High Dose Gammaglobulin for Intractable Childhood Epilepsy

Ariizumi, Motomizu; Shiihara, Hiroaki; Hibio, Shuiichi; Ryo, Shigeo; Baba, Kazuhiko; Ogawa, Kimio; Suzuki, Yutaka; Momoki, Toshiro

doi:10.1016/s0140-6736(83)90144-7

Cited by 80 publications

(18 citation statements)

References 4 publications

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“…We could not confirm previous reports concerning markers predicting a favourable IVIg response, like IgG2 deficiency [17,39,46], duration of epilepsy [6], low serum IgA values [6,41] or low CD4 values [41]. However, a trend for a negative correlation existed between serum IgM concentration, and both clinical seizure reduction and behavioural improvement.…”

Section: Discussioncontrasting

confidence: 89%

“…Since then, the hypothesis has emerged that immune mechanisms may play a role in triggering, maintaining or even improving (intractable) epilepsies [1,32,35,47]. The favourable results of immunosuppressive drugs like ACTH and corticosteroids, in epilepsies usually of the WS type but also LGS, have favoured an immunological approach, and many studies have reported generally successful intravenous immunoglobulin (IVIg) treatment in epilepsies [6,8,10,12,17,19,22,29,39,41,42,45,46]. Nevertheless, heterogeneity concerning patient inclusion criteria, types of epilepsy, route and schedule of IVIg administration, followup period and criteria for the evaluation of the response, make the various results difficult to interpret.…”

Section: Introductionmentioning

confidence: 99%

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High-dose intravenous immunoglobulin treatment in cryptogenic West and Lennox-Gastaut syndrome; an add-on study

et al. 1994

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In an add-on pilot study, a group of 15 children with cryptogenic and intractable West syndrome (3) and Lennox-Gastaut syndrome (12) received intravenous immunoglobulin (IVIg, 0.4 g/kg body weight per day for 5 consecutive days, followed by the same dose once every 2 weeks for 3 months). Five patients had been treated previously with ACTH without success. The reduction in clinical seizures averaged 70%. Electroencephalographic (EEG) recordings revealed a mean reduction in epileptic discharges of 40%. In all 15 patients, acceleration of EEG background activity occurred, and psychomotor development improved. Prior to IVIg administration, CSF examinations were normal. After IVIg administration, the serum total IgG concentration increased by an average of 76%, and the CSF IgG concentration by 44%. According to our data, IVIg crosses the blood-CSF barrier, and might be effective in the treatment of West syndrome and Lennox-Gastaut syndrome. We suggest it should be considered when other treatments, such as ACTH, have failed.

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Section: Discussioncontrasting

confidence: 89%

Section: Introductionmentioning

confidence: 99%

High-dose intravenous immunoglobulin treatment in cryptogenic West and Lennox-Gastaut syndrome; an add-on study

et al. 1994

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“…On the other hand, alternative treatments (e.g. gamma globulin, lorazepam) for ehildhood status epileptieus, serial seizures and severe epilepsy (2,3,6) have not proven to be more or, in many eases, even equally effeetive as the introduetion of long barbiturate anaesthesia. Barbiturate anaesthesia often remains the last choice of treatment for many ehildren with otherwise intractable seizures.…”

Section: Discussionmentioning

confidence: 99%

Effects of Long Barbiturate Anaesthesia on Eight Children with Severe Epilepsy

Eriksson¹,

Baer²,

Kilpinen³

et al. 1993

Neuropediatrics

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Frequent epileptic seizures in children are often related to delayed psychomotor development, and status epilepticus is always a neurological emergency. In both situations barbiturate anaesthesia has been used for status epilepticus since the 1960s, and for intractable seizures in children since the 1980s. However, the clinical results on the effectiveness of barbiturate anaesthesia in children with chronic epileptic disorders remain contradictory. Between 1986 and 1991 in Tampere University Hospital in Finland long barbiturate anaesthesia was introduced--using thiopentone sodium--to eight children with very severe epilepsy. Children were 10 months to 7 years 11 months of age and the mean time from the onset of seizures to the introduction of BA was 2 years 8 months. Effects upon seizure frequency, antiepileptic medication and/or psychomotor development were clearly positive in three patients, slightly positive in one patient and in four patients there was no effect. Good effect seemed to be associated with an anaesthesia which is deep and long enough to produce loss of consciousness and spontaneous reactions, and an electroencephalographic pattern of burst-suppression. Positive results were also more often achieved when the treatment lag was less than 12 months. Physical and neurophysiological properties of barbiturates make their effectiveness as anticonvulsants understandable, but there is only little evidence to explain the mechanism of this action.

