Treatment Evaluation Process

Some important HH characteristics and symptoms to consider when evaluating a potential HH patient include:
  • seizures
  • precocious (early) puberty
  • hormone imbalances
  • cognitive impairment
  • behavioral problems
  • emotional difficulties
Seizures caused by a hypothalamic hamartoma usually begin in infancy, most often as brief and frequent gelastic (or laughing) seizures. Between the ages of 4 and 10 years old, seizures may become more disabling, with the emergence of different seizure types, such as:
  • complex partial seizures (diminished or altered consciousnesswith involuntary but coordinated movements)
  • generalized convulsions (involuntary shaking, twitching, anddisorganized movements)
  • drop or atonic seizures (brief loss of muscle tone leading to falls)
In the early stage, the child may display signs of progressive cognitive impairment, worsening school performance, and the following behavioral problems:
  • tantrums
  • rage attacks
  • social isolation
Some children with a hypothalamic hamartoma may have endocrine (hormonal) disturbances. Central precocious puberty (early puberty) is the most common sign of these disturbances, and may be the only symptom of hypothalamic hamartoma that a child has. Not all people with a hypothalamic hamartoma have the same symptoms or the same age of onset. While you might notice gelastic seizures in a newborn, it is also possible that seizures will not develop until later in life. Seizures caused by a hypothalamic hamartoma generally do not respond to medication. View Treatment Algorithm here