Cover page


Review Article


Neurologic Manifestations of Childhood Rheumatic Diseases

Reza SHIARI

Iranian Journal of Child Neurology, Vol. 6 No. 4 (2012), 2 October 2012, Page 1-7
https://doi.org/10.22037/ijcn.v6i4.3914

How to Cite this Article: Shiari R. Neurologic Manifestations of Childhood Rheumatic Diseases.  Iran
J Child Neurol Autumn 2012; 6(4): 1-7.

Children with rheumatic disorders may have a wide variety of clinical features ranging from fever or a simple arthritis to complex multisystem autoimmune diseases. Information about the prevalence of neurological manifestations in children with rheumatologic disorders is limited. This review describes the neurologic complications of childhood Rheumatic disease either solely or combined with symptoms of other organs involvement, as a primary manifestation or as a part of other symptoms, additionally.

 

References

1. Benseler S, Schneider R. Central nervous system vasculitisin children. Curr Opin Rheumatol. 2004 Jan;16(1):43-50.

2. Benseler SM. Central nervous system vasculitis in children. Curr Rheumatol Rep. 2006 Dec;8(6):442-9.

3. Mc Carthy HJ, Tizard EJ. Clinical practice: Diagnosis and management of Henoch-Schönlein purpura. Eur J Pediatr.2010 Jun;169(6):643-50. Epub 2009 Dec 12.

4. Robson WL, Leung AK. Henoch-Schönlein purpura. AdvPediatr. 1994;41:163-94.

5. Ostergaard JR, Storm K, Neurologic manifestations of Schönlein-Henoch purpura. Acta Paediatr Scand. 1991Mar;80(3):339-42.

6. Bakkaloğlu SA, Ekim M, Tümer N, Deda G, ErdenI, Erdem T. Cerebral vasculitis in Henoch-Schönlein purpura. Nephrol. Dial Transplant. 2000 Feb;15(2):246-8.

7. Mattoo TK, al-Mutair A, al-Khatib Y, Ali A, al-SohaibaniMO. Group A beta-haemolytic streptococcal infection and Henoch-Schonlein purpura with cardiac, renal and neurological complications. Ann Trop Pediatr. 1997Dec;17(4):381-6.

8. Ha TS, Cha SH. Cerebral vasculitis in Henoch-Schönlein purpura: a case with sequential magnetic resonance imaging. Pediatr Nephrol. 1996;10:634-6.

9. Saket S, Mojtahedzadeh S, Karimi A, Shiari R, ShirvaniF. Relationship between electrolyte abnormalities, ESR,CRP and platelet count with severity of Kawasaki disease.Yafteh. 2009;11(3):5-14.

10. Halimiasl A, Hosseini AH, Shiari R, Ghadamli P,Mojtahedzadeh S. Concomitant Coronary Artery Aneurysm and Myocarditis as a Rare Manifestation of Kawasaki Disease: A Case Report. J Compr Ped.2012;3(1):34-6.

11. Ichiyama T, Nishikawa M, Hayashi T, Koga M, Tashiro N, Furukawa S. Cerebral hypoperfusion during acute Kawasaki disease. Stroke. 1998 Jul;29(7):1320-1.

12. Muneuchi J, Kusuhara K, Kanaya Y, Ohno T, Furuno K,Kira R, Mihara F, Hara T. Magnetic resonance studies of brain lesions in patients with Kawasaki disease. Brain Dev. 2006 Jan;28(1):30-3.

13. Scolding NJ, Wilson H, Hohlfeld R, Polman C, Leite I,Gilhus N. The recognition, diagnosis and management of cerebral vasculitis: a European survey. Eur J Neurol. 2002Jul;9(4):343-7.

14. Guillevin L, Durand-Gasselin B, Cevallos R, Gayraud M, Lhote F, Callard P et al. Microscopic polyangiitis:clinical and laboratory findings in eighty-five patients.Arthritis Rheum. 1999 Mar;42(3):421-30.

15. Rahman A, Isenberg DA. Systemic lupus erythematosus.N Engl J Med. 2008 Feb;358(9):929-39.

16. Farivar S, Shiari R, Nejad Hosseinian M, Eshtad S. New genetic finding in systemic lupus erythematosus. Genet inthe 3rd Millen 2008;6(2):1333-8.

17. Sibbitt WL Jr, Brandt JR, Johnson CR, Maldonado ME,Patel SR, Ford CC et al. The incidence and prevalence of neuropsychiatric syndromes in pediatric onset systemic lupus erythematosus. J Rheumatol. 2002 Jul;29(7):1536-42.

18. Harel L, Sandborg C, Lee T, von Scheven E.Neuropsychiatric manifestations in pediatric systemic lupus erythematosus and association with antiphospholipid antibodies. J Rheumatol. 2006 Sep;33(9):1873-7.

19. Hawro T, Bogucki A, Sysa-Jedrzejowska A, BogaczewiczJ, Wozniacka A. [Neurological disorders in systemic lupus erythematosus patients]. Pol Merkur Lekarski. 2009 Jan;26(151):43-8.

20. Greenberg BM. The neurologic manifestations of systemic lupus erythematosus. Neurologist. 2009May;15(3):115-21.21. Sofat N, Malik O, Higgens CS. Neurological involvementin patients with rheumatic disease. QJM 2006 Feb;99(2):69-79.

22. Shiari R, Farivar S. Juvenile Systemic Lupus Erythematosus associated with Klinefelter’s syndrome: A case report. Reumatol Clin. 2010 Jul-Aug;6(4):212-3.

23. Yuan H, Ni JD, Pan HF, Li LH, Feng JB, Ye DQ. Lack of association of FcyRIIIb polymorphisms with systemic lupus erythematosus: a meta-analysis. Rheumatol Int.2011 Aug;31(8):1017-21.

24. Shiari R, Kobayashi I, Toita N, Hatano N, Kawamura N, Okano M et al. Epitope mapping of anti-alpha-fodrinauto antibody in juvenile Sjögren’s syndrome: differencein major epitopes between primary and secondary cases. J Rheumatol. 2006 Jul;33(7):1395-400.

25. Ozen S. Pediatric onset Behçet disease. Curr Opin Rheumatol. 2010 Sep;22(5):585-9.

26. Wadia N, Williams E. Behçet’s syndrome with neurological complications. Brain. 1957 Mar;80(1):59-71.

27. Ai-Araji A, Kidd DP. Neuro-Behçet’s disease:epidemiology, clinical characteristics, and management.Lancet Neurol. 2009 Feb;8(2):192-204.

28. Namer IJ, Karabudak R, Zileli T, Ruacan S, KücükaliT, Kansa E. Peripheral nervous systems involvement inBehçet’s disease. Case report and review of the literature.Eur Neurol. 1987;26(4):235-240.

29. Takeuchi A, Kodama M, Takatsu M, Hashimoto T,Miyashita H. Mononeuritis multiplex in incompleteBehçet’s disease a case report and the review of theliterature. Clin Rheumatol 1989 Sep;8(3):375-380.

