Investigation of Early Puberty in Neurology Patients

Investigation of Early Puberty in Neurology Patients

Puberty is a complex process of development with many biological and nutritional influences. In children with neurologic problems or impairments, onset of puberty is often exceptionally anxiety provoking. When parents see the first signs of puberty, the natural tendency is wonder if their child's neurologic condition or treatments are involved in the begining of puberty. The following information was organized with significant help from Dr. John Parks, Chief, Section of Endocrinology, in the Emory Department of Pediatrics. Staging of puberty is based on changes described by Marshall and Tannner.

When does puberty normally start?
      Normative data: 50% of Anglo-American girls have
  • Breast budding by their tenth birthday
  • Pubic hair by 11 years and 3 months
  • Menarche by 12 years and 9 months of age

      Normative data: 50% of African-American girls have
  • Breast budding by 8 years and 9 months of age
  • Pubic hair by 8 years and 9 months of age
  • One or the other by 8 years and 0 months
  • Menarche by 12 years and 1 month of age

Menarche occurs a good 3 years after the first signs of puberty

What is early?
      Sort of: More than 24 months before the median
      Real: More than 36 months before the median
      Possibility of three distinct groups
  1. Hypertrichosis:
    • Generalized hairiness may be related to drugs such as pheyntoin (Dilantin). If the hair on the front looks like the hair on the back, no workup is indicated.
    • "Flip-side Test" look on one side of the body, "flip" and look on the other side
  2. Pubarche:
    • Sexual hair development begins on labia majora
    • No point in working up early Tanner 2 at any age
    • Consider evaluating Tanner 3 if present before age 5 years with:
      • Bone age
      • Testosterone to exclude serious virilization
      • DHEAS to implicate adrenarche as source
  3. Breast Development: Hypothesis is that there is a release of inhibition of puberty in children with disorganized brains, in general It is driven by pulsatile release of LHRH that entrains LH and FSH release
    • Work up with:
      • Bone age (BA)
      • Baseline serum LH, ultrasensative test
    • Recomendations:
      • If BA is within 2 yrs of Chronologic Age (CA) and LH is < 0.5, observe
      • If BA is > 2yrs above CA and LH is higher than 0.5, consider whether there is a good rationale for giving monthly intramuscular shots of LUPRON DEPOT to hold off pubertal progression until the child and family may be more ready for it.
      • Endocrine referral if family wants to continue discussion of stopping puberty
Role of MRI in Evaluating early Puberty in Neurologic patients
  • Will generally have been done.
  • Hypothalamic hamartoma is the most important lesion
Role of Pelvic U/S in Evaluating early Puberty in Neurologic patients
  • Can gauge size of ovaries
  • Needed to exclude rare granulosa cell tumor
  • Can give an idea of how close the child is to starting periods