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Hypothalmic Hamartoma
What are the symptoms?
HH is associated with a number of features including difficult epilepsy, particularly gelastic (laughing) seizures, behavioural problems and precocious (early) puberty. The range and severity of these features varies widely between individuals.
Gelastic seizures are a rare type of seizure characterised by highly distinctive episodes of laughter. Seizures are both unpredictable and unprovoked, lasting for between five to sixty seconds, and occurring frequently. Because the seizures resemble natural laughter, parents and doctors initially may not recognize the child's behaviour as a seizure, thus delaying a diagnosis of HH.
In later childhood, the laughing seizures become longer and more severe, taking on less natural sounds and the pattern of seizure may change. Gelastic seizures may progress to complex partial seizures, tonic, atonic or generalized tonic-clonic seizures. In a generalized tonic-clonic (also known as a grand mal) seizure, individuals usually cry out, lose consciousness and fall to the floor. Their muscles stiffen (tonic phase) and then their arms and legs will jerk and twitch (clonic phase). The development of seizures associated with HH varies widely between individuals.
Later in development a child with HH may start to show outbursts of aggression and rage (often referred to as hypothalamic rage) and poor social adjustment. Increasing problems with learning often occurs. Some HH children display signs of autism. The frequency of the seizures and the subsequent disruption to the brain often makes deterioration of the child's mental state unavoidable.
Individuals with HH may show precocious puberty (see entry, Premature Sexual Maturation). This is the onset of puberty with the development of secondary sex characteristics before the age of eight years in girls and nine years in boys. Girls may begin their periods before nine years of age. Early onset of puberty is associated with a number of problems. Although an early growth spurt can initially cause tall stature, rapid bone maturation causes growth to cease too early resulting in short adult stature. The early appearance of breasts or periods in girls and increased libido in boys may cause emotional distress for both children and parents.
Background
| How is it treated? 
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