Presentation
A 38-year-old man presented with chronic hypothermia. He also had evidence of hypothalamo-pituitary dysfunction and CT scans showed a discrete hypothalamic lesion. [ncbi.nlm.nih.gov]
The third patient presented diabetes insipidus associated with corticotropic insufficiency (7). A solitary hypothalamopituitary lesion was demonstrated on MR imaging. [path.upmc.edu]
All cases in present study responded well to pharmacotherapy and surgery was not required. [annalsofian.org]
Headache is often a prominent symptom at presentation of sellar or parasellar lesions. [journals.lww.com]
Entire Body System
- Fever
Fever is the most common disorder of thermoregulation. [nba.uth.tmc.edu]
By reducing the activity of warm-sensitive neurons and increasing the activity of cold-sensitive neurons, pyrogens cause fever, a state in which all thermoregulatory responses have elevated set-point temperatures. [ncbi.nlm.nih.gov]
[…] in a dog, antithyroid antibodies led to hypothyroidism and adipsia, both of which resolved with levothyroxine therapy. [6] "Sickness behavior" is a condition in animals in which systemic infection leads to a highly regulated set of responses such as fever [emedicine.medscape.com]
How is body temperature regulated and what is fever? https://www.ncbi.nlm.nih.gov/books/NBK279457/ Reddy, S., & Sharma S. (2018). [news-medical.net]
[…] oxytocin levels reduce repetitive behaviors and improved ability to interpret emotions water inteake (thirst center) inhibit food intake (satiety center); docility homeostasis-- also respiratory center regulates response to heat --> PNS outflow GnRH, LHRH, fever [quizlet.com]
- Hypothermia
A 38-year-old man presented with chronic hypothermia. He also had evidence of hypothalamo-pituitary dysfunction and CT scans showed a discrete hypothalamic lesion. [ncbi.nlm.nih.gov]
Figure 3.7 Emergencies of Thermoregulation Hypothermia is defined as a core temperature of 35 degrees or lower and represents a potential medical emergency. [nba.uth.tmc.edu]
Although periodic hypothermia or hyperthermia has been occasionally reported in MS, hyperthemia and disturbances in the sleep-wake cycle and amenorrhea as a result of hypothalamic disease have not been previously reported among subjects with MS. [ajnr.org]
Periodic disorders. 28.1 Kleine-Levin syndrome (periodic somnolence and morbid hunger). 28.2 Spontaneous periodic fever, hypothermia, Shapiro syndrome and periodic Cushing's syndrome. 28.3 Acute intermittent porphyria. 28.4 Narcolepsy. 28.5 Epileptic [elsevier.com]
[…] hypokalemia低カリウム血[症] hypoparathyroidism上皮小体[機能]低下[症]、副甲状腺[機能]低下[症] hypopituitarism下垂体機能低下症 hypothalamic hormone視床下部ホルモン hypothalamohypophysial portalvein視床下部-下垂体門脈 hypothalamohypophysial system視床下部-下垂体系 hypothalamus-pituitary-adrenalaxis視床下部-下垂体-副腎系 hypothermia [tokyo-med.ac.jp]
Gastrointestinal
- Increased Appetite
DYSTROPHIA ADIPOSOGENITALIS Characterized by increased appetite and depressed secretion of gonadotropin Obesity and sexual infantilism Associated with dwarfism (if the condition occurs during growing period) It is also called Frohlich’s syndrome [de.slideshare.net]
This explains why depressed individuals may be more susceptible to infection Other Regionalized Functions of the Hypothalamus the lateral hypothalamus is important in increasing appetite; lesions to this area cause a decrease in body weight the medial [brainaacn.org]
He had increased appetite likely due to hypothalamic dysfunction, which resulted in body weight gain and fatty liver (Fig. 4b and c). He then started showing a gradual decline in cognitive function and activities of daily living. [bmcendocrdisord.biomedcentral.com]
Neurologic
- Headache
CONSUMERS: Click here for the Consumer Version Patients with hypothalamic-pituitary lesions generally present with some combination of Symptoms and signs of a mass lesion: headaches, altered appetite, thirst, visual field defects—particularly bitemporal [merckmanuals.com]
Tumor symptoms may include headache or loss of vision. If the thyroid is affected, there may be symptoms of an underactive thyroid (hypothyroidism). Symptoms may include feeling cold all the time, constipation, fatigue, or weight gain, among others. [medlineplus.gov]
Papillary craniopharyngioma in a 39-year-old man with headache. [pubs.rsna.org]
Patients with pituitary apoplexy present with sudden headache, meningeal signs, unilateral or bilateral cavernous sinus syndrome, visual loss, hypotension, and depressed level of consciousness. [brainaacn.org]
Women who are postmenopausal will not have obvious symptoms such as these and may first present with headache or loss of vision. Men may also suffer sexual dysfunction as a result of gonadotropin deficiency. [medical-dictionary.thefreedictionary.com]
- Lethargy
