Presentation and evolution of organic central precocious puberty according to the type of CNS lesion.
OBJECTIVE
To evaluate the influence of the type and treatment of CNS lesion causing central precocious puberty (CPP) on the presentation, hypothalamic-pituitary function and final height.


METHODS
One hundred patients with CPP caused by central nervous system (CNS) lesion.


RESULTS
The CPP was the presenting symptom of the lesion in 25 (10 boys) and occurred in 75 patients (23 boys) previously treated for lesions. These were optic glioma or astrocytoma (n = 45), hydrocephalus (n = 22), hypothalamic hamartoma (n = 15), suprasellar arachnoid cyst (n = 10) and others (n = 8). The percentages of patients with increased height, bone age advance, testicular volume, LH/FSH peaks ratio after gonadotrophin-releasing hormone (GnRH) test and plasma testosterone concentration in boys and oestradiol in girls varied from one aetiology to another. The boys with hamartoma were significantly taller and had greater bone age advance, LH peak and testosterone than boys with optic glioma. The girls with hamartoma and suprasellar arachnoid cyst were significantly younger and had greater LH peak than girls in the other groups. All patients treated for optic glioma had hypothalamic-pituitary deficiencies, including GH (100%), thyrotrophin (71.4%), corticotrophin (12.5%) and pubertal (34.3%) deficiencies. Sixty percent of those with suprasellar cysts lacked GH. Final height was below -2 SD in 15/59 (25%) patients, including 5/11 not treated with GnRH analogue, 3/5 not treated with GH despite GH deficiency, and 2 with hydrocephalus as a result of meningomyelocele.


CONCLUSIONS
The type of CNS lesion influences the presentation of CPP. This is probably caused by differences in the mechanisms inducing puberty and to the hypothalamic-pituitary deficiencies associated with the CPP as a result of a lesion and/or its treatment.
Trivin
Christine
C
AP-HP, Hôpital Necker-Enfants Malades, Service d'Explorations Fonctionnelles, Paris, France.
Couto-Silva
Ana-Claudia
AC
Sainte-Rose
Christian
C
Chemaitilly
Wassim
W
Kalifa
Chantal
C
Doz
François
F
Zerah
Michel
M
Brauner
Raja
R
D000293:Adolescent; D000328:Adult; D016080:Arachnoid Cysts / Q000097:blood / Q000150:complications; D001254:Astrocytoma / Q000097:blood / Q000150:complications; D001827:Body Height; D001932:Brain Neoplasms / Q000097:blood / Q000150:complications*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005640:Follicle Stimulating Hormone / Q000097:blood; D012739:Gonadal Steroid Hormones / Q000097:blood; D013006:Growth Hormone / Q000097:blood; D013007:Growth Hormone-Releasing Hormone; D006222:Hamartoma / Q000097:blood / Q000150:complications; D006801:Humans; D006849:Hydrocephalus / Q000097:blood / Q000150:complications; D006854:Hydrocortisone / Q000097:blood; D007027:Hypothalamic Diseases / Q000209:etiology*; D007029:Hypothalamic Neoplasms / Q000097:blood / Q000150:complications; D007986:Luteinizing Hormone / Q000097:blood; D008297:Male; D008591:Meningomyelocele / Q000097:blood / Q000150:complications; D020339:Optic Nerve Glioma / Q000097:blood / Q000150:complications; D010900:Pituitary Diseases / Q000097:blood / Q000209:etiology*; D011629:Puberty, Precocious / Q000097:blood / Q000209:etiology*; D018709:Statistics, Nonparametric
D012739:Gonadal Steroid Hormones; D007986:Luteinizing Hormone; D005640:Follicle Stimulating Hormone; D013006:Growth Hormone; D013007:Growth Hormone-Releasing Hormone; D006854:Hydrocortisone