile. At 2:30 a.m. the doctors finally found an effective medication, the tranquilizer Haldol. The doctor was worried that all these medications might make Cody unable to breathe on his own, but by 6:30 Cody was up and fighting, just as before. The hospital called in Mark Romereim, a pediatric psychiatrist, who ordered Cody to be put on Risperdal, eventually increasing Cody’s dosage to that of an average adult. Kore Liow, an epileptologist, was then called in to consult. He ordered a video EEG at the Epilepsy Center and, after five days of monitoring Cody, diagnosed him with Landau-Kleffner syndrome.
No one in Cody’s family had heard of Landau-Kleffner syndrome (LKS). A disorder in children often occurring between the ages of 3 and 7, LKS affects the child’s ability to understand and use language. All children with LKS suffer from abnormal electrical brain waves, just like those in Cody’s EEG. These children may suffer epileptic seizures at night, hence Cody’s awakening sick and dazed. Hypersensitivity and aggression can accompany the disorder.
Before there was LKS, there was William M. Landau, neurologist, and Frank R. Kleffner, speech pathologist, two doctors who observed six children with acquired aphasia and convulsive disorder at the Central Institute for the Deaf. Landau and Kleffner studied the effects of drug and speech treatment on the children, noting in their summary: “In all cases a severe paroxysmal electroencephalographic abnormality, usually diffuse, is observed; electroencephalographic improvement tends to parallel improvement in speech re-education.” “Syndrome of Acquired Aphasia With Convulsive Disorder in Children” was published in 1957—50 years ago this month—and caused quite a stir, opening the door for some 160 diagnoses between 1957 and 1990. It was Landau, along with John F. Mantovani, M.D., who published a follow-up study in 1980 of nine patients evaluated 10 to 28 years after the onset of LKS; the first six were from the original Landau-?Kleffner study. Landau, who is still practicing at Washington University School of Medicine, wishes more progress had been made over the past five decades. “I’m glad to have survived the past 50 years,” he says, wryly, but adds: “I’m very discouraged—we haven’t learned much at all. We know nothing about cause yet.” In terms of what went wrong, he says: “I think research has not been done adequately. We need multi-institutional organization of research programs that will examine potential causes and potential treatment in a systematic way. I’d like to tease the current population of potential investigators to get off their butts.”
“The hallmark feature of Landau-Kleffner syndrome is a loss of receptive and expressive speech and language skills,” says Sharon Willig, associate director of speech-language pathology for the American Speech-Language-Hearing Association (ASHA). The inability of those affected to understand the spoken word eventually hinders their own language skills, thereby rendering most of these children gradually or suddenly mute. It’s for this reason that LKS children are often misidentified as developmentally delayed or possibly hearing impaired.
