Kshitij Mankad1, Despina Eleftheriou2,3, Harry Knights4,5, Elizabeth Minas6, Faraan Khan7, Lindsay Shaw8, Muthana Al Obaidi2. 1. Department of Radiology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK. 2. Department of Paediatric Rheumatology, University College London Institute of Child Health and Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK. 3. Centre for Adolescent Rheumatology Versus Arthritis, University College London, London, UK. 4. Department of Paediatric Rheumatology, University College London Institute of Child Health and Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK. hdk116@ic.ac.uk. 5. Imperial College School of Medicine, Imperial College London, London, UK. hdk116@ic.ac.uk. 6. Department of Radiology, Charing Cross Hospital, London, UK. 7. Department of Radiology, St George's University Hospitals NHS Foundation Trust, London, UK. 8. Department of Paediatric Dermatology, University College London Institute of Child Health and Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
Abstract
BACKGROUND: The aim of this study was to: (i) describe the abnormalities seen on brain imaging in a group of children with en coup de sabre (EDCS) with/without Parry-Romberg syndrome (PRS); and (ii) identify clinical predictors of brain imaging abnormalities. METHODS: This was a single centre (Great Ormond Street Hospital, London) retrospective case series of patients with ECDS/PRS seen from 2000 to 2018. We identified patients with cutaneous manifestations consistent with the clinical descriptions of ECDS/PRS. Presenting clinical, laboratory, and radiological brain findings are described. Results are expressed as medians and ranges or frequencies and percentages. Fisher's exact test was used to identify clinical associations with magnetic resonance imaging (MRI) abnormalities. RESULTS: Fourteen patients were studied: 6 males and 8 females; median age 14 years (range 3-20). We observed neuroimaging abnormalities in 2/6 ECDS and 5/8 ECDS/PRS patients. White matter signal abnormality, dystrophic calcification, leptomeningeal enhancement, and sulcal crowding were the typical findings on brain imaging. A total of 50% of patients had no MRI abnormality despite some of these patients having neurological symptoms. The presence of seizures was significantly associated with ipsilateral enhanced white matter signalling on MRI (p < 0.05). CONCLUSIONS: In summary, we observed several distinct radiographic patterns associated with ECDS/PRS. Seizure disorder was strongly associated with the presence of ipsilateral enhanced white matter signalling. Improved neuroimaging techniques that combine morphological with functional imaging may improve the detection rate of brain involvement in children with ECDS/PRS in the future.
BACKGROUND: The aim of this study was to: (i) describe the abnormalities seen on brain imaging in a group of children with en coup de sabre (EDCS) with/without Parry-Romberg syndrome (PRS); and (ii) identify clinical predictors of brain imaging abnormalities. METHODS: This was a single centre (Great Ormond Street Hospital, London) retrospective case series of patients with ECDS/PRS seen from 2000 to 2018. We identified patients with cutaneous manifestations consistent with the clinical descriptions of ECDS/PRS. Presenting clinical, laboratory, and radiological brain findings are described. Results are expressed as medians and ranges or frequencies and percentages. Fisher's exact test was used to identify clinical associations with magnetic resonance imaging (MRI) abnormalities. RESULTS: Fourteen patients were studied: 6 males and 8 females; median age 14 years (range 3-20). We observed neuroimaging abnormalities in 2/6 ECDS and 5/8 ECDS/PRSpatients. White matter signal abnormality, dystrophic calcification, leptomeningeal enhancement, and sulcal crowding were the typical findings on brain imaging. A total of 50% of patients had no MRI abnormality despite some of these patients having neurological symptoms. The presence of seizures was significantly associated with ipsilateral enhanced white matter signalling on MRI (p < 0.05). CONCLUSIONS: In summary, we observed several distinct radiographic patterns associated with ECDS/PRS. Seizure disorder was strongly associated with the presence of ipsilateral enhanced white matter signalling. Improved neuroimaging techniques that combine morphological with functional imaging may improve the detection rate of brain involvement in children with ECDS/PRS in the future.
Entities:
Keywords:
En coup de sabre; Epilepsy; Linear scleroderma; Magnetic resonance imaging; Parry-Romberg syndrome
Authors: C De la Garza-Ramos; A Jain; S A Montazeri; L Okromelidze; R McGeary; A A Bhatt; S J S Sandhu; S S Grewal; A Feyissa; J I Sirven; A L Ritaccio; W O Tatum; V Gupta; E H Middlebrooks Journal: AJNR Am J Neuroradiol Date: 2022-06 Impact factor: 4.966