deep sleep disorder
Introduction
Introduction The posterior hypothalamic lesion can cause deep sleep: sleep for several days or weeks, in the middle can be woken up to eat or go to the bathroom, and then sleep again. 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, and 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 have been identified in hypothalamic neurons.
Cause
Cause
(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, etc.
3. Degenerative changes: brain softening, tuberous sclerosis, gliosis and so on.
4. Brain metabolic diseases: carbon dioxide anesthesia, acute intersexual hemophilic porphyria.
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: craniocerebral 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, reserpine, chlorpromazine and contraceptives can cause gluest-menopausal 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 be associated with central diabetes insipidus and decreased growth hormone secretion. MRI showed abnormalities in the pituitary gland and skull. After administration of exogenous growth hormone, 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 human life activities (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, leading to hypothalamic dysfunction, secondary pituitary and target gland dysfunction, there will be a group of endocrine and metabolic disorders, body temperature and sleep regulation abnormalities, autonomic dysfunction as the main performance Clinical syndrome.
Examine
an examination
Related inspection
EEG Dynamic Blood Pressure Monitoring (ABPM)
The first symptom is closely related to the cause of the disease. Among the 70 cases of hypothalamic syndrome reported by Sasonglin, the onset of diabetes mellitus is the most common, followed by headache, vision loss, sexual dysfunction (including precocious puberty, delayed development, hypoplasia and non-development), obesity and lethargy. More common. Uncommon first symptoms include fever, mental retardation, abnormal feeding (hyperphagia, anorexia), mental or emotional disturbances, and 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 tumor in the front of the third ventricle is easily invaded and the hypothalamus, which can cause diabetes insipidus, vision loss, headache, vomiting, and increased intracranial pressure. Such as tuberculous meningitis, there are hypothermia, night sweats, increased erythrocyte sedimentation rate, neck stiffness, positive Klinefelter signs 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. There are the following types of common sleepiness:
A. Narcolepsy: Patients sleep at any time and on the site, ranging from minutes to hours. Most common. Mostly caused by brain trauma, encephalitis and so on.
B. Deep sleep disorder (parasomnia): Sleep episodes can last from several days to several weeks, but you can wake up to eat, urinate, and then fall asleep. More common in the lower part of the 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 kleine-levin syndrome have uncontrollable episodes of sleep. Each sleep lasts for several hours and several days. After waking up, binge drinking and overeating, the food intake increases several times or more. Hypothalamic (central) obesity.
2 eating disorders: A. lesions involving the ventromedial nucleus or nodules, often lead to more food and obesity, often with sexual organ dysplasia, called obesity - reproductive incompetence syndrome. Obesity is most prominent in the face, neck and trunk. The proximal part of the limb is second, the fingers are slender, the skin is delicate, the bones are too long, the intelligence is diminished, the sexual organ development is disordered, and diabetes insipidus can be complicated. B. The lesion involves the lateral hypothalamic, ventrolateral nucleus, often anorexia, weight loss, hair loss, skin atrophy, muscle weakness, bradycardia, chills, decreased basal metabolic rate, and even cachexia.
3 abnormal body temperature regulation: A. hypothermia: below 36.0 °C. Can be seen 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 aminopyrine can be antipyretic. (Please 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 psychiatric symptoms: A. acute lesions, often showing 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 disorder. The consciousness is clear, and even a transient paralysis may occur 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. In the acute lesions of the hypothalamus and brainstem, myocardial infarction-like electrocardiogram changes may occur, T wave is low-level, inverted, QT interval is prolonged, and U wave is obvious. The above changes in ECG have no significant relationship with body temperature and 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 cranial trauma, hypothalamic teratoma, cerebral glioma patients, can occur in the epileptic seizures. At the time of onset, the skin on the face and upper chest is flushed, sweating, drooling, tearing, urinating, defecation, convulsions, tremors, and unconscious disturbances, ranging from a few minutes to 1