Magnetic resonance imaging findings in children with Parry-R
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The aim of this study is to explain the abnormalities seen on brain imaging in a group of children with en coup de sabre (EDCS) with or without Parry-Romberg syndrome (PRS) and to find clinical predictors of these abnormalities.

This was a retrospective case series of patients with ECDS/PRS from a single center. Patients with cutaneous manifestations were found in line with ECDS/PRS clinical definitions. The clinical, laboratory, and radiological findings in the brain are identified. The data is presented in the form of medians and ranges, as well as frequencies and percentages. Medical associations with magnetic resonance imaging (MRI) anomalies were identified using Fisher's exact test.

Results:
--14 patients were studied: 6 males and 8 females; median age 14-years (range 3–20).

--Neuroimaging abnormalities was observed 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.

In conclusion, there were several distinct radiographic trends associated with ECDS/PRS. The development of ipsilaterally enhanced white matter signaling was closely linked to seizure disorder. In the future, improved neuroimaging techniques that combine morphological and functional imaging can increase the rate of brain involvement detection in children with ECDS/PRS.

Source: https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-021-00512-6
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