Review Article

Posterior Reversible Encephalopathy Syndrome in Pediatric Hematologic- Oncologic Disease: Literature Review and Case Presentation

Mohammad Thaghi ARZANIAN, Bibi Shahin SHAMSIAN*, Parvaneh KARIMZADEH, Mohammad KAJIYAZDI, Fatima MALEK, Mohammad HAMMOUD

Iranian Journal of Child Neurology, Vol. 8 No. 2 (2014), , Page 1-10

How to Cite This Article: Arzanian MT, Shamsian BSh, Karimzadeh P, Kajiyazdi M, Malek F, Hammoud M. Posterior Reversible Encephalopathy Syndrome in Pediatric Hematologic-Oncologic Disease: Literature Review and Case Presentation. Iran J Child Neurol. 2014 Spring 8(2):1-10.


Posterior reversible encephalopathy syndrome (PRES) is a cliniconeuroradiological disease entity, which is represented by characteristic magnetic resonance imaging (MRI) findings of subcortical/cortical hyperintensity in T2-weighted sequences. It is more often seen in parietaloccipital lobes, and is accompanied by clinical neurological changes. PRES is a rare central nervous system (CNS) complication in patients with childhood hematologic-oncologic disese and shows very different neurological symptoms between patients, ranging from numbness of extremities to generalized seizure.
In this article, we will review PRES presentation in hematologic-oncologic patients. Then, we will present our patient, a 7-year-old boy with Evans syndrome on treatment with cyclosporine, mycophenolate mofetil (MMF) and prednisone, with seizure episodes and MRI finding in favour of PRES.



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Research Article

Efficacy of Chloral Hydrate-Hydroxyzine and Chloral Hydrate-Midazolam in Pediatric Magnetic Resonance Imaging Sedation

Razieh FALLAH, Nafiseh FADAVI, Shekofah BEHDAD, Mohammad FALLAH TAFTI*

Iranian Journal of Child Neurology, Vol. 8 No. 2 (2014), , Page 11-17

How to Cite This Article: Fallah R, Fadavi N, Behdad Sh, Fallah Tafti M. Efficacy of Chloral Hydrate-Hydroxyzine and Chloral Hydrate-Midazolam in Pediatric Magnetic Resonance Imaging Sedation. Iran J Child Neurol. 2014 Spring 8(2):11-17.


Magnetic resonance imaging (MRI) is a useful diagnostic tool for the evaluation of congenital or acquired brain lesions. But, in all of less than 8-year-old children, pharmacological agents and procedural sedation should be used to induce
motionless conditions for imaging studies. The purpose of this study was to compare the efficacy and safety of combination of chloral hydrate-hydroxyzine (CH+H) and chloral hydrate-midazolam (CH+M) in pediatric MRI sedation.

Materials & Methods
In a parallel single-blinded randomized clinical trial, sixty 1-7-year-old children who underwent brain MRI, were randomly assigned to receive chloral hydrate in a minimum dosage of 40 mg/kg in combination with either 2 mg/kg of
hydroxyzine or 0.5 mg/kg of midazolam. The primary outcomes were efficacy of adequate sedation (Ramsay sedation score of five) and completion of MRI examination. The secondary outcome was clinical side-effects.

Twenty-eight girls (46.7%) and 32 boys (53.3%) with the mean age of 2.72±1.58 years were studied. Adequate sedation and completion of MRI were achieved in 76.7% of CH+H group. Mild and transient clinical side-effects, such as vomiting of one child in each group and agitation in 2 (6.6 %) children of CH+M group, were also seen. The adverse events were more frequent in CH+M group.

Combinations of chloral hydrate-hydroxyzine and chloral hydrate-midazolam were effective in pediatric MRI sedation; however, chloral hydrate-hydroxyzine was safer.



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Acquired CNS Demyelinating Syndrome in Children Referred to Shiraz Pediatric Neurology Ward

Soroor INALOO*, Saeedeh HAGHBIN, Mehrpoor MORADI, Hassan DASHTI, Nazila Nazila SAFARI

Iranian Journal of Child Neurology, Vol. 8 No. 2 (2014), , Page 18-23

How to Cite This Article: Inaloo S, Haghbin S, Moradi M, Dashti H, Safari N. Acquired CNS Demyelinating Syndrome in Children Referred to Shiraz Pediatric Neurology Ward. Iran J Child Neurol. 2014 Spring; 8(2):18-23.

Incidence of CNS acquired demyelinating syndrome (ADS), especially multiple sclerosis (MS) in children, appears to be on the rise worldwide. The objective of this study was to determine prevalence, clinical presentation, neuroimaging
features, and prognosis of different types of ADS in Iranian children.

