hypothalamic diseases

Summary

Summary: Neoplastic, inflammatory, infectious, and other diseases of the hypothalamus. Clinical manifestations include appetite disorders; AUTONOMIC NERVOUS SYSTEM DISEASES; SLEEP DISORDERS; behavioral symptoms related to dysfunction of the LIMBIC SYSTEM; and neuroendocrine disorders.

Top Publications

  1. ncbi Psychological correlates of functional hypothalamic amenorrhea
    M D Marcus
    Department of Psychiatry, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA
    Fertil Steril 76:310-6. 2001
  2. pmc A genetic basis for functional hypothalamic amenorrhea
    Lisa M Caronia
    Harvard Center for Reproductive Endocrine Sciences and Reproductive Endocrine Unit and the Department of Medicine, Massachusetts General Hospital, Boston, USA
    N Engl J Med 364:215-25. 2011
  3. ncbi Functional brain mapping of ictal activity in gelastic epilepsy associated with hypothalamic hamartoma: a case report
    Alberto J R Leal
    Department of Neurophysiology, Hospital Júlio de Matos, Lisbon, Portugal
    Epilepsia 50:1624-31. 2009
  4. ncbi The histopathology of hypothalamic hamartomas: study of 57 cases
    Stephen W Coons
    Division of Neuropathology, Barrow Neurological Institute, Phoenix, Arizona 85013, USA
    J Neuropathol Exp Neurol 66:131-41. 2007
  5. ncbi Hypothalamic hamartoma: basic mechanisms of intrinsic epileptogenesis
    Kristina A Fenoglio
    Division of Neurology and Pediatric Neurology, Barrow Neurological Institute and Children s Health Center, St Joseph s Hospital and Medical Center, Phoenix, AZ 85013, USA
    Semin Pediatr Neurol 14:51-9. 2007
  6. ncbi The hypothalamic hamartoma: a model of subcortical epileptogenesis and encephalopathy
    John F Kerrigan
    Division of Neurology, Barrow Neurological Institute, St Joseph s Hospital and Medical Center, Phoenix, AZ 85013, USA
    Semin Pediatr Neurol 12:119-31. 2005
  7. ncbi GABAA receptor-mediated activation of L-type calcium channels induces neuronal excitation in surgically resected human hypothalamic hamartomas
    Do Young Kim
    Division of Pediatric Neurology, Barrow Neurological Institute, St Joseph s Hospital and Medical Center, Phoenix, AZ 85013, USA
    Epilepsia 49:861-71. 2008
  8. ncbi Hypothalamic hamartomas associated with epilepsy: ultrastructural features
    John Beggs
    Division of Neuropathology, and The Comprehensive Epilepsy Center, Barrow Neurological Institute, St Joseph s Hospital and Medical Center, Phoenix, AZ 85013, USA
    J Neuropathol Exp Neurol 67:657-68. 2008
  9. ncbi Hypothalamic hamartoma with refractory epilepsy: surgical procedures and results in 18 patients
    Martine Fohlen
    unité de neurochirurgie pédiatrique, Fondation Ophtalmologique A de Rothschild, Paris, France
    Epileptic Disord 5:267-73. 2003
  10. ncbi Status gelasticus after temporal lobectomy: ictal FDG-PET findings and the question of dual pathology involving hypothalamic hamartomas
    Andre Palmini
    Neurology Service and Epilepsy Surgery Program, Hospital Sao Lucas da PUCRS, Porto Alegre, Brazil
    Epilepsia 46:1313-6. 2005

Detail Information

Publications199 found, 100 shown here

  1. ncbi Psychological correlates of functional hypothalamic amenorrhea
    M D Marcus
    Department of Psychiatry, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA
    Fertil Steril 76:310-6. 2001
    ..To determine whether mood, attitudes, or symptoms of disordered eating discriminated women with functional hypothalamic amenorrhea (FHA) from those with organic causes of amenorrhea and eumenorrhea...
  2. pmc A genetic basis for functional hypothalamic amenorrhea
    Lisa M Caronia
    Harvard Center for Reproductive Endocrine Sciences and Reproductive Endocrine Unit and the Department of Medicine, Massachusetts General Hospital, Boston, USA
    N Engl J Med 364:215-25. 2011
    ..We hypothesized that mutations in genes involved in idiopathic hypogonadotropic hypogonadism, a congenital form of GnRH deficiency, are associated with hypothalamic amenorrhea...
  3. ncbi Functional brain mapping of ictal activity in gelastic epilepsy associated with hypothalamic hamartoma: a case report
    Alberto J R Leal
    Department of Neurophysiology, Hospital Júlio de Matos, Lisbon, Portugal
    Epilepsia 50:1624-31. 2009
    ..The EEG/fMRI method has the spatial-temporal resolution to study the dynamics of seizure activity, with detailed demonstration of origin and propagation pathways...
  4. ncbi The histopathology of hypothalamic hamartomas: study of 57 cases
    Stephen W Coons
    Division of Neuropathology, Barrow Neurological Institute, Phoenix, Arizona 85013, USA
    J Neuropathol Exp Neurol 66:131-41. 2007
    ..Nodules of these small neurons were a universal feature of the microarchitecture of HH lesions associated with epilepsy. Characterization of these neurons may aid in understanding the mechanism of seizure development in HH...
  5. ncbi Hypothalamic hamartoma: basic mechanisms of intrinsic epileptogenesis
    Kristina A Fenoglio
    Division of Neurology and Pediatric Neurology, Barrow Neurological Institute and Children s Health Center, St Joseph s Hospital and Medical Center, Phoenix, AZ 85013, USA
    Semin Pediatr Neurol 14:51-9. 2007
    ..These observations constitute the basis for future investigations aimed at elucidating the mechanisms of subcortical epileptogenesis...
  6. ncbi The hypothalamic hamartoma: a model of subcortical epileptogenesis and encephalopathy
    John F Kerrigan
    Division of Neurology, Barrow Neurological Institute, St Joseph s Hospital and Medical Center, Phoenix, AZ 85013, USA
    Semin Pediatr Neurol 12:119-31. 2005
    ..Refractory epilepsy associated with HH is the premier human model for subcortical epilepsy and an excellent model for secondary epileptogenesis and epileptic encephalopathy...