In some cases, the obstruction seemed enough to cause abnormal accumulation of CSF within the ventricles (hydrocephalus). 5 Impaired CSF flow can generate conditions like severe headache, neurological injury or lethargy. [massagemag.com]
[…] nervousness, insomnia, weight loss, tremor, excessive sweating, poor temperature regulation, and frequent bowel movements thyroid opthalmopathy – inflammation of orbital tissues proximal muscle weakness, tremor, dyskinesias and dementia Hypothyroidism lethargy [brainaacn.org]
Urogenital
- Amenorrhea
Report American Journal of Neuroradiology September 2011, 32 (8) E153-E155; DOI: https://doi.org/10.3174/ajnr.A2278 Abstract SUMMARY: This study describes a case of a patient with OSMS who presented with somnolence, periodic fever, memory impairment, and amenorrhea [ajnr.org]
Página 82 - M. (1980): Pregnancies following chronic intermittent (pulsatile) administration of Gn-RH by means of a portable pump ("Zyklomat") - a new approach to the treatment of infertility in hypothalamic amenorrhea. [books.google.es]
(round moon-shaped facies and truncal obesity) and acne, hirsutism, purplish skin striae, thin-appearing skin, easy bruising, poor wound healing, hypertension, diabetes, edema, immunosuppression, osteoporosis, avascular necrosis of the femoral head, amenorrhea [brainaacn.org]
[…] androgens and estrogens *other areas influence the production of sex hormones thorugh their regulation of anterior pituitary * before puberty * hypothalamic lesions--> may arret sexual development * after puberty * hypothalamic lesion s may result in amenorrhea [quizlet.com]
Case 2 A 21-year-old woman was referred for secondary amenorrhea. She had been treated for seven years with DDAVP for DI. Details of the initial investigation were not available. [em-consulte.com]
- Sexual Dysfunction
Men may also suffer sexual dysfunction as a result of gonadotropin deficiency. In acquired gonadotropin deficiency, both men and women may notice loss of body hair. [medical-dictionary.thefreedictionary.com]
Patients may seek treatment for evidence of gonadal dysfunction, secondary hypothyroidism, and even cortisol deficiency. Growth failure and lack of secondary sexual development may be seen in children. [journals.lww.com]
- Secondary Amenorrhea
Case 2 A 21-year-old woman was referred for secondary amenorrhea. She had been treated for seven years with DDAVP for DI. Details of the initial investigation were not available. [em-consulte.com]
Workup
Serum
- Hyperprolactinemia
It is the reason why masses other than adenomas can cause hyperprolactinemia. This is also why an unenhanced MRI scan suffices in a patient with hyperprolactinemia: it is not the size of the microadenoma, but ruling out other pathology that matters. [radiologyassistant.nl]
With stalk interruption hyperprolactinemia can occur, and diabetes insipidus is found in approx-imately 25% of patients [ 6,7 ]. [journals.lww.com]
Rarely, some children have adipsia without a definable structural lesion (essential adipsia). [9, 10, 11] A constellation of adipsia, obesity, hyperprolactinemia, and hypothyroidism was reported in one child. [11] In hypodipsia, the exact pathological [emedicine.medscape.com]
[…] change Disease, diseased - see also Syndrome hypophysis E23.7 pituitary E23.7 (gland) Disorder (of) - see also Disease pituitary gland E23.7 Dysfunction hypophysis E23.7 Lesion (s) (nontraumatic) hypothalamic E23.7 ICD-10-CM Codes Adjacent To E23.7 E22.1 Hyperprolactinemia [icd10data.com]
Two years earlier, investigation of impotence had shown mild hyperprolactinemia [prolactin 30 ng/ml (normal range = 5-15)] and hypogonadism [total plasma testosterone 2 m g/l (normal range = 3-10)]. [em-consulte.com]
Treatment
They play a vital role in planning and implementing treatment. As a neurosurgeon I am trained in the operative and non- operative diagnosis, management, prevention, and treatment of neurological disorders. [123helpme.com]
Rapidly consult with trusted authorities thanks to new expert-opinion treatment strategies and recommendations. Zero in on the most relevant and useful references with the aid of a more focused, concise bibliography. [books.google.de]
Patient remained seizure free with treatment. Last follow-up was done at about six months since start of therapy. [annalsofian.org]
The mass was no longer detected after the treatment (b). [bmcendocrdisord.biomedcentral.com]
A small number of patients have transient temperature irregularities following treatment or experience a temporary increase in seizure frequency several weeks after treatment. [rarediseases.org]
Prognosis
Prognosis The prognosis for most patients with hypopituitarism is excellent. As long as therapy is continued, many experience normal life spans. [medical-dictionary.thefreedictionary.com]
