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Washington University Experience | MYELIN (IMMUNE-MEDIATED) | MS - Secondary Progressive (SPMS), fulminant | 1A0 Case 1 History
Case 1 History --- This patient was a 47 year old woman with a 10 year history of MS, initially relapsing and remitting and eventually progressive and fulminant. She was first seen in 2006 with bilateral lower extremity numbness, tonic spasms and falls. Physical exam was noteworthy for bilateral temporal retinal pallor, Lhermitte’s sign, abnormal vibratory sensation, positive Romberg sign, ataxia, gait unsteadiness and bilateral ankle clonus with upgoing toes. Strength was normal. Brain MRI in 2006 showed at least 10 hemispheric white matter lesions and innumerable patchy T2 signal abnormalities with contrast enhancement in C2-T10 spinal cord. She was treated for her diagnosis of MS unsuccessfully with beta-interferon and received Tysabri (natalizumab), from 11/2007 to 2/2010 (always JC virus negative) but stopping after losing confidence in its efficacy. Copaxone (glatiramer acetate injection), Rituximab and Gilenya (fingolimod) did not prevent relapses. In March of 2015, she was doing relatively well when she suddenly began to experience worsening weakness, dysmetria, vertigo, nausea, intermittent diplopia, dysphagia and urinary retention and now needed help with all activities of daily living. A 5 day course of IV methylprednisolone was minimally effective. MRI showed multiple new multiple punctate and confluent white matter T2 hyperintensities involving the corpus callosum, centrum semiovale, frontal and biparietal subcortical white matter, left cerebral peduncle, midbrain, pons and bilateral superior and middle cerebellar peduncles, many of which showed contrast enhancement. Cord lesions appeared unchanged. In May she had weakness of all 4 limbs, numerous cranial nerve abnormalities and decreased hearing, treated with plasma exchange. Plasma exchange X5 only resulted in slight improvement. In late May there were numerous cranial nerve abnormalities including abnormal motility, marked bidirectional nystagmus, dysarthria, and significant hearing loss. It was recommended she stop Gilenya and start Tysabri. JCV became positive but there was a low index of suspicion that PML accounted for the clinical picture. Seen in the clinic on 7/21/15, she had continued to decline and was unable to move her arms and legs or hold up her head. She was again hospitalized in late July 2015, which showed no evidence for any other cause of her rapid decline other than aggressive multiple sclerosis (NMO-IgG was negative) and she expired.
