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GLOBUS PALLIDUS HIGH–SIGNAL LESIONS: A PREDOMINANT MRI FINDING IN CHILDREN WITH NEUROFIBROMATOSIS TYPE 1
  1. Aravindhan Baheerathan
  1. University of Leicester

    Abstract

    Introduction Lesions of the brain recognised as unidentified bright objects (UBOs) are commonly observed as areas of increased T2–weighted signal intensity on Magnetic resonance imaging (MRI) in children with neurofibromatosis type 1 (NF1). Identification of these lesions is not currently encompassed in the National Institute of Health (NIH) diagnostic criteria for NF1.

    Objective We aimed to determine the prevalence of UBOs in children with NF1 and identify areas of the brain that are commonly affected by these lesions; allowing us to evaluate whether UBOs should be included in the diagnostic criteria for the diagnosis of NF1.

    Methods We reviewed the cranial MRI scans of 22 children who had been diagnosed with sporadic or familial NF1 in accordance to the criteria established by NIH. UBOs were present in 81% of children with NF1.

    Results These lesions have a predilection for specific areas of the brain, including the globus pallidus (72%), cerebellum (66%), brainstem (27%) and cerebral hemispheres (16%). The prevalence of UBOs identified varied significantly with age and sex; they were infrequent in children less than 4 years of age but were common in those aged between 4 and 12 years of age. UBOs were more commonly seen in males (66.6%) compared to females (33.3%). Repeat MRI scan on a subset of these patients with UBOs did not show any significant changes despite a worsening in clinical symptoms.

    Conclusion and discussion We have shown that UBOs are a common finding in children with NF1 and are most prevalent between the ages of 4 and 12. Many sites of the brain are affected by these lesions, most notably the globus pallidus and the cerebellum. Based on our findings and those of other published literature, we suggest that UBOs are incorporated as one of the criterion for the diagnosis of neurofibromatosis type 1 as it is likely to enhance the sensitivity of its clinical detection.

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