XXXX : a study of familial and sporadic cases.

@entity1 @entity230 @entity1737 @entity71193
Chronic @entity71193 gravis ( @entity1737 ) is a rare entity. We describe six such @entity1 (F:M 4:2) who constituted 5% of 120 @entity1737 cases in a seven year study. The disease was familial in four and sporadic in two. No @entity1 had ocular muscle weakness either at presentation (mean of 18.2 months after onset of illness) or during a mean follow up period of 36 months. Diagnosis was established by a positive decremental response on repetitive stimulation of a proximal muscle. Muscle biopsy was essentially normal in all five @entity1 . All @entity1 responded to acetylcholinesterase inhibitors, although to varying degrees. Four @entity1 also received @entity230 . One @entity1 with @entity1737 had transient worsening but others showed partial improvement. It is noteworthy that the initial diagnosis in these @entity1 was other than @entity1737 . Diagnosis of @entity71193 needs to have a strong index of suspicion as it has therapeutic implications.
