Prepubertal anti-Musk positive myasthenia gravis with long remission.
Anti-MuSK positive myasthenia gravis (MuSK-MG) is rare prepuberty. We report a female patient with MuSK-MG starting at 3.5years with ptosis as the sole symptom for 2years. A brief period of generalization was followed by complete stable remission for 6years. Prolonged ocular symptoms and long remissions are not features of MuSK-MG, but are often seen in prepubertal onset MG. The patient then presented at age 12 with moderately severe symptoms which were almost confined to oculobulbar muscles and were unresponsive to pyridostigmine. She was dependent on corticosteroids and thymectomy did not seem to be effective. She was later noted to have tongue atrophy after a period without treatment. Our patient thus presented with features seen in many prepubertal patients, but the later course was quite typical of MuSK-MG.
Department of Neurology, Istanbul University, Istanbul Medical Faculty, Capa 34390, Istanbul, Turkey. Electronic address: ozlemgtuncer@hotmail.com.
Ozlem
O
Gungor-Tuncer
Department of Neurology, Istanbul University, Istanbul Medical Faculty, Capa 34390, Istanbul, Turkey.
Elif Kocasoy
EK
Orhan
Department of Neurology, Istanbul University, Istanbul Medical Faculty, Capa 34390, Istanbul, Turkey.
Vuslat
V
Yilmaz
Department of Neurology, Istanbul University, Istanbul Medical Faculty, Capa 34390, Istanbul, Turkey.
Yesim
Y
Parman
Department of Neurology, Istanbul University, Istanbul Medical Faculty, Capa 34390, Istanbul, Turkey.
Piraye
P
Oflazer
Department of Neurology, Istanbul University, Istanbul Medical Faculty, Capa 34390, Istanbul, Turkey.
Guher
G
Saruhan-Direskeneli
Department of Neurology, Istanbul University, Istanbul Medical Faculty, Capa 34390, Istanbul, Turkey.
Feza
F
Deymeer
D000293:Adolescent; D000906:Antibodies / Q000097:blood; D018450:Disease Progression; D005260:Female; D006801:Humans; D009157:Myasthenia Gravis / Q000175:diagnosis* / Q000276:immunology; D020794:Receptor Protein-Tyrosine Kinases / Q000276:immunology*; D011950:Receptors, Cholinergic / Q000276:immunology*; D012074:Remission Induction
D000906:Antibodies; D011950:Receptors, Cholinergic; C095714:MUSK protein, human; D020794:Receptor Protein-Tyrosine Kinases
Juvenile; MuSK positive; Myasthenia gravis; Prepubertal