Iranian Journal of Child Neurology

How to Cite this Article: Bhadbhade A, Cheng DW. Amyloid Precursor Protein Processing in Alzheimer’s Disease. Iranian Journal of Child Neurology2012;6(1):1-5.

Alzheimer’s disease (AD) is a progressive neurodegenerative disorder and a leading cause of dementia. The AD is characterized by presence of intraneuronal tangles and extracellular plaques in the brain. The plaques are composed of dense and mostly insoluble deposits of amyloid beta peptide (Aβ), formed by sequential cleavage of the Amyloid Precursor Protein (APP), by two pathways amyloidogenic and non-amyloidogenic. Tangles are composed of paired helical fragments, which aggregate to form, microtubular protein tau. Although Aβ plaques are established to be the cause of the disease, there exist genetic factors and other pathological identifications in addition to these which are an integral part of the disease. This article gives an overview into the mechanism of APP action, genetic factors and other pathological identifications contributing to Alzheimer’s disease formation.

References

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2. Hebert LE, Scherr PA, Bienias JL, Bennett DA, Evans DA. Alzheimer disease in the US population. Arch Neurol 2003;60(8):1119-22.
3. Möller HJ, Graeber M. The case described by Alois Alzheimer in 1911. European Archives of Psychiatry and Clinical Neuroscience 1998:248(3):111-122.
4. Selkoe D J. (2002). Deciphering the genesis and fate of amyloid beta-protein yields novel therapies for Alzheimer disease. J Clinic Investigat 2002;110(10): 1375-82.
5. Wolfe MS. Tau mutations in neurodegenerative diseases. J Biolog Chem 2009;284(10):6021.
6. Selkoe DJ. Alzheimer’s disease: genes, proteins, and therapy. Physiological reviews 2001;81(2):741.
7. Selkoe DJ. The cell biology of [beta]-amyloid precursor protein and presenilin in Alzheimer’s disease. Trends in Cell Biology 1998;8(11):447-453.
8. Thinakaran G, Koo EH. Amyloid precursor protein trafficking, processing, and function. Journal of Biological Chemistry 2008;283(44):29615.
9. Zhang YW, Thompson R, Zhang H, Xu H. APP processing in Alzheimer’s disease. Mol Brain 2011;4:3. doi: 1756- 6606-4-3 [pii] 10.1186/1756-6606-4-3.
10. Nunan J, Small DH. Regulation of APP cleavage by α-,β- and γ-secretases. J Biolog Chem 2000:483(1):6-10.
11. Pearson HA, Peers C. Physiological roles for amyloid peptides. J Physiology 2006;575(1):5-10.
12. Wang Y, Ha Y. The X-ray structure of an antiparallel dimer of the human amyloid precursor protein E2 domain. Molecular Cell 2004;15(3):343-353.
13. Quitschke WW, Goldgaber D. The amyloid b-protein precursor promoter. Journal Biolog Chem 1992;267(24):17362-17368.
14. Vostrov AA, Taheny MJ, Izkhakov N, Quitschke WW. A nuclear factor-binding domain in the 5’-untranslated region of the amyloid precursor protein promoter: implications for the regulation of gene expression. BMC Research Notes 2010;3:4.
15. Ghosala K,Vogta D, Lianga M, Shenb Y, Lamba BT, Sanjay W, Pimplikara SW. Alzheimer’s disease-like pathological features in transgenic mice expressing the APP intracellular domain. Proceedings of National Academy of Sciences 2009;106(43):18367-77.

How to Cite this Article: Karimzadeh P, Ghazavi A. Comparison of Deflazacort and Prednisone in Duchenne Muscular Dystrophy. IranianJournal of Child Neurology 2012;6(1):5-12.

Objective

Duchenne muscular dystrophy (DMD) is a degenerative disease that usually becomes clinically detectable in childhood as progressive proximal weakness. No cure is yet available for DMD, but the use of steroids improves muscle strength and function. This study has been carried out to select the best steroid for the management of DMD.

Materials & Methods

This study is a single-blind, randomized clinical trial with a sample volume of 34 DMD patients. Half of these patients were treated with deflazacort (0.9 mg/kg daily) and the other half with prednisone (0.75 mg/kg daily) for a period of 18 months. The motor function score and excess body weight were registered one year after the start and also at the end of the study and compared between the two groups.

Results

Deflazacort was more effective in the improvement of motor function after one year, but there was no significant difference between the two drugs at the end of the study (18 months after start). Weight gain after one year and at the end of the study was higher in prednisone group and steroid treatment with deflazacort appears to cause fewer side effects than prednisone regarding weight gain.

Conclusion

Deflazacort seems to be more effective than prednisone in the improvement of motor function causing fewer side effects, particularly weight gain. This medication may be important for the improvement of motor function and could be used as the best steroidal treatment for Duchenne muscular dystrophy.