Materials & Methods
During the period 2002-2012, all the patients (aged 1-18 years) with ADS, such as MS, acute disseminated encephalomyelitis (ADEM), optic neurotic (ON), Devic disease, and transverse myelitis (TM), referred to the pediatric neurology ward, Nemazee Hospital, Shiraz University of Medical Sciences, were included
in this study. Demographic data, clinical signs and symptoms, past and family history, preclinical findings, clinical course, and outcome were obtained.

We identified 88 patients with ADS in our center. The most prevalent disease was MS with 36.5% (n=32), followed by AEDM 26.1% (n=31), ON 17% (n=13), TM 15.9% (n=14), and Devic disease 4.5% (n=4). MS, ON, TM were more
common among females while ADEM was more common in males. Children with ADEM were significantly younger than those with other types of ADS.
Family history was positive in 10% of patients with MS.
Previous history of recent infection was considerably seen in cases with ADEM.
Clinical presentation and prognosis in this study was in accordance with those in previous studies on children.

In this study, the most common type of ADS was MS, which was more common in female and older age cases. ADEM was more common in male and younger children. ADEM and ON had the best and Devic disease had the worst prognosis.


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The Etiology, Clinical Type, and Short Outcome of Seizures in Newborns Hospitalized in Besat Hospital/Hamadan/ Iran

Mohammad Kazem SABZEHEI, Behnaz BASIRI, Hassan BAZMAMOUN*

Iranian Journal of Child Neurology, Vol. 8 No. 2 (2014), , Page 24-28

How to Cite This Article: Sabzehei MK, Basiri B, Bazmamoun H. The Etiology, Clinical Type, and Short Outcome of Seizures in Newborns Hospitalized in Besat Hospital/ Hamadan/Iran. Iran J Child Neurol. 2014 Spring 8(2):24-28.

Seizures in neonates are very different from those of older children and adults.
The aim of this study was to determine the etiology, clinical presentation, and outcome of seizures in hospitalized neonates of Besat Hospital, Hamadan, Iran.

Material & Methods
In this retrospective study, we evaluated all neonates with seizures (aged 0-28 days) admitted to the Besat hospital, Hamadan, over a period of three years from September 2008 to September 2011. The data were obtained from hospital records and analyzed using SPSS 12.

Seizures were reported in 102/1112 (9.1%) neonatal admissions. Among neonates with seizures, 57% were male and 23.5% were preterm. The mean birth weight was 2936±677 grams and the mean gestational age was 37.60±1.94 weeks. 16.7% of them were LBW and 2.9% VLBW. In terms of seizure type, subtle seizures were observed in 38.2%, tonic in 29.4%, clonic in 26.4%, and
myoclonic in 5.9% of cases. The main diagnosis in neonates with seizures included hypoxic-ischemic encephalopathy (HIE) (34.3%), infections (24.4%), intracranial hemorrhage (6.9%), hypoglycemia (5.9%), hypocalcemia (2.9%), inborn error of metabolism (1%), and unknown cause (24.5%). The mortality rate was 14.7%.

Neonatal seizures indicate a significant underlying disease. HIE was the most common cause of neonatal seizures in our study. Therefore, efforts should be made to improve care during childbirth.


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Prevalence of Epileptiform Discharges in Children with Sensori-Neural Hearing Loss and Behavioral Problems Compared to Their Normal Hearing Peers


Iranian Journal of Child Neurology, Vol. 8 No. 2 (2014), , Page 29-33

How to Cite This Article: Amirsalari S, Radfar Sh, Ajallouyean M, Saburi A, Yousefi J, Noohi S, Tavallaie SA, Hassanalifard M, Ghazavi Y. Prevalence of Epileptiform Discharges in Children with Sensori-Neural Hearing Loss and Behavioral Problems Compared to Their Normal Hearing Peers. Iran J Child Neurol. 2014 Spring 8(2):29-33.

Overactivity and behavioral problems are common problems in children with prelingually profound sensorineural hearing loss (SNHL). Data on epileptiform electroencephalography (EEG) discharges in deaf children with psychological
disorders are so limited. The primary focus of this study was to determine the prevalence of epileptiform discharges (EDs) in children with SNHL and overactivity or behavioral problems.

Materials & Methods
A total of 262 patients with prelingually profound SNHL who were referred to our cochlear implantation center between 2008 and 2010 were enrolled in this study. Children with SNHL who had diagnosis of overactivity and/or behavioral
problems by a pediatric psychiatrist, underwent electroencephalography (EEG).
EEG analysis was carried out by a board-certified pediatric neurologist. The control group consisted of 45 cases with overactivity or behavioral problems and normal hearing.

One hundred thirty-eight children with mean age of 3.5±1.23 year were enrolled in the case group, of whom 88 cases (63.7%) were boy. The control group consisted of 45 cases with mean age of 3.2±1.53 years, of whom 30 (66.6%)
cases were male. EDs were detected in 28 (20.02%) children of the case group (with SNHL) in comparison with 4 (8.88%) in the control group (without SNHL), which was statistically significantly different.

