Spencer S. Eccles Health Sciences Library, University of Utah
Abstract
A 36 year old woman presented with history of Myasthenia Gravis (MG) since the age of 18. She was known to have dry eyes and hypothyroidism. Past surgical history included a thymectomy (age 23 years). The MG had been difficult to control with past medications including corticosteroids, azathioprine, methotrexate and mycophenolate. Eighteen months prior to this presentation she was enrolled in a double blind placebo controlled trial with Eculizumab, a terminal complement inhibitor. She had required intravenous immunoglobin (IV IG) as rescue therapy initially and went on to open label Eculizumab 9 months prior to this admission. She had subsequently developed Alopecia areata (no family history), followed by nummular dermatitis, and then Alopecia totalis. The decision was made to have a drug holiday. This precipitated a worsening of the MG requiring IV IG. She restarted Eculizumab and 3 weeks later developed swinging fevers, nausea and vomiting and generalised polyarthralgia. On admission her vision (6/5 OU) and colour vision were normal. She had longstanding symptomatic left limitation of abduction, with normal saccades. She had no relative afferent pupillary defect and enlarged blind spots on Humphrey visual field testing. Dilated examination showed bilateral papilloedema. There was no other cranial neuropathy. Blood tests on admission were normal, including inflammatory markers and three sets of blood cultures with no growth. Inflammatory antibody tests were normal, as was HIV and testing for Tuberculosis. Lumbar puncture opening pressure was 23 cm CSF, with raised protein (0.93g/dl), low CSF glucose compared to serum, a mononuclear pattern, oligoclonal bands in the CSF only and PCR for viral and bacterial factors were negative. Initial MRI imaging was normal. CT thorax, abdomen and pelvis was normal. FDG-PET imaging showed increased uptake along the spinal cord. Bone marrow biopsy was normal. A brain biopsy and one further diagnostic test was performed.KBDautoimmuneopticneuropath
