Iranian Journal of Child Neurology

How to Cite This Article: Babaknejad N, Sayehmiri F, Sayehmiri K, Mohamadkhani A, Bahrami S. The Relationship between Zinc Levels
and Autism: Systematic Review and Meta-analysis. Iran J Child Neurol. Autumn 2016; 10(4):1-9.

Abstract

Objective

Autism is a complex behaviorally defined disorder. There is a relationship between zinc (Zn) levels in autistic patients and development of pathogenesis, but the conclusion is not permanent.

Materials & Methods

The present study conducted to estimate this probability using meta-analysis method. In this study, Fixed Effect Model, twelve articles published from 1978 to 2012 were selected by searching Google scholar, PubMed, ISI Web of Science, and Scopus and information were analyzed. I² statistics were calculated to examine heterogeneity. The information was analyzed using R and STATA Ver. 12.2.

Results

There was no significant statistical difference between hair, nail, and teeth Zn levels between controls and autistic patients: -0.471 [95% confidence interval (95% CI): -1.172 to 0.231]. There was significant statistical difference between plasma Zn concentration and autistic patients besides healthy controls: -0.253 (95% CI: 0.498 to -0.007). Using a Random Effect Model, the overall Integration of data from the two groups was -0.414 (95% CI: -0.878 to -0.051).

Conclusion

Based on sensitivity analysis, zinc supplements can be used for the nutritional therapy for autistic patients.

References

1. Arnold LE, Farmer C, Kraemeret HC, et al. Moderators, mediators, and other predictors of Risperidoneresponse in children with Autistic Disorder and Irritability. J Child Adolesc Psychopharmacol 2012; 20(2): 83-92.

2. Karimzadeh P. Recent finding about etiology of autism. Rehabilitation 2000; 1(2):58-63.

3. Dufault R, Schnoll R, Lukiw WJ, et al. Mercury exposure, nutritional deficiencies and metabolic disruptions may affect learning in children. Behav Brain Funct 2009; 5(44): 1-15.

4. Morris CR, Agin CM. Syndrome of allergy, apraxia, and malabsorption: characterization of a neurodevelopmental phenotype that responds to omega 3 and vitamin E supplementation. Altern Ther Health Med 2009; 15(4): 34-43.

5. An centers for Disease Control and Prevention (2012). Prevalence of autism spectrum disorders-Autism and Developmental Disabilities Monitoring Network, Prevalence of Autism Spectrum Disorders (ASDs) Among Multiple Areas of the United States in 2008, United States, Morbidity and Mortal Weekly Report (MMWR); Vol. 61(3).

6. Dufault R, Lukiw WJ, Crider R, et al. A macro epigenetic approach to identify factors responsible for the autism epidemic in the United States. Clin Epigenetics 2012; 4(6): 2-12.

7. Faber S, Zinn GM, Kern GC, et al. The plasma zinc/ serum copper ratio as a biomarker in children with autism spectrum disorders. Biomarkers 2009; 14(3): 171–180.

8. Cornish E. Gluten and casein free diets in autism: a study of the effects on food choice and nutrition. J Hum Nutr Dietet 2012; 15: 261-268.

9. De Palma G, Catalani S, Franco A, et al. Lack of correlation between metallic elements analyzed in hair by ICP-MS and Autism. J Autism Dev Disord 2012; 42(3):342–353.

10. Adams JB, Romdalvik J, Ramanujam VM, Legator MS, et al. Mercury, Lead, and Zinc in Baby teeth of children with Autism versus controls. J Toxicol Environ Health A 2007;7(12): 1046-1051.

11. Blaurock-Busch E, Amin OR, Rabah T. Heavy metals and Trace elements in hair and urine of a sample of Arab children with Autistic Spectrum Disorder. Maedica (Buchar) 2011;6(4): 247-252.

12. Russo AJ, Devito R. Analysis of Copper and Zinc Plasma Concentration and the efficacy of Zinc therapy in individuals with Asperger’s syndrome, pervasive Developmental Disorder Not Otherwise Specified (PDDNOS) and Autism. Biomarker Insights 2011; 6:127–133.

13. Shearer TR, Larson K, Neuschwander J, Gedney B. Minerals in the hair and nutrient intake of Autistic children. J Autism Dev Disord 1982; 12(1): 25-30.

14. Liberati A, Tetzlaff J, Mulrow C, et al. The PRISMA statement for reporting systematic reviews and metaanalyses of studies that evaluate healthcare interventions: explanation and elaboration. BMJ 2009; 21: 339- b2700.

15. Hartung J, Knapp G, Sinha BK. Statistical Meta- analysis with application. John Willey and Sons 2008, INC, USA.

16. Babaknejad N, Sayehmiri F, Sayehmiri K, et al. The relationship between selenium levels and breast cancer: a systematic review and meta-analysis. Biol Trace Elem Res 2014;159(1-3):1-7.

17. Al-Ayadehi LY. Heavy metals and trace elements in hair samples of autistic children in central Saudi Arabia. Neurosciences (Riyadh) 2005; 10(3):213-8.

18. Blaurock-Busch E, Amin OR, Dessoki HH, Rabah T. Toxic metals and essential elements in hair and severity of symptoms among children with Autism. Mædica J Clin Med 2012;7(1): 38-47.

19. Elsheshtawy E, Tobar S, Sherra K, et al, Study of some biomarkers in hair of children with autism. MECPsych 2011;18 18:6–10.

20. Russo AJ. Increased Copper in individuals with Autism normalizes post Zinc therapy more efficiently in Individuals with in current GI Disease. Nutr Metab Insights 2011;4: 49–54.

21. Jackson MJ, Garrod PJ. Plasma Zinc, Copper, and Amino Acid levelsin the blood of Autistic Children. J Autism Child Schizophr 1978; 8(2): 203-206.

22. Priya MDL, Geetha A. Level of trace elements (Copper, Zinc, Magnesium and Selenium) and toxic elements

(Lead and Mercury)in the Hair and Nail of Children with Autism. Biol Trace Elem Res 2011; 142(2): 148–158.

23. Wecker L, Miller SB, Cochran SR, Dugger DL, Johnson WD. Trace element concentrations in hair from autistic children. J Ment Defic Res 1985; 29(1): 15-22.

24. Adams JB, Audhya T, McDonough-Means S, et al. Nutritional and metabolic status of children with autism vs. neurotypical children, and the association with autism severity. Nutr Metab (Lond) 2011; 8(34): 1-30.

25. Adams JB, Holloway CE, George F, Quig D. Analyses of toxic metals and essential minerals in the hair of Arizona Children with Autism and associated conditions, and their mothers. Biol Trace Elem Res 2006; 110: 194-207.

26. Al-Farsi YM, Waly MI, Al-Sharbati MM, et al. Levels of heavy metals and essential minerals in hair samples of children with Autism in Oman: a Case–Control Study. Biol Trace Elem Res 2013;151(2): 181-6.

27. Russo AJ. Decreased serum Cu/Zn SOD in children with Autism. Nutr Metab Insights 2009; 2: 27-35.

28. Xia W, Zhou Y, Sun C, Wang J, Wu L. A preliminary study on nutritional status and intake in Chinese children with autism. Eur J Pediatr 2010; 169(10):1201-1205.

29. Russo AJ, Bazin AP, Bigega R, et al. Plasma Copper and Zinc Concentration in Individuals with Autism Correlate with Selected Symptom Severity. Nutr Metab Insights 2012;5: 41–47.

30. Bjørklund G. The role of zinc and copper in autism spectrum disorders. Acta Neurobiol Exp 2013; 73: 225–236.

31. Yasuda H, Yoshida K, Yasuda Y, Tsutsui T. Infantile zinc deficiency: Association with autism spectrum disorders. Sci Rep 2011; 1(129): 1-4.

32. Frye RE, Rossignol D2, Casanova MF, et al. A review of traditional and novel treatments for seizures in autism spectrum disorder: findings from a systematic review and expert panel. Front Public Health 2013; 1(31): 1-17.

33. Yasuda H, Tsutsui T. Assessment of Infantile Mineral Imbalances in Autism Spectrum Disorders (ASDs). Int J Environ Res Public Health 2013;10(11): 6027–6043.

How to Cite This Article: Akbari M, Joghataei MT, Poorbakhat A, Jenabi MS. Contra-lateral auditory Brainstem Responses in Dyslexia. Iran J Child Neurol. Autumn 2016; 10(4): 10-15.

Abstract

Objective

Dyslexia is a neurological dysfunction (also known as a learning disability) that characterized by disability in reading in spite of normal intelligence. Bothe genetic and environmental risk factors are contributing into the condition.

Diagnosis of dyslexia is based on examination and investigation of the patient’s memorial, spelling, visual, and reading skills. It is the most common learning disability, affecting 3%–10% of the school population. Modern neuroimaging techniques such as functional magnetic resonance imaging (fMRI) have shown a correlation between both functional and structural differences in the brains of children with reading difficulties. Hence, to address this issue, the auditory brainstem responses of children with dyslexia were investigated.

Materials &Methods

Fifty two children with dyslexia (30 males, 22 females) were selected after examination by speech therapist. In addition, fifty two control children were included as well. The IPSI and contralateral ABR tests were conducted on both cases and controls. Click stimuli were used at 75 nHL intensity. The study focused on absolute latency of wave V in both groups.

Results

Absolute latency of wave V in contralateral showed differences between children with dyslexia and control group, but no significant results were found in IPSI testing.

Conclusion

The current data provide an evidence for brainstem and its function in signal processing and the role of brainstem nucleus on processing and delivering the information to each hemisphere.

References

1. Lyon GR, Shaywitz SE, Shaywitz BA. A definition of dyslexia: partI - defining dyslexia, comorbidity, teachers’ knowledge of language and reading. Ann Dyslexia; 2003: 53:1-14.

2. Vellutino, F, Fletcher, J,Snowling, M, &Scalon, D. Specific reading disability (dyslexia): what have we learnt  in the past four decades? Child Psychology and Psychiatry; 2004: 45: 2–40

3. Demonet JF, Taylor MJ, Chaix Y. Developmental dyslexia. Lancet; 2004.

4. Wible B, NicolT, Kraus N. Correlation between brainstem and cortical auditory Brain. 2005; 128: 417–42.

5. Tzounopoulos T, Kraus N. Neuron. Learning to Encode Timing: Mechanisms of Plasticity in the Auditory Brainstem 2009; 62(4): 463–469.

6. Jeffery A, Winer.Decoding the auditory corticofugal systems Hearing Research (2005).207; 1–9.

7. Kraus N, Nicol T. Brainstem origins for cortical “what” and “where” pathways in the auditory system. Trends in Neurosciences. 2005; 28: 176-181.

8. KrishnanaA, XuaY, Gandoura J, Carianib P. Encoding of pitch in the human brainstem is sensitive to language experience. Cognitive Brain Research. 2005; 161 – 168.

