Nonneoplastic congenital gray matter heterotopia in region of tuber cinereum of hypothalamus
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Bourdillon P et al: Surgical treatment of hypothalamic hamartomas. Neurosurg Rev. ePub, 2020
Gadgil N et al: Staged magnetic resonance-guided laser interstitial thermal therapy for hypothalamic hamartoma: analysis of ablation volumes and morphological considerations. Neurosurgery. 86(6):808-16, 2020
Roodakker KR et al: Ecstatic and gelastic seizures relate to the hypothalamus. Epilepsy Behav Rep. 14:100358, 2020
Wang S et al: Stereotactic radiofrequency thermocoagulation and resective surgery for patients with hypothalamic hamartoma. J Neurosurg. 1-8, 2020
Youngerman BE et al: Magnetic resonance imaging-guided laser interstitial thermal therapy for epilepsy: systematic review of technique, indications, and outcomes. Neurosurgery. 86(4):E366-82, 2020
Ferrand-Sorbets S et al: Seizure outcome and prognostic factors for surgical management of hypothalamic hamartomas in children. Seizure. 75:28-33, 2019
Fujita A et al: Pathogenic variants of DYNC2H1, KIAA0556, and PTPN11 associated with hypothalamic hamartoma. Neurology. 93(3):e237-51, 2019
Ler GYL et al: Teaching NeuroImages: hypothalamic hamartoma and polydactyly: think Pallister-Hall syndrome. Neurology. 93(23):e1016-7, 2019
Roland JL et al: Recent advances in the neurosurgical treatment of pediatric epilepsy: JNSPG 75th Anniversary Invited Review Article J Neurosurg Pediatr. 23(4):411-21, 2019
Fayed I et al: MR-guided laser interstitial thermal therapy for medically refractory lesional epilepsy in pediatric patients: experience and outcomes. Pediatr Neurosurg. 53(5):322-9, 2018
Du VX et al: Laser interstitial thermal therapy: a first line treatment for seizures due to hypothalamic hamartoma? Epilepsia. 58 Suppl 2:77-84, 2017
Démurger F et al: New insights into genotype-phenotype correlation for GLI3 mutations. Eur J Hum Genet. 23(1):92-102, 2015
Wu J et al: Mechanisms of intrinsic epileptogenesis in human gelastic seizures with hypothalamic hamartoma. CNS Neurosci Ther. 21(2):104-11, 2015
Li CD et al: Classification of hypothalamic hamartoma and prognostic factors for surgical outcome. Acta Neurol Scand. 130(1):18-26, 2014
Alves C et al: Giant hypothalamic hamartoma: case report and literature review. Childs Nerv Syst. 29(3):513-6, 2013
Mittal S et al: Hypothalamic hamartomas. Part 1. Clinical, neuroimaging, and neurophysiological characteristics. Neurosurg Focus. 34(6):E6, 2013
Pati S et al: Diagnosis and management of epilepsy associated with hypothalamic hamartoma: an evidence-based systematic review. J Child Neurol. 28(7):909-16, 2013
Parvizi J et al: Gelastic epilepsy and hypothalamic hamartomas: neuroanatomical analysis of brain lesions in 100 patients. Brain. 134(Pt 10):2960-8, 2011
Beggs J et al: Hypothalamic hamartomas associated with epilepsy: ultrastructural features. J Neuropathol Exp Neurol. 67(7):657-68, 2008
Kos S et al: Hydrometrocolpos, postaxial polydactyly, and hypothalamic hamartoma in a patient with confirmed Pallister-Hall syndrome: a clinical overlap with McKusick-Kaufman syndrome. Pediatr Radiol. 38(8):902-6, 2008
Ng YT et al: Endoscopic resection of hypothalamic hamartomas for refractory symptomatic epilepsy. Neurology. 70(17):1543-8, 2008
Ng YT: Clarification of the term "status gelasticus" and treatment and prognosis of gelastic seizures. Pediatr Neurol. 38(4):300-1; author reply 301-2, 2008