30. Aksoyek S, Aytemir K, Ozer N, Ozcebe O, Oto A.Assessment of autonomic nervous system functionin patients with Behçet’s disease by spectral analysisof heart rate variability. J Auton Nerv Syst. 1999 Sep24;77(2):190-4.

31. Borhani-Haghighi A, Samangooie S, Ashjazadeh N,Nikseresht, A Shariat A, Yousefipour G et al. Neurological manifestations of Behçet’s disease. Neurosciences(Riyadh). 2006 Oct;11(4):260-4.

32. Ideguchi H, Suda A, Takeno M, Kirino Y, Ihata A,Ueda A et al. Neurological manifestations of Behçet’s disease in Japan: a study of 54 patients. J Neurol. 2010 Jun;257(6):1012-20. Epub 2010 Feb 3.

33. Cassidy JT, Levinson JE, Bass JC, Baum J, Brewer EJJr., Fink CW et al. A study of classification criteria fora diagnosis of juvenile rheumatoid arthritis. Arthritis Rheum. 1986 Feb;29(2):274-81.

34. Ravelli A, Martini A. Juvenile idiopathic arthritis.Lancet. 2007 Mar 3;369(9563):767-78.

35. Farivar S, Shiari R, Hadi E. Genetic susceptibility tojuvenile idiopathic arthritis in Iranian children. Arch Med Res. 2011 May;42(4):301-4.

36. Unal O, Ozçakar L, Cetin A, Kaymak B. Severe bilateral carpal tunnel syndrome in juvenile chronic arthritis.Pediatr Neurol. 2003 Oct;29(4):345-8.

37. Ueno H, Katamura K, Hattori H, Yamaguchi Y,Nakahata T. Acute lethal encephalopathy in systemic juvenile rheumatoid arthritis. Pediatr Neurol. 2002 Apr;26(4):315-7.

38. Duzova A, Bakkaloglu A. Central nervous system involvement in pediatric rheumatic diseases:current concepts in treatment. Curr Pharm Des.2008;14(13):1295-301.

39. Laiho K, Savolainen A, Kautiainen H, Kekki P, Kauppi M. The cervical spine in juvenile chronic arthritis. Spine J. 2002 Mar-Apr;2(2):89-94.

40. De Cunto CL, Liberatore DI, San Román JL, Goldberg JC, Morandi AA, Feldman G. Infantile-onset multisystem inflammatory disease: a differential diagnosis of systemic juvenile rheumatoid arthritis. J Pediatr. 1997 Apr;130(4):551-6.

41. Farivar S, Shiari R, Hadi E. Molecular analysis of MEFV gene in Iranian children with familial Mediterranean fever. Ind J Rheumato. 2010 Jun;5(2):66-8.

42. Kalyoncu U, Eker A, Oguz KK, Kurne A, Kalan I,Topcuoglu AM et al. Familial Mediterranean fever and central nervous system involvement: a case series. Medicine (Baltimore). 2010 Mar;89(2):75-84.

43. Radice A, Sinico RA. Antineutrophil cytoplasmic antibodies (ANCA). Autoimmunity. 2005 Feb;38(1):93-103.

44. Nishino H, Rubino FA, DeRemee RA, Swanson JW,Parisi JE. Neurological involvement in Wegener’s granulomatosis: an analysis of 324 consecutive patients at the Mayo Clinic. Ann Neurol. 1993 Jan;33(1):4-9.

45. Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA,Arend WP et al. The American College of Rheumatology1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis).Arthritis Rheum. 1990 Aug;33(8):1094-100.

46. Sehgal M, Swanson JW, DeRemee RA, Colby TV.Neurologic manifestations of Churg-Strauss syndrome.Mayo Clin Proc. 1995 Apr;70(4):337-41.

47. Twardowsky AO, Paz JA, Pastorino AC, Jacob CM,Marques-Dias MJ, Silva CA. Chorea in a child with Churg-Strauss syndrome. Acta Reumatol Port. 2010 Jan-Mar;35(1):72-5.

48. Kumar N, Vaish AK. Hemiplegia due to Churg Strauss syndrome in a young boy. J Assoc Physicians India.2011 Mar;59:172-3.

49. Pagnoux C, Seror R, Henegar C, Mahr A, Cohen P,Le Guern V et al. Clinical features and outcomes in 348 patients with polyarteritis nodosa: a systematic retrospective study of patients diagnosed between 1963 and 2005 and entered into the French Vasculitis Study Group Database. Arthritis Rheum. 2010 Feb;62(2):616-26.

50. Valeyrie L, Bachot N, Roujeau JC, Authier J, Gherardi R,Hosseini H. Neurological manifestations of polyarteritis nodosa associated with the antiphospholipid syndrome.Ann Med Interne (Paris). 2003 Nov;154(7):479-82.

51. Cellucci T, Benseler SM. Diagnosing central nervous system vasculitis in children. Curr Opin Pediatr. 2010 Dec;22(6):731-8.

52. Matsell DG, Keene DL, Jimenez C, Humphreys P. Isolated angiitis of the central nervous system in childhood. Can J Neurol Sci. 1990 May;17(2):151-4.

53. Calabrese LH, Furlan AJ, Gragg LA, Ropos TJ. Primary angiitis of the central nervous system: diagnostic criteria and clinical approach. Cleve Clin J Med. 1992 May- Jun;59(3):293-306.

54. Cekinmez EK, Cengiz N, Erol I, Kizilkilic O, Uslu Y. Unusual cause of acute neurologic deficit in childhood: primary central nervous system vasculitis presenting with basilar arterial occlusion. Childs Nerv Syst. 2009 Jan;25(1):133-6.

55. Benseler SM, Silverman E, Aviv RI, Schneider R, Armstrong D, Tyrrell PN. Primary central nervous system vasculitis in children. Arthritis Rheum. 2006 Apr;54(4):1291-7.

56. Yaari R, Anselm IA, Szer IS, Malicki DM, Nespeca MP, Gleeson JG. Childhood primary angiitis of the central nervous system: two biopsy-proven cases. J Pediatr.2004 Nov;145(5):693-7.

 

 

 

Research Article


Effects of Piracetam on Pediatric Breath Holding Spells: A Randomized Double Blind Controlled Trial

Ali ABBASKHANIAN, Sara EHTESHAMI, Sadegh SAJJADI, Mohammad Sadegh REZAI

Iranian Journal of Child Neurology, Vol. 6 No. 4 (2012), 2 October 2012, Page 9-15
https://doi.org/10.22037/ijcn.v6i4.3915

How to cite this article:Abbaskhanian A, Ehteshami S, Sajjadi S, Rezai MS. Effects of Piracetam on Pediatric Breath Holding Spells: A Randomized Double Blind Controlled Trial. Iran J Child Neurol Autumn 2012; 6(4): 9-15.

 

Abstarct:

Objective

Breath holding spells (BHS) are common paroxysmal non-epileptic eventsin the pediatric population which are very stressfull despite their harmlessnature. There has been no specific treatment found for the spells yet. The aimof this study was to evaluate the efficacy of piracetam (2-oxo-l-pyrrolidine)on these children.

