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Washington University Experience | MYELIN (NON-IMMUNE MEDIATED) | CADASIL | 2A0 Case 2 History
Case 2 History ---- The patient is a 41-year-old man who carried a diagnosis of multiple sclerosis up to the time of admission, who presented to our hospital with complaints of worsening right hemiparesis and respiratory distress. The patient had been diagnosed with multiple sclerosis in 1992, manifested in an apparent relapsing, remitting course. At baseline, the patient had left hemiparesis with contractures and spasms. In the week before admission, the patient began to deteriorate in strength on the right side with falls and problems with transferring from site to site. The patient also developed quiet speech and intermittent disorientation. The patient had been started on Avonex six weeks previous to his admission. In June 2002, the examination showed left-sided weakness with increased tone and no cranial nerve or optic nerve dysfunction. His strength worsened in August and November 2002. An MRI at that time showed restricted diffusion in the left corona radiata and small punctate areas of restricted effusion in the right subcortical posterior parietal region and left cerebellar hemisphere. The large region of restricted effusion at the left corona radiata demonstrated increased signal on T2 with no corresponding enhancement or T1 abnormality. At an outside hospital, the patient had difficulty handling his respiratory secretions and was intubated. The patient was given Zosyn empirically as well as loaded with Fosphenytoin, following 20 seconds of observed right arm spasm and shaking with gaze deviation. The patient's relapsing, remitting multiple sclerosis was changed to a diagnosis of secondary progressive multiple sclerosis in the fall of 2002. In early 2003 he was biopsied and a diagnosis of CADASIL established. He expired in 2006 of respiratory failure secondary to prior strokes and diabetes.