Parent AS et al: Gene expression profiling of hypothalamic hamartomas: a search for genes associated with central precocious puberty. Horm Res. 69(2):114-23, 2008
Pleasure SJ et al: Hypothalamic hamartomas and hedgehogs: not a laughing matter. Neurology. 70(8):588-9, 2008
Prigatano GP et al: Intellectual functioning in presurgical patients with hypothalamic hamartoma and refractory epilepsy. Epilepsy Behav. 13(1):149-55, 2008
Romanelli P et al: Radiosurgery for hypothalamic hamartomas. Neurosurg Focus. 24(5):E9, 2008
Schulze-Bonhage A et al: Outcome and predictors of interstitial radiosurgery in the treatment of gelastic epilepsy. Neurology. 71(4):277-82, 2008
Shahar E et al: Intractable gelastic seizures during infancy: ictal positron emission tomography (PET) demonstrating epileptiform activity within the hypothalamic hamartoma. J Child Neurol. 23(2):235-9, 2008
Shim KW et al: Treatment modality for intractable epilepsy in hypothalamic hamartomatous lesions. Neurosurgery. 62(4):847-56; discussion 856, 2008
Castori M et al: Reassessment of holoprosencephaly-diencephalic hamartoblastoma (HDH) association. Am J Med Genet A. 143(3):277-84, 2007
Castro LH et al: Epilepsy syndromes associated with hypothalamic hamartomas. Seizure. 16(1):50-8, 2007
Cheung CS et al: Gelastic seizures: not always hypothalamic hamartoma. Epileptic Disord. 9(4):453-8, 2007
Coons SW et al: The histopathology of hypothalamic hamartomas: study of 57 cases. J Neuropathol Exp Neurol. 66(2):131-41, 2007
Homma J et al: Stereotactic radiofrequency thermocoagulation for hypothalamic hamartoma with intractable gelastic seizures. Epilepsy Res. 76(1):15-21, 2007
Kerrigan JF et al: Hypothalamic hamartoma and infantile spasms. Epilepsia. 48(1):89-95, 2007
Régis J et al: Gamma knife surgery for epilepsy related to hypothalamic hamartomas. Semin Pediatr Neurol. 14(2):73-9, 2007
Rosenfeld JV et al: Hypothalamic hamartoma treatment: surgical resection with the transcallosal approach. Semin Pediatr Neurol. 14(2):88-98, 2007
Schulze-Bonhage A et al: Treatment options for gelastic epilepsy due to hypothalamic hamartoma: interstitial radiosurgery. Semin Pediatr Neurol. 14(2):80-7, 2007
Shahar E et al: Pediatric-onset gelastic seizures: clinical data and outcome. Pediatr Neurol. 37(1):29-34, 2007
Veendrick-Meekes MJ et al: Neuropsychiatric aspects of patients with hypothalamic hamartomas. Epilepsy Behav. 11(2):218-21, 2007
Vieira TC et al: Central precocious puberty associated with pituitary duplication and midline defects. J Pediatr Endocrinol Metab. 20(10):1141-4, 2007
Ali S et al: Psychiatric comorbidity in adult patients with hypothalamic hamartoma. Epilepsy Behav. 9(1):111-8, 2006
Amstutz DR et al: Hypothalamic hamartomas: correlation of MR imaging and spectroscopic findings with tumor glial content. AJNR Am J Neuroradiol. 27(4):794-8, 2006
Maixner W: Hypothalamic hamartomas--clinical, neuropathological and surgical aspects. Childs Nerv Syst. 22(8):867-73, 2006
Procaccini E et al: Surgical management of hypothalamic hamartomas with epilepsy: the stereoendoscopic approach. Neurosurgery. 59(4 Suppl 2):ONS336-44; discussion ONS344-6, 2006
Trivin C et al: Presentation and evolution of organic central precocious puberty according to the type of CNS lesion. Clin Endocrinol (Oxf). 65(2):239-45, 2006
