Report of a Turkish child with Sjören-Larsson syndrome associated with peripheral nerve involvement.
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.
Akdeniz
Necmettin
N
Department of Dermatology, Yüzüncü Yil University Faculty of Medicine, Van, Turkey.
Calka
Omer
O
Anlar
Omer
O
Akbayram
Sinan
S
Caksen
Hüseyin
H
Metin
Ahmet
A
Cinal
Adnan
A
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