Case of Landau‐Kleffner Syndrome Secondary to Inflammatory Demyelinating Disease

Abstract

A 6-year old girl developed acquired aphasia with epilepsy and a paroxysmal EEG (Landau-Kleffner syndrome). Isoelectric CSF focusing showed oligoclonal IgG bands. Small lesions were visualized in periventricular left frontal white matter and right parietal lobe centrum semiovale with magnetic resonance imaging (MRI). After a week of ACTH therapy, the EEG paroxysmal activity disappeared; during the next few months, the language disorder improved. Further MRI examination showed a decrease in size and signal of the left frontal lesions, with localized white matter atrophy, dilatation of the subarachnoidal spaces, and disappearance of the right parietal lesion. The clinical and neuroradiologic features and the laboratory data suggest an acute disseminated encephalomyelitis.

The Landau-Kleffner syndrome: A review

“…However, the biopsies performed by McKinney and McGreal (1974) and Dugas et al (1982) were normal, as well as the surgically removed material by Cole et al (1988). Recently toxoplasma gondii infection (Michalowicz et al, 1988(Michalowicz et al, -1989, otitis media (Gordon, 1989), neurocysticercosis (Oter0 et al, 1989), cerebral arteritis (Pascual-Castroviej0 et al, 1992), inflammatory demyelating illness (Pemiola et al, 1993), temporal lobe tumor (Solomon et al, 1993;Nass et al, 1993) has also been postulated as a possible cause of LKS, following concomitant occurrence of these conditions and LKS. Benefits from corticosteroid treatment (McKinney & McGreal, 1974;Kellerman, 1978;VanDe SandtKoenderman et al, 1984;Marescaux et al, 1990;Lerman et al, 1991) may reveal an autoimmune process as the underlying pathology.…”

Landau Kleffner Syndrome: Neuroradiology Aspect

“…Neither the evoked potentials nor auditory test have contributed useful data for diagnosis (Zouari & Choyakh, 1997;Pablo et al, 2002). The analysis of cerebral fluid is almost always normal, although case in which a slight elevation of total protein has been reported (Perniola,et al,1993). 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).…”

“…The analysis of cerebral fluid is almost always normal, although case in which a slight elevation of total protein has been reported (Perniola,et al,1993). 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).…”

Autistic regression and Landau‐Kleffner syndrome: progress or confusion?

“…Clinical improvement has also typically followed suppression of epileptiform activity by anticonvulsants, corticosteroids, and surgical elimination of epileptogenic discharge 40,47,48 . The prognosis of LKS is variable but generally better in those with fluctuating aphasia and worse in those with onset before ageIn LKS, standard brain imaging with computerized axial tomography or magnetic resonance imaging is usually normal, although there have been rare associations with localized infections, demyelination, and tumors [51][52][53] . Case series of children with LKS using positron emission tomography and SPECT have shown abnormalities of glucose uptake and cerebral perfusion in the temporal lobes [54][55][56][57] .…”