Anita A Kohli1, Arastoo Vossough2, Robert M Mallery3, John H Woo4, Claire A Sheldon5, Grace L Paley6, Kathleen B Digre7, Deborah I Friedman8, John T Farrar9, Shana E McCormack10, Grant T Liu11, Christina L Szperka12. 1. Department of Ophthalmology and Visual Science, Yale School of Medicine, New Haven, Connecticut. 2. Division of Radiology at the Children's Hospital of Philadelphia, Philadelphia, Pennsylvania. 3. Department of Neurology, Brigham and Women's Hospital, Boston, Massachusetts. 4. Department of Radiology, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania. 5. Department of Ophthalmology & Visual Sciences, The University of British Columbia, Vancouver, British Columbia Canada. 6. Department of Ophthalmology, Washington University School of Medicine in St. Louis, St. Louis, Missouri. 7. John A. Moran Eye Center, University of Utah, Salt Lake City, Utah. 8. Department of Neurology & Neurotherapeutics and Ophthalmology, University of Texas Southwestern Medical Center, Dallas, Texas. 9. Center for Clinical Epidemiology & Statistics, University of Pennsylvania Perelman School of Medicine, Philadelphia, Pennsylvania. 10. Division of Endocrinology & Diabetes, Children's Hospital of Philadelphia, 3401 Civic Center Blvd, Philadelphia, Pennsylvania. 11. Division of Ophthalmology, Neuro-Ophthalmology Service, Children's Hospital of Philadelphia, 3401 Civic Center Blvd, Philadelphia, Pennsylvania; The Scheie Eye Institute, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania; Department of Neurology, Hospital of the University of Pennsylvania Philadelphia, Pennsylvania. 12. Department of Neurology, Hospital of the University of Pennsylvania Philadelphia, Pennsylvania; Division of Neurology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania. Electronic address: Szperka@email.chop.edu.
Abstract
BACKGROUND: Revised diagnostic criteria for pseudotumor cerebri syndrome require three of four neuroimaging findings in the absence of papilledema. We examined the sensitivity and specificity of three or more of four of these magnetic resonance imaging (MRI) findings for pseudotumor cerebri syndrome in children. METHODS: As part of clinical care, patients in whom there was suspicion for pseudotumor cerebri syndrome underwent neurological and fundoscopic examinations, lumbar puncture, MRI, or magnetic resonance venogram. For this retrospective study, we used this information to classify 119 subjects into definite (n = 66) or probable pseudotumor cerebri syndrome (n = 12), elevated opening pressure without papilledema (n = 23), or controls who had normal opening pressure without papilledema (n = 24). A neuroradiologist, unaware of the clinical findings or original MRI report, reviewed MRIs for pituitary gland flattening, flattening of the posterior sclera, optic nerve sheath distention, and transverse venous sinus stenosis. RESULTS: The presence of three or more MRI findings has a sensitivity of 62% (95% confidence interval: 47% to 75%) and a specificity of 95% (95% confidence interval: 77% to 100%), compared with controls. Two of three (transverse venous sinus stenosis, pituitary gland flattening, flattening of the posterior sclera) had a similar sensitivity and specificity. Transverse venous sinus stenosis alone had a slightly higher sensitivity (74%, 95% confidence interval: 60% to 85%) and specificity (100%, 95% confidence interval: 80% to 100%). CONCLUSIONS: In children, three of four of the proposed neuroimaging criteria and transverse venous sinus stenosis alone have a moderate sensitivity and robust specificity for pseudotumor cerebri syndrome. MRIs should be reviewed for these criteria, and their presence should raise suspicion for pseudotumor cerebri syndrome in children, particularly if the presence of papilledema is uncertain.
BACKGROUND: Revised diagnostic criteria for pseudotumor cerebri syndrome require three of four neuroimaging findings in the absence of papilledema. We examined the sensitivity and specificity of three or more of four of these magnetic resonance imaging (MRI) findings for pseudotumor cerebri syndrome in children. METHODS: As part of clinical care, patients in whom there was suspicion for pseudotumor cerebri syndrome underwent neurological and fundoscopic examinations, lumbar puncture, MRI, or magnetic resonance venogram. For this retrospective study, we used this information to classify 119 subjects into definite (n = 66) or probable pseudotumor cerebri syndrome (n = 12), elevated opening pressure without papilledema (n = 23), or controls who had normal opening pressure without papilledema (n = 24). A neuroradiologist, unaware of the clinical findings or original MRI report, reviewed MRIs for pituitary gland flattening, flattening of the posterior sclera, optic nerve sheath distention, and transverse venous sinus stenosis. RESULTS: The presence of three or more MRI findings has a sensitivity of 62% (95% confidence interval: 47% to 75%) and a specificity of 95% (95% confidence interval: 77% to 100%), compared with controls. Two of three (transverse venous sinus stenosis, pituitary gland flattening, flattening of the posterior sclera) had a similar sensitivity and specificity. Transverse venous sinus stenosis alone had a slightly higher sensitivity (74%, 95% confidence interval: 60% to 85%) and specificity (100%, 95% confidence interval: 80% to 100%). CONCLUSIONS: In children, three of four of the proposed neuroimaging criteria and transverse venous sinus stenosis alone have a moderate sensitivity and robust specificity for pseudotumor cerebri syndrome. MRIs should be reviewed for these criteria, and their presence should raise suspicion for pseudotumor cerebri syndrome in children, particularly if the presence of papilledema is uncertain.
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