Materials & Methods

In this randomized double blind clinical trial study, 150 children with severe BHS referred to our pediatric outpatient service were enrolled from August2011 to July 2012. The patients were randomized into two equal groups.One received 40mg/kg/day piracetam and the other group received placebo,twice daily. Patients were followed monthly for three months. The numberof attacks/month before and after treatment were documented.

Results

Of the enrolled patients, 86 were boys. The mean age of the patients was17 months (range, 6 to 24 months). In the piracetam group, 1 month after treatment an 81% response to treatment was found. In the placebo group,none of the patients had complete remission and 7% of the cases had partialremission. Overall, control of breath-holding spells was observed in 91%of the patients in the group taking piracetam as compared with 16% in the group taking placebo at the end of the study. There wasd nosignificant difference detected between the groups regarding the prevalenceof drug side effects.

Conclusion

A significant difference was detected between piracetam and placebo in prevention and controlling BHS. Piracetam (40mg/kg/day) had a good effecton our patients.

 

References

  1. Di Mario FJ Jr. Prospective study of children with cyanotic and pallid breath-holding spells. Pediatrics. 2001 Feb;107(2):265-9.
  2. Kotagal P, Costa M, Wyllie E, Wolgamuth B. Paroxysmal non epileptic events in children and adolescents. Pediatrics. 2002 Oct:110(4):e46.
  3. Kolkiran A, Tutar E, Atalay S, Deda G, Cin S. Autonomic nervous system functions in children with breath-holding spells and effects of iron deficiency. Acta Pediatric. 2005 Sep;94(9):1227-31.
  4. Hüdaoglu O, Dirik E, Yiş U, et al. Parental attitude of mothers, iron deficiency anemia, and breath-holding spells. Pediatr Neurol. 2006:Jul;35(1):18-20.
  5. Ahmad Bhat M, Ali W, Mohidin K, Sultana M. Prospective study of severe breath holding spells and role of iron. J Pediatr Neurol. 2007;5(1):27-32.
  6. Lombroso CT, Lerman P. Breath holding spells (cyanotic and pallid infantile syncope). Pediatrics. 1967 Apr;39(4):563-81.
  7. Gouliaev AH, Senning A. Piracetam and other structurally related nootropics. Brain Res Rev. 1994 May;19(20:180-222.
  8. Azam M, Bhatti N, Shahab N. Piracetam in severe breath holding spells. Int J Pschyiatry Med. 2008;38(2):195-201.
  9. Garg RK. Piracetam  for the treatment of breath holding spells. Indian Pediatrics.1998 Oct;35(10):1034-5.
  10. Donma MM. Clinical efficacy of piracetam in treatment of breath holding spells. Pediatr Neurol. 1998 Jan;18(1):41-5.
  11. Murata R, Matsuoka O, Hattori H, Kawawaki H, Nakajima S, Nakamura M et al. Efficacy of Kan-baku-taiso-to (TJ-72) on breath-holding spells in children. Am J Chin Med. 1988;16(3-4):155-8.
  12. Kelly AM, Porter CJ, Mc Goon MD, Espinosa RE, Osborn MJ, Hayes DL. Breath-holding spells associated with significant bradycardia: successful treatment with permanent pacemaker implantation. Pediatrics. 2001 Sep;108(3):698-702.
  13. McWilliam RC, Stephenson JB. Atropine treatment of reflex anoxic seizures. Arch Dis Child. 1984 May;59(5):473-5.
  14. Ashrafi MR, Mohammadi M, Shervin Badve R.        Efficacy of piracetam in treatment of breath-holding spells Iran J Pediatr. 2002;12(4):33-6.
  15. Daoud AS, Batieha A, al-Sheyyab M, Abuekteish F, Hijazi S. Effectiveness of iron therapy on breath-holding spells. J Pediatr. 1997 Apr;130(4):547-50.
  16. Ziaullah Nawaz S, Shah S, Talaat A. Iron deficiency anemia as a cause of breath holding spells. J Postgrad Med Instit. 2005;19(2):171-4.
  17. Di lanni M, Wilsher CR, Blank MS, Conners CK, Chase CH, Funkenstein HH et al. The effects of piracetam in children with dyslexia. J Clin Psychopharmacol. 1985 Oct;5(5):272-8.
  18. Wilsher CR, Bennett D, Chase CH, Conners CK, Dilanni M, Feagans L et al. Piracetam and dyslexia: effects on reading tests. J Clin Psychopharmacol. 1987 Aug;7(4):230-7.
  19. DiMario FJ Jr, Sarfarazi M. Family pedigree analysis of children with severe breath-holding spells. J Pediatr. 1997 Apr;130(4):647-51.
  20. Winnicka K, Tomasiak M, Bielawska A. Piracetam-an old drug with novel properties. Acta Pol Pharm. 2005 Sep-Oct:62(5):405-9.
  21. Winblad, B. Piracetam: a review of pharmacological properties and clinical uses. CNS drug rev. 2005 Summer:11(2):169-82.


Atypical Findings of Guillain-Barré Syndrome in Children

Parvaneh KARIMZADEH, Mohammadkazem BAKHSHANDEH BALI, Mohammad Mahdi Nasehi, Seyedeh Mohaddese Taheri Otaghsara, Mohammad Ghofrani

Iranian Journal of Child Neurology, Vol. 6 No. 4 (2012), 2 October 2012, Page 17-22
https://doi.org/10.22037/ijcn.v6i4.3916

How to cite this article: Karimzadeh P, Bakhshandeh Bali MK, Nasehi MM, Taheri Otaghsara SM, Ghofrani M. Atypical Findings of Guillain-Barré Syndrome in Children. Iran J Child Neurol Autumn 2012;6(4):17-22.

 

Abstract

Objective

Guillain-Barre syndrome (GBS) is an immune-mediated polyneuropathy that occurs mostly after  prior infection. The diagnosis of this syndrome is dependent heavily on the history and examination, although cerebrospinal fluid analysis and electrodiagnostic testing usually confirm the diagnosis. This is a retrospective study which was performed to investigate the atypical features of GBS.

Materials & Methods

Thirty three patients (21/63.6% males and 12/36.4% females) with GBS were retrospectively studied and prospectively evaluated at the Child Neurology institute of Mofid Children Hospital of Shahid Beheshti University of Medical Sciences between May 2011 and September 2012.

Results

The mean age was 5.4 years (range, 1.5-10.5).Twenty one patients (87.9 %) had previous history of infections. Eight patients (24.2%) admitted with atypical symptoms like upper limb weakness (3%), ptosis (3%), neck stiffness (3%), inability to stand (proximal weakness) (9.1%), headache (3%) and dysphagia (3%).According to disease process, weakness was ascending in 26 (78.8%), descending in 5 (15.2%) and static in 2 (6.1%) patients. Cranial nerve involvement was found in 8(24.3%) children, most commonly as facial palsy in 3 (9.1%).

