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Neuropediatrics 2019; 50(02): 116-121
DOI: 10.1055/s-0038-1676514
Short Communication
Georg Thieme Verlag KG Stuttgart · New York

Muscle-Specific Kinase Autoimmune Myasthenia Gravis: Report of a Pediatric Case and Literature Review

Thouraya Ben Younes
1   Research Unit UR12 SP24, Department of Child and Adolescent Neurology, National Institute Mongi Ben Hmida of Neurology, Tunis, Tunisia
,
Hanene Benrhouma
1   Research Unit UR12 SP24, Department of Child and Adolescent Neurology, National Institute Mongi Ben Hmida of Neurology, Tunis, Tunisia
2   Faculté de Médecine de Tunis, Université de Tunis El Manar, Tunis, Tunisia
,
Hedia Klaa
1   Research Unit UR12 SP24, Department of Child and Adolescent Neurology, National Institute Mongi Ben Hmida of Neurology, Tunis, Tunisia
2   Faculté de Médecine de Tunis, Université de Tunis El Manar, Tunis, Tunisia
,
Rania Ben Aoun
1   Research Unit UR12 SP24, Department of Child and Adolescent Neurology, National Institute Mongi Ben Hmida of Neurology, Tunis, Tunisia
2   Faculté de Médecine de Tunis, Université de Tunis El Manar, Tunis, Tunisia
,
Aida Rouissi
1   Research Unit UR12 SP24, Department of Child and Adolescent Neurology, National Institute Mongi Ben Hmida of Neurology, Tunis, Tunisia
2   Faculté de Médecine de Tunis, Université de Tunis El Manar, Tunis, Tunisia
,
Melika Ben Ahmed
2   Faculté de Médecine de Tunis, Université de Tunis El Manar, Tunis, Tunisia
3   Department of Immunology, Pasteur Institute, Tunis, Tunisia
,
Ichraf Kraoua
1   Research Unit UR12 SP24, Department of Child and Adolescent Neurology, National Institute Mongi Ben Hmida of Neurology, Tunis, Tunisia
2   Faculté de Médecine de Tunis, Université de Tunis El Manar, Tunis, Tunisia
,
Ilhem Ben Youssef-Turki
1   Research Unit UR12 SP24, Department of Child and Adolescent Neurology, National Institute Mongi Ben Hmida of Neurology, Tunis, Tunisia
2   Faculté de Médecine de Tunis, Université de Tunis El Manar, Tunis, Tunisia
› Author Affiliations
Further Information

Publication History

21 March 2018

30 October 2018

Publication Date:
21 December 2018 (online)

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Abstract

Myasthenia gravis (MG) with antibodies to the muscle-specific tyrosine kinase (MuSK-MG) receptor is a rare entity. It represents 5 to 8% of all MG patients. Few pediatric cases were reported. Clinical presentation is often atypical. It is characterized by predominant involvement of cranial, bulbar, and axial muscles and early respiratory crises. Myokymia and fasciculation are suggestive of MuSK-MG. The clinical course of patients with MuSK-MG is worse than other types of MG. Responses to standard therapies are variable. We report clinical, neurophysiological, serological, and outcome profile of a Tunisian child with MuSK-MG.

Keywords

muscle-specific tyrosine kinase - myasthenia gravis - children - myokymia
 

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