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Abstract
RÉSUMÉ
Le Locked-In-Syndrome (LIS) chez l’enfant est un trouble neurologique rare et grave associant : la présence d’une ouverture permanente des paupières, une conscience conservée, des capacités cognitives relativement intactes, une aphonie, une quadriplégie et une communication basée principalement sur des mouvements oculopalpébraux. A moins que l’équipe médicale ne soit familière avec le LIS, cette affection peut être méconnue à la phase initiale ce qui allonge le délai diagnostique et retarde la prise en charge spécifique. Nous rapportons le cas d’une fillette de 10 ans atteinte d’un LIS secondaire à une tumeur du tronc cérébral diagnostiquée à l’IRM cérébrale 5 mois après le début des symptômes. Une corticothérapie à forte dose a été débutée dès le diagnostic et une prise en charge en radiothérapie envisagée. La patiente est décédée 10 jours après le début du traitement. Ce cas clinique soulève les difficultés diagnostiques et thérapeutiques de cette affection dans le contexte camerounais.
ABSTRACT
Locked-In-Syndrome (LIS) in children is a rare and severe neurological disorder combining: the presence of a permanent opening of the eyelids, a preserved consciousness, relatively intact cognitive abilities, aphonia, quadriplegia and a communication based mainly on oculopalpebral movements. Unless the medical team is familiar with the LIS, this disease can be unheeded in the initial phase thereby lengthening the time to diagnosis and delaying the specific management. We report the case of a 10-year-old girl presenting with a LIS secondary to a brain stem tumor diagnosed with cerebral MRI 5 months after onset of symptoms. High dose corticosteroids were initiated as soon as diagnosis was made and radiotherapy was considered. The patient died 10 days after starting treatment. This clinical case raises the diagnostic and therapeutic difficulties of this condition in a Cameroonian context.
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References
- American Congress of Rehabilitation Medecine. Recommendations for use of uniform nomenclature pertinent to patients with severe alterations of consciousness. Arch Phys Med Rehabil. 1995 Feb; 76(2): 205-9.
- Bauer G, Gerstenbrand F, Rumpl E. Varieties of the locked-in syndrome. J Neurol. 1979 Aug 1; 221(2): 77–91.
- Gosseries O, Bruno M-A, Vanhaudenhuyse A, Laureys S, Schnakers C. Consciousness in the locked-in syndrome. Neurol Conscious Cogn Neurosci Neuropathol. 2009; 191–203.
- León-Carrión J, Eeckhout P van, Domínguez-Morales M del R. Review of subject: The locked-in syndrome: a syndrome looking for a therapy. Brain Inj. 2002 Jan 1; 16(7): 555–69.
- Inci S, Özgen T. Locked-in Syndrome Due to Metastatic Pontomedullary Tumor. Neurol Med Chir (Tokyo). 2003; 43(10): 497–500.
- Bassetti, C., Mathis, J., & Hess, C. W. Multimodal electrophysiological studies including motor evoked potentials in patients with locked-in syndrome: report of six patients. J Neurol Neurosurg Psychiatry. 1994 Nov; 57(11): 1403-6.
- Laureys S, Pellas F, Van Eeckhout P, Ghorbel S, Schnakers C, Perrin F, Berré J, Faymonville ME, Pantke KH, Damas F, Lamy M, Moonen G, Goldman S. The locked-in syndrome: what is it like to be conscious but paralyzed and voiceless? Prog Brain Res. 2005; 150: 495-511.
- Bruno M-A, Schnakers C, Damas F, Pellas F, Lutte I, Bernheim J, et al. Locked-In Syndrome in Children: Report of Five Cases and Review of the Literature. Pediatr Neurol. 2009 Oct; 41(4): 237–46.
- Patterson JR, Grabois M. Locked-in syndrome: a review of 139 cases. Stroke. 1986 Jul 1; 17(4): 758–64.
- Bruno, M. A., Pellas, F., Schnakers, C., Van Eeckhout, P., Bernheim, J., Pantke, K. H., ... & Laureys, S. Le Locked-In Syndrome : la conscience emmurée. Revue Neurologique. 2008; 322–35.
- Albrecht, G. L., & Devlieger, P. J. The disability paradox: high quality of life against all odds. Social science & medicine. 1999; 977–88.
References
American Congress of Rehabilitation Medecine. Recommendations for use of uniform nomenclature pertinent to patients with severe alterations of consciousness. Arch Phys Med Rehabil. 1995 Feb; 76(2): 205-9.
Bauer G, Gerstenbrand F, Rumpl E. Varieties of the locked-in syndrome. J Neurol. 1979 Aug 1; 221(2): 77–91.
Gosseries O, Bruno M-A, Vanhaudenhuyse A, Laureys S, Schnakers C. Consciousness in the locked-in syndrome. Neurol Conscious Cogn Neurosci Neuropathol. 2009; 191–203.
León-Carrión J, Eeckhout P van, Domínguez-Morales M del R. Review of subject: The locked-in syndrome: a syndrome looking for a therapy. Brain Inj. 2002 Jan 1; 16(7): 555–69.
Inci S, Özgen T. Locked-in Syndrome Due to Metastatic Pontomedullary Tumor. Neurol Med Chir (Tokyo). 2003; 43(10): 497–500.
Bassetti, C., Mathis, J., & Hess, C. W. Multimodal electrophysiological studies including motor evoked potentials in patients with locked-in syndrome: report of six patients. J Neurol Neurosurg Psychiatry. 1994 Nov; 57(11): 1403-6.
Laureys S, Pellas F, Van Eeckhout P, Ghorbel S, Schnakers C, Perrin F, Berré J, Faymonville ME, Pantke KH, Damas F, Lamy M, Moonen G, Goldman S. The locked-in syndrome: what is it like to be conscious but paralyzed and voiceless? Prog Brain Res. 2005; 150: 495-511.
Bruno M-A, Schnakers C, Damas F, Pellas F, Lutte I, Bernheim J, et al. Locked-In Syndrome in Children: Report of Five Cases and Review of the Literature. Pediatr Neurol. 2009 Oct; 41(4): 237–46.
Patterson JR, Grabois M. Locked-in syndrome: a review of 139 cases. Stroke. 1986 Jul 1; 17(4): 758–64.
Bruno, M. A., Pellas, F., Schnakers, C., Van Eeckhout, P., Bernheim, J., Pantke, K. H., ... & Laureys, S. Le Locked-In Syndrome : la conscience emmurée. Revue Neurologique. 2008; 322–35.
Albrecht, G. L., & Devlieger, P. J. The disability paradox: high quality of life against all odds. Social science & medicine. 1999; 977–88.