  7. ncbi GABAA receptor-mediated activation of L-type calcium channels induces neuronal excitation in surgically resected human hypothalamic hamartomas
    Do Young Kim
    Division of Pediatric Neurology, Barrow Neurological Institute, St Joseph s Hospital and Medical Center, Phoenix, AZ 85013, USA
    Epilepsia 49:861-71. 2008
    ..Here, we examined the role of GABAA receptors in surgically resected HH tissue...
  8. ncbi Hypothalamic hamartomas associated with epilepsy: ultrastructural features
    John Beggs
    Division of Neuropathology, and The Comprehensive Epilepsy Center, Barrow Neurological Institute, St Joseph s Hospital and Medical Center, Phoenix, AZ 85013, USA
    J Neuropathol Exp Neurol 67:657-68. 2008
    ....
  9. ncbi Hypothalamic hamartoma with refractory epilepsy: surgical procedures and results in 18 patients
    Martine Fohlen
    unité de neurochirurgie pédiatrique, Fondation Ophtalmologique A de Rothschild, Paris, France
    Epileptic Disord 5:267-73. 2003
    ..To study the surgical procedures and results on seizures, in 18 patients with refractory epilepsy due to hypothalamic hamartoma...
  10. ncbi Status gelasticus after temporal lobectomy: ictal FDG-PET findings and the question of dual pathology involving hypothalamic hamartomas
    Andre Palmini
    Neurology Service and Epilepsy Surgery Program, Hospital Sao Lucas da PUCRS, Porto Alegre, Brazil
    Epilepsia 46:1313-6. 2005
    ....
  11. ncbi From hypothalamic hamartoma to cortex: what can be learnt from depth recordings and stimulation?
    Philippe Kahane
    Neurophysiopathologie de l Epilepsie, CHU de Grenoble, France
    Epileptic Disord 5:205-17. 2003
    ..Whether this proposal of secondary epileptogenesis is valid or not remains a major issue, since it could provide arguments on the moment to discuss surgery...
  12. pmc GABA(A) receptor-mediated excitation in dissociated neurons from human hypothalamic hamartomas
    Jie Wu
    Division of Neurology, NRC 445, Barrow Neurological Institute, St Joseph s Hospital and Medical Center, Phoenix, AZ 85013 4496, USA
    Exp Neurol 213:397-404. 2008
    ..Taken collectively, we provide novel evidence for and mechanistic insights into HH epileptogenicity: GABA(A)R-mediated excitation...
  13. ncbi Presentation and evolution of organic central precocious puberty according to the type of CNS lesion
    Christine Trivin
    AP HP, Hopital Necker Enfants Malades, Service d Explorations Fonctionnelles, Paris, France
    Clin Endocrinol (Oxf) 65:239-45. 2006
    ..To evaluate the influence of the type and treatment of CNS lesion causing central precocious puberty (CPP) on the presentation, hypothalamic-pituitary function and final height...
  14. ncbi Successful resection of Hypothalamic Hamartoma with intractable gelastic seizures--by transcallosal subchoroidal approach
    Krishnapundha Bunyaratavej
    Chulalongkorn Comprehensive Epilepsy Program, Bangkok, Thailand
    J Med Assoc Thai 89:1269-76. 2006
    ..This appears to be the first report of this surgical approach for HH, which is less likely to disturb memory function compared to previously described interfoniceal approach...
  15. ncbi Treatment options for gelastic epilepsy due to hypothalamic hamartoma: interstitial radiosurgery
    Andreas Schulze-Bonhage
    Epilepsy Centre, University Hospital Freiburg, Freiburg, Germany
    Semin Pediatr Neurol 14:80-7. 2007
    ..A persistent weight gain was noted in 3 patients, which was severe in 1 (20 kg). There were no other neurologic, neuropsychologic, or neuropsychiatric side effects, which compares favorably with most surgical series...
  16. ncbi Psychiatric aspects of patients with hypothalamic hamartoma and epilepsy
    Ghislaine Savard
    Montreal Neurological Hospital, McGill University Health Centre, Montreal, QC, Canada
    Epileptic Disord 5:229-34. 2003
    ..The anxious and social features of the aggression suggest that psychiatric interventions, which have been neglected as the emphasis has been on seizure control, are worthwhile in the overall management of this difficult case...
  17. ncbi Positron emission tomography in epileptogenic hypothalamic hamartomas
    Philippe Ryvlin
    Department of Functional Neurology and Epileptology, Neurology Hospital, Lyon, France
    Epileptic Disord 5:219-27. 2003
    ..Whether these cortical abnormalities only reflect the propagation of ictal discharges, or a potentially independent seizure onset zone remains unknown...
  18. ncbi Stereotactic radiosurgery for hypothalamic hamartomas
    F Unger
    Department of Neurosurgery, Karl Franzens University, Graz, Austria
    Acta Neurochir Suppl 84:57-63. 2002
    ..Gamma Knife radiosurgery can be an effective and safe alternative treatment modality for HH capable of achieving good seizure control and improving behavioural disorders in selected cases...
  19. ncbi Gamma knife surgery for epilepsy related to hypothalamic hamartomas
    J Regis
    Stereotactic and Functional Neurosurgery Department, Timone Hospital, Marseilles, France
    Acta Neurochir Suppl 91:33-50. 2004
    ..A first retrospective study has shown a very good safety and efficacy level but for a more reliable evaluation a prospective study would be required...
  20. ncbi Hypothalamic hamartoma and epilepsy in children: illustrative cases of possible evolutions
    Alexis A Arzimanoglou
    Epilepsy Unit, Child Neurology and Metabolic Diseases Department, University Hospital Robert Debré, Paris, France
    Epileptic Disord 5:187-99. 2003
    ....
  21. ncbi [A case of hypothalamic hamartoma with refractory seizures: successful treatment with gamma knife surgery]
    Wakako Ogino
    Department of Development and Neurology, Kobe Children s Hospital, Kobe
    No To Hattatsu 35:65-9. 2003
    ..Focal radiosurgery by a gamma knife of the hamartoma successfully controlled the seizures with no neurological complications, and his mental function improved...
  22. ncbi Association of morphological characteristics with precocious puberty and/or gelastic seizures in hypothalamic hamartoma
    H Jung
    Clinical Research, Internal Medicine, and Endocrinology, Lilly Deutschland GmbH, Bad Homburg, Germany
    J Clin Endocrinol Metab 88:4590-5. 2003
    ..Male gender was associated with an intrahypothalamic HH and epilepsy, suggesting a sexually dimorphic developmental pattern of this heterotopic mass...
