Neuromyelitis optica in a patient with pemphigus foliaceus.
Neuromyelitis optica (NMO, also eponymously known as Devic's disease) is an immune-mediated demyelinating disease of the central nervous system that can lead to significant disability. Pediatric NMO is a rare disorder often reported after an infection. The authors report a 16 year-old female patient with pemphigus foliaceus who developed subacute optic neuritis followed by cervical transverse myelitis. Restricted distribution of the lesions in the optic nerve and spinal cord was confirmed by ophthalmological evaluation and magnetic resonance imaging of the brain and spinal cord. She was started on intravenous methylprednisolone and then given a maintenance oral prednisone. Subsequently, she was treated with a nonsteroidal immunosuppressant, mycophenolate mofetil, with a target dose of 1000 mg twice a day. Over the course of months, patient noted significant recovery of previous deficits and resolution of the cervical cord enhancement, expansion and cystic dilatation that was previously seen. This case is noteworthy for being the first patient reported with neuromyelitis optica associated with pemphigus foliaceus.
Department of Neurology, Henry Ford Hospital, Detroit, MI 48202, USA. ricsalmont@hotmail.com
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Salazar
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Cerghet
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Farhat
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Lim
D000293:Adolescent; D001921:Brain / Q000473:pathology*; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008775:Methylprednisolone / Q000627:therapeutic use; D009173:Mycophenolic Acid / Q000031:analogs & derivatives / Q000627:therapeutic use; D009471:Neuromyelitis Optica / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D009900:Optic Nerve / Q000473:pathology; D010392:Pemphigus / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D013116:Spinal Cord / Q000473:pathology*; D016896:Treatment Outcome
D007166:Immunosuppressive Agents; D009173:Mycophenolic Acid; D008775:Methylprednisolone