Comorbidities of Rare Epilepsies: Results from the Rare Epilepsy Network

Rare Epilepsy Network Steering Committee

Research output: Contribution to journalArticle

Abstract

Objective: To describe the prevalence and characteristics of comorbidities in persons with rare epilepsies. Study design: Persons with rare epilepsies and caregivers of those affected were recruited through the Epilepsy Foundation and more than 30 rare epilepsy advocacy organizations affiliated with the Rare Epilepsy Network (REN). A web-based survey was conducted using a questionnaire consisting of core sections to collect data from affected persons on various aspects, including comorbidities. Comorbidity information was grouped into 15 classes, 12 of which had a stem question followed by detailed branch questions and 3 that were created from a combination of related questions. Results: Of 795 persons with more than 30 different rare epilepsy diagnosis groups, one-half had ≥5 comorbidity classes and 97% were classified as complex chronic disease (C-CD). The highest number of comorbidity classes reported per person were persons with Aicardi syndrome, Phelan-McDermid syndrome (median, 7.0; IQR, 5.0-9.0), and tuberous sclerosis complex (median, 6.0; IQR, 4.0-8.0). The most common comorbidity classes were learning/developmental disability (71%), mental health issues (71%), sleep disorders (60%), brain abnormalities (52%), oral issues (49%), bone-joint issues (42%), hyper/hypotonia (42%), and eye-vision disorders (38%). The prevalence of brain abnormalities, hyper/hypotonia, eye, and cardiac disorders was significantly higher in persons first diagnosed with epilepsy at a younger age (<9 months) than in those first diagnosed at an older age (P <.05 for trend). Conclusions: Nearly all persons with rare epilepsies are medically complex, with a high prevalence of multiple comorbidities, especially those who were diagnosed with epilepsy in the first year of life. Comorbidities should be carefully considered in the diagnosis and management of persons with rare epilepsies.

Original languageEnglish (US)
Pages (from-to)249-258.e5
JournalJournal of Pediatrics
Volume203
DOIs
StatePublished - Dec 1 2018

Fingerprint

Comorbidity
Epilepsy
Muscle Hypotonia
Aicardi Syndrome
Developmental Disabilities
Tuberous Sclerosis
Learning Disorders
Vision Disorders
Brain
Caregivers
Mental Health
Chronic Disease
Joints
Organizations
Bone and Bones

Keywords

  • comorbidity
  • prevalence
  • questionnaire
  • rare epilepsy

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Comorbidities of Rare Epilepsies : Results from the Rare Epilepsy Network. / Rare Epilepsy Network Steering Committee.

In: Journal of Pediatrics, Vol. 203, 01.12.2018, p. 249-258.e5.

Research output: Contribution to journalArticle

Rare Epilepsy Network Steering Committee. / Comorbidities of Rare Epilepsies : Results from the Rare Epilepsy Network. In: Journal of Pediatrics. 2018 ; Vol. 203. pp. 249-258.e5.
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abstract = "Objective: To describe the prevalence and characteristics of comorbidities in persons with rare epilepsies. Study design: Persons with rare epilepsies and caregivers of those affected were recruited through the Epilepsy Foundation and more than 30 rare epilepsy advocacy organizations affiliated with the Rare Epilepsy Network (REN). A web-based survey was conducted using a questionnaire consisting of core sections to collect data from affected persons on various aspects, including comorbidities. Comorbidity information was grouped into 15 classes, 12 of which had a stem question followed by detailed branch questions and 3 that were created from a combination of related questions. Results: Of 795 persons with more than 30 different rare epilepsy diagnosis groups, one-half had ≥5 comorbidity classes and 97{\%} were classified as complex chronic disease (C-CD). The highest number of comorbidity classes reported per person were persons with Aicardi syndrome, Phelan-McDermid syndrome (median, 7.0; IQR, 5.0-9.0), and tuberous sclerosis complex (median, 6.0; IQR, 4.0-8.0). The most common comorbidity classes were learning/developmental disability (71{\%}), mental health issues (71{\%}), sleep disorders (60{\%}), brain abnormalities (52{\%}), oral issues (49{\%}), bone-joint issues (42{\%}), hyper/hypotonia (42{\%}), and eye-vision disorders (38{\%}). The prevalence of brain abnormalities, hyper/hypotonia, eye, and cardiac disorders was significantly higher in persons first diagnosed with epilepsy at a younger age (<9 months) than in those first diagnosed at an older age (P <.05 for trend). Conclusions: Nearly all persons with rare epilepsies are medically complex, with a high prevalence of multiple comorbidities, especially those who were diagnosed with epilepsy in the first year of life. Comorbidities should be carefully considered in the diagnosis and management of persons with rare epilepsies.",
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author = "{Rare Epilepsy Network Steering Committee} and Ho, {Nhan Thi} and Barbara Kroner and Zachary Grinspan and Brandy Fureman and Kathleen Farrell and Jingzhou Zhang and Janice Buelow and Hesdorffer, {Dale C.} and Brianne McDonald and Monica Weldon and Juliann Bradish and Vanessa Vogel-Farley and Paige Nues and Tracy Dixon-Salazar and Geraldine Bliss and Yssa DeWoody and Nakagawa, {Jo Anne} and Barbara Kroner and Michael Harris and Glen Arm and Heather Jackson and Ilene Miller and Gina Vozenilek and Nora Wong and Audrey Davidow and Kira Wagner and Hecker, {Jay Etta} and Lynn Egan and Heidi Grabenstatter and Meskis, {Mary Anne} and Mike Bartenhagen and Scotty Sims and Julie Walters and Lisa Schoyer and Kim Nye",
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AU - Rare Epilepsy Network Steering Committee