9. Adams, M. Beginning to read: thinking and learning about print. Cambridge, Mass. MIT Press. 1990.

10. Demb, JB, Boynton G, M., &Heeger, D. J. Functional magnetic resonance imaging of early visual pathways in dyslexia. Neurosci. 1998; 18(17): 6939-6951.

11. Fiez, JA,Petersen, SE. Neuroimaging studies of word reading. PNAS. 1998; 95:914-921.

12. Hinshelwood J. Congenital word blindness. London: Lewis. 1917.

13. Morgan, WP. A case of congenital word blindness. The British Medical Journal. 1896; 2: 1378.

14. Dejerine, Suruncas de cectieverbale avec agraphie, suivid’autopsie. CR Soc. 1891; 43: 197–201.

15. Drake WE. Clinical and pathological findings in a child with a developmental learning disability. J Learn Disabil. 1968; 1: 486–502.

16. Orton ST. Reading, writing, and speech problems in children and selected papers. Austin: ProEd. 1937.

17. Geschwind, N, Galaburda, AM. Cerebral lateralization: Biological mechanisms, associations, and pathology. Cambridge, MA: MIT Press. 1987.

18. Weintraub S, Mesulam MM. Developmental learning disabilities. Arch Neurol. 1983;40(8):463-8.

19. Cohen L, Dehaene S. Neuroimage Specialization within the ventral stream.2004; 22(1):466-76.

20. Simos PG1, Fletcher JM, Bergman E, Breier JI, Foorman BR, Castillo EM, Davis RN, Fitzgerald M, Papanicolaou AC. Neurology. 2002;58(8):1203-13.

21. Wible, B, Nicol, T, Kraus N. Correlation between brainstem and cortical auditoryprocesses in normal and language-impaired children. Brain. 2005; 128: 417–423.

22. Starr A, Picton T, Sininger Y, Hood I, Berlin C. Auditory neuropathy. Brain. 1996; 119:741-753.

23. Zhang, Y. and Suga, N. Corticofugal feedback for collicular plasticity evoked by electric stimulation of the inferior colliculus. Neurophysiol. 2005; 94: 2676–2682.

24. Hood L J. Clinical applications of the auditory brainstem response. Singular Publishing Group. 1998; 49-63.

25. Dobie R A, Berlin C I. Binaural interaction in human auditory evoked responses. Archives of Otolaryngology. 1979; 105: 391-398.

26. MollerA R, Jannetta P J. Auditory evoked potentials recorded from the cochlearnucleus and its vicinity in man. Journal of Neurosurgery. 1983; 59(6): 1013–1018.

27. Moller A R, Jho H D, Yokota M, Janetta P J. Contribution from crossed and uncrossed brainstem structures to the brainstem auditory evoked potentials: a study in humans. Laryngoscope. 1995; 105(6): 596–605.

28. Wible B, Nicol T, Kraus N. Correlation between brainstem and cortical auditory processes in normal and language-impaired children. Brain. 2005; 128, 417–423.

29. Stone J L, Calderon-Arnulphi M, Watson K S. Brainstem auditory evoked potentials- a review and modified studies in healthy subjects. Journal of Clinical Neurophysiology. 2009; 26(3): 167–175.

30. Parkkonen L, Fujiki N, Mäkelä J P. Sources of auditory brainstem responses21 revisited: contribution by magnetoencephalography. Human Brain Mapping. 2009; 30(6): 1772–1782.

31. Møller AR, Jho HD, Yokota M, Jannetta PJ. Contribution from crossed and uncrossed brainstem structures to the brainstem auditory evoked potentials: a study in humans. Laryngoscope. 1995; 105(6):596–605.

32. Voordecker P, Brunko E, Beyl Z D. Selective unilateral absence or attenuationof wave V of brain-stem auditory evoked potentials with intrinsic brain-stem lesions. Archives of Neurology.1988; 45(11), 1272–1276.

33. Litovsky R Y, Fligor B J, Tramo M J. Functional role of the human inferiorcolliculus in binaural hearing. Hearing Research.2002; 165(1–2): 177–188.

34. Tzounopoulos T, Kraus N. Learning to encode timing: mechanisms of plasticity in the auditory brainstem. Neuron. 2009; 62(4): 463–469.

35. Hatanaka T, Shuto H, Yasuhara A, Kobayashi Y. Ipsilateral and contralateral recordings of auditory brainstem responses to monaural stimulation. Pediatric Neurology. 1988; 4(6): 354–357.

How to Cite This Article: Rafiey H, Soleimani F, Torkzahrani Sh, Salavati M, NASIRI M. Scale Development and Psychometrics for Parents’
Satisfaction with Developmental Care in Neonatal Intensive Care Unit. Iran J Child Neurol. Autumn 2016; 10(4):16-24.

Abstract

Objective

Developmental care comprises a wide range of medical and nursing interventions used in the neonatal intensive care unit (NICU) to mitigate and reduce stressors affecting preterm or ill neonates. Because patient satisfaction survey is a valuable quality improvement tool, we aimed to develop and test the psychometric properties of a tool for measuring parent satisfaction of developmental care in the NICU.

Materials &Methods

In this psychometric methodological study, the item pool and initial questionnaire were designed based on a comprehensive literature review and exploring NICU parent satisfaction questionnaires. The validity of the designed questionnaire was determined using face, content (qualitative and quantitative), and construct validity. Exploratory factor analysis was performed using responses from 400 parents of infants hospitalized in the NICUs of 34 hospitals in 2015 in Tehran, Iran. The reliability of the questionnaire was identified using Cronbach’s alpha and stability measures.

Results

The initial questionnaire was designed with 72 items in five domains. After testing the face validity, 3 items were omitted. The results of validity testing were acceptable. The exploratory factor analysis was performed on 69 items, and 5 factors (care and treatment with 20 items, information with 15 items, hospital facilities with 9 items, parental education with 7 items, and parental participation with 8 items) were extracted. The reliability was supported by high internal consistency (α = 0.92).

Conclusion

This questionnaire could be valid and reliable tool for measuring parents’ satisfaction.

References

1. Prakash B. Patient satisfaction. J Cutan Aesthet Surg 2010;3(3):151-5.

2. Ware JE, Snyder MK, Wright WR, Davies AR. Defining and measuring patient satisfaction with medical care. Eval Program Plann 1983;6(3):247-63.

3. Miles MS, Burchinal P, Holditch-Davis D, Brunssen S, Wilson SM. Perceptions of stress, worry, and support in Black and White mothers of hospitalized, medically fragile infants.J Pediatr Nurs 2002;17(2):82-8.

4. Pinelli J. Effects of family coping and resources on family adjustment and parental stress in the acute phase of the NICU experience. Neonatal Netw 2000;19(6):27-37.

5. Butt ML, McGrath JM, Samra HA, Gupta R. An integrative review of parent satisfaction with care provided in the neonatal intensive care unit. J Obstet Gynecol Neonatal Nurs 2013;42(1):105-20.

6. Wielenga JM, Smit BJ, Unk LK. How satisfied are parents supported by nurses with the NIDCAP® model of care for their preterm infant? J Nurs Care Qual 2006;21(1):41-8.

7. Gay G, Franck LS. Toward a standard of care for parents of infants in the neonatal intensive care unit. Crit Care Nurse 1998;18(5):62.

8. McGrath JM, Samra HA, Kenner C. Family-centered developmental care practices and research: what will the next century bring? J Perinat Neonatal Nurs 2011;25(2):165-70.

9. Sizun J, Westrup B. Early developmental care for preterm neonates: a call for more research. Arch Dis Child Fetal Neonatal Ed 2004;89(5):F384-F8.

10. Lucas N. Developmental care in the neonatal unit. Sri Lanka J Child Health 2015;44(1):45-52.

11. Lester BM, Miller RJ, Hawes K, Salisbury A, Bigsby R, Sullivan MC, et al. Infant neurobehavioral development. Semin Perinatol 2011;35(1):8-19.

12. Als H. A synactive model of neonatal behavioral organization: framework for the assessment of neurobehavioral development in the premature infant and for support of infants and parents in the neonatal intensive care environment. Phys Occup Ther Pediatr 1986;6(3-4):3-53.

13. Altimier L, Phillips RM. The Neonatal Integrative Developmental Care Model: Seven Neuroprotective Core Measures for Family-Centered Developmental Care. Newborn Infant Nurs Rev 2013;13(1):9-22.

14. Ramachandran S, Dutta S. Early developmental care interventions of preterm very low birth weight infants. Indian Pediatri 2013;50(8):765-70.

15. Voos KC, Park N. Implementing an Open Unit Policy in a Neonatal Intensive Care Unit: Nurses’ and Parents’ Perceptions. J Perinat Neonatal Nurs 2014;28(4):313-8.

16. Johnston CC, Filion F, Campbell-Yeo M, Goulet C, Bell L, McNaughton K, et al. Kangaroo mother care diminishes pain from heel lance in very preterm neonates: a crossover trial. BMC Pediatr 2008;8(1):13.

17. Blackington SM, McLauchlan T. Continuous quality improvement in the neonatal intensive care unit: evaluating parent satisfaction. J Nurs Care Qual 1995;9(4):78-85.

18. Conner JM, Nelson EC. Neonatal intensive care: satisfaction measured from a parent’s perspective. Pediatrics 1999;103(Supplement E1):336-49.

19. Salehi Z, Mokhtari Nouri J, Khademolhoseyni SM, Ebadi A. Designing and determining psychometric characteristics of satisfaction measurement questionnaire of the parents’ infants, hospitalized in Neonatal Intensive Care Unit. Iran J Crit Care Nurs 2014;7(3):176-83.

20. Latour JM, Duivenvoorden HJ, Tibboe D, Hazelzet Jan A. The shortened Empowerment of Parents in The Intensive Care 30 questionnaire adequately measured parent satisfaction in pediatric intensive care units. J Clin Epidemiol 2013;9(66):1045–1050.

21. Hajizadeh E, & Asghari, M. Statistical Methods and Analyses in Health and Biosciences, A Research Methodological Approch. Tehran: University Jahad Publishing Corrporation; 2012.

22. Carolyn Feher Waltz OLS, Elizabeth R. Lenz,. Measurement in Nursing and Health Research. Fourth ed. USA: Springer Publishing Company, LLC; 2010.

23. Hyrkäs K, Appelqvist-Schmidlechner K, Oksa L. Validating an instrument for clinical supervision using an expert panel. Int J Nurs Stud 2003;40(6):619-25.

24. Polit DF, Beck CT, Owen SV. Is the CVI an acceptable indicator of content validity? Appraisal and recommendations. Res Nurs Health 2007;30(4):459-67.

25. Lawshe CH. A quantitative approach to content validity1. Personnel Psychol 1975;28(4):563-75.

26. Rattray J, Jones MC. Essential elements of questionnaire design and development. J Clin Nurs 2007;16(2):234- 43.

27. Munro BH. Statistical methods for health care research: Lippincott Williams & Wilkins; 2005.

28. DeVellis RF. Scale Development Theory and Applications. 2nd ed: Sage Publications, Inc,Thous and Oaks.; 2003.

29. Pascoe GC. Patient satisfaction in primary health care: a literature review and analysis. Eval Program Plann 1983;6(3):185-210.

