page_title: Tuber cinereum hamartoma
section_title: Signs and symptoms
hierarchical_section_title: Tuber cinereum hamartoma / Signs and symptoms
caption_attribution_description: English: The image is of Douglas Myers (myself), i took it at Dalhousie Life Sciences Center on a research study investigating responses to startling. I do not care who uses the image or how, it was taken using a cell phone camera
context_page_description: Tuber cinereum hamartoma is a benign tumor in which a disorganized collection of neurons and glia accumulate at the tuber cinereum of the hypothalamus on the floor of the third ventricle. It is a congenital malformation, included on the spectrum of gray matter heterotopias. Formation occurs during embryogenesis, typically between days 33 and 41 of gestation. Size of the tumor varies from one to three centimeters in diameter, with the mean being closer to the low end of this range. It is estimated to occur at a frequency of one in one million individuals.
context_section_description: The classic presentation is gelastic or laughing epilepsy, a disorder characterized by spells of involuntary laughter with interval irritability and depressed mood. The tumor can be associated with other seizure types as well as precocious puberty and behavioral disorders. Gelastic epilepsy has been more classically associated with sessile lesions and precocious puberty reported with pedunculated morphology. More recent epidemiologic studies have found these associations to be less consistent, with gelastic epilepsy predominant in the majority of patients regardless of morphology.
  Hypothalamic hamartomas are found in 33% of patients with true precocious puberty. The etiology of this relationship is unclear, but it is suspected in some cases to be due to a nonphysiological secretion of GnRH. A case of hamartoma has also been reported to secrete CRH, causing excessive ACTH production.
  Seizures often begin when patients are young, although studies have shown adult onset as well. Many causes of the epilepsy have been theorized, with EEG often finding the hamartoma itself as the source of electrical activity, or epileptogenic focus. With chronic seizures, cognitive decline can develop, which can manifest as poor school performance, decreased nervous stimulus IQ, or limited socialization. Other symptoms of this tumor type include visual disturbances, such as the appearance of motion from a stationary object, or inappropriate color perception of the entire visual field.
caption: Electroencephalography is used to find the source of electrical activity causing the gelastic seizure.
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