References

1. Lankester BJA, Whitehouse MR, Gargan MF. Duchenne muscular dystrophy. Current Orthopedics 2007;21:298- 300.
2. Wenger DR, Rang M. The art and practice of children’s orthopedics. Philadelphia, PA: Lippincott; Baltimore: Williams and Wilkins; 1993.
3. Sussman M. Duchenne muscular dystrophy. J Am Acad Orthop Surg 2002 Mar-Apr;10(2):138-51.
4. Escolar DM, Leshner RT. Muscular dystrophies. In: Swaiman KF, Ashwal S, Ferriero DM. Pediatric Neurology. 4th ed. Philadelphia: Mosby; 2006. p. 1967-86.
5. Sarnat HB, Menkes JH. Disease of the motor unit. In: Menkes JH, Sarnat HB, editors. Child Neurology. 7th ed. Philadelphia: Lippincott Williams & Wilkins; 2005. p. 984-9.
6. Biggar WD, Politano L, Harris VA, Passamano L, Vajsar J, Alman B, et al. Deflazacort in duchenne muscular dystrophy: a comparison of two different protocols. Neuromuscul disord 2004 Sep;14(8-9):476-82.
7. Radley HG, De Luca A, Lynch GS, Grounds MD. Duchenne muscular dystrophy: focus on pharmaceutical and nutritional interventions. Int J Biochem Cell Biol 2007;39(3):469-77.
8. Biggar WD, Klamut HJ, Demacio PC, Stevens DJ. Duchenne muscular dystrophy: current knowledge, treatment and future prospects. Clin Orthop Relat Res 2002 Aug;401:88-106.
9. Sohn RL, Gussoni E. Stem cell therapy for muscular dystrophy. Expert Opin Biol Ther 2004 Jan;4(1):1-9.
10. Houde S, Filiatrault M, Fournier A, Dube J, D’Arcy S, Berube D et al. Deflazacort use in duchenne muscular dystrophy: an 8-year follow-up. Pediatr Neurol 2008 Mar;38(3):200-6.
11. Bonifati MD, Ruzza G, Bonometto P, Berardinelli A, Gorni K, Orcesi K et al. A multicenter double-bind randomized trial of deflazacort versus prednisone in Duchenne muscular dystrophy: analysis after 2 years. Basic Appl Myol 2000;10:171-5.
12. Reitter B. Deflazacort vs. prednisone in Duchenne muscular dystrophy: trends of an ongoing study. Brain Dev 1995;17 Suppl:39-43.
13. Polgar G, Promadhat V. Standard values. In: pulmonary function testing in children: techniques and standards. Philadelphia: W.B. Saunders, 1971:102-3.
14. Biggar WD, Gingras M, Febling DL, Harris VA, Steele CA. Deflazacort treatment of Duchenne muscular dystrophy. J Pediatr 2001 Jan;138(1):45-50.

How to Cite this Article: Asadi-Pooya AA, Torabi-Nami M. Knowledge and Attitude Towards Epilepsy Among Biology Teachers in Fars Province, Iran. IranianJournal of Child Neurology 2012;6(1):13-18.

Objective

This study investigates the awareness and perception on “epilepsy” amongst biology teachers in Fars province, Iran.

Materials & Methods

A sample of high school biology teachers in Fars province, Iran, filled out an investigator designed questionnaire including questions about their knowledge and attitude concerning “epilepsy”. There were 17 questions in the questionnaire. Nine questions addressed the knowledge and the rest were about attitude and perception.

Results

Forty two teachers completed the questionnaires. More than two-thirds of the participants had a fairly desirable awareness about the definition; whereas, only approximately 40% knew something about the etiology and treatment of epilepsy. More than two-thirds of the participants had a positive attitude towards epilepsy; however, misconceptions and negative attitudes were observed.

Conclusion

Educational programs for biology teachers and also other teachers are necessary to improve their knowledge, attitude and perception about epilepsy.