30. Ahmad I, Nawaz A, Khan S, Khan H, Rashid MA, Khan MH. Predictors of patient satisfaction. Gomal J Med Sci 2012;9(2).

31. Marley KA, Collier DA, Meyer Goldstein S. The role of clinical and process quality in achieving patient satisfaction in hospitals. Decision Sci 2004;35(3):349- 69.

32. Urden LD. Patient satisfaction measurement: current issues and implications. Prof Case Manag 2002;7(5):194- 200.

33. Jenkinson C, Coulter A, Bruster S, Richards N, Chandola T. Patients’ experiences and satisfaction with health care: results of a questionnaire study of specific aspects of care. Qual Saf Health Care 2002;11(4):335-9.

34. Al-Abri R, Al-Balushi A. Patient satisfaction survey as a tool towards quality improvement. Oman Med J 2014;29(1):3-7.

35. Hagen IH, Vadset TB, Barstad J, Svindseth MF. Development and validation of Neonatal Satisfaction Survey–NSS-13. Scand J Caring Sci 2015;29(2):395- 406.

36. Saad Andaleeb S. Determinants of customer satisfaction with hospitals: a managerial model. Int J Health Care Qual Assur 1998;11(6):181-7.

37. Dunn MS, Reilly MC, Johnston AM, Hoopes RD, Abraham MR. Development and dissemination of potentially better practices for the provision of family centered care in neonatology: the family-centered care map. Pediatrics 2006;118(Supplement 2):S95-S107.

38. Streiner DL, Saigal S, Burrows E, Stoskopf B, Rosenbaum P. Attitudes of parents and health care professionals toward active treatment of extremely premature infants. Pediatrics 2001;108(1):152-7.

39. Bracht M, O’Leary L, Lee SK, O’Brien K. Implementing family-integrated care in the NICU: a parent education and support program. Adv Neonatal Care 2013;13(2):115-26.

40. Sweeney JK, Heriza CB, Blanchard Y, Dusing SC. Neonatal physical therapy. Part II: Practice frameworks and evidence-based practice guidelines. Pediatr Phys Ther 2010;22(1):2-16.

How to Cite This Article: Hassanzadeh A, Aminzadeh V. The Comparison between Effect of Chloralhydrate and Diphenhydramine on Sedating for Electroencephalography. Iran J Child Neurol. Autumn 2016; 10(4):25-29.

Abstract

Objective

Electroencephalography (EEG) is the most effective diagnostic tool in distinguishing epileptic seizure. Chloral hydrate (CH) is a sedative hypnotic drug, commonly used as a method of sedation in children aged<3 yr.

Furthermore, diphenhydramine (DH) is a first generation antihistaminic drug (H1 receptor blocker) with anti-cholinergic effect. In this study, we aimed to compare the effects of CH and DH on sedating for EEG.

Materials & Methods

This retrospective cohort study was conducted on patients’ records of aged 15-72 months undergone an EEG and required sedation. Overall, 200 children were assessed including 100 patients in group 1 (CH) and 100 patients in group 2 (DH). Data were gathered by a form including age, sex, the cause of EEG, complication, success rate, first dose success, as well as sleep and awake latency.

Data were reported by descriptive statistics (mean, standard deviation, number, and percent) and analyzed by t-test and chi-square using SPSS 19.

Results

Totally, 113(56%) male patients with the mean age of 35.62±14.00 months participated in this study. Vomiting and agitation were the most frequent complications in CH and DH groups, respectively. Most of patients in both group indicated successful sedation. CH indicated higher rate of success by first dose toward DH. In addition, CH mentioned lower sleep latency and significant difference was noted between groups. The mean duration of awake latency was higher in DH groups which showed significant difference.

Conclusion

CH might be a more effective drug in comparison with the DH for sedation. According to the availability and low cost of DH, investigators are advised to perform further investigations. 

References

1. Marcdante K, Kliegman RM, Behrman RE, Jenson HB. Nelson essentials of pediatrics: Elsevier Health Sciences; 2010.

2. Sahyoun C, Krauss B. Clinical implications of pharmacokinetics and pharmacodynamics of procedural sedation agents in children. Curr Opin Pediatr 2012;24(2):225-32.

3. Temme J, Anderson J, Matecko S. Sedation of children for CT and MRI scanning. Radiol Technol. 1990; 61(4):283- 5.

4. Soni H. Martindale: The complete drug reference–38^th edition. Nursing Standard 2014;28(47):32-.5. Gauillard J, Cheref S, Vacherontrystram M, Martin J. Chloral hydrate: a hypnotic best forgotten? Encephale 2002;28(3 Pt 1):200-4.

6. Kay GG, Berman B, Mockoviak SH, Morris CE, Reeves D, Starbuck V, et al. Initial and steady-state effects of diphenhydramine and loratadine on sedation, cognition, mood, and psychomotor performance. Arch Intern Med 1997;157(20):2350-6.

7. Roehrs T, Zwyghuizen-Doorenbos A, Roth T. Sedative effects and plasma concentrations following single doses of triazolam, diphenhydramine, ethanol and placebo. Sleep 1993;16(4):301-5.

8. Engorn B, Flerlage J. The Harriet Lane Handbook: Mobile Medicine Series, Expert Consult: Elsevier Health Sciences; 2014.

9. Malviya S, Voepel-Lewis T, Tait A, Merkel S, Tremper K, Naughton N. Depth of sedation in children undergoing computed tomography: validity and reliability of the University of Michigan Sedation Scale (UMSS). Br J Anaesth 2002;88(2):241-5.

10. Fallah R, Jalili S, Golestan M, Karbasi SA, Jarahzadeh M-H. Efficacy of chloral hydrate and promethazine for sedation during electroencephalography in children; a randomised clinical trial. Iran J Pediatr 2013;23(1):27-31.

11. Bektas O, Arıca B, Teber S, Yılmaz A, Zeybek H, Kaymak S, et al. Chloral hydrate and/or hydroxyzine for sedation in pediatric EEG recording. Brain Dev. 2014;36(2):130- 6.

12. Roach CL, Husain N, Zabinsky J, Welch E, Garg R Moderate sedation for echocardiography of preschoolers. Pediatr Cardiol 2010;31(4):469-73.

13. Fávero ML, Ponce FAS, Pio MRB, Tabith Junior A, Silva FLC. Chloral hydrate to study auditory brainstem response. Braz J Otorhinolaryngol 2010;76(4):433-6.

14. Ashrafi MR, Mohammadi M, Tafarroji J, Shabanian R, Salamati P, Zamani GR. Melatonin versus chloral hydrate for recording sleep EEG. Eur J Paediatr Neurol 2010;14(3):235-8.

15. Heistein LC, Ramaciotti C, Scott WA, Coursey M, Sheeran PW, Lemler MS. Chloral hydrate sedation for pediatric echocardiography: physiologic responses, adverse events, and risk factors. Pediatrics 2006;117(3):e434-41.

16. Cengiz M, Baysal Z, Ganidagli S. Oral sedation with midazolam and diphenhydramine compared with midazolam alone in children undergoing magnetic resonance imaging. Paediatr Anaesth 2006;16(6):621-6.

How to Cite This Article: Rashnonejad A, Onay H, Atik T, Atan Sahin O, Gokben S, Tekgul H, Ozkinay F. Molecular Genetic Analysis of Survival Motor Neuron Gene in 460 Turkish Cases with Suspicious Spinal Muscular Atrophy Disease. Iran J Child Neurol. Autumn 2016; 10(4):30-35.

Abstract

Objective

To describe 12 yr experience of molecular genetic diagnosis of Spinal Muscular Atrophy (SMA) in 460 cases of Turkish patients.

Materials & Methods

A retrospective analysis was performed on data from 460 cases, referred to Medical Genetics Laboratory, Ege University’s Hospital, Izmir, Turkey, prediagnosed as SMA or with family history of SMA between 2003 and 2014.

The PCR-restriction fragment length polymorphism (RFLP) and the Multiplex ligation–dependent probe amplification (MLPA) analysis were performed to detect the survival motor neuron (SMN)1 deletions and to estimate SMN1 and SMN2 gene copy numbers.

Results

Using PCR-RFLP test, 159 of 324 postnatal and 18 of 77 prenatal cases were detected to have SMN1 deletions. From positive samples, 88.13% had a homozygous deletion in both exon 7 and exon 8 of SMN1. Using MLPA, 54.5% of families revealed heterozygous deletions of SMN1, and 2 or 3 copies of SMN2, suggesting a healthy SMA carrier. Among patients referred for SMA testing, the annual percentage of patients diagnosed as SMA has decreased gradually from 90.62% (2003) down to 20.83% (2014).

Conclusion

Although PCR-RFLP method is a reliable test for SMA screening, MLPA is a necessary additional test and provide relevant data for genetic counseling of families having previously affected child. The gradual decrease in the percentage of patients molecularly diagnosed as SMA shows that clinicians have begun to use genetic tests in the differential diagnosis of muscular atrophies. Cost and availability of these genetic tests has greatly attributed to their use. 

References

1. Brichta L, Holker I, Haug K, Klockgether T, Wirth B. In vivo activation of SMN in spinal muscular atrophy carriers and patients treated with valprotae. Ann Neurol 2006;59:970-5.

2. Prior TW, Krainer AR, Hua Y, Swoboda KJ, Snyder PC, Bridgeman SJ, et al. A positive modifier of spinal muscular atrophy in the SMN2 gene. Am J Hum Genet 2009;85:408-13.

3. Striano P, Boccella P, Sarappa C, Striano S. Spinal muscular atrophy and progressive myoclonic epilepsy: one case report and characteristics of the epileptic syndrome. Seizure 2004;13:582-6.

4. Wirth B. An update of the mutation spectrum of the survival motor neuron gene (SMN1) in autosomal recessive spinal muscular atrophy (SMA). Hum Mutat 2000;15:228-37.

5. Van der Steege G, Grootscholten PM, Van der Vlies P, Draaijers TG, Osinga J, Cobben JM, et al. PCR-based DNA test to confirm clinical diagnosis of autosomal recessive spinal muscular atrophy. Lancet 1995;345:985-6.

6. Rekik I, Boukhris A, Ketata S, Amri M, Essid N, Feki I, et al. Deletion analysis of SMN and NAIP genes in Tunisian patients with spinal muscular atrophy. Ann Indian Acad Neurol 2013;16:57-61.

7. de Souza Godinho FM, Bock H, Gheno TC, Saraiva-Pereira ML. Molecular Analysis of Spinal Muscular Atrophy: A genotyping protocol based on TaqMan realtime PCR. Genet Mol Biol 2012;35:955-9.

8. Burghes AH. When deletion is not a deletion? When it is converted? Am J Hum Genet 1997;61:9-15.

9. Kubo Y, Nishio H, Saito K. A new method for SMN1 and hybrid SMN gene analysis 1. in spinal muscular atrophy using long-range PCR followed by sequencing. J Hum 2. Genet 2015;60:233-9.

