Childhood Neurological Disorders
- Association of Child Brain Research
- Aug 17, 2021
- 3 min read
Updated: Aug 17, 2021
CASE 1:
A 3.2 year old girl was observed with stuttering. She was diagnosed as having benign myoclonic epilepsy of infancy at 3.2 months aged and treated with valproate. Her electroencephalogram (EEG) returned to normal at the top of the primary year. The therapy was stopped after a 2 year seizure-free period. She began to stutter prominently 3 months after the discontinuation of anti-epilepsy drugs. She had no verbal agnosia. Her EEG revealed multiple spike and wave discharges.
She was diagnosed as having Landau Kleffner syndrome. Her previous epilepsy history had contributed to her having obtained an EEG within the early period.
Things to remember:
If a toddler with normal language function starts to stutter, it's going to be related to convulsive seizures and extra difficulties with behavior, social interaction, motor skills, and learning. it's not usually life-threatening but can impact greatly on the quality of life unless it responds well to treatment. It occurs approximately in one child in a million. The disease is more common in boys and doesn't usually run in families. Landau Kleffner syndrome (LKS) may be rare epilepsy. It occurs in children usually between the ages of three and nine years and is characterized by loss of language skills and silent electrical seizures during sleep.
SYMPTOMS:
LKS could also be mentioned as Acquired aphasia of childhood with seizure, Epileptic aphasia, and verbal agnosia. In LKS, the child generally loses language understanding then speech. This loss could even be sudden (even overnight), or gradual over a period of months and is typically mistaken for deafness initially. Many children compensate by using visual cues, gestures, and by copying others and will initially hide their difficulty. The deterioration in skills is known as a regression because the kid appears to have returned to an earlier stage in their development. LKS was initially thought to be specific to language, but it's now clear that other abilities are also often affected. Behavioral changes are commonly reported and will include overactivity, reduced concentration span, irritability, tantrums, and difficulties with social interaction. Nonverbal thinking (cognitive) skills are usually relatively unaffected, although there could even be an isolated difficulty, as an example with thinking speed.
The child also can have problems with fine motor coordination and movement, like dribbling, messy eating, loss of speech clarity, clumsiness, and shakiness. These difficulties are thought to be an instantaneous result of the disease process, rather than simply an emotional reaction to the loss of language. It's hard to understand what the kid experiences, but some later have described being conscious of muffled sounds but were unable to form any words, or other unusual auditory experiences just like the sound of wind rushing or indistinct loud voices. Most children have clinically obvious seizures and these often start before the initial regression. Seizures are generally short and do not show a thorough relationship to language difficulties.
DIAGNOSIS:
This syndrome is extremely difficult to diagnose and it's often misdiagnosed as autism, pervasive development disorder, learning disorder, hearing disorder, auditory or verbal processing disorder, childhood schizophrenia, intellectual disability, attention deficit hyperactivity disorder, or emotional or behavioral problems. In many cases of patients that are exhibiting LKS, there'll show tons of abnormal brain activity in both the proper and therefore the left hemispheres of the brain which occurs frequently when sleeping. The best way to confirm the presence of LKS is by obtaining overnight sleep EEGs and EEGs together sleeping stages should be recorded. In some conditions like demyelination and brain tumors, they will be ruled out with the utilization of resonance imaging (MRI).
PROGNOSIS:
Landau Kleffner Syndrome affects some parts of the brain that help in controlling comprehension and speech. The parts of the brain that carry these functions include Broca’s areas and therefore the Wernicke’s areas. This disorder mostly occurs in children between the ages of three to seven years. There's slight male dominance within the diagnosis of the syndrome. The ratio to which this syndrome occurs between males to females is 1:7. Children with Landau Kleffner syndrome grow and develop normally, but with time they lose their language skills. Many of the affected individuals usually have clinical seizures while others experience only electrographic seizures that include electrographic epilepsy of sleep (ESES). The prognosis varies in several cases. Some affected children may have permanent severe language disorder while others regain language abilities after a while. The prognosis is improved when the onset of the disorder is after the age of six and when therapy is started early.
REFERENCES:
Tutuncuoglu Sarenur, Serdaroglu Gul and Kadoglu Bengu (2002) Landau-Kleffner Syndrome Beginning With Stuttering: Case Report, (Volume 17, Issue 10, 2002), Journal of Child Neurology, Sage Journals.
Article “Landau Kleffner Syndrome”, in NHS Foundation Trust, Great Ormond Street Hospital for children.
Attend upcoming Workshop on - Epilepsy in Children by International Faculty
Case from- Dr. Rahul Bharat's Paediatric Neurology Clinic
Written by- Ms. Divya Khare
Edited by Dr. Uzaina
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