Corticosteroids for the Treatment of Landau-Kleffner Syndrome and Continuous Spike-Wave Discharge During Sleep

Sinclair, D. Barry; Snyder, Thomas

doi:10.1016/j.pediatrneurol.2004.12.006

Cited by 106 publications

(61 citation statements)

References 21 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…25,26 Conversely, patients with neuronal antibodies who fail to respond to immunotherapy may still have an autoimmune basis for their seizures, as is likely the case in patients with onconeural antibodies targeting intracellular proteins. Our study confirms prior published data demonstrating that PMA Abs predict a positive response to immunotherapy 15,21 but beneficial outcomes were observed in both antibody-positive and antibody-negative patients.…”

mentioning

confidence: 99%

Utility of an immunotherapy trial in evaluating patients with presumed autoimmune epilepsy

et al. 2014

View full text Add to dashboard Cite

Objective: To evaluate a trial of immunotherapy as an aid to diagnosis in suspected autoimmune epilepsy. Method:We reviewed the charts of 110 patients seen at our autoimmune neurology clinic with seizures as a chief complaint. Twenty-nine patients met the following inclusion criteria: (1) autoimmune epilepsy suspected based on the presence of $1 neural autoantibody (n 5 23), personal or family history or physical stigmata of autoimmunity, and frequent or medically intractable seizures; and (2) initiated a 6-to 12-week trial of IV methylprednisolone (IVMP), IV immune globulin (IVIg), or both. Patients were defined as responders if there was a 50% or greater reduction in seizure frequency.Results: Eighteen patients (62%) responded, of whom 10 (34%) became seizure-free; 52% improved with the first agent. Of those receiving a second agent after not responding to the first, 43% improved. A favorable response correlated with shorter interval between symptom onset and treatment initiation (median 9.5 vs 22 months; p 5 0.048). Responders included 14/16 (87.5%) patients with antibodies to plasma membrane antigens, 2/6 (33%) patients seropositive for glutamic acid decarboxylase 65 antibodies, and 2/6 (33%) patients without detectable antibodies. Of 13 responders followed for more than 6 months after initiating long-term oral immunosuppression, response was sustained in 11 (85%).Conclusions: These retrospective findings justify consideration of a trial of immunotherapy in patients with suspected autoimmune epilepsy. Classification of evidence:This study provides Class IV evidence that in patients with suspected autoimmune epilepsy, IVMP, IVIg, or both improve seizure control. Neurology ® 2014;82:1578-1586 GLOSSARY AED 5 antiepileptic drug; CASPR2 5 contactin-associated protein-like 2; CC 5 calcium channel; gAChR 5 neuronal acetylcholine receptor, ganglionic-type; GAD65 5 glutamic acid decarboxylase 65; IgG 5 immunoglobulin G; IVIg 5 IV immune globulin; IVMP 5 IV methylprednisolone; LGI1 5 leucine-rich, glioma-inactivated 1; PMA Abs 5 antibodies to neural plasma membrane antigen; VGKC 5 voltage-gated potassium channel.Approximately one-third of epilepsy cases are intractable to antiepileptic drug (AED) therapy.

show abstract

mentioning

confidence: 99%

Utility of an immunotherapy trial in evaluating patients with presumed autoimmune epilepsy

et al. 2014

View full text Add to dashboard Cite

show abstract

“…Anti-epileptic drugs (AEDs) are the first line pharmacologic treatment for LKS and have been found in many cases to reduce epileptiform discharges with inconsistent effects on language [15,31]. Some studies indicate that high dose corticosteroids for a short period (3-6 months) result in improvement in language and EEG disturbances [32][33][34]; others have shown mixed results for language [35]. Sideeffects of corticosteroids can be problematic [35].…”

Section: Rehabilitation Issues In Landau-kleffner Syndromementioning

confidence: 99%

“…Some studies indicate that high dose corticosteroids for a short period (3-6 months) result in improvement in language and EEG disturbances [32][33][34]; others have shown mixed results for language [35]. Sideeffects of corticosteroids can be problematic [35]. Intravenous immunoglobulin has been used both with good effect [36] and no effect [37].…”

Section: Rehabilitation Issues In Landau-kleffner Syndromementioning

confidence: 99%

Rehabilitation issues in Landau–Kleffner Syndrome

Kuriakose

Lang

Boyer

et al. 2012

Developmental Neurorehabilitation

View full text Add to dashboard Cite

“…Steroids also are effective in some paediatric epilepsy syndromes, like infantile spasms, LandauKleffner syndrome and Rasmussen encephalitis [340][341][342]. The reported clinical experience in adult patients is small, but some cases with RSE responded to treatment with steroids [343,344].…”

Section: S C H W E I Z E R a R C H I V F ü R N E U R O L O G I E U N mentioning

confidence: 99%

Non-convulsive status epilepticus in adults - an overview

Rüegg¹

2008

Swiss Arch Neurol Psychiatr

View full text Add to dashboard Cite

show abstract

Corticosteroids for the Treatment of Landau-Kleffner Syndrome and Continuous Spike-Wave Discharge During Sleep

Cited by 106 publications

References 21 publications

Utility of an immunotherapy trial in evaluating patients with presumed autoimmune epilepsy

Utility of an immunotherapy trial in evaluating patients with presumed autoimmune epilepsy

Rehabilitation issues in Landau–Kleffner Syndrome

Non-convulsive status epilepticus in adults - an overview

Contact Info

Product

Resources

About