10. Ogino S, Leonard DG, Rennert H, Wilson RB. Spinal Muscular Atrophy Genetic Testing Experience at an Academic Medical Center. J Mol Diagn 2002;4:53-8.

11. Baumbach-Reardon L, Sacharow S, Ahearn ME. Spinal Muscular Atrophy, X-Linked Infantile. Gene Review 1993.

12. Khaniani MS, Derakhshan SM, Abasalizadeh S. Prenatal diagnosis of spinal muscular atrophy: clinical experience and molecular genetics of SMN gene analysis in 36 cases. J Prenat Med 2013;7:32-4.

13. Lin SP, Chang JG, Jong YJ, Yang TY, Tsai CH, Wang NM, et al. Prenatal prediction of spinal muscular atrophy in Chinese. Prenat Diagn 1999;19:657-61.

14. Cobben JM, Scheffer H, De visser M, Van der Steege G, Verhey JB, Osigna J, et al. Prenatal prediction of spinal muscular atrophy. Experience with linkage studies and consequences of present SMN deletion analysis. Eur J Hum Genet 1996;4:231-6.

15. Miskovic M, Lalic T, Radivojevic D, Cirkovic S, Ostojic S, Guc-Scekic M. Ten years of experience in molecular prenatal diagnosis and carrier testing for spinal muscular atrophy among families from Serbia. Int J Gynaecol Obstet 2014;124:55-8.

16. Mailman MD, Heinz JW, Papp AC, Snyder PJ, Sedra MS, Burghes AHM, Wirth B, Prior TW. Molecular analysis of spinal muscular atrophy and modification of the phenotype by SMN2. Genet Med 2002;4:20–26.

17. Ogino S, Leonard DG, Rennert H, Ewens WJ, Wilson RB. Genetic risk assessment in carrier testing for spinal muscular atrophy. Am J Med Genet 2002;110:301-7.

18. Wirth B. An update on the mutation spectrum of the survival motor neuron gene (SMN1) in autosomal recessive spinal muscular atrophy (SMA). Hum Mutat 2000;15:228–37.

How to Cite This Article: Vameghi R, Amir Ali Akbari S, Sajedi F, sajjadi H, Alavi Majd H. Path Analysis Association between Domestic Violence, Anxiety, Depression and Perceived Stress in Mothers and Children’s Development. Iran J Child Neurol. Autumn 2016; 10(4):36-48.

Abstract

Objective

Given that several factors involved in the incidence or exacerbation of developmental disorders in children, the present study aimed to investigate the relationship between some of the risk factors affecting mothers’ health and development in children using path analysis.

Materials & Methods

The present cross-sectional analytical study was conducted on 750 mothers and their children in health centers in Tehran, Iran in 2014 enrolled through multi-stage random sampling. Data were collected using a demographic and personal information questionnaire, the Perceived Stress Scale, Beck’s depression Inventory, Spielberger’ anxiety inventory, the WHO domestic violence questionnaire and an ages & stages questionnaire for assessing children’s development. Data were analyzed using SPSS.19 (Chicago, IL, USA) and Lisrel 8.8.

Results

Developmental delay was observed in 12.1% of the children. The mean stress score was 23.94±8.62 in the mothers, 50.7% of whom showed mild to severe depression, 84.2% moderate to severe anxiety and 35.3% had been subjected to domestic violence. The path analysis showed that children’s development was affected directly by perceived stress (β=-0.09) and depression (β=-0.17) and indirectly by domestic violence (β=-0.05278) and anxiety (β=-0.0357). Of all the variables examined, depression had the biggest influence on development in the children (β=-0.17). The proposed model showed a good fit (GFI=1, RMSEA=0.034).

Conclusion

Children’s development was influenced indirectly by domestic violence and anxiety and directly by perceived stress and depression in mothers. It is thus suggested that more concern and attention be paid to women’s mental health and the domestic violence they experience. 

References

1. R. de Moura D, Costa JC, Santos IS, D. Barros AJ, Matijasevich A, Halpern R, and et al. Risk factors for suspected developmental delay at age 2 years in a Brazilian birth cohort. Paediatr Perinat Epidemiol 2010; 24(3): 211–221.

2. Karimzadeh P, Kuimarsi A, Yousefi M. A Survey of Pediatrics Resident Knowledge of Growth & Development. Iran J Child Neurol 2011; 5(2):11-16.

3. Baker R. Pediatric Primary Care Well-Child Care. USA. Lippincott Williams and Wilkins Publish. 2001.

4. Sachdeva S, Amir A, Alam S, Khan Z, Khalique N, Ansari MA. Global developmental delay and its determinants among urban infants and toddlers: a cross sectional study. Indian J Pediatr 2010; 77(9):975-80.

5. Natale JE, Joseph JG, Bergen R, Thulasiraj RD, Rahmathullah L. Prevalence of childhood disability in a southern Indian city: independent effect of small differences in social status. Int J Epidemiol 1992; 21(2):367–72.

6. Bello AI, Quartey JN, Appiah LA. Screening for developmental delay among children attending a rural community welfare clinic in Ghana. BMC Pediatr 2013; 13: 119.

7. Pradilla AG, VesgaAB, Leon-Sarmiento FE. [National neuroepidemiological study in Colombia (EPINEURO)]. Rev Panam Salud Publica 2003; 14(2):104–11.

8. Guimarães AF, Carvalho DV, Machado NÁ, Baptista RA, Lemos SM. Risk of developmental delay of children aged between two and 24 months and its association with the quality of family stimulus. Rev Paul Pediatr 2013; 31(4):452-8.

9. To T, Guttmann A, Dick PT, Rosenfield JD, Parkin PC, Tassoudji M and et al. Risk markers for poor developmental attainment in young children: results from a longitudinal national survey. Arch Pediatr Adolesc Med 2004; 158(7):643–9.

10. Earls MF, Hay SS. Setting the stage for success: implementation of developmental and behavioural screening and surveillance in primary care practicethe North Carolina Assuring Better Child Health and Development (ABCD) project. Pediatrics 2006; 118(1):e183–e188.

11. Simpson GA, ColpeL, Greenspan S. Measuring functional developmental delay in infants and young children: prevalence rates from the NHIS-D. Paediatr Perinat Epidemiol 2003; 17(1):68–80.

12. Sajedi F, Vameghi R, Kraskian Mujembari A. Prevalence of undetected developmental delays in Iranian children. Child Care Health Dev 2014; 40(3):379-88.

13. Vameghi R, Amir Ali Akbari S, Sajjadi H, Sajedi F, Alavimajd H. Correlation between mothers’ depression and developmental delay in infants aged 6-18 months. Glob J Health Sci 2015; 23; 8(5):50060.

14. Shahshahani S, Vameghi R, Azari N, Sajedi F, KazemnejadA.Validity and Reliability Determination of Denver Developmental Screening Test-II in 0-6 Year-Olds in Tehran. Iran J Pediatr 2010; 20(3):313-22.

15. Yaghini O, Danesh F, Mahmoudian T, Beigi B. Evaluation of developmental delay in infants who came in for 6^th month vaccination in Isfahan city health centers. Iran J Child Neurol 2012; 6(2): 29-32.

16. Shahshahani S, Vameghi R, Azari N, Sajedi F, Kazemnejad A. Comparing the results of developmental screening of 4-60 months old children in Tehran using ASQ & PDQ. Iran Rehab J 2011; 9 (S1):3-7.

17. Ali SS.A brief review of risk-factors for growth and developmental delay among preschool children in developing countries. Adv Biomed Res 2013; 2:91.

18. Cheng ER, Poehlmann-Tynan J, Mullahy J, Witt WP. Cumulative social risk exposure, infant birth weight, and cognitive delay in infancy. Acad Pediatr 2014; 14(6):581-8.

19. Petterson S M, Albers A.Effects of poverty and maternal depression on early child development. Child Development 2001; 72(6).1794-1813.

20. Ordway M R. Depressed mothers as informants on child behavior: Methodological Issues. Res Nurs Health 2011; 34(6): 520–532.

21. Ali NS, Mahmud S, Khan A, Ali BS. Impact of postpartum anxiety and depression on child’s mental development from two peri-urban communities of Karachi, Pakistan: a quasi-experimental study. BMC Psychiatry 2013; 13:274.

22. Ahlfs-Dunn SM, Huth-Bocks AC. Intimate partner violence and infant socioemotional development: the moderating effects of maternal trauma symptoms. Infant Ment Health J 2014;35(4):322-35.

23. O’Connor TG, Heron J, Glover V: Antenatal anxiety predicts child behavioral/emotional problems independently of postnatal depression. J Am Acad Child Adolesc Psychiatry 2002, 41(12):1470-1477.

24. Murray L, Cooper PJ: Effects of postnatal depression on infant development. Arch Dis Child 1997; 77(2):99-101.

25. Latendresse G. The interaction between chronic stress and pregnancy: preterm birth from a biobehavioral perspective. J Midwifery Womens Health 2009;54(1):8-17.

26. Herring S, Gray K, Taffe J, Tonge B, Sweeney D, Einfeld S. Behavior and emotional problems in toddlers with pervasive developmental disorders and developmental delay: associations with parental mental health and family functioning. J Intellect Disabil Res 2006; 50(12):874-882.

27. Van den Bergh BR, Mulder EJ, Mennes M, Glover V. Antenatal maternal anxiety and stress and the neurobehavioural development of the fetus and child: links and possible mechanisms. A review. Neurosci Biobehav Rev 2005; 29(2):237-58.

28. Berg-Nielsen TS, Vika A, Dahl AA. When adolescents disagree with their mothers: CBCL–YSR discrepancies related to maternal depression & adolescent self-esteem. Child Care Health Dev 2003; 29(3):207-213.

29. Propper C, Rigg J. Socio-Economic Status and Child Behaviour: Evidence from a contemporary UK cohort. Centre for Analysis of Social Exclusion (CASE/125).2007. London School of Economics. http://sticerd.lse.ac.uk/case.

30. Allison SJ, Stafford J, Anumba DO. The effect of stress and anxiety associated with maternal prenatal diagnosis on feto-maternal attachment. BMC Women’s Health 2011; 11:33.

31. O’Connor TG, Heron J, Golding J, Beveridge M, Glover V. Maternal antenatal anxiety and children’s behavioural/emotional problems at 4 years: Report from the Avon Longitudinal Study of Parents and Children. Br J Psychiatry 2002; 180:502-508.

32. Brouwors Evelien PM, Baar AnneloesLVan, Pop victor JM. Maternal anxiety during pregnancy and subsequent infant development. Infant Behav Develop 2001; 24(1): 95-106.

33. Dolatian M, Hesami K, Shams J, Alavi Majd H. Relationship between violence during pregnancy and postpartum depression. Iran Red Crescent Med J 2010;12(4):377-383.

34. Ali AA, Yassin K, Omer R. Domestic violence against women in Eastern Sudan. BMC Public Health 2014; 14:1136.

35. Adeodato VG, CarvalhoRdos R, de Siqueira VR, de Matos e Souza FG. Quality of life and depression in women abused by their partners. Rev Saude Publica 2005; 39(1):108-13.

