Hypothalamic Hamartoma

FACTS

 
 

HPI

  • gelastic seizures (90% patients) --> can become uncontrollable causing marked deterioriation of cognitive/behavioral abilities
  • precocious puberty (hamartomas release GnRH)
  • developmental delay (from all the seizures)
  • aggressive behavior/rage attacks
  • visual changes

PHYSICAL EXAM

universal exam

IMAGING

  • Shape of lesion generally correlates with clinical manifestation (also changes type of craniotomy that is appropriate).
  • T1 isotense, Do NOT enhance
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Pedunculated (parahypothalamic)
Intrathalamic (sessile)
Morphology
narrower base attached to hypothalamus
arise within hypothalamic or broad attachment to it
Predominant Sx
precocious puberty > seizures
seizures > precocious puberty
laterobasilar crani
transcallosal interforniceal
A/P
  • GnRH analog for precocious puberty
  • Craniotomy, type as above
  • Counsel: this is non-cancerous