Unilateral Neuroimaging Findings in Pediatric Craniofacial Scleroderma: Parry-Romberg Syndrome and En Coup de Sabre.

Journal of Child Neurology
Ronald R SeeseKathryn S Torok

Abstract

Parry-Romberg syndrome (PRS) and en coup de sabre (ECDS) are subtypes of craniofacial localized scleroderma. Systematic analyses of central nervous system imaging findings and their clinical associations in children are lacking. Here, we aim to characterize neuroimaging findings and associated neurological symptoms in these conditions. Neuroimaging and neurological symptoms of children evaluated at our institution with a diagnosis of PRS or ECDS were retrospectively reviewed. Laterality, location, stability, and number of lesion(s) were evaluated, as was the presence of susceptibility lesion(s) and contrast enhancement. History of seizures or headaches was noted. From 2003 to 2019, 80 patients with PRS or ECDS were followed at our institution. Neuroimaging was completed in 73 and found to be abnormal in 25. In 12 (48%) of these 25 cases, headaches and/or seizures were present. In the vast majority of these cases (22/25, 88%), lesions were ipsilateral to skin findings. White matter was involved in 19 (76%) patients. MRI abnormalities preceded a rheumatological diagnosis in 7 (28%). Susceptibility lesions were noted in 11 (44%), and 8 (73%) of these patients endorsed a history of headaches. Most lesions were in the supratentorial...Continue Reading

References

Feb 22, 2001·Journal of Neurology, Neurosurgery, and Psychiatry·J StoneM H Johnson
Aug 28, 2002·Neurology·Anna PichiecchioFederico Zappoli
Aug 13, 2003·Neurology·J R ShahH T Chugani
Dec 6, 2005·Pediatrics·Kristen E HollandBeth A Drolet
Oct 11, 2007·AJNR. American Journal of Neuroradiology·K A TongE M Haacke
Mar 20, 2009·Current Rheumatology Reports·Jo Nadine FlemingStephen Mark Schwartz
May 15, 2010·Interventional Neuroradiology : Journal of Peritherapeutic Neuroradiology, Surgical Procedures and Related Neurosciences·T BosmanP A Brouwer
Aug 31, 2010·Journal of Neurology, Neurosurgery, and Psychiatry·Brian D MoseleyCheolsu Shin
Mar 13, 2012·Journal of the American Academy of Dermatology·Jessica El-KehdyNelly Rubeiz
Jun 13, 2015·AJNR. American Journal of Neuroradiology·M WongD R Shatzkes
May 8, 2018·European Journal of Pediatrics·Tamás ConstantinKathryn S Torok
Mar 4, 2019·Annals of the Rheumatic Diseases·Francesco ZulianIvan Foeldvari
May 23, 2019·Frontiers in Immunology·Kathryn S TorokTheresa T Lu

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Citations

Dec 22, 2021·Pediatric Dermatology·Daniel H GlaserKathryn S Torok

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