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[Hypothalamic hamartoma: detailed presentation of a case]
S Daigneault1, C M Braun, J L Montes
1Département de Psychologie, Hôpital de Montréal pour Enfants.
Insights
A rare hypothalamic hamartoma caused severe behavioral issues in a child, including aggression and self-injury, despite typical epilepsy symptoms. This case highlights the profound psychological impact of small brain lesions.
Area of Science:
- Neuroscience
- Pediatric Neurology
- Clinical Psychology
Background:
- Hypothalamic hamartomas are rare congenital tumors.
- Classical symptoms include gelastic seizures, precocious puberty, and behavioral disturbances.
- Diagnosis is typically confirmed via MRI.
Observation:
- A seven-year-old child presented with severe behavioral issues, including aggression, self-injury, coprophagia, and coprolalia.
- The patient exhibited gelastic laughter and compulsive behaviors, but not precocious puberty.
- Despite various medications, no improvement was observed.
Findings:
- Magnetic Resonance Imaging (MRI) revealed a hypothalamic hamartoma consistent with typical profiles.
- Partial resection confirmed the diagnosis.
- The small lesion, approximately cherry-sized, correlated with extreme psychological and behavioral consequences.
Implications:
- This case underscores that even small hypothalamic hamartomas can lead to severe morbid psychological outcomes.
- Management challenges include the difficulty of institutionalization due to severe aggression.
- Further research into neuropsychological evaluation, electroencephalographic traits, and neurosurgical interventions is warranted.
Abstract:
We describe a seven year old child with a hypothalamic hamartoma. Classical symptoms of hypothalamic hamartoma include gelastic epileptic laughter, precocious puberty, aggressiveness, and progressively worsening epilepsy. After a normal first few years of life, this case presents all these symptoms except the precocious puberty. He has a markedly morbid personality disorder: he assaults strangers and relatives, bites people, spits in their faces unpredictably, is coprolalic and coprophagic, has gelastic laughter, puts pencils, erasers, and other non-comestible objects in his mouth, chews and ingests them, has tics (plays noisily with his saliva, empty chewing, compulsive spitting) and is self-injurious. None of the medications attempted to date have been of any help. Medical prognosis is somber, and this case is difficult to institutionalize, the more "congenial" institutions being insufficiently equipped to protect him and the beneficiaries and staff from his aggressive behavior. MRI showed the typical profile of hypothalamic hamartoma, and the diagnosis was confirmed with partial resection. This case illustrates that a tiny lesion, the size of a small cherry, can have extremely morbid psychological consequences. Detailed neuropsychological evaluation, certain unusual electroencephalographic traits and neurosurgical issues are discussed.