Therapeutic Effects of Adrenocorticotropic Hormone ACTH in Children with Severely Intractable Seizure

How to Cite This Article: Nasiri J, Sarajan A, Salari M, Sedghi M. Therapeutic Effects of Adrenocorticotropic Hormone ACTH in Children withSeverely Intractable Seizure. Iran J Child Neurol. Summer 2017; 11(3):19-26.AbstractObjectiveTreatment of intractable seizures other than spasms is difficult and controversial.There are few studies on efficacy of adrenocorticotropic hormone (ACTH) in treatment of patients with intractable seizure.Materials & MethodsTwenty-five patients with intractable seizure other than spasm including 14 boys and 11 girls with median age of 58 months referred to university clinics of Pediatric Neurology in Isfahan, Iran, during 2014-2015 were prospectively investigated. ACTH was administrated according to our protocol. All cases were followed regularly and assessed for response to treatment and probable side effects, 3 wk after beginning of ACTH therapy and three months after the ACTH therapy. EEG finding were recorded before and three months after the end of ACTH therapy. Statistical analysis using Freidman test and Wilcoxon signed – rank test were performed in order to compare seizure frequency and EEG changes, respectively.ResultsMean A significant reduction (>80%) in seizure frequency in 11 cases (44%) and moderate reduction (50%-80%) in 7 (28%) after 3 wk of ACTH therapy.Despite initial positive response, recurrence of seizure was observed in 7 out of 18 cases with favorable initial response within 3 months after ACTH therapy cessation. The comparison of EEG finding before and 3 months after ACTH therapy using Wilcoxon signed – rank test showed  significant differences.ConclusionACTH therapy may be useful in treatment of children with intractable seizures who are resistant to usual antiepileptic drugs. However further studies should be performed to determine the long-term efficacy of ACTH in treatment of intractable seizure.References1. Dunin-Wąsowicz D, Mazurkiewicz-Bełdzińska M, Steinborn B, Wheless J, Jóźwiak S. Treatment of pediatric epilepsy in Poland. Eur J Paediatr Neurol 2015;19(3):320-6.2. Oka E, Ohtsuka Y, Yoshinaga H, Murakami N, Kobayashi K, Ogino T. Prevalence of Childhood Epilepsy and Distribution of Epileptic Syndromes: A Population-based Survey in Okayama, Japan. Epilepsia 2006;47(3):626-30.3. Beleza P. Refractory epilepsy: a clinically oriented review. Eur Neurol 2009; 62(2):65-71.4. Pentella K, Bachman D, Sandman CA. Trial of an ACTH4-9 Analogue (ORG 2766) in children with intractable seizures. Neuropediatrics 1982;13(2):59-62.5. Snead OC, Benton JW, Myers GJ. ACTH and prednisone in childhood seizure disorders. Neurology 1983;33(8):966-70.6. Okumura A, Tsuji T, Kato T, Natsume J, Negoro T, Watanabe K. ACTH therapy for generalized seizures other than spasms. Seizure 2006;15(7):469-75.7. Verhelst H, Boon P, Buyse G, Ceulemans B, D’Hooghe M, De Meirleir L, et al. Steroids in intractable childhood epilepsy: clinical experience and review of the literature. Seizure 2005;14(6):412-21.8. Oguni H, Funatsuka M, Sasaki K, Nakajima T, Yoshii K, Nishimura T, et al. Effect of ACTH therapy for epileptic spasms without hypsarrhythmia. Epilepsia 2005;46(5):709-15.9. Haberlandt E, Weger C, Sigl SB, Rauchenzauner M, Scholl-Bürgi S, Rostásy K, et al. Adrenocorticotropic hormone versus pulsatile dexamethasone in the treatment of infantile epilepsy syndromes. Pediatr Neurol 2010;42(1):21-7.10. Inutsuka M, Kobayashi K, Oka M, Hattori J, Ohtsuka Y. Treatment of epilepsy with electrical status epilepticus during slow sleep and its related disorders. Brain Dev 2006;28(5):281-6.11. Fujii A, Oguni H, Hirano Y, Osawa M. Atypical benign partial epilepsy: recognition can prevent pseudocatastrophe. Pediatr Neurol 2010;43(6):411-9.12. Inui T, Kobayashi T, Kobayashi S, Sato R, Endo W, Kikuchi A, et al. Efficacy of long term weekly ACTH therapy for intractable epilepsy. Brain Dev 2015;37(4):449-54.13. Kalra V, Sharma S, Arya R. ACTH therapy in refractory generalized epilepsy. Indian J Pediatr 2009;76(1):91-3.14. Kurian M, Korff CM. Steroids in pediatric epilepsy: infantile spasms and beyond. Epileptologie 2011; 28(1):15-20.15. Rogawski MA, DS R. Neurosteroids and infantile spasms: The deoxycorticosterone hypothesis. In: JMR PAS, editor. International Review of Neurobiology Volume 49: Academic Press; 2002. p. 199-219.16. Snead OC. How does ACTH work against infantile spasms? Bedside to bench. Ann Neurol 2001;49(3):288-9.17. Jacobson L, Sapolsky R. The Role of the Hippocampus in Feedback Regulation of the Hypothalamic-Pituitary- Adrenocortical Axis. Endocr Rev 1991;12(2):118-34.18. Sinclair DB. Prednisone therapy in pediatric epilepsy. Pediatr Neurol 2003;28(3):194-8

