caa a22 4500 465767087 CHVBK 20180323111917.0 cr unu---uuuuu 170327e19900401xx s 000 0 eng 10.1007/BF00294250 doi (NATIONALLICENCE)springer-10.1007/BF00294250 Hypoplasia of posterior spinal roots and dorsal spinal tracts with arthrogryposis multiplex congenita [Elektronische Daten] [H. Vogel, D. Halpert, D. Horoupian] Summary: We report a male infant with arthrogryposis multiplex congenita (AMC) who survived for 19 weeks following birth at 36 weeks gestational age. No heritable or acquired cause of neuromuscular disease was found. He manifested joint contractures of upper and lower extremities, diffuse hypotonia requiring ventilatory support, and areflexia; the general examination also showed facial dysmorphisms, and an ichthyotic rash. Pathological examination of the brain and spinal cord revealed severe hypoplasia of dorsal roots and posterior columns, nondecussation of pyramidal tracts, and anterior horns of an unusual configuration: the brain was normal, and the cerebellum contained Purkinje cell heterotopias. Muscle spindles could not be identified. To our knowledge, these spinal cord abnormalities in association with AMC have not been previously reported, thus raising interesting speculations about the possible role of such abnormalities in the pathogenesis of AMC. Springer-Verlag, 1990 Arthrogryposis multiplex congenita nationallicence Spinal cord dysplasia nationallicence Hypoplasia, posterior spinal roots nationallicence Spinal root ganglia, rudimentary nationallicence Dysplasia, inferior olivary nuclei nationallicence Vogel H. Department of Pathology (Neuropathology), Stanford University Medical Center, 94305, Stanford, CA, USA aut Halpert D. Department of Pathology (Neuropathology), Stanford University Medical Center, 94305, Stanford, CA, USA aut Horoupian D. Department of Pathology (Neuropathology), Stanford University Medical Center, 94305, Stanford, CA, USA aut Acta Neuropathologica Springer-Verlag 79/6(1990-04-01), 692-696 0001-6322 79:6<692 1990 79 401 https://doi.org/10.1007/BF00294250 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/BF00294250 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Vogel H. Department of Pathology (Neuropathology), Stanford University Medical Center, 94305, Stanford, CA, USA aut NATIONALLICENCE 700 1- Halpert D. Department of Pathology (Neuropathology), Stanford University Medical Center, 94305, Stanford, CA, USA aut NATIONALLICENCE 700 1- Horoupian D. Department of Pathology (Neuropathology), Stanford University Medical Center, 94305, Stanford, CA, USA aut NATIONALLICENCE 773 0- Acta Neuropathologica Springer-Verlag 79/6(1990-04-01), 692-696 0001-6322 79:6<692 1990 79 401 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer