Pulse Methylprednisolone Therapy in Landau-Kleffner Syndrome

Aykut-Bingöl, Canan; Arman, Azim; Tokol, O.; Afşar, Nazire; Aktan, Sevinç

doi:10.1016/0896-6974(96)00026-6

Cited by 9 publications

(8 citation statements)

References 7 publications

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“…Although the Cochrane review on corticosteroids for childhood epilepsy other than epileptic spasms found no evidence for their efficacy or safety in treating childhood epilepsies (Gayatri et al, 2007), our results, along with those of other case reports and retrospective studies on this therapeutic option (Aykut-Bingol et al, 1996;Tsuru et al, 2000;Okuyaz et al, 2005; Steroid pulse therapy in epilepsy © C I C E d i z i o n i I n t e r n a z i o n a l i Sevilla-Castillo et al, 2009;Lichtenfeld et al, 2010;Heyman et al, 2012), confirm the usefulness of this approach. In particular, Heyman et al (2012) found clinical and electroencephalographic improvements in more than 50% of patients; both Aykut-Bingol et al (1996) and Tsuru et al (2000) reported suppression of EEG abnormalities and language improvement in Landau-Kleffner patients. The same results were found by Okuyaz et al (2005) in their patient with continuous spike-and-waves during slow-wave sleep (CSWS) and partial epilepsy.…”

Section: Discussionsupporting

confidence: 53%

“…However, the available evidence is limited, since it comes from uncontrolled trials and case reports and there is, as yet, no consensus on treatment dosages and durations (Veggiotti et al, 2012). Over the past two decades, high-dose intravenous corticosteroid pulse therapy (an approach already used to treat several neurological syndromes such as multiple sclerosis) has been used in various studies in the field of epilepsy in order to avoid the development of side effects and maintain long-term efficacy (Aykut-Bingol et al, 1996;Tsuru et al, 2000;Okuyaz et al, 2005;Sevilla-Castillo et al, 2009;Lichtenfeld et al, 2010;Mytinger et al, 2010;Heyman et al, 2012;Almaabdi et al, 2014). Administered according to different regimens (dosages ranging from 15 mg/kg/day for three or five days to 30 mg/kg/day for two or three days), repeated at different intervals (weekly or monthly) (Donetti Dontin et al, 2013), it has been shown to be successful in different disorders (Landau-Kleffner syndrome, absence seizures, infantile spasms, epileptic encephalopathies in general).…”

Section: Introductionmentioning

confidence: 99%

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Intravenous methylprednisolone pulse therapy for children with epileptic encephalopathy

Pera

Randazzo

Masnada

et al. 2015

Functional Neurology

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SummaryThe aim of this retrospective study of children affected by epileptic encephalopathy was to evaluate seizure frequency, electroencephalographic pattern and neuropsychological status, before and after intravenous methylprednisolone therapy. Eleven children with epileptic encephalopathy were administered one cycle of intravenous methylprednisolone (15-30 mg/kg/day for three consecutive days, once a month for four months) in addition to constant dosages of their regular antiepileptic drugs. The treatment resulted in statistically significant reductions of generalized slow spike-and-wave discharges (p<0.0028) and seizure frequency (p<0.013), which persisted even after methylprednisolone pulse therapy was stopped. A globally positive outcome was noted in 9/11 patients (81.8%). This methylprednisolone treatment regimen did not cause significant or persistent adverse effects. We suggest that children with epileptic encephalopathy without an underlying structural lesion could be the best candidates for intravenous methylprednisolone pulse therapy.

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Section: Discussionsupporting

confidence: 53%

Section: Introductionmentioning

confidence: 99%

Intravenous methylprednisolone pulse therapy for children with epileptic encephalopathy

Pera

Randazzo

Masnada

et al. 2015

Functional Neurology

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“…Aykut-Bingol et al [44] 500mg infusion over 3h daily for 5 days, 1/1 Improvement started 2 wks after initial therapy, and after 2mo language followed by 250mg over 2h once per month level increased and EEG abnormalities were suppressed Tsuru et al [6] 20 mg/kg/day for 3 consecutive days, 2/2 Improvement started after the first or second 3-day injection (to nearly repeated three times at 4-day intervals, normal understanding and expression of speech in one patient 1-2 wks followed by oral prednisolone 2 mg/kg/day for after the third course) 1mo then gradually withdrawn…”

Section: Methylprednisolone (Intravenous)mentioning

confidence: 98%

“…Aykut-Bingol et al [44] reported an 8-year-old girl with LKS who received intravenous high-dose methylprednisolone (500mg) over 3 hours daily for 5 days. This regimen was followed by a 250mg infusion given over 2 hours once a month.…”

