OBJECTIVE: To investigate the clinic characters and diagnosis of Susac syndrome. METHODS: Two cases were systematically studied by the authors. RESULTS: All the two cases were young women. The clinical manifestations include acute and subacute multifocal and diffuse encephalopathic symptoms, hearing loss, and visual loss. Diagnosis is facilitated by demonstration of retinal arteriolar occlusions without uveitis or keratoconjunctivitis, mid-to-low frequency unilateral or bilateral sensorineural hearing loss, and numerous small foci of increased signal in the white and gray matter on T2-weighted brain magnetic resonance imaging. CONCLUSIONS: This rare syndrome often can be identified at an early stage with a careful history and physical examination. MRI, SPECT, retinal fluorescein angiography and audiometry will contribute to diagnosis.
OBJECTIVE: To investigate the clinic characters and diagnosis of Susac syndrome. METHODS: Two cases were systematically studied by the authors. RESULTS: All the two cases were young women. The clinical manifestations include acute and subacute multifocal and diffuse encephalopathic symptoms, hearing loss, and visual loss. Diagnosis is facilitated by demonstration of retinal arteriolar occlusions without uveitis or keratoconjunctivitis, mid-to-low frequency unilateral or bilateral sensorineural hearing loss, and numerous small foci of increased signal in the white and gray matter on T2-weighted brain magnetic resonance imaging. CONCLUSIONS: This rare syndrome often can be identified at an early stage with a careful history and physical examination. MRI, SPECT, retinal fluorescein angiography and audiometry will contribute to diagnosis.
Authors: Jan Dörr; Sarah Krautwald; Brigitte Wildemann; Sven Jarius; Marius Ringelstein; Thomas Duning; Orhan Aktas; Erich Bernd Ringelstein; Friedemann Paul; Ilka Kleffner Journal: Nat Rev Neurol Date: 2013-04-30 Impact factor: 42.937