Conclusion

In this study, 24.3% of our patients presented with atypical symptoms of GBS as upper limb weakness, ptosis, neck stiffness, inability to stand (proximal weakness), headache and dysphagia

 

References:

  1. Hughes RA, Cornblath DR. Guillain-Barré syndrome. Lancet. 2005 Nov 5;366(9497):1653-66.
  2. McGillicuddy DC, Walker O, Shapiro NI, Edlow JA. Guillain-Barré syndrome in the emergency department. Ann Emerg Med. 2006 Apr;47(4):390-3.
  3. Cosi V, Versino M. Guillain-Barré syndrome. Neurol Sci. 2006;27(Suppl 1):S47-51.
  4. Hughes RA, Cornblath DR. Guillain-Barré syndrome. Lancet. 2005 Nov;366(9497):1653-66.
  5. Barzegar M, Dastgiri S, Karegarmaher MH, Varshochiani A. Epidemiology of childhood Guillan Barre syndrome in the north west of Iran. BMC Neurol. 2007 Aug 5;7:22.
  6. Vriesendorp FJ, Mishu B, Blaser MJ, Koski CL. Serum antibodies to GM1, GD1b, peripheral nerve myelin, and Campylobacter jejuni in patients with Guillain-Barre syndrome and controls: correlation and prognosis. Ann Neurol. 1993 Aug;34(2):130-5.
  7. Asbury AK, Cornblath DR. Assessment of current diagnostic criteria for Guillain-Barre syndrome. Ann Neurol. 1990;27 Suppl:S21-4.
  8. Hughes RA, Cornblath DR. Guillain-Barre syndrome. Lancet. 2005 Nov;366(9497):1653-66.
  9. Govoni V, Granieri E. Epidemiology of the Guillain-Barre syndrome. Curr Opin Neurol. 2001 Oct;14(5):605-13.
  10. Gordon PH, Wilbourn AJ. Early electrodiagnostic findings in Guillain-Barré syndrome. Arch Neurol. 2001 Jun;58(6):913-7.
  11. Darabi K, Abdel-Wahab O, Dzik WH. Current usage of intravenous immune globulin and the rationale behind it: the Massachusetts General Hospital data and a review of the literature. Transfusion. 2006 May;46(5):741-53.
  12. Hughes RA, Raphaël JC, Swan AV, van Doorn PA. Intravenous immunoglobulin for Guillain-Barré syndrome. Cochrane Database Syst Rev. 2006 Jan 25;(1):CD002063.
  13. Hughes RA, Swan AV, van Koningsveld R, van Doorn PA. Corticosteroids for Guillain-Barré syndrome. Cochrane Database Syst Rev. 2006 Apr 19;(2):CD001446.
  14. Asbury AK, Cornblath DR. Assessment of Current Diagnostic Criteria for Guillain-Barre syndrome. Ann Neurol. 1990; 27 Suppl:S21-4.
  15. van Doorn PA, Ruts L, Jacobs BC. Clinical features, pathogenesis, and treatment of Guillain-Barré syndrome. Lancet Neurol. 2008 Oct;7(10):939-50.
  16. Levin KH. Variants and mimics of Guillain-Barré Syndrome. Neurologist. 2004 Mar;10(2):61-74.
  17. Winer JB. Guillain-Barré syndrome: clinical variants and their pathogenesis. J Neuroimmunol. 2011 Feb;231(1-2):70-2.
  18. Susuki K, Koga M, Hirata K, Isogai E, Yuki N. A Guillain-Barré syndrome variant with prominent facial diplegia. J Neurol. 2009 Nov;256(11):1899-905.
  19. Jin Park H, Hyang Lee K. Atypical Miller-Fisher Syndrome Presenting as an Isolated Internal Ophthalmoplegia Following Epstein-Barr Virus Infection. J Pediatr Care for review 2012 Mar; 20(1):39-42.
  20. Etem Pişkin I, Calık M, Yarımay G, Adresi Y. Neck stiffness in Guillaine-Barre syndrome subsequent to cytomegalovirus Infection. Dicle Med J. 2011;38(1):104-6.
  21. Koul R, Chacko A, Ahmed R, Varghese T, Javed H, Al-Lamki Z. Ten year prospective study (clinical spectrum) of childhood Guillain Barré syndrome in the Arabian peninsula: comparison of  outcome in patients in the pre and post intravenous immunoglobulin eras. J Child Neurol. 2003;18(11):767-71.
  22. Linden V, da Paz JA, Casella EB, Marques-Dias MJ. Guillain-Barré syndrome in children: clinic, laboratorial and epidemiologic study of 61 patients. Arq Neuropsiquiatr. 2010 Feb;68(1):12-7.
  23. Winer JB, Hughes RA, Anderson MJ, Jones DM, Kangro H, Watkins RP. A prospective study of acute idiopathic neuropathy: II antecedent events. J Neurol Neurosurg Psychiatry. 1988 May;51(5):613-18.
  24. Ho TW, Mishu B, Li CY, Gao CY, Cornblath DR, Griffin JW et al. Guillain-Barre syndrome in northern China. Relationship to Campylobacter jejuni infection and anti-glycolipid antibodies. Brain. 1995 Jun;118(Pt 3):597-605.
  25. Bahou YG, Biary N, al Deeb S. Guillain-Barre syndrome: a series observed at Riyadh Armed Forces Hospital. J Neurol. 1996 Feb;243(2):147-52.
  26. Cosi V, Versino M. Guillain-Barré syndrome. J Neurol Sci. 2006;27 (Suppl 1):S47-S51.
  27. Winer JB. Guillain Barré syndrome. Mol Pathol. 2001 Dec;54(6):381-5.
  28. The prognosis and main prognostic indicators of Guillain-Barré syndrome: A multicentre prospective study of 297 patients. The Italian Guillan-Barre Study Group. Brain. 1996 Dec;119(Pt 6):2053-61.


Efficacy of Levothyroxine in Migraine Headaches in Children with Subclinical Hypothyroidism

Mehrdad MIROULIAEI, Razieh FALLAH, Nasrollah BASHARDOOST, Mina PARTOVEE, Mahtab ORDOOEI

Iranian Journal of Child Neurology, Vol. 6 No. 4 (2012), 2 October 2012, Page 23-26
https://doi.org/10.22037/ijcn.v6i4.3917

How to Cite this article: Mirouliaie M, Fallah R, Partovee M, Ordooei M. Efficacy of Levothyroxine in Migraine Headaches in Children with Subclinical Hypothyroidism. Iran J Child Neurol Autumn 2012;6(4):23-26.

 

Abstract

Objective

Hypothyroidism may be an exacerbating factor for  primary headaches and migraine is one of the most common primary headaches in childhood. Thepurpose of this study was to evaluate the effect of treatment of subclinical hypothyroidism on children with migraine headache.

Materials & Methods

In a quasi-experimental study, the severity and monthly frequency of headache of 25 migraineur  children with subclinical hypothyroidism who were referred to the pediatric neurology clinic of Shahid Sadoughi University of Medical Sciences, Yazd, Iran between January 2010 and February 2011and were treated with levothyroxine for two months were evaluated.

