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VOLUME 36, ISSUE 02

PRECOCIOUS PUBERTY AND OBESITY IN CHILDHOOD NARCOLEPSY WITH CATAPLEXY
High Prevalence of Precocious Puberty and Obesity in Childhood Narcolepsy with Cataplexy

http://dx.doi.org/10.5665/sleep.2366

Francesca Poli, MD, PhD1; Fabio Pizza, MD, PhD1; Emmanuel Mignot, MD, PhD2; Raffaele Ferri, MD3; Uberto Pagotto, MD, PhD4; Shahrad Taheri, MB, BS, PhD, FRCP5; Elena Finotti, MD1; Filippo Bernardi, MD6; Piero Pirazzoli, MD6; Alessandro Cicognani, MD6; Antonio Balsamo, MD6; Lino Nobili, MD, PhD7; Oliviero Bruni, MD8; Giuseppe Plazzi, MD, PhD1

1Department of Neurological Sciences, University of Bologna - IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy; 2Stanford Center for Sleep Sciences, Stanford University, Stanford, CA; 3Department of Neurology I.C., IRCCS Oasi Institute for Research on Mental Retardation and Brain Aging, Troina, Italy; 4Endocrinology Unit, Department of Clinical Medicine, S.Orsola-Malpighi General Hospital, Bologna, Italy; 5School of Clinical and Experimental Medicine, University of Birmingham, United Kingdom; 6Pediatric Clinic, S. Orsola-Malpighi General Hospital, Bologna, Italy; 7Centre for Epilepsy Surgery “C. Munari”, Centre of Sleep Medicine, Department of Neuroscience, Niguarda Hospital, Milan, Italy; 8Department of Developmental Neurology and Psychiatry, Sapienza University, Rome, Italy



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Study Objectives:

We analyzed the potential predictive factors for precocious puberty, observed in some cases of childhood narcolepsy with cataplexy (NC) and for obesity, a much more common feature of NC, through a systematic assessment of pubertal staging, body mass index (BMI), and metabolic/endocrine biochemical analyses.

Design:

Cross-sectional on consecutive recruitment.

Setting:

Hospital sleep center and pediatric unit.

Patients:

Forty-three children and adolescents with NC versus 52 age-matched obese children as controls.

Interventions:

N/A.

Measurements and Results:

Patients underwent clinical interview, polysomnographic recordings, cerebrospinal fluid hypocretin-1 measurement, and human leukocyte antigen typing. Height, weight, arterial blood pressure, and Tanner pubertal stage were evaluated. Plasma lipid and glucose profiles were analyzed. When an altered pubertal development was clinically suspected, plasma concentrations of hypothalamic-pituitary-gonadal axis hormones were determined. Children with NC showed a high prevalence of overweight/obesity (74%) and a higher occurrence of precocious puberty (17%) than obese controls (1.9%). Isolated signs of accelerated pubertal development (thelarche, pubic hair, advanced bone age) were also present (41%). Precocious puberty was significantly predicted by a younger age at first NC symptom onset but not by overweight/obesity or other factors. In addition, overweight/obesity was predicted by younger age at diagnosis; additional predictors were found for overweight/obesity (short disease duration, younger age at weight gain and lower high-density lipoprotein cholesterol), which did not include precocious puberty. NC symptoms, pubertal signs appearance, and body weight gain developed in close temporal sequence.

Conclusions:

NC occurring during prepubertal age is frequently accompanied by precocious puberty and overweight/obesity, suggesting an extended hypothalamic dysfunction. The severity of these comorbidities and the potential related risks require a multidiagnostic approach and a tailored therapeutic management.

Citation:

Poli F; Pizza F; Mignot E; Ferri R; Pagotto U; Taheri S; Finotti E; Bernardi F; Pirazzoli P; Cicognani A; Balsamo A; Nobili L; Bruni O; Plazzi G. High prevalence of precocious puberty and obesity in childhood narcolepsy with cataplexy. SLEEP 2013;36(2):175–181.

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