36. Malta LA, McDonald SW, Hegadoren KM, Weller CA, Tough SC. Influence of interpersonal violence on maternal anxiety, depression, stress and parenting morale in the early postpartum: a community based pregnancy cohort study. BMC Pregnancy Childbirth 2012; 12:153.

37. Forouzan AS, Dejman M, Eftekhari Baradaran M. Direct costs assault against women in Forensic Medicine in Tehran city. Payesh 2006; 5(3):201-206 (Persian).

38. Greeson MR, Kennedy AC, Bybee DI, Beeble M, Adams AE, Sullivan C. Beyond deficits: intimate partner violence, maternal parenting, and child behavior over time. Am J Community Psychol 2014; 54(1-2):46-58.

39. Holmes MR. Aggressive behavior of children exposed to intimate partner violence: an examination of maternal mental health, maternal warmth and child maltreatment. Child Abuse Negl 2013; 37(8):520-30.

40. Vameghi R, Sajedi F, Kraskian Mojembari A, Habiollahi A, Lornezhad HR, Delavar B. Cross-Cultural adaptation, validation and standardization of ages and stages questionnaire (ASQ) in Iranian Children. Iran J Public Health 2013; 42(5), 522-528.

41. Glascoe FP. Screening for developmental and behavioral problems. Mental Retard Dev Disabil Res Rev 2005; 11(3): 173–179.

42. Ahsan S, Murphy G, Kealy S, Sharif F. Current developmental surveillance: is it time for change? Irish Med J 2008; 101(4):110-2.

43. Earls MF, Hay SS. Setting the stage for success: implementation of developmental and behavioral screening and surveillance in primary care practice–the North Carolina Assuring Better Child Health and Development (ABCD) Project. Pediatrics 2006; 118 (1):e183-e188.

44. Cohen S, Kamarck T, Mermelstein R. A global measure of perceived stress. J Health Soc Behav 1983; 24(4):385-96.

45. Bastani F, Rahmatnejad L, Jahdi F, Haghani H. Breastfeeding self-efficacy and perceived stress in primiparous mothers. Iran J Nurs 2008; 21(54):9-24.(Persian)

46. Mirabzadeh A, Dolatian M, Forouzan A S, Sajjadi H, AlaviMajd H, Mahmoodi Z. Path Analysis Associations Between Perceived Social Support, Stressful Life Events and Other Psychosocial Risk Factors During Pregnancy and Preterm Delivery. Iran Red Crescent Med J 2013; 15(6): 507- 14.

47. Mohammadi Yeganeh L, Bastani F, Feizi Z, AgilarVafaie M, HaghaniH. The Effect of stress management training on mood and perceived stress in women consuming contraceptive pills. Iran J Nurs 2008; 21(53): 63-73. (Persian)

48. MomeniJavid F, Simbar M, Dolatian M, AlaviMajd H. Comparison of Pregnancy Self-Care, Perceived Social Support and Perceived Stress of Women with Gestational Diabetes and Healthy Pregnant Women. Iran J Endocrinol Metabol 2014; 16(3):156-164. (Persian).

49. Mazloom RS, Darban F,Vaghei S, Modaresgharavi M, Kashanilotfabadi M, Shad M. The effect of Stress Inoculation Program (SIP) on nurses’ Perceived stress in psychiatric wards. Evidence Based Care 2012; 2: 42-54. (Persian)

50. Bech P, Gormsen L, Loldrup D, Lunde M.The clinical effect of clomipramine in chronic idiopathic pain disorder revisited using the Spielberger State Anxiety Symptom Scale (SSASS) as outcome scale. J Affect Disord 2009; 119 (1): 43-51.

51. Court H, Greenland K, Margrain T. Measuring Patient Anxiety in Primary Care: Rasch Analysis of the 6-item Spielberger State Anxiety Scale. Value Health 2010;13(6):813-819.

52. Nasiri Amiri F. Salmalian H, Hajiahmadi M. Association between prenatal anxiety and spontaneous preterm birth. JBUMS 2009; 11 (4); 42-48.(Persian)

53. Zhang J, Gao Q. Validation of the trait anxiety scale for state-trait anxiety inventory in suicide victims and living controls of Chinese rural youths. Arch Suicide Res 2012; 16(1):85-94.

54. Razavi SH, Razavi-Ratki SK, Nojomi MM, Namiranian N. Depression and general anxiety in the prisoner of war’s children: a cross sectional study. Med J Islam Repub Iran 2012; 26(4):179-84.

55. Jakšić N, Ivezić E, Jokić-Begić N, Surányi Z, StojanovićŠpehar S. Factorial and diagnostic validity of the Beck Depression Inventory-II (BDI-II) in Croatian primary health care. J Clin Psychol Med Settings 2013; 20(3):311-22.

56. Hall BJ, Hood MM, Nackers LM, Azarbad L, Ivan I, Corsica J. Confirmatory factor analysis of the Beck Depression Inventory-II inbariatric surgery candidates. Psychol Assess 2013; 25(1):294-9.

57. Hajian S, Vakilian K, Mirzaii Najm-abadi K, Hajian P, Jalalian M. Violence against Women by Their Intimate Partners in Shahroud in Northeastern Region of Iran. Glob J Health Sci 2014; 6(3)117-130.

58. Hooper D, Coughlan J, Mullen M. Structural equation modelling: guidelines for determining model fit. EJBRM 2008; 6 (1) 53-60.

59. Deyessa N, Berhane Y, Emmelin M, Ellsberg MC, Kullgren G, Högberg U. Joint effect of maternal depression and intimate partner violence on increased risk of child death in rural Ethiopia. Arch Dis Child 2010; 95(10):771-5.

60. Avdibegović E, Sinanović O. Consequences of domestic violence on women’s mental health in Bosnia and Herzegovina. Croat Med J 2006; 47(5):730-741.

61. Burke JG, Lee LC, O’Campo P. An exploration of maternal intimate partner violence experiences and infant general health and temperament. Matern Child Health J 2008; 12(2):172-9.

62. Milgrom J, Holt C. Early intervention to protect the mother-infant relationship following postnatal depression: study protocol for a randomized controlled trial. Trials 2014;15:385.

63. Feldman R, Granat A, Pariente C, Kanety H, Kuint J, Gilboa-Schechtman E. Maternal depression and anxiety across the postpartum year and infant social engagement, fear regulation, and stress reactivity. J Am Acad Child Adolesc Psychiatry 2009; 48(9):919-27.

64. Pawlby S, Sharp D, Hay D, O’Keane V. Postnatal depression and child outcome at 11 years: the importance of accurate diagnosis. J Affect Disord 2008;107(1-3):241-5.

65. Gutteling BM, de Weerth C, Willemsen-Swinkels SH, Huizink AC, Mulder EJ, Visser GH, et al. The effects of prenatal stress on temperament and problem behavior of 27-month-old toddlers. ESCAP 2005; 14(1):41-51.

66. Laplante DP, Brunet A, Schmitz N, Ciampi A, King S. Project Ice Storm: prenatal maternal stress affects cognitive and linguistic functioning in 5 1/2-year-old children. J Am Acad Child Adolesc Psychiatry 2008; 47(9):1063-72.

67. Ciciolla L, Crnic KA, West SG. Determinants of Change in Maternal Sensitivity: Contributions of Context, Temperament, and Developmental Risk. Parent Sci Pract 2013; 13(3):178-195.

68. Ramchandani PG, Richter LM, Norris SA, Stein A. Maternal prenatal stress and later child behavioral problems in an urban South African setting. J Am Acad Child Adolesc Psychiatry 2010; 49(3):239-47.

69. O’Connor TG, Heron J, Glover V. Antenatal anxiety predicts child behavioral/emotional problems independently of postnatal depression. J Am Acad Child Adolesc Psychiatry 2002; 41(12):1470-7.

70. Tang, E, Luyten P, Casalin S, Vliegen N. Parental Personality, Relationship Stress, and Child Development: A Stress Generation Perspective. Inf Child Dev 2015;25(2):179-197.

How to Cite This Article: Amini M, Aliabadi F, Alizade M, Kalani M, Qorbani M. The Relationship between Motor Function and Behavioral Function in Infants with Low Birth Weight. Iran J Child Neurol. Autumn 2016; 10(4):49-55.

Abstract

Objective

Nowadays, the evaluation of all aspects of infant development is important. However, in practice, some of these assessments, especially those requiring more manipulation on high-risk infants, may impose additional stress on them.

Therefore, sometimes it is essential to utilize the results of a developmental assessment for the prediction of some other aspects of development. This study evaluated the relationship between the scores of the behavioral tests and the motor function test.

Materials & Methods

This cross-sectional study and was undertaken in the Neonatal Intensive Care Center and Clinic of Shahid Akbar Abadi Hospital, Tehran, Iran. A group of 50 infants with low birth weights was selected based on the easy non-contingency method and the inclusion criteria, and served as the participants. In order to assess the motor function and the behavioral performance, the motor function test (a test of infant motor performance (TIMP)) and the neonatal behavioral assessment scale (neonatal behavioral assessment scale (NBAS)) were used respectively. TIMP has both stimulation and observation sections. The items include habituation, social interaction, motor system, state organization, state regulation, autonomic system, smile, supplementary items, and the reflex.

Results

No significant association was found between the items of the habituation of behavioral testing and the observation of the movement test. There was no statistically significant relationship between the habituation and stimulation sections as well as between the system autonomous of the behavioral test and the observation section of the motor test (P>0.05). The relationship between other variables was statistically significant (P<0.05).

Conclusion

The scores of some behavioral performance items could be a good predictor of the scores of the motor function items for low birth weight infants in the neonatal period.

References

1. Wright-Ott C. Mobility. In: Case-smith J, editors. Occupational Therapy for Children. 5th ed. USA: Mosbey; 2005.P. 657-684.

2. Case-smith J. Fine motor outcomes in preschool children who receive occupational therapy services. Am J Occup Ther 1996;50(6):466-74.

3. Soleimani F, Zaheri F, Abdi F. Long-term neurodevelopmental outcomes after preterm birth. Iran Red Crescent Med J 2014;16(6):1-8.

4. Hunter JG. Neonatal Intensive Care Unite. In: Case-smith J, editors. Occupational Therapy for Children. 5th ed. USA: Mosbey; 2005.P. 688-754.

5. Arpino C, Compagnone E, Montanaro ML, Cacciatore D, De Luca A, Cerulli A, Di Girolamo S, Curatolo P. Preterm birth and neurodevelopmental outcome: a review. Childs Nerv Syst 2010;50:10-20.

6. Pedersen SJ, Sommerfelt K, Markestad T. Early motor development of premature infants with birthweight less than 2000 grams. Acta Pediatr 2000;89:1450-61.

7. Aliabadi F, Amini M, Alizade M, Kalani M, Qorbani M. Prediction of infant motor performance through performance evaluation of behavior. J Modern Rehab 2011;5 (3): 54-59.