  23. ncbi Hypothalamic hamartoma secreting corticotropin-releasing hormone. Case report
    Jean Marc Voyadzis
    Department of Neurosurgery, Georgetown University Medical Center, Washington DC 20007, USA
    J Neurosurg 100:212-6. 2004
    ..Postoperatively, his hormone levels normalized and his behavioral disturbances abated. The radiographic and clinical characteristics of hypothalamic hamartomas are reviewed and therapeutic considerations discussed...
  24. ncbi [Clinical and electroencephalogram study of 5 children with hypothalamic hamartoma]
    Eiko Otsuka
    Departments of Pediatrics, Tokyo Women s Medical University, Tokyo
    No To Hattatsu 37:387-93. 2005
    ....
  25. ncbi Hypothalamic hamartoma and epilepsy: the pathway of discovery
    Frederick Andermann
    Epileptic Disord 5:173-5. 2003
  26. ncbi The anatomy and embryology of the hypothalamus in relation to hypothalamic hamartomas
    Jeremy L Freeman
    Children s Epilepsy Program, Department of Neurology and Murdoch Childrens Research Institute, Royal Children s Hospital, Parkville, Victoria, Australia
    Epileptic Disord 5:177-86. 2003
    ..Potential environmental antecedents to HH development are discussed, and the significance for sporadic, isolated HH of several syndromes associated with HH is explored...
  27. ncbi Gelastic epilepsy and hypothalamic hamartoma
    S Syamlal
    Indian Pediatr 43:454-5. 2006
  28. pmc Cranial MRI scans are indicated in all girls with central precocious puberty
    S M Ng
    Endocrinology Department, Royal Liverpool Chidren s Hospital, Liverpool, UK
    Arch Dis Child 88:414-8; discussion 414-8. 2003
    ..1) To assess the value of cranial magnetic resonance imaging (MRI) scans in the investigation of girls with central precocious puberty (CPP); and (2) to determine the clinical predictors of abnormal cranial MRI scans in these patients...
  29. ncbi Endoscopic surgery for hypothalamic hamartomas causing medically refractory gelastic epilepsy
    Harold L Rekate
    Division of Neurological Surgery, Barrow Neurological Institute, St Joseph s Hospital and Medical Center, Phoenix, AZ, USA
    Childs Nerv Syst 22:874-80. 2006
    ..The purpose of this study is to report our experiences with endoscopic removal of these hamartomas...
  30. ncbi Disconnecting surgical treatment of hypothalamic hamartoma in children and adults with refractory epilepsy and proposal of a new classification
    Olivier Delalande
    unité de neurochirurgie pédiatrique, Fondation Ophtalmologique A de Rothschild, Paris, France
    Neurol Med Chir (Tokyo) 43:61-8. 2003
    ..Endoscopic disconnection seems to be a very safe way to treat hamartomas in intraventricular locations...
  31. ncbi Pathogenesis of precocious puberty in hypothalamic hamartoma
    Heike Jung
    Clinic of Pediatrics and Genetics, University Hospital Hannover, Germany
    Horm Res 57:31-4. 2002
    ..These results imply that some HHs induce sexual precocity by activating endogenous LHRH secretion via astroglial-derived factors...
  32. ncbi Central precocious puberty associated with pituitary duplication and midline defects
    Teresa C Vieira
    Division of Neuroendocrinology, Department of Medicine, Escola Paulista de Medicina, Universidade Federal de Sao Paulo, Brazil
    J Pediatr Endocrinol Metab 20:1141-4. 2007
    ..Sellar and cervical column MRIs revealed pituitary duplication and rudimentary intervertebral disks. We present the clinical and imaging observations of this case, and a thorough literature review of this rare developmental abnormality...
  33. ncbi [Surgical disconnection of hypothalamic hamartomas]
    G Dorfmuller
    Service de Neurochirurgie, unité de neurochirurgie pédiatrique, Fondation Ophtalmologique A de Rothschild, 25 29 rue Manin, 75940 Paris Cedex 19, France
    Neurochirurgie 54:315-9. 2008
    ..Alternative surgical routes and techniques have therefore been proposed, especially for broadly attached lesions and for those with a third ventricular location...
  34. ncbi Outcome and predictors of interstitial radiosurgery in the treatment of gelastic epilepsy
    A Schulze-Bonhage
    Epilepsy Center, Neurocentre, University Hospital Freiburg, Breisacher Str 64, D 79106 Freiburg, Germany
    Neurology 71:277-82. 2008
    ..Here we report the effects of interstitial radiosurgery in the treatment of this generally pharmacoresistant epilepsy syndrome...
  35. ncbi Stereotactic surgical approach to hypothalamic hamartomas
    Ruben I Kuzniecky
    UAB Epilepsy Center, Department of Neurology, University of Alabama at Birmingham, Birmingham, Alabama, USA
    Epileptic Disord 5:275-80. 2003
    ..Epilepsy improved in 60% of patients with minimal morbidity. This approach was less successful in large lesions. These results indicate that this approach should be considered in patients with small/medium lesions...
  36. ncbi Hypothalamic hamartomas and seizures: distinct natural history of isolated and Pallister-Hall syndrome cases
    Eilis A Boudreau
    Clinical Epilepsy Section and EEG and Sleep Section, National Institute of Neurological Disorders and Stroke, Bethesda, Maryland 20892 1408, USA
    Epilepsia 46:42-7. 2005
    ..However, some patients, particularly those with other findings associated with Pallister-Hall syndrome (PHS), have a more benign course...
  37. ncbi Interstitial radiosurgery in the treatment of gelastic epilepsy due to hypothalamic hamartomas
    A Schulze-Bonhage
    Epilepsy Center, University Clinics of Freiburg, Germany
    Neurology 62:644-7. 2004
    ..At follow-up at least 1 year after interstitial radiotherapy, two patients had become seizure-free within 2 months, and two others had only persisting auras. There were no major perioperative or postoperative side effects...
