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BMJ Case Rep ; 13(9)2020 Sep 13.
Article in English | MEDLINE | ID: mdl-32928818

ABSTRACT

A 30-year-old woman presented with recurrent hiccups, vomiting and painful diminution of vision and gait instability for 1 day. She had one-and-a-half syndrome, bilateral seventh cranial nerve paresis with bilateral symptomatic optic neuritis and left-sided ataxic haemiparesis. We described her disorder as the 'twenty syndrome' (11/2+7+7+2+2+½=20). MRI of her brain revealed demyelination predominantly in right posterolateral aspect of pons, medulla and bilateral optic nerves. Serum antiaquaporin-4 antibody came out positive. Thus, she was diagnosed as neuromyelitis optica spectrum disorder (NMOSD). She responded brilliantly to immunosuppressive therapy. This is the first ever reported case of the 'twenty syndrome' secondary to cerebral NMOSD.


Subject(s)
Cerebellar Ataxia/immunology , Facial Paralysis/immunology , Immunosuppressive Agents/therapeutic use , Neuromyelitis Optica/diagnosis , Ocular Motility Disorders/immunology , Optic Neuritis/immunology , Adult , Aquaporin 4/immunology , Autoantibodies/blood , Autoantibodies/immunology , Cerebellar Ataxia/blood , Cerebellar Ataxia/diagnosis , Cerebellar Ataxia/drug therapy , Facial Paralysis/blood , Facial Paralysis/diagnosis , Facial Paralysis/drug therapy , Female , Humans , Magnetic Resonance Imaging , Neuromyelitis Optica/blood , Neuromyelitis Optica/complications , Neuromyelitis Optica/immunology , Ocular Motility Disorders/blood , Ocular Motility Disorders/diagnosis , Ocular Motility Disorders/drug therapy , Optic Nerve/diagnostic imaging , Optic Nerve/immunology , Optic Neuritis/blood , Optic Neuritis/diagnosis , Optic Neuritis/drug therapy , Pontine Tegmentum/diagnostic imaging , Pontine Tegmentum/immunology , Syndrome , Treatment Outcome
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