In this study, we obtained higher frequency of EDs in deaf children with overactivity and/or behavioral problem compared to the children without SNHL. Further studies are required to evaluate the possible association of SNHL with
EDs in overactive children.


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20. Borusiak P, Zilbauer M, Jenke AC. Prevalence of epileptiform discharges in healthy children--new data from a prospective study using digital EEG. Epilepsia 2010;51(7):1185-8.

21. Daneshi A, Hassanzadeh S. Cochlear implantation in prelingually deaf persons with additional disability. J Laryngol Otol 2007;121(7):635-8.

22. Dye MW, Bavelier D. Attentional enhancements and deficits in deaf populations: an integrative review. Restor Neurol Neurosci 2010;28(2):181-92.


A Study of Methadone-Poisoned Children Referred to Hamadan’s Besat Hospital/Iran

Hassan BAZMAMOUN, Afshin FAYYAZi*, Ali KHAJEH, Mohammad Kazem SABZEHEI, Fuzieh KHEZRIAN

Iranian Journal of Child Neurology, Vol. 8 No. 2 (2014), , Page 34-37

How to Cite This Article: Bazmamoun H, Fayyazi A, Khajeh A, Sabzehei MK, Khezrian F. A Study of Methadone-Poisoned Children Referred to Hamadan’s Be’sat Hospital Iran. Iran J Child Neurol. 2014 Spring 8(2):34-37.

Increasing use of methadone in withdrawal programs has increased methadone poisoning in children. This research aimed to study the causes of incidence of poisoning in children and its side-effects.

Materials & Methods
In this research, The hospital records of all methadone-poisoned children referred to Hamadan’s Be’sat Hospital from June 2007 to March 2013, were studied. Children with a definite history of methadone use or proven existence
of methadone in their urine, were studied.

During 5 years, 62 children with the mean age of 53.24±29.50 months were hospitalized due to methadone use. There was a significant relationship between
delayed referral to hospital and increased bradypnea. According to their history, 25.8% and 58.1% of the children had been poisoned by methadone tablet and syrup, respectively. The most common initial complaint expressed by parents, was decreased consciousness (85.5%). During the initial examination, decreased consciousness, meiosis, and respiratory depression were observed in 91.9%,
82.3%, and 69.4% of the cases, respectively. Nine patients required mechanical ventilation. There was a significant relationship between the need for mechanical ventilation and seizure with initial symptom of emesis. There were two cases of death (3.2%), both of which were secondary to prolonged hypoxia and brain death. There was a significant relationship between poor patient prognosis (death) and presence of cyanosis in early symptoms, seizure, hypotension, duration of decreased consciousness, and duration of mechanical ventilation.

This research indicated that the occurrence of seizure, hypotension, and cyanosis in the early stages of poisoning is associated with an increased risk of sideeffects and death and are serious warning signs. Early diagnosis and intervention can improve outcomes of methadone-poisoned children.


1. Nazari H. Clinical approach to methadone intoxication. J Addict 2007;2:18-20.

2. Fayyazi A, Bagheri M, Khajeh A, Ahmadi S. Acute hydrocephaly following methadone intoxication in a child. Iran J Child Neurol. 2012;6(1):35-8.

3. Binchy JM, Molyneux EM, Manning J. Accidental ingestion of methadone by children in Merseyside. BMJ.1994; 308(6940):1335-6.

4. Li L, Levine B, Smialek JE. Fatal methadone poisoning in children: Maryland 1992–1996. Subst Use Misuse 2000;35(9):1141-8.

5. Riascos R, Kumfa P, Rojas R, Cuellar H, Descartes F. Fatal methadone intoxication in a child. Emerg Radiol 2008;15(1):67-70.

6. Milroy CM, Forrest AR. Methadone deaths: a toxicological analysis. J Clin Pathol 2000;53(4):277-81.

7. Farnaghi F, Jafari N, Mehregan FF. Methadone Poisoning among Children Referred to Loghman-Hakim Hospital in 2009. Pajoohandeh Journal 2012;16(6):299-303.

8. Zamani N, Sanaei-zadeh H, Mostafazadeh B. hallmarks of opium poisoning in infants and toddlers. Trop Doct 2010;40 (4):220-2.

9. Malloy S, Soh C, Williams TL Reversible delayed post hypoxic leukoencephalopathy. Am J Neuroradiol 2006; 27(8):1763–5.

10. Geibprasert S, Gallucci M and KringsT. Addictive Illegal Drugs: Structural Neuroimaging. Am J Neuroradiol 2010;31(5):803-8.


Iron-deficiency Anemia in Children with Febrile Seizure: A Case-Control Study

Fateme GHASEMI, Fateme VALIZADEH*, Nadere TAEE

Iranian Journal of Child Neurology, Vol. 8 No. 2 (2014), , Page 38-44

How to Cite This Article: Ghasemi F, Valizadeh F, Taee N. Iron-deficiency Anemia in Children with Febrile Seizure: A Case-Control Study. Iran J Child Neurol. 2014 Spring 8(2):38-44.

