Preliminary neurocognitive outcomes in Jeavons syndrome.
Jeavons syndrome (JS, eyelid myoclonia with absences [EMA]) consists of a triad of symptoms including eyelid myoclonia that may be accompanied by absence seizures, eye closure-induced EEG paroxysms or seizures, and photosensitivity. The age of onset ranges between 2 and 14 years with symptoms peaking between 6 and 8 years of age. Though investigation of the clinical, EEG, and neurological features of JS has occurred, neurocognitive functioning has not been well-delineated despite suggestion that a subtype of the syndrome is characterized in part by cognitive impairment. The purpose of this study was to define neurocognitive functioning in a more detailed manner by examining global IQ and relevant neurocognitive domains (i.e., verbal and nonverbal reasoning, attention, executive functioning, memory) in pediatric patients. The sample (N=6, 4 females) ranged in age from 8 to 15 years (M=11, SD=2.82). All participants completed neuropsychological evaluations. Statistical analyses revealed performance that was below average on measures of global IQ, processing speed and rote, verbal learning coupled with average nonverbal reasoning, and sustained attention. There was also evidence of impaired higher-level verbal reasoning. While global IQ ranged from low average to borderline impaired, no participant could be accurately described as impaired or having intellectual disability (ID) given the consistently average performance noted on some higher-order tasks including nonverbal reasoning.
Department of Psychology, Neuropsychology Section, St. Jude Children's Research Hospital, Memphis, TN, USA. Electronic address: Ashley.Fournier@StJude.org.
Ashley S
AS
Fournier-Goodnight
Jane and John Justin Neurosciences Center, Cook Children's Medical Center, Fort Worth, TX, USA.
Marsha
M
Gabriel
Jane and John Justin Neurosciences Center, Cook Children's Medical Center, Fort Worth, TX, USA.
M Scott
MS
Perry
D000293:Adolescent; D017668:Age of Onset; D001288:Attention; D002648:Child; D002675:Child, Preschool; D003072:Cognition Disorders / Q000209:etiology / Q000523:psychology*; D004569:Electroencephalography; D004832:Epilepsy, Absence / Q000523:psychology*; D056344:Executive Function; D005260:Female; D006801:Humans; D007361:Intelligence Tests; D007858:Learning; D008297:Male; D008568:Memory; D008606:Mental Processes; D009207:Myoclonus / Q000523:psychology*; D009483:Neuropsychological Tests; D013577:Syndrome; D014888:Wechsler Scales
Eyelid myoclonia with absences; Jeavons syndrome; Neurocognitive outcomes; Neuropsychological profile