References

1. Sander JW, Shorvon SD. Incidence and prevalence studies in epilepsy and their methodological problems: a review. J Neurol Neurosurg Psychiatry 1987;50:829-39.
2. Saraceno B. The WHO world health report 2001 on mental health. Epidemiol Psychiatr Soc 2002;11(2):83-7.
3. Kim MK, Cho KH, Shin J, Kim SJ. A study of public attitudes towards epilepsy in Kwang-Juarea. J Kor Neurol Assoc 1994;12:410-27.
4. DiIorio C, Shafer PO, Letz R, Henry T, Schomer DL, Yeager K, etal. The association of stigma with self-management and perception of health care among adults with epilepsy. Epilepsy Behav 2003;4(3):259-67.
5. Aziz H, Akhtar SW, Hasan KZ. Epilepsy in Pakistan: stigma and psychological problems: a population-based epidemiologic study. Epilepsia 1997;38:1069-73.
6. Ablon. The nature of stigma and medical conditions. Epilepsy Behav 2002;3:2-9.
7. Jacoby A. Stigma, epilepsy and quality of life. Epilepsy Behav 2002;3:10-20.
8. McLin WM, de Boer HM. Public perceptions about epilepsy. Epilepsia 1995;36:957-9.
9. Daoud A, Al-Safi S, Otoom S, Wahba L, Alkofahi A. Public knowledge and attitudes towards epilepsy in Jordan. Seizure 2007;16:521-6.
10. Ndour D, Diop AG, Ndiaye M, Niang C, Sarr MM, Ndiaye IP. A survey of school teachers’ knowledge and behaviour about epilepsy, in a developing country such as Senegal. [Article in French]. Aert Rev Neurol 2004;160(3):338-41.
11. Millogo A, Siranyan AS. Knowledge of epilepsy and attitudes towards the condition among school-teachers in Bobo-Dioulasso (Burkina Faso). Epileptic Disord 2004;6(1):21-6.
12. Masoudnia E. Awareness, understanding and attitudes towards epilepsy among Iranian ethnic groups. Seizure 2009;18(5):369-73.
13. Fernandes PT, Noronha AL, Araújo U, Cabral P, Pataro R, de Boer HM, Prilipko L, Sander JW, Li LM. Teachers’ perception about epilepsy. Arq Neuropsiquiatr 2007;65(1):28-34.
14. Aydin K, Yildiz H.Teacher’s perceptions in central turkey concerning epilepsy and asthma and short term effect of a brief education on the perception of epilepsy. Epilepsy Behav 2007;10:280-90.
15. Caixeta J, Frenandes PT, Bell GS, Sander JW, Li LM. Epilepsy perception amongst university students. A survey. Arq Neuropsiquiatr 2007;65 (suppl 1):43-8.

How to Cite this Article: Bani hashem A, Heydarian F, Gharavi M, Khoshnod M. Postdural Puncture Headache: Incidence and Risk Factors in Children FollowingIntrathecal Chemotherapy. Iranian Journal of Child Neurology 2012;6(1):19-22.

Objective

To evaluate the incidence and risk factors of development of postdural puncture headache in children who had intrathecal chemotherapy injection.

Materials & Methods

Two-hundred eighty patients (mean age, 7.23±3.92 years) who had intrathecal chemotherapy injection were studied prospectively during 2008-2009 in the pediatric ward of Dr. Sheikh hospital in Mashhad. Patients who had lumbar puncture for their chemotherapy drug injections were assessed daily for four days to detect postdural puncture headache.

Results

There were 172 (61, 4%) male patients and the remainder were female. Postdural puncture headache was detected in 41 patients (14.6%). The body mass index did not show any significant difference between the two groups. Lumbar puncture (LP) attempts had a significant association with postdural puncture headache. The size and shape of the needle did not have a significant association with postdural puncture headache.

Conclusion

LP attempts have a significant relationship with postdural puncture headache.

References:

1. Bolder PM. Postlumbar puncture headache in pediatriconcology patients. Anesthesiology. 1986;65:696-8.

2. Ozyalcin NS, Menda F. Regional anesthesia and analgesiaapplications in children and infants – II. Agri 2004;16:29-34, 37-42.

3. Oilver A. Dural punctures in children: what should wedo? Paediatr Anaesth. 2002;12:473-7.

4. Kokki H, Hendolin H, Turunen M. Postdural punctureheadache and transient neurologic symptoms in childrenafter spinal anaesthesia using cutting and pencil pointpaediatric spinal needles. Acta Anaesthesiol Scand1998;42:1076-82.

5. Lowery S, Oliver A. Incidence of postdural punctureheadache backache following diagnostic therapeuticlumbar puncture using a 22G cutting spinal needle, andafter introduction of a 25G pencil point spinal needle.Pediatr Anesth. 2008;18:230-4

6. Apiliogulari S, Duman A, Gok F, Akilliouglu L.Spinal needle design and size affect the incidence ofposdural puncture headache in children. Pediatr Anesth2010;20:177-82.

7. Kokki H, Heikkinen M, Turunen M. Needle design doesnot affect the success rate of spinal anaesthesia or theincidence of postpuncture complications in children. ActaAnaesthesiol Scand 2000;44:210-3.

8. Hammond ER, Wang Z, Bhulani N, McArthur JC, LevyM. Needle Type and the risk of post-lumber punctureheadache in the outpatient neurology. Clin J Neurol Sci;306(2011):24-8.

9. Lybecker H, Djernes M, Schimidt JF. Postdural punctureheadache (PDPH): onset, duration, severity and associatedsymptoms. An analysis of 75 consecutive patients withPDPH. Acta Anaesthesiol Scand 1995;39:605-12.

10. Seeberger MD, Kaufman M, Staender S, Schneider M,Scheidegger D. Repeated dural punctures increase theincidence of postdural puncture headache. Anesth Analg1996;82:302-5.

11. Thomas SR, Jamison DR, Muir KW. Randomizedcontrolled trial of atraumatic versus standard needles fordiagnostic lumbar puncture. BMJ 2000;3210:986-90.

