Dermato myo myelitis: a case of concurrent anti-synthetase syndrome and NMOSD

ABSTRACT

A 50 year old Afro-Caribbean gentleman with a background of Ro52 and MDA5 antibody positive anti- synthetase syndrome on prednisolone and mycophenolate mofetil presented with a tight band like pain around his thorax and abdominal pain. Examination identified severe dysaesthesia with a T6 spinal level, as well as lower limb and truncal weakness. MRI showed an expansile T2 hyperintense lesion from C7 to T8 with patchy enhancement in keeping with a NMOSD. He had no optic nerve involvement, confirmed by OCT. The weakness and dysaesthesia responded rapidly to high dose methylprednisolone however he developed constipation, recurrent neuropathic pain and tonic spasms. Serum AQP4 antibodies were present. At presentation serum CK was also raised.Up to 25% of patients with NMOSD have co-existing autoimmunity. However, there are very few reports of anti-synthetase syndrome and NMOSD. Ro-antibodies are well documented in studies on Sjögren’s disease and transverse myelitis. Patients with autoimmune connective tissue diseases who develop neurological symptoms should be investigated for neurological autoantibodies too as they are at risk of multiple autoimmune disorders. Furthermore, neurologists should be observant to other systems when presented with a patient with NMOSD as they may have other diseases which are currently asymptomatic or minimally symptomatic.