Cover page

Review Article

Approach To The First Unprovoked Seizure- PART II


Iranian Journal of Child Neurology, Vol. 7 No. 4 (2013), , Page 1-5

How to Cite This Article: Ghofrani M. Approach To The First Unprovoked Seizure- PART II. Iran J Child Neurol. 2013 Autumn; 7(4):1-5.

The approach to a child who has experienced a first unprovoked generalized tonic-clonic seizure is challenging and at the same time controversial.

How to establish the diagnosis, ways and means of investigation and whether treatment is appropriate, are different aspects of this subject. 

In this writing the above mentioned matters are discussed.


31.Berg AT, Testa FM., Levy SR, Shinnar S. Neuroimaging in children with newly diagnosed epilepsy. A community based study. Pediatrics 2000;106:527-532.

32.Shinnar S, Odell C. Treating childhood seizure; when and for how long. In: Shinnar S, Amir N, Branski D (Eds). Childhood seizure. S Karger Basel. 1995. P.100-110.

33.Shinnar S, Berg AT, Moshe Sl, et al. Risk of Seizure recurrence following a first unprovoked seizure in childhood; A prospective study. Pediatrics 1990;85:1076-2085.

34.Shinnar S, Berg At, Moshe SL, et al. The risk of seizure recurrence after a first unprovoked febrile seizure in childhood: An extended follow up. Pediatrics 1996:98:216-225.

35.Hauser WA, Rich SS, Annegers JF, Anderson VE. Seizure recurrence after a first unprovoked seizure: An extended follow up. Neurology 1990;40:1163-1170.

36.Stroink H, Brouwer O F, Arts WF, Greets AT, Peter AC, Van Donselaar CA. The First unprovoked, untreated seizure in childhood: A hospital based study of the accuracy of diagnosis, rate of recurrence, and long term outcome after recurrence. Dutch study of epilepsy in childhood. J Neurol Neurosurg Psychiatry 1998;64:595-600.

37.Shinnar S, Berg AT, O’Dell C. Newstein D, et al. Predictors of multiple seizure in a cohort of children prospectively followed from the time of their first unprovoked seizure, Ann Neurol 2000; 48:140-147.

38.Martinovie Z, Jovic N. Seizure recurrence after a first generalized tonic-clonic seizure in children, adolescents and young adult. Seizure 1997;6:461-565.

39.Berg AT, Shinnar S, Levy SR, Testa FM, et al. Early development of intractable epilepsy in children: A prospective study. Neurology 2001;56:1445-1452.

40.Berg At, Shinnar S. The risk of seizure recurrence following a first unprovoked seizure: A quantitative review. Neurology 1991;41:955-972.

41.Camfield PR, Camfield CS, Dooley JM, et al. Epilepsy after a first unprovoked seizure in childhood, Neurology 1985;35:1657-1660.

42.Commission on epidemiology and prognosis. International League Against Epilepsy. Guidelines for epidemiologic studies on epilepsy. Epilepsia 1993;37:592-596.

43.Annegers JF, Shirts SB, Hauser WA, et al. Risk of recurrence after an initial unprovoked seizure. Epilepsia 1986;27:43-50.

44.Shinnar S, Berg AT, Moshe SL, Shinnar R. How long do new –onset seizures in children last? Ann Neurol 2001;49:659-664.

45.Camfield P, Camfield C, Dooley J, et al. A randomized study of carbamazepine versus no medication after a first unprovoked seizure in childhood. Neurology 1989;39:851-852.

46.Chandra B. First Seizure in adult: to treat or not to treat. Clin Neurol Neurosurg 1992;94:861-863.

47.Musico M, Beghi E, Solari A, Viani F. Treatment of first Tonic-Clonic Seizure does not improve the prognosis of epilepsy. Neurology 1997;49:991-998.

48.American Academy of Pediatrics. Behavioral and cognitive effects of anticonvulsant therapy (RE9537). Approach To The First Unprovoked Seizure-PART II. Pediatrics 1995;96:538-540.

49.Yerby MS. Teratogenic effects of antiepileptic drugs: what do we advise patients? Epilepsia 1997;38-957-958.

50.Vinig EP, Melits ED. Dorsen MM, et al. Psychologic and behavioral effects of antiepileptic drugs in children: A double-blind comparison between Phenobarbital and valproic acid. Pediatrics 1987;80: 165-174.

51.Berg I, Butler A, Ellis M, Foster J. Psychiatric aspects of epilepsy in childhood treated with carbamazepine, phenytoin, or sodium valporate: a random trial. Dev Med Child Nerol 1993;35:149-157.

52.Aman MG, Werry JS, Paxton JW, et al. Effects of carbamazepine on psychomotor performance in children as a function of drug concentration, seizure type, and time of medication. Epilepsia 1990;31:51-60.

53.Aman MG, Werry JS, Turbott SH. Effects of phenytoin on cognitive-motor performance in children as a function of drug concentration, seizure type and time of medication. Epilepsia 1994;35:172-180.

54.Thilothammal N, Banu K, Tatnam BS. Comparison of Phenobarbiton, phenytion with sodium valproate. Randomized double blind study. Indian Pediatr 1996;33:549-555.


Research Article

The Efficacy and Safety of Topiramate for Prophylaxis of Migraine in Children


Iranian Journal of Child Neurology, Vol. 7 No. 4 (2013), , Page 7-11

How to Cite This Article: Fallah R, Akhavan Karbasi S, Shajari A, Fromandi M. The Efficacy and Safety of Topiramate for Prophylaxis of Migraine in Children. Iran J Child Neurol. 2013 Autumn; 7(4):7-11.

Migraine is the most common acute intermittent primary headache in children and prophylactic therapy is indicated in children with frequent or disabling headaches. The purpose of this study was to evaluate the efficacy and safety of topiramate (TPM) for migraine prophylaxis in children.

Materials & Methods
In a quasi-experimental study, monthly frequency, severity and duration of headache, migraine disability, and side-effects were evaluated in 100 children who were referred to the Pediatric Neurology Clinic of Shahid Sadoughi University of Medical Sciences, Yazd, Iran from April 2011 to March 2012, and were treated with 3 mg/kg/day of TPM for three months.

Fifty eight (57.4%) girls and 42 (41.6%) boys with the mean age of 10.46±2.11 years were evaluated. Monthly frequency, severity, and duration of headache decreased with treatment from 15.34±7.28 to 6.07±3.16 attacks, from 6.21±1.74 to 3.15±2.22, and from 2.28±1.55 to 0.94±0.35 hours, respectively, and the Pediatric Migraine Disability Assessment score reduced with TPM from 32.48±9.33 to 15.54±6.16. Transient side-effects were seen in 21% of the patients, including hyperthermia in 11%, anorexia and weight loss in 6%, and drowsiness in 4%. No serious side-effects were reported. 

