Juvenile limb-girdle myasthenia gravis.
Limb-girdle myasthenia is a rare disorder which includes familial and autoimmune forms. Myasthenia gravis is an uncommon disease in children and its diagnosis may be difficult. We report here five cases of autoimmune juvenile LGM starting before the age of 16 years with attention to clinical diagnostic difficulties, evolution, laboratory and instrumental data as well as response to treatment. Diagnosis of limb-girdle myasthenia requires a strong index of suspicion also in childhood. We suggest that this form be suspected in children with unclassifiable myopathy, mostly affecting deltoid muscles and lower extremities.
Rodolico
C
C
Department of Neurosciences, Psychiatry and Anaesthesiology, University of Messina, Italy. crodolico@unime.it
Pastura
C
C
Sinicropi
S
S
Girlanda
P
P
Toscano
A
A
Messina
C
C
Vita
G
G
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D002800:Cholinesterase Inhibitors / Q000627:therapeutic use; D022062:Electrophysiologic Techniques, Cardiac; D005260:Female; D006801:Humans; D008297:Male; D018482:Muscle, Skeletal / Q000473:pathology / Q000503:physiopathology; D049288:Muscular Dystrophies, Limb-Girdle / Q000150:complications* / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D009157:Myasthenia Gravis / Q000150:complications* / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy
D002800:Cholinesterase Inhibitors