Serologie HLA typing in cryptogenic Lennox-Gastaut syndrome

Engelen, B.G.M. van; Waal, L. P. de; Weemaes, C.M.R.; Renier, W.O.

doi:10.1016/0920-1211(94)90078-7

Cited by 16 publications

(6 citation statements)

References 23 publications

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“…We do not believe that LTG contributed to the infections our patients reported. Instead, the high rate of infections is consistent with both our routine clinical experience involving LGS patients and the hypothesis that immunogenic mechanisms play a role in triggering or maintaining some cases of LGS (6, 26,27).…”

Section: Discussionsupporting

confidence: 81%

Lamotrigine Adjunctive Therapy in Childhood Epileptic Encephalopathy (the Lennox Gastaut Syndrome)

1997

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Summary: Purpose:We assessed efficacy and safety of adjunctive lamotrigine (LTG) therapy in patients with the Lennox-Gastaut syndrome (LGS).Methods: The study was a single-center, retrospective chart review of open-label adjunctive LTG therapy in patients with LGS. Initial LTG dose and titration was dependent on concomitant antiepileptic drugs (AEDs). Efficacy was based on the change in seizure frequency between the initiation of LTG therapy and December 1, 1995 (or LTG discontinuation). Seizure diaries were used to count patient seizures. A secondary evaluation of efficacy was a parental or guardian assessment of the patient's global status. The evaluation of safety involved chart review for treatment-emergent adverse events (AE).Results: Data from 16 LGS patients were analyzed. Fiftythree percent (8 of 15) had a >50% reduction in seizure frequency with LTG adjunctive therapy. Tonic, atonic, generalized tonic-clonic (GTCS), and atypical absence seizure frequency but not myoclonic seizure frequency decreased significantly during LTG therapy. Fifty-three percent of the patient's parents (8 of 15) reported that their child's quality of life (QOL) was much or very much improved during the study. The major treatment-emergent AE were infection (50%, 8 of 16) and sleep disturbance (19%, 3 of 16). A rash was noted in 13% (2 of 16) of the patients and resulted in LTG discontinuation in 1. No clinically significant changes were noted in neurologic examination or laboratory tests during the study.Conclusions: Our results indicate that LTG adjunctive therapy is effective and well tolerated in patients with LGS.

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Section: Discussionsupporting

confidence: 81%

Lamotrigine Adjunctive Therapy in Childhood Epileptic Encephalopathy (the Lennox Gastaut Syndrome)

1997

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“…The LGS group did not differ from any of the control groups with regard to percentage of B cells (mean 11,3%, standard deviation (SD) 5.5; individual values: 15,14,10,17,5,21,8,14,7,6,3, and 15%), CD3 positive cells (mean 64,5%, SD 6,4; individual values: 66, 73,65,72, 70,60,60,60, 70, 65, 61, and 52%) or CD4/CD8 ratio (mean 2,8, SD 0.9; individual values: 1.8, 5.2, 2.9, 2.8, 2.4, 2,1, 2,3, 2,1, 2,3, 3.1, 3,1, and 3.1), Proliferation of peripheral blood lymphocytes to phytohaemagglutinin and pokeweed mitogen was normal in both the LGS and the epilepsy control groups.…”

Section: Resultsmentioning

confidence: 85%

“…Various pathophysiologic mechanisms have been suggested to explain this condition including the hypothesis that it results from an aberrant immune function. Indications supportive for this hypothesis include the reports on anti bodies to brain tissue [2,3], the suggested effect of intravenous immunoglobulin [4], and the reported associations with certain HLA class II alleles in patients with LGS [5]. The publications on humoral and cellular immunity are conflict ing, impaired immunity (predominantly IgG2 and IgA defi ciencies) [6][7][8], normal immunity [9], and increased humoral or cellular immunity [7,10] have been described.…”

Section: Introductionmentioning

confidence: 99%

A Dysbalanced Immune System in Cryptogenic Lennox‐Gastaut Syndrome

Engelen

Weemaes²,

Renier

et al. 1995

Scand J Immunol

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In children with cryptogenic Lennox‐Gastaut syndrome we found a functionally impaired humoral immune response to a primary antigen (haemocyanin), despite signs of a triggered immune system consisting of elevated IgG concentrations. This combination of immunological findings, considered to be the expression of a dysbalanced‐triggered as well as functionally impaired‐immune system, has also been described in an auto‐immune disease like systemic lupus erythaematodes in humans, and in genetically epilepsy‐prone rats. The interactions between the immune system and the nervous system in Lennox‐Gastaut syndrome will be discussed.

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“…Reports of an activated but functionally impaired immune system,15 increased serum immunoglobulins16and a HLA DR5 association in Lennox-Gastaut syndrome further supports immunological involvement 17…”

Section: Childhood Epilepsy Syndromesmentioning

confidence: 82%

Epilepsy: an autoimmune disease?

Palace

2000

Journal of Neurology, Neurosurgery &Amp; Psychiatry

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Serologie HLA typing in cryptogenic Lennox-Gastaut syndrome

Cited by 16 publications

References 23 publications

Lamotrigine Adjunctive Therapy in Childhood Epileptic Encephalopathy (the Lennox Gastaut Syndrome)

Lamotrigine Adjunctive Therapy in Childhood Epileptic Encephalopathy (the Lennox Gastaut Syndrome)

A Dysbalanced Immune System in Cryptogenic Lennox‐Gastaut Syndrome

Epilepsy: an autoimmune disease?

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