TPM could be considered as a safe and effective drug in pediatric migraine prophylaxis.



  1. Hershey AD. Migraine. In: Kliegman RM, Stanton BF, Schor NF, St. Geme JW, Behrman RE, editors Nelson Textbook of Pediatrics. 19th ed. Philadelphia: Saunders;
    2011. p. 2040-5.
  2. The International Classification of Headache Disorders:2nd ed. Headache Classification Subcommittee of the International Headache Society. Cephalalgia 2004;24 Suppl 1:9-160.
  3. Hershey AD, Winner PK. Pediatric migraine: recognition and treatment. J Am Osteopath Assoc 2005;105(4 Suppl 2):2S-8S.
  4. Jayapal S, Maheshwari N. Question 3. Topiramate for chronic migraine in children. Arch Dis Child  2011;96(3):318-21.
  5. Fallah R. Topiramate as a new antiepileptic drug in epileptic children in Iran. Indian J Pediatr 2006;73(12):1073-5.
  6. Hershey AD, Powers SW, Vockell ALB, LeCates SL, Kabbouche MA, Maynard MK. PedMIDAS: Development of a questionnaire to assess disability of migraines in children. Neurology 2001;57(11):2034-9.
  7. Wewers ME, Lowe NK. A critical review of visual analogue scales in the measurement of clinical phenomena. Res Nurs Health 1990;13(4):227-36.
  8. Ashtari F, Shaygannejad V, Akbari M. A doubleblind, randomized trial of low-dose topiramate vs propranolol in migraine prophylaxis. Acta Neurol Scand 2008;118(5):301-5.
  9. Tonekaboni SH, Ghazavi A, Fayyazi A, Khajeh A, Taghdiri MM, Abdollah Gorji F, Azargashb E.Prophylaxis of childhood migraine: Topiramate versus Propranolol. Iran J Child Neurol 2013 ; 7(1):9-14.
  10. Winner P, Pearlman EM, Linder SL, Jordan DM, Fisher AC, Hulihan J; Topiramate Pediatric Migraine Study Investigators. Topiramate for migraine prevention in children: a randomized, double-blind, placebo-controlled trial. Headache 2005;45(10):1304-12.
  11. Lewis D, Winner P, Saper J, Ness S, Polverejan E, Wang S, et al. Randomized, double-blind, placebo-controlled study to evaluate the efficacy and safety of topiramate for migraine prevention in pediatric subjects 12 to 17 years of age. Pediatrics 2009;123(3):924-34.
  12. Borzy JC, Koch TK, Schimschock JR. Effectiveness of topiramate in the treatment of pediatric chronic daily headache. Pediatr Neurol 2005;33(5):314-6.
  13. Campistol J, Campos J, Casas C, Herranz JL. Topiramate in the prophylactic treatment of migraine in children. J Child Neurol 2005;20(3):251-3.
  14. Cruz MJ, Valencia I, Legido A, Kothare SV, Khurana DS, Yum S, et al. Efficacy and tolerability of topiramate in pediatric migraine. Pediatr Neurol 2009;41(3):167-70.
  15. Aydin M, Kabakus N, Bozdag S, Ertugrul S. Profile of children with migraine. Indian J Pediatr 2010;77(11):1247-51.
  16. Unalp A, Uran N, Oztürk A. Comparison of the effectiveness of topiramate and sodium valproate in pediatric migraine. J Child Neurol 2008;23(12):1377-81.
  17.  Lakshmi CV, Singhi P, Malhi P, Ray M. Topiramate in the prophylaxis of pediatric migraine: a double-blind placebo-controlled trial. J Child Neurol 2007;22(7):829-35.
  18. Vollono C, Ferraro D, Valeriani M. Antiepileptic drugs in the preventive treatment of migraine in children and adolescents. Drug Development Research 2007;68:355-9. 
    19. Ferraro D, Di Trapani G. Topiramate in the prevention of pediatric migraine: literature review. J Headache Pain 2008;9(3):147-50.

The Impacts of Hinged and Solid Ankle-Foot Orthoses on Standing and Walking in Children with Spastic Diplegia


Iranian Journal of Child Neurology, Vol. 7 No. 4 (2013), , Page 12-19

How to Cite This Article: Dalvand H, Dehghan L, Feizi A, Hosseini SA, Amirsalari S. Iran J Child Neurol. 2013 Autumn; 7(4):12-19.

Objective The purpose of this study was to examine the impacts of hinged and solid anklefoot orthoses (AFOs) on standing and walking abilities in children with spastic diplegia.

Materials & Methods
In a quasi-experimental design, 30 children with spastic diplegia, aged 4-6 years were recruited. They were matched in terms of age, IQ, and level of GMFCS E&R. Children were randomly assigned into 3 groups: a hinged AFO group (n=10) plus occupational therapy (OT), a solid AFO group (n=10) plus OT, a control group who used only OT for three months. Gross motor abilities were
measured using Gross Motor Measure Function (GMFM).

We obtained statistically significant differences in the values between baseline and after treatment in all groups. The groups were also significantly different in total GMFM after intervention. Furthermore, there were differences between hinged AFOs and solid AFOs groups, and between hinged AFOs and control groups.

We concluded that gross motor function was improved in all groups; however, hinged AFOs group appears to improve the gross motor function better than solid AFOs and control groups.


