Iranian Journal of Child Neurology

How to Cite This Article: Dara N, Sayyari AA, Imanzadeh F. Hepatic Encephalopathy: Early Diagnosis in Pediatric Patients With Cirrhosis. Iran J Child Neurol. 2014 Winter; 8(1):1-11.

Objective

As acute liver failure (ALF) and chronic liver disease (cirrhosis) continue to increase in prevalence, we will see more cases of hepatic encephalopathy.
Primary care physician are often the first to suspect it, since they are familiar with the patient’s usual physical and mental status. This serious complication typically occurs in patients with severe comorbidities and needs multidisciplinary evaluation and care. 
Hepatic encephalopathy should be considered in any patient with acute liver failure and cirrhosis who presents with neuropsychiatric manifestations, decrease level of consciousness (coma), change of personality, intellectual
and behavioral deterioration, speech and motor dysfunction.
Every cirrhotic patient may be at risk; potential precipitating factors should be addressed in regular clinic visits. The encephalopathy of liver disease may be prominent, or can be present in subtle forms, such as decline of school performance, emotional outbursts, or depression.
“Subtle form” of hepatic encephalopathy may not be obvious on clinical examination, but can be detected by neurophysiologic and neuropsychiatric testing.

References:

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How to Cite This Article: Fallah R, Salor F, Akhavan Karbasi S, Motaghipisheh H. Randomised Clinical Efficacy Trial of Topiramate and Nitrazepam in Treatment of Infantile Spasms. Iran J Child Neurol. 2014 Winter; 8(1):12-19.

Objective
Infantile spasms (IS) are among the most catastrophic epileptic syndromes of infancy. The purpose of this study was to compare efficacy and safety of topiramate (TPM) and nitrazepam (NZP) as first-line drugs in the treatment of
IS.

Materials & Methods

In a parallel single-blinded randomized clinical trial, 50 patients with IS referred to Pediatric Neurology Clinic of Shahid Sadoughi University of Medical Sciences, Yazd, Iran, were evaluated from September 2008 to March 2010.
Patients were randomly assigned to two groups to be treated with TPM or with NZP for 6 months. The primary endpoint was efficacy in cessation of all spasms or reduction of more than 50% in weekly seizure frequency, which was evaluated
before and 6 months after the drug use. Secondary outcome was clinical sideeffects of the drugs.

Results
Twenty boys (40%) and 30 girls (60%) with the mean age of 9.4±3.8 months were evaluated. Cessation of all spasms occurred in 12 (48%) infants in TPM group and 4(16%) in NZP group. Eight (32%) children in TPM group and 7 (28%) in NZP group had more than 50% reduction in spasms frequency. So,
TPM was more effective. Side effects were seen in 32% of TPM and in 36% of NZP groups.

Conclusion
Topiramate is an effective and safe drug, which might be considered as the firstline drug for the treatment of ISs.

References:

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2. Tsao CY. Current trends in the treatment of infantile spasms. Neuropsychiatr Dis Treat 2009;5:289-99.
3. Sankar R, Koh S, Wu J, Menkes JH. Paroxysmal disorders. In: Menkes JH, Sarnat HB, Maria BL, editors Child Neurology,7th ed. Philadelphia: Lippincott; 2006.p. 877.
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8. Jaseja H. Justification of vigabatrin administration in West syndrome patients? Warranting a re-consideration for improvement in their quality of life. Clin Neurol Neurosurg 2009;111(2):111-14.
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How to Cite This Article: Karimzadeh P, Rezayi A, Togha M, Ahmadabadi F, Derakhshanfar H, Azargashb E, Khodaei F. The Best Time for EEG Recording in Febrile Seizure. Iran J Child Neurol. 2014 Winter; 8(1):20-25.

Objective
Some studies suggest that detection of epileptic discharge is unusual during the first postictal week of febrile seizure and others believe that EEGs carried out on the day of the seizure are abnormal in as many as 88% of the patients. In this
study, we intend to compare early and late EEG abnormalities in febrile seizure.

Materials & Methods 
EEG was recorded during daytime sleep, 24-48 hours (early EEG) and 2 weeks (late EEG) after the seizure in 36 children with febrile seizure (FS), aged between 3 months and 6 years. EEGs that showed generalized or focal spikes, sharp, spike wave complex, and slowing were considered as abnormal EEG.
Abnormalities of the first EEG were compared with those of second EEG.

Results
The most common abnormal epileptiform discharges recorded in the early EEG were slow waves (27.6%) and sharp waves in late EEG (36%). Distribution of abnormalities in early and late EEG showed no significant statistical difference.

Conclusion
The early and late EEG recording had the same results in patient with febrile seizure.