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“…Previous studies have suggested immunosuppressive effect of ACTH and corticosteroids, together with anti-inflammatory properties, as well as direct inhibitory impact on CNS excitability and possible inhibition of endogenous proconvulsant factor synthesis (Baram & Hatalski 1998, Riikonen 2000, Brunson, Khan, Eghbal-Ahmadi & Baram 2001, Joels & Baram 2009, Vezzani, French, Bartfai & Baram 2011), although ACTH has been reported to be more efficient (Snead 2001). In addition, therapy with immune-modulatory agents such as ACTH and corticosteroids or high-dose immunoglobulin has shown efficacy on spasms cessation in some patients with IS of infectious etiology, however it was suggested that steroid therapy should be avoided in patients with a history of CMV and herpes simplex in the past (Ariizumi, Shiihara, Hibio, Ryo, Baba, Ogawa et al 1983, Ariizumi, Baba, Hibio, Shiihara, Michihiro, Ogawa et al 1987, Riikonen 1993, Wise, Rutledge & Kuzniecky 1996, Hattori 2001). …”

Section: Are There Any Lead Candidates or Unexplored Targets For Fmentioning

confidence: 99%

Inflammation in Epileptic Encephalopathies

Shandra

Moshé

Galanopoulou

2017

Stress and Inflammation in Disorders

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West syndrome (WS) is an infantile epileptic encephalopathy (EE) that manifests with infantile spasms, hypsarrhythmia (in ~60% of infants) and poor neurodevelopmental outcomes. The etiologies of WS can be structural-metabolic pathologies (~60%), genetic (12–15%) or of unknown origin. The current treatment options include hormonal treatment [adrenocorticotropic hormone (ACTH) and high dose steroids], the GABA aminotransferase inhibitor vigabatrin, while ketogenic diet can be given as add-on treatment in refractory IS. There is a need to identify new therapeutic targets and more effective treatments for WS. Theories about the role of inflammatory pathways in the pathogenesis and treatment of WS have emerged, being supported by both clinical and preclinical data from animal models of WS. Ongoing advances in genetics have revealed numerous genes involved in the pathogenesis of WS, including genes directly or indirectly involved in inflammation. Inflammatory pathways also interact with other signaling pathways implicated in WS, such as the neuroendocrine pathway. Furthermore, seizures may also activate pro-inflammatory pathways raising the possibility that inflammation can be a consequence of seizures and epileptogenic processes. With this targeted review we plan to discuss the evidence pro and against the following key questions. Does activation of inflammatory pathways in the brain cause epilepsy in WS and does it contribute to the associated comorbidities and progression? Can activation of certain inflammatory pathways be a compensatory or protective event? Are there interactions between inflammation and the neuroendocrine system that contribute to the pathogenesis of West syndrome? Does activation of brain inflammatory signaling pathways contribute to the transition of WS to Lennox-Gastaut syndrome? Are there any lead candidates or unexplored targets for future therapy development for WS targeting inflammation?

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High Dose Gammaglobulin for Intractable Childhood Epilepsy

Cited by 80 publications

References 4 publications

High-dose intravenous immunoglobulin treatment in cryptogenic West and Lennox-Gastaut syndrome; an add-on study

High-dose intravenous immunoglobulin treatment in cryptogenic West and Lennox-Gastaut syndrome; an add-on study

Effects of Long Barbiturate Anaesthesia on Eight Children with Severe Epilepsy

Inflammation in Epileptic Encephalopathies

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