The idiopathic intracranial hypertension treatment trial: clinical profile at baseline

Michael Wall, Mark J Kupersmith, Karl D Kieburtz, James J Corbett, Steven E Feldon, Deborah I Friedman, David M Katz, John L Keltner, Eleanor B Schron, Michael P McDermott, NORDIC Idiopathic Intracranial Hypertension Study Group, Michael Wall, James Corbett, Steven Feldon, Deborah Friedman, John Keltner, Karl Kieburtz, Mark Kupersmith, Michael P McDermott, Eleanor B Schron, David Katz, Tippi Hales, Cindy Casaceli, Sanjay Kedhar, Flora Levin, Jonathan Feistmann, Katy Tai, Karen Tobias, Melissa Rivas, Lorena Dominguez, Reid Longmuir, Matthew Thurtell, Trina Eden, Randy Kardon, Robert Lesser, Danielle Rudich, Barbara Hart, Bonnie Carlstrom, Susan Allman, Anne Haroldsen, Byron L Lam, Joshua Pasol, Potyra R Rosa, Alexis Morante, Jennifer Verriotto, David Katz, Tracy Asbury, Robert Gerwin, Mary Barnett, Steven Hamilton, Caryl Tongco, Beena Gangadharan, Eugene May, Dean A McGee, Anil Patel, R Michael Siatkowsk, Heather Miller, Vanessa Bergman, Kammerin White, Steven O'Dell, Joseph Andrezik, Timothy Tytle, Kenneth Shindler, Joan Dupont, Rebecca Salvo, Jonathan Lo, Stephanie Engelhard, Sami Khella, Madhura Tamhankar, Gregory Van Stavern, Jamie Kambarian, Renee Van Stavern, Karen Civitelli, Beau B Bruce, Valérie Biousse, Nancy J Newman, Judy Brower, Linda Curtis, Lanning Kline, Roy McDonald, J Givre, Tippi Hales, Kenneth M Carnes, Kimberly James, Marisol Ragland, John T Lind, George O'Gara, Kari Steinmetz, Mare Perevich, Elisabeth Carter, Rajeev Ramchandran, Steven Katz, Marc Criden, Andrea Inman, Prem S Subramanian, N Hoffman, M Michaele Hartnett, Madiha Siddiqui, Ellen Arnold, Jeff Boring, Neil R Miller, Peter Quiros, Sylvia Ramos, Margaret Padilla, Lupe Cisneros, Carlos Filipe Chicani, Kevin Na, Rosa Tang, Laura Frishman, Priscilla Cajavilca, Sheree Newland, Liat Gantz, Maria Guillermo Prieto, Anastas Pass, Nicky R Holdeman, Michael S Lee, Helen Roemhild, Wendy Elasky, Jody Fissgus, Julie Falardeau, William Hills, Cristi Bryant, Donna Kim, Rebecca Armour, Lori Higginbotham, Steven A Newman, Kristina Holbrook, Laura D Cook, Janis Beall, William Hall, Debbie Hamilton, Jeannie Reimer, Jeri Nickerson, Fiona Costello, Vivian Rismondo-Stankovich, Maureen Flanagan, Allison Jensen, Ann Marie, Ruth Tenzler, Robert Honkanen, Joseph Rizzo, Dean Cestari, Neal Snebold, Brian Vatcher, Christine Matera, Edward Miretsky, Judith Oakley, Josyane Dumser, Ursula Bator, Barbara Barrett, Charlene Callahan, Sarah Brett, Kamella Zimmerman, Marcia Grillo, Karen Capaccioli, M Tariq Bhatti, LaToya Greene, Maria Cecilia Santiago-Turla, Noreen McClain, John E Carter, Patrick O'Connor, Daniel Mojica, Joan Smith, Yolanda Trigo, Sherry Slayman Kellogg, Alexandra Martinez, Paul Comeau, Andres Sanchez, Nathan McCarthy, Erika Perez, Carlos Bazan, H Logan Brooks Jr, Ronda Gorsica, Brian Sherman, Joel Kramer, Larry Frohman, Amanda Ribeiro, Kathryn Boschert, Yu fei Tu, Susan Rivera, Roger Turbin, Craig Simms, Mary Kemp, Jim Farmer, Robert Granadier, Tammy Osentoski, Bobbie Lewis, Lori Stec, Jorge C Kattah, John Pula, Mary Rose Buttice, Kimberly DuPage, Kimberly Cooley, Judith Beck, Lynn Bannon, Cynthia Guede, Luis Mejico, Melissa Ko, Burk Jubelt, Megan Grosso, Mark Chilton, Mary LouWatson, Jennifer Moore, Tim Martin, Cara Everhart, Joan Fish, Lori Cooke, J Paul, Marie D Acierno, Amy Thomassie, Aravinda Rao, Trisha Mary Chiasson, Janet C Rucker, Ilana Katz-Sand, Deepali Rajguru, Sachin Kedar, Stephanie Morris, Andrew Pearson, Mike Hanson, Betty Kovacs, Xavier Pi-Sunyer, William Fisher, Dorothea Castillo, Valerie Davis, Lourdes Fagan, Rachel Hollar, Peter MacDowell, John Keltner, Kim Plumb, Danielle Harvey, Kim Plumb, Laura Leming, Shan Gao, Xin Tu, Debbie Baker, Karen Helles, Larry Preston, Deborah Friedman, O Iyore Ayanru, Pravin Patel, William