Thalamic Ventral Intermediate Nucleus Deep Brain Stimulation for Orthostatic Tremor

Background: Orthostatic tremor (OT) was first described in 1977. It is characterized by rapid tremor of 13–18 Hz and can be recorded in the lower limbs and trunk muscles. OT remains difficult to treat, although some success has been reported with deep brain stimulation (DBS). Case Report: We report a 68-year-old male with OT who did not improve significantly after bilateral thalamic stimulation. Discussion: Although some patients were described who improved after DBS surgery, more information is needed about the effect of these treatment modalities on OT, ideally in the form of randomized trial data

Evaluation of Effective Factors on Home Delivery from Women Perspective under Health Comprehensive Services Centers of Kerman Medical Sciences University

Background & Objectives: Maternal mortality rate due to complications of pregnancy and delivery is one of the most important indicators of the development status of countries which has a direct relationship with delivery conditions. Preposition home delivery increase, this study has been done for the purpose of study of mothers views who are under Kerman Medical Sciences University for causes detecting of delivery at home. Methods: This study was a descriptive-analytical one that was performed cross-sectionally on 503 mothers who are under comprehensive health service centers of Kerman University of Medical Sciences who were included in the study using convenience sampling method. The data were collected using a researcher-made questionnaire and were analyzed by descriptive statistics as well as logistic regression in SPSS software Results: The most causes of home delivery were the high cost of delivery at hospitals (71.4%) and family’s decision (33.4%). Unfavorable weather conditions (0.6%) had the least important role in home birth. The highest number of home births was related to non-Iranian population (98.4%). Based on the goodness of fit of logistic regression model, only the number of pregnancies and number of stillbirths had a significant effect on home birth. Conclusion: Since the high cost of delivery at hospital is the major reason preventing non-Iranian women from referring to the hospital, it is necessary to adopt new policies for admission of non-Iranian women in public hospitals and provide insurance to them so that they would be encouraged to have delivery at hospital. Key¬words: Home Birth, Effective factors, Women, Comprehensive Health Service Centers, Health Centers Citation: Tabasinezhad N, Safizadeh M, Hassanzadeh M, Salari S, Saber Mahani M, Damadi B. Evaluation of Effective Factors on Home Delivery from Women Perspective under Health Comprehensive Services Centers of Kerman Medical Sciences University. Journal of Health Based Research 2020; 6(1): 15-24. [In Persian