Reference:

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How to Cite This Article: Tonekaboni Sh, Jafari Naeini S, Khajeh A, Yaghini O, Ghazavi A, Abdollah Gorji F. Use of Complementary and Alternative Medicine for Epileptic Children in Tehran: A Cross-Sectional Study (2009-2011). Iran J Child Neurol. 2014 Winter; 8(1):26-31.

Objective
Although the use of Complementary and Alternative Medicine (CAM) has been evaluated globally, there are few studies in our country on this subject. The purpose of this study was to determine the prevalence, pattern of use, parental sources of information, and benefits of CAM in epileptic children in Tehran.

Materials & Methods
One hundred thirty-three parents or relatives of epileptic children who were referred to outpatient clinics or admitted in neurologic ward of four major hospitals in Tehran, were interviewed by our researcher based on a structured questionnaire; from 2009 to 2010. The information obtained comprised the demographic data of patients and their parents, frequency and morphology of convulsions, the type and sources of CAM and finally, the benefits and adverse
effects of this practice.

Results
Forty-four percent of the respondents had used CAM methods either alone or in combination with other methods. The most frequently used CAM was written prayers followed by oral herbs and special diets. CAM was mainly introduced to them by relatives. Only 16.7% of these parents had discussed this matter with their children’s physicians. No efficacy to control seizure was observed for most of these methods.

Conclusion
This study showed that use of CAM in our study group is relatively common and may have a potentially hazardous role in the treatment process. So, it is necessary for physicians to have enough information about CAM practice in their patients.

References:

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3. Michi CA. The use of herbal remedies in Jamaica. Ann Trop Paediatr 1992;12(1):31-6.
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7. Khonsari A, Gorji K, Abdollahpur F, Dehestani S, Mousavi A, Delfan B et al. Study of international approaches on treatment of common diseases using different methods of complementary medicine. J Ilam Uni Med Sci 2009;4:37-45. (Full text in Persian)
8. Kwan P, Brodie MJ. Early identification of refractory epilepsy. N Engl J Med 20003;342(5):314-9.
9. Oshikoya KA, Senbanjo IO, Njokanma OF, Soipe A. Use of complementary and alternative medicine for children with chronic health conditions in Lagos, Nigeria. BMC Complement Altern Med 2008;8:66.
10. Liow K, Ablah E, Nguyen JC, Sadler T, Wolfe D, Tran KD et al. Pattern and frequency of use of complementary and alternative medicine among patients with epilepsy in the mid western United States. Epilepsy Behav 2007;10(4):576-82.
11. Tehrani Banihashemi SA, Asgharifard H, Haghdoost AA, Barghamdi M, Mohammadhosseini N. The use of complementary/ Alternative medicine among the general population in Tehran /Iran. Payesh 2008;7(4):355-62.(Full text in Persian)
12. Ekici B, That B, Abah S, Aydinli N, Ozmen M. Application of complementary and alternative medicine in epileptic children at a tertiary pediatric neurology center in Turkey. Eur J Integr Med 2011;4:e71-5.
13. Soo I, Mah JK, Barlow K, Hamiwka L, Wirrell E. Use of complementary and alternative medical therapies in pediatric neurology clinic. Can J Neurol Sci 2005;32(4):524-28.
14. Cuzzolin L, Zaffani S, Murgia V, Gangemi M, Meneghelli G, Chiamenti G, et al. Patterns and perceptions of complementary/alternative medicine among paediatricians and patients’ mothers: a review of the literature. Eur J Pediatr 2003;162(12):820-7.

How to Cite This Article: Heidari MM , Khatami M, Pourakrami J. Novel Point Mutations in Frataxin Gene in Iranian Patients with
Friedreich’s Ataxia. Iran J Child Neurol. 2014 Winter; 8(1):32-36.

Objective
Friedreich’s ataxia is the most common form of hereditary ataxia with autosomal recessive pattern. More than 96% of patients are homozygous for GAA repeat extension on both alleles in the first intron of FXN gene and the remaining
patients have been shown to be heterozygous for a GAA extension in one allele and point mutation in other allele.

Materials & Methods
In this study, exons of 1, 2, 3, and 5 of frataxin gene were searched by single strand conformation polymorphism polymerase chain reaction (PCR-SSCP) in 5 patients with GAA extension in one allele. For detection of exact mutation,
samples with band shifts were sent for DNA sequencing.

Results 
Three novel point mutations were found in patients heterozygous for the GAA repeat expansion, p.S81A, p.Y123D, and p.S192C. 

Conclusion
Our results showed that these point mutations in one allele with GAA extension in another allele are associated with FRDA signs. Thus, these results emphasize the importance of performing molecular genetic analysis for point mutations in
FRDA patients.

