Dr. Victor M. Pedro†
Department of Clinical Sciences University of Bridgeport, Bridgeport, CT, USA and Victor Pedro Institute Cranston, R.I. USA
Department of Psychology The College of Staten Island of the City University of New York Staten Island, NY 10314 USA
Center for Clinical Ergonomics and Rehabilitation Sciences
School of Engineering and the Faculty of Health Science
Ben Gurion University of the Negev, Be’er Sheva, Israel
A case study is presented of a 14-year old right-handed Caucasian female diagnosed with the Landau-Kleffner Syndrome (LKS) at the age 3½ years. Her LKS symptoms presented with abrupt disruption in language after normal development, electroencephalogram (EEG) brain-wave abnormality, seizure activity, inability to read, and impairment in her motor skills. After 11 years of pharmacological and special education interventions with no significant improvement in any measurable area of function, a multimodal approach using techniques purportedly aimed at facilitating inter-hemispheric communication was provided. At completion of the program, EEG was controlled, reading, language, and auditory processing improved and objective behavioral-social measures improved significantly.
KEY WORDS: Landau-Kleffner Syndrome, hemispheric integration therapy
Landau-Kleffner Syndrome (LKS) also known as acquired epileptic aphasia was introduced in 1957 when Landau and Kleffner first reported the cases of six children with developmentally normal language functions who then developed aphasia in association with a convulsive disorder (Sobel et al., 2000). The syndrome appears in children between the ages of three and seven years and is identified with abnormal EEG brain waves, an interruption in language and other higher cortical functions, and is associated with epilepsy and spike-wave discharges that increase during sleep (Landau & Kleffner, 1957). Case studies have noted that males are twice as likely as females to acquire LKS (National Organization for Rare Disorders, 1995), with the cause remaining unknown. Symptoms also include behavioral disturbances that are apparent in two-thirds of LKS cases and they range from aggression and attention disorders to autistic like behaviors such as such as avoidance and withdrawal, resistance to change in daily activity, gestural stereotypes, echolalia and echopraxia, hyperlexia, and even psychotic-like disturbances (Zivi et al., 1990). These symptoms can lead to misdiagnosis, which makes the EEG examination an important determinant in the differential diagnosis.
The LKS literature points out clear variability in the presentation of symptoms of severity, duration, and comorbidity. The presentation of various symptoms in some reports correlate and in others do not with the presentation of aberrant EEG, seizure activity, and symptoms of aphasia or related manifestations (i.e. various nonverbal signs have been associated with LKS including reports of agnosia for non-speech sounds). The child may appear deaf, yet audiograms are generally normal (Feekery et al., 1993; Lanzi et al., 1994; Paquier et al., 1992). Intellectual capacity deteriorates in some of the children (Lanzi et al., 1994; Paquier et al., 1992; Roulet et al., 1991). Behavioral disturbances are frequent and may include attention disorders, hyperactivity, aggression, apathy, or depression (Bishop, 1985; Dugas et al., 1982; Soprano et al., 1994), even autistic (Rapin, 1995; Van Hout, 1997) or psychotic regression (Roulet et al., 1991). Manual and oral dyspraxia (Bulteau et al., 2000) visuo-spatial deficits (Bulteau et al., 2000) have also been reported in some children.
The onset of LKS may be sudden or insidious (Deonna, 1995; Soprano et al., 1994). In rare instances precipitating events, such as rubella (Veggiotti et al., 1995) have been reported. The pathogenesis of LKS is not known.
The extent to which the aphasic symptoms are the result of the epileptic activity is controversial (Deonna, 1995). In many children, EEG recordings have demonstrated spikes during slow sleep (Deonna, 1995; Lanzi et al., 1994; Paquier et al., 1992; Roulet et al., 1991) and seizure control is reported to be unrelated to aphasic symptoms (Feekery et al., 1993). EEG abnormalities are reported predominantly over bi-temporal, temporal-parietal, and parietal-occipital leads (Feekery et al., 1993; Nakano et al., 1989).
MRI scans are normal in most cases (Bureau, 1995), but PET and SPECT studies have demonstrated focal or multifocal changes or perfusion asymmetries predominantly affecting, but not restricted to the temporal regions (Maquet et al., 1995; Mouridsen et al., 1993; O’Tuama et al., 1992). A primary symptom in LKS is considered to be impaired phonological decoding and expressive disorders are seen as secondary or concomitant (Lanzi et al., 1994; Soprano et al., 1994). However, agnosia for non-speech sounds has also been reported. It is therefore possible that the deficit underlying aphasia will be a generalized auditory amnesia instead of a phonological decoding deficiency, a view supported in a study by Klein and colleagues (1995). Young adults with acquired aphasia were shown to have slowed reaction times and a delay of certain components of evoked potentials in response to both non-verbal and phonological stimuli. The results contradict, to some extent, those obtained by Frumkin and Rapin (1980) and Notoya et al. (1991) of poorer perception of consonants than of vowels. The findings would indicate that there is some degree of specificity of impairment within the auditory domain.
Research has weighed an important focus on auditory disruption, which is an integral focus in the Cortical Integrative Therapy. The primary deficit of the receptive aphasia is considered to be impaired phonological decoding, while expressive disorders are seen as secondary (Lanzi et. al, 1994; Soprano et al., 1994 as taken from Korkman et al. 1998).