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>Journals >Acta Pediátrica de México >Year 2012, Issue 5

Mata-Pérez K, Teja-Ángeles E, Durán-Gutiérrez LA
Oral rehabilitation under general anesthesia in a boy with Hallervorden-Spatz syndrome. Report of a case
Acta Pediatr Mex 2012; 33 (5)

Language: Español
References: 25
Page: 227-231
PDF: 208.05 Kb.

Full text


Hallervorden-Spatz syndrome is a rare neuro-degenerative disorder characterized by high levels of iron deposits in the brain. Patients exhibit extrapyramidal and cognitive impairment and dystonia, seizures, spasticity, dysarthria, rigidity of limbs and visual disturbances. We report a case of a patient with this syndrome, who had multiple carious lesions and self-inflicted injuries of the mucosa of the lower lip and tongue, produced during periods of intense orofacial spasms. Oral rehabilitation was performed under general anesthesia. Dental treatment was given to promote dental hygiene, pain relief, and elimination of infections.

Key words: Hallervorden-Spatz syndrome, neurodegenerative disease, orofacial spasm, self-inflicted injuries, oral rehabilitation.


  1. Castelnau P, Cif L, Valente EM, Vayssiere N, Hemm S, Gannau A, et al. Pallidal stimulation improves pantothenate kinase-associated neurodege-neration. Ann Neurol 2005;57(5):738-41.

  2. Sheehy EC, Longhurst P, Pool D, Dandekar M. Self-inflicted injury in a case of Hallervorden-Spatz disease. Int J Paediatr Dent 1999;9(4):299-302.

  3. Dooling EC, Schoene WC, Richardson EPJr. Hallervorden- Spatz syndrome. Arch Neurol 1974;30(1):70-83

  4. Gregory A, Hayflick SJ. Neurodegeneration with brain iron accumulation. Folia Neuropathol 2005;43(4):286-96.

  5. Quezada CG, Quezada CSE, Mercado PG, Durán BM. Un caso de la enfermedad de Hallervorden-Spatz. Rev Mex Pediatr 2008;75(6):274-6.

  6. Chan KY, Lam CW, Lee LP, Tong SF, Yuen YP. Pantothenate kinase-associated neurodegeneration in two Chinese children: identification of a novel PANK2 gene mutation. Hong Kong Med J 2008;14(1):70-3.

  7. Del Valle-López P, Pérez-García R, Sanguino-Andrés R, González- Pablos E. Adult onset of Hallervorden-Spatz disease with psychotic symptoms. Actas Esp Psiquiatr 2011;39(4):260-2.

  8. Galvin JE, Giasson B, Hurtig HI, Lee VM, Trojanowski JQ. Neurodegeneration with brain iron accumulation, type 1 is characterized by alpha-, beta-, and gamma-synuclein neuropathology. Am J Pathol 2000;157(2):361-8.

  9. Hayflick SJ, Hartman M, Coryell J, Gitschier J, Rowley H. Brain MRI in neurodegeneration with brain iron accumulation with and without PANK2 Mutations. AJNR Am J Neuroradiol 2006;27(6):1230–3

  10. Perry TL, Norman MG, Yong VW, Whiting S, Crichton JU, Hansen S, et al. Hallervorden-Spatz disease: cysteine accumulation and cysteine dioxygenase deficiency in the globus pallidus. Ann Neurol 1985;18(4):482-9.

  11. Bonnet C, Roubertie A, Doummar D, Bahi-Buisson N, Cochen de Cock V, Roze E. Developmental and benign movement disorders in childhood. Mov Disord 2010;25(10):1317-34.

  12. Guimarães J, Santos JV. Generalized freezing in Hallervorden- Spatz syndrome: case report. Eur J Neurol 1999;6(4):509-13.

  13. Sunwoo YK, Lee JS, Kim WH, Shin YB, Lee MJ, Cho IH, et al. Psychiatric disorder in two siblings with Hallervorden-Spatz disease. Psychiatry Investig 2009;6(3):226-9.

  14. Berg D, Hochstrasser H. Iron metabolism in Parkinsonian syndromes. Mov Disord 2006;21(9):1299-310.

  15. Hartig MB, Hörtnagel K, Garavaglia B, Zorzi G, Kmiec T, Klopstock T, et al. Genotypic and phenotypicspectrum of PANK2 mutations in patients with neurodegeneration with brain iron accumulation. Ann Neurol 2006;59(2):248-56.

  16. Awasthi R, Gupta RK, Trivedi R, Singh JK, Paliwal VK, Rathore RK. Diffusion tensor MR imaging in children with pantothenate kinase-associated neurodegeneration with brain iron accumulation and their siblings. AJNR Am J Neuroradiol 2010;31(3):442-7.

  17. Chacko B, Seshadri P, Sudarsanam TD. Generalized dystonia: clinical diagnosis is possible. Hong Kong Med J 2009;15(3):224-6.

  18. Parashari UC, Aga P, Parihar A, Singh R, Joshi V. Case report: MR spectroscopy in pantothenate kinaseassociated neurodegeneration. Indian J Radiol Imaging 2010;20(3):188-91.

  19. Sethi KD, Adams RJ, Loring DW, Gammal T. Hallervorden- Spatz syndrome: clinical and magnetic resonance imaging correlations. Ann Neurol 1988;24(5):692-4.

  20. Hartinger M, Tripoliti E, Hardcastle WJ, Limousin P. Effects of medication and subthalamic nucleus deep brain stimulation on tongue movements in speakers with Parkinson’s disease using electropalatography: a pilot study. Clin Linguist Phon 2011;25(3):210-30.

  21. Møller E, Bakke M, Dalager T, Werdelin LM. Oromandibular dystonia involving the lateral pterygoid muscles: four cases with different complexity. Mov Disord 2007;22(6):785-90.

  22. Ford B, Louis ED, Greene P, Fahn S. Oral and genital pain syndromes in Parkinson’s disease. Mov Disord 1996;11(4):421-6.

  23. Frucht S, Fahn S, Shannon KM, Waters CH. A 32-year-old man with progressives pasticity and parkinsonism. Mov Disord 1999;14(2):350-7.

  24. Almeida D, Da Costa KN, Castro R de A, Almeida ML, Vianna R, Antonio AG. Self-inflicted oral injury in an infant with transverse myelitis. Spec Care Dentist 2009;29(6):254-8.

  25. Littlewood SJ, Mitchell L. The dental problems and management of a patient suffering from congenital insensitivity to pain. Int J Paediatr Dent 1998;8(1):47-50.

>Journals >Acta Pediátrica de México >Year 2012, Issue 5

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