References:

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2. Harding AE, Zilkha KJ. ‘Pseudo-dominant’ inheritance in Friedreich’s ataxia. J Med Genet 1981;18(4):285-7.
3. Schulz JB, Boesch S, Burk K, Durr A, Giunti P, Mariotti C, et al. Diagnosis and treatment of Friedreich ataxia: a European perspective. Nat Rev Neurol 2009;5(4):222-34.
4. Campuzano V, Montermini L, Lutz Y, Cova L, Hindelang C, Jiralerspong S, et al. Frataxin is reduced in Friedreich ataxia patients and is associated with mitochondrial membranes. Hum Mol Genet 1997;6(11):1771-80.
5. Sharma R, De Biase I, Gomez M, Delatycki MB, Ashizawa T, Bidichandani SI. Friedreich ataxia in carriers of unstable borderline GAA triplet-repeat alleles. Ann Neurol 2004;56(6):898-901.
6. Durr A, Cossee M, Agid Y, Campuzano V, Mignard C, Penet C, et al. Clinical and genetic abnormalities in patients with Friedreich’s ataxia. N Engl J Med 1996;17;335(16):1169-75.
7. Filla A, De Michele G, Cavalcanti F, Pianese L, Monticelli A, Campanella G, et al. The relationship between trinucleotide (GAA) repeat length and clinical features in Friedreich ataxia. Am J Hum Genet 1996;59(3):554-60.
8. Lamont PJ, Davis MB, Wood NW. Identification and sizing of the GAA trinucleotide repeat expansion of Friedreich’s ataxia in 56 patients. Clinical and genetic correlates. Brain 1997;120 ( Pt 4):673-80.
9. Montermini L, Richter A, Morgan K, Justice CM, Julien D, Castellotti B, et al. Phenotypic variability in Friedreich ataxia: role of the associated GAA triplet repeat expansion. Ann Neurol 1997;41(5):675-82.
10. Monros E, Molto MD, Martinez F, Canizares J, Blanca J, Vilchez JJ, et al. Phenotype correlation and intergenerational dynamics of the Friedreich ataxia GAA trinucleotide repeat. Am J Hum Genet 1997;61(1):101-10.
11. Zuhlke CH, Dalski A, Habeck M, Straube K, Hedrich K, Hoeltzenbein M, et al. Extension of the mutation spectrum in Friedreich’s ataxia: detection of an exon deletion and novel missense mutations. Eur J Hum Genet 2004;12(11):979-82.
12. Heidari MM, Houshmand M, Hosseinkhani S, Nafissi S, Scheiber-Mojdehkar B, Khatami M. A novel mitochondrial heteroplasmic C13806A point mutation associated with Iranian Friedreich’s ataxia. Cell Mol Neurobiol 2009;29(2):225-33.
13. Heidari MM, Houshmand M, Hosseinkhani S, Nafissi S, Scheiber-Mojdehkar B, Khatami M. Association between trinucleotide CAG repeats of the DNA polymerase gene (POLG) with age of onset of Iranian Friedreich’s ataxia patients. Neurol Sci 2008; 29(6):489-93.
14. Heidari MM, Houshmand M, Hosseinkhani S, Nafissi S, Khatami M. Complex I and ATP content deficiency in lymphocytes from Friedreich’s ataxia. Can J Neurol Sci 2009;36(1):26-31.
15. Campuzano V, Montermini L, Molto MD, Pianese L, Cossee M, Cavalcanti F, et al. Friedreich’s ataxia: autosomal recessive disease caused by an intronic GAA triplet repeat expansion. Science 1996;271(5254):1423-7.
16. Sambrook J, Russel DW. Chapter 13: Detection of Mutations by Single-strand Conformational Polymorphism and Heteroduplex Analysis. Molecular cloning: a laboratory manual. 3eded. New York: Cold Spring Harborn Laboratory Press; 2001. P.49-59.
17. Kyte J, Doolittle RF. A simple method for displaying the hydropathic character of a protein. J Mol Biol 1982;157(1):105-32.
18. Harding AE, Hewer RL. The heart disease of Friedreich’s ataxia: a clinical and electrocardiographic study of 115 patients, with an analysis of serial electrocardiographic changes in 30 cases. Q J Med 1983;52(208):489-502.
19. Cavalier L, Ouahchi K, Kayden HJ, Di Donato S, Reutenauer L, Mandel JL, et al. Ataxia with isolated vitamin E deficiency: heterogeneity of mutations and phenotypic variability in a large number of families. Am J Hum Genet 1998;62(2):301-10.
20. Anheim M, Mariani LL, Calvas P, Cheuret E, Zagnoli F, Odent S, et al. Exonic deletions of FXN and early-onset Friedreich ataxia. Arch Neurol 2012;69(7):912-6.
21. Li H, Gakh O, Smith DYt, Ranatunga WK, Isaya G. Missense mutations linked to friedreich ataxia have different but synergistic effects on mitochondrial frataxin isoforms. J Biol Chem 2013;288(6):4116-27.
22. Evans-Galea MV, Corben LA, Hasell J, Galea CA, Fahey MC, du Sart D, et al. A novel deletion-insertion mutation identified in exon 3 of FXN in two siblings with a severe Friedreich ataxia phenotype. Neurogenetics 2011;12(4):307-13.

