Immunoglobulin therapy in the West syndrome

Ariizumi, Motomizu; Baba, Kazuhiko; Hibio, Shuichi; Shiihara, Hiroaki; Michihiro, Narumi; Ogawa, Kimio; Okubo, O

doi:10.1016/s0387-7604(87)80117-1

Cited by 52 publications

(24 citation statements)

References 3 publications

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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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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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“…All 6 patients with cryptogenic West syndrome showed complete remission in accordance with normalized electroencephalogram. Of the 5 patients with symptomatic West syndrome, 1 showed cessation of clinical spasms in agreement with EEG improvement and 2 others revealed transient cessation of spasms with recurrence 99. Five of 23 patients with infantile spasms experienced cessation of infantile spasms with 1 g/kg of IVIg for 2 days, repeated every 3 weeks for 6 months 100…”

Section: Intravenous Immunoglobulin (Ivig)mentioning

confidence: 71%

Current trends in the treatment of infantile spasms

Tsao¹

2009

NDT

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Infantile spasms are an epilepsy syndrome with distinctive features, including age onset during infancy, characteristic epileptic spasms, and specific electroencephalographic patterns (interictal hypsarrhythmia and ictal voltage suppression). Adrenocorticotropic hormone (ACTH) was first employed to treat infantile spasms in 1958, and since then it has been tried in prospective and retrospective studies for infantile spasms. Oral corticosteroids were also used in a few studies for infantile spasms. Variable success in cessation of infantile spasms and normalization of electroencephalograms was demonstrated. However, frequent significant adverse effects are associated with ACTH and oral corticosteroids. Vigabatrin has been used since the 1990s, and shown to be successful in resolution of infantile spasms, especially for infantile spasms associated with tuberous sclerosis. It is associated with visual field constriction, which is often asymptomatic and requires perimetric visual field study to identify. When ACTH, oral corticosteroids, and vigabatrin fail to induce cessation of infantile spasms, other alternative treatments include valproic acid, nitrazepam, pyridoxine, topiramate, zonisamide, lamotrigine, levetiracetam, felbamate, ganaxolone, liposteroid, thyrotropin-releasing hormone, intravenous immunoglobulin and a ketogenic diet. Rarely, infantile spasms in association with biotinidase deficiency, phenylketonuria, and pyridoxine-dependent seizures are successfully treated with biotin, a low phenylalanine diet, and pyridoxine, respectively. For medically intractable infantile spasms, some properly selected patients may have complete cessation of infantile spasms with appropriate surgical treatments.

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“…In 2001, Hattori summarized the spontaneous remission of West syndrome in Japan and described most remission cases as being due to a viral infection from 1970 to 2000. 6 On the other hand, Riikonen reported that West syndrome continued for several weeks after infection in 10% of the viral encephalitis cases owing to cytomegalovirus, human herpesvirus, and rubella. However, the details are unclear, and most of them are considered to be a spontaneous remission.…”

Section: Discussionmentioning

confidence: 99%

Remission Associated With Human Herpesvirus Infection in West Syndrome

2006

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Generally, West syndrome is an intractable epileptic syndrome in infancy, although spontaneous remission has been reported in some cases. An immunologic response to infection might be one of the factors involved in the remission of West syndrome, but the mechanisms remain unknown. On the other hand, exanthema subitum is a common disease occurring in infancy with the characteristics of fever and rash. Two kinds of human herpesvirus, 6 and 7, have been isolated as causal agents of exanthema subitum. We experienced one symptomatic case and three cryptogenic cases of West syndrome that showed spontaneous remission. In the symptomatic case, the subject showed a temporary remission; however, in the other cases, the remissions were long term. In the present study, we report the patients' improvement and electroencephalographic (EEG) findings. In all of our cases, hypsarrythmia disappeared on the EEG findings, the human herpesvirus 6 IgG antibodies increased in all four cases, and the herpesvirus 7 IgG antibodies increased in two cases. We postulate that the remission of the four cases proceeded from infection by exanthema subitum. The changes in serum antibody values suggest that the spontaneous remission of West syndrome was related to human herpesvirus 6 and 7 infections.

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Immunoglobulin therapy in the West syndrome

Cited by 52 publications

References 3 publications

Inflammation in Epileptic Encephalopathies

Inflammation in Epileptic Encephalopathies

Current trends in the treatment of infantile spasms

Remission Associated With Human Herpesvirus Infection in West Syndrome

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