Genetic Testing in Parkinson's Disease

Genetic testing for persons with Parkinson's disease is becoming increasingly common. Significant gains have been made regarding genetic testing methods, and testing is becoming more readily available in clinical, research, and direct-to-consumer settings. Although the potential utility of clinical testing is expanding, there are currently no proven gene-targeted therapies, but clinical trials are underway. Furthermore, genetic testing practices vary widely, as do knowledge and attitudes of relevant stakeholders. The specter of testing mandates financial, ethical, and physician engagement, and there is a need for guidelines to help navigate the myriad of challenges. However, to develop guidelines, gaps and controversies need to be clearly identified and analyzed. To this end, we first reviewed recent literature and subsequently identified gaps and controversies, some of which were partially addressed in the literature, but many of which are not well delineated or researched. Key gaps and controversies include: (1) Is genetic testing appropriate in symptomatic and asymptomatic individuals without medical actionability? (2) How, if at all, should testing vary based on ethnicity? (3) What are the long-term outcomes of consumer- and research-based genetic testing in presymptomatic PD? (4) What resources are needed for clinical genetic testing, and how is this impacted by models of care and cost-benefit considerations? Addressing these issues will help facilitate the development of consensus and guidelines regarding the approach and access to genetic testing and counseling. This is also needed to guide a multidisciplinary approach that accounts for cultural, geographic, and socioeconomic factors in developing testing guidelines.</p

Defining the causes of sporadic Parkinson's disease in the global Parkinson's genetics program (GP2)

The Global Parkinson’s Genetics Program (GP2) will genotype over 150,000 participants from around the world, and integrate genetic and clinical data for use in large-scale analyses to dramatically expand our understanding of the genetic architecture of PD. This report details the workflow for cohort integration into the complex arm of GP2, and together with our outline of the monogenic hub in a companion paper, provides a generalizable blueprint for establishing large scale collaborative research consortia

Multi-ancestry genome-wide association meta-analysis of Parkinson?s disease

Although over 90 independent risk variants have been identified for Parkinson’s disease using genome-wide association studies, most studies have been performed in just one population at a time. Here we performed a large-scale multi-ancestry meta-analysis of Parkinson’s disease with 49,049 cases, 18,785 proxy cases and 2,458,063 controls including individuals of European, East Asian, Latin American and African ancestry. In a meta-analysis, we identified 78 independent genome-wide significant loci, including 12 potentially novel loci (MTF2, PIK3CA, ADD1, SYBU, IRS2, USP8, PIGL, FASN, MYLK2, USP25, EP300 and PPP6R2) and fine-mapped 6 putative causal variants at 6 known PD loci. By combining our results with publicly available eQTL data, we identified 25 putative risk genes in these novel loci whose expression is associated with PD risk. This work lays the groundwork for future efforts aimed at identifying PD loci in non-European populations

Can COVID‐19 accelerate neurodegeneration?

Abstract COVID‐19 may accelerate neurodegeneration in patients with neurodegenerative disease

Atypical parkinsonism and self‐mutilation: A new lens on the old concept

Abstract We report a case of atypical parkinsonism and self‐mutilation

Magnetic resonance imaging findings in diagnosis and prognosis of Wilson disease

Wilson disease (WD) is a rare autosomal recessive disorder characterized by excessive copper deposition in the body, principally in the liver and the brain. There is a wide spectrum of clinical presentations, but the most significant and basic symptoms of the disease can be divided into hepatic, neurologic, and psychiatric manifestations. Magnetic resonance imaging (MRI) provides more detailed anatomical information than computed tomography of the brain, especially of the structure of the basal ganglia and brain stem. In this review, we want to evaluate the correlation between MRI findings and clinical features of WD

Botulinum Toxin Induced Atrophy: An Uncharted Territory

Botulinum neurotoxins (BoNTs) produce local chemo-denervation by cleaving soluble N-ethylmaleimide-sensitive factor activating protein receptor (SNARE) proteins. Botulinum neurotoxins are therapeutically indicated in several neurological disorders and have been in use for three decades. The long-term efficacy, safety, and side effects of BoNTs have been well documented in the literature. However, the development of muscle atrophy following chronic exposure to BoNTs has not received sufficient attention. Muscle atrophy is not only cosmetically distressing, but also has an impact on future injections. An extensive literature search was conducted on atrophy and mechanisms of atrophy. Five hundred and four relevant articles in the English language were reviewed. This review revealed the surprising lack of documentation of atrophy within the literature. In addition, as demonstrated in this review, the mechanisms and the clinical factors that may lead to atrophy have also been poorly studied. However, even with this limited information it is possible to indicate factors that could modify the clinical approach to botulinum toxin injections. This review highlights the need for further study of atrophy following BoNT injections