How to Cite This Article: Jalili M, Jahangiri N, Yazdi Aa, Ashrafzadeh F. The Effects of Imitative Vs. Cognitive Methods on The Speech Development of Children With Autism. Iran J Child Neurol. 2014 Winter; 8(1):37-46.

Objective
The present study was performed to examine the effects of two speech therapy methods on six verbal behaviors of autistic children, including oral speech, listening, organizing, speaking, semantics, and syntax.

Materials & Methods
In this study, thirty 6-8 years old children with autism were assigned to one of two groups: imitative and cognitive groups. Before starting the main procedures of the study, the children of both groups were homogenized concerning their autism level. In the first phase of the study, the speech development level of the two groups was measured in a pre-test, in which both groups showed similar results. Then, both groups of children received 6 months of speech therapy instruction, during which one group was taught using an imitative method, while the other group was being worked with cognitive method.

Results
After 6-month treatment period, a post-test was done, and the t-tests based on the data of the two groups revealed a significant difference between the results.

Conclusion
The statistics showed that after the teaching period, autistic that worked with cognitive method gained a better development in their speech abilities, comparing to those worked with the imitative method.

Reference:

1. Samadi SA, Mahmoodizadeh A, McConkey R. A national study of the prevalence of Autism among five year old children in Iran. Autism 2012;16(1):5-14.
2. Fombonne E, Simmons H, Ford T, Meltzer H, Goodman R. Prevalence of pervasive developmental disorders in the British nationwide survey of child mental health. J Am Acad Child Adolesc Psychiatry 2001;40(7):820-7.
3. Fombonne, E. The changing epidemiology of Autism. J Appl Res Intellect Disabil 2005,18(4):281-94.
4. Filipek PA, Accardo PJ, Ashwal S, Baranek GT, Cook EH Jr, Dawson G, et al. Practice parameter: screening and diagnosis of autism: report of the Quality Standards Subcommittee of the American Academy of Neurology and the Child Neurology Society. Neurology 2000;55(4):468-79.
5. Samadi, SA. Comparative policy brief: status of intellectual disabilities in the Islamic republic of Iran. J Policy Pract Intellect Disabil 2008;5:129-32.
6. Rogers SJ, An examination of the imitation deficit in autism. In: Nadel J, Butterworth G, editors. Imitation in infancy. Cambridge: Cambridge University Press; 1999. p.254-83.
7. Ozonoff S, Pennington BF, Rogers SJ. Executive function deficits in high-functioning autistic individuals: Relationship to theory of mind. J Child Psychol Psychiatry 1991;32(7):1081-105.
8. Williams JH, Whiten A, Suddendorf T, Perrett DI. Imitation, mirror neurons and autism. Neurosci Biobehav Rev 2001;25(4):287-95.
9. Rogers SJ, Hepburn SL, Stackhouse T, Wehner E. Imitation performance in toddlers with autism and those with other developmental disorders. J Child Psychol Psychiatry 2003;44(5):763-81.
10. Gena A, Krantz PJ, McClannahan LE, Poulson CL. Training and generalization of affective behavior displayed by youth with autism. J Appl Behav Anal 1996;29(3):291-304.
11. Ingenmey R, Van Houten R. Using time delay to promote spontaneous speech in an autistic child. J Appl Behav Anal 1991;24(3):591-6.
12. Ayzenson J. Language and speech disorders in children. Tehran: Roshd publications; 2010.
13. De Giacomo A, Fombonne E. Parental recognition of developmental abnormalities in autism. Eur Child Adolesc Psychiatry 1998;7(3):131-6.
14. Hodgdon LA. Solving behavior problems in autism: improving communication with visual strategies. Quirkroberts publishing; 2001. P.32.
15. Bertrand J, Mars A, Boyle C, Bove F, Yeargin-Allsopp M, Decoufle P. Prevalence of autism in a United States population: the Brick Township, New Jersey, investigation. Pediatrics 2001;108(5):1155-61.
16. Siegel B. Helping children with autism learn: a guide to treatment approaches for parents and professionals. Oxford: Oxford Press Inc; 2002. P.3.
17. Plimley L, Bowen M. Social skills and autism spectrum disorders, London: Paul Chapman publishing; 2007.
18. Mohammadi M, Mesgarpour B, Sahimi Izadian E, Mohammadi M. Psychiatric tests for children and young adults: CARS test. Tehran: Teimourzadeh publications; 2006.
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21. Dequinzio JA. Generalized Imitation of Facial Models by Children with Autism. J Appl Behav Anal 2007;40(4):755-9.
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How to Cite This Article: Esmaeeli M, Erfani Sayar R, Saghebi A, Elmi Saghi, Rahmani Sh, Elmi S, Rabbani Javadi A. Screening for Depression in Hospitalized Pediatric Patients. Iran J Child Neurol. 2014 Winter; 8(1):47-51.