Boudreau EA et al: Hypothalamic hamartomas and seizures: distinct natural history of isolated and Pallister-Hall syndrome cases. Epilepsia. 46(1):42-7, 2005
Kizilkilic O et al: Hypothalamic hamartoma associated with a craniopharyngeal canal. AJNR Am J Neuroradiol. 26(1):65-7, 2005
Saxonhouse MA et al: Neonatal hypothalamic hamartoma: a differentiating nonlethal hamartoblastoma. J Neurosurg. 103(3 Suppl):277-81, 2005
Striano S et al: The clinical spectrum and natural history of gelastic epilepsy-hypothalamic hamartoma syndrome. Seizure. 14(4):232-9, 2005
Yamura M et al: Evaluation of small hypothalamic hamartomas with 3D constructive interference in steady state (CISS) sequence. Neuroradiology. 47(3):204-8, 2005
Booth TN et al: Pre- and postnatal MR imaging of hypothalamic hamartomas associated with arachnoid cysts. AJNR Am J Neuroradiol. 25(7):1283-5, 2004
Freeman JL et al: MR imaging and spectroscopic study of epileptogenic hypothalamic hamartomas: analysis of 72 cases. AJNR Am J Neuroradiol. 25(3):450-62, 2004
Voyadzis JM et al: Hypothalamic hamartoma secreting corticotropin-releasing hormone. Case report. J Neurosurg. 100(2 Suppl Pediatrics):212-6, 2004
Freeman JL: The anatomy and embryology of the hypothalamus in relation to hypothalamic hamartomas. Epileptic Disord. 5(4):177-86, 2003
Mullatti N et al: The clinical spectrum of epilepsy in children and adults with hypothalamic hamartoma. Epilepsia. 44(10):1310-9, 2003
Boyko OB et al: Hamartomas of the tuber cinereum: CT, MR, and pathologic findings. AJNR Am J Neuroradiol. 12(2):309-14, 1991
Related Anatomy
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Related Differential Diagnoses
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Synonyms
Tuber cinereum hamartoma, diencephalic hamartoma
Definitions
Nonneoplastic congenital gray matter heterotopia in region of tuber cinereum of hypothalamus
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Bourdillon P et al: Surgical treatment of hypothalamic hamartomas. Neurosurg Rev. ePub, 2020
Gadgil N et al: Staged magnetic resonance-guided laser interstitial thermal therapy for hypothalamic hamartoma: analysis of ablation volumes and morphological considerations. Neurosurgery. 86(6):808-16, 2020
Roodakker KR et al: Ecstatic and gelastic seizures relate to the hypothalamus. Epilepsy Behav Rep. 14:100358, 2020
Wang S et al: Stereotactic radiofrequency thermocoagulation and resective surgery for patients with hypothalamic hamartoma. J Neurosurg. 1-8, 2020
Youngerman BE et al: Magnetic resonance imaging-guided laser interstitial thermal therapy for epilepsy: systematic review of technique, indications, and outcomes. Neurosurgery. 86(4):E366-82, 2020
Ferrand-Sorbets S et al: Seizure outcome and prognostic factors for surgical management of hypothalamic hamartomas in children. Seizure. 75:28-33, 2019
Fujita A et al: Pathogenic variants of DYNC2H1, KIAA0556, and PTPN11 associated with hypothalamic hamartoma. Neurology. 93(3):e237-51, 2019
Ler GYL et al: Teaching NeuroImages: hypothalamic hamartoma and polydactyly: think Pallister-Hall syndrome. Neurology. 93(23):e1016-7, 2019
Roland JL et al: Recent advances in the neurosurgical treatment of pediatric epilepsy: JNSPG 75th Anniversary Invited Review Article J Neurosurg Pediatr. 23(4):411-21, 2019
Fayed I et al: MR-guided laser interstitial thermal therapy for medically refractory lesional epilepsy in pediatric patients: experience and outcomes. Pediatr Neurosurg. 53(5):322-9, 2018
