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A 14-year-old girl presented with progressive upper and lower extremity weakness. Symptoms started 5 months prior with intermittent bilateral calf pain coincident with onset of lower extremity weakness. Initial evaluation revealed normal complete blood count, complelete metabolic panel, inflammatory markers, muscle enzymes and MRI brain and spine with/without contrast. Electromyography demonstrated an acute to subacute polyradiculitis affecting the upper and lower extremities.
She was admitted for expedited evaluation. Examination was significant for lower extremity weakness and absent reflexes. Lumbar puncture (LP) showed lymphocytic pleocytosis and markedly elevated protein. Thorough infectious, oncological and rheumatological evaluations were all unremarkable. Most notable was that CSF Neuromyelitis Optica IgG and paraneoplastic antibody panel were negative, and serum ACE was normal. She was started on intravenous immunoglobulin for empiric treatment of chronic inflammatory demyelinating polyneuropathy (CIDP).
While hospitalised, she developed left eye blurred vision. Ophthalmological examination demonstrated bilateral optic nerve oedema. Repeat MRI brain showed enhancement of multiple cranial nerves (figure 1). Initial MRI spine was reinterpreted as diffuse enhancement and …
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