Hypothalamic syndrome

Introduction to hypothalamic syndrome The hypothalamus weighs about 4g and is small in size. In function, the hypothalamus is the center of neuroendocrine and autonomic regulation of the body. On the whole, the hypothalamus is both a nerve center and an endocrine organ. It is a hub for the nervous system to control the interconnection between endocrine and endocrine organs and internal and external receptors. At least 11 species of secretory hormones in hypothalamic neurons have been identified. basic knowledge The proportion of illness: 0.0035% Susceptible people: no specific population Mode of infection: non-infectious Complications: Diabetes insipidus Hypernatremia

Cause of hypothalamic syndrome

(1) Causes of the disease

The cause is congenital and acquired, organic and functional.

1. Infection and inflammation: tuberculous meningitis, purulent meningitis, epidemic encephalitis, viral encephalitis, meningitis, brain abscess, smallpox, measles, chickenpox, rabies vaccination, tissue cytoplasmosis, etc.

2. Tumor: Common craniopharyngioma and its deformation (epenocarcinoma and epidermoid cyst), pineal tumor and ectopic pineal tumor, astrocytoma, funnel tumor, pituitary tumor spread to the saddle ( Development, growth, elongation), neurofibromatosis, ganglioneuroma, plasmacytoma, medulloblastoma, germ cell tumor, hemangioma, malignant hemangioendothelioma, third ventricular cyst, meningioma, lipoma, metastasis Sexual cancer, leukemia, lymphoma and hamartoma, teratoma and so on.

3. Degenerative changes: brain softening, tuberous sclerosis, gliosis and so on.

4. Brain metabolic diseases: carbon dioxide anesthesia, acute gap episodes of hematoporphyria.

5. Vascular lesions: cerebral arteriosclerosis, cerebral aneurysms, cerebral embolism, cerebral hemorrhage, systemic lupus erythematosus and other causes of intracerebral vasculitis, pituitary apoplexy, hemangioma, arteriovenous malformation.

6. Physical factors: Traumatic trauma or brain surgery causes the pituitary stalk to break or damage the hypothalamus. Radiation therapy of head and neck tumors causes necrosis of hypothalamic nerve tissue.

7. Drugs: Long-term (large) doses of domperidone, risperidone, chlorpromazine and contraceptives can cause glucer- amenorrhea syndrome (gactorrhea-amenorrhea syndrome).

8. Granulomatous lesions: tuberculoma, sarcoidosis, eosinophilic granuloma, reticuloendothelial cell proliferation, etc.

9. Functional disorders: Neurogenic amenorrhea or impotence accompanied by thyroid function and/or adrenal insufficiency may occur due to trauma, environmental changes, and the like.

10. Congenital lesions or hereditary

(1) Sexual dysplasia and olfactory loss syndrome: Kalmanns syndrome, familial olfactory-sexual hypoplasia syndrome, osteoporosis with precocious puberty (congenital polyfibrillar hyperplasia) , also known as AIbright syndrome).

(2) Obesity-reproductive incompetence syndrome (also known as Frohlich syndrome).

(3) Sexual naive-multi-finger malformation syndrome (also known as laurence-moon-biedl syndrome)

11. Others: Recently, patients with kabuki make-up syndrome have been reported to have central diabetes insipidus and growth hormone secretion dysfunction. MRI shows pituitary gland and head abnormalities. After exogenous growth hormone is given, Physical development reached normal levels.

(two) pathogenesis

The physiological function of the hypothalamus is complex and can be summarized as the following three aspects: synthesis and secretion of hormones that regulate pituitary function, release of inhibitory hormones, and antidiuretic hormones that regulate water and salt metabolism; hypothalamus is the highest regulation of sympathetic and parasympathetic nerves. The central hypothalamus is one of the centers of important life activities of the human body (such as energy balance and nutrient intake, awakening and sleep, thermoregulation, emotional behavior, sexual function, biological clock, etc.). Therefore, the above various causes involve the hypothalamus. In order to cause hypothalamic dysfunction, secondary pituitary and target gland dysfunction, there will be a group of clinical syndromes with endocrine and metabolic disorders, abnormalities such as body temperature and sleep, and autonomic dysfunction.

Hypothalamic syndrome prevention

There are no effective preventive measures for this disease.