Considering the recurrence of febrile seizure and costs for families, many studies have attempted to identify its risk factors. Some recent studies have reported that anemia is more common in children with febrile convulsion, whereas others have reported that iron deficiency raises the seizure threshold. This study was done to compare iron-deficiency anemia in children with first FS with children having febrile illness alone and with healthy children.

Materials & Methods
This case-control study evaluated 300 children in three groups (first FS, febrile without convulsion, and healthy) in Khoramabad Madani Hospital from September 2009 to September 2010. Body temperature on admission was
measured using the tympanic method. CBC diff, MCV, MCH, MCHC, serum iron, plasma ferritin and TIBC tests were performed for all participants. Data were analyzed by frequency, mean, standard deviation, ANOVA, and chi-square statistical tests. Odds ratios were estimated by logistic regression at a confidence level of 95%.

Forty percent of the cases with FS had iron-deficiency anemia, compared to 26% of children with febrile illness without seizure and 12% of healthy children. The Odds ratio for iron-deficiency anemia in the patients with FS was 1.89 (95% CI, 1.04-5.17) compared to the febrile children without convulsion and 2.21 (95% CI, 1.54-3.46) compared to the healthy group. 

Children with FS are more likely to be iron-deficient than those with febrile illness alone and healthy children. Thus, iron-deficiency anemia could be a risk factor for FS.


1. Østergaard J R. Febrile Seizures. Acta Pædiatr 2009;98(5):771-3.

2. Jones T, Jacobsen SJ. Childhood Febrile Seizures: Overview and Implications. Int J Med Sci. 2007; 4(2):110-4.

3. Flury T, Aebi C, Donati F. Febrile Seizures and Parental Anxiety: Does Information Help? Swiss Med Wkly 2001;131:556–60.

4. Kliegman RM, Stanton BF, St Geme JW, Schor NF, Behrman RE. Nelson Textbook of Pediatrics. 19th ed. Philadelphia (PA): WB Saunders Company; 2011. p.2017.

5. Pisacane A, Sansone R, Impagliazzo N, Coppola A, Rolando P, D’Apuzzo A, et al. Iron Deficiency Anemia and Febrile Convulsions: Case-control Study in Children under 2 Years. BMJ 1996;313 (7053):343.

6. Lozoff B, Beard J, Connor J, Barbara F, Georgieff M, Schallert T, et al. Long-lasting Neural and Behavioral Effects of Iron Deficiency in Infancy. Nutr Rev 2006;64(5 Pt 2):34–43.

7. Parks YA, Wharton BA. Iron Deficiency and the Brain. Acta Paediatr Scand 1989;361:(Suppl 1):71–7.

8. Ur-Rehman N, Billoo AG. Association between Iron Deficiency Anemia and Febrile Seizures. J Coll Physicians Surg Pak 2005;15(6):338-40.

9. Daoud AS, Batieha A, Abu-Ekteish F, Gharaibeh N, Ajlouni S, Hijazi S. Iron Status: A Possible Risk Factor for the First Febrile Seizure. Epilepsia 2002;43(7):740-3.

10. Hartfield DS, Tan J, Yager JY, Rosychuk RJ, Spady D, Haines C, et al. The Association between Iron Deficiency and Febrile Seizures in Childhood. Clin Pediatr (Phila) 2009;48(4):420-6.

11. Momen A, Nikfar R, Karimi B. Evaluation of Iron Status in 9-month to 5-year-old Children with Febrile Seizures: A Case-control Study in the South West of Iran. Iran J

Child Neurol 2010;4(2):45-50.

12. Talebian A, Momtazmanesh N. Febrile Seizures and Anemia. Iran J Child Neurol 2007;31-3.

13. Kobrinsky NL, Yager JY, Cheang MS, Yatscoff RW, Tenenbein M. Does Iron Deficiency Raise the Seizure Threshold? J Child Neurol 1995;10(2):105–9.

14. Salehi Omran MR, Tamaddoni A, Nasehi MM, Babazadeh H, Alizadeh Navaei R. Iron Status in Febrile Seizure: A Case-control Study. Iran J Child Neurol 2009:3(3):40-3.

15. Amirsalari S, Keihanidost Z, Ahmadi M, Sabouri A, Kavemanesh Z, Afshar P, et al. Relationship between Iron Deficiency Anemia and Febrile Seizures. Iran J Child Neurol 2010;14(1):27-30.

16. Bidabadi E, Mashouf M. Association between Iron Deficiency Anemia and First Febrile Convulsion: A Case–control Study. Seizure 2009;18:347-51.