  38. ncbi Gelastic epilepsy and precocious puberty due to hypothalamic hamartoma
    G Bruninx
    Department of Radiology, CHU Charleroi, Boulevard Paul Janson 92, B 6000 Charleroi, Belgium
    JBR-BTR 86:146-7. 2003
  39. ncbi Hypothalamic hamartoma with gelastic seizures in Swedish children and adolescents
    Göran Brandberg
    Department of Pediatrics, County Hospital, Falun Sweden
    Eur J Paediatr Neurol 8:35-44. 2004
    ..Hypothalamic hamartoma with gelastic seizures (HHGS) is an uncommon, often unrecognized, epileptic syndrome with onset of symptoms during childhood...
  40. ncbi Endoscopic disconnection for hypothalamic hamartoma with intractable seizure. Report of four cases
    Joong Uhn Choi
    Department of Neurosurgery and Neurology, Brain Research Institute, Yonsei University College of Medicine, Seoul, Korea
    J Neurosurg 100:506-11. 2004
    ..The authors advocate the endoscopic disconnection surgery as a safe and effective treatment for HH-related epilepsy by blocking the spread of epileptic discharges from the lesion...
  41. ncbi Pre- and postnatal MR imaging of hypothalamic hamartomas associated with arachnoid cysts
    Timothy N Booth
    Department of Radiology, Children s Medical Center of Dallas and University of Texas Southwestern Medical Center, Dallas, TX, USA
    AJNR Am J Neuroradiol 25:1283-5. 2004
    ..The lesion could also be confused with a cystic tumor such as pilocytic astrocytoma. Thorough evaluation is required in patients with precocious puberty, gelastic seizures, and the presence of a suprasellar arachnoid cyst...
  42. ncbi Radiosurgery for hypothalamic hamartomas
    Pantaleo Romanelli
    Functional Neurosurgery, IRCCS Neuromed, Pozzilli, Italy
    Neurosurg Focus 24:E9. 2008
    ..Radiosurgery is an emerging treatment modality for HHs, which provides excellent seizure outcomes and no lasting complications to date...
  43. ncbi Hypothalamic hamartoma: MR imaging and MR spectroscopic features
    Ashu Seith
    Singapore Med J 49:366-7. 2008
  44. ncbi Gelastic seizures: not always hypothalamic hamartoma
    Christina S Cheung
    Epilepsy Programme, Department of Clinical Neurological Sciences, University of Western Ontario, London, Canada
    Epileptic Disord 9:453-8. 2007
    ..This case report presents a 29-year-old woman who was successfully rendered free of gelastic seizures after resection of a frontal cortical dysplasia, localized through MRI and SPECT imaging.[Published with video sequences]...
  45. ncbi Gelastic seizure with tectal tumor, lobar holoprosencephaly, and subependymal nodules: clinical report
    Cigdem Inan Akman
    Division of Pediatric Neurology, State University of New York, Health Science Center at Brooklyn, USA
    J Child Neurol 17:152-4. 2002
    ..She developed seizures during the newborn period and presented with gelastic seizure and simple partial seizure at 3 months of age...
  46. ncbi The endocrinology of hypothalamic hamartoma surgery for intractable epilepsy
    Jeremy L Freeman
    Children s Epilepsy Program, Royal Children s Hospital, Parkville, Victoria, Australia
    Epileptic Disord 5:239-47. 2003
    ..Long term follow-up of growth and sexual development in a large series of patients is required...
  47. ncbi Somatic mutations in GLI3 can cause hypothalamic hamartoma and gelastic seizures
    R H Wallace
    Queensland Brain Institute, University of Queensland, Brisbane, QLD, 4072 Australia
    Neurology 70:653-5. 2008
  48. ncbi Hypothalamic hamartoma: comparison of clinical presentation and magnetic resonance images
    C Debeneix
    Pediatric Endocrinology, , Assistance Publique-Hopitaux de Paris, France
    Horm Res 56:12-8. 2001
    ..The hypothalamic-pituitary function in these cases is normal, which suggests that the absence of CPP is not due to gonadotropin deficiency. GnRH analog treatment preserves the growth potential in those with CPP...
  49. ncbi Hypothalamic hamartoma associated with a craniopharyngeal canal
    Osman Kizilkilic
    Department of Radiology, Adana Teaching and Medical Research Center, Baskent University, Adana, Turkey
    AJNR Am J Neuroradiol 26:65-7. 2005
    ....
  50. ncbi Gene expression profiling of hypothalamic hamartomas: a search for genes associated with central precocious puberty
    Anne Simone Parent
    Division of Neuroscience, Oregon National Primate Research Center, Beaverton, OR 97006, USA
    Horm Res 69:114-23. 2008
    ..Because HHs might express genes similar to those required for the initiation of normal puberty, we used cDNA arrays to compare the gene expression profile of an HH associated with CPP with three HHs not accompanied by sexual precocity...
  51. ncbi Hypothalamic hamartoma: the role of surgery in a series of eight patients
    C Mottolese
    Service de Neurochirurgie B, Hĵpital Neurologique et Neurochirurgical Pierre Wertheimer, Lyon, France
    Childs Nerv Syst 17:229-36; discussion 237-8. 2001
    ..hypothalamic hamartoma can be surgically treated when patients fail to respond to medical treatment, when the length of the treatment cannot be tolerated by the chidren and their families, and when there are uncontrolled gelastic seizures..
  52. ncbi Transcallosal resection of hypothalamic hamartomas in patients with intractable epilepsy
    A Simon Harvey
    Children s Epilepsy Program, Royal Children s Hospital, Parkville, Victoria, Australia
    Epileptic Disord 5:257-65. 2003
    ..The operative risks include stroke, short-term memory disturbance, weight gain and minor endocrine disturbances. Based on published data, the transcallosal approach appears to be safer and more effective than other operative strategies...
  53. ncbi Long-term treatment with growth hormone improves final height in a patient with Pallister-Hall syndrome
    C Galasso
    Department of Pediatrics, Tor Vergata University, Rome, Italy
    Am J Med Genet 99:128-31. 2001
    ..We conclude that children with Pallister-Hall syndrome and short stature be evaluated carefully for spontaneous somatotropic function and, if necessary, treated with growth hormone...
  54. ncbi Operative technique: the anterior transcallosal transseptal interforniceal approach to the third ventricle and resection of hypothalamic hamartomas
    Jeffrey V Rosenfeld
    Children s Epilepsy Program, Royal Children s Hospital, Parkville, Vic, Australia
    J Clin Neurosci 11:738-44. 2004
    ..This operative approach is applicable to other pathology in the third ventricle or hypothalamic region and has advantages compared with the standard transcallosal approach to the third ventricle...