  1. Miller F. Cerebral palsy. 1st ed. New York: Springer Science & Business Media; 2005.
  2. Wren TL, Rethlefsen S, Kay RM. Prevalence of specific gait abnormalities in children with cerebral palsy: Influence of cerebral palsy subtype, age, and previous surgery. J Pediat Orthoped 2005;25(1):79-83.
  3. Knutosn L, Clark D. Orthotic devices for ambulation in children with cerebral palsy and myelomeningocele. Phys Ther 1991;71:947-60.
  4. Figueiredo EM, Ferreira GB, Maia Moreira RC, Kirkwood R, Fetters L. Efficacy of Ankle-Foot Orthoses on Gait of Children with Cerebral Palsy: Systematic Review of Literature. Pediat Phys Ther 2008; 20(3):207-23.
  5. Brehm MA, Harlaar J, Schwartz M. Effect of ankle-foot orthoses on walking efficiency and gait in children with cerebral palsy. J Rehabil Med 2008;40(7):529-34.
  6. Abel MF, Juhl GA,Vaughan CL, Damiano DL. Gait assessment of fixed ankle-foot orthoses in children with spastic diplegia. Arch Phys Med Rehabil 1998;79(2):126-33.
  7. Balaban B, Yasar E, Dal U, Yazicioglu K, Mohur H, Kalyon TA. The effect of hinged ankle-foot orthosis on gait and energy expenditure in spastic hemiplegic cerebral palsy. Disabil Rehabil 2007;29(2):139-44.
  8. Wilson H, Haideri N, Song K, Telford D. Ankle-foot orthoses for preambulatory children with spastic diplegia. J Pediat Orthoped 1997;17(3):370-6.
  9. Romkes J, Hell AK, Brunner R. Changes in muscle activity in children with hemiplegic cerebral palsy while walking with and without ankle-foot orthoses. Gait Posture 2006;24(4):467-74.
  10. Radtka SA, Skinner SR, Dixon DM, Johanson ME. A comparison of gait with solid, dynamic, and no ankle-foot orthoses in children with spastic cerebral palsy. Phys Ther 1997;77(4):395-409.
  11. Radtka SA, Skinner SR, Johanson ME. A comparison of gait with solid and hinged ankle-foot orthoses in children with spastic diplegic cerebral palsy. Gait Posture 2005;21:303-10.
  12. Hayek S, Hemo Y, Chamis S, Bat R, Segev E, Wientroub S, et al. The effect of community prescribed ankle–foot orthoses on gait parameters in children with spastic cerebral palsy. J Children’s Orthoped 2007;1(6):325-32.
  13. Burtenr PA, Woollactt MM, Qualls C. Stance balance control with orthoses in a group of children with spastic cerebral palsy. Dev Med Child Neurol 1999;41(11):748-57.
  14. Bjornson KF, Schmale GA, Adamczyk-Foster A, McLaughlin J. The effect of dynamic ankle foot orthoses on function in children with cerebral palsy. J Pediat Orthoped 2006;26(6):773-6.
  15. Hassani S, Ferdjallah M, Reiners K, Johnson C, Smith P, Harris G. Motor performance comparison of the hinged and dynamic ankle-foot orthotics. Dev Med Child Neurol 2002;44(91):4.
  16. Smith PA, Hassani S, Graf A, Flanagan A, Reiners K. Brace evaluation in children with diplegic cerebral palsy with a jump gait pattern. J Bone Joint Surg Am 2009;91(2):356-65.
  17. Zhang J, Wang Y, Yang Z. Comparison of dynamic and solid ankle-foot orthosis configurations for cerebral palsy children with spastic diplegia. Chine J Rehabil Med 2009;24(1):45-8.
  18. Case Smith J. Occupational therapy for children. 5th ed. Michigan: Elsevier Mosby; 2005.
  19. Palisano R, Rosenbaum P, Walter S, Russell D, Wood E, Galuppi B. Development and reliability of a system to classify gross motor function in children with cerebral palsy. Dev Med Child Neurol 1997; 39(4):214–23.
  20. Wood E, Rosenbaum P. The gross motor function classification system for cerebral palsy: a study of reliability and stability over time. Dev Med Child Neurol 2000;42(5):292-6.
  21. Dehghan L, Dalvand H, Abdolvahab M, Bagheri H, Faghih zade S. Inter rater reliability of Persian version Gross Motor Function Classification System Expanded & Revised in patients with cerebral palsy. Bimonthly Official Publication Medical Daneshvar 2011;18(91):2-8.
  22. Russell DJ, Rosenbaum PL, Avery LM, Lane M. Gross Motor Function Measure (GMFM’66&GMFM’88) user’s manual. 1st ed: London; Mac Keith Press; 2002.
  23. Russell DJ, Goster JW. Assessing functional differences in gross motor skills in children with cerebral palsy who use an ambulatory aid or orthoses: can the GMFM-88 help? Dev Med Child Neurol  2005;47(7):462-7.
  24. Drouin LM, Malouin F, Richards CL, Marcoux S. Correlation between the gross motor function measure scores and gait spatiotemporal measures in children with neurological impairments. Dev Med Child Neurol 1996;38(11):1007-19.
  25. Buckon CE, Thomas SS, Jakobson-Huston S, Sussman M, Aiona M. Comparison of three ankle-foot orthosis configurations for children with spastic hemiplegia. Dev Med Child Neurol 2001;43(6):371-8.
  26. Middleton EA, Hurley GRB, McIlwain JS. The role of rigid and hinged polypropylene ankle-foot-orthoses in the management of cerebral palsy: A case study. Prosthetics and Orthotics International 1988;12(3):129-35.
  27. Rethlefsen S, Kay R, Dennis S, Forstein M, Tolo V. The effects of fixed and articulated ankle-foot orthoses on gait patterns in subjects with cerebral palsy. J Pediat Orthoped 1999;19(4):470-4.
  28. Neumann DA. Kinesiology of the musculoskeletal system foundations for physical rehabilitation. 1st ed. Michigan: Mosby; 2002.
  29. Buckon CE, Thomas SS, Jakobson-Huston S, Moor M, Sussman M, Aiona M. Comparison of three ankle-foot orthosis configurations for children with spastic diplegia. Dev Med Child Neurol 2004;46(9):590-8.
  30. Rodda JM, Graham HK, Carson L, Galea MP, Wolfe R. Sagittal gait patterns in spastic diplegia. J Bone Joint Surg Br 2004;86(2):251-8.

Zinc Status in Febrile Seizure: A Case-Control Study

MohammadReza SALEHIOMRAN, Masumeh Masoumeh MAHZARI

Iranian Journal of Child Neurology, Vol. 7 No. 4 (2013), , Page 20-23

How to Cite This Article: Salehiomran MR, Mahzari M. Zinc Status in Febrile Seizure: A Case-Control Study. Iran J Child Neurol. 2013 Autumn; 7(4):20-23.

Febrile seizure is the most common type of seizure in children. Their incidence is 2-5%. There are different hypotheses about relationship between neurotransmitters and trace elements (such as zinc) and febrile seizure. Zinc, as
a major element of some enzymes, plays an important role in the central nervous system (CNS) and can affect some inhibitory mechanisms of CNS. The aim of the present study was to determine whether there were any changes in serum
zinc level in children with febrile seizure in comparison with febrile children without seizure.

Materials & Methods
This case-control study was performed on 100 patients aged 6 months to 6 years.
This study was conducted between January and August 2012, on 50 children with febrile seizures (case) and 50 febrile children without seizures (control), that were referred to Amirkola Children Hospital (a referral hospital in the north
of Iran). Two groups were matched for age and sex. The serum zinc levels in the both groups were determined by atomic absorption spectrophotometry method.

The mean serum zinc level was 0.585±0.166 mg/L and 0.704±0.179 mg/L in the case group and the control group, respectively (p=0.001). The mean serum zinc level was significantly lower in the febrile seizure group compared to the
control groups. 


Our findings revealed that serum zinc level was significantly lower in children with simple febrile seizure in comparison with febrile children without seizure. 

It can emphasize the hypothesis that there is a relation between serum zinc level and febrile seizure in children.



