Landau-Kleffner syndrome (LKS) is a rare neurodevelopmental disorder that affects language and behaviour in children between the ages of 3 and 7 years.
Children with LKS often show normal development before diagnosis, although some have delayed language milestones. LKS commonly begins with difficulty understanding spoken language. In some cases speech is also affected. Seizures are usually infrequent and sleep-related. An EEG can be useful in both diagnosis and the analysis of Electrical Status in Slow-Wave Sleep (ESES). These sleep activation discharges can become continuous in LKS. Treatment of LKS can be difficult and the prognosis is quite variable.
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Case Study: Ali’s story
Our daughter Ali was a perfect baby and little girl until she got her first ear infection. We went to the pediatrician and he put her on antibiotics. After a week, Ali still wasn’t feeling well. We went back and Ali still had an infection so he changed the antibiotic. Ali wasn’t getting better so I called an ENT. We brought her right over and tubes were placed in her ears the next day. Everyone said Ali would get better but she never did. We tried everything and we asked everyone if they ever saw a normal child lose her speech and could not fight infections. In May of 1995, our search came to an end and Ali was diagnosed with Landau Kleffner Syndrome.
It was bitter sweet: we now had a diagnosis but there was no cause or a definite treatment for LKS. The doctor put Ali on 2 rounds of high level steroids. We saw a little improvement in her speech but our little girl was trapped in her body. You could see her trying to get the words out but nothing would come out. We tried many years of speech therapy and special educational programs but Ali never got better. Over time Ali lost her speech. It was a sad slow process. When Ali was 10 years old, she had 14 grand mal seizures in 12 hours. Our neurologists told us that there was nothing that they could do for Ali but to keep her comfortable. I knew that it wasn’t Ali’s time to leave us. Ali woke up 3 days later and could not even hold up her head, but Ali’s eyes were bright and her smile was big. I stayed with Ali for 5 weeks in two different hospitals and Ali worked so hard day and night to walk out of the hospital on that brisk fall day in October.
Through puberty and middle school Ali had many seizures and was very sick but her eyes and smile were still bright and big. We saw a lot of specialists all working together as a team. Ali had all the new brain scans that were coming out. We never left one stone unturned. When it all came together the doctors agreed on one thing, to do everything that we want to now and don’t wait. This wasn’t the first time I heard this and we decided as a family to live and for Ali to live and make every day the best. As long as Ali was happy, we were happy. Happy to us means love, laughter, Ali’s pain under control, no hypoglycemia and Ali’s seizures under control.
Ali knew everything that was happening but couldn’t express herself with words. Ali’s nonverbal communication became very strong. Ali always let you know what she wanted to say, with her communication device or her big and beautiful smile. Ali loves to go to school and be with friends. When Ali turned 20 years old, things started to get a little easier. Ali loved school and being with her friends. Ali was able to communicate with her IPAD. Ali went to all of her proms, graduated High School and is a cheerleader for the BIG 33 football game. Ali goes to Travel Club, a community based day program and loves every minute of it. Ali’s smile tells us her health status; if Ali is happy, everyone is happy.
Ali had accomplished things in life that her doctors never thought that she would do or even be alive to do. Everybody loves Ali. Ali brings out the best in others in the fewest number of words. Ali lives every day to the fullest. Ali’s only consistent word is “please”. How lucky are we to walk the path of life with Ali? It’s the best and Ali is our greatest teacher!
It’s been a hard road for all of us, the Super 4. Why the Super 4 because every day Ali’s dad, mom and sister need to power up to get through the day because we don’t know what the day will bring for Ali. That’s the hardest part, the roller coaster. Ali’s life/health is consistently inconsistent. Ali has focal and partial seizures every day. Ali’s seizure triggers are identified and we address them when they happen and that is hard. It’s hard to be prepared for the worst. We take lemons and make lemonade and we don’t live for Ali to die but we live for our family to live. We have so much love for each other and we are a very happy family. Isn’t that what life is all about?
If your child has recently been diagnosed with LKS, never give up. Never leave any stone unturned. Ask your doctors questions, be an advocate for your child at school and in their community. Laugh and love your child like they are “normal”. Be there to help them with their frustrations and pain. Treat all of your children the same and make special time for your other children. Never give up hope because one day you could meet a very special person whose research finds a gene, a cause, a treatment or a cure for LKS.
President, Ali Paris Fund for LKS Research and Education