Objective
In chronically ill children who are hospitalized, many mood changes occur. For example, in children with cancer or renal failure, prolonged hospitalization and chemotherapy can lead to depression. With the improved survival of childhood
malignancies, the effect of treatment on child’s psychosocial well-being becomes increasingly relevant. In this study, we examined the prevalence of depression in hospitalized children with chronic and acute conditions in Dr Sheikh Pediatrics Hospital in Mashhad.

Materials & Methods
After receiving the approval from the Ethics Committee of Mashhad University of Medical Sciences, we did this cross-sectional descriptive study, from April to June 2012 in Dr Sheikh Pediatric Hospital in Mashhad. Ninety children, aged between 8 to 16 years, were screened for depression. The sampling method was census. Children with a history of depressive or other mental disorders were excluded.
Three groups of children (children with chronic renal disease, malignancy, and acute disease) were evaluated for depression using standard Children Depression Inventory Questionnaire (CDI). Two specifically trained nurses with the supervision
of a psychiatrist filled out the questionnaires at patients’ bedside. Depression scores were then analyzed by SPSS software.

Results
Of 90 children, 43(47.7%) were male and 47(52.2%) were female. The Children’s mean age was 11±2.3 years, and the mean length of hospitalization was 8±5.3 days.
Depression was detected in various degrees in 63% of patients (N=57), and 36.6% of children (N=32) had no symptoms of depression. Severe depression was not seen
in any of the patients with acute illness. More than half of patients with cancer and chronic kidney disease had moderate to severe depression.
There was a significant statistical relationship between the duration of illness and severity of depression. There was also a significant correlation between severity of depression and frequency of hospitalization. Children who had been hospitalized more than 3 times in the last year, experienced more severe levels of depression. We also found a significant correlation between pubertal age and severity of depression
in patients with cancers and chronic renal failure.

Conclusion
Children who are hospitalized due to chronic conditions are at a higher risk for mood disorders in comparison with the ones with acute conditions. It is therefore advisable to consider more practical plans to improve the care for hospitalized
children’s mental health.

References

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2. Rutter M. Relationship between mental disorders in children and adulthood. Acta Psychiatr Scand 1995;91(2):73-85.
3. Naerde A, Tambs K, Mathiesen KS Dalgard OS. Symptoms of anxiety and depression among mothers of pre-school children: effect of chronic strain related to children and child care-taking. J affect Discord 2000;58(3):181-99
4. Ernest J, Weissflog G, Brahler E, Romer G, Götze H. Depression in children of cancer patients over time and dependence of parental distress. Prax Kinderpsychol Kinderpsychiatr 2012;61(6):432-46.
5. Gordijn MS, van Listenburg RR, Gemke RJ, Huisman J. Sleep, fatigue, depression, and quality of life in survivors of childhood acute lymphoblastic leukemia. Pediatr Blood Cancer 2013;60(3):479-85.
6. De Sousa A. Psychiatric issues in renal failure and dialysis. Indian J Nephrol 2008;18(2):47-50
7. Bakr A, Amr M, Sarhan A, Hammad A. Psychiatric disorders in children with chronic renal failure. Pediatr Nephrol 2007;22(1):128-31.
8. Goldstein SL, Rosburg NM, Warady BA, Seikaly M, McDonald R, Limbers C, et al. Pediatric end stage renal disease health-related quality of life differs by modality: a PedsQL ESRD analysis. Pediatr Nephrol 2009;24(8):1553-60.
9. Thomalla G, Barkmann C, Romer G. Psychosocial symptoms in children of hemodialysis patients. Prax Kinderpsychol Kinderpsychiatr 2005;54(5):399-416.
10. Laffond C, Dellatolas G, Alapetite C, Puget S, Grill J, Habrand JL, et al. Quality of life, mood and executive functioning after childhood craniopharyngioma treated with surgery and proton beam therapy. Brain Inj 2012;26(3):270-81.
11. Chung TK, Lynch ER, Fiser CJ, Nelson DA, Agricola K, Tudor C, et al. Psychiatric coomorbidity and treatment response in patients with tuberous sclerosis complex. Ann Clin Psychiatry 2011;23(4):263-9.
12. Kinahan KE, Sharp LK, Seidel K, Leisenring W, Didwania A, Lacouture ME, et al. Scarring, disfigurement, and quality of life in long-term survivors of childhood cancer: a report from the Childhood Cancer Survivor study. J Clin Oncol 2012;30(20):2466-74.
13. Adduci A, Jankovic M, Strazzer S, Massimino M, Clerici C, Poggi G. Parent-child communication and psychological adjustment in children with a brain tumor. Pediatr Blood Cancer 2012;59(2):290-4
14. Szabo A, Mezei G, Kovari E, Cserhati E. Depressive symptoms amongst asthmatic children caregivers. Peditr Allergy Immunol 2012;21(4 Pt 2):e667-73.
15. Arabiat DH, Elliot B, Draper P. The prevalence of depression in pediatric oncology patients undergoing chemotherapy treatment in Jordan. Pediatr Oncol Nurs 2012;29(5):283-8.
16. Li HC, Williams PD, Lopez V, Chung JO. Relationships among therapy-related symptoms, depressive symptoms, and quality of life in Chinese children hospitalized with cancer: an exploratory study. Cancer Nurs 2013;36(5):346-54.

