Hippocampal atrophy and developmental regression as first sign of linear scleroderma "en coup de sabre"

European Journal of Paediatric Neurology : EJPN : Official Journal of the European Paediatric Neurology Society
Helene E VerhelstRudy N Van Coster

Abstract

An 8-year-old girl with linear scleroderma "en coup de sabre" is reported who, at preschool age, presented with intractable simple partial seizures more than 1 year before skin lesions were first noticed. MRI revealed hippocampal atrophy, controlaterally to the seizures and ipsilaterally to the skin lesions. In the following months, a mental and motor regression was noticed. Cerebral CT scan showed multiple foci of calcifications in the affected hemisphere. In previously reported patients the skin lesions preceded the neurological signs. To the best of our knowledge, hippocampal atrophy was not earlier reported as presenting symptom of linear scleroderma. Linear scleroderma should be included in the differential diagnosis in patients with unilateral hippocampal atrophy even when the typical skin lesions are not present.

References

Feb 22, 2001·Journal of Neurology, Neurosurgery, and Psychiatry·J StoneM H Johnson
Sep 13, 2002·Journal of the European Academy of Dermatology and Venereology : JEADV·L Orozco-CovarrubiasR Ruiz-Maldonado
Dec 15, 2004·Neurology·Gregory Y ChangOh-Sang Kwon
Dec 21, 2004·The British Journal of Dermatology·S SatoK Takehara
Dec 22, 2004·Pediatric Allergy and Immunology : Official Publication of the European Society of Pediatric Allergy and Immunology·D C SalpietroM Vaccaro
Aug 19, 2005·Early Human Development·Sandra Rees, Terrie Inder
Oct 7, 2005·International Journal of Dermatology·Yoshihiro MimraKunihiko Tamaki
Dec 6, 2005·Pediatrics·Kristen E HollandBeth A Drolet
Dec 22, 2005·Rheumatology·F ZulianUNKNOWN Juvenile Scleroderma Working Group of the Pediatric Rheumatology European Society (PRES)
Jan 28, 2006·Annals of the Rheumatic Diseases·S AppenzellerF Cendes
Sep 26, 2006·Pediatric Neurology·Tom F D FarrowRichard A Grünewald
May 12, 2007·Epilepsy & Behavior : E&B·Christian G Bien, Christian E Elger

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Citations

Oct 30, 2015·Neurotherapeutics : the Journal of the American Society for Experimental NeuroTherapeutics·Eleni I KampylafkaAthanasios G Tzioufas
Jul 6, 2013·Seminars in Arthritis and Rheumatism·Tiago Nardi AmaralSimone Appenzeller
Feb 10, 2012·Autoimmune Diseases·Tiago Nardi AmaralSimone Appenzeller
May 8, 2016·Neurologia i neurochirurgia polska·Elzbieta Klimiec, Aleksandra Klimkowicz-Mrowiec
Apr 25, 2018·The Journal of Dermatology·Yoshihide AsanoHironobu Ihn
Jul 17, 2015·Medicine·Justine VixJean-Philippe Neau
Feb 12, 2020·Seizure : the Journal of the British Epilepsy Association·Ruben Rocha, Marios Kaliakatsos
Dec 17, 2009·Current Opinion in Rheumatology

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