  1. Varma RR. Febrile seizures. Indian J Pediatr 2002; 69(8); 697-700.
  2. Talebian A, Vakili Z, Talar SA, Kazemi M, Mousavi GA. Assessment of the relation between serum zinc and magnesium levels in children with febrile convulsion. Iranian j pathol 2009;4(4):157-60.
  3. Mollah MA, DEy PR, Tarafdar SA, Akhter S, Ahmed S, Hassan T, et al. Zinc in CSF of patients with febrile convulsion. Indian J Pediatr 2002;69(10):859-61.
  4. Auvichayapat P, Auvichayapat N, Jedsrisuparp A, Thinkhamrop B, Sriroj S, Piyakulmala T, et al. Incidence of febrile seizures in thalassemic patients. J Med Assoc Thai 2004;87(8):970-3.
  5. Nelson KB, Ellenberg JH. Prenatal and perinatal antecedents of febrile seizures. Ann Neurol 1990;27(2):127-31.
  6. Udani V.Pediatric epilepsy - An Indian perspective. Indian J Pediatr 2005; 72(4):309-13.
  7. Ehsanipour F, Talebi-Taher M, Harandi N, Kani k. Serum zinc level in children with febrile convulsion and its comparison with that of control group. Iranian J Pediatr 2009;199:65-8.
  8. Heydarian F, Ashrafzadeh F, Kam S. Simple febrile seizure: The role of serum sodium levels in prediction of seizure recurrence during the first 24 hours. Iran J Child Neurol 2009;3(2):31-4.
  9. Jun-Hwa Lee, Jeong Hyun Kim. Comparison of Serum Zinc Levels Measured by Inductively Coupled Plasma Mass Spectrometry in Preschool Children with Febrile and Afebrile Seizures. Ann Lab Med 2012;32(3):190-3.
  10. Heydarian F, Ashrafzadeh F, Ghasemian A. Serum zinc level in patient with simple febrile seizure. Iran J Child Neurol 2010;4(2):41-3.
  11. Garty B, Olomucki R, Lerman ST, Nitzan M. Cerebrospinal fluid zinc concentration in febrile convulsion. Arch Dis Child 1995;73(4):338-41.
  12. Daoud AS, Batieha A, Abu-Ekteish F, Gharaibeh N, Ajlouni S, HijaziS. Iron status: a possible risk factor for the first febrile seizure. Epilepsia 2002;43(7):740-3.
  13. Ganesh R, Janakiraman L, Meenakshi B. Serum zinc levels are low in children with simple febrile seizures compared with those in children with epileptic seizures and controls. Ann Trop Paediatr 2011;31(4):345-9.
  14. Cho WJ, Son BH, Kim SW. Levels of Sodium and Zinc concentration in febrile convulsion. Korean Child Neural Soc1999;7(2):214-9.
  15. Burtis carl A, Ashwood, Edwhard R; Brun, David E. Tietz fundamentals of clinical chemistry. 6th ed. Philadelphia: Saunders Elsevier; 2008. P. 505-7.
  16. Smart TG, Hosie AM, Miller PS. Zn2+ ions: modulators of excitatory and inhibitory synaptic activity. Neuroscientist 2004;10(5):432-4.
  17. Mollah MA, Rakshit SC, Anwer KS, Arslan MI, saha N, Ahmeds, et al. Zinc concentration in serum and cerebrospinal fluid simultaneously decrease in children with febrile seizure: Finding from a Prospective study in Bangladesh. Acta pediatr 2008;97(12):1707-11.




Neuroimaging Findings in First Unprovoked Seizures: A Multicentric Study in Tehran


Iranian Journal of Child Neurology, Vol. 7 No. 4 (2013), , Page 24-31

How to Cite This Article: Molla Mohammadi M, Tonekaboni SH, Khatami AR, Azargashb E, Tavasoli A, Javadzadeh M, Zamani GR. Neuroimaging Findings in First Unprovoked Seizures: A Multicentric Study in Tehran. Iran J Child Neurol. 2013 Autumn; 7(4):24-31.


Seizure is an emergency in pediatrics. It really matters to the parents of the involved child to have information about the causes, management and prognosis.

First unprovoked seizures (FUS) are seizures that occur in patients without fever, trauma or infection. Due to the rapid improvement in diagnostic techniques in the last few decades, the etiology will be revealed and this term will no longer exist. This Study was designed to evaluate brain imaging findings in FUS patients.


Materials & Methods

Ninety-six children with FUS, who were admitted in three major children’s hospitals in Tehran, underwent brain imaging and were enrolled into the study.

The decision about the type of imaging (CT or MRI) was based on the patient’s medical and financial conditions. An expert radiologist in the field of pediatric neuroimaging interpreted the images.



Altogether, 27.1% had abnormal findings of which 29.2% were in the brain MRI group and 14.3% were in the brain CT scan group.

Abnormal results were gliosis (10.4%), hemorrhage (4.2%), dysgenesis (2.1%), dysmyelination (7.3%), encephalomalacy (1%), atrophy (5.2%) and infarction (2.1%). In some patients, the lesions were in 2 or 3 sites and some had more than one type of lesion.

There was no association between the duration, age and type of seizure and imaging abnormlities. However, we found an association between the location of the lesion and the type of seizure.



We recommend brain imaging in all patients with FUS and apart from some exceptions, brain MRI is superior to CT.