Hypothalamic syndrome complications Complications diabetes insipidus hypernatremia

A large number of low specific gravity urine, urine volume more than 3L / d, specific gravity below 1.006, polydipsia and polydipsia, especially good cold drink, in addition to burnout, fatigue, affect sleep, generally does not affect growth and development, according to the severity of the disease, can be divided into parts Diabetes insipidus and complete diabetes insipidus, caused by tumors in the sellar region, trauma, surgery, such as the function of the pituitary gland at the same time, may be accompanied by partial or complete hypogonadal hypofunction, congenital diabetes insipidus and Trauma, surgery, and saddle area tumors can affect the thirst center, making patients more urinary but not thirsty, easy to dehydrate leading to hypernatremia, hyperosmotic state, which can be accompanied by fever, convulsions and even cerebrovascular accidents.

Partial pituitary diabetes insipidus can be given hydrochlorothiazide (hydrochlorothiazide) 25 ~ 50mg, oral, 3 times / d, while avoiding coffee, etc., clofibrate (clofibrate) 0.25 ~ 0.5g, oral, 3 times / d, Carbamazepine 0.1g, oral, 3 times / d, may have white blood cell reduction, liver damage, lethargy, dizziness, rash and other adverse reactions, complete pituitary diabetes insipidus should be supplemented with vasopressin, commonly used oily niacin plus Pressure hormone (long-acting urine collapse) (5U/ml), starting from 0.1ml, deep intramuscular injection, generally 0.3 ~ 0.5ml, to maintain about 5 days is appropriate, adverse reactions have headache, high blood pressure, abdominal pain, etc. , powder after the pituitary powder (50U / ml) 5 ~ 10U, nasal suction, every 4 ~ 6 hours, can cause chronic rhinitis and affect the efficacy, 1-cysteine-8-dextro-arginine pressure It increases antidiuretic activity and reduces adverse reactions. Starting from 0.1mg/d, the dosage is gradually increased according to the amount of urine. The dose adjusted to a urine volume of about 2000ml/d is maintained, usually 0.1-0.2mg, orally, 2~. 3 times / d, or 4mg / ml, intramuscular injection, 2 ~ 3 times / d, alert to excessive water poisoning, such as pale, abdominal pain, elevated blood pressure, due to tumor introduction Who should surgery or radiation therapy, can also be tested nephrogenic diabetes insipidus hydrochlorothiazide, the same dosage of the former.

Symptoms of hypothalamic syndrome Common symptoms Deep sleepy precocious puberty laparoscopic diplopia intracranial pressure increased tremor hair loss chills diarrhea narcolepsy sleep

The first symptom is closely related to the etiology of this disease. Among the 70 cases of hypothalamic syndrome reported by Saxon forest, the onset of diabetes mellitus is the most common, followed by headache, vision loss, sexual dysfunction (including precocious puberty, delayed development, development). Incomplete and undeveloped), obesity and lethargy are common, rare first symptoms are fever, mental retardation, abnormal feeding (eat more, anorexia), mental or emotional disorders, coma.

1. Abnormal performance and dysfunction of the hypothalamic primary disease

(1) The manifestations of hypothalamic primary disease: related to the etiology of hypothalamic syndrome, such as the tumor in the upper saddle area, the anterior tumor of the third ventricle is easily invaded and the hypothalamus, which can cause diabetes insipidus, vision loss, headache, Vomiting, increased intracranial pressure, such as tuberculous meningitis, there are low fever, night sweats, increased erythrocyte sedimentation rate, neck stiffness, Klinefelter positive and other meningeal irritation.

(2) hypothalamic dysfunction: different parts of the hypothalamic nucleus neurons are damaged, showing different regulatory dysfunction.

1 sleep disorder: in the posterior hypothalamic lesions, most patients show lethargy, early insomnia, common types of sleepiness have the following types

A. Narcolepsy: Patients sleep at any time and on the site, ranging from a few minutes to a few hours, the most common, mostly due to brain trauma, encephalitis and so on.

B. Deep sleep syndrome (parasomnia): sleep episodes can last for several days to several weeks, but can wake up to eat, urinate, and then fall asleep, more common in the posterior hypothalamus, the upper end of the brain stem disease.

C. Sleep upside down: daytime sleepiness, nighttime excitement, can be seen in the posterior hypothalamic infection.

D. Patients with periodic kleine-levin syndrome have uncontrollable, paroxysmal sleep, each sleep lasts for several hours, several days, wake up after binge drinking, overeating, and the food intake increases several times or more, also known as Hypothalamic (central) obesity.

2 eating disorders:

A. The lesion involves the ventromedial nucleus or nodule, often resulting in more food and obesity, often accompanied by dysplasia of the sexual organs, called obesity-reproductive incompetence syndrome, obesity to face, neck and trunk are most prominent, followed by proximal limbs, Slim fingers, delicate skin, too long bones, mental retardation, sexual organ development disorders, can be complicated by diabetes insipidus.