Results

Thirteen girls (52%) and 12 boys (48%) with the mean age of 10.2 ± 2.76years were evaluated.In children with hypothyroidism, the monthly frequency of headache (mean± SD: 17.64 ± 9.49 times vs. 1.2 ± 1.1 times) and the severity of headache(mean± SD: 6.24±1.8 scores vs. 1.33 ± 0.87 scores) were significantly decreased by treatment.

Conclusion

Based on the results of this study, treatment of subclinical hypothyroidism was effective in reducing migraine headaches. Therefore, it is logical to check thyroid function tests in migraineur  children. 

References:

  1. Jan MM. Updated overview of pediatric headache and migraine. Saudi Med J. 2007 Sep;28(9):1324-9.
  2. Hershey AD. Headaches. In: Kliegman RM, Stanton BF, Schor NF, St. Geme JW, Behrman RE. Nelson Textbook of Pediatrics. 19th ed. Philadelphia: Saunders; 2011. p. 2039-42.
  3. Hershey AD. Current approaches to the diagnosis and management of paediatric migraine. Lancet Neurol. 2010 Feb;9(2):190-204.
  4. Bigal ME, Gladstone J. The metabolic headaches. Curr Pain Headache Rep. 2008 Aug;12(4):292-5.
  5. Tepper DE, Tepper SJ, Sheftell FD, Bigal ME. Headache attributed to hypothyroidism. Curr Pain Headache Rep. 2007 Aug;11(4):304-9.
  6. Toprak D, Demirkukan K, Ellidokuz H. Is it important to test thyroid function tests in migraineurs? TJFMPC. 2007;4:47-51.
  7. Hagen K, Bjøro T, Zwart JA, Vatten L, Stovner LJ, Bovim G. Low headache prevalence amongst women with high TSH values. Eur J Neurol. 2001 Nov;8(6):693-9.
  8. Oleson J. The International Classification of Headache Disorders: 2nd edition. Headache Classification Subcommittee of the International Headache Society. Cephalalgia 2004; 24 (Suppl) : 9-160.
  9. LaFranchi S. Disorders of the Thyroid Gland. In: Kliegman RM, Stanton BF, Schor NF, St. Geme JW, Behrman RE. Nelson Textbook of Pediatrics. 19th ed. Philadelphia: Saunders; 2011. p. 1894-908.
  10. Mavromichalis I, Anagnostopoulos D, Metaxas N, Papanastassiou E. Prevalence of migraine in schoolchildren and some clinical comparisons between migraine with and without aura. Headache. 1999 Nov-Dec;39(10):728-36.
  11. Zwart JA, Dyb G, Holmen TL, Stovner LJ, Sand T. The prevalence of migraine and tension-type headaches among adolescents in Norway. The Nord-Trøndelag Health Study (Head-HUNT-Youth), a large population-based epidemiological study. Cephalalgia. 2004 May;24(5):373-9.
  12. Bigal ME, Lipton RB, Winner P, Reed ML, Diamond S, Stewart WF. Migraine in adolescents: association with socioeconomic status and family history. Neurology. 2007 Jul;69(1):16-25.
  13. Singh SK. Prevalence of migraine in hypothyroidism. J Assoc Physicians India. 2002 Nov;50:1455-6.
  14. Moreau T, Manceau E, Giroud-Baleydier F, Dumas R, Giroud M. Headache in hypothyroidism. Prevalence and outcome under thyroid hormone therapy. Cephalalgia.1998 Dec;18(10):687-9.
  15. Iwasaki Y, Kinoshita M, Ikeda K, Takamiya K, Shiojima T. Thyroid function in patients with chronic headache. Int J Neurosci. 1991 Apr;57(3-4):263-7.
  16. Abend NS, Younkin D. Medical Causes of Headache in Children. Curr Pain Headache Rep. 2007 Oct;11(5):401-7.
  17. Abend NS, Younkin D, Lewis DW. Secondary headaches in children and adolescents. Semin Pediatr Neurol. 2010 Jun;17(2):123-33.

Iron Status and Febrile Seizure- A Case Control Study in Children Less Than 3 Years

Mansour SADEGHZADEH, Parisa KHOSHNEVIS ASL, Esrafil MAHBOUBI

Iranian Journal of Child Neurology, Vol. 6 No. 4 (2012), 2 October 2012, Page 27-31
https://doi.org/10.22037/ijcn.v6i4.3918

 

How to cite this article: 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 Autumn 2012; 6(4):27-31.

Abstract

Objective:

Febrile seizure is one of the most common neurological conditions of childhood. Several theories, such as iron deficiency anemia have been proposed as the pathogenesis of this condition. The aim of this study was to find the association between iron deficiency anemia and febrile seizures in children aged 6 months to 3 years admitted in Valie Asr hospital in Zanjan.

Materials &Methods

Hemoglobin (Hb), mean corpuscular volume (MCV), serum iron (SI), total iron binding capacity (TIBC) and SI/TIBC ratio were assessed in one hundred children with febrile seizures and compared to the values of one hundred healthy children presenting in a heath care center in the same period as the control group.

Results

A total of 6% of cases had iron deficiency anemia which was similar to the control group. In the case group SI/TIBC ratio below 12% was seen in 58% of children which was significantly higher than that of the control group (29%).

Conclusion

The results of this study suggest that although anemia was not common among febrile seizure patients, iron deficiency was more frequent in these patients.

 