  1. Johnston MV. Siezure in childhood. In: Kliegman RM, Behrman RE, editors. Nelson text book of pediatrics. 18th ed. Philadelphia: Saunders; 2010. p. 2457-70.
  2. Bluestein JS, Moshe SL. First unprovoked seizure. In: Maria BL, editor. Currents in management in child neurology. 3rd ed. Hamilton: BC Decker; 2005. p. 89-92.
  3. Khodapanahandeh F, Hadizadeh H. Neuroimaging in children with first afebrile seizures: to order or not to order? Arch Iran Med 2006 Apr;9(2):156-8.
  4. Alawaneh H, Bataineh HA. Urgent neuroimaging in children with first nonfebrile seizures. Middle East JFam Med 2008 Feb;6(1):24-6.
  5. Shinnar S, O’Dell C, Mitnick R, Berg AT, Moshe SL. Neuroimaging abnormalities in children with an apparent first unprovoked seizure. Epilepsy Res 2001 Mar;43(3):261-9.
  6. Kalnin AJ, Fastenau PS, deGrauw TJ, Musick BS, Perkins SM, Johnson CS, et al. Magnetic resonance imaging findings in children with a first recognized seizure. Pediatr Neurol 2008 Dec;39(6):404-14.
  7. King MA, Newton MR, Jackson GD, Fitt GJ, Mitchell LA, Silvapulle MJ et al. Epileptology of the first-seizure presentation: a clinical, electroencephalographic, and magnetic resonance imaging study of 300 consecutive patients. Lancet 1998 Sep 26;352(9133):1007-11.
  8. Pohlmann-Eden B, Beghi E, CarnfieldC, Carnfield P. The first seizure and its management in adults and children. BMJ 2006 Feb;332(11):339-34.
  9. Raman S, Susan K, Joyce W. Paroxysmal disorders.In: Menkes J, editor. Child neurology. 7th ed. Philadelphia: Lipincott; 2006. p. 857-942.
  10. Wical B. The first unprovoked seizure.Gillette Children’sSpecialty Healthcare. A PediatricPerspective 1999 Mar;8(3).
  11. Bano S, Yadav SN.Neuroimaging in epilepsy.Medi-Focus2010 Apr-Sep;9(3&4):2-4.
  12. Rauch DA,Carr E, Harrington J.Inpatient brain MRI for new-onset seizures: utility and cost effectiveness.Clin Pediatr (Phila) 2008 Jun;47(5):457-60.
  13. Gaillard WD, Chiron C, Cross JH, Harvey AS, Kuzniecky R, Hertz-Pannier L et al. Guidelines for imaging infants and children with recent-onset epilepsy. Epilepsia 2009 Sep;50(9):2147-53.
  14. Barkovich AJ. Techniques and methods in pediatric neuroimaging. 4thed. Philadelphia: Lippincott Williams &Wilkins; 2005. p. 4-7.
  15. Doescher JS, deGrauw TJ, Musick BS, Dunn DW, Kalnin AJ, Egelhoff JC et al. Magnetic resonance imaging and electroencephalic findings in a cohort of normal children with newly dignosed seizures. J Child Neurol 2006 Jun; 21(6):490-5.



Accidental Children Poisoning With Methadone: An Iranian Pediatric Sectional Study

Sayena JABBEHDARI, Fariba FARNAGHI, Fakhreddin SHARIATMADARI, Narjes JAFARI, Fatemeh-Fereshteh MEHREGAN, Parvaneh KARIMZADEH

Iranian Journal of Child Neurology, Vol. 7 No. 4 (2013), , Page 32-34

How to Cite This Article: Jabbehdari S, Farnaghi F, Shariatmadari SF, Jafari J, Mehregan FF, Karimzadeh P. Accidental Children Poisoning With Methadone: An Iranian Pediatric Sectional Study. Iran J Child Neurol. 2013 Autumn;7(7): 32-34.


Toxic poisoning with methadone is common in children in Iran. Our study was carried out due to the changing pattern of methadone poisoning in recent years and increasing methadone toxicity.


Materials & Methods

In this descriptive-sectional study, all of the methadone poisoned children younger than 12 years who were admitted to the Loghman Hakim Hospital in 2012, were assessed. Clinical symptoms and signs, para-clinical findings, and treatment were evaluated.



In this study, 16 boys and 15 girls who had been poisoned by methadone were enrolled. The mean age of patients was 55 months. All patients had been poisoned randomly or due to parent’s mistakes. The mean time of symptoms onset after methadone consumption was 1 hour and 30 Min, indicating a relatively long time after onset of symptoms.

Clinical findings were drowsiness (75%), miotic pupil (68 %), vomiting (61%), rapid shallow breathing (57%) and apnea (40%). In paraclinical tests, respiratory acidosis (69%) and leukocytosis (55.2%) were seen. The most important finding was increase in distance of QT in ECG (23.8%). The mean time of treatment with naloxone infusion was 51 hours. Three percent of patients had a return of symptoms after discontinuation of methadone. In patients with apnea, a longer course of treatment was required, and this difference was significant. Also, 17% of patients with apnea had aspiration pneumonia, which was statistically significant.



We suggest long time treatment with naloxone and considering the probability of return of symptoms after discontinuation of methadone.


  1. Goldfrank L, Flomenbaum N, Lewin N. Goldfrank’s Toxicologic Emergencies. 7th ed. McGraw–Hill 2002; p. 590-607.
  2. Schelble DT. Phosgene and phosphine. In: Haddad LM, Shannon MW, Winchester J, eds. Clinical Management of Poisoning and Drug Overdose. 3rd ed. Philadelphia: WB Saunders; 2007. p. 640-7.
  3. Jennifer C, Gibson A. Accidental methadone poisoning in children: A call for Canadian research action. Child Abuse Negl;2010;34(8):553-4.
  4. Binchy JM, Molyneux E, Manning J. Accidental ingestion of methadone by children in Merseyside. BMJ 1994;308(6940:1335-6.
  5. Zamani N, Sanaei-Zadeh H, Mostafazadeh B. Hallmarks of opium poisoning in infants and toddlers. Trop Doct 2010;40(4):220-2.
  6. LoVecchio F, Pizon A, Riley B, Sami A, D’Incognito C. Onset of symptoms after methadone overdose. Am J Emerg Med 2007;25(1):57-9.
  7. Thanavaro KL, Thanavaro JL. Methadone-induced torsades de pointes: a twist of fate. Heart Lung 2011;40(5):448-53.
  8. Gaalen FA, Compier EA, Fogteloo AJ. Sudden hearing loss after a methadone overdose. Eur Arch Otorhinolaryngol 2009;266(5):773-4.
  9. Lynch RE, Hack RA. Methadone-induced rigid-chest syndrome after substantial overdose.Pediatrics. 2010; 126(1):232-4.
  10. Sidlo J, Valuch J, Ocko P, Bauerová J. Fatal methadone intoxication in a 11-month-old male infant. Soud Lek 2009;54(2):23-5.


How to Cite This Article: Heydarian F, Rezaeian A. Relationship between Changes in Serum Sodium Level and Seizures Occurrence in Children with Hypernatremic Dehydration. Iran J Child Neurol. 2013 Autumn;7(4): 35- 40.



To assess any relationship between serum sodium changes and seizure occurrence in children aged 2 months to 5 years with hypernatremic dehydration.


Materials & Methods

This cross-sectional study was performed on 63 patients aged 2 months to 5 years from 20 March 2006 to 15 March 2012 at Ghaem Hospital and Dr. Sheikh Hospital in Mashhad, Iran. Patients were divided into 2 groups: case group with hypernatremic dehydration and seizure occurrence, and control group with hypernatremic dehydration and no seizures.



The mean age of patients was 10.38 (2-48) months. Thirteen patients had seizures, 11 out of them, before admission and 2 during hospital staying. Serum sodium level at admission in those 2 patients with seizure occurrence after hospitalization was 169 (158-180) mmol/L, and in 50 patients without seizure was 162.8 (148-207) mmol/l. Also, the rate of decrease of serum sodium levels in these 2 cases within the first 12 hours after admission was 1.12, and in those without seizure was 0.54 (mmol/L/hour), and it was 0.47 and 0.53 (mmol/l/hour) after 24 hours of admission, respectively. Severe dehydration was seen in 38.5% of cases and 14% of controls.