B. lesions involving the lateral hypothalamic, ventrolateral nucleus, often anorexia, weight loss, hair loss, skin atrophy, muscle weakness, bradycardia, chills, basal metabolic rate, and even cachexia.

3 abnormal temperature regulation:

A. hypothermia: down to 36.0 ° C, visible in hemangioma.

B. Low heat: usually around 37.0 °C.

C. High fever: Relaxation type or irregular type of high heat, up to 41.0 °C, change day and night, hot body heat, while limbs are cold, breathing and heart rate are normal, general antipyretics are invalid, but chlorpromazine and large The dose of amino-pyrene can be antipyretic, (pay attention to the side effects of the drug when using the drug) physical cooling such as alcohol rubbing bath, ice water enema, ice pack cold compress is also effective.

4 mental symptoms:

A. When acute lesions, often express excitement, crying and laughing, directional loss, hallucinations, irritability, convulsions and so on.

B. When the nipples are damaged on both sides, the Korsakov syndrome, also known as amnestic syndrome, is a near-forgotten, fictional and disorientation, with a clear consciousness, and occasionally a transient state of paralysis at night. .

C. When the front of the hypothalamus is damaged, it can also cause mania, which can be seen in craniocerebral surgery and trauma.

D. After the encephalitis can also appear morbid personality, mental abnormalities, mostly due to lesions invading the hypothalamus.

5 cardiovascular symptoms: clinical manifestations are diverse, and are volatility, related to the cause and location of the lesion.

A. Hypothalamic lesions: periodic hypotension, paroxysmal hypertension, paroxysmal supraventricular tachycardia, sinus tachycardia, bradycardia, etc.

B. Saddle-third ventricle tumors, orthostatic hypotension with intermittent seizures.

C. Acute hypothalamic and brainstem lesions, myocardial infarction-like ECG changes, T wave low level, inverted, QT interval prolonged, U wave is obvious, the above ECG changes have no significant relationship with body temperature, blood electrolyte levels.

6 stomach and duodenal ulcer: acute hypothalamic lesions often occur in the stomach and duodenal ulcer and bleeding, perforation or peritonitis.

7 craniocerebral trauma, hypothalamic teratoma, cerebral glioma patients, can occur in the epileptic seizures, on the face, facial and upper chest skin flushing, a lot of sweating, runny, tears, urination, defecation, Convulsions, tremors, with or without unconscious disturbances, ranging from a few minutes to 1 to 2 hours, after a seizure, the body is weak.

2. Hypothalamic-pituitary-target gland endocrine dysfunction

Hypothalamic syndrome can cause hypothalamic release (inhibition) of hormone secretion disorders, pituitary and target gland endocrine dysfunction.

(1) Complete hypothalamic hormone secretion deficiency can cause total glandular pituitary dysfunction.

(2) Lack of hyperthyroid hormone secretion or hyperthyroidism:

1 growth hormone releasing hormone (growth release hormone) secretion is lacking, causing the secretion of pituitary growth hormone to decrease, pituitary dwarfism occurs; when hypersecretion occurs, giant disease occurs, acromegaly.

2 prolactin releasing hormone and prolactin release inhibit hormone secretion deficiency, can cause prolactin secretion reduction; when hypersecretion, can cause galactorrhea or galactorrhea-menopausal syndrome: men's breast development.

3 Gonadotropin-releasing hormone (GnRH) secretion is lacking: female amenorrhea, male libido, impotence, secondary sexual characteristics and sexual organs are not developed, Kallmann syndrome; sexual precocity occurs when hypersecretion occurs.

4 When the thyroid-stimulating hormone releasing hormone is lacking, it causes hypothalamic hypothyroidism; when hypersecretion occurs, it causes hypothalamic hyperthyroidism.

5 When the secretion of corticotropin-releasing hormone is lacking, it causes adrenal insufficiency; when hypersecretion occurs, it causes adrenal hyperplasia-type hypercortisolism.

6 When the secretion of vasopressin is lacking, it causes diabetes insipidus.

3. Vision, visual impairment

Hypothalamic syndrome occurs in the anterior hypothalamic tumor, which can cause vision loss, visual field disorder (temporal hemianopia, unilateral hemianopia, tubular vision) diplopia, optic nerve atrophy, optic disc edema, and even blindness.