Section: Prednisone (Oral)mentioning

confidence: 99%

Management of Landau-Kleffner Syndrome

Mikati

Shamseddine

2005

Pediatric Drugs

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Landau-Kleffner syndrome (LKS) is an acquired epileptic aphasia disorder in which children, usually 3-8 years of age who have developed age-appropriate speech, experience language regression with verbal auditory agnosia, abnormal epileptiform activity, behavioral disturbances, and sometimes overt seizures. There are no controlled clinical trials investigating the therapeutic options for LKS. Only open-label data are available. Early diagnosis and initiation of prompt medical treatment appear to be important to achieving better long-term prognosis.Several antiepileptic drugs have been reported to be beneficial in treating this syndrome. These include valproic acid (valproate sodium), diazepam, ethosuximide, clobazam, and clonazepam. Reports on the efficacy of lamotrigine, sultiame, felbamate, nicardipine, vigabatrin, levetiracetam, vagal nerve stimulation, and a ketogenic diet are few and more experience is needed. Carbamazepine and possibly phenobarbital and phenytoin have been reported to occasionally exacerbate the syndrome. As initial therapy, valproic acid or diazepam is often empirically chosen. Subsequently, other antiepileptic drugs, corticosteroids, or intravenous immunoglobulin (IVIG) therapy are often used. Corticosteroid therapy should probably not be delayed more than 1-2 months after the initial diagnosis. Various corticosteroid regimens including oral prednisone and, recently, high doses of intravenous pulse corticosteroids, as well as corticotropin (adrenocorticotropic hormone) have been reported to be effective in LKS. Oral corticosteroids are used more often and usually need to be maintained for a long period of time to prevent relapses. The use of IVIG has been associated with an initial dramatic response in only a few patients. In our experience, a long-term worthwhile improvement has been noted in only 2 of 11 patients. These two patients had an immediate response to IVIG initially and after relapses before eventually achieving a long-term sustained remission. Surgical treatment by multiple subpial transection, which is reserved for patients who have not responded to multiple medical therapies, has been followed in selected cases by a marked improvement in language skills and behavior. However, a widely accepted consensus about suitable candidates for this surgery and about its efficacy is still lacking. Speech therapy, including sign language, and a number of classroom and behavioral interventions are helpful in managing LKS, and should be used in all patients.

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“…Other steroid treatment series of regressed language in autistic spectrum patients diagnosed with LKS variant showed improved language with pulse-dose steroids. 73,76,77 A single case report documenting language and social improvement after daily high-dose steroids in a regressive case of autism with normal EEG findings was based on abnormal cortical response to frequency-modulated, auditory-evoked potentials based on work from Stefanatos et al 78 This patient had a robust steroid response to prednisone. There has not been any follow-up or similar use of this technique to document steroid response, and although long-term outcome did show improved language and social function, the autism diagnosis remained.…”

Section: Corticosteroidsmentioning

confidence: 99%

Immune Therapy in Autism: Historical Experience and Future Directions with Immunomodulatory Therapy

Chez

Guido-Estrada

2010

Neurotherapeutics

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Summary: Autism affects 1 in 110 new births, and it has no single etiology with uniform agreement. This has a significant impact on the quality of life for individuals who have been diagnosed with autism. Although autism has a spectrum quality with a shared diagnosis, it presents a uniquely different clinical appearance in each individual. Recent research of suspected immunological factors have provided more support for a probable immunological process or for processes that may play a role in the acquisition of an autistic condition. These factors include prenatal, genetic, and postnatal findings, as well as the discovery of a dysfunctional chronic pro-inflammatory state in brain tissue and cerebrospinal fluid in subsets of autistic patients. These findings offer new theories that may lead to the development of disease modification or preventative therapeutic options in the near future. This article reviews prenatal, genetic, and observed immune aspects of the autism condition that may be risk factors in the presentation of the autistic clinical phenotype. Historical immune interventions in autism are reviewed and potential new therapies and interventions are discussed.

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Pulse Methylprednisolone Therapy in Landau-Kleffner Syndrome

Cited by 9 publications

References 7 publications

Intravenous methylprednisolone pulse therapy for children with epileptic encephalopathy

Intravenous methylprednisolone pulse therapy for children with epileptic encephalopathy

Management of Landau-Kleffner Syndrome

Immune Therapy in Autism: Historical Experience and Future Directions with Immunomodulatory Therapy

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