8. Orton J, Spittle A, Doyle L, Anderson P, Boyd R. Do early intervention programs improve cognitive and motor outcomes for preterm infants after discharge: a systematic review. Dev Med Child Neurol 2009;51(11):851-9.

9. Spittle AJ, Doyle LW, Boyd RN. A systematic review of clinimetric properties of neuromotor assessment for preterm infants during the first year of life. Dev Med Child Neurol 2008;50(10):254-66.

10. Brazeltone B. Neonatal Behavioral Assessment Scale. 3rd ed. University of Masschusetts and Harvard Medical School: Mac Keit Press; 1995. 8-10, 67, P. 104-5.

11. Falk B, Eliakim A, Dotan R, Liebermann DG, Regev R, Bar-Or O. Birth weight and physical ability in 5- to -8-yr old healthy children born prematurely. Med Sci Sports Exerc 1997;29(9):1124-30.

12. Burns Y, O’Callaghan M, McDonell B, Rogers Y. Movement and motor development in ELBW infant at 1 year is related to cognitive and motor abilities at 4 years. Early Hum Dev 2004;80:19-29.

13. Tavasoli A, Aliabadi F, Eftekhari R. Motor Developmental Status of Moderately Low Birth Weight Preterm Infants. Iran J Pediatr 2014;24 (5), 581-586.

14. Islam M. The Effects of Low Birth Weight on School Performance and Behavioral Outcomes of Elementary School Children in Oman. Oman Med J 2015;30(4):241-51.

15. Ohgi S, Arisawa K, Takahashi T, Kusumoto T, Goto Y, Akiyama T, Saito H. Neonatal behavioral assessment scale as a predictor of later developmental disabilities of low-birth weight and/or premature infants. Brain Dev 2003;25:313-21.

16. Ho YB, Lee RS, Chow CB, Pang MY. Impact of massage therapy on motor outcomes in very low-birth weight infants: Randomized controlled pilot study. Pediatr Int 2010; 52(3):378-85.

17. Craciunoiu O, Holsti L. A Systematic Review of the Predictive Validity of Neurobehavioral Assessments During the Preterm Period. Phys Occup Ther Pediatr 2016; 17:1-16.

18. Tirosh E, Abadi J, Berger A, Cohen A. Relationship between neonatal behavior and subsequent temperament. Acta Pediatr 1992;81(8):29-31.

How to Cite This Article: Salehi B, Yousefichaijan P, Safi Arian S, Ebrahimi S, Naziri M. Comparison of Relation between ADHD in Children with And without Simple Febrile Seizure Admitted in Arak Amir-Kabir Hospital on 2010-2011. Iran J Child Neurol. Autumn 2016; 10(3):56-61.

Abstract

Objective

Febrile seizure is one of the most prevalent childhood convulsions with the most common age of onset at 14-18 mo old. Fever decreases the brain threshold for seizure. Attention Deficit Hyperactivity Disorder (ADHD) is also a neurologic-behavioral problem defined by attention deficit and hyperactivity according to DSM-IV criteria in which the child must have these signs in two different environments. There is controversy on the possible relation between febrile seizure and ADHD; while some studies approve a strong relation, some exclude any relation and some attribute ADHD to the side effects of other reasons.

Materials & Methods

This descriptive-analytic study enrolled all children of 3-12 yr old with febrile seizure (according to Nelson Pediatrics Textbook diagnosed by the pediatrician in charge) referring to Amir Kabir Hospital, Arak, central Iran in 2010-2011.

Overall, 103 of them with no corporeal or psychological disorder (like depression, anxiety, schizophrenia and other CNS maternal disease) were compared to 103 children of the same age and gender admitted due to disease other than febrile seizure utilizing DSM IV criteria for ADHD. Data were analyzed using SPSS version 18.

Results

The hyperactivity disorder in the control and case group was 34.3% and 16.7%, respectively, denoted a significant relation between simple febrile seizure and hyperactivity.

Conclusion

Hyperactivity has a significant relation with febrile seizure in male gender, making further investigation in these children prudent for early diagnosis and management.

References

1. Cunningham NR, Jensen P. ADHD. In: Kliegman RM, Stanton BF, Geme III JW, Schor NF, Behrman RE, eds. Nelson Textbook of pediatrics. 19th ed. Philadelphia: WB Saunders; 2011. p. 108-12.

2. Greenhill LL, Hechtman LI. Attention- Deficit Disorders. In: Sadock BJ, Sadock VA, Ruiz P, Kaplan HI, editors. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 9th ed. Philadelphia: Wolters Kluwer Health/ Lippincott Williams & Wilkins; 2009.p.1223-1231.

3. Biederman J, Faraone SV, Keenak K, Steinjard R, Tsuang M. Familial association between ADHD and anxiety disorders. APA 1991; 48: 633–642.

4. Austin JK, Perkins SM, Johnson CS, Fastenau PS. Behavior problems in children at time of first recognized seizure and changes over the following years. Epilepsy Behave 2011;21(4):373-81.

5. Baum KT, Byars AW, deGrauw TJ, Dunn DW. The effect of temperament and neuropsychological functioning on behavior problems in children with new onset seizures. Epilepsy Behave 2010; 17(4):467-73.

6. Fastenau PS, Johnson CS, Perkins SM, Byars AW. Neuropsychological status at seizure onset in children: risk factors for early cognitive deficits. Neurology 2009;73(7):526-34

7. Anna W, Kelly B, Cynthia S, Ton J. The relationship between sleep problems and neuropsychological functioning in children with first recognized seizures Epilepsy Behave 2008; 13(4):607-13.

8. Ying C, Nai W, Chao C, Shan T, Jing J. Neurocognitive Attention and Behavior Outcome of School-Age Children with a History of Febrile Convulsions: A Population Study. Epilepsies 2000; 41(4):412-20.

9. Chang YC, Guo NW, Wang ST, Huang CC. Working memory of school- aged children with a history of febrile convulsions: a population study. Neurology 2001; 57(1):37-42.

10. Hesdorffer DC, Ludvigsson P, Olafsson E, Gudmundsson G. ADHD as a Risk Factor for Incident Unprovoked Seizures and Epilepsy in Children. Arch Gen Psychiat 2004; 61(7):731-6.

11. Dunn D, Harezlak J, Ambrosius W, Austin J. Teacher assessment of behavior in children with new – onset seizures. Seizure 2002; 11(3):169-75.

12. Tsai JJ, Wang ST. Assessing the behavioral and cognitive effects of seizures on the developing brain. Prog Brain Res 2002; 135: 377-90.

13. Austin J, Harezlak J, Dunn D, Huster G. Behavior Problems in children before first recognized seizures. Pediatrics 2001; 115-122.

14. Yousefichaijan P, Eghbali A, Rafiei M, Sharafkhah M, Zol M, Firouzifar M. The Relationship between iron deficiency anemia and simple febrile convulsion in children. J Pediatr Neurosci 2014; 9: 110-114.

15. Yousefichaijan P, Salehi B, Firouzifar M, Sheikholslami H. The correlation between ADHD and enuresis in children with nocturnal enuresis. I.U.M.S, 2nd week, 2012. 184(30): 8-14.

How to Cite This Article: Mozafari H, Taghikhani M, Khatami Sh, Alaei MR, Vaisi-Raygani A, Rahimi Z. Chitotriosidase Activity and Gene Polymorphism in Iranian Patients with Gaucher Disease and Sibling Carriers. Iran J Child Neurol. Autumn 2016; 10(4):62-70.

Abstract

Objective

Chitotriosidase (CT) activity is a useful biomarker for diagnosis and monitoring of Gaucher disease (GD). Its application is limited by some variants in the CT gene. Two main polymorphisms are 24 bp duplication and G102S led to reduce CT activity. The aim of this study was to determine these variants influencing on plasma CT activity.

Materials & Methods

Blood samples were collected from 33 patients with GD, 15 sibling carriers and 105 healthy individuals serving as controls. CT activity was measured using 4-methylumbelliferyl-β-D-N,N′,N″triacetylchitotrioside substrate in plasma samples. The CT genotypes of 24 bp duplication and G102S variants were determined using PCR and PCR-RFLP.

Results

Untreated GD patients had a significantly higher CT activity compared to treated patients (P = 0.021). In addition, chitotriosidase activity in carriers was higher rather than controls. Allele frequencies of 24 bp duplication in GD patients, sibling carriers and controls were 0.21, 0.266 and 0.29 and for G102S were 0.318, 0.366 and 0.219, respectively. Different G102S genotypes had not significant effect on CT activity. Chitotriosidase activity has a positive correlation with age in normal group, carriers, and negative correlation with hemoglobin in GD patients. Using cut-off level of 80.75 nmol/ml/h, sensitivity and specificity of CT activity were 93.9% and 100%, respectively.

Conclusion

Chitotriosidase activity is a suitable biomarker for diagnosis and monitoring of GD. Determination of 24 bp duplication is helpful for more accurate monitoring the GD patient’s therapy. However, it seems that, specifying of the G102S polymorphism is not required for Iranian GD patients.

References

1. Bennett LL, Mohan D. Gaucher disease and its treatment  options. Ann Pharmacother 2013;47(9):1182-93.

2. Shrestha B, Devgan A, Sharma M. Gaucher’s disease: rare presentation of a rare disease. J Child Neurol 2013;28(10):1296-8.

3. Kanneganti M, Kamba A, Mizoguchi E. Role of chitotriosidase (chitinase 1) under normal and disease conditions. J Epithel Biol Pharmacol 2012;5:1-9.

4. Adly AA, Ismail EA, Ibraheem TM. Macrophagederived soluble CD163 level in young patients with Gaucher disease: relation to phenotypes, disease severity and complications. Int Immunopharmacol 2015;24(2):416-22.

5. Irún P, Alfonso P, Aznarez S, Giraldo P, Pocovi M.

Chitotriosidase variants in patients with Gaucher disease.  Implications for diagnosis and therapeutic monitoring. Clin Biochem 2013;46(18):1804-7.

6. Grace ME, Balwani M, Nazarenko I, Prakash- Cheng A, Desnick RJ. Type 1 Gaucher disease: null and hypomorphic novel chitotriosidase mutationsimplications for diagnosis and therapeutic monitoring. Hum Mutat 2007;28(9):866-73.

7. Woo KH, Lee BH, Heo SH, Kim JM, Kim GH, Kim YM, et al. Allele frequency of a 24 bp duplication in exon 10 of the CHIT1 gene in the general Korean population and in Korean patients with Gaucher disease. J Hum Genet 2014;59(5):276-9.

8. Wajner A, Michelin K, Burin MG, Pires RF, Pereira ML, Giugliani R, et al. Comparison between the biochemical properties of plasma chitotriosidase from normal individuals and from patients with Gaucher disease, GM1-gangliosidosis, Krabbe disease and heterozygotes for Gaucher disease. Clin Biochem 2007;40(5-6):365-9.