12. Strupp M, Schueler O, Straube A. Atroumatic Sprotteneedle reduces the incidence of post-lumbar punctureheadaches. Neurology 2001;57:2310-2.

13. Kleyweg RP, Hetzberger LI, Carbat PA. Significantreduction in post-lumber puncture headache using an atraumatic needle. Cephalalgia 1998;18:635-7.

14. Lybecker H, Moller JT, May O. Incidence and predictionof postural puncture headache. A prospective study of1021 spinal Anesthesias. Anesth Analg 1990;70:389-94.15. Helper S, Preston R. Postdural puncture headache andspinal needle design. Meta analyses. Anesthesiology1994:81:1376-83.

16. Parker RK, White PF. A microscopic analysis of cutbevelversus pencil-point spinal needles. Anesth Analg1997;85:1101-4.

17. Reina MA, de leon Casasola OA, Lopez A. An in vitrostudy of dural lesions produced by 25-gauge Quinckeand Whitacre needles evaluated by scanning electronmicroscopy. Reg Anesth Pain Med 2000;393-402.

How to Cite this Article: Mahyar A, Ayazi P, Dalirani R, Bakhtiyari H, Daneshi Khohan MM, Javadi A. A Case-Control Study of the Association Between SerumCopper Level and Febrile Seizures in Children. Iranian Journal of Child Neurology 2012;6(1):23-28.

Objective

Febrile seizures are the most common cause of seizure in children. Identification of risk factors is very important. This study was conducted to determine the association between the serum copper level and simple febrile seizure in children.

Materials & Methods

In this study, 30 children with simple febrile seizures (case group) were compared with 30 children with febrile illness without seizures (control group) regarding serum copper level. This study was conducted in Qazvin children’s hospital (Qazvin, Iran).

Results

The mean serum copper levels in the case and control groups were 141.41±30.90 and 129.43±18.97 mcg/dl, respectively. This difference was not significant statistically.

Conclusion

This study revealed that there is no association between serum copper levels and febrile seizures. It seems that copper deficiency is not a risk factor for febrile seizures in children.

References

1. Mikati MA. Febrile seizures in: Kliegman RM, Stanton BF, GemeIII JWS, Schor NF, Behrman RE. Nelson textbookof pediatrics.19th edition. Philadelphia: Saunders; 2011.p. 2017-19.

2. Ferrie C, Newton R, Martland T. Febrile seizure in:Mclntosh N, Helms PJ, Smyth RL, Logan S. Forfar& Arneils textbook of pediatrics, London: ChurchillLivingstone; 2008. p. 860-1.

3. Mahyar A, Ayazi P, Fallahi M, Javadi A.Risk factors ofthe first febrile seizures in Iranian children. Int J Pediatr2010;2010:862897.[Epub 2010 Jun 24].

4. Siqueira LF. Febrile seizures: update on diagnosisand management. Rev Assoc Med Bras 2010 Jul-Aug;56(4):489-92.

5. Daoud A, Batieha A. Iron status a possible risk factor forthe first seizure. Epilepsy 2002;43(7):740-43.

6. Hartfield DS, Tan J, Yager JY, Rosychuk RJ, SpadyD, Haines C, et al. The association between irondeficiency and febrile seizures in childhood. Clin Pediatr(Phila) 2009;48(4):420-6.

7. Vaswani RK, Dharaskar PG, Kulkarni S, Ghosh K. Irondeficiency as a risk factor for first febrile seizure. IndianPediatr 2010;47(5):437-9.

8. Amiri M, Farzin L, Moassesi ME, Sajadi F. Serum traceelement levels in febrile convulsion. Biol Trace Elem Res2010;135(1-3):38-44.

9. Ganesh R, Janakiraman L, Meenakshi B. Serum zinclevels are low in children with simple febrile seizurescompared with those in children with epileptic seizuresand controls. Ann Trop Paediatr 2011;31(4):345-9.

10. Mahyar A, Ayazi P, Fallahi M, Javadi A.Correlationbetween serum selenium level and febrile seizures. PediatrNeurol 2010;43(5):331-4.

11. Anderson JB. Copper in: Mahan KL, Stump SE. Krause,sFood, Nutrition,& Diet Therapy 9th ed, Phila, Saunders;2004:150-4.

12. Gaggelli E, Kozlowski H, Valensin G. Copperhomeostasis and neurodegenerative disorders. Chem Rev2006;106:1995-2044.

13. Lazarchick J. Update on anemia and neutropenia incopper deficiency. Curr Opin Hematol 2012 ;19(1):58-60.

14. Zatta P, Frank A. Copper deficiency and neurologicaldisorders in man and animals, Brain Res Rev2006;54(1):19-23.

15. Tapiero H, Townsend DM, Tew KD. Trace elementsin human physiology and pathology. Copper. BiomedPharmacother 2003;57(9):386-98.

