Intractable vomiting and hiccups as the presenting symptom of neuromyelitis optica
A 24-year-old lady developed recurrent projectile vomiting, 8-10 times/day for initial few days and 2-3 episodes/day later, associated with hiccups which used to come intermittently. Symptoms continued for 1.5 months. There were no other systemic or neurological complaints. Initially patient was seen by a general practitioner and was treated symptomatically. She did not experience weight loss, dehydration, or electrolyte disturbances as she was being treated with intravenous (IV) fluids. Later, she was evaluated by an ear, nose, and throat (ENT) specialist and a neurologist. A computed tomography (CT) scan was done which was normal again she was continued on symptomatic treatment. Later she was evaluated by another neurologist and MRI of brain was done, which showed T2/fluid attenuated inversion recovery (FLAIR) hyperintensity in periaqueductal grey matter of midbrain, midbrain tectum, medulla, and right temporal periventricular region [Figure and ]. The diagnosis considered were Wernicke's encephalopathy/acute disseminated encephalomyelitis (ADEM). There were no symptoms/signs of encephalopathy. Electroencephalogram and lumbar puncture were not performed. Vomiting and hiccups stopped after a 5-day course of IV methylprednisolone (IVMP). She was continued on oral steroids 1 mg/kg (weight 62 kg) initially and later on tapered for 5-6 months and she was asymptomatic subsequently. Five months later (11 months after initial symptom) she developed numbness of the right upper limb followed by right lower limb without facial involvement over 2 weeks. There was no weakness of limbs and bowel bladder disturbances. She was again given 5-day course of IVMP, but there was no significant improvement. Oral steroids were not given. Patient was independent for her daily activities.\nThree months later she developed bilateral visual loss which improved completely over 1 month following another course of IVMP. Oral steroids 1 mg/kg were given initially and gradually tapered over 1 year along with single dose of IV methylprednisolone injection every month. Later (2 years 2 months after onset of first symptom and 1.5 years of steroid therapy) she developed painful restriction of movements of left hip and imaging with MRI was done and was diagnosed to have avascular necrosis of femur both sides for which she underwent core decompression. Two and half years after the first symptom again she developed weakness of all four limbs requiring two person's support to walk, numbness below the neck, and urinary retention. This time she presented to our institute. MRI of spine showed longitudinally extensive transverse myelitis (LETM) involving first to fifth cervical vertebra []. We considered the diagnosis of NMO. Her NMO antibody test was positive. Other autoantibodies were negative. Cerebrospinal fluid examination showed six lymphocytes and protein of 41 mg/dL. Human immunodeficiency virus (HIV) and venereal disease research laboratory (VDRL) tests were nonreactive. Vitamin B12 level was normal. She was treated with five cycles of large volume plasmapheresis and she made significant improvement (expanded disability status scale was 6.5 at the time of admission and it was 3.5 during last follow-up 2 months later). For immunomodulation, azathioprine has been started, after explaining the need, short and long term side effects.