Posted On :
30 May 2005
12 yr old female presented with generalised joint contractures including inability to open mouth fully and generalised muscle wasting which has developed over the last 3 years,prior to which she was absolutely normal. Facial appearance has a puckered look with tight skin. Her fingers and toes show features of arachnodactyl. No h,o Raynauds phenomenon. No h,o dysphagia. No abnormal perinatal events. No h,o delayed mile stones.B owel,micturition-normal. Investigations revealed atlanto axial subluxation. CXR NORMAL. USG abdomen normal. ESR 65MM. RA FACTOR -VE. ANA plus VE. What could be the diagnosis?
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