There was not any relationship between changes in serum sodium level and seizure occurrence in children with hypernatremic dehydration.



  1. Okumura A, Ishiguro Y, Sofue A, Suzuki Y, Maruyama K, Kubota T, et al. Treatment and outcome in patients with febrile convulsion associated with epileptiform discharges on electroencephalography. Brain Dev 2004;26(4):241-4.
  2. Heydarian F, Vatankhah H. The role of anemia n first simple febrile seizure in children aged 6 months to 5 years old. Neurosciences (Riyadh) 2012;17(3):226-9.
  3. Heydrian F, Ashrafzadeh F, Ghasemian A. Serum zinc level in patients with simle febrile seizure. Iran J Child Neurol 2010;4(2):41-4.
  4. Dad MI, Ismael GA, Al-Oufi AA, Al-Mohammadi AH. Clinical pattern of seizures in hospitalized children. Neurosciences (Riyadh) 2003;8(2):107-9.
  5. Heydarian F, Ashrafzadeh F, Kam S. Simple febrile seizure: The role of serum sodium levels in prediction of seizure occurrence during the first 24 hours, Iran J Child Neurol 2009;3;31-4.
  6. Robertson G, Carrihill M, Hatherill M, Waggir Z,Reynolds L, Argent A. Relationship between fluid management, changes in serum sodium and outcome in hypernatremia associated with gastroenteritis, J Paediatr Child Health 2007;43(4):291-6.
  7. De Petris L, Luchetti A, Emma F. Cell volume regulationand transport mechanisms across the brain blood barrier: Implications for the management of hypernatremic states. Eur J pediatr 2000;160(2):71-7.
  8. Ross O. The management of extreme hypernatremia secondary to salt poisoning in an infant. Pediatr Anesth 2000;10:110-5.
  9. Hatherill M. Rubbing salt in the wound. Arch Dis Child 2004;89(5):414-8.
  10. Moritz ML, Ayus JC. Prevention of hospital acquired hyponatremia: A case for using isotonic saline. Pediatrics 2003;111(2):227-30.
  11. Taylor D, Durward A. Pouring salt on troubled waters. Arch Dis Child 2004;89(5):411-4.
  12. Fang C, Mao J, Dai Y, Xia Y, Fu H, Chen Y, et al. Fluid management of hypernatremic dehydration to prevent cerebral edema: A retrospective case control study of 97 children in China. J Paediatr Child Health 2010;46(6):301-3.
  13. El-Bayoumi MA, Abdelkader AM, El-Assmy MMA, Alwakeel AA, El-Tahan HM. Normal saline is a safe initial rehydration fluid in children with diarrhea-related hypernatremia. Eur J Pediatr 2012;171(2):383-8.
  14. Arampatzis S, Frauchiger B, FiedlerGM, Leichtle AB, Buhl D, Schwarz C, et al. Characteristics, Symptoms, and Outcome of severe Dysnatremias Present on Hospital Admission. Am J Med 2012;125(11):1125.e1-1125.e7.
  15. Chisti MJ, Pietroni MA, Smith JH, Bardhan PK, Salam MA, Predictors of death in under-five children with diarrhea admitted to a critical care ward in an urban hospital in Bangladesh. Am J Med 2012;125(11):1125. e1-1125.e7.
  16. Moritz ML, Ayus JC Preventing neurological complications from dysnatremias in children. Pediatr Nephrol 2005;20(12):1687-700.


Neuroimaging in Cerebral Palsy – Report from North India


Iranian Journal of Child Neurology, Vol. 7 No. 4 (2013), , Page 41-46

How to Cite This Article: Aggarwal A, Mittal H, Debnath SKR, Rai A. Neuroimaging in Cerebral Palsy–Report from North India. Iran J Child Neurol. 2013 Autumn; 7(3):41- 46.



Only few Indian reports exist on neuroimaging abnormalities in children with cerebral palsy (CP) from India.


Materials & Methods

We studied the clinico-radiological profile of 98 children diagnosed as CP at a tertiary centre in North India. Relevant investigations were carried out to determine the etiology.



Among the 98 children studied, 80.5% were males and 22.2% were premature. History of birth asphyxia was present in 41.9%. Quadriplegic CP was seen in 77.5%, hemiplegic in 11.5%, and diplegic in 10.5%. Other abnormalities were microcephaly (60.5%), epilepsy (42%), visual abnormality (37%), and hearing abnormality (20%). Neuroimaging was abnormal in 94/98 (95.91%).

Abnormalities were periventricular white matter abnormalities (34%), deep grey matter abnormalities (47.8%), malformations (11.7%), and miscellaneous lesions (6.4%). Neuroimaging findings did not relate to the presence of birth asphyxia, sex, epilepsy, gestation, type of CP, or microcephaly.



Neuroimaging is helpful for etiological diagnosis, especially malformations.




  1. Singhi PD, Ray M, Suri G. Clinical spectrum of cerebral palsy in north India-an analysis of 1000 cases. J Trop Pediatr 2002 48(3); 162-6.
  2. Sharma P, Sharma U, Kabra A. Cerebral Palsy-Clinical Profile and Predisposing Factors. Indian Pediatr 1999;36(10):1038-42.
  3. Nelson KB, Ellenberg JH. Antecedents of cerebral palsy. Multivariate analysis of risk. N Engl J Med 1986 315(2):81-6.
  4. Krägeloh-Mann I, Horber V. The role of magnetic resonance imaging in elucidating the pathogenesis of cerebral palsy: a systematic review. Dev Med Child Neurol 2007; 49(2):144-51.
  5. Rosenbaum P, Paneth N, Leviton A, Goldstein M, Bax M, Damiano D, et al. A report: the definition and classification of cerebral palsy April 2006. Dev Med Child Neurol Suppl 2007;109:8-14.
  7. Rikomen R, Raumanvrita S, Sinivuori E, Seppala T. Changing pattern of cerebral palsy in southwest region of Finland. Acta Pediatr Scand 1989; 78(4):581-7.
  8. Pharaoh POD, Plat MJ, Cooke T. The changing epidemiology of cerebral palsy. Arch Dis Child 1996;75(3): F169-73.
  9. Eischer PS, Batshaw M. Cerebral Palsy. Ped Clin North Am 1993;40(3):537-51.
  10. Bax M, Tydeman BA, Flodmark O. Clininical and MRI correlates of cerebral palsy: the European Cerebral PalsyStudy. JAMA 2006; 296(13):1602-08.
  11. Korzeniewski SJ, Birbeck G, DeLano MC, Potchen MJ, Paneth N. A systematic review of neuroimaging for cerebral palsy. Journal of Child Neurology 2008;23(2):216-27.
  12. Robinson MN, Peake LJ, Ditchfield MR, Reid SM. Magnetic Resonance imaging findings in population based cohort of children with cerebral palsy Dev Med Child Neurol 2009; 51(1):39-45.
  13. Shevell M, Ashwal S, Donley D, Flint J, Gingold M, Hirtz D, et al. Practice parameter: Evaluation of the child with global developmental delay: Report of the Quality Standards Subcommittee of the American Academy of Neurology and The Practice Committee of the Child Neurology Society. Neurology 2003; 60(3); 367-80.
  14. Ashwal S, Russman BS, Blasco PA, Miller G, Sandler A, Shevell M, et al. Practice parameter: diagnostic assessment of the child with cerebralpalsy: report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology 2004; 23;62(6): 851-63.