17. Oski FA, Brugnara C, Nathan DG. A Diagnostic Approach to the Anemic Patients. In: Nathan DG, Orkin SH, editors. Nathan and Oski’s Hematology of Infancy and Childhood. 7th ed. Philadelphia: WB Saunders Company; 2008. Appendix 11.

18. Vaswani RK, Dharaskar PG, Kulkarni S, Ghosh K. Iron Deficiency as a Risk Factor for First Febrile Seizure. Indian Pediatr 2009;47(5):437-9.

19. Sadeghzadeh M, Khoshnevis P, Mahboubi E. Iron Status and Febrile Seizure- A Case Control Study in Children Less Than 3 Years. Iran J Child Neurol 2012;6(4):27-31.

20. Idro R, Gwer S, Williams TN, Otieno T, Uyoga S, Fegan G, et al. Iron Deficiency and Acute Seizures: Results from Children Living in Rural Kenya and a Metaanalysis. PLoS One 2010;5(11):e14001.

21. 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.


Associations between Manual Abilities, Gross Motor Function, Epilepsy, and Mental Capacity in Children with Cerebral Palsy

Ewa GAJEWSKA*, Magdalena SOBIESKA, Włodzimierz SAMBORSKI

Iranian Journal of Child Neurology, Vol. 8 No. 2 (2014), , Page 45-52

How to Cite This Article: Gajewska E, Sobieska M, Samborski W. Associations between Manual Abilities, Gross Motor Function, Epilepsy, and Mental Capacity in Children with Cerebral Palsy. Iran J Child Neurol. 2014 Spring 8(2):45-52.

This study aimed to evaluate gross motor function and hand function in children with cerebral palsy to explore their association with epilepsy and mental capacity. 

Material & Methods
The research investigating the association between gross and fine motor function and the presence of epilepsy and/or mental impairment was conducted on a group of 83 children (45 girls, 38 boys). Among them, 41 were diagnosed
with quadriplegia, 14 hemiplegia, 18 diplegia, 7 mixed form, and 3 athetosis.
A neurologist assessed each child in terms of possible epilepsy and confirmed diagnosis in 35 children. A psychologist assessed the mental level (according to
Wechsler) and found 13 children within intellectual norm, 3 children with mild mental impairment, 18 with moderate, 27 with severe, and 22 with profound.
Children were then classified based on Gross Motor Function Classification System and Manual Ability Classification Scale.

The gross motor function and manual performance were analysed in relation to mental impairment and the presence of epilepsy. Epilepsy was found to disturb conscious motor functions, but also higher degree of mental impairment was
observed in children with epilepsy.

The occurrence of epilepsy in children with cerebral palsy is associated with worse manual function. The occurrence of epilepsy is associated with limitations in conscious motor functions. There is an association between epilepsy in children with cerebral palsy and the degree of mental impairment.
The occurrence of epilepsy, mainly in children with hemiplegia and diplegia is associated with worse mental capacities.


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  2. Sellier E, Uldall P, Calado E, Sigurdardottir S, Torrioli MG, Platt MJ, et al. Epilepsy and cerebral palsy: characteristics and trends in children born in 1976-1998. Eur J Paediatr Neurol 2012;16(1):48-55.
  3. Wallace SJ. Epilepsy in cerebral palsy. Dev Med Child Neurology 2001;43(10):713-7.
  4. Bax MCO. Cerebral palsy. In: Aicardi J, editor. Disease of the nervous system in childhood. 2nd ed. London: Mc Keith Press; 1998. p. 210-39.
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  6. 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 Neurology 2007;109:8-14.
  7. Palisano R, Rosenbaum P, Bartlett D, Livingston M. Content validity of the expanded and revised Gross Motor Function Classification System. Dev Med Child Neurology 2008;50(10):744-50.
  8. Öhrvall AM, Eliasson AC. Parents and therapists perceptions about the content and construct of Manual Ability Classification System, MACS. Scand J Occup Ther 2010;17(3):209-16.
  9. Marszał E.Występowanie, diagnostyka i leczenie padaczki u dzieci z mózgowym porażeniem dziecięcym. Neurologia dziecięca;2006:15, 30:65-8.
  10. Blair E, Watson L. Epidemiology of cerebral palsy. Semin Fetal Neonatal Medicine 2006;11(2):117-25.
  11. Meberg A, Broch H. Etiology of cerebral palsy. J Perinat Med 2004;32(5):434-9.
  12. Blair E. Epidemiology of the cerebral palsies. Orthop Clin North Am 2010;41(4):441-55.
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  14. Arnould C, Penta M, Renders A, Thonnard JL. Abilhand-Kids: a measure of manual ability in children with cerebral palsy. Neurology 2004;63(6):1045-52.
  15. Carnahan KD, Arner M, Hägglund G. Association between gross motor function (GMFCS) and manual ability (MACS) in children with cerebral palsy. A population-based study of 359 children. BMC Musculoskeletal Disorders 2007;21:50.
  16. Bax MC, Keith Brown J. The spectrum of disorders known as CP. In: Scrutton D, Damiano D, Mayston M, editors. Management of the Motor Disorders of Children with CP. Clinics in Developmental Medicine London: Mac Keith Press; 2004. p. 83-140.
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  20. McLellan A. Epilepsy – an additional risk factor for psychological problems in cerebral palsy. Dev Med Child Neurology 2008;50(10):727.
  21. Kulak W, Sobaniec W, Smigielska-Kuzia J, Kubas B, Walecki J. A comparison of spastic diplegic and tetraplegic cerebral palsy. Pediatr Neurol 2005;32(5):311-7.
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Neurometabolic Disorder Articles