Du VX et al: Laser interstitial thermal therapy: a first line treatment for seizures due to hypothalamic hamartoma? Epilepsia. 58 Suppl 2:77-84, 2017
Démurger F et al: New insights into genotype-phenotype correlation for GLI3 mutations. Eur J Hum Genet. 23(1):92-102, 2015
Wu J et al: Mechanisms of intrinsic epileptogenesis in human gelastic seizures with hypothalamic hamartoma. CNS Neurosci Ther. 21(2):104-11, 2015
Li CD et al: Classification of hypothalamic hamartoma and prognostic factors for surgical outcome. Acta Neurol Scand. 130(1):18-26, 2014
Alves C et al: Giant hypothalamic hamartoma: case report and literature review. Childs Nerv Syst. 29(3):513-6, 2013
Mittal S et al: Hypothalamic hamartomas. Part 1. Clinical, neuroimaging, and neurophysiological characteristics. Neurosurg Focus. 34(6):E6, 2013
Pati S et al: Diagnosis and management of epilepsy associated with hypothalamic hamartoma: an evidence-based systematic review. J Child Neurol. 28(7):909-16, 2013
Parvizi J et al: Gelastic epilepsy and hypothalamic hamartomas: neuroanatomical analysis of brain lesions in 100 patients. Brain. 134(Pt 10):2960-8, 2011
Beggs J et al: Hypothalamic hamartomas associated with epilepsy: ultrastructural features. J Neuropathol Exp Neurol. 67(7):657-68, 2008
Kos S et al: Hydrometrocolpos, postaxial polydactyly, and hypothalamic hamartoma in a patient with confirmed Pallister-Hall syndrome: a clinical overlap with McKusick-Kaufman syndrome. Pediatr Radiol. 38(8):902-6, 2008
Ng YT et al: Endoscopic resection of hypothalamic hamartomas for refractory symptomatic epilepsy. Neurology. 70(17):1543-8, 2008
Ng YT: Clarification of the term "status gelasticus" and treatment and prognosis of gelastic seizures. Pediatr Neurol. 38(4):300-1; author reply 301-2, 2008
Parent AS et al: Gene expression profiling of hypothalamic hamartomas: a search for genes associated with central precocious puberty. Horm Res. 69(2):114-23, 2008
Pleasure SJ et al: Hypothalamic hamartomas and hedgehogs: not a laughing matter. Neurology. 70(8):588-9, 2008
Prigatano GP et al: Intellectual functioning in presurgical patients with hypothalamic hamartoma and refractory epilepsy. Epilepsy Behav. 13(1):149-55, 2008
Romanelli P et al: Radiosurgery for hypothalamic hamartomas. Neurosurg Focus. 24(5):E9, 2008
Schulze-Bonhage A et al: Outcome and predictors of interstitial radiosurgery in the treatment of gelastic epilepsy. Neurology. 71(4):277-82, 2008
Shahar E et al: Intractable gelastic seizures during infancy: ictal positron emission tomography (PET) demonstrating epileptiform activity within the hypothalamic hamartoma. J Child Neurol. 23(2):235-9, 2008
Shim KW et al: Treatment modality for intractable epilepsy in hypothalamic hamartomatous lesions. Neurosurgery. 62(4):847-56; discussion 856, 2008
Castori M et al: Reassessment of holoprosencephaly-diencephalic hamartoblastoma (HDH) association. Am J Med Genet A. 143(3):277-84, 2007
Castro LH et al: Epilepsy syndromes associated with hypothalamic hamartomas. Seizure. 16(1):50-8, 2007
Cheung CS et al: Gelastic seizures: not always hypothalamic hamartoma. Epileptic Disord. 9(4):453-8, 2007
Coons SW et al: The histopathology of hypothalamic hamartomas: study of 57 cases. J Neuropathol Exp Neurol. 66(2):131-41, 2007