16. Prasad R, Singh A, Das BK, Upadhyay RS,Singh TB,Mishra OP. Cerebrospinal Fluid And Serum Zinc, Copper,Magnesium And Calcium Levels In Children WithIdiopathic Seizure. JCDR 2009;3(6):1841-6.

17. Sholomo S. Febrile seizures In: Swaiman KF, Ashwal S,Ferriero DM. Pediatric neurology: principles and practice.4th ed. Philadelphia: Mosby; 2006. p. 1079-86.

18. Ashrafi MR, Shabanian R, Abbaskhanian A, NasirianA, Ghofrani M, Mohammadi M, et al. Selenium andintractable epilepsy: is there any correlation? PediatrNeurol 2007;36(1):25-9.

19. Shenkin A, Baines M, Fell GS, Lyon TDG. Vitaminsand Trace Elements In: Burtis CA, Ashwood ER, BrunsDE. Tietz textbook of clinical chemistry and moleculardiagnostics. 4th ed. Phila: WB Saunders, 2006:1126-30.

20. Mishra OP, Singhal D, Upadhyay RS, Prasad R, etal. Cerebrospinal fluid zinc, magnesium, copper andgamma-aminobutyric acid levels in febrile seizures. JPN2007;5(1):39-44.

21. Wu J, Ricker M, Muench J. Copper deficiency as causeof unexplained hematologic and neurologic deficits inpatient with prior gastrointestinal surgery.J Am BoardFam Med 2006;19(2):191-4.

22. Ilhan A, Özerol E, Güleç M, Bünyamin Isik B, IlhanN, Ilhan N, et al. The comparison of nail and serumtrace elements in patients with epilepsy and healthysubjects . Prog Neuropsychopharmacol Biol Psychiatry2004;289(1):99-104.

How to Cite this Article: Soltanifar A, Ashrafzadeh F, Mohareri F, Mokhber N. Depression and Anxiety in Iranian Mothers ofChildren with Epilepsy. Iranian Journal of Child Neurology 2012;6(1):29-34.

Objective

Epilepsy is a common neurological disorder in children. Parents with epileptic children have many psychosocial care needs. So the main goal of this study was to evaluate depression and anxiety in Iranian mothers with epileptic children.

Materials & Methods

We identified 30 mothers of children with epilepsy and 30 mothers of children without epilepsy with children aged between 8 and 12 years who met the study criteria. In all children with epilepsy, the mothers were the main caregivers and all these children lived in two-parent families. Children in the control group were in the same age. Ninety-eight percent of children in the control group lived in two-parent families with the mother as the main caregiver. All mothers fulfilled the Beck Depression Inventory (BDI) and Spielberger State-Trait Anxiety Inventory.

Results

According to these data, BDI scores were significantly higher in the mothers of epileptic children (mean of Beck score=16.5) compared to the control group (mean of Beck score=9.8). The total, Spielberger State-Trait Anxiety Inventory scores for mothers of children with epilepsy were 100.3, 51.7 and 48.6. However, these scores in the control group were 86.9, 45.1 and 41.8. These differences were statistically significant.In a second analysis, using the demographic data, we did not find any statistically significant relation between anxiety or depression and the mothers’ job, children’s medication and other demographic variables.

Conclusion

Neurologists and psychiatrists need to develop better programs for adequate management of psychiatric disorders in mothers with epileptic children.

References

1. Cowan LD. The epidemiology of the epilepsies inchildren. Ment Retard Dev Disabil Res Rev 2002;8:171-81.

2. Schiariti V, Farrell K, Hoube JS, Lisonkova S. Periodprevalence of epilepsy in children in BC: a population-basedstudy. Can J Neurol Sci 2009 Jan;36(1):36-41.

3. Otero S. Psychopathology and psychological adjustmentin children and adolescents with epilepsy. World J Pediatr2009 Feb;5(1):12-7.

4. Rodenburg HR, Stams GJJM, Meijer AM, Aldenkamp AP,Dekovic´ M. Psychopathology in children with epilepsy:a metaanalysis. J Pediatr Psychol 2005 Sep;30(6):453-68.Epub 2005 Mar 3.

5. Rodenburg R, Meijer AM, Dekovic M, AldenkampAP. Family factors and psychopathology in childrenwith epilepsy: a literature review. Epilepsy Behav 2005Jun;6(4):488-503.

6. Lovejoy M, Graczyk PA, O_Hare E, Neuman G. Maternaldepression and parenting behavior: a meta-analytic reviewClin Psychol Rev 2000;20:561-92.

7. Shore CP, Buelow JM, Austin JK, Johnson CS.Continuing psychosocial care needs in children with newonsetepilepsy and their parents. J Neurosci Nurs 2009Oct;41(5):244-50.

8. Pianta RC, Lothman DJ. Predicting behavior problemsin children with epilepsy: child factors, disease factors,family stress, and child-mother interaction. Child Dev1994 Oct;65(5):1415-28.