4. Other diseases with hypothalamic symptoms

It is recognized that there are obesity, sexual dysplasia, low intelligence, abnormal bone development, abnormal glucose tolerance (or diabetes) and hypothalamic diseases, such as: 1 Prader-Willi syndrome (also known as low muscle) Tension-low intelligence-gonad development-obesity syndrome; 2Albright syndrome, also known as Albright bone dystrophy, bone fiber abnormal proliferation sign, multiple bone fiber malnutrition sign; 3 periodicity Periodical somnolence and morbid hunger syndrome (also known as Klein-Levin syndrome); 4 obesity-reproductive syndrome (adiposogenital syndrome) also known as Fleury syndrome (Frö - lich syndrome).

Examination of hypothalamic syndrome

Cerebrospinal fluid

When the tumor causes the disease, the protein content in the cerebrospinal fluid can be increased, the pressure of the cerebrospinal fluid can be increased, and the number of cells can be increased due to inflammation. The embryonic tissue tumor is located on the saddle, the tumor cells can fall off into the ventricle and the subarachnoid space, and the cerebrospinal fluid After ultracentrifugation, tumor cells can be found, -FP and hHCG- can be elevated in serum and cerebrospinal fluid, tuberculosis, tuberculous meningitis, protein content in cerebrospinal fluid is increased, acid-fast bacilli may be found, or tuberculosis cultured in cerebrospinal fluid Positive.

2. Determination of endocrine function of pituitary and target glands

In order to understand the presence or absence of pituitary dysfunction and gonads, thyroid, adrenal cortical secondary dysfunction, serum hormone levels can be measured separately.

(1) gonad: FSH, LH, testosterone, estradiol can be measured.

(2) Thyroid: TSH, TT3, TT4 can be measured.

(3) Adrenal cortex: ACTH, cortisol, and 24h urinary-17-hydroxycorticosteroid (170HCS), urinary free cortisol and 17-ketosteroid (17-KS) can be measured.

3. Hypothalamic-pituitary function determination

(1) TRH stimulation test: Serum TSH levels were measured before and after 15, 30, 60, 90, and 120 minutes before and after intravenous injection of TRH 200-500 g. The results were judged according to: 1 If the lesion is in the pituitary, TSH has no elevated response, 2 If the lesion is in the hypothalamus, a delayed increase may occur.

(2) LH-RH stimulation test: LH-RH 100g (dissolved in 5ml normal saline), blood LH (or HCG-LH) before and after intravenous injection, 15, 30, 60min, and then intravenous injection or After intramuscular injection of LH 100g for a total of 3 times, repeat the above blood test to determine LH, the results judged: 1 when the lesion is in the pituitary, the HCG-LH base value is low, after LH-RH injection, it shows low or no response, 2 lesions are under In the thalamus, the HCG-LH has a low baseline value and a normal or delayed response after injection of LH-RH.

(3) CRH stimulation test: first intravenous intubation, blood is taken every 15 minutes after 45 minutes, 2ml each time, a total of 2 times, as a basic value, after 100g of CRH dissolved in 1ml of acidic saline (pH 7.0, to prevent CRH In the syringe and tube wall adsorption, intravenous infusion within 1min, blood is taken every 15min within the first hour after instillation; in the second hour, blood is taken every 30min, plasma ACTH and cortisol are determined, the result is judged :1 lesions in the pituitary, ACTH, cortisol did not increase the response; 2 lesions in the hypothalamus, all showed a delayed increase in response.

(4) Insulin hypoglycemia test (insulin tolerance test): Insulin can cause hypoglycemia reaction to stimulate the release of ACTH and GH, so as to observe changes in ACTH and GH levels before and after insulin injection.

(5) Direct measurement of hypothalamic release hormone levels: such as CRH, TRH, LH-RH.

4. EEG

The EEG can be seen as a one-way normal phase spike diffuse abnormality of 14 times per second, paroxysmal release, alternating high amplitude waves, and so on.

5. Imaging examination

Feasible skull X-ray film, cerebral angiography, ventriculography, gas angiography, CT scan, MRI, endoscopic stereotactic technique, transcranial Doppler color ultrasound, etc. to detect the location and nature of intracranial lesions.

Diagnosis and diagnosis of hypothalamic syndrome

Diagnostic criteria

A major feature of hypothalamic syndrome is the diversity and complexity of symptoms, so it is not easy to diagnose. The complete diagnosis of this disease should include several aspects, namely, initial diagnosis, cause diagnosis, injury site and pathological diagnosis.

Preliminary diagnosis

(1) The premise for the diagnosis of hypothalamic syndrome is that the hypothalamus can be considered after the single target organ or pituitary lesions and systemic diseases have been excluded.