  55. ncbi Gene-transfer technology: a preventive neurotherapy to curb obesity, ameliorate metabolic syndrome and extend life expectancy
    Satya P Kalra
    Department of Neuroscience, College of Medicine, University of Florida McKnight Brain Institute, Gainesville, FL 32610, USA
    Trends Pharmacol Sci 26:488-95. 2005
    ..Thus, the novel approach of central gene-transfer technology has distinct advantages over current therapies and has the potential to correct or slow the progression of inherited or acquired hypothalamic diseases.
  56. ncbi Surgical treatment of hypothalamic hamartoma causing central precocious puberty: long-term follow-up
    Chun de Li
    Department of Neurosurgery, Beijing Tiantan Hospital, Capital University of Medical Sciences, Beijing, People s Republic of China
    J Neurosurg Pediatr 12:151-4. 2013
    ..Central precocious puberty due to HH can be successfully treated with resection. In the 3 cases presented, this approach was associated with both short- and long-term efficacy. ..
  57. ncbi The use of stereotactic radiosurgery to treat intractable childhood partial epilepsy
    Catalina Dunoyer
    Department of Neurology, Miami Children s Hospital, Miami, Florida 33155, USA
    Epilepsia 43:292-300. 2002
    ..Stereotactic radiosurgery is a noninvasive procedure that effectively treats patients with vascular malformations and brain tumors, but its efficacy for epileptogenic foci is limited, especially in children...
  58. ncbi Subarachnoid haemorrhage
    Ilonka Kreitschmann-Andermahr
    Lancet 369:903-4. 2007
  59. ncbi Index of suspicion
    Raemma Paredes Luck
    Temple University Children s Medical Center, Philadelphia, PA, USA
    Pediatr Rev 28:111-7. 2007
  60. ncbi An audit of 500 subcutaneous glucagon stimulation tests to assess growth hormone and ACTH secretion in patients with hypothalamic-pituitary disease
    K S Leong
    Diabetes Centre, Whiston Hospital, Warrington Road, Prescot, Merseyside L35 5DR, UK
    Clin Endocrinol (Oxf) 54:463-8. 2001
    ..The 240 minute sample added little additional information and could be omitted...
  61. ncbi Hypothalamic obesity in children
    A Bereket
    Department of Pediatrics, Marmara University, Istanbul, Turkey
    Obes Rev 13:780-98. 2012
    ..Understanding the central role of the hypothalamus in the regulation of feeding and energy metabolism will help us gain insights into the pathogenesis and management of common obesity...
  62. ncbi Giant solid-cystic hypothalamic hamartoma. Case report
    Christian Dorfer
    Medical University of Vienna, Department of Neurosurgery, Wahringer Gurtel, Vienna, Austria
    Neurosurg Focus 30:E7. 2011
    ..The authors report on an exceptionally large solid-cystic hamartoma that led to hydrocephalus, precocious puberty, and intractable gelastic seizures. They discuss potential mechanisms of the development of hypothalamic hamartomas...
  63. ncbi Nature of changes in adrenocortical function in chronic hyperleptinemic female rats
    Mario Perello
    Neuroendocrine Unit, Multidisciplinary Institute on Cell Biology CONICET CICPBA, 1900 La Plata, Argentina
    Endocrine 24:167-75. 2004
    ..Our study supports that adrenal leptin resistance could be responsible, at least in part, for enhanced glucocorticoid circulating levels in this phenotype of obesity...
  64. ncbi Body fat, leptin, and hypothalamic amenorrhea
    Rexford S Ahima
    Division of Endocrinology, Diabetes, and Metabolism, University of Pennsylvania School of Medicine, Philadelphia, USA
    N Engl J Med 351:959-62. 2004
  65. ncbi Gelastic seizures due to hypothalamic hamartoma: rapid resolution after endoscopic tumor disconnection
    Gemma Incorpora
    a Unit of Pediatrics, Hospital Garibaldi, Catania, Italy
    Neurocase 19:458-61. 2013
    ..Brain magnetic resonance study revealed an hypothalamic hamartoma. Endoscopic tumor disconnection of the hamartoma resulted in rapid resolution of neurological symptomatology. ..
  66. ncbi [Case of severe motor and intellectual disabilities presenting with partial central diabetes insipidus triggered by infection]
    Miyuki Serizawa
    Department of Pediatrics, National Hospital Organization West Kofu National Hospital, Kofu, Yamanashi
    No To Hattatsu 36:414-8. 2004
    ..5-2.0 pg/ml) and urine osmolarity (peak level, 552 mOsm/kgH2O) despite the elevation of plasma osomolarity, suggesting latent partial central DI. Water balance and serum electrolyte levels should be closely monitored in cases of SMID...
  67. pmc Hypocretin deficiency develops during onset of human narcolepsy with cataplexy
    Andri Savvidou
    Paediatric Department, Blekinge Hospital, Karlskrona, Sweden
    Sleep 36:147-8. 2013
    ..Although hypothesized through animal studies, a temporal and causal association between hypocretin deficiency and the onset of narcolepsy with cataplexy (NC) has never been proven in humans...
  68. ncbi Mood disorders and sexual functioning in women with functional hypothalamic amenorrhea
    Carolyn M Dundon
    Psychology Department, University of Vermont, Burlington, Vermont 05405, USA
    Fertil Steril 94:2239-43. 2010
    ..To investigate the sexual function of women with functional hypothalamic amenorrhea (FHA) and to test the mediating effects of depression and anxiety on the sexual functioning of women with FHA...
  69. ncbi Hypothalamic--pituitary-- adrenal axis dysregulation in women with irritable bowel syndrome in response to acute physical stress
    Leah Z Fitzgerald
    University of California, Los Angeles, USA
    West J Nurs Res 31:818-36. 2009
    ..The women with IBS display blunted ACTH and cortisol responses to the LP along with a profile of affective responsiveness suggestive of chronic psychosocial stress, although no CRF(CSF) differences between groups are observed...
  70. ncbi Mind over matter: psychological factors and the menstrual cycle
    Leroy C Edozien
    St Mary s Hospital, Manchester, UK
    Curr Opin Obstet Gynecol 18:452-6. 2006
    ..Increasingly, gynaecologists are becoming aware of the impact of psychosocial factors on women's health generally, and on the menstrual cycle in particular. This review highlights developments in this field in the last triennium...
  71. ncbi Craniospinal Langerhans cell histiocytosis in children: 30 years' experience at a single institution
    Laurence Davidson
    Division of Neurosurgery, Children s Hospital Los Angeles, Keck School of Medicine, University of Southern California, Los Angeles, CA 90033, USA
    J Neurosurg Pediatr 1:187-95. 2008
    ..The goal of this study was to review a large series of patients with Langerhans cell histiocytosis (LCH) who had craniospinal lesions to assess the long-term course, outcome, and efficacy of treatment of the disease...
  72. ncbi Hypothalamic-pituitary dysfunction in critically ill patients with traumatic and nontraumatic brain injury
    Ioanna Dimopoulou
    Second Department of Critical Care Medicine, Attikon Hospital, Medical School, National and Kapodistrian University of Athens, Greece
    Intensive Care Med 31:1020-8. 2005
    ..A significant number of studies have shown that critically ill patients with brain injury (BI) frequently exhibit abnormal pituitary hormonal responses during the immediate postinjury period...
  73. ncbi Ictal movement disorders and hypothalamic hamartoma
    William Szurhaj
    Department of Clinical Neurophysiology and Neurology, CHRU Lille, France
    Epileptic Disord 7:43-8. 2005
    ....
  74. pmc New questions regarding bioequivalence of levothyroxine preparations: a clinician's response
    William L Green
    Division of Endocrinology, Metabolism, and Nutrition, VA Puget Sound Health Care System, Seattle, WA 98108, USA
    AAPS J 7:E54-8. 2005
    ..Such methods should include assessment of the changes in TSH induced by each preparation in athyrotic patients...
  75. ncbi MR imaging and spectroscopic study of epileptogenic hypothalamic hamartomas: analysis of 72 cases
    Jeremy L Freeman
    Children s Epilepsy Program, Department of Neurology, Royal Children s Hospital, Flemington Road, Parkville, Victoria 3052, Australia
    AJNR Am J Neuroradiol 25:450-62. 2004
    ..We aimed to detail the relationship of hypothalamic hamartomas to surrounding structures, to determine the frequency and nature of associated abnormalities, and to gain insight into mechanisms of epileptogenesis...
  76. ncbi A multimodality approach to the treatment of craniopharyngiomas avoiding hypothalamic morbidity: a UK perspective
    Helen A Spoudeas
    London Centre for Paediatric Endocrinology, Great Ormond Street Hospital, London, UK
    J Pediatr Endocrinol Metab 19:447-51. 2006
    ..Because of the rarity of craniopharyngiomas, international collaborative trials are necessary to properly inform future therapies...
  77. ncbi [Syndrome of hypothalamic obesity]
    Hironobu Yoshimatsu
    Department of Anatomy, Biology and Medicine, Internal Medicine 1, Faculty of Medicine, Oita University
    Nihon Rinsho . 2006
  78. ncbi Hypothalamic hamartoma treatment: surgical resection with the transcallosal approach
    Jeffrey V Rosenfeld
    Department of Neurosurgery, The Alfred Hospital and Monash University, Victoria, Australia
    Semin Pediatr Neurol 14:88-98. 2007
    ..The transcallosal resection of HH is an effective and safe treatment, but there is a small risk of short-term memory impairment. The endoscopic approach is an alternative to the transcallosal approach for smaller HH...
  79. ncbi An evaluation of the thyrotrophin-releasing hormone stimulation test in paediatric clinical practice
    Patricia M Crofton
    Department of Paediatric Biochemistry, Royal Hospital for Sick Children, Edinburgh, UK
    Horm Res 69:53-9. 2008
    ..The aim of this retrospective study was to evaluate the clinical usefulness of the thyrotropin-releasing hormone (TRH) test in children with suspected hypothalamic or pituitary dysfunction...
  80. ncbi Hepatic, cardiovascular, and endocrine outcomes of the histological subphenotypes of nonalcoholic fatty liver disease
    Eitan Rubinstein
    Division of Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, University of California, San Diego, Rady Children s Hospital, San Diego, California 92103 8450, USA
    Semin Liver Dis 28:380-5. 2008
    ....
  81. ncbi Effects of an oral contraceptive (norgestimate/ethinyl estradiol) on bone mineral density in women with hypothalamic amenorrhea and osteopenia: an open-label extension of a double-blind, placebo-controlled study
    Michelle P Warren
    Columbia University, College of Physicians and Surgeons, Department of Obstetrics and Gynecology, PH 16 128, New York, NY 10032, USA
    Contraception 72:206-11. 2005
    ..The effects of long-term triphasic oral contraceptive administration on bone mineral density (BMD) were investigated in premenopausal women with hypothalamic amenorrhea (HA) and osteopenia...
  82. ncbi Adrenal medullary function and expression of catecholamine-synthesizing enzymes in mice with hypothalamic obesity
    Andréia C P Martins
    Department of Cell Biology and Genetics, State University of Maringa, Maringa, PR, Brazil
    Life Sci 74:3211-22. 2004
    ..We conclude that in MSG obesity the low total catecholamine content is directly related to a decrease of key catecholamine-synthesizing enzymes, which by its turn may lead to a defective catecholamine secretion...
  83. ncbi Generation of a phenotypic array of hypothalamic neuronal cell models to study complex neuroendocrine disorders
    Denise D Belsham
    Department of Physiology, University of Toronto, Toronto, Ontario, Canada M5S 1A8
    Endocrinology 145:393-400. 2004
    ..Such studies may contribute information necessary for the strategic design of therapeutic interventions for complex neuroendocrine disorders, such as obesity...
  84. ncbi Hypothalamic hamartoma and seizures: a treatable epileptic encephalopathy
    Samuel F Berkovic
    Epilepsy Research Institute and Department of Neurology, Austin and Repatriation Medical Centre, Heidelberg Melbourne, Australia
    Epilepsia 44:969-73. 2003
    ..The previously dismal outlook for children with severe seizures associated with this lesion has now dramatically changed. These insights may have implications for other epileptic encephalopathies of childhood...
  85. ncbi Hypothalamic digitalis-like substance is released with sodium-loading in rats
    H Takahashi
    2nd Department of Medicine, Kyoto Prefectural University of Medicine, Japan
    Am J Hypertens 1:146-51. 1988
    ..Thereby, the plasma level of the EDLS was significantly greater than the control rats that received only the artificial CSF.(ABSTRACT TRUNCATED AT 250 WORDS)..
  86. ncbi Adrenal insufficiency in the critically ill: a new look at an old problem
    Paul E Marik
    Department of Critical Care Medicine, University of Pittsburgh, Pittsburgh, PA 15261, USA
    Chest 122:1784-96. 2002
    ..HPA dysfunction is a common and underdiagnosed disorder in the critically ill. We review the risk factors, pathophysiology, diagnostic approach, and management of HPA dysfunction in the critically ill...
  87. ncbi Severity of osteopenia in estrogen-deficient women with anorexia nervosa and hypothalamic amenorrhea
    S Grinspoon
    Neuroendocrine Unit, Massachusetts General Hospital and Harvard Medical School, Boston 02114, USA
    J Clin Endocrinol Metab 84:2049-55. 1999
    ..Lean body mass, independent of the duration or severity of estrogen deficiency, is an important predictor of bone loss among women with AN...
  88. ncbi Gamma knife radiosurgery for hypothalamic hamartomas in patients with medically intractable epilepsy and precocious puberty. Report of two cases
    F Unger
    Department of Neurosurgery, Karl Franzens University, Graz, Austria
    J Neurosurg 92:726-31. 2000
    ..Follow-up MR imaging has not revealed significant changes in the sizes of the lesions. Gamma knife radiosurgery can be an effective and safe treatment modality for achieving good seizure control in patients with hypothalamic hamartomas...
  89. ncbi High prevalence of fibromyalgia-associated symptoms in patients with hypothalamic-pituitary disorders
    Birgit Harbeck
    Department of Medicine I, University of Lubeck, Lubeck, Germany
    Clin Exp Rheumatol 31:S16-21. 2013
    ..The aim of this study was to evaluate whether patients with hypothalamic-pituitary diseases are at increased risk for fibromyalgia syndrome (FMS)...
  90. ncbi Serum leptin and leptin binding activity in children and adolescents with hypothalamic dysfunction
    L Patel
    Department of Child Health, University of Manchester, Booth Hall Children s Hospital, UK
    J Pediatr Endocrinol Metab 15:963-71. 2002
    ..This along with low leptin binding activity and hence higher free leptin levels would be consistent with central leptin insensitivity...
  91. ncbi Resective surgery for hypothalamic hamartoma
    Charles E Polkey
    Academic Neurosciences Centre Institute of Psychiatry, London, UK
    Epileptic Disord 5:281-6. 2003
    ..Alternate strategies include stereotactic radiosurgery and radiofrequency ablation under stereotactic control...
  92. ncbi [Assessment of endocrine disorders of the hypothalamic-pituitary axis by nuclear medicine techniques]
    M Schmidt
    Klinik und Poliklinik fur Nuklearmedizin, Universitat zu Koln, Deutschland
    Nuklearmedizin 41:80-90. 2002
    ..g. localization of small ACTH-producing pituitary adenomas, tumor localization in ectopic ACTH syndrome, localization of recurrent pituitary tissue, assessment of small incidentalomas, can not be definitely given yet...
  93. ncbi Gonadotropin-releasing hormone for infertility in women with primary hypothalamic amenorrhea. Toward a more-interventional approach
    A Kesrouani
    Department of Obstetrics and Gynecology, Hotel Dieu de France University Hospital, Paris, France
    J Reprod Med 46:23-8. 2001
    ..To assess the effectiveness of a protocol of pulsatile gonadotropin releasing-hormone (GnRH) in treating infertility in women with primary hypothalamic amenorrhea...
  94. ncbi Nonalcoholic fatty liver disease among patients with hypothalamic and pituitary dysfunction
    Leon A Adams
    Division of Gastroenterology and Hepatology, Mayo Clinic and Foundation, Rochester, Minnesota, USA
    Hepatology 39:909-14. 2004
    ..The novel evidence that hypothalamic/pituitary dysfunction may be accompanied by progressive NAFLD has important implications for the work-up and management of patients with hypothalamic/pituitary disease...
  95. ncbi 11 beta-Hydroxysteroid dehydrogenase activity in hypothalamic obesity
    Dov Tiosano
    Departments of Pediatrics, Meyer Children s Hospital, Haifa 31096, Israel
    J Clin Endocrinol Metab 88:379-84. 2003
    ..We propose that deficiency of hypothalamic messengers after surgical injury induces a paracrine/autocrine effect of enhanced glucocorticoid activity due to up-regulated 11 beta-HSD1 activity...
  96. ncbi [Sarcoidosis: the involvement of anterior pituitary hormones is poorly recognized]
    Hélène Bihan
    Hopital Avicenne, Service d endocrinologie, diabétologie et maladies métaboliques, 93009 Bobigny, France
    Presse Med 41:e524-9. 2012
    ..The objective of this study was to evaluate the impact on the detection of hypothalamic-pituitary sarcoidosis in patients hospitalized for this condition in a lung disease sensibilized department...
  97. ncbi Giant hypothalamic hamartoma: case report and literature review
    Cresio Alves
    Pediatric Endocrinology Unit, Hospital Universitario Prof Edgard Santos, Faculty of Medicine, Federal University of Bahia, Salvador, Bahia, Brazil
    Childs Nerv Syst 29:513-6. 2013
    ..Hypothalamic hamartomas are very rare (1:200,000) and range in size from 17.9 to 18 mm. When their dimensions exceed 30-40 mm, they are classified as giant hypothalamic hamartomas...
  98. ncbi Neurochemical and behavioral analyses of the lateral hypothalamic syndrome: a look back
    Edward M Stricker
    Department of Neuroscience, University of Pittsburgh, Pittsburgh, PA 15260, United States
    Behav Brain Res 231:286-8. 2012
    ..I write in behalf of a grateful field to salute his many great contributions...
  99. ncbi Endocrine disorders & female infertility
    David Unuane
    Department of Endocrinology, Universitair Ziekenhuis Brussel, UZ Brussel, Vrije Universiteit Brussel, Belgium
    Best Pract Res Clin Endocrinol Metab 25:861-73. 2011
    ..We will propose an algorithm for the diagnosis and approach of the female patient presenting with infertility on the basis of the available evidence in literature...
  100. ncbi Monozygotic twins discordant for ROHHAD phenotype
    Pallavi P Patwari
    Department of Pediatrics, Northwestern University Feinberg School of Medicine, Center for Autonomic Medicine in Pediatrics, Children s Memorial Hospital, 2300 Children s Plaza, Box 165, Chicago, IL 60614, USA
    Pediatrics 128:e711-5. 2011
    ..These cases confound the hypothesis of a monogenic etiology for ROHHAD and indicate alternative etiologies including autoimmune or epigenetic phenomenon or a combination of genetic predisposition and acquired precipitant...
  101. ncbi Hypothalamic-pituitary-adrenal axis suppression in asthmatic school children
    Ekkehard Werner Zöllner
    Paediatric Endocrine Unit, Tygerberg Children s Hospital, University of Stellenbosch, Cape Town, South Africa
    Pediatrics 130:e1512-9. 2012
    ..Hypothalamic-pituitary-adrenal axis suppression (HPAS) when treating children with corticosteroids is thought to be rare. Our objective was to determine the prevalence of and predictive factors for various degrees of HPAS...

Research Grants22

  1. PATHOGENESIS OF FUNCTIONAL HYPOTHALAMIC AMENORRHEA
    Sarah Berga; Fiscal Year: 2001
    ..We expect that the results of this proposal will lead directly to the PA-91-lOO objective of developing a "psychosocial intervention for infertility." ..
  2. GABAergic excitation in human hypothalamic hamartoma
    Jie Wu; Fiscal Year: 2007
    ..Knowledge about excitatory GABAA receptor function of HH neurons will also aid selection of superior strategies for pharmaceutical development to prevent and control human gelastic seizures. [unreadable] [unreadable]..
  3. REPRODUCTIVE PHYSIOLOGY OF OVARIAN FAILURE
    Nanette Santoro; Fiscal Year: 2003
    ..In this manner, we hope to elucidate the critical mechanisms that may cause or be the consequence of the hormonal dynamics of the early stages of the menopausal transition. ..
  4. Androgen Effects in HIV-Infected Women
    Steven Grinspoon; Fiscal Year: 2007
    ..The studies outlined in this renewal constitute an important investigative initiative of a targeted, gender-specific therapy in this largely understudied population. ..
  5. Chronic Prostatitis Collaborative Research Network
    Michel Pontari; Fiscal Year: 2007
    ..Issues in developing a clinically relevant definition of urologic chronic pelvic pain syndromes are discussed. ..
  6. Adolescent Gastric By-Pass and Diabetic Precursors
    Thomas Inge; Fiscal Year: 2006
    ....
  7. Clinical trial of leptin for hypothalamic amenorrhea
    Christos Mantzoros; Fiscal Year: 2005
    ..Support is needed to finalize key study design issues, establish collaborative arrangements for a multi-center study, develop an infrastructure for conducting a RCT, and plan subject recruitment strategies. ..
  8. GENE THERAPY, OBESITY AND HYPOTHALAMIC SIGNALING
    Satya Kalra; Fiscal Year: 2005
    ..This new knowledge will be applied to the ultimate goal of site-directed gene delivery aimed at the newly identified vulnerable loci in hypothalamic signaling to curb overeating and abnormal weight gain. ..
  9. Design and Analysis of Multi-Staged Association Studies Using Pooled Genomic DNA
    David Craig; Fiscal Year: 2008
    ..unreadable] [unreadable] [unreadable]..
  10. Stress and Risk for Substance Abuse in Adolescents
    Uma Rao; Fiscal Year: 2008
    ..The identification of specific vulnerabilities and associated biological alterations will aid in the development of more focused and effective pharmacological and psychosocial interventions to reduce the risk of SUD in such youth. ..
  11. Consequences and Treatment of Growth Hormone Deficiency in the Metabolic Syndrome
    STEVEN KYLE GRINSPOON; Fiscal Year: 2010
    ..Lay Description: This grant will study how commonly low GH level are seen and contribute to increased cardiac risk in patients with abdominal fat and whether increasing low GH levels will improve cardiac risk in this population. ..
  12. Neuronal Risk Markers for Nicotine Dependence in Youth
    Uma Rao; Fiscal Year: 2010
    ..abstract_text> ..
  13. Stress Response and Smoking Cessation in Depressed Youth
    Uma Rao; Fiscal Year: 2007
    ..Results of the proposed study might lead to treatment for both depression and nicotine dependence in adolescent patients. ..
  14. Androgen deficiency and Osteopenia in Anorexia Nervosa
    Karen Miller; Fiscal Year: 2002
    ..In the third phase of the study, we will examine the effects of administering testosterone on bone density and body mass. ..
  15. Markers of Risk for Nicotine Dependence in Youngsters
    Uma Rao; Fiscal Year: 2006
    ..Even if only a small subgroup of youngsters at risk for ND can be identified by such means, because of the many direct as well as indirect negative consequences associated with ND, this will be of great public health importance. ..
  16. Role of Leptin in the Neuroendocrine Response to Fasting
    Christos S Mantzoros; Fiscal Year: 2011
    ..abstract_text> ..
  17. Reproductive Hormones and Pre-Clinical CVD in Women
    C Bairey Merz; Fiscal Year: 2004
    ..abstract_text> ..
  18. Alcohol and Stress: Interactive Effects
    Joanne Weinberg; Fiscal Year: 2007
    ....
  19. Effects of Traditional Acupuncture on Mechanisms of CHD
    C Bairey Merz; Fiscal Year: 2009
    ..abstract_text> ..
  20. MECHANISMS AND STRATEGIES FOR INSULIN RESISTANCE IN AIDS
    Steven Grinspoon; Fiscal Year: 2004
    ..The proposed studies represent a significant collaborative effort between clinical researchers, adipocyte biologists, epidemiologists and nutritional biochemists in satisfaction of the mandate of the RFA. ..
  21. Risk for Substance Abuse in Depressed Adolescents
    Uma Rao; Fiscal Year: 2006
    ..More focused and effective treatments potentially might be helpful in reducing the risk for SUD in depressed youth. Moreover, such treatment strategies can be extended for testing in non-depressed youth. ..