Study on MRI Changes in Phenylketonuria in Patients Referred to Mofid Hospital/Iran


Iranian Journal of Child Neurology, Vol. 8 No. 2 (2014), , Page 53-56

How to Cite This Article: Karimzadeh P, Ahmadabadi F, Jafari N, Shariatmadari F, Nemati H, Ahadi A, Karimi Dardashti S, Mirzarahimi M, Dastborhan Z, Zare Noghabi J. Study on MRI Changes in Phenylketonuria in Patients Referred to Mofid Hospital. Iran J Child Neurol. 2014 Spring 8(2):53-56.

Phenylketonuria is one of the most common metabolic disorders and the first known cause of mental retardation in pediatrics. As Screening for phenylketonuria (PKU) is not a routine neurometabolic screening test for neonates in Iran, many PKU cases may be diagnosed after developing the clinical symptoms. One of the findings of PKU is myelination disorders, which is seen as hypersignal regions in T2-weighted (T2W) and FLAIR sequences of brain MRI. The aim of our study was to assess MRI changes in PKU patients referred to Mofid Children’s Hospital, 2010-2011.

Materials & Methods
We studied all PKU cases referred to our clinic as a referral neurometabolic center in Iran for brain MRI and assessed the phenylalanine level at the time of Imaging. The mean phenylalanine level (in one year), clinical manifestations,
and MRI pattern based on Thompson scoring, were evaluated.

The mean age of our study group was 155±99 months and the mean diagnosis age was 37±27.85 months. There were 15 patients with positive and 15 with negative family history. The mean phenylalanine level at the time of imaging was 9.75±6.28 and the mean 1 year phenylalanine level was 10.28±4.82. Seventy percent of our patients had MRI involvement, in whom 20% showed atrophic changes, in addition to white matter involvement. Based on modified Thompson scoring, the score for our study group was 4.84.
The maximum involvement in MRI was in occipital region, followed by parietal, frontal, and temporal zones. There was not any correlation between MRI score and patients’ age. But we found significant relationship between MRI score and
the age of regimen cessation. No correlation was seen between phenylalanine level (at the time of Imaging) and MRI score. But there was a relationship between mean 1 year phenylalanine level and MRI score.

According to the results of this study, brain MRI and white matter involvement can be used for evaluation of long-term control of phenylalanine level in PKU cases.



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3. Rezvani I, Melvin JJ. Defects in metabolism of amino acids. In: Kliegman RM, Stanton BF, St. Geme J, Behrman RE, editors. Nelson Textbook of Pediatrics. 19th ed. USA: Elsevier; 2011. p. 418-22.

4. Menkes J, Wilcox WR. Inherited Metabolic Diseases of nervous system. In: Menkes JH, editor Child neurology. 7th ed. Philadelphia: Lippincott Williams&Wilkins; 2006. p. 34-36.

5. Aicardi J. Diseases of the nervous system in childhood. London: Mac Keith press; 2009.

6. Enns GM, Cowan TM, Klein O, Packman S. Aminoacidemias and organic acidemias. In: Swaiman KF. Swaimans Pediatric Neurology principle and practice. 5th ed. China: Saunders; 2012. p. 330-7.

7. Barkovich J. Toxic and Metabolic Brain disorders. In: Barkovich J, editor Pediatric Neuroimaging. 4th ed. USA: Lippincott William&Wilkins;2005. p. 88-92.

8. Van der knaap MS. Phenylketonuria. In: Van der knaap MS, Valk J, editor. Magnetic resonance of Myelination and Myelin Disorders. 3rd ed. Germany: Springer; 2005. p. 285-90.

9. Manara R, Burlina AP, Citton V, Ermani M, Vespignani F, Carollo C, et al. Brain MRI diffusion-Weighted imaging in patients with classical phenylketonuria: Neuroradiology (2009)51:803-12.

10. Möller HE, Weglage J, Bick U, Wiedermann D, Feldmann R, Ullrich K. Brain imaging and proton Magnetic Resonance Spectroscopy in Patients with Phenylketonuria Pediatrics 2003;112(6 Pt 2):1580-3.

