CEREBRAL PRECOCIOUS PUBERTY
 
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CEREBRAL PRECOCIOUS PUBERTY

 

DEFINITION:

A group of disorders characterized by the orderly development of secondary sexual characteristics prior to the age of 8 and 8.5 years in females and males, respectively.

EPIDEMIOLOGY:

  • incidence: ?
  • age of onset:
    • childhood
  • risk factors:
    • M > F

PATHOGENESIS:

1. Background

  • due to the premature awakening of the hypothalamic-pituitary-gonadal axis by a variety of intracranial lesions by unknown mechanisms
  • considered a true or central (complete) precocious puberty
    • gonadotropin-dependent
    • development of all secondary sexual characteristics (isosexual)
    • increased size and activity of gonads
  • the earlier the presentation of true precocious puberty, the greater the likelihood of an intracranial lesion

2. Etiologic Factors

1. Trauma

2. Tumors

  • hypothalamic hamartoma
  • astrocytomas
  • ependymomas
  • optic gliomas
  • pineal teratoma
  • tuber cinereum
  • ventricular hamartoma

3. Infectious/Inflammatory Lesions

  • encephalitis (postencephalitic scars)
  • meningitis
  • meningoencephalitis (tuberculous)

4. Others

  • cysts
  • hydrocephalus

CLINICAL FEATURES:

1. Endocrine Manifestations

1. Complete Precocious Puberty

  • development of all secondary sexual characteristics
    • isosexual
  • increased size and activity of the gonads

2. Hypothalamic Manifestaitons

  • adipsia
  • cachexia
  • diabetes insipidus
  • hyperthermia
  • obesity

2. Neurological Manifestations

1. Infratentorial

  • obstructive hydrocephalus with increased intracranial pressure
  • cerebellar signs
  • coning

2. Supratentorial

  • focal neurological signs
  • personality changes
  • seizures

INVESTIGATIONS:

1. Imaging Studies

1. Skeletal X-Rays

  • advanced bone age

2. MRI/CT

  • intracranial lesions

3. Pelvic Ultrasound

  • enlargement of the uterus
  • multicystic appearance of ovaries

2. Serum

  • elevated testosterone (M) and estradiol (F)
  • elevated FSH and LH on serial determinations
  • pulsatile secretion of FSH and LH
  • LHRH stimulation test
    • brief LH response as in puberty
    • best test for evidence of central precocious puberty

MANAGEMENT:

1. Surgery

  • treat underlying condition

2. LHRH Analogues

1. Luprolide Acetate (Lupron Depot)

  • 0.2-0.3 mg/kg (max. 7.5 mg) IM q4weeks
  • suppresses hypothalamic-pituitary-gonadal function
    • down-regulates receptors
  • regression of secondary sexual characteristics
    • cessation of menses
  • delays short stature by delaying the closure of epiphyses and normalizes rate of growth

 

 

Pediatric Database - CEREBRAL PRECOCIOUS PUBERTY

 

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