Neurometabolic Disorder Articles

Biotinidase Deficiency: A Reversible Neurometabolic Disorder (An Iranian Pediatric Case Series)

Parvaneh KARIMZADEH, Farzad AHMADABADI, Narjes JAFARI, Sayena JABBEHDARI, Mohammad Reza ALAEE, Mohammad GHOFRANI, Mohammad-Mahdi TAGHDIRI, Seyed Hassan TONEKABONI

Iranian Journal of Child Neurology, Vol. 7 No. 4 (2013), , Page 47-52


How to Cite This Article: Karimzadeh P, Ahmadabadi F, Jafari N, Jabbehdari S, Alaee MR, Ghofrani M, Taghdiri MM, Tonekaboni SH. Biotinidase Deficiency: A Reversible Neurometabolic Disorder (An Iranian Pediatric Case Series). Iran J Child Neurol. 2013 Autumn; 7(4):47- 52.



Biotinidase deficiency is one of the rare congenital metabolic disorders with autosomal recessive inheritance. If this disorder is diagnosed in newborn period, could be prevented well from mental and physical developmentaldelay and most of clinical manifestations.

Materials & Methods

The patients were diagnosed as biotinidase deficiency in Neurology Department of Mofid Children’s Hospital in Tehran, Iran, between 2009 and 2012 were included in this study. This study was conducted to define the age, gender, past medical history, developmental status, general appearance, clinical manifestations, neuroimaging findings, and response to treatment in 16 patients with biotinidase deficiency in this department.


In clinical presentation, cutaneous lesions were not found in 37% of the patients and 43% patients had not alopecia. 75% patients had abnormal neuroimaging that in 56% of them, generalized brain atrophy and myelination delay were found. Results of the present study showed the efficacy of biotin in early diagnosed patients with seizure and dermatological manifestations. The seizure and skin manifestations were improved after biotin therapy.


According to the results of this study, we suggest that early assessment and diagnosis have an important role in the prevention of disease progression and clinical signs.



  1. Wolf B.Disorders of biotin metabolism. In: Scriver CR,Beaudet AL, Sly W, et al.,eds. The Metabolic and MolecularBases of Inherited Disease, 8thed. New York,NY:McGraw-Hill;2001: 3935-3962.
  2. Rathi N, RathiM.Biotinidase deficiency with hypertonia as unusual feature.IndianPediatr. 2009;46(1):65-67.
  3. Wolf B.Worldwide survey of neonatal screening for biotinidasedeficiency.J Inherit Metab Dis. 1991;14(6):923-7.
  4. Dahiphale R, Jain S, AgrawalM.Biotinidasedeficiency. IndianPediatr. 2008;45(9):777-779.
  5. Heard GS,SecorMcVoy JR,Wolf B.A screening method for biotinidase deficiency in newborns.Clin Chem. 1984;30(1):125–7.
  6. Desai S, Ganesan K, HegdeA.Biotinidase deficiency: a reversible metabolic encephalopathy. neuroimaging and MR spectroscopic findings in a series of four patients. PediatrRadiol. 2008;38(8):848-856. Epub 2008 Jun 11.
  7. Wolf B.The neurology of biotinidasedeficiency.Mol Genet Metab. 2011;104(1-2):27-34. Epub 2011 Jun 12.
  8. Wastell HJ, Bartlett K, Dale G, et al. Biotidinase deficiency: a survey of 10 cases. Arch Dis Child. 1998;63(10):1244-1249.
  9. Wolf B, Pomponio RJ, Norrgard KJ, et al. Delayedonset profound biotinidase deficiency. J Pediatr.1998; 132(2):362–365.
  10. Grunewald S, Champion MP, Leonard JV, et al. Biotinidase deficiency: a treatable leukoencephalopathy. Neuropediatrics. 2004; 35(4):211–216.
  11. Wolf B, Spencer R, Gleason T. Hearing loss is a common feature of symptomatic children with profound biotinidase deficiency. J Pediatr.2002; 140(2):242–246.


Case Report

Hypoparathyroidism as the first manifestation of Kearns-sayre syndrome. A case report


Iranian Journal of Child Neurology, Vol. 7 No. 4 (2013), , Page 53-57

Hypoparathyroidism as the First Manifestation of Kearns-Sayre Syndrome: A Case Report


How to Cite This Article: Ashrafzadeh F, Ghaemi N, Akhondian J, Beiraghi Toosi M, Elmi S. Hypoparathyroidism as the First Manifestation of Kearns-Sayre Syndrome: A Case Report. Iran J Child Neurol. 2013 Autumn;7(4):53-57.




Kearns-Sayre syndrome is a mitochondrial myopathy, which was first described by Tomas Kearn in 1958. Diagnostic symptoms include retinitis pigmentosa, chronic and progressive external ophthalmoplegia plus one or more of following factors: heart conduction system disorders, cerebellar ataxia, or cerebrospinal fluid (CSF) protein content above 100 mg/dL. The nature of this uncommon disease is yet to be clarified. In this paper, we report a case of Kearns-Sayre syndrome. According to the previous records, the first manifestation of Kearns-Sayre syndrome as hypoparathyroidism is uncommon and in this article, we report a case with this problem.