11. Phillips MD, McGraw P, Lowe MJ, Mathews VP, Hainline BE. Diffusion-Weighted Imaging of White Matter Abnormalities in Patients with Phenylketonuria. AJNR Am J Neuroradiol 2001 Sep;22(8):1583-6.

12. Cleary MA, Walter JH, Wraith JE, Jenkins JP, Alani SM, Tyler K, et al. Magnetic resonance Imaging of the Brain in Phenyl ketonuria. Lancet 1994;344(8915):87-90.

Case Report

Levatiracetam for the management of Lance-Adams syndrome

Faik ILIK*, Mustafa Kemal ILIK, İlker ÇOVEN

Iranian Journal of Child Neurology, Vol. 8 No. 2 (2014), , Page 57-59

How to Cite This Article: Ilik F, Ilik MK, Çöven I. Levatiracetam for the management of Lance-Adams syndrome. Iran J Child Neurol. 2014 Spring 8(2):57-59.


Chronic post-hypoxic myoclonus, also known as Lance-Adams syndrome (LAS) is a neurological complication characterized by uncontrolled myoclonic jerks following cardiac arrest. In this article, clinical manifestation and symptomatic treatment options are discussed especially concerning the rationale of use of levatiracetam in patients with Lance-Adams syndrome. Clinical presentation is action myoclonus associated with cerebellar ataxia, postural imbalance, and very mild intellectual deficit.

An 18-year-old female patient was admitted to our intensive care unit in a coma. She had a cardiorespiratory arrest after a splenectomy in a local hospital. Then, myoclonic movements were continuously observed over the entire body, including the face.

On day 14 of hospitalization, we started levatiracetam 1000 mg daily. The frequency of convulsion movements was reduced. The patient level of consciousness was 15 on the Glasgow coma scale (GCS) on the Mini-Mental State Examination (MMSE) score was 23 out of 30. She was later transferred to the rehabilitation department.

Vigilance is required to ensure early diagnosis and timely intervention for the myoclonic jerks. In conclusion, we would like to emphasize that LAS should be considered in patients with the myoclonic jerks following cardiac arrest and that levatiracetam therapy may be useful as treatment.


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2. Guo XH, Yu SY, Liu J, Wu WP, Pu CQ, Zhu K. Posthypoxic myoclonus treated with 5-hydroxytryptophan: a case report. J Clin Neurol 2002; 15: 313-6.

3. Arpesella R, Dallocchio C, Arbasino C, Imberti R, Martinotti R, Frucht SJ. A patient with intractable posthypoxic myoclonus (Lance-Adams syndrome) treated with sodium oxybate. Anaesth Intensive Care 2009; 37:314-8.

4. Werhahn KJ, Brown P, Rompson PD, Marsden CD. The clinical features and prognosis of chronic post-hypoxic myoclonus. Mov Disord 1997; 12: 216-20.

5. Frucht SJ, Trost M, Ma Y, Eidelberg D. The metabolic topography of post-hypoxic myoclonus. Neurology 2004; 62: 1879-1881.

6. Frucht S, Fahn S. The clinical spectrum of post-hypoxic myoclonus. Mov Disord 2000; 15 (Suppl 1): 2-7.

7. Matsumoto RR, Truong DD, Nguyen KD, Dang AT, Hoang TT, Vo PQ, Sandroni P. Involvement of GABA(A) receptors in myoclonus. Mov Disord 2000; 15(Suppl1):47-52.

The Acrocallosal Syndrome in A Neonate With Further Widening of Phenotypic Expression

Ravish SINGHAL*, Sadbhavna PANDIT, Ashok Saini, Paramjit SINGH, Neeraj DHAWAN

Iranian Journal of Child Neurology, Vol. 8 No. 2 (2014), , Page 60-64

How to Cite This Article: Singhal R, Pandit S, Saini A, Singh P, Dhawan N. The Acrocallosal Syndrome in A Neonate With Further Widening of Phenotypic Expression. Iran J Child Neurol. 2014 Spring;8(2):60-64.


The presentation of the typical characteristics of the acrocallosal syndrome (ACLS) are hypoplasia/agenesis of corpus callosum, moderate to severe mental retardation, characteristic craniofacial abnormalities, distinctive digital
malformation, and growth retardation in a neonate.
An Indian neonate presented on day 1 of life (youngest in the literature to be reported) with combination of abnormalities consistent with the acrocallosal syndrome and some additional findings. The baby, born to non-consanguineous, healthy parents, presented with macrocephaly, prominent forehead, hypertelorism, polydactyly of the hands and feet, duplication of hallux, hypotonia, recurrent cyanotic episodes, rib anomalies, dextro-positioning of heart, and delayed fall
of umbilical cord.
As the mode of inheritance of ACLS is autosomal recessive, the risk of recurrence is 25%. Genetic counselling is of prime importance, Polydactyly, and central nervous system malformations can be detected by ultrasonography in the second trimester, but due to variability of presentation, prenatal diagnosis may not always be possible.


