Is Cerebral Arteritis the Cause of the Landau-Kleffner Syndrome? Four Cases in Childhood with Angiographic Study

Abstract

ABSTRACT:Four children with Landau-Kleffner syndrome were studied over a six year period. They presented with acquired aphasia, epilepsy, and focal or generalized EEG discharges which were exacerbated during sleep. In addition, cerebral angiography demonstrated isolated arteritis of some branches of the carotid arteries in all cases. Computed tomographic and magnetic resonance images were normal. Nicardipine in a dose of 1 to 2 mg/kg/day, added to conventional anticonvulsant drugs provided effective supplementary control of seizures, of paroxysmal EEG discharges, and of language and behavioural disturbances, even several years after the onset of the disorder and in patients whose response to other medications, including steroids, had been poor. Interruption of nicardipine administration was followed by relapse of the language disorder. Repeat angiography was performed in all four patients and showed recanal-ization of obstructed vessels in two cases. Focal cerebral vasculitis may be the pathogenesis of the Landau-Kleffner syndrome and calcium channel blockers such as nicardipine may be effective and specific therapy.

Response to immunotherapy in a patient with Landau‐Kleffner syndrome and GRIN2A mutation

“…Autoantibodies to myelin (Nevsímalová et al, 1992), to CNS endothelial cells (Connolly et al, 1999), and to rat brain auditory cortex (Boscolo et al, 2005) have been found in children with LKS. Moreover, LKS has been linked to other disorders of autoimmunity, including inflammatory demyelinating disease (Perniola et al, 1993) and cerebral arteritis (Pascual-Castroviejo et al, 1992). LKS has been found to respond to both steroids and IVIG (Mikati et al, 1998(Mikati et al, , 2000(Mikati et al, , 2002(Mikati et al, , 2010Gallagher et al, 2006;Geva-Dayan et al, 2012).…”

The Landau-Kleffner Syndrome

“…Other biopsied cases did not have encephalitic changes (22,39). Angiography has occasionally suggested isolated arteritis of small and medium‐sized vessels in some studies (40), but not others (22,37).…”

Epilepsy and epileptiform EEG: Association with autism and language disorders

“…There have been anecdotal reports of successful treatment with some of the newer AEDs, such as vigabatrin [Appleton et al, 1993]. Pascual-Castroviejo et al [1992] reported improvements in four children treated with nicardipine and AED. There are also anecdotal reports of improved language functioning following treatment with intravenous immunoglobulins [Fayad et al, 1997;Lagae et al, 1998].…”