A case report of infantile striatal necrosis with an acute onset |
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| Authors: | S Yasukohchi T Akabane T Mori K Tamagawa Y Morimatsu |
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| Affiliation: | 1. Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Nagano;2. Department of Pediatrics, National Sanatorium Higashi-Matsumoto Hospital, Matsumoto, Nagano;3. Department of Pediatrics, Tokyo Metropolitan Neurological Hospital, Fuchu, Tokyo;4. Division of Clinical Neuropathology, Tokyo Metropolitan Institute for Neuroscience, Fuchu, Tokyo;1. Service de rhumatologie, hôpital Saint-Camille, 2, rue des Pères Camilliens, 94360 Bry-sur-Marne, France;2. Service de médecine vasculaire, groupe hospitalier Paris Saint-Joseph, 185, rue Raymond-Losserand, 75014 Paris, France |
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| Abstract: | We report here an autopsy case, an 8-year-old boy diagnosed as having infantile striatal necrosis, characterized by a preceding febrile illness followed by acute encephalopathy with abrupt obtundation, seizures and dystonia, with remarkable improvement of the disturbed consciousness and intelligence after TRH-T therapy. These clinical symptoms were linked with bilateral necrosis of the striata on CT scanning. The presented case belonged to a newly described subgroup of the heredogenous disorders that produce necrosis of the putamina in children. |
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