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AU - Kroner, Barbara

AU - Grinspan, Zachary

AU - Fureman, Brandy

AU - Farrell, Kathleen

AU - Zhang, Jingzhou

AU - Buelow, Janice

AU - Hesdorffer, Dale C.

AU - McDonald, Brianne

AU - Weldon, Monica

AU - Bradish, Juliann

AU - Vogel-Farley, Vanessa

AU - Nues, Paige

AU - Dixon-Salazar, Tracy

AU - Bliss, Geraldine

AU - DeWoody, Yssa

AU - Nakagawa, Jo Anne

AU - Kroner, Barbara

AU - Harris, Michael

AU - Arm, Glen

AU - Jackson, Heather

AU - Miller, Ilene

AU - Vozenilek, Gina

AU - Wong, Nora

AU - Davidow, Audrey

AU - Wagner, Kira

AU - Hecker, Jay Etta

AU - Egan, Lynn

AU - Grabenstatter, Heidi

AU - Meskis, Mary Anne

AU - Bartenhagen, Mike

AU - Sims, Scotty

AU - Walters, Julie

AU - Schoyer, Lisa

AU - Nye, Kim

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N2 - Objective: To describe the prevalence and characteristics of comorbidities in persons with rare epilepsies. Study design: Persons with rare epilepsies and caregivers of those affected were recruited through the Epilepsy Foundation and more than 30 rare epilepsy advocacy organizations affiliated with the Rare Epilepsy Network (REN). A web-based survey was conducted using a questionnaire consisting of core sections to collect data from affected persons on various aspects, including comorbidities. Comorbidity information was grouped into 15 classes, 12 of which had a stem question followed by detailed branch questions and 3 that were created from a combination of related questions. Results: Of 795 persons with more than 30 different rare epilepsy diagnosis groups, one-half had ≥5 comorbidity classes and 97% were classified as complex chronic disease (C-CD). The highest number of comorbidity classes reported per person were persons with Aicardi syndrome, Phelan-McDermid syndrome (median, 7.0; IQR, 5.0-9.0), and tuberous sclerosis complex (median, 6.0; IQR, 4.0-8.0). The most common comorbidity classes were learning/developmental disability (71%), mental health issues (71%), sleep disorders (60%), brain abnormalities (52%), oral issues (49%), bone-joint issues (42%), hyper/hypotonia (42%), and eye-vision disorders (38%). The prevalence of brain abnormalities, hyper/hypotonia, eye, and cardiac disorders was significantly higher in persons first diagnosed with epilepsy at a younger age (<9 months) than in those first diagnosed at an older age (P <.05 for trend). Conclusions: Nearly all persons with rare epilepsies are medically complex, with a high prevalence of multiple comorbidities, especially those who were diagnosed with epilepsy in the first year of life. Comorbidities should be carefully considered in the diagnosis and management of persons with rare epilepsies.

AB - Objective: To describe the prevalence and characteristics of comorbidities in persons with rare epilepsies. Study design: Persons with rare epilepsies and caregivers of those affected were recruited through the Epilepsy Foundation and more than 30 rare epilepsy advocacy organizations affiliated with the Rare Epilepsy Network (REN). A web-based survey was conducted using a questionnaire consisting of core sections to collect data from affected persons on various aspects, including comorbidities. Comorbidity information was grouped into 15 classes, 12 of which had a stem question followed by detailed branch questions and 3 that were created from a combination of related questions. Results: Of 795 persons with more than 30 different rare epilepsy diagnosis groups, one-half had ≥5 comorbidity classes and 97% were classified as complex chronic disease (C-CD). The highest number of comorbidity classes reported per person were persons with Aicardi syndrome, Phelan-McDermid syndrome (median, 7.0; IQR, 5.0-9.0), and tuberous sclerosis complex (median, 6.0; IQR, 4.0-8.0). The most common comorbidity classes were learning/developmental disability (71%), mental health issues (71%), sleep disorders (60%), brain abnormalities (52%), oral issues (49%), bone-joint issues (42%), hyper/hypotonia (42%), and eye-vision disorders (38%). The prevalence of brain abnormalities, hyper/hypotonia, eye, and cardiac disorders was significantly higher in persons first diagnosed with epilepsy at a younger age (<9 months) than in those first diagnosed at an older age (P <.05 for trend). Conclusions: Nearly all persons with rare epilepsies are medically complex, with a high prevalence of multiple comorbidities, especially those who were diagnosed with epilepsy in the first year of life. Comorbidities should be carefully considered in the diagnosis and management of persons with rare epilepsies.

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KW - prevalence

KW - questionnaire

KW - rare epilepsy

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