9. Rosén C, Andersson CH, Andreasson U, Molinuevo JL, Bjerke M, Rami L, et al. Increased Levels of Chitotriosidase and YKL-40 in Cerebrospinal Fluid from Patients with Alzheimer’s Disease. Dement Geriatr Cogn Dis Extra 2014;31;4(2):297-304.

10. Malaguarnera L. Chitotriosidase: the yin and yang. Cell Mol Life Sci 2006;63(24):3018-29.

11. Pagliardini V, Pagliardini S, Corrado L, Lucenti A, Panigati L, Bersano E, et al. Chitotriosidase and lysosomal enzymes as potential biomarkers of disease progression in myotrophic lateral sclerosis: A survey clinic-based study. J Neurol Sci 2015;15;348(1-2):245-50.

12. Fusetti F, von Moeller H, Houston D, Rozeboom HJ, Dijkstra BW, Boot RG, et al. Structure of human chitotriosidase. Implications for specific inhibitor design and function of mammalian chitinase-like lectins. J Biol Chem 2002;277:25537–25544.

13. Sista RS, Wang T, Wu N, Graham C, Eckhardt A, Bali D, et al. Rapid assays for Gaucher and Hurler diseases in dried blood spots using digital microfluidics. Mol Genet Metab 2013;109(2): 218–220.

14. Hollak CE, van Weely S, van Oers MH, Aerts JM. Marked elevation of plasma chitotriosidase activity. A novel hallmark of Gaucher disease. J Clin Invest 1994;93(3):1288–1292.

15. Old JM, Higgs DR. Gene analysis. In: Weatherall DJ, editor. Methods in hematology. The thalassemias. Vol. 6. London: Churchill Livingstone; 1983. pp.74 – 101.

16. Sinha S, Singh J, Jindal SK, Birbian N, Singla N. Association of 24 bp duplication of human CHIT1 gene with asthma in a heterozygous population of north India: a case-control study. Lung 2014;192(5):685-91.

17. Manno N, Sherratt S, Boaretto F, Coico FM, Camus CE, Campos CJ, et al. High prevalence of chitotriosidase  deficiency in Peruvian Amerindians exposed to chitinbearing food and enteroparasites. Carbohydr Polym 2014;26;113:607-14.

18. Adelino TE, Martins GG, Gomes AA, Torres AA, Silva DA, Xavier VD, et al. Biochemical and Molecular Chitotriosidase Profiles in Patients with Gaucher Disease Type 1 in Minas Gerais, Brazil: New Mutation in CHIT1 Gene. JIMD Rep 2013;9:85-91.

19. van Dussen L, Hendriks EJ, Groener JE, Boot RG, Hollak CE, Aerts JM. Value of plasma chitotriosidase to assess non-neuronopathic Gaucher disease severity and progression in the era of enzyme replacement therapy. J Inherit Metab Dis 2014;37(6):991-1001.

20. Weinreb NJ, Aggio MC, Andersson HC, Andria G, Charrow J, Clarke JT, et al. Gaucher disease type 1: revised recommendations on evaluations and monitoring for adult patients. Semin Hematol 2004;41:15–22.

21. Czartoryska B, Tylki-Szymańska A, Górska D. Serum chitotriosidase activity in Gaucher patients on enzyme replacement therapy (ERT). Clin Biochem 1998;3(5):417-20.

22. Arndt S1, Hobbs A, Sinclaire I, Lane AB. Chitotriosidase deficiency: a mutation update in an african population. JIMD Rep 2013;10:11-6.

23. Lee P, Waalen J, Crain K, Smargon A, Beutler E. Human chitotriosidase polymorphisms G354R and A442V associated with reduced enzyme activity. Blood Cells Mol Dis 2007;39(3):353-60.

24. Chien YH, Chen JH, Hwu WL. Plasma chitotriosidase activity and malaria. Clin Chim Acta 2005 ;353(1-2):215 25. Bussink AP, Verhoek M, Vreede J, Ghauharalivan der Vlugt K, Donker-Koopman WE, Sprenger RR, et al. Common G102S polymorphism in chitotriosidase differentially affects activity towards 4-methylumbelliferyl substrates. FEBS J 2009;276(19):5678-88.

26. Aerts JM, Kallemeijn WW, Wegdam W, Joao Ferraz M, van Breemen MJ, Dekker N, et al. Biomarkers in the diagnosis of lysosomal storage disorders: proteins, lipids, and inhibodies. J Inherit Metab Dis 2011;34(3):605-19.

27. Giraldo P, Cenarro A, Alfonso P, Pérez-Calvo JI, Rubio- Félix D, Giralt M, et al. Chitotriosidase genotype and plasma activity in patients type 1 Gaucher’s disease and their relatives (carriers and non carriers). Haematologica 2001;86(9):977-84.

28. Pocovi M, Cenarro A, Civeira F, Torralba MA, Perez- Calvo JI, Mozas P, et al. Beta-glucocerebrosidase gene locus as a link for Gaucher’s disease and familial hypoalpha- lipoproteinaemia. Lancet 1998;351(9120):1919-23.

29. Fluiter K, van der Westhuijzen DR, van Berkel TJ. In vitro regulation of scavenger receptor BI and the selective uptake of high density lipoprotein choles-teryl esters in rat liver parenchymal and Kupffer cells. J Biol Chem 1998; 273:8434-8.

30. Ries M, Schaefer E, Lührs T, Mani L, Kuhn J, Vanier MT, et al. Critical assessment of chitotriosidase analysis in the rational laboratory diagnosis of children with Gaucher disease and Niemann-Pick disease type A/B and C. J Inherit Metab Dis 2006;29:647–652.

31. Kurt I, Abasli D, Cihan M, Serdar MA, Olgun A, Saruhan E, et al. Chitotriosidase Levels in Healthy Elderly Subjects. Ann N Y Acad Sci 2007;1100:185-8.

32. Tamanaha P, D’Almeida V, Calegare BF, Tomita LY, Bittencourt LR, Tufik S. 24 bp duplication of CHIT1 gene and determinants of human chitotriosidase activity among participants of EPISONO, a population-based cross-sectional study, São Paulo, Brazil. Clin Biochem 2013;46(12):1084-8.

33. Dodelson de Kremer R, Paschini de Capra A, Angaroni CJ, Giner de Ayala A. Plasma chitotriosidase activity in Argentinian patients with Gaucher disease, various lysosomal diseases and other inherited metabolic disorders. Medicina (B Aires). 1997;57(6):677-84.

34. Goldim MP, Garcia Cda S, de Castilhos CD, Daitx VV, Mezzalira J, Breier AC, et al. Screening of high-risk Gaucher disease patients in Brazil using miniaturized dried blood spots and leukocyte techniques. Gene 2012;508(2):197-8.

How to Cite This Article: Shiari R, Tabatabaei Nodushan SMH, Mohebbi MM, Karimzadeh P, Javadzadeh M. Moyamoya Syndrome Associated with Henoch-Schönlein Purpura. Iran J Child Neurol. Autumn 2016; 10(4):71-74.

Abstract

Some reports have shown the association between Moyamoya syndrome and
autoimmune diseases. Herewith, we present a 3.5 yr old girl with Henoch-
Schönleinpurpura (HSP) who was treated with steroids because of sever
colicky abdominal pain. However, central nervous system manifestations such
as headache, ataxia and vision impairment developed during 6 months of her
outpatient follow-up. More evaluation using MRA revealed intracranial stenosis
of internal carotid artery and arterial collaterals that were in favor of Moyamoya
syndrome. To our knowledge, this is the first report of Moyamoya syndrome
following henoch-schönleinpurpura.

References

1. Aicardi J. Diseases of the nervous system in childhood, 2nd ed. London: Mac Keith Press, 1998. pp. 554–556.

2. Currie S, Raghavan A, Batty R, Connolly DJ, Griffiths PD. Childhood moyamoya disease and moyamoya syndrome: a pictorial review. Pediatr Neurol 2011; 44:401–13.

3. Suzuki J, Takaku A. Cerebrovascular, “moyamoya” disease. Disease showing abnormal net-like vessels in  base of brain. Arch Neurol 1969; 20:288–99.

4. Smith ER, Scott RM. Spontaneous occlusion of the circle of Willis in children: pediatric moyamoya summary with proposed evidence-based practice guidelines. A review. J Neurosurg Pediatr. 2012 Apr;9(4):353-60.

5. Ozen S, Ruperto N, Dillon MJ, Bagga A, Barron K, Davin JC, et al. EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides. Ann Rheum Dis. 2006; 65(7): 936-941.

6. Gardner-Medwin JM, Dolezolova P, Cummins C, Southwood TR. Incidence of Henoch-Schonleinpurpura, Kawasaki disease, and rare vasculitides in children of different ethnic origins. Lancet. 2002; 360: 1197-1202.

7. Aalberse J, Dolman K, Ramnath G, Periera RR, Davin JC. Henoch-Schönleinpurpura in children: an epidemiological study among Dutch paediatricians on incidence and diagnostic criteria. Ann Rheum Dis. 2007; 66(12): 1648-1650.

8. Saulsbury FT. Henoch-Schönleinpurpura in children. Report of 100 patients and review of the literature. Medicine (Baltimore). 1999; 78(6): 395-409.

9. Tarvin SE, Ballinger S. Henoch-Schonleinpurpura. Current Paediatrics. 2006; 16: 259-263.

10. Cahide Y, Hu¨seyin, Şükrü Arslan C et al. Bilateral brachial plexopathy complicating henoch-schönleinpurpura. Brain & Development 28 (2006) 326–328.

11. Bellman AL, Leicher CR, Moshe SL, Mezey AP. Neurologic manifestations of henoch–schönleinpurpura: Report of three cases and review of the literature. Pediatrics 1985; 75:687-92.

12. Reza Shiari. Neurologic Manifestations of Childhood Rheumatic Diseases. Iran J Child Neurol 2012; 6(4): 1-7.

13. Lee YJ, Yeon GM, Nam SO, Kim SY. Moyamoya syndrome occurred in a girl with an inactive systemic lupus erythematosus. Korean J Pediatr 2013; 56(12): 545–549.

14. Seol HJ, Wang KC, Kim SK, Hwang YS, Kim KJ, Cho BK. Headache in pediatric moyamoya disease: review of 204 consecutive cases. J Neurosurg 2005; 103: Suppl: 439-42.

15. Suzuki J, Kodama N. Moyamoyadisease — a review. Stroke 1983; 14:104-9.

16. Baba T, Houkin K, Kuroda S. Novel epidemiological features of moyamoya disease. J Neurol Neurosurg Psychiatry 2008; 79:900-4.

17. Tanghetti B, Capra R, Giunta F, Marini G, Orlandini A. Moyamoya syndrome in only one of two identical twins: case report. J Neurosurg 1983; 59:1092-4.

18. Guey S, Tournier-Lasserve E, Kossorotoff M. Moyamoya disease and syndromes: from genetics to clinical management. Appl Clin Genet 2015; 8: 49–68.