1. Schinzel A. Postaxial polydactyly, hallux duplication, absence of corpus callosum, macencephaly and severe

mental retardation: a new syndrome? Helv Paediatr Acta 1979:34:141-146.

2. Schinzel A. The acrocallosal syndrome: expansion of the phenotypic spectrum. Clin Dysmorphol 1994;3:31-34.

3. Schinzel A, Schmid W. Hallux duplication, postaxial Polydactyly, absence of corpus callosum, severe mental retardation and additional anomalies in two unrelated patients: a new syndrome. Am J Med Genet 1980;6:241-249.

4. Moeschler JB, Pober BR, Holmes LB, Graham JM Jr. Acrocallosal syndrome: new findings. Am J Med Genet 1989;32:195-199.

5. Schinzel A, Kaufmann U. The acrocallosal syndrome in sisters. Clin Genet 1986;30:399-405.

6. Koenig R, Bach A, Woelki U, Grzeschik KH, Fusch S. Spectrum of acrocallosal syndrome. Am J Med Genet 2002;108:7-11.

7. Schinzel A. The acrocallosal syndrome in first cousins: widening of the spectrum of clinical features and further support for automal inheritance. J Med Genet 1988;25:332-336.

8. Hendriks HJE, Brunner HG, Haagen TAM, Hamel BCJ. Acrocallosal syndrome. Am J Med Genet 1990;35:443-446.

9. Yuksel M, Caliskan M, Ogur G, Ozmen M, Dolunary G, Apak S. The acrocallosal syndrome in a Turkish Boy. J Med Genet 1990;27:48-49.

10. Casamassima AC, Beneck D, Gewitz MH, Horowitz MA, Woolf PK, Pettersen IM, Shapiro LR. Acrocallosal Syndrome: additional manifestation. Am J Med Genet 1989;32:311-317.

11. Courtens W, Vamos E, Christophe C, Schinzel A. Acrocallosal syndrome in an Algerian boy born to consanguineous parents: review of the literature and further delineation of the syndrome. Am J Med Genet 1997;69:17-22.

12. Bassam R Ali, Jennifer L Silhavy, Nadia A Akawi, Joseph G Gleeson Lihadh Al-Gazali. A mutation in KIF7 is responsible for te autosomal recessive syndrome of macrocephaly, multiple epiphyseal dysplasia and distinctive facial appearance. Orphanet J Rare Dis 2012 May 15;7:27.

How to Cite This Article: Akhondian J, Ashrafzadeh F, Beiraghi Toosi M, Hashemi N. A Rare Presentation of Neurobrucellosis in A Child with Recurrent Transient Ischemic Attacks and Pseudotumor Cerebri (A Case Report and Review of Literature). Iran J Child Neurol. 2014 Spring; 8(2):65-69.


Brucellosis is a multi-system infectious disease that presents with various manifestations and complications. Neurobrucellosis is an uncommon but serious presentation of brucellosis that can be seen in all stages of the disease. High
index of suspicion, especially in endemic areas is essential to prevent morbidity from this disease.
The case was an 11- year -old female patient who was admitted with a severe headache that was worsening over a period of 2 months. The day after each attack, she experienced transient right hemiparesia that was lasting less than one hour (TIA) as well as blurred vision and bilateral papilledema. Laboratory findings revealed serum agglutination Wright test positive at 1/320 and 2ME test positive at 1/160. A lumbar puncture showed a clear CSF with increased opening pressure (32 cmH2O), CSF examination was within normal range (pseudotumor cerebri).To our knowledge, there has been no report for recurrent TIA in pediatric neurobrucellosis in the base of pseudotumor cerebri.
In endemic areas like Iran, unexplained neurological signs or symptoms should be evaluated for brucellosis.



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A Report of Guillain–Barré Syndrome With Myalgia and Mild Weakness


Iranian Journal of Child Neurology, Vol. 8 No. 2 (2014), , Page 70-72

How to Cite This Article: Aminzadeh V, Hassanzadeh Rad A. A Report of Guillain–Barré Syndrome With Myalgiaand Mild Weakness. Iran J Child Neurol. 2014 Spring; 8(2):70-72.


We report a rare case that revealed severe myalgia as the chief complaint that is not mentioned in the list of frequent symptoms of Guillain Barré.
Guillain-Barré syndrome (GBS) is an acute inflammatory demyelinating polyneuropathy (AIDP).Required features for diagnosis of GBS are progressive motor weakness of more than one limb and areflexia. We report an 11-yearold
boy who was referred to the emergency department with complaints of generalized body pain and gate problem.
It seems that if myalgias are the chief complaint and weakness is mentioned as a less important symptom, clinicians should consider GBS after ruling out other
reasons for myalgia especially inflammatory myositis.



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