The Indian Journal of Pediatrics

, Volume 73, Issue 3, pp 225–226 | Cite as

Juvenile amyotrophic lateral sclerosis

  • Anju Aggarwal
  • Shashiraj
Clinical Brief


Juvenile amytrophic lateral sclerosis (JALS) is a type of motor neuron disease presenting before 25 years of age. It is characterized by a combination of upper and lower motor signs. It may be familial or sporadic. We are reporting a sporadic case of JALS with onset of symptoms at 4 years of age. Diagnostic criteria and a brief review of literature are presented.

Key words

Juvenile amyotrophic lateral sclerosis Early onset 


  1. 1.
    Ben Hamida M, Hentati F, Ben Hamida C. Hereditary motor system diseases (chronic juvenile amyotrophic lateral sclerosis).Brain 1990; 13: 347–363.CrossRefGoogle Scholar
  2. 2.
    Bradley WG, Krasin F. A new hypothesis of etiology of amytrophic lateral sclerosis: the DNA hypothesis.Arch Neurol 1982; 39: 677–680.PubMedGoogle Scholar
  3. 3.
    Refums, Skillicon SA. Amyotrophic familial spastic paraplegia.Neurology 1954; 4: 40–47.Google Scholar
  4. 4.
    Rabin BA, Griffin JW, Crain BJ. Autosomal dominant juvenile amyotrophic lateral sclerosis.Brain 1999; 122: 1539–1550.PubMedCrossRefGoogle Scholar
  5. 5.
    World Federation of neurology research group on motor neuron disease. Revised criteria for diagnosis of amytropic lateral sclerosis. 1998.htm.Google Scholar
  6. 6.
    Gouriedevi M, Suresh TG, Shankar SK. Pattern of motor neuron disease in south India and monomelic amyotrophy. (A benign atypical form). In Gouriedevi M, eds.Motor Neuron Disease. Oxford and IBH Publishing Co Pvt Ltd India 1984; 171–190.Google Scholar
  7. 7.
    Prabhakar S, Chopra JS, Banerjee AKet al. Wasted leg syndrome. A clinical, electrophysiological and histological study.Clinical Neuro Neurosurg 1981; 83: 19.CrossRefGoogle Scholar
  8. 8.
    Panagariya A, Garg A, Sharma B. Juvenile amyotrophic lateral sclerosis with unusual presentation: A case report.Neurol India 2003; 51: 413–414.PubMedGoogle Scholar
  9. 9.
    Emery AEH, Holloway S. Familial motor neuron disease. In Rowland LP, ed.Human motor neuron disease. Advances in Neurology. New York; Raven Press; 1982. Vol. 36. 139–47.Google Scholar
  10. 10.
    Rossen DR, Siddique T, Patterson D, Figlewicz DA, Sapp P, Hentati Aet al. Mutations in the Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis.Nature 1993; 362:59–62.CrossRefGoogle Scholar
  11. 11.
    Hentati A, Bejaoui K, Pericak-Vance MA, Hentati F, Speer MC, Hung W-Yet al. Linkage of recessive familial amyotrophic lateral sclerosis to chromosome 2q33–q35.Nat Genet 1994b; 7: 425–428.PubMedCrossRefGoogle Scholar
  12. 12.
    Jonghe PD, Grumbach MA, Wagner IK, Plecko B, Kennerson M, Zhu Det al. Autosomal dominant juvenile amytrophic lateral sclerosis and distal hereditary motor neuronopathy with pyramidal tract signs: synonyms for same disorder?Brain 2002; 125: 1320–1325.PubMedCrossRefGoogle Scholar
  13. 13.
    Engel WK, Siddique T, Nicloff JT. Effect on weakness and spasticity in amyotropic lateral sclerosis of thyrotropin releasing hormone.Lancet 1983; 73–75.Google Scholar
  14. 14.
    Olarte MR, Schoenfeldt MG, Rowland LP. Plasmapheresis in amytrophic lateral sclerosis.Ann Neurol 1980; 8: 644–645.PubMedCrossRefGoogle Scholar

Copyright information

© Dr. K C Chaudhuri Foundation 2006

Authors and Affiliations

  1. 1.Department of Pediatrics, University College of Medical SciencesGuru Tegh Bahadur HospitalDelhiIndia

Personalised recommendations