19. Scott RM, Smith JL, Robertson RL, Madsen JR, Soriano SG, Rockoff MA. Long-term outcome in children with moyamoya syndrome after cranial revascularization by pialsynangiosis. J Neurosurg 2004; 100: Suppl: 142-9.

20. Lubman DI, Pantelis C, Desmond P, Proffitt TM, Velakoulis D. Moyamoya disease in a patient with schizophrenia. J Int Neuropsychol Soc 2003; 9:806-10.

21. Smith ER, Scott RM. Moyamoya Disease and Moyamoya Syndrome. N Engl J Med 2009; 360:1226-37.

22. Research Committee on the Pathology and Treatment of Spontaneous Occlusion of the Circle of Willis. Guidelines for diagnosis and treatment of moyamoya disease Neurol Med Chir (Tokyo) 2012; 52(5):245–266.

23. Fung LW, Thompson D, Ganesan V. Revascularisation surgery for paediatricmoyamoya: a review of the literature. Childs Nerv Syst 2005; 21(5):358–364.

How to Cite This Article: Saeedi Borujeni MJ, Esfandiary E, Almasi Dooghaee M. Childhood Neurogenic Stuttering Due to Bilateral Congenital Abnormality in Globus Pallidus: A Case Report and Review of the Literature. Iran J Child Neurol. Autumn 2016; 10(4):75-79.

Abstract

Objective

The basal ganglia are a group of structures that act as a cohesive functional unit. They are situated at the base of the forebrain and are strongly connected with the cerebral cortex and thalamus. Some speech disorders such as stuttering can resulted from disturbances in the circuits between the basal ganglia and the language motor area of the cerebral cortex. Stuttering consists of blocks, repetitive, prolongation or cessation of speech. We present a 7.5 -year-old male child with bilateral basal ganglia lesion in globus pallidus with unclear reason.

The most obvious speech disorders in patient was stuttering, but also problems in swallowing, monotone voice, vocal tremor, hypersensitivity of gag reflex and laryngeal dystonia were seen. He has failed to respond to drug treatment, so he went on rehabilitation therapy when his problem progressed.

In this survey, we investigate the possible causes of this type of childhood neurogenic stuttering.

References

1. Daliri A, Prokopenko RA, Flanagan JR, Max L. Control and Prediction Components of Movement Planning in Stuttering Versus Nonstuttering Adults. J Speech Lang Hear Res 2014;57(6):2131-41.

2. Kasbi F, Mokhlesin M, Maddah M, Noruzi R, Monshizadeh L, Khani MMM. Effects of Stuttering on Quality of Life in Adults Who Stutter. Middle East J Rehab Health 2015;2(1). e43352, DOI: 10.17795/ mejrh-25314

3. Alm PA. Stuttering and the basal ganglia circuits: a critical review of possible relations. J Communicat Disord 2004;37(4):325-69.

4. Groenewegen HJ. The basal ganglia and motor control. Neural Plasticity 2003;10(1-2):107-20.

5. Van Schouwenburg MR, Onnink AMH, Ter Huurne N, Kan CC, Zwiers MP, Hoogman M, et al. Cognitive flexibility depends on white matter microstructure of the basal ganglia. Neuropsychologia 2014;53:171-7.

6. Albin RL, Young AB, Penney JB. The functional anatomy of basal ganglia disorders. Trends Neurosci 1989;12(10):366-75.

7. Tani T, Sakai Y. Analysis of five cases with neurogenic stuttering following brain injury in the basal ganglia. J Fluency Disord 2011;36(1):1-16.

8. Ludlow CL, Loucks T. Stuttering: a dynamic motor control disorder. J Fluency Disord 2004;28(4):273-95.

9. Van Borsel J, Van Der Made S, Santens P. Thalamic stuttering: A distinct clinical entity? Brain Language 2003;85(2):185-9.

10. Nambu A. Functional Circuitry of the Basal Ganglia. Deep Brain Stimulation for Neurological Disorders: Springer; 2015. p. 1-11.

11. Jin X, Tecuapetla F, Costa RM. Basal ganglia subcircuits distinctively encode the parsing and concatenation of action sequences. Nature Neurosci 2014;17(3):423-30.

12. Volkmann J, Hefter H, Lange H, Freund H-J. Impairment of temporal organization of speech in basal ganglia diseases. Brain Lang 1992;43(3):386-99.

13. Civier O, Bullock D, Max L, Guenther FH. Computational modeling of stuttering caused by impairments in a basal ganglia thalamo-cortical circuit involved in syllable selection and initiation. Brain Lang 2013;126(3):263-78.

14. Giraud A-L, Neumann K, Bachoud-Levi A-C, von Gudenberg AW, Euler HA, Lanfermann H, et al. Severity of dysfluency correlates with basal ganglia activity in persistent developmental stuttering. Brain Lang 2008;104(2):190-9.

15. Mink JW. The basal ganglia and involuntary movements: impaired inhibition of competing motor patterns. Arch Neurol 2003;60(10):1365-8.

16. Watkins KE, Smith SM, Davis S, Howell P. Structural and functional abnormalities of the motor system in developmental stuttering. Brain 2008;131(1):50-9.

17. Allert N, Kelm D, Blahak C, Capelle H-H, Krauss JK. Stuttering induced by thalamic deep brain stimulation for dystonia. J Neural Transm (Vienna) 2010;117(5):617-20.

18. Dietz J, Noecker AM, McIntyre CC, Mikos A, Bowers D, Foote KD, et al. Stimulation region within the globus pallidus does not affect verbal fluency performance. Brain Stimulation 2013;6(3):248-53.

19. Ullman MT. Is Broca’s area part of a basal ganglia thalamocortical circuit? Cortex 2006;42(4):480-5.

How to Cite This Article: Tavasoli AR, Gharib B, Alizadeh H, Farshadmoghaddam H, Memarian S, Ashrafi MR, Sharifzade M. Brain on FIRES: Super refractory seizure in a 7 year old boy. Iran J Child Neurol. Autumn 2016; 10(4):80-85.

Abstract

We present a 7 year old boy afflicted with super-refractory seizure that responded poorly to antiepileptic drugs and sustained a long course of hospitalization and complications of high doses of medications as well as longstanding stay in hospital. The differential diagnoses were, fever-induced refractory epileptic encephalopathy (FIRES), and infectious and autoimmune encephalitis.

However, work-ups had not revealed any evidence of any specific diagnosis, so we assumed that he was afflicted by viral infectious encephalitis as he had, fever, vomiting, and prodromal symptoms of infectious (most probably viral) disease prior to onset of the seizure attacks.

References

1. William H. Theodore, Epilepsy and Viral Infections, Epilepsy Currents, Vol. 14, No. 1 Supplement 2014 pp. 35–42

2. Rima Nabbout, AnnamariaVezzani, Olivier Dulac, Catherine Chiron. Acute encephalopathy with inflammation-mediated status epilepticus. Lancet Neurol 2011; 10: 99–108.

3. Uri Kramer, Ching-Shiang Chi, Kuang-Lin Lin, Nicola Specchio, Mustafa Sahin, Heather Olson, Rima Nabbout, Gerhard Kluger, Jainn-Jim Lin, Andreas van Baalen. Febrile infection–related epilepsy syndrome (FIRES): Pathogenesis, treatment, and outcome, A multicenter study on 77 children. Epilepsia ; 201152(11):1956–1965.

4. Yael Hacohen, Sukhvir Wright, Patrick Waters, Shakti Agrawal, Lucinda Carr, Helen Cross et al. Paediatric autoimmune encephalopathies: clinical features, laboratory investigations and outcomes in patients with or without antibodies to known central nervous system autoantigens. J Neurol Neurosurg Psychiatry 2012;0:1–8.

5. Pedro J, Serrano-Castro, Pablo Quiroga-Subirana, Manuel Payan-Ortiz, Javier Fernandez-Perez. The expanding spectrum of febrile infection-related epilepsy syndrome (FIRES). Seizure 22 (2013), 153-155.

6. R Kneen BD, Michael E, Menson B, Mehta A, Easton C, Hemingway PE, Klapper A, Vincent M, Lim E, Carrol T. Solomon, On behalf of the National Encephalitis Guidelines Development and Stakeholder Groups. Management of suspected viral encephalitis in children e Association of British Neurologists and British Paediatric Allergy Immunology and Infection Group National Guidelines. J Infect 2012; 64, 449-477.

7. Raoul Sutter, Stephan Rüegg. Refractory Status Epilepticus: Epidemiology, Clinical Aspects and Management of a Persistent Epileptic Storm. Epileptologie 2012; 29: 186 – 193.

8. Simon Shorvon. Super-refractory status epilepticus: An approach to therapy in this difficult clinical situation. Epilepsia, 52(Suppl. 8):53–56, 2011.

9. Mustafa AM, Salih Heba Y, El Khashab Hamdy H, Hassan Amal Y. Kentab, Sara S. Al Subaei, Radwan M. Zeidan, Mohammed N. Al-Nasser, Saleh A. Othman. A Study on Herpes Simplex Encephalitis in 18 Children, Including 3 Relapses. Open Pediatr Med J 2009, 3, 48-57.

10. Thais Armangue, Josep O. Dalmau. Autoimmune Encephalitis. In: Robert M. Kliegman, Bonita F. Stanton, Joseph W. St. Geme III, Nina F. Schor, Richard E. Behrman, et al, editors. Nelson Textbook of Pediatrics, 20th Edition. Philadelphia, United States of America: Saunders; 2016: p. 2905-2910.

How to Cite This Article: Harsh V, Chengazhacherril R.B, SharmaK, Kalakoti P, Gupta U, Ahmad W, Kumar A. Sciatic Nerve Injection Palsy in Children. Iran J Child Neurol. Autumn 2016; 10(4):86-87.

Letter to Editor 

Pls see PDF file.

References

1. Toopchizadeh V, Barzegar M, Habibzadeh A. Sciatic Nerve Injection Palsy in Children, Electrophysiologic Pattern and Outcome: A Case Series Study. Iran J Child Neurol 2015 Summer;9(3):69-72.

2. Sunderland S. Miscellaneous causes of nerve injury. London: Churcill Livingstone; 1991.

3. Lehmann HC, Zhang J, Mori S, Sheikh KA. Diffusion tensor imaging to assess axonal regeneration in peripheral nerves. Exp Neurol 2010;223(1):238-44.

4. Takagi T, Nakamura M, Yamada M, Hikishima K, Momoshima S, Fujiyoshi K, et al. Visualization of peripheral nerve degeneration and regeneration: monitoring with diffusion tensor tractography. NeuroImage 2009 ;44(3):884-92.

5. Barry JM, Harsh V, Kumar A, Patil S. Injection nerve palsy: What’s to blame? J Neurosci Rural Pract 2013;4:481.

6. Greensmith JE, Murray WB. Complications of regional anesthesia. Current opinion in anaesthesiology. 2006;19(5):531-7.

7. Barry JM, Harsh V, Patil S. Are our intramuscular injections nerve-friendly? What are we missing? Simple techniques to prevent, recognize and manage nerve injection injuries. Int J Stud Res 2014;4(2):25-8.