title: Report of a Turkish child with Sjören-Larsson syndrome associated with peripheral nerve involvement.
abstract: Sjören-Larsson syndrome is a rare hereditary neurocutaneous disorder characterized by ichthyosis, spastic di- or tetra-plegia, and mild to moderate mental retardation. In this article, we present a nine-year-old girl with the classical features of the syndrome associated with peripheral nerve involvement because of its rare presentation. To the best of our knowledge, only three cases of Sjören-Larsson syndrome with peripheral nerve involvement have been previously reported in the literature. We assume that Sjören-Larsson syndrome involves extensive disorders of the ectodermal tissues, including the peripheral nerves as well as the skin and the central nervous system.
authors.affiliation: Department of Dermatology, Yüzüncü Yil University Faculty of Medicine, Van, Turkey.
authors.forename: Necmettin
authors.initials: N
authors.lastname: Akdeniz
authors.forename: Omer
authors.initials: O
authors.lastname: Calka
authors.forename: Omer
authors.initials: O
authors.lastname: Anlar
authors.forename: Sinan
authors.initials: S
authors.lastname: Akbayram
authors.forename: Hüseyin
authors.initials: H
authors.lastname: Caksen
authors.forename: Ahmet
authors.initials: A
authors.lastname: Metin
authors.forename: Adnan
authors.initials: A
authors.lastname: Cinal
mesh_terms: D002648:Child; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D009128:Muscle Spasticity / Q000150:complications / Q000175:diagnosis; D020335:Paraparesis / Q000150:complications / Q000175:diagnosis; D010523:Peripheral Nervous System Diseases / Q000150:complications / Q000175:diagnosis*; D035583:Rare Diseases; D012720:Severity of Illness Index; D016111:Sjogren-Larsson Syndrome / Q000150:complications / Q000175:diagnosis*; D014421:Turkey