Homma J et al: Stereotactic radiofrequency thermocoagulation for hypothalamic hamartoma with intractable gelastic seizures. Epilepsy Res. 76(1):15-21, 2007
Kerrigan JF et al: Hypothalamic hamartoma and infantile spasms. Epilepsia. 48(1):89-95, 2007
Régis J et al: Gamma knife surgery for epilepsy related to hypothalamic hamartomas. Semin Pediatr Neurol. 14(2):73-9, 2007
Rosenfeld JV et al: Hypothalamic hamartoma treatment: surgical resection with the transcallosal approach. Semin Pediatr Neurol. 14(2):88-98, 2007
Schulze-Bonhage A et al: Treatment options for gelastic epilepsy due to hypothalamic hamartoma: interstitial radiosurgery. Semin Pediatr Neurol. 14(2):80-7, 2007
Shahar E et al: Pediatric-onset gelastic seizures: clinical data and outcome. Pediatr Neurol. 37(1):29-34, 2007
Veendrick-Meekes MJ et al: Neuropsychiatric aspects of patients with hypothalamic hamartomas. Epilepsy Behav. 11(2):218-21, 2007
Vieira TC et al: Central precocious puberty associated with pituitary duplication and midline defects. J Pediatr Endocrinol Metab. 20(10):1141-4, 2007
Ali S et al: Psychiatric comorbidity in adult patients with hypothalamic hamartoma. Epilepsy Behav. 9(1):111-8, 2006
Amstutz DR et al: Hypothalamic hamartomas: correlation of MR imaging and spectroscopic findings with tumor glial content. AJNR Am J Neuroradiol. 27(4):794-8, 2006
Maixner W: Hypothalamic hamartomas--clinical, neuropathological and surgical aspects. Childs Nerv Syst. 22(8):867-73, 2006
Procaccini E et al: Surgical management of hypothalamic hamartomas with epilepsy: the stereoendoscopic approach. Neurosurgery. 59(4 Suppl 2):ONS336-44; discussion ONS344-6, 2006
Trivin C et al: Presentation and evolution of organic central precocious puberty according to the type of CNS lesion. Clin Endocrinol (Oxf). 65(2):239-45, 2006
Boudreau EA et al: Hypothalamic hamartomas and seizures: distinct natural history of isolated and Pallister-Hall syndrome cases. Epilepsia. 46(1):42-7, 2005
Kizilkilic O et al: Hypothalamic hamartoma associated with a craniopharyngeal canal. AJNR Am J Neuroradiol. 26(1):65-7, 2005
Saxonhouse MA et al: Neonatal hypothalamic hamartoma: a differentiating nonlethal hamartoblastoma. J Neurosurg. 103(3 Suppl):277-81, 2005
Striano S et al: The clinical spectrum and natural history of gelastic epilepsy-hypothalamic hamartoma syndrome. Seizure. 14(4):232-9, 2005
Yamura M et al: Evaluation of small hypothalamic hamartomas with 3D constructive interference in steady state (CISS) sequence. Neuroradiology. 47(3):204-8, 2005
Booth TN et al: Pre- and postnatal MR imaging of hypothalamic hamartomas associated with arachnoid cysts. AJNR Am J Neuroradiol. 25(7):1283-5, 2004
Freeman JL et al: MR imaging and spectroscopic study of epileptogenic hypothalamic hamartomas: analysis of 72 cases. AJNR Am J Neuroradiol. 25(3):450-62, 2004
Voyadzis JM et al: Hypothalamic hamartoma secreting corticotropin-releasing hormone. Case report. J Neurosurg. 100(2 Suppl Pediatrics):212-6, 2004
Freeman JL: The anatomy and embryology of the hypothalamus in relation to hypothalamic hamartomas. Epileptic Disord. 5(4):177-86, 2003
Mullatti N et al: The clinical spectrum of epilepsy in children and adults with hypothalamic hamartoma. Epilepsia. 44(10):1310-9, 2003
Boyko OB et al: Hamartomas of the tuber cinereum: CT, MR, and pathologic findings. AJNR Am J Neuroradiol. 12(2):309-14, 1991
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