References

  1. Bidabadi E, Mashouf M.. Association between iron deficiency anemia and first febrile convulsion: A case-control study. Seizure. 2009 Jan;18(5):347-51.
  2. Sadeghzadeh M, Khoshnevisasl P, Moussavinassab N, Koosha A, Norouzi M. The Relation Between Serum Zinc Level and Febrile Seizures in Children Admitted to Zanjan Valie-Asr Hospital. J Zanjan Uni Med Sci. 2011;19(74):17-24.
  3. Kumari PL, Nair MK, Nair SM, Kailas L, Geetha S. Iron deficiency as a risk factor for simple febrile seizures-a case control study. Indian Pediatr. 2012 Jan;49(1):17-9.
  4. Pisacane A, Sansone R, Impagliazzo N, Coppola A, Rolando P, D'Apuzzo A, Tregrossi C. Iron deficiency anaemia and febrile convulsions: case-control study in children under 2 years. BMJ. 1996 Aug;313(7053):343.
  5. Yadav D, Chandra J. Iron deficiency: beyond anemia. Indian J Pediatr. 2011 Jan;78(1):65-72.
  6. Sherjil A, us Saeed Z, Shehzad S, Amjad R. Iron deficiency anaemia-a risk factor for febrile seizures in children. J Ayub Med Coll Abbottabad. 2010 Jul-Sep;22(3):71-3.
  7. Erikson KM, Jones BC, Hess EJ, Zhang Q, Beard JL. Iron deficiency decreases dopamine D1 and D2 receptors in rat brain. Pharmacol Biochem Behav. 2001 Jul-Aug;69(3-4):409-18.
  8. Beard JL, Chen Q, Connor J, Jones BC. Altered monoamine metabolism in caudate-putamen of iron-deficient rats. Pharmacol Biochem Behav. 1994 Jul;48(3):621-4.
  9. Chen Q, Beard JL, Jones BC. Abnormal rat brain monoamine metabolism in iron deficiency anemia. J Nutr Biochem. 1995;6(9):486-93.
  10. Youdim MB, Ben-Shachar D, Yehuda S. Putative biological mechanisms of the effect of iron deficiency on brain biochemistry and behavior. Am J Clin Nutr. 1989 Sep;50(3 Suppl):607-15.
  11. Felt BT, Beard JL, Schallert T, Shao J, Aldridge JW, Connor JR et al. Persistent
  12. 12.  neurochemical and behavioral abnormalities in adulthood despite early iron supplementation for perinatal iron deficiency anemia in rats. Brain Behav Res. 2006 Aug;171(2):261-70.
  13. Beard JL, Felt B, Schallert T, Burhans M, Connor JR, Georgieff MK. Moderate iron deficiency in infancy: biology and behavior in young rats. Behav Brain Res. 2006 Jun 30;170(2):224-32.
  14. Beard JL, Connor JR. Iron status and neural functioning. Ann Rev Nutr. 2003;23:41-58.
  15. Johnston MV. Iron deficiency, febrile seizures and brain development. Indian Pediatr. 2012;49(16):13-4.
  16. Lozoff B, Georgieff MK. Iron deficiency and brain development. Semin Pediatr Neurol. 2006 Sep;13(3):158-65.
  17. 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 meta-analysis. PLoS One. 2010 Nov 16;5(11):e14001
  18. 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):39-42
  19. Momen AA, Hakimzadeh M. Case-control study of the relationship between anemia and febrile convulsion in children between 9 months to 5 years of age. Sci Med J Ahwaz Uni Med Sci. 2003;1(4):54-50.
  20. 19. Stoltzfus RJ, Mullany L, Black RE(2004). Iron deficiency anaemia. In: Ezzati M, Lopez AD, Rodgers A, et al. editors. Comparative quantification of health risks: global and regional burden of disease attributable to selected major risk factors. Geneva: World Health Organization; 2004. p. 163-209.
  21. Brotanek JM, Halterman JS, Auinger P, Flores G, Weitzman M. Iron deficiency, prolonged bottle-feeding, and racial/ethnic disparities in young children. Arch Pediatr Adolesc Med. 2005 Nov;159(11):1038-42.
  22. 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 May;48(4):420-6.
  23. 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 Jul;43(7):740-3.
  24. Naveed-ur-Rehman, Billoo AG. Association between iron deficiency anemia and febrile seizures. J Coll Physicians Surg Pak. 2005 Jun;15(6):338-40.
  25. Vaswani RK, Dharaskar PG, Kulkarni S, Ghosh K. Iron deficiency as a risk factor for first febrile seizure. Indian Pediatr. 2010 May;47(5):437-9.
  26. Ozaydin E, Arhan E, Cetinkaya B, Ozdel S, Değerliyurt A, Güven A et al. Differences in iron deficiency anemia and mean platelet volume between children with simple and complex febrile seizures. Seizure. 2012 Apr;21(3):211-4.
  27. Abdurrahman KN, Al-atrushi AM. The association between iron deficiency anemia and first febrile seizure: a case-control study. Duhok Med J. 2010;4(1):60-6.
  28. Amirsalari S, Keihani doust ZT, Ahmadi M, Sabouri A, Kavemanesh Z, Afsharpeyman SH et al. Relationship between iron deficiency anemia and febrile seizures. Iran J Child Neurol. 2010;4(1):27-30.
  29. Kobrinsky NL, Yager JY, Cheang MS, Yatscoff RW, Tenenbein M. Does iron deficiency raise the seizure threshold? J Child Neurol. 1995 Mar;10(2):105-9.
  30. Abbaskhanian A,Vahidshahi k, Parvinnejad N. The association between iron deficiency and the first episode of febrile seizure. J Babol Uni Med Sci.2009;11(3):32-6.

 

Febrile Seizure: Demographic Features and Causative Factors

Hamed ESMAILI GOURABI, Elham BIDABADI, Fatemeh CHERAGHALIPOUR, Yasaman AARABI, Fatemeh SALAMAT

Iranian Journal of Child Neurology, Vol. 6 No. 4 (2012), 2 October 2012, Page 33-37
https://doi.org/10.22037/ijcn.v6i4.3919

How to cite this article: Esmaili Gourabi H, Bidabadi E, Cheraghalipour  F, Aarabi  Y, Salamat F. Febrile Seizure: Demographic Features and Causative Factors. Iran J Child Neurol Autumn 2012; 6(4):33-37.

Abstract

Objective

Because of geographical and periodical variation, we prompted to determine the demographic features and causative factors for febrile seizure in Rasht.

Materials & Methods

In this cross-sectional study, all 6–month- to 6-year-old children with the diagnosis of febrile seizure admitted to 17 Shahrivar hospital in Rasht, from August, 2009 to August, 2010 were studied. Age, sex, family history of the disease, seizure types, body temperature upon admission and infectious causes of the fever were recorded. All statistical analysis was performed with SPSS software, version 16.

Results

Of the 214 children (mean age, 25.24±15.40 months), 124 were boys and 109 had a positive family history. Complex seizures were seen in 39 cases. In patients with a complex febrile seizure, 59% had the repetitive type, 20.5% had the focal type and 20.5% had more than 15 minutes duration of seizures. Most of the repetitive seizures (78.3%) occurred in patients under 2 years old; the difference between under and over 2-year-old patients was statistically significant (P=0.02). Study results did not show significant differences between the two genders for simple or complex seizures. The mean body temperature upon admission was 38.2±1.32◦C (38.31±0.82 degrees in boys and 38.04±1.78 in girls). Upper respiratory infections were seen in most patients (74.29%). All cases of lower respiratory infections were boys. There was a statistically significant difference between boys and girls in causes of fever.

Conclusion

Most of the children had a positive family history and the most common causative factor was upper respiratory infection.