9. Dunn DW, Austin JK, Huster GA. Symptoms ofdepression in adolescents with epilepsy. J Am Acad ChildAdolesc Psychiatr 1999;38:1133-8.

10. Shore CP, Austin JK, Huster GA, Dunn DW. Identifyingrisk factors for maternal depression in families ofadolescents with epilepsy. J Specialists Pediatr Nurs2002;7:71-80.

11. Yongli, Cheng-Ye ji, Jiong Qin, Zhi-Xiang Zhang.Parental anxiety and quality of Life of epileptic children.Biomed Environ Sci 2008 Jun;21(3):228-32.

12. Williams J, Steel C, Sharp GB, DelosReyes E, PhillipsT, Bates S, et al. Parental anxiety and quality of life inchildren with epilepsy. Epilepsy Behav 2003;4:483-6.

13. Lv R, Wu L, Jin L, Lu Q, Wang M, Qu Y, Liu H. Depression,anxiety and quality of life in parents of children withepilepsy. Acta Neurol Scand 2009 Nov;120(5):335-41.

14. Baki O, Erdogan A, Kantarci O, Akisik G, KayaalpL, Yalcinkaya C. Anxiety and depression in childrenwith epilepsy and their mothers. Epilepsy Behav 2004Dec;5(6):958-64.

15. Yam WK, Ronen GM, Cherk SW, Rosenbaum P, ChanKY, Streiner DL, et al. Health-related quality of lifeof children with epilepsy in Hong Kong: how does itcompare with that of youth with epilepsy in Canada?Epilepsy Behav 2008 Apr;12(3):419-26.

16. Ghassemzadeh H, Mojtabai R, Karamghadiri N,Ebrahimkhani N. Psychometric properties of a Persianlanguageversion of the Beck Depression Inventory Second edition: BDI-II-PERSIAN. Depress Anxiety2005;21(4):185-92.

17. Hojat M, Shapurian R, Mehryar AH. Psychometricproperties of a Persian version of the short form of theBeck Depression Inventory for Iranian college students.Psychol Rep 1986 Aug;59(1):331-8.

18. Kalkhoran MA, Karimollahi M. Religiousness andpreoperative anxiety: a correlational study. Ann GenPsychiatry 2007;6:17.

19. Mu PF, Kuo HC, Chang KP. Boundary ambiguity, copingpatterns and depression in mothers caring for children withepilepsy in Taiwan. Int J Nurs Stud 2005 Mar;42(3):273-82.

20. Lv R, Wu L, Jin L, Lu Q, Wang M, Qu Y, et al. Depression,anxiety and quality of life in parents of children withepilepsy. Acta Neurol Scand 2009 Nov;120(5):335-41.

21. (Wood LJ, Sherman E, Hamiwka LD, Blackman M,Wirrell E. Depression, anxiety, and quality of life insiblings of children with intractable epilepsy. EpilepsyBehav 2008 Jul;13(1):144-8.

22. Tosun A, Gokcen S, Ozbaran B, Serdaroglu G, Polat M,Tekgul H, et al. The effect of depression on academicachievement in children with epilepsy. Epilepsy Behav2008 Oct;13(3):494-8.

23. Rodenburg R, Meijer AM, Dekovic M, Aldenkamp AP. Family factors and psychopathology in childrenwith epilepsy: a literature review. Epilepsy Behav 2005Jun;6(4):488-503.

24. Wirrell EC, Wood L, Hamiwka LD, Sherman EM. Parenting stress in mothers of children with intractableepilepsy. Epilepsy Behav 2008 Jul;13(1):169-73.

How to Cite this Article: Fayyazi A, Khajeh A, Bagheri M, Ahmadi S. Acute Hydrocephaly Following Methadone Intoxication in a Child. Iranian Journal of ChildNeurology 2012;6(1):35-38.

Infantile methadone intoxication has been on the rise since the usage of methadone in opioid detoxification programs. We report a 30-month-old child with encephalopathy and acute hydrocephaly following methadone intoxication.

References:

1. Nazari H. Clinical approach to methadone toxication.Quarterly journal of Addiction 2007;2:18-20.

2. Plummer JL, Gourlay GK, Cherry DA, Cousins MJ.Estimation of methadone clearance: application in themanagement of cancer pain. Pain 1988 Jun;33(3):313-22.

3. Davies D, DeVlaming D, Haines C. Methadoneanalgesia for children with advanced cancer. PediatrBlood Cancer 2008 Sep;51(3):393-7.

4. Riascos R, Kumfa P, Rojas R, Cuellar H, Descartes F.Fatal methadone intoxication in a child. Emerg Radiol2008 Jan;15(1):67-70.

5. Binchy JM, Molyneux EM, Manning J. Accidental ingestion of methadone by children in Merseyside.BMJ 1994 May 21;308(6940):1335-6.

6. Li L, Levine B, Smialek JE. Fatal methadone poisoningin children: Maryland 1992-1996. Subst Use Misuse2000 Aug;35(9):1141-8.

