| Cause . | Symptoms and signs . | Evaluation . |
|---|---|---|
Due to a CNS lesion | ||
Hypothalamic hamartoma | May be associated with gelastic (laughing attacks), focal or tonic-clonic seizures. | MRI: Mass in the floor of the third ventricle iso-intense to normal tissue without contrast enhancement |
Other hypothalamic tumours: Glioma involving the hypothalamus and/or the optic chiasm Astrocytoma Ependymoma Pinealoma Germ cell tumours | May include headache, visual changes, cognitive changes, symptoms/signs of anterior or posterior pituitary deficiency (e.g. decreased growth velocity, polyuria/polydipsia), fatigue, visual field defects. If CNS tumour (glioma) associated with neurofibromatosis, may have other features of neurofibromatosis (cutaneous neurofibromas, café au lait spots, Lisch nodules) | MRI: contrast-enhanced mass that may involve the optic pathways (chiasm, nerve, tract), or the hypothalamus (astrocytoma, glioma), or that may involve the hypothalamus and pituitary stalk (germ cell tumour), may have evidence of intracranial hypertension May have signs of anterior or posterior pituitary deficiency (e.g. hypernatremia) If germ cell tumour: ßhCG can be detectable in blood or CSF |
Cerebral malformations involving the hypothalamus: Suprasellar archnoid cyst, Hydrocephalus, Septo-optic dysplasia, Myelomeningocele, Ectopic neurohypophysis. | May have neurodevelopmental deficits, large head size, visual impairment, nystagmus, obesity, polyuria/polydipsia, decreased growth velocity | May have signs of anterior or posterior pituitary deficiency (e.g. hypernatremia) or hyperprolactinaemia |
Acquired injury: Cranial irradiation, Head trauma, Infections, Perinatal insults. | Relevant history. Symptoms and signs of anterior or posterior pituitary deficiency may be present. | MRI may reveal condition-specific sequelae or may be normal |
Idiopathic–No CNS lesion | ≈ 92% of girls and ≈ 50% of boys. History of familial precocious puberty or adoption may be present. | No hypothalamic abnormality on the head MRI. The anterior pituitary may be enlarged. |
Secondary to early exposure to sex steroids | ||
After cure of any cause of gonadotropin-independent precocious puberty. | Relevant history. | |
| Cause . | Symptoms and signs . | Evaluation . |
|---|---|---|
Due to a CNS lesion | ||
Hypothalamic hamartoma | May be associated with gelastic (laughing attacks), focal or tonic-clonic seizures. | MRI: Mass in the floor of the third ventricle iso-intense to normal tissue without contrast enhancement |
Other hypothalamic tumours: Glioma involving the hypothalamus and/or the optic chiasm Astrocytoma Ependymoma Pinealoma Germ cell tumours | May include headache, visual changes, cognitive changes, symptoms/signs of anterior or posterior pituitary deficiency (e.g. decreased growth velocity, polyuria/polydipsia), fatigue, visual field defects. If CNS tumour (glioma) associated with neurofibromatosis, may have other features of neurofibromatosis (cutaneous neurofibromas, café au lait spots, Lisch nodules) | MRI: contrast-enhanced mass that may involve the optic pathways (chiasm, nerve, tract), or the hypothalamus (astrocytoma, glioma), or that may involve the hypothalamus and pituitary stalk (germ cell tumour), may have evidence of intracranial hypertension May have signs of anterior or posterior pituitary deficiency (e.g. hypernatremia) If germ cell tumour: ßhCG can be detectable in blood or CSF |
Cerebral malformations involving the hypothalamus: Suprasellar archnoid cyst, Hydrocephalus, Septo-optic dysplasia, Myelomeningocele, Ectopic neurohypophysis. | May have neurodevelopmental deficits, large head size, visual impairment, nystagmus, obesity, polyuria/polydipsia, decreased growth velocity | May have signs of anterior or posterior pituitary deficiency (e.g. hypernatremia) or hyperprolactinaemia |
Acquired injury: Cranial irradiation, Head trauma, Infections, Perinatal insults. | Relevant history. Symptoms and signs of anterior or posterior pituitary deficiency may be present. | MRI may reveal condition-specific sequelae or may be normal |
Idiopathic–No CNS lesion | ≈ 92% of girls and ≈ 50% of boys. History of familial precocious puberty or adoption may be present. | No hypothalamic abnormality on the head MRI. The anterior pituitary may be enlarged. |
Secondary to early exposure to sex steroids | ||
After cure of any cause of gonadotropin-independent precocious puberty. | Relevant history. | |
This PDF is available to Subscribers Only
View Article Abstract & Purchase OptionsFor full access to this pdf, sign in to an existing account, or purchase an annual subscription.