Hart Jr, Joanne Katz, David Kaufman, Michael Wall, Mark J Kupersmith, Karl D Kieburtz, James J Corbett, Steven E Feldon, Deborah I Friedman, David M Katz, John L Keltner, Eleanor B Schron, Michael P McDermott, NORDIC Idiopathic Intracranial Hypertension Study Group, Michael Wall, James Corbett, Steven Feldon, Deborah Friedman, John Keltner, Karl Kieburtz, Mark Kupersmith, Michael P McDermott, Eleanor B Schron, David Katz, Tippi Hales, Cindy Casaceli, Sanjay Kedhar, Flora Levin, Jonathan Feistmann, Katy Tai, Karen Tobias, Melissa Rivas, Lorena Dominguez, Reid Longmuir, Matthew Thurtell, Trina Eden, Randy Kardon, Robert Lesser, Danielle Rudich, Barbara Hart, Bonnie Carlstrom, Susan Allman, Anne Haroldsen, Byron L Lam, Joshua Pasol, Potyra R Rosa, Alexis Morante, Jennifer Verriotto, David Katz, Tracy Asbury, Robert Gerwin, Mary Barnett, Steven Hamilton, Caryl Tongco, Beena Gangadharan, Eugene May, Dean A McGee, Anil Patel, R Michael Siatkowsk, Heather Miller, Vanessa Bergman, Kammerin White, Steven O'Dell, Joseph Andrezik, Timothy Tytle, Kenneth Shindler, Joan Dupont, Rebecca Salvo, Jonathan Lo, Stephanie Engelhard, Sami Khella, Madhura Tamhankar, Gregory Van Stavern, Jamie Kambarian, Renee Van Stavern, Karen Civitelli, Beau B Bruce, Valérie Biousse, Nancy J Newman, Judy Brower, Linda Curtis, Lanning Kline, Roy McDonald, J Givre, Tippi Hales, Kenneth M Carnes, Kimberly James, Marisol Ragland, John T Lind, George O'Gara, Kari Steinmetz, Mare Perevich, Elisabeth Carter, Rajeev Ramchandran, Steven Katz, Marc Criden, Andrea Inman, Prem S Subramanian, N Hoffman, M Michaele Hartnett, Madiha Siddiqui, Ellen Arnold, Jeff Boring, Neil R Miller, Peter Quiros, Sylvia Ramos, Margaret Padilla, Lupe Cisneros, Carlos Filipe Chicani, Kevin Na, Rosa Tang, Laura Frishman, Priscilla Cajavilca, Sheree Newland, Liat Gantz, Maria Guillermo Prieto, Anastas Pass, Nicky R Holdeman, Michael S Lee, Helen Roemhild, Wendy Elasky, Jody Fissgus, Julie Falardeau, William Hills, Cristi Bryant, Donna Kim, Rebecca Armour, Lori Higginbotham, Steven A Newman, Kristina Holbrook, Laura D Cook, Janis Beall, William Hall, Debbie Hamilton, Jeannie Reimer, Jeri Nickerson, Fiona Costello, Vivian Rismondo-Stankovich, Maureen Flanagan, Allison Jensen, Ann Marie, Ruth Tenzler, Robert Honkanen, Joseph Rizzo, Dean Cestari, Neal Snebold, Brian Vatcher, Christine Matera, Edward Miretsky, Judith Oakley, Josyane Dumser, Ursula Bator, Barbara Barrett, Charlene Callahan, Sarah Brett, Kamella Zimmerman, Marcia Grillo, Karen Capaccioli, M Tariq Bhatti, LaToya Greene, Maria Cecilia Santiago-Turla, Noreen McClain, John E Carter, Patrick O'Connor, Daniel Mojica, Joan Smith, Yolanda Trigo, Sherry Slayman Kellogg, Alexandra Martinez, Paul Comeau, Andres Sanchez, Nathan McCarthy, Erika Perez, Carlos Bazan, H Logan Brooks Jr, Ronda Gorsica, Brian Sherman, Joel Kramer, Larry Frohman, Amanda Ribeiro, Kathryn Boschert, Yu fei Tu, Susan Rivera, Roger Turbin, Craig Simms, Mary Kemp, Jim Farmer, Robert Granadier, Tammy Osentoski, Bobbie Lewis, Lori Stec, Jorge C Kattah, John Pula, Mary Rose Buttice, Kimberly DuPage, Kimberly Cooley, Judith Beck, Lynn Bannon, Cynthia Guede, Luis Mejico, Melissa Ko, Burk Jubelt, Megan Grosso, Mark Chilton, Mary LouWatson, Jennifer Moore, Tim Martin, Cara Everhart, Joan Fish, Lori Cooke, J Paul, Marie D Acierno, Amy Thomassie, Aravinda Rao, Trisha Mary Chiasson, Janet C Rucker, Ilana Katz-Sand, Deepali Rajguru, Sachin Kedar, Stephanie Morris, Andrew Pearson, Mike Hanson, Betty Kovacs, Xavier Pi-Sunyer, William Fisher, Dorothea Castillo, Valerie Davis, Lourdes Fagan, Rachel Hollar, Peter MacDowell, John Keltner, Kim Plumb, Danielle Harvey, Kim Plumb, Laura Leming, Shan Gao, Xin Tu, Debbie Baker, Karen Helles, Larry Preston, Deborah Friedman, O Iyore Ayanru, Pravin Patel, William Hart Jr, Joanne Katz, David Kaufman

