Brain Single Photon Emission Computed Tomography Imaging in Landau‐Kleffner Syndrome

Abstract

Five right-handed children with Landau-Kleffner syndrome (LKS) who had disease onset between the ages of 3 and 9 years were studied with EEG and single-photon emission computed tomography (SPECT) before and, in four cases, after 6 months of corticosteroid treatment. EEG findings included both focal and generalized spikes as well as spike-wave discharges with bilateral temporal predominance. These increased markedly during sleep in 1 child, and continuous spike-and-wave complexes appeared during slow-wave sleep in another patient. Neuropsychological testing demonstrated verbal auditory agnosia. Magnetic resonance imaging (MRI) was performed in 4 children and was normal. Brain SPECT imaging demonstrated abnormal perfusion in the left temporal lobe in all patients. The response to corticosteroid therapy was mixed. Our findings reinforce the concept that LKS is a functional disease affecting the language-dominant brain areas. We conclude that SPECT imaging may be of diagnostic assistance in the evaluation of this syndrome of unknown etiology.

Atypical Evolutions of Benign Localization‐Related Epilepsies in Children: Are They Predictable?

“…Abnormalities were found in the temporal lobes of patients with LKS but not in children with BCECTS (82)(83)(84)(85).…”

Síndrome de Landau-Kleffner e regressão autística: a importância do diagnóstico diferencial

“…Estudos funcionais, tais como tomografia por emissão de pósitron (PET) e tomografia por emissão de fóton único (SPECT) têm mostrado alterações funcionais (hipoperfusão) nos lobos temporais, sobrepondo-se à região preferencial das descargas epileptiformes ao EEG 10,11 . O estudo com SPECT interictal não revelou as alterações observadas; deve-se, entretanto, ressaltar que este exame foi realizado após sedação do paciente com anestésico, o que pode ter resultado em um falso-negativo 10 .…”

“…O estudo com SPECT interictal não revelou as alterações observadas; deve-se, entretanto, ressaltar que este exame foi realizado após sedação do paciente com anestésico, o que pode ter resultado em um falso-negativo 10 .…”

Landau Kleffner Syndrome: Neuroradiology Aspect

“…Axial computer tomography and magnetic imaging are always normal, although non-specific finding, such as white matter changes or structural damage, have been reported (Perniola,et al,1993). In a small number of patients single photon emission computed tomography and positron emission tomography have revealed perfusion and glucose metabolism abnormalities in the temporal lobes (Luat,et al,2006;Guerreiro,et al,1996;Da Silva et al,1997).There have been case report about the use of MEG in patients with LKS (Sobel,et al,2000). Magnetoencephalography can provide excellent pre-surgery localization of epileptiform activity as well as functional language mapping if surgical treatment, such as multiple subpial transection, the role MEG in evaluating patients with epilepsy continues to evoive.…”