7. Milroy CM, Forrest AR. Methadone deaths: atoxicological analysis. J Clin Pathol 2000 Apr;53(4):277-81.

8. Afzali S, Jafari MR. One year study of chest X-raychanges in opiate-poisoned patients in Hamadan. JQom Uni Med Sci 2010; 4(2):3-7.

9. Zamani N, Sanaei-Zadeh H, Mostafazadeh B. Hallmarksof opium poisoning in infants and toddlers. Trop Doct2010 Oct; 40(4):220-2.

10. Besharat S, Besharat M, Akhavan Masouleh A, JabbariA, Yazdi HR. Opium intoxication in children under 5years old, Golestan- Iran (2006-07). J Gorgan Uni MedSci Spring 2010;12(1):85-9.

11. Izadi Mood N, Gheshlaghi F, Sharafi SE. Fatalpoisoning cases admitted to the emergency departmentof poisoning, Noor Hospital, Isfahan. J Legal Med IslRep Iran 2003;9(31):122-26.

12. Boushehri B, Yekta Z, Zareei-Kheirabad A, Kabiri SH,Kazempour A. Determining the frequency of poisoningwith chemical agents and drugs in hospitalized patientsof Taleghani Hospital Uromieh, 2001-2004. J LegalMed Isl Rep Iran2004;10(35):126-131.

13. Cheraghali F, Taymori M. Epidemiological study ofdrug intoxication in children. Acta Medica Iranica2006;44(1):37-40.

14. Iran Ministry of Health report: some case reports ofoccasional methadone toxication in infants. QuarterlyJ Addiction 2007;2:26.

15. Geibprasert S, Gallucci M, Krings T. Addictiveillegal drugs: structural neuroimaging. AJNR Am JNeuroradiol 2010 May;31(5):803-8.

16. Malloy S, Soh C, Williams TL. Reversible delayed posthypoxic leukoencephalopathy. AJNR Am J Neuroradiol2006 Sep;27(8):1763-5.

17. Glatstein M, Finkelstein Y, Scolnik D. Accidentalmethadone ingestion in an infant: case report andreview of the literature. Pediatr Emerg Care 2009Feb;25(2):109-11.

How to Cite this Article: Ravi A, Veerabhadra D, Richa G, Ngangbam S. A Rare Occurrence of Shagreen Patch on the Face of aPediatric Patient with Tuberous Sclerosis. Iranian Journal of Child Neurology 2012;6(1):39-42.

Tuberous sclerosis is a neurocutaneous syndrome characterized by a clinical spectrum varying from severe mental retardation and incapacitating seizures to normal intelligence and lack of seizures, often within the same family(1). The younger the patients present with symptoms and signs of TSC (tuberous sclerosis complex), the greater are the likelihood of mental retardation (2). Skin features are very characteristic of the disease and help in the early diagnosis of the disease. Shagreen patch is one of the major diagnostic features of the disease. It is a less common skin lesion consisting of an excess amount of fibrous tissue similar to that found in scar tissues (3). It is a section of thickened, elevated pebbly skin like an orange peel. The name is derived from the French phrase “peau chagrinee”. It is usually found on the lower back, buttock, thigh and the nape of the neck commonly. In this case report we report a characteristic skin lesion, the shagreen patch in a six-year-old child with TSC at an unusual site (the cheek) never reported before with the best of our knowledge.

References:

1. Datta AK, Mandal S, Bhattacharya S. Autism and mentalretardation with convulsion in tuberous sclerosis: a casereport. Cases J 2009;2:7061.

2. Haslam RHA. Neurocutaneous syndrome. In KliegmanRM, Behrman RE, Jenson HB, Stanton BF (editors).Nelson Text book of Pediatrics,18th Edition. NewDelhi: Elsevier. 2008.P. 2485-6.

3. Mallory SB (editor). Illustrated manual of pediatricdermatology, diagnosis and management, 1st Edition.UK:Taylor & Francis. 2005.P. 369-89.

4. Roach ES, DiMario FJ, Kandt RS, Northrup H. TuberousSclerosis Consensus Conference: recommendationsfor diagnostic evaluation. National Tuberous SclerosisAssociation. J Child Neurol 1999 Jun;14(6):401-7.

5. Roach ES, Gomez MR, Northrup H. Tuberous sclerosiscomplex consensus conference: revised clinicaldiagnostic criteria. J Child Neurol 1998 Dec;13(12):624-8.

6. Raymond SK. Tuberous sclerosis complex andneurofibromatosis type 1: the two most commonneurocutaneous diseases. Neurol Clin N Am2002:20:941-64.

7. Prabhu S, Mahesh KP. Tuberous sclerosis with oralangiofibroma: Case report. Br J Oral Maxillofac Surg2010 Apr;48(3):205-7.