How to Cite This Article: Nazari Sh, Moradi N, Sadeghi Koupaei MT. Evaluation of The Effects of Psychotherapy on Anxiety Among Mothers of Children With Leukemia. Iran J Child Neurol. 2014 Winter; 8(1):52-57.

Objective
Children with leukemia and their families face a long period of medical treatment and uncertainty about the future. These families may suffer from short- and long-term emotional problems. The aim of the present study was to assess the
effect of supportive psychotherapy on the anxiety of mothers whose children suffer from leukemia.

Materials & Methods
The current research were performed on mothers who had a child with leukemia hospitalized in Mofid Children’s Hospital, Tehran, Iran. They were randomly selected. The research method was a pseudo-experimental study with pretest/
posttest design. The pretest Kettles’ anxiety questionnaire was given to all the mothers and after seven sessions of supportive psychotherapy, the posttest was performed and the grades were compared.

Results
Ten mothers finished all seven therapeutic sessions. There was a statistically significant difference between the pretest and posttest mean scores, confirming the mothers’ reduced anxiety level.

Conclusion
Finding effective and newer approaches to improve the well-being of parents with a sick child is an important challenge of today’s medical researches. Based on our findings, it is possible to reduce the anxiety in mothers of children with
leukemia through supportive psychiatric therapies.

References

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2. Pourfathollah AA, Rakhshan M, Ahmadi M, Izadyar M. FAB Classification of childhood leukemia in Iran. Med J Islam Repub Iran 1988;4(2):263-4.
3. Brown R, Madan-Swain A, Lambert R. Posttraumatic stress symptoms in adolescent survivors of childhood cancer and their mothers. J Trauma Stress 2003;16(4):309-18.
4. Dolgin M, Phipps S, Fairclough D, Sahler OJ, Askins M, Noll R, et al. Trajectories of adjustment in mothers of children with newly diagnosed cancer: A natural history investigation. J Pediatr Psychol 2007;32(7):771-82.
5. Patiño-Fernández AM, Pai A, Alderfer M, Hwang WT, Reilly A, Kazak A. Acute stress in parents of children newly diagnosed with cancer. Pediatr Blood Cancer 2008;50(2):289-92.
6. Kazak A, Boeving A, Alderfer M, Hwang WT, Reilly A. Posttraumatic stress symptoms during treatment in parents of children with cancer. J clin oncol 2005;23(30):7405-10.
7. Brown S, Yalom I. Interactional group psychotherapy with alcoholic patients. J Stud Alcohol 1977;38(3):426-456.
8. Corraze J. An outline of General Psychopathology (Mental Diseases).Translated to Persian by Mansour M and Dadsetan P. Tehran: Roshd Publication; 2008. p.210-27. (Text in Persian)
9. Sahler OJ, Fairclough D, Phipps S, Mulhern R, Dolgin M, Noll R, et al. Using problem-solving skills training to reduce negative affectivity in mothers of children with newly diagnosed cancer: Report of a multisite randomized trial. J Consult Clin Psychol 2005 Apr;73(2):272-83.
10. Streisand R, Rodrique J, Houck C, Graham-Pole J, Berlant N. Brief Report: Parents of children undergoing bone marrow transplantation: Documenting stress and piloting a psychological intervention program. J Pediatr Psychol 2000;25(5):331-7.
11. Wijnberg-Williams BJ, Kamps WA, Klip EC, Hoekstra-Weebers JE. Psychological distress and the impact of social support on fathers and mothers of pediatric cancer patients: Long-term prospective results. Journal J Pediatr Psychol 2006;31(8):785-92.
12. Kazak AE, Alderfer MA, Streisand R, Simms S, Rourke MT, Barakat LP, et al. Treatment of posttraumatic stress symptoms in adolescent survivors of childhood cancer and their families: a randomized clinical trial. J Fam Psychol 2004;18(3):493-504.
13. Young B, Dixon Woods M, Findly M, Heney D. Parenting in a crisis: conceptualizing of mothers of children with cancer. Soc Sci Med 2002;55(10):1835-47.
14. Fisher HR. The need of parents with chronically sick children: a literature review. J Adv Nurs 2001;36(4):600-7.
15. Stewart M, Davidson K, Meade D, Hirth A, Weld-Viscount P. Group support for couples coping with a cardiac condition. J Adv Nurs 2001;33(2):190-9.
16. Kyngas H, Rissanen M. Support as a crucial predictor of good compliance of adolescents with a chronic disease. J Clin Nurs 2001;10:767-74.

