Treatment for both Landau-Kleffner syndrome (LKS) and autistic epileptiform regression (AER), when instituted, has generally consisted of traditional antiepileptic drugs and steroids or ACTH, immunoglobulins,119,120 and calcium channel blockers.
Adrenocorticotropic hormone (ACTH) probably has the greatest efficacy, although detailed prospective outcome studies are lacking.28,94,112 Some have suggested that AER and infantile spasms lie on the same spectrum, given their phenotypic overlap. If this is true, the neuroendocrine underpinnings of infantile spasms121 provide the backdrop for the efficacy of ACTH and, possibly, steroids. Several investigators suggest that early treatment is extremely important.92,94
Surgical treatment has also been tried recently. Magnetoencephalography (MEG) provides useful presurgical information about the cortical spike dynamics in LKS patients.45 Morrell et al.81 first used multiple subpial transection (MST) in 14 children with aphasia, seizures, and markedly abnormal EEGs. Of the 14 patients, 7 recovered age-appropriate speech, are in regular classes in school, and no longer require speech therapy. Another 4 showed marked improvement and were speaking and understanding verbal instruction, but were still receiving speech therapy. Thus, three-fourths of the patients, none of whom had used language to communicate for at least 2 years, improved significantly after MST.
Sawhney et al.122 reported on the efficacy of MST in 21 patients, 3 of whom had LKS. MST was carried out mainly in the precentral and postcentral regions. The three patients with LKS, who were mute before operation, exhibited substantial recovery of speech.
Gillberg et al.123 also reported positive responses to surgery in two preadolescent autistic boys with tuberous sclerosis. One had a corticectomy in addition to the MST.
Neville et al.124 reported two preschoolers with autistic regression and intractable epilepsy. One improved after left temporal lobectomy and the other after MST.
In the series by Grote et al.,125 11 of 14 children demonstrated significant postoperative improvement on measures of receptive or expressive vocabulary. Results indicate that early diagnosis and treatment optimize outcome and that gains in language function are most likely to be seen years, rather than months, postoperatively. The best predictor of postoperative improvements in language function was the length of time since surgery.125
In one of the patients of Rintahaka et al.,117 continuous spikes and waves during slow sleep (CSWS) disappeared and language function improved after MST surgery.
Nass et al.126 reported 7 patients (derived from a cohort of 36 children who had been referred originally for video EEG monitoring) with refractory epilepsy and AER who responded to MST in varying degrees after medical management had failed. Surgical treatment variously involved MST of the left neocortex in temporal, parietal, and frontal regions, often including regions within the classic perisylvian language areas. One patient also had a left temporal lobectomy. In all 7 patients, seizure control or EEG features (or both) improved after MST. Language, social, and overall behavior improved to a moderate degree, although most improvements were temporary.
In another cohort, 12 of 18 AER patients showed improved behavior or language ability postoperatively, despite the multifocal nature of the epileptiform activity on MEG.44
Reviewed and revised May 2004 by Steven C. Schachter, MD, epilepsy.com Editorial Board.
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