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Central tegmental tract lesion in a girl with holoprosencephaly presenting with West syndrome
Authors:Hideto Yoshikawa  Kyoko Nakano  Shuei Watanabe
Affiliation:1. School of Health and Rehabilitation Sciences, Physical Therapy Division, The Ohio State University, Columbus, OH, United States;2. Biomedical Engineering, The Ohio State University, Columbus, OH, United States;3. Center for Brain and Spinal Cord Repair, The Ohio State University, Columbus, OH, United States;1. Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea;2. Department of Pediatrics, Samsung Changwon Hospital, Sungkyunkwan University School of Medicine, Changwon, Republic of Korea;1. Department of Pediatrics, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan;2. Department of Radiology, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan;1. Neurology Section, Department of Pediatrics, University of Arkansas for Medical Sciences, 1 Children’s Way, Little Rock, AR 72202, USA;2. Department of Pathology, University of Arkansas for Medical Sciences, Little Rock, AR, USA;3. Division of Neurosurgery, Arkansas Children’s Hospital, and Department of Neurosurgery, University of Arkansas for Medical Sciences, Little Rock, AR, USA
Abstract:We described a 16-month-old female patient who developed West syndrome at 3 months of age. MRI revealed a holoprosencephaly with incomplete fusion of the cerebrum, associated with central tegmental tract (CTT) lesions. At 1 year of age, the CTT lesion was still present on T2-weighted MRI. The CTT represents an important projection pathway of the extrapyramidal tract and the CTT lesions have rarely been reported using MRI in patients with neonatal hypoxic–ischemic encephalopathy and several inborn errors of metabolism. Although the exact mechanism remains obscure, we suggest that disturbances in midbrain fibers that connect to the basal ganglia, may have contributed to the etiology of West syndrome in this patient.
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