| Identifier |
walsh_2018_s1_c3 |
| Title |
Gee...What's Causing that Pap? - Video |
| Creator |
Susan Mollan; Daniel White; Santhosh Nagaraju; Swarupsinh Chavda; Tom Hayton; Saiju Jacob |
| Affiliation |
(SM) Birmingham Neuro-Ophthalmology, Queen Elizabeth Hospital, Birmingham, United Kingdom; (DW) (SN) (SC (TH)( SJ) University Hospitals Birmingham NHS Foundation Trust, Birmingham, United Kingdom |
| Subject |
Papilledema; Meningo-Encephalitis; Vision Loss |
| Description |
Investigation and initial treatment was aimed at the differential diagnosis of an infectious aetiology, an inflammatory or aseptic meningitis and lymphoma. Brain biopsy showed a non-specific meningo-encephalitis. She was treated subsequently with corticosteroids, cyclophosphamide, mycophenolate, IV IG, plasma exchange, and rituximab. Initial she improved, but this was followed by progressive decline with quadriplegia and bilateral blindness; ventilator dependency and she succumbed to her illness. Her CSF was positive for an antibody directed against Glial Fibrillary Acidic Protein (GFAP) with a titre of 1:512 (normal range <1:2). This is a novel astrocytic autoantibody identified as a biomarker of a relapsing autoimmune meningoencephalomyelitis Co-Authors[1]. Transient serum antibodies have been described in traumatic brain injury, brain tumours, autism, lead exposed workers and diabetes mellitus. Autoimmune GFAP astrocytopathy has been characterized by the Mayo clinic series Co-Authors[2] with similar findings to our patient, who was young and female with a prodromal flu-like illness with co-existing autoimmune disorder of MG. Another small series has also expressed treatment difficult with poor response to immunosuppression Co-Authors[3], as experienced in this case. |
| History |
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. |
| Disease/Diagnosis |
Autoimmune Glial Fibrillary Acidic Protein (GFAP) Astrocytopathy |
| Presenting Symptom |
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. |
| Date |
2018-03 |
| References |
1 Fang B, McKeon A, Hinson SR, Kryzer TJ, Pittock SJ, et al. Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy: A Novel Meningoencephalomyelitis JAMA Neurol, 73,1297-1307, 2016 2 Flanagan EP, Hinson SR, Lennon VA, Fang B, Aksamit AJ, et al. Glial Fibrillary Acidic Protein Immunoglobulin G as Biomarker of Autoimmune Astrocytopathy: Analysis of 102 Patients. Ann Neurol, 81, 298-309, 2017 3 Yang X, Liang J, Huang Q, Xu H, Gao C, et al. Treatment of Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy: Follow-Up in 7 Cases. Neuroimmunomodulation, 24, 113-119, 2017 |
| Language |
eng |
| Format |
video/mp4 |
| Type |
Image/MovingImage |
| Source |
2018 North American Neuro-Ophthalmology Society Annual Meeting |
| Relation is Part of |
NANOS Annual Meeting 2018 |
| Collection |
Neuro-Ophthalmology Virtual Education Library: Walsh Session Annual Meeting Archives: https://novel.utah.edu/Walsh/ |
| Publisher |
North American Neuro-Ophthalmology Society |
| Holding Institution |
Spencer S. Eccles Health Sciences Library, University of Utah |
| Rights Management |
Copyright 2018. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
| ARK |
ark:/87278/s6xd5468 |
| Setname |
ehsl_novel_fbw |
| ID |
1320243 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s6xd5468 |
