<p>Patients with hypothalamic hamartomas (HH) can suffer from a&#xa0;broad spectrum of clinical manifestations, including epileptic seizures, endocrine disturbances, developmental delays and behavioral abnormalities. The onset, severity and combination of these symptoms vary significantly between individuals. This review provides a&#xa0;comprehensive overview of the current literature on cognitive impairments (especially of memory and executive functions up to developmental delay and intellectual disability in 50–80%) and behavioral abnormalities (particularly externalized behavioral problems, such as outbursts of rage in 40–80%) in patients with HH and epilepsy. Furthermore, potential factors influencing neurodevelopmental and behavioral outcomes over the course of the disease are discussed. Finally, contemporary surgical approaches to epilepsy treatment in the context of HH are presented.</p>

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Zwischen Anfall und Alltagsleben: kognitive und Verhaltensmerkmale bei Epilepsie aufgrund eines hypothalamischen Hamartoms

  • Kathrin Wagner,
  • Victoria San Antonio-Arce,
  • Andreas Schulze-Bonhage,
  • Kerstin Alexandra Klotz

摘要

Patients with hypothalamic hamartomas (HH) can suffer from a broad spectrum of clinical manifestations, including epileptic seizures, endocrine disturbances, developmental delays and behavioral abnormalities. The onset, severity and combination of these symptoms vary significantly between individuals. This review provides a comprehensive overview of the current literature on cognitive impairments (especially of memory and executive functions up to developmental delay and intellectual disability in 50–80%) and behavioral abnormalities (particularly externalized behavioral problems, such as outbursts of rage in 40–80%) in patients with HH and epilepsy. Furthermore, potential factors influencing neurodevelopmental and behavioral outcomes over the course of the disease are discussed. Finally, contemporary surgical approaches to epilepsy treatment in the context of HH are presented.