Abstract
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.
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References
Ben Hamida M, Hentati F, Ben Hamida C. Hereditary motor system diseases (chronic juvenile amyotrophic lateral sclerosis).Brain 1990; 13: 347–363.
Bradley WG, Krasin F. A new hypothesis of etiology of amytrophic lateral sclerosis: the DNA hypothesis.Arch Neurol 1982; 39: 677–680.
Refums, Skillicon SA. Amyotrophic familial spastic paraplegia.Neurology 1954; 4: 40–47.
Rabin BA, Griffin JW, Crain BJ. Autosomal dominant juvenile amyotrophic lateral sclerosis.Brain 1999; 122: 1539–1550.
World Federation of neurology research group on motor neuron disease. Revised criteria for diagnosis of amytropic lateral sclerosis. www.wfnals.org/Articles/esescorial 1998.htm.
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.
Prabhakar S, Chopra JS, Banerjee AKet al. Wasted leg syndrome. A clinical, electrophysiological and histological study.Clinical Neuro Neurosurg 1981; 83: 19.
Panagariya A, Garg A, Sharma B. Juvenile amyotrophic lateral sclerosis with unusual presentation: A case report.Neurol India 2003; 51: 413–414.
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.
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.
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.
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.
Engel WK, Siddique T, Nicloff JT. Effect on weakness and spasticity in amyotropic lateral sclerosis of thyrotropin releasing hormone.Lancet 1983; 73–75.
Olarte MR, Schoenfeldt MG, Rowland LP. Plasmapheresis in amytrophic lateral sclerosis.Ann Neurol 1980; 8: 644–645.
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Aggarwal, A., Shashiraj Juvenile amyotrophic lateral sclerosis. Indian J Pediatr 73, 225–226 (2006). https://doi.org/10.1007/BF02825486
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DOI: https://doi.org/10.1007/BF02825486