Effects of initial polytherapy in newly diagnosed West syndrome

Jia, Fei‐Yong; Jiang, Hao-Bo; Du, Lin; Li, Honghua; Niu, Chen

doi:10.1109/hhbe.2011.6027919

Cited by 3 publications

(3 citation statements)

References 11 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In our experience (unpublished data), initial polytherapy with low dose of ACTH and TPM was effective to suppress ISS in most patients, allowing to leave TPM-after removal of ACTH-as AEDs of choice to prevent recurrence of the ISS. This combined treatment has been endorsed by other authors [206,207].…”

Section: Calcium Current Inhibitorsmentioning

confidence: 63%

GABAergic Interneurons in Severe Early Epileptic Encephalopathy with a Suppression-Burst Pattern: A Continuum of Pathology

Carrascosa-Romero¹,

Cabo²

2016

Epileptology - The Modern State of Science

View full text Add to dashboard Cite

Early infantile epileptic encephalopathy (EIEE) and early myoclonic encephalopathy (EME) are catastrophic epilepsies starting in the neonatal period. The International League Against Epilepsy classifies both of them as generalized symptomatic epilepsies of nonspecific etiology, characterized by early onset, presence of burst-suppression EEG pattern and serious prognosis. The critical difference lies in their presumed etiologies and the prevailing clinical seizure type at onset: EIEE (known as Ohtahara syndrome) usually manifests with tonic seizures, while EME is mainly associated with myoclonic seizures. However, the distinction between those two pathologies is not always easy due to clinical and etiological overlap. Both mutations in the ARX gene for EIEE (OMIM 308350) and disruption in the neuregulin receptor ErbB4 for EME (OMIM 609304) impair interneuron migration and alter the number of GABAergic interneurons in the postnatal cortex. These findings could explain the occurrence of severe epileptic encephalopathy with a burst-suppression pattern and represent a continuum of progressive pathology. In the present chapter we review the genes involved in EIEE and EME including their possible mechanisms of action, particularly via GABAegic interneurons. Their clinical manifestations are myoclonic or tonic seizures, which represent the expression of the underlying pathology and correlate with degree of brain damage.

show abstract

Section: Calcium Current Inhibitorsmentioning

confidence: 63%

GABAergic Interneurons in Severe Early Epileptic Encephalopathy with a Suppression-Burst Pattern: A Continuum of Pathology

Carrascosa-Romero¹,

Cabo²

2016

Epileptology - The Modern State of Science

View full text Add to dashboard Cite

show abstract

“…Prospective study at Severance Children’s Hospital also demonstrated the potential of a treatment protocol using polytherapy(vigabatrin and prednisolone) for West syndrome [ 20 ]. Other studies also showed that initial polytherapy with ACTH and other AED or topiramate followed by low-dose ACTH are effective and well-tolerated options in IS [ 21 , 22 , 23 ]; however, it is worth noting that the randomized, open-label, controlled trial denied the effectiveness of combination of hormonal therapy(prednisone) and moderate dose of to piramate [ 24 ].…”

Section: Discussionmentioning

confidence: 99%

Effectiveness of ACTH in Patients with Infantile Spasms

et al. 2022

View full text Add to dashboard Cite

(1) Background: West syndrome is a severe, refractory, epileptic syndrome that usually appears in infancy or early childhood. ACTH is one of the more effective drugs for treating this condition. (2)Aim of the study and methods: The objective of our study was to examine short-term efficacy (during treatment schedule) and long-term outcome of intramuscular 0.02 mg/kg/day ACTH (tetracosactide) depot, used concomitantly with other antiepileptic drugs (AEDs) in patients with infantile spasms who did not achieve seizure cessation or relapse when taking only the AEDs. The drug efficacy was evaluated in retrospective and prospective analyses of 50 patients diagnosed with infantile spasms. (3) Results: Complete cessation of spasms was achieved in 42 cases (84%). EEG improvement was seen in 41 (82%) patients who responded to ACTH therapy. Information on the clinical course of 28 patients was obtained duringlong-term follow-up. In 17 (60.7%) cases, seizures were still present. Normal or near-normal development was observed in 11 out of 28 children (39%). ACTH used concomitantly with other AEDis a highly effective treatment with acceptable side effects. (4) Conclusion: Randomized controlled clinical trialswith long-term follow-up are needed to compare the effectiveness of ACTH in polytherapy and monotherapy.Dyskinesias as a potential side effect observed in our study group should be investigated in the following studies.

show abstract

“…corticotropin is not available in the region where the patient was treated. Rather, an approved shortacting formulation of animal-derived natural corticotropin (Shanghai The First Biochemical & Pharmaceutical Co Ltd, Shanghai, China) was available 14 and was used after the Institutional Review Board approved the proposal and the patient provided written informed consent. The initial regimen consisted of subcutaneous injections of 25 IU of short-acting natural corticotropin given daily at 9 am with reference to the Columbia corticotropin gel therapy (80 IU twice a week) regimen for nephrotic glomerulopathies.…”

Section: Figurementioning

confidence: 99%

Acquired Resistance to Corticotropin Therapy in Nephrotic Syndrome: Role of De Novo Neutralizing Antibody

Wang

Zhang

Wang³

et al. 2017

Pediatrics

View full text Add to dashboard Cite

There is increasing evidence supporting the use of corticotropin as an alternative treatment of refractory proteinuric glomerulopathies. The efficacy of short-acting corticotropin, however, remains unknown and was tested here in an adolescent with steroid-dependent nephrotic syndrome caused by minimal change disease. After developing Cushing syndrome and recently being afflicted with severe cellulitis, the patient was weaned off all immunosuppressants, including corticosteroids. This resulted in a relapse of generalized anasarca, associated with massive proteinuria and hypoalbuminemia. Subsequently, mono-therapy with short-acting animal-derived natural corticotropin was initiated and resulted in a rapid response, marked by substantial diuresis, reduction in body weight, and partial remission of proteinuria. Ten days later, the patient developed mild skin rash and subcutaneous nodules at injection sites. A relapse followed despite doubling the dose of corticotropin, consistent with delayed-onset resistance to treatment. Immunoblot-based antibody assay revealed de novo formation of antibodies in the patient's serum that were reactive to the natural corticotropin. In cultured melanoma cells known to express abundant melanocortin receptors, addition of the patient's serum strikingly mitigated dendritogenesis and cell signaling triggered by natural corticotropin, denoting neutralizing properties of the newly formed antibodies. Collectively, short-acting natural corticotropin seems effective in steroid-dependent nephrotic syndrome. De novo formation of neutralizing antibodies is likely responsible for acquired resistance to corticotropin therapy. The proof of concept protocols established in this study to examine the anticorticotropin neutralizing antibodies may aid in determining the cause of resistance to corticotropin therapy in future studies.

show abstract

Effects of initial polytherapy in newly diagnosed West syndrome

Cited by 3 publications

References 11 publications

GABAergic Interneurons in Severe Early Epileptic Encephalopathy with a Suppression-Burst Pattern: A Continuum of Pathology

GABAergic Interneurons in Severe Early Epileptic Encephalopathy with a Suppression-Burst Pattern: A Continuum of Pathology

Effectiveness of ACTH in Patients with Infantile Spasms

Acquired Resistance to Corticotropin Therapy in Nephrotic Syndrome: Role of De Novo Neutralizing Antibody

Contact Info

Product

Resources

About