8. Hake S. Cutaneous manifestation of tuberous sclerosis.Ochsner J 2010 Fall;10(3):200-4.

How to Cite this Article: Ravi Torkaman M, Amirsalari S, Saburi A. Thyroid Function Test Imbalance in Epileptic ChildrenUnder Anticonvulsive Therapy. Iranian Journal of Child Neurology 2012;6(1):43-44.

Dear Editor,

There have been many studies regarding the impact of antiepileptic drugs(AEDs) on thyroid function. There are some challenging scopes which must beconsidered for conducting the study adressing the focused question. “Which oneof the thyroid hormones is related to the AEDs consumption?”. Some studiesdemonstrated that there may be alterations in all thyroid function tests (T3, T4 andTSH) after antiepileptic therapy in children (1). Some studies concluded that longtermprescription of anticonvulsive medications resulted in a decline in serum T4levels, although it had no effect on serum TSH levels. However, changes in serumT3 level was challenging and it must be investigated further (2).There were some confounding factors which may interfere with the conclusion.One of them is the type of the study. There are various study plans for this purposesuch as cross-sectional, case-control, experimental, self-controlled cohort anddouble-blind randomized clinical trial studies. It seems that the proper protocol ofstudy for this propose is a double-blind randomized clinical trial study. By usingother designs, the authors cannot interpret the effect of AEDs on thyroid function;however, they can discuss the prevalence of thyroid hormone imbalance and thecoordination among T3, T4 and TSH.Moreover, one of the confounding factors is the thyroid binding globulin (TBG)effect. It has appeared that some of the AEDs may change the amount of TBGand in this way may affect the amount of thyroid hormones (3). Clonazepamand valproic acid do not have any enzyme inducing effects, but phenobarbital,carbamazepine, phenytoin and primidone may induce the hepatic enzyme (4-6). Therefore, it seems necessary to analyze each group of patients based on thetype of drug which is prescribed and also by using the free amount of thyroidhormones, the researcher will be able to exclude the TBG effect. In addition, ageis an important factor which should be considered. The epileptic patients showextra-thyroid adverse effects such as vitamin D and bone metabolism disordersdue to antiepileptic drugs (7). Other secondary clinical disorders which interactwith the thyroid metabolism should be considered and overruled in the study withthe mentioned proposal, especially in the older population.It appears that the confounding effect of the duration of AED intake mustbe adjusted regarding the children’s age using multivariate analysis such asregression model or partial correlation test. The etiological mechanism of serumconcentrations of thyroid hormone change by AEDs has not been clarified clearlyand besides, patients with a personal or family history of thyroid disorders mayprogress to overt thyroid hormone imbalance secondary to AED consumption (6). Therefore, it is suggested that the pure etiologicalmechanism of thyroid disorders due to AEDs shouldbe studied in such cases. Previous reports suggestedthat return to normal of all parameters was observedafter withdrawal of anticonvulsive therapy and thisreversibility of the thyroid hormone imbalance may bea clue for further investigations in order to study thepatho-physiologic mechanism of this disorder (6).Recently, new pharmaceutical drugs have beensuccessfully used for epileptic patients. It is expectedthat the therapeutic role of these new medications willbecome more prominent in these patients in the futureand future studies should be focused on their adverseeffects.

Disclosures: None.

References:

1. Talebian A, Eslamian MR, Shiasi K, Moravveji A,Khodayari M, Abedi AR. Changing in thyroid functiontest in children underwent antiepileptic therapy. Iran JChild Neurol 2010;4(1):17-22.

2. Mahyar A, Ayazi P, Dalirani R, Hosseini SM, DaneshiKohan MM. Serum thyroid hormone levels in epilepticchildren receiving ant-convulsive drugs. Iran J ChildNeurol 2011;5(4):21-4.

3. Eirís-Puñal J, Del Río-Garma M, Del Río-Garma MC,Lojo-Rocamonde S, Novo-Rodríguez I, Castro-GagoM. Long-term treatment of children with epilepsy withvalproate or carbamazepine may cause subclinicalhypothyroidism. Epilepsia 1999;40(12):1761-6.

4. Kirimi E, Karasalihoglu S, Boz A. Thyroid functions inchildren under long-term administration of antiepilepticdrugs. Eastern J Med 1999;4(1):23-6.

5. Amirsalari S, Kayhanidost ZT, Kavemanesh Z, Torkman M, Beiraghdar F, Teimoori M, et al. Thyroid functionin epileptic children who receive carbamazepine,primidone, phenobarbital and valproic acid. Iranian JChild Neurol 2011;5(2):15-20.

6. Verrotti A, Di corcia G, Trotta D, Chiarelli F. Thyroidfunction in children treated with antiepilepticdrugs: effects of treatment withdrawal. Ital J Pediatr 2003;29:242-6.

7. Pack AM, Morrell MJ, McMahon DJ, Shane E. Normal vitamin D and low free estradiol levels in women onenzyme-inducing antiepileptic drugs. Epilepsy Behav 2011;21(4):453-8.