RELAPSE OF NMO NEGATIVE NEUROMYELITIS OPTICA SPECTRUM DISORDER IN A MIDDLE AGED INDIAN FEMALE-A CASE REPORT

Background: Neuromyelitis Optica Spectrum Disorder(NMOSD) is a central nervous system inammatory disease usually with a relapsing course, that has a particular predilection to cause Optic Neuritis and Longitudinally extensive Transverse Myelitis (LETM).Approximately 70% of cases are associated with antibodies to Aquaporin 4 immunoglobulin G(AQ4IG-G). A 45 year old female resented to Civil Hospital Ahmedabad Case presentation: with complain(s) of bilateral lower limb weakness, sub acute in onset ,progressive in nature, complain of loss of touch and pain sensation in bilateral lower limbs, urinary incontinence, all since 10 days. She had a history of similar illness 9 year ago and a history of sudden onset painful loss of vision in left eye consistent with optic neuritis in one year ago. On Neurological examination, there was loss of sensation below nipples, lower limbs was 0,DeepTendon reex of bilateral lower limbs were exaggerated, Plantar were upwards bilaterally.Labs: Complete Blood count ,Liver Function Tests, Renal Function Tests all were within normal range .Serum B12 was within normal range.ANA by Indirect Immuno uorescence was negative.HIV was negative. On routine CSF examination, protein was 18 mg/dl, cells were 2/hpf.CSF showed no unique Oligoclonal bands. Serum Aquaporin 4 immunoglobulin G(AQ4IG-G)negative, Myelin Oligodendrocyte Glycoprotein(MOG) antibody negative. Fundus examination was normal.MRI dorsal spine was suggestive of Longitudionally Extensive Transverse Myelitis(LETM), from D2-D12 level with diffuse grey and white matter involvement,hyperintense on T2 and STIR. MRI brain was suggestive of chronic small vessel ischemic changes.Patient was diagnosed Acute Transverse Myelitis due to relapse of AQ4IG-G negative Neuromyelitis Optica Spectrum Disorder(NMOSD). She was started on high dose methylprednisolone and Plasma Exchange. The patient improved signicantly after starting the treatment. She was discharged on oral steroids and Azathioprine. Summary: Relapses in NMOSD tend to be more severe and disabling .The recovery is often incomplete with incremental disability. It is very important to diagnose this disease early and distinguish it from Multiple Sclerosis (MS)because of a number of drugs used for MS are ineffective and potentially harmful for NMOSD. Management of NMOSD is directed for rescue treatment for acute attacks which includes combination of Plasma exchange and steroids and maintenance immune therapies.