Miller Fisher Syndrome With Papilledema and Antecedent Helicobacter pylori Infection

Abstract

A 16-year-old girl from Guinea (in the United States one year) with a history of malaria treated as a child and mild anemia, developed symptoms of headache, meningismus, malaise, upper then lower extremity weakness, diarrhea, nausea, emesis, and polydipsia resulting in multiple outside emergency department visits beginning 3 weeks before presentation to our institution. One week before her presentation to us, she was noted to have altered mental status and was admitted to an outside hospital. A lumbar puncture provided temporary headache relief and yielded clear cerebrospinal fluid (CSF) with an opening pressure of 55 cm/H 2 O, with one white blood cell, 41 red blood cells, protein 23.4 mg/dL, and glucose 70 mg/dL. She was started on acetazolamide (dose 500-1000 mg daily). She developed syndrome of inappropriate antidiuretic hormone. She had a normal computed tomography of the head and MRI of the spine. MRI of the brain one day after admission revealed flattened posterior globes with bulging papillae and a partial empty sella. Headaches recurred and she complained of blurred vision. Papilledema was noted on ophthalmologic examination and repeat lumbar puncture showed an opening pressure of at least 35 cm/H20 (column max 35 cm/H20 in instrument used), with one white blood cell, protein 32.2 mg/dL, glucose 77 mg/dL, negative venereal disease research laboratory test, and negative Borrelia burgdorferi DNA. A ventriculoperitoneal shunt was placed with intraoperative opening pressure noted to be 50 cm/H 2 O. The CSF testing for Escherichia coli K1, Haemophilus influenzae, Listeria monocytogenes, Neisseria meningitidis, Streptococcus agalactiae, Streptococcus pneumoniae, Cytomegalovirus, Epstein-Barr virus, herpes simplex virus 1, 2, and 6, Parechovirus, Varicella zoster, Lyme's western blot and Cryptococcus was normal. Interferon gamma release assay was negative. Serum testing for HIV, complement levels, antiproteinase 3 antibody, antimyeloperoxidase antibody, and antinuclear antibody were normal. Antidouble stranded DNA was weakly positive. One

Severe Vision Loss and Optic Disc Edema Associated With GAD-65 Antibody Positive Miller Fisher Syndrome

“…Although a few cases of GAD-65 Ab–positive MFS have been reported (1–3), to the best of our knowledge, this is the first reported case of GAD-65 Ab–positive MFS with severe and persistent vision loss and optic disc edema. Optic disc edema in GQ-1b Ab–positive MFS has been found in a few reported cases and is usually associated with severe headaches and resolution of optic disc edema with IVIG (4,5). By contrast, our patient had only transient moderate holocephalic headache.…”