Table 1: Timeline of events.

Date Symptoms Lab findings AQP4-ab Status MRI findings Therapy administered
May 2010 Bulbar dysfunction, dysphagia, dysphonia, pulmonary aspirations CSF: normal protein and glucose; no RBCs, 2 nucleated cells; OBs negative Negative Large brainstem lesion;
Five days later multiple areas of hyperintensities (Figure 1a)
IV methylprednisone 1 g/day;
IV IgG 0.4 mg/kg for 5 days
October 2010 Gait difficulties, repeated falls, distal paresthesia CSF: No RBCs, 7 nucleated cells, Glucose: 45 mg/dl, Protein: 26 mg/dl Negative Improvement in previous brainstem lesion; new lesion from C2-C7 (Figure 1b) IV methylprednisone 1 g/day;
October 2010-April 2015 None - Negative Stable MMF 1500 g/day
June 2015 None - 123 U/mL (Positive) Stable MMF 500 mg twice per day
September 2015 Pain, visual problems, subcutaneous nodules No OBs, Ig G/albumin index: 5.9, Glucose: 63 mg/dl, Protein: 19 mg/dl, VEP prolonged conduction on the left side 122 U/mL (Positive) Stable IV methylprednisone 1 g/day
November 2015-December 2015 LLE paresthesia, blurry vision ANA titer 1:2560. NMDA < 1.1. Glucose: 58 mg/dl, Protein: 30 mg/dl; IgG/albumin index: 0.89, low C3 Pachymeningeal enhancement (Figure 2) IV methylprednisone 1 g/day; MMF 1000 mg/day
December 2015 Five days after hospital discharge, the patient has rapid deterioration in affect, withdrawn, mute, she required assistance with feeding and activities of daily living, became incontinent and was re-admitted. On physical exam, did not follow commands, had midline gaze without tracking, generalized swelling, persistent subcutaneous skin nodules and persistent variable tachycardia with occasional fever. ANA titer 1:2560; thyroid microsomal
Elevated, ENA
ENA RNP Antibodies positive,
LOW C3, CSF IgG/ albumin index 3.0,
123 units/mL Diffuse pachymeningeal enhancement Plasmapheresis;
IV methylprednisone 1 g/day;
5 day PLEX;
IV rituximab;
MMF 3000 mg/day;
IV IgG 0.4 mg/kg/day for 5 days