How to Cite This Article: Karimzadeh P, Jafari N, Ahmad Abadi F, Jabbehdari S, Taghdiri MM, Alaee MR, Ghofrani M, Tonekaboni SH, Nejad Biglari H. Propionic Acidemia: Diagnosis and Neuroimaging Findings of This Neurometabolic Disorder. Iran J Child Neurol. 2014 Winter; 8(1):58-61.

Objective
Propionic acidemia is one of the rare congenital neurometabolic disorders with autosomal recessive inheritance. This disorder is caused by a defect in the propionyl-CoA carboxylase enzyme and can be presented with life-threatening ketoacidosis, lethargy, failure to thrive, and developmental delay.

Materials & Methods
The patients diagnosed as having propionic acidemia in Neurology Department of Mofid Children’s Hospital in Tehran, Iran, between 2002 and 2012 were include in our study. This disorder was confirmed by clinical manifestations, neuroimaging findings, and neurometabolic assessment in the reference laboratory in Germany. Our study was conducted to define the sex, age, gender, past medical history, developmental status, clinical findings, and  neuroimaging manifestations in 10 patients with propionic acidemia.

Results
Seventy percent of patients were offspring of consanguineous marriages. In this study, only one patient had microcephaly at birth, but at detection time, 30% of patients had head circumference and weight below the 3rd percentile. 
The patients were followed for approximately 5 years and this follow-up showed that the patients with early diagnosis had a more favorable clinical response.
Neuroimaging findings included brain atrophy, white matter and globus pallidus involvement.

Conclusion
Finally we suggest that early diagnosis and treatment have an important role in the prevention of disease progression.

References:

1. Ramachandran R, Pietz J. Propionic acidemia without acidemia: a case report. J Perinatol 1995;15(1):71-3.
2. Figueras Aloy J, Ribes Rubio A, Vilaseca Busca MA, Lluch Mir M, Brines Godino P, Jiménez González R, et al. [The neonatal form of propionic acidemia]. An Esp Pediatr 1988;29(6):459-62.
3. Yuan L. [Propionic acidemia: one case report]. Zhongguo Yi Xue Ke Xue Yuan Xue Bao 1991;13(2):141-3.
4. Hsu WC, Lin SP, Huang FY, Wang PA, Hsiao KJ. [Propionic acidemia: report of a case that is successfully managed by peritoneal dialysis and sodium benzoate therapy]. Zhonghua Yi Xue Za Zhi (Taipei) 1990;46(5):306-10.
5. Schreiber J, Chapman KA, Summar ML, Ah Mew N, Sutton VR, MacLeod E, et al. Neurologic considerations in propionic acidemia. Mol Genet Metab 2012;105(1):10-5.
6. Chapman KA, Gropman A, MacLeod E, Stagni K, Summar ML, Ueda K, et al. Acute management of propionic acidemia. Mol Genet Metab 2012;105(1):16-25.
7. Ryu J, Shin YH, Ko JS, Gwak MS, Kim GS.Intractable metabolic acidosis in a child with propionic academia undergoing liver transplantation -a case report. Korean J Anesthesiol 2013;65(3):257-61.
8. Fernandes J, Saudubray JM, Berghe GVD, Walter JH. Inborn Metabolic Diseases. 4th ed. Berlin: Springer;2006. P.247–256.
9. Sutton VR, Chapman KA, Gropman AL, MacLeod E, Stagni K, Summar ML, et al. Chronic management and health supervision of individuals with propionic acidemia. Mol Genet Metab 2012;105(1):26-33.
10. Lehnert W, Sperl W, Suormala T, Baumgartner ER. Propionic acidaemia: clinical, biochemical and therapeutic aspects. Experience in 30 patients. Eur J Pediatr 1994;153(7 Suppl. 1):S68–S80.
11. Ozand PT, Rashed M, Gascon GG, Youssef NG, Harfi H, Rahbeeni Z, et al. Unusual presentations of propionic acidemia. Brain Dev 1994;16(Suppl):46-57.
12. Feliz B, Witt DR, Harris BT. Propionic acidemia: a neuropathology case report and review of prior cases. Arch Pathol Lab Med 2003;127(8):e325-8.
13. Hamilton RL, Haas RH, Nyhan WL, Powell HC, Grafe MR. Neuropathology of propionic acidemia: a report of two patients with basal ganglia lesions. J Child Neurol 1995;10:25-30.
14. Brismar J, Ozand PT. CT and MR of the brain in disorders of the propionate and methylmalonate metabolism. Am J Neuroradiol 1994;15(8):1459-73.
15. Bergman AJ, Van der Knaap MS, Smeitink JA, Duran M, Dorland L, Valk J, et al. Magnetic resonance imaging and spectroscopy of the brain in propionic acidemia: clinical and biochemical considerations. Pediatr Res 1996;40(3):404-9.

