Landau-Kleffner Syndrome

My childhood with LKS, starts in spring 1984 and worn out mid fall 1995. Just in time before goes to junior high school and learns to drive car. My communication skills are getting better one step at time since 1995 and will takes while to full recovery. I continues work hard on my best to gets grammer and communication better. I'm 29 year old, still have little trouble with grammer because people believes my grammer act likes third grade student. I can't help it, thats way I am but getting better at it anyway.

Understanding Landau-Kleffner Syndrome
by Timothy Lesaca, M.D.
January 2000, Vol. XVII, Issue 1

The loss of language in childhood, particularly when verbal comprehension is affected, creates a catastrophic barrier to normal development. One uncommon yet debilitating language disorder is Landau-Kleffner syndrome (LKS), also known as acquired epileptiform aphasia. There have been over 160 cases of LKS reported in the medical literature (Tuchman, 1997).
Landau-Kleffner syndrome is associated with epileptic manifestations and electroencephalogram (EEG) abnormalities. LKS's epileptic nature may not be apparent unless an EEG is performed; consequently, many children with LKS will present initially to providers of psychiatric care due to language and behavioral difficulties. Behavioral problems such as hyperkinesis and aggressiveness have been associated with LKS, but are thought to be secondary to the child's frustrations in dealing with the loss of communication skills.
The history of LKS began in 1957 when Landau and Kleffner described six children with unusual aphasia without a definitive etiology. In most LKS cases, the aphasia appears after normal language development, with auditory verbal agnosia and reduction of spontaneous speech developing between 3 and 7 years of age. Children exhibit progressively decreased responsiveness to verbal language and eventually become unresponsive to verbal communication. This loss of language can occur abruptly or insidiously, and it can be marked with remissions and exacerbations.
Many children with LKS use gestures and other forms of nonverbal communication to effectively express their needs (Deonna, 1991). Those children with LKS who do not lose all verbal communication skills are able to use very simple sentences, but sometimes will manifest complete aphasia. Older children with reading and writing skills who develop LKS have sometimes been shown to lose these skills as well. LKS initially presents after 9 years of age in only 5% of cases, and only very rarely does it present after 12 years of age. Males are affected twice as often as females.
While LKS is, by definition, always associated with an abnormal EEG, it is important to note that 25% of children with LKS do not have seizures (Tuchman, 1997). It is unclear if there is a causal relationship between the aphasia of LKS and the associated EEG abnormalities and seizures (Gordon, 1997; Nass et al., 1998).
The EEG abnormalities seen in LKS are nonspecific and consist of a variety of epileptiform patterns, including continuous spike waves during slow sleep, focal sharp waves with spikes and centrotemporal spikes (Nass et al., 1998). At some time during LKS development, all patients have bilateral spike waves for more than 85% of the sleep period (Deonna, 1991). Although there is variation in the topography of the main discharge focus, most studies find predominance in the temporal or parietal regions. A single normal tracing, particularly during wakefulness, does not rule out the diagnosis, since discharges are not always present or might occur only during sleep (Paquier et al., 1992).
The clinical seizure activity associated with LKS is heterogeneous and seizure frequency is quite variable. Generalized tonic-clonic, focal motor, nocturnal simple partial motor, atypical absence, myoclonic-astatic and atonic seizures have been reported. It is unusual for the seizures to become difficult to control, and they usually resolve by 15 years of age. A family history of epilepsy is found in 12% of LKS cases (Gordon, 1990).
The precise etiology of LKS is not well understood. No demonstrable structural pathology has been consistently seen in children with LKS. Audiography, auditory evoked potentials and neuroimaging studies are typically normal, although studies using positron emission tomography have revealed metabolic abnormalities in the temporal lobes (da Silva et al., 1997).
It is thought that the aphasia of LKS might be caused by demyelinating or encephalopathic processes, infectious or inflammatory illnesses, unilateral brain lesions, or autoimmune diseases. The most compelling etiologic theory, however, relates back to Landau and Kleffner's original article. In it, they advanced the notion that persistent abnormal electrical discharges in brain areas associated with linguistic communication result in the functional ablation of those areas (Landau and Kleffner, 1957). As a consequence, recently acquired language skills that have not yet become fully impressed into long-term memory can be lost, leading to impaired or regressed language abilities. This might explain why children who develop LKS after 5 or 6 years of age have a better prognosis than those who acquire it at a younger age.
EEG and other clinical similarities between LKS and epilepsy with continuous spike waves during slow sleep (CSWS) are further evidence of an epileptogenic etiology for LKS. These disorders differ, however, in that CSWS is associated with behavioral, intellectual and learning difficulties rather than with language disturbances. Also, the characteristic EEG of CSWS has a predominantly frontal focus.
Although the diagnosis of LKS should be considered in cases of arrested or regressed language development associated with EEG abnormalities or seizures, it must be differentiated from other more common disorders that are also identified with communication problems. For example, audiometric testing can rule out hearing loss and deafness. Selective mutism would manifest only in particular social situations rather than globally. Children with autism or autistic spectrum disorders do not demonstrate the sociability and proficiency at nonverbal communication seen in children with LKS. Finally, developmental language disorders and mental retardation do not generally present after the loss of previously acquired functioning.
If epileptic activity is accepted as a probable etiology for LKS, then there are strong reasons to advocate antiepileptic medication for its treatment (Gordon, 1997). These agents have disappointing results, however, as they often alleviate the seizures but not the aphasia (Paquier et al., 1992).
The lack of efficacy of antiepileptic medication has led to antidotal trials of numerous other treatments, including corticosteroids, subpial resection, calcium channel agents and adrenocorticotropic hormone (Gordon, 1997; Paquier et al., 1992). These treatments also have mixed results, yet they appear to be more effective than the antiepileptics. Most recently, the use of intravenous immunoglobulins has demonstrated some promise in improving both the clinical and EEG abnormalities of LKS and will likely be a direction for future research (Lagae et al., 1998).
The prognosis for children with LKS is quite varied and is probably related to age of onset. Early age of onset, particularly prior to 5 years of age, implies a more severe epileptic tendency, a longer duration of symptoms and a more guarded prognosis for recovery of language. Some affected children never fully recover, while others completely regain speech over the course of months or years. Improvement, when it does occur, typically is seen toward late childhood or early adolescence. About one-third of children with LKS make a good recovery. Early recognition and intervention yield the best hope for a favorable prognosis.
Dr. Lesaca is a child and adolescent psychiatrist with Heritage Valley Healthcare Systems in Sewickley, Penn.