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

Thouraya Ben Younes; Hanene Benrhouma; Hedia Klaa; Rania Ben Aoun; Aida Rouissi; Melika Ben Ahmed; Ichraf Kraoua; Ilhem Ben Youssef-Turki

doi:10.1055/s-0038-1676514

Neuropediatrics, Table of Contents

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

¹Research Unit UR12 SP24, Department of Child and Adolescent Neurology, National Institute Mongi Ben Hmida of Neurology, Tunis, Tunisia

,

Hanene Benrhouma

¹Research Unit UR12 SP24, Department of Child and Adolescent Neurology, National Institute Mongi Ben Hmida of Neurology, Tunis, Tunisia

²Faculté de Médecine de Tunis, Université de Tunis El Manar, Tunis, Tunisia

,

Hedia Klaa

¹Research Unit UR12 SP24, Department of Child and Adolescent Neurology, National Institute Mongi Ben Hmida of Neurology, Tunis, Tunisia

²Faculté de Médecine de Tunis, Université de Tunis El Manar, Tunis, Tunisia

,

Rania Ben Aoun

¹Research Unit UR12 SP24, Department of Child and Adolescent Neurology, National Institute Mongi Ben Hmida of Neurology, Tunis, Tunisia

²Faculté de Médecine de Tunis, Université de Tunis El Manar, Tunis, Tunisia

,

Aida Rouissi

¹Research Unit UR12 SP24, Department of Child and Adolescent Neurology, National Institute Mongi Ben Hmida of Neurology, Tunis, Tunisia

²Faculté de Médecine de Tunis, Université de Tunis El Manar, Tunis, Tunisia

,

Melika Ben Ahmed

²Faculté de Médecine de Tunis, Université de Tunis El Manar, Tunis, Tunisia

³Department of Immunology, Pasteur Institute, Tunis, Tunisia

,

Ichraf Kraoua

¹Research Unit UR12 SP24, Department of Child and Adolescent Neurology, National Institute Mongi Ben Hmida of Neurology, Tunis, Tunisia

²Faculté de Médecine de Tunis, Université de Tunis El Manar, Tunis, Tunisia

,

Ilhem Ben Youssef-Turki

¹Research Unit UR12 SP24, Department of Child and Adolescent Neurology, National Institute Mongi Ben Hmida of Neurology, Tunis, Tunisia

²Faculté de Médecine de Tunis, Université de Tunis El Manar, Tunis, Tunisia

› Author Affiliations

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

Full Text

References

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