 

References:

  1. Huang MC, Huang CC, Thomas K. Febrile convulsions: development and validation of a questionnaire to measure parental knowledge, attitudes, concerns and practices. J Formos Med Assoc. 2006 Jan;105(1):38-48.
  2. Vaswani RK, Dharaskar PG, Kulkarni S, Ghosh K. Iron deficiency as a risk factor for first febrile seizure. Indian Pediatr. 2010 May;47(5):437-9.
  3. Sadleir LG, Scheffer IE. Febrile seizures. BMJ. 2007 Feb;334(7588):307-11.
  4. Mohebbi MR, Holden KR, Butler IJ. FIRST: a practical approach to the causes and management of febrile seizures. J Child Neurol. 2008 Dec;23(12):1484-9.
  5. Salehi Omran M, Khalilian E, Mehdipour E et al. Febrile seizures in North Iranian children: Epidemiology and clinical feature. J Pediatr Neurol. 2008;6(1):39-42.
  6. Bidabadi E, Mashouf M. Association between iron deficiency anemia and first febrile convulsion. A case-control study. Seizure. 2009 Jun;18(5):347-51.
  7. Vahidnia F, Eskenazi B, Jewell N. Maternal smoking, alcohol drinking, and febrile convulsion. Seizure. 2008 Jun;17(4):320-6.
  8. Ashrafzade F, Hashemzadeh A, Malek A. Acute otitis Media in Children with Febrile Convulsion. Iran J Otorhinolaryngol. 2002;16(35):33-9.
  9. Millichap JJ, Gordon Millichap J. Methods of investigation and management of infections causing febrile seizures. Pediatr Neurol. 2008 Dec;39(6):381-6.
  10. Hosseini Nasab A, Dai pariz M, Alidousti K. Demographic characteristics and predisposing factors of febrile seizures in children admitted to Hospital No. 1 of Kerman University of Medical Sciences. J Med Counc Islam Repub Iran. 2006;24(2):107-12.
  11. Keller A, Saucier D, Sheerin A, Yager J. Febrile convulsions affect ultrasonic vocalizations in the rat pup. Epilepsy Behav. 2004 Oct;5(5):649-54.
  12. Ogihara M, Shirakawa S, Miyajima T, Takekuma K, Hoshika A. Diurnal variation in febrile convulsions. Pediatr Neurol. 2010 Jun;42(6):409-12.
  13. Fallah R, Akhavan S, Mir Sadat Nasseri F. Clinical and demographic characteristics of first febrile seizure in children. J Shaeed Sdoughi Uni Med Sci Yazd. 2009;16(5):61-5.
  14. Khodapanahande F, VahidHarandi N, Esmaeli F. Evaluation of seasonal variation and circadian rhythm of febrile seizures in children admitted to the  pediatric ward of Rasoul-e-Akram hospital. Razi J Med Sci. 2008;15(59):59-66.
  15. Hassanpour onje H, Ghofrani M, Taheri N. Risk factors of recurrent febrile seizures in children admitted to hospital with the children of Hazrat Ali Asghar. J Iran Uni Med Sci. 2006;16(65):46-54.
  16. Habib Z, Akram S, Ibrahim S, Hasan B. Febrile seizures: factors affecting risk of recurrence in Pakistani children presenting at the Aga Khan University Hospital. J Pak Med Assoc. 2003 Jan;53(11):11-7.
  17. Abaskhanian A, Vahid Shahi K, Parvinnejad N. The Association between Iron Deficiency and the First Episode of Febrile Seizure. J Babol Univ Med Sci. 2009;11(3):32-6.
  18. Mahyar A, Ayazi P, Fallahi M, Javadi A. Risk factors of the first febrile seizures in Iranian children. Int J Pediatr. 2010 2010:862897.
  19. Kolahi AA, Tahmooreszadeh S. First febrile convulsions: inquiry about the knowledge, attitudes and concerns of the patients’ mothers. Eur J Pediatr. 2009 Feb;168(2):167-71.
  20. Talebian A, Honarpishe A, Mohajeri S, et al. Risk factors associated with incidence of first febrile seizure in children. Faiz. 2003;7(2):55-8.
  21. Sanaee Dashty A, Akhlaghi AK, Pazoki R. Clinical risk factors of febrile seizure in children in a university hospital in Bushehr port. Iranian south medical journal (Teb-e-Jonoob). 2007;9(2):168-74.
  22. Golestan M, Fallah R, Akhavan S. Evaluation of CSF in 100 children admitted with febrile seizures. J Shaeed Sadoughi Uni Med Sci Yazd. 2009;16(5):3-7.

 

Effects of Stress on Mothers of Hospitalized Children in a Hospital in Iran

Tayebeh HASAN TEHRANI, Mohammad HAGHIGHI, Hasan BAZMAMOUN

Iranian Journal of Child Neurology, Vol. 6 No. 4 (2012), 2 October 2012, Page 39-45
https://doi.org/10.22037/ijcn.v6i4.3920

How to cite this article: Hasan Tehrani T, Haghighi M, Bazmamoun H. Effects of Stress on Mothers of Hospitalized Children in a Hospital in Iran. Iran J Child Neurol Autumn 2012;6(4):39-45.

Abstract

Objective

Hospitalization of a child can cause severe anxiety and stress in the parents, especially for the mother. This stress consequently affects the treatment course of the child. Hereby, we investigate the impact of different stressors in mothers of hospitalized children.

Materials & Methods

In this cross-sectional study, 225 mothers of hospitalized children in the pediatric ward of Besat hospital were randomly selected and studied. Data collection tool was a two-part questionnaire gathered by interviewing the mother. The first part included demographic information of the patients. The second part included questions regarding stressors in four different categories; child-related factors, environmental factors, socioeconomic factors and health professional factors. SPSS 16.5 was used for statistical analysis and data were analyzed by one way ANOVA and T test.

Results

In the child-related factor category, fear of child death (84%); in the socioeconomic factor category, fear of disease in the other siblings (84%); in the environmental factor category, unpleasant odors in the ward (56%); and in the health professional category, not enough explanation about inserting IV lines, (54.2%) constituted the most important factors.

There was a meaningful correlation between the stressors and the mothers’ age and occupation, child age, days of hospitalization, types of admission and health insurance coverage, but there was no meaningful correlation between stressors and other factors.

Conclusion

Professional and in depth training programs should be provided for health care providers and nursing staff regarding dealing with mothers of hospitalized children.

 

References:

  1. Marilyn JH, David W. Wong's essentials of pediatric nursing. 8th ed. Canada: Mosby; 2008. p. 659.
  2. Commodari E. Children staying in hospital: a research on psychological stress of caregivers. Ital J Pediatr. 2010 May 25;36:40.
  3. Burke SO, Handley-Derry MH, Costello EA, Kauffmann E, Dillon MC. Stress-point intervention for parents of children of repeatedly hospitalized children with chronic conditions. Res Nurs Health. 1997 Dec;20(6): 475-85.
  4. Kristensson-Hallstrom I. Parental participation in pediatric surgical care. AORN J. 2000 May;71(5): 1021-4, 1026-9.
  5. Garro A, Thurman SK, Kerwin ME, Ducette JP. Parent/caregiver stress during pediatric hospitalization for chronic feeding problems. J Pediatr Nurs. 2005 Aug;20(4):268-75.
  6. Shields L, Kristensson-Hallström  I. We have needs, too: parental needs during a child’s hospitalisation. Online Brazilian Journal of Nursing (OBJN-ISSN 1676-4285) [online] 2004  December; 3(3) Available in: www.uff.br/nepae/objn303shieldsetal.htm.
  7. Hallstrom I, Runesson I, Elander G. Obeserved parental needs during their child’s hospitalization. J Pediatr Nurs. 2002 Apr;17(2):140-8.
  8. Little L. Differences in stress and coping for mothers and fathers of childrens with Aspergers syndrome and nonverbal learning disorders. Pediatr Nurs. 2002 Nov-Dec;28(6):565-70.
  9. Soderback M, Christensson K. family involvement in the care of a hospitalized child. Int J Nurs Stud. 2008 Dec;45(12):1778-88.
  10. Mwangi R, Chandler C, Nasuwa F, Mbakilwa H, Poulsen A, Bygbjerg IC et al. Perceptions of mothers and hospital staff paediatric care in 13 public hospitals in northern Tanzania. Trans R Soc Trop Med Hyg. 2008 Aug;102(8):805-10.
  11. Chilman AM, Thomas M. Understanding nursing care. Third edition. Edinburgh: Churchill Livingstone; 1987. p. 636.
  12. Esmaeilzadeh H. Stressors of mothers of hospitalized neonates in Qods hospital JQUMS. 2003;6(4):40-5.
  13. Miles MS, Burchinal P, Holditch-Davis D, Brunssen S, Wilson SM. Perceptions of stress, worry and support in Black and White mothers of hospitalized, medically fragile infants. J Pediatr Nurs. 2002 Apr;17(2):82-8.
  14. Lam LW, Chang AM, Morrissey J. Parents’ experiences of participation in the care of hospitalized children: a qualitative study. Int J Nurs Stud. 2006 Jul;43(5):535-45.