  1. Ashizawa T, Subramony SH. What is Kearns-Sayer syndrome after all? Arch Neurol 2001;58(7):1053-4.
  2. Barragan-Campos HM, Vallee JN, Lo D, Barrera-Ramirez CF, Argote-Greene M, Sanchez-Guerrero J, et al. Brain magnetic resonance imaging findings in patients with mitochondrial cytopathies. Arch Neurol 2005;62(5):737-42.
  3. Amemiya S, Hamamoto M, Goto Y, Komaki H, Nishino I, Nonaka I, et al. Psychosis and progressive dementia: presenting features of a mitochondriopathy. Neurology 2000;55(4):600-1.
  4. Katsanos KH, Pappas CJ, Patsouras D, Michalis LK, Kitsios G, Elisaf M, et al. Alarming atrioventricular block and mitral valve prolapse in the Kearns-Sayer syndrome. Int J Cardiol 2002;83(2):179-81.
  5. Tiranti V, Viscomi C, Hildebrandt T, Di Meo I, Mineri R, Tiveron C, et al. Loss of ETHE1, a mitochondrial dioxygenase, causes fatal sulfide toxicity in ethylmalonic encephalopathy. Nat Med 2009;15(2):200–5.
  6. Chinnery PF, DiMauro S, Shanske S, Schon EA, Zeviani M, Mariotti C, et al. Risk of developing a mitochondrial DNA deletion disorder. Lancet 2004;364(9434):592–6.
  7. Bosbach S, Kornblum C, Schröder R, Wagner M. Executive and visuospatial deficits in patients with chronic progressive external ophthalmoplegia and Kearns-Sayer syndrome. Brain 2003;126(Pt 5):1231-40.
  8. Berenberg RA, Pellock JM, DiMauro S, Schotland DL, Bonilla E, Eastwood A, et al. Lumping or splitting? “Ophthalmoplegia-plus” or Kearns-Sayer syndrome? Ann Neurol 1977;1(1):37-54.
  9. Welzing L, von Kleist-Retzow JC, Kribs A, Eifinger F, Huenseler C, Sreeram N. Rapid development of life threatening complete atrioventricular block in Kearns-Sayer syndrome. Eur J Pediatr 2009;168(6):757-9.
  10. Berio A, Piazzi A. Kearns-Sayer syndrome with GH deficiency. Pediatr Med Chir 2000;22:43-6.
  11. Schmiedel J, Jackson S, Schäfer J, Reichmann H. Mitochondrial cytopathies. J Neurol 2003;250(3):267-77.
  12. Chu BC, Terae S, Takahashi C, Kikuchi Y, Miyasaka K, Abe S, et al. MRI of the brain in the Kearns-Sayer syndrome: report of four cases and a review. Neuroradiology 1999;41(10):759-64.
  13. Altunbaşak S, Bingöl G, Ozbarlas N, Akçören Z, Hergüner O. Kearns-Sayer syndrome. A case report. Turk J Pediatr 1998;40(2):255-9.
  14. Chawla S, Coku J, Forbes T, Kannan S. Kearns-Sayer syndrome presenting as complete heart block. Pediatr Cardiol 2008;29(3):659-62.
  15. Gregoratos G, Abrams J, Epstein AE, Freedman RA, Hayes DL, Hlatky MA, et al. ACC/AHA/NASPE 2002 guideline update for implantation of cardiac pacemakers and antiarrhythmia devices: summary article: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (ACC/ AHA/NASPE Committee to Update the 1998 Pacemaker Guidelines). Circulation 2002;106(16):2145-61.
  16. Basu AP, Posner E, McFarland R, Turnbull DM. Kearnsayre syndrome. Medscape reference. Feb 4, 2010.

How to Cite This Article: Shervin Badv R, Niksirat A. Downward Vertical Gaze Palsy As A Prominent Manifestation Of Episodic Ataxia Type 2: A Case Report. Iran J Child Neurol. 2013 Autumn; 7(4):58- 60.



Episodic ataxia type 2 (EA2) is an inherited autosomal dominant disorder characterized by intermittent ataxia, nausea, vomiting, dysarthria, or nystagmus.

We report a case of EA2, which downward gaze palsy exists as a common sign in all her attacks. Responsiveness of EA2 to acetazolamide was observed in this patient.



  1. Ouvrier R, Aicardi J. Disorders of the peripheral nerves. In: Aicardi J, Bax M, Gillberg C, editors.Diseases of the nervous system in Childhood. 3rd ed. London: Mackeith Press; 2009.
  2. Swaiman KF, Ashwal S, Ferriero DM, Schor NF. Pediatric neurology: principles & practice. 5th ed. London: Elsevier Saunders; 2012.
  3. National Ataxia Foundation. Minneapolis: National Ataxia Foundation; 2007 (cited 2007 Feb). Available from: URL:
  4. Subramony SH, Schott K, Raike RS, Callahan J, Langford LR, Christova PS, et al. Novel CACNA1A mutation causes febrile episodic ataxia with interictal cerebellar deficits. Ann Neurol. 2003;54(6):725-31.
  5. Brunt ER, van Weerden TW. Familial paroxysmal kinesigenic ataxia and continuous myokymia. Brain 1990;113(5):1361-82.
  6. Jen J. Familial Episodic Ataxias and Related Ion Channel Disorders. Curr Treat Options Neurol 2000;2(5):429-31.
  7. Fenichel M. Clinical Pediatric Neurology: A Signs and Symptoms Approach. 6th ed. Philadelphia: Elsevier Saunders; 2009. P.227-247.
  8. Griggs RC, Moxley RT 3rd, Lafrance RA, McQuillen J. Hereditary paroxysmal ataxia: response to Acetazolamide. Neurology 1978;28(12):1259-64.
  9. Scoggan KA, Friedman JH, Bulman DE. CACNA1A mutation in a EA-2 patient responsive to acetazolamide and valproic acid. Can J Neurol Sci 2006;33(1):68-72.
  10. Kim JM, Kim JS, Ki CS, Jeon BS. Episodic Ataxia Type 2 due to a Deletion Mutation in the CACNA1A Gene in a Korean Family. J Clin Neurol 2006;2(4):268-71.
  11. Bain PG, O’Brien MD, Keevil SF, Porter DA. Familial periodic cerebellar ataxia: a problem of cerebellar intracellular pH homeostasis. Ann Neurol 1992;31(2):147-54.
  12. Gancher ST, Nutt JG. Autosomal dominant episodic ataxia: a heterogeneous syndrome. Mov Disord. 1986;1(4):239-53.
  13. Lubbers WJ, Brunt ER, Scheffer H, Litt M, Stulp R, Browne DL, et al. Hereditary myokymia and paroxysmal ataxia linked to chromosome 12 is responsive to acetazolamide. J Neurol Neurosurg Psychiatry 1995;59(4):400-5.
  14. Shapiro MS, Gomeza J, Hamilton SE, Hille B, Loose MD, Nathanson NM, et al. Identification of subtypes of muscarinic receptors that regulate Ca2+ and K+ channel activity in sympathetic neurons. Life Sci 2001;68(22-23):2481-7.
  15. Baloh RW. Episodic vertigo: Central nervous system causes. Curropin Neurol 2002;15(1):17-21
  16. VanDyke DH, Griggs RC, Murphy MJ, Goldstein MN. Hereditary myokymia and periodic ataxia. J Neurolog Sci 1975;25(1):109-18.
  17. Jen J, Kim GW, Baloh RW. Clinical spectrum of episodic ataxia type 2. Neurology 2004;62(1):17-22.
  18. Singhvi JP, Prabhakar S, Singh P. Episodic ataxia: a case report and review of literature. Neurol India 2000;48(1):78-80.