Abstract

Importance: To our knowledge, there are no large prospective cohorts of untreated patients with idiopathic intracranial hypertension (IIH) to characterize the disease.

Objective: To report the baseline clinical and laboratory features of patients enrolled in the Idiopathic Intracranial Hypertension Treatment Trial.

Design, setting, and participants: We collected data at baseline from questionnaires, examinations, automated perimetry, and fundus photography grading. Patients (n = 165) were enrolled from March 17, 2010, to November 27, 2012, at 38 academic and private practice sites in North America. All participants met the modified Dandy criteria for IIH and had a perimetric mean deviation between -2 dB and -7 dB. All but 4 participants were women.

Main outcomes and measures: Baseline and laboratory characteristics.

Results: The mean (SD) age of our patients was 29.0 (7.4) years and 4 (2.4%) were men. The average (SD) body mass index (calculated as weight in kilograms divided by height in meters squared) was 39.9 (8.3). Headache was the most common symptom (84%). Transient visual obscurations occurred in 68% of patients, back pain in 53%, and pulse synchronous tinnitus in 52%. Only 32% reported visual loss. The average (SD) perimetric mean deviation in the worst eye was -3.5 (1.1) dB, (range, -2.0 to -6.4 dB) and in the best eye was -2.3 (1.1) dB (range, -5.2 to 0.8 dB). A partial arcuate visual field defect with an enlarged blind spot was the most common perimetric finding. Visual acuity was 85 letters or better (20/20) in 71% of the worst eyes and 77% of the best eyes. Quality of life measures, including the National Eye Institute Visual Function Questionnaire-25 and the Short Form-36 physical and mental health summary scales, were lower compared with population norms.

Conclusions and relevance: The Idiopathic Intracranial Hypertension Treatment Trial represents the largest prospectively analyzed cohort of untreated patients with IIH. Our data show that IIH is almost exclusively a disease of obese young women. Patients with IIH with mild visual loss have typical symptoms, may have mild acuity loss, and have visual field defects, with predominantly arcuate loss and enlarged blind spots that require formal perimetry for detection.

Trial registration: clinicaltrials.gov Identifier: NCT01003639.