 


 

 

Case Report


A Case of Schizencephaly and Septo-Optic Dysplasia Presenting with Anterior Encephalocele

Kaveh FADAKAR, Sahar DADKHAHFAR, Arash ESMAEILI, Zarrintaj KEYHANIDOUST

Iranian Journal of Child Neurology, Vol. 6 No. 4 (2012), 2 October 2012, Page 47-50
https://doi.org/10.22037/ijcn.v6i4.2599

How to cite this article: Fadakar K, Dadkhahfar S, Esmaeili A, Keyhanidoust Z. A Case of Schizencephaly and Septo-Optic Dysplasia Presenting with Anterior Encephalocele. Iran J Child Neurol Autumn 2012; 6(4):47-50.

 

Abstract

Schizencephaly is a rare central nervous system disorder with variable presentations. Here we report a patient with a huge bilateral schizencephaly and septo-optic dysplasia presenting with anterior encephalocele.

 

References:

  1. Yakovlev PI, Wadsworth RC. Schizencephalies; a study of the congenital clefts in the cerebral mantle; clefts with hydrocephalus and lips separated. J Neuropathol Exp Neurol. 1946 Jul;5(3):169-206.
  2. Yakovlev PI, Wadsworth RC. Schizencephalies; a study of the congenital clefts in the cerebral mantle; clefts with fused lips. J Neuropathol Exp Neurol. 1946 Apr;5:116-30.
  3. Brunelli S, Faiella A, Capra V, Nigro V, Simeone A,Cama A et al. Germline mutations in the homeobox gene EMX2 in patients with severe schizencephaly. Nat Genet. 1996 Jan;12(1):94-6.
  4. Montenegro MA, Guerreiro MM, Lopes-Cendes I,Guerreiro CA, Cendes F. Interrelationship of genetics and prenatal injury in the genesis of malformations of cortical development. Arch Neurol. 2002 Jul;59(7):1147-53.
  5. Granata T, Freri E, Caccia C, Setola V, Taroni F,Battaglia G. Schizencephaly: clinical spectrum, epilepsy, and pathogenesis. J Child Neurol. 2005 Apr;20(4):313-8.
  6. Lee HK, Kim JS, Hwang YM, Lee MJ, Choi CG, Suh DC et al. Location of the primary motor cortex in schizencephaly. AJNR Am J Neuroradiol. 1999 Jan;20(1):163-6.
  7. De Morsier G. [Studies on malformation of cranio-encephalic sutures. III. Agenesis of the septum lucidum with malformation of the optic tract]. Schweiz Arch Neurol Psychiatr. 1956;77(1):267-92.
  8. Hoyt WF, Kaplan SL, Grumbach MM, Glaser JS. Septo-optic dysplasia and pituitary dwarfism. Lancet. 1970 Apr;1(7652):893-4.
  9. Webb EA, Dattani MT. Septo-optic dysplasia. Eur J Hum Genet. 2010 Apr;18(4):393-7.
  10. Ellenberger C Jr, Runyan TE. Holoprosencephaly with hypoplasia of the optic nerves, dwarfism, and agenesis of the septum pellucidum. Am J Ophthalmol. 1970 Dec;70(6):960-7.

How to cite this article: Shakiba M, Nejad Biglari H, Alaee MR. Digital and Dental Malformation and Short Stature in a Patient with Neurological Problems: A Variant of the Oculodentodigital Dysplasia Syndrome or a New Syndrome?Iran J Child Neurol Autumn 2012; 6(4): 51-54.

 

Abstract

Several syndromes have been recognized with digital abnormality and CNS involvement such as oculodentodigital dysplasia (ODDD), Mohr syndrome and Joubert syndrome. We report a patient who was referred to us because of the neurological signs suspicious of metabolic disorders. This case was a 22-year-old woman whose problems began 4 years ago with shortening of memory, ataxia, abnormal gait and diplopia which progressed slowly. She consulted many neurologists and was on treatment with the suspicion of vasculitis, but no response was detected. She had severe short stature, hypoplasia of the middle and distal phalanges of the first, second and third fingers, clinodactyly, abnormal toes, abnormal enamel and missing teeth. She had no characteristic faces of ODDD and ophthalmological abnormality. Our patient might be a variant of ODDD or a new syndrome with somatic and neurologic signs.

References:

  1. Lohmann W, Beitrag zur Kenntnis des reinen Mikrophthalmus. Arch Augenheilkunde.1920;86:136-41.
  2. Berliner ML. Unilateral microphthalmia with congenital anterior synechiae and syndactyly. Arch Ophthalm. 1941;26:653-60.
  3. Bauer KH. Homoio transplantation von Epidermis bei eineiigen Zwillingen. Beitr Klin Chir. 1927;141:442-7.
  4. Pitter J, Svejda J. [The effect of x-rays as a cause of fetal misdevelopment]. Ophthalmologica. 1952 Jun;123(6):386-93.
  5. Judisch GF, Martin-Casals A, Hanson JW, Olin WH. Oculodentodigital dysplasia. Four new reports and a literature review. Arch Ophthalmol. 1979 May;97(5):878-84.
  6. Reardon W, Harbord MG, Hall-Craggs MA, Kendall B, Brett EM, Baraitser M. Central nervous system malformation in Mohr´s syndrome. J Med Genet. 1989 Oct;26(10):659-63.
  7. Ciliz D, Czturk S,Sakman B. Joubert syndrome case presentation. J Neurol Scie. (Turkish) 2010;27:214-8.
  8. Loddenkemper T, Grote K, Evers S, Oelerich M, Stogbauer F. Neurological manifestations of the oculodentodigital dysplasia syndrome. J Neurol. 2002 May;249(5):584-95.
  9. Aminabadi NA, Pourkazemi M, Oskouei SG, Jamali Z. Dental management of oculodentodigital dysplasia:a case report. J Oral Sci. 2010 Jun;52(2):337-42.