(2) According to domestic and foreign data, the most common clinical manifestations of hypothalamic diseases are: sexual dysfunction, diabetes insipidus, polyphagia, and mental disorders. Therefore, if three of them coexist, the disease should be highly suspected.

(3) In general, endocrine function and metabolic disorder are the main manifestations of hypothalamic syndrome, and have the greatest significance for diagnosis. When patients have other symptoms (such as nervous system manifestations, mental disorders, headache, fever) The disease should be highly suspected. Conversely, when patients are treated with the above symptoms, their endocrine function should be checked for abnormalities. The thin layered slices of MRI and CT sometimes show the location and nature of the lesion.

2. Cause diagnosis

(1) In terms of incidence, tumors rank first, the most common of which are craniopharyngioma and ectopic pineal tumors; followed by trauma and congenital diseases; again inflammation, granuloma and physical factors .

(2) When symptoms of increased intracranial pressure such as headache, accompanied by abnormal vision or visual field, and progressive diabetes insipidus and sexual dysfunction, tumor factors should be considered first, MRI, CT sometimes helps a lot.

(3) Some congenital lesions may have chain symptoms: such as loss of smell, deformity, developmental delay, may be Kallmann syndrome.

(4) The diagnosis of trauma, drugs and radiological factors requires detailed medical history. However, many patients still cannot find the cause, and the doctor's experience is very important.

3. Damage site

The patient's clinical manifestations sometimes reflect the location of the hypothalamic lesions, which can be referenced as follows:

Damage to the anterior region: autonomic dysfunction.

Damage to the anterior region of the hypothalamus: high fever.

Anterior hypothalamus: eating disorders.

Anterior hypothalamic and supraoptic nucleus, paraventricular nucleus, diabetes insipidus, idiopathic hypernatremia.

Abdominal lateral area damaged: anorexia, weight loss.

Impaired ventral region: bulimia, obesity, personality changes.

The ventral medial aspect of the hypothalamus is impaired in the median bulge: low sexual function, abnormal secretion of ACTH, GH, PRL, and diabetes insipidus.

Damage to the posterior hypothalamus: altered consciousness, lethargy, hypothermia, and decreased motor function.

Papillary body: mental disorders, memory disorders.

Pituitary stalk: Diabetes insipidus, partial or total hypopituitarism.

4. Pathological diagnosis

There should be a clear pathological diagnosis after tumor surgery or autopsy.

Differential diagnosis

1. hypothalamic syndrome (HTS): clinical features of endocrine and metabolic disorders, accompanied by autonomic dysfunction, its etiology and clinical manifestations vary widely, clinical symptoms and signs can not Use a single pituitary or single-target gland to explain the damage or other causes, so it is necessary to identify the specific symptoms and the results of the test.

2. The hypothalamic thyroid stimulating hormone, the standard test of luteinizing hormone releasing hormone helps identify the pituitary disease.

3. When pheochromocytoma is measured, the blood urinary catecholamine and its metabolites are increased, the histamine stimulation test is positive, and the phentolamine inhibition test is positive, which can be distinguished from this sign.

4. Some symptoms of hypothalamic syndrome can also be differentiated from neurosis, hypoglycemia, and temporal lobe epilepsy.

5. Primary pituitary disease: Does not have symptoms of hypothalamic autonomic dysfunction, insomnia, no abnormal appetite and body temperature, and no diabetes insipidus.

6. Hypothalamic functional disorder: trauma, environmental changes can also be associated with anorexia, weight loss and hypoglandular dysfunction, such as amenorrhea, impotence, etc., but no organic damage, change the environment, etc. can make it recover, under Acute dysfunction of the thalamus is seen in low-temperature, anesthesia, burns, trauma, sepsis and other emergency stress states, often accompanied by hyperglycemia, which can be restored after stress. The severity and duration of hyperglycemia are related to prognosis, occasionally There are people with hypoglycemia.

7. Eating disorders: gluttony with obesity should be distinguished from hypoglycemia, anorexia with weight loss should be differentiated from the weight loss caused by chronic wasting diseases, such as chronic infectious diseases (tuberculosis, etc.), malignant tumors (primary Liver cancer, stomach cancer) or blood diseases.

8. Obesity: Attention is distinguished from simple obesity and hypercortisolism.

9. Fever: It is necessary to exclude fever caused by other causes, such as various infectious diseases, autoimmune diseases, and tumors.

10. Drowsiness: Pay attention to the identification of hypothyroidism.

11. Emotional and mental abnormalities: Primary psychosis, hyperthyroidism, etc. should pay attention to the difference.

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