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; MMF |
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December 2015 | Five days after hospital discharge, the patient has rapid deterioration in affect, withdrawn, mute, she required assistance with feeding and activities of daily living, became incontinent and was re-admitted.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 Antibodies Elevated, ENA SSA, 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 |