How to Cite This Article: Shiari R, Hassase Yegane M, Farivar S, Javadi Parvaneh V, Mirjavadi SA. Neuropsychiatric symptoms as the first manifestation of Juvenile Systemic Lupus Erythematosus: A complicated case with Klinefelter’s syndrome. Iran J Child Neurol. 2014 Winter; 8(1):62-65.

Abstract

Systemic Lupus Erythematosus (SLE) is an autoimmune, multisystem disorder with various manifestations. There are limited reports on the neuropsychiatric findings as the first manifestation of SLE in children. Herein, we reported a
14-year-old Iranian boy with a two-year history of cognitive dysfunction and behavioural problems as well as a recent history of epistaxis. The patient workup ended with a diagnosis of Klinefelter’s syndrome associated with juvenile
SLE. Patients with Klinefelter’s syndrome may exhibit behavioural problems and psychological disease. These psychiatric disorders could be complicated with lupus in children. In fact, psychiatric symptoms may occur as the first
manifestation of juvenile SLE. Specially, if accompanied with Klinefelter’s syndrome. We suggest the diagnosis of SLE must be strongly considered in all children with neuropsychiatric manifestations.

References:

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2. Yu CH, Lee MB, Tseng MM, Liao SC. Obsessive-Compulsive symptoms as a manifestation of neuropsychiatric SLE. J Formus Med Assoc 2008;107:68-72.
3. Harel L, Sandborg Ch, Lee T, Scheven E. Neuropsychiatric manifestations in paediatric SLE and association with antiphospholipid antibodies. J Rheumatol 2006;33:1873-7.
4. Bernatsky S, Clarke A, Gladman DD, Urowitz M, Fortin PR, Barr SG, et al. Mortality related to Cerebrovascular disease in SLE. Lupus 2006; 15:835-9.
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How to Cite This Article: Talebian A, Goudarzi RM, Mohammadzadeh M , Mirzadeh AS. Vincristine-Induced Cranial Neuropathy. Iran J Child Neurol. 2014 Winter; 8(1):66-68.

Abstract
Vincristine (VCR) is a vinca alkaloid that is used for treatment of many malignancies.
The vinca alkaloids are neurotoxic, usually causing a peripheral neuropathy, but cranial neuropathies are rare as side effects. 
Described here is the case of a 2.5-year-old boy, a known case of Wilms’ tumor, treated by vincristine (0/067 mg/kg/day) and dactinomycin (0/045 mg/kg/day) after surgery. Three weeks after treatment, he presented with bilateral ptosis.
Neurological examination revealed bilateral ptosis with normal pupillary reflex and eye movement. He received 3.015 mg cumulative dose of vincristine before development of ptosis.
Treatment with pyridoxine (150 mg/m2 p.o. BID) and pyridostigmine (3 mg/kg p.o. BID) started as neuroprotective agents, and after 7 days the problem disappeared.
The treatment continued for 6 weeks and there were no signs of ptosis or a recurrence in follow up 2 months later.

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How to Cite This Article: Inaloo S, Katibeh P. Guillain-Barre Syndrome Presenting With Bilateral Facial Nerve Palsy. Iran J Child Neurol. 2014 Winter;8(1):69-71.

Objective
This case study is about an 11-year-old girl with bilateral facial weakness, abnormal taste sensation, and deep tendon reflexes of both knees and ankles were absent. However, the muscle power of the lower and upper extremities across all muscle groups was normal. After 2 days, she developed paresthesia and numbness in the lower extremities. Other neurologic examinations, such as fundoscopic evaluation of the retina were normal with the muscle power of both upper- and lower-extremities intact. A lumbar puncture revealed albumincytological dissociation. EMG and NCV were in favor of Guillain-Barre syndrome, for which IVIG was prescribed and the abnormal sensations in the lower limbs rapidly improved. Bilateral facial diplegia without weakness and paresthesia is a variant of Guillain-Barre syndrome that mostly presents with
acute onset, rapid progression with or without limb weakness, paresthesia, and decreased or absent DTR and albumin-cytological dissociation.

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