Follow up Follow up of Horner’s syndrome depends on the etiology of Horner’s syndrome Prognosis Prognosis depends on the etiology of Horner’s syndrome AAO, Basic and Clinical Science Course. Section 5: Neuro-ophthalmology, 2015-2016. [eyewiki.aao.org]
Long-term follow-up evaluation of magnetic resonance imaging in the prognosis of permanent GH deficiency. Eur J Endocrinol 2000 ; 143 : 493 –96 Carr DH, Sandler LM, Joplin GF. Computed tomography of sellar and parasellar lesions. [ajnr.org]
If removal of the tumor was successful then the patient can expect a positive prognosis regarding recovery rate and expected mortality. [123helpme.com]
Yamakawa K, Shitara N, Genka S, et al.: Clinical course and surgical prognosis of 33 cases of intracranial epidermoid tumors. Neurosurgery 1989; 24: 568–73. 17. [journals.lww.com]
Etiology
Etiology The etiology of Horner’s syndrome varies with the patient age and site of lesion. The etiology remains unknown in 35-40% of cases. [eyewiki.aao.org]
We examined an obese 58-year-old patient with a bilateral posterior hypothalamic lesion of unknown etiology. A 24-hour polysomnography revealed a markedly increased total sleep time (17.6 h). During daytime, only 3 continuous wake phases occurred. [ncbi.nlm.nih.gov]
Number of Figures: 1 Number of Tables: 1 ISSN: 0014-3022 (Print) eISSN: 1421-9913 (Online) For additional information: https://www.karger.com/ENE Abstract We examined an obese 58-year-old patient with a bilateral posterior hypothalamic lesion of unknown etiology [karger.com]
A genetic etiology has also been suggested. [14] Gelastic Seizure may also be observed in patients without MRI lesions and with normal neurologic status. [annalsofian.org]
Serum levels of pituitary hormones are lower than normal. hypopituitarism Endocrinology A condition characterized by ↓ secretion of one or more anterior pituitary or adenohypophyseal hormones Etiology Idiopathic, pituitary tumor, hypothalamic or infundibular [medical-dictionary.thefreedictionary.com]
Epidemiology
[…] thyroid hormones 3.1.3 Clinical assessment of the thyroid patient 3.1.4 Thyroid function tests and the effects of drugs 3.1.5 Nonthyroidal illness 3.1.6 Thyroid imaging: nuclear medicine techniques 3.1.6.1 Thyroid imaging: nonisotopic techniques 3.1.7 Epidemiology [oxfordmedicine.com]
ICD-9 337.9 Epidemiology In a population based study of Horner’s syndrome in the pediatric age group, the incidence of Horner’s syndrome was estimated to be 1.42 per 100 000 patients younger than 19 years, with a birth prevalence of 1 in 6250 for those [eyewiki.aao.org]
Pathophysiology
Hypothalamic dysfunction (hypothalamic syndromes) 2.4.2 Craniopharyngiomas 2.4.3 Perisellar tumours including cysts, hamartomas, and vascular tumours 2.4.4 Lymphocytic hypophysitis and other inflammatory conditions of the pituitary 2.5 Pineal physiology and pathophysiology [oxfordmedicine.com]
Recently, it was discovered that the pathophysiology of idiopathic narcolepsy-cataplexy is linked to orexin ligand deficiency in the brain and cerebrospinal fluid. [ci.nii.ac.jp]
Pathophysiology Horner's syndrome results from a lesion to the sympathetic pathways that supply the head and neck, including the oculosympathetic fibers. Relevant anatomy The pupil is innervated by sympathetic and parasympathetic fibers. [eyewiki.aao.org]
The pathophysiologic basis of secondary narcolepsy and hypersomnia. Curr Neurol Neurosci Rep. 2011;11(2):235–41. 2. Suzuki K, Miyamoto M, Miyamoto T, Matsubara T, Inoue Y, Iijima M, Mizuno S, Horie J, Hirata K, Shimizu T, et al. [bmcneurol.biomedcentral.com]
Report of a case and review of the literature with a pathophysiological explanation of the precocious sexual syndrome. Arch. Intern. Med., 67:762-792, 1941. 79. Wilber, I. F., Montoya, F., Plotnikoff, N. [jpgmonline.com]
Prevention
During the light cycle, a series of mechanisms deriving from the retinal ganglionic cells to the SCN prevent the secretion of melatonin. By preventing the release of the hormone, individuals are motivated to stay away during daylight hours. [news-medical.net]
Primary prevention Prevention strategies depend on the etiology of Horner’s syndrome. These may include prevention of trauma (especially head trauma), control of cardiovascular risk factors and smoking cessation. [eyewiki.aao.org]
Prevention There is no known prevention of hypopituitarism, except for prevention of damage to the pituitary/hypothalamic area from injury. Key terms Adenoma — A benign (not threatening or cancerous) tumor that originates in a gland. [medical-dictionary.thefreedictionary.com]
As a neurosurgeon I am trained in the operative and non- operative diagnosis, management, prevention, and treatment of neurological disorders. [123helpme.com]
Careful history of surgical complications in relatives and identification of other contributing conditions is the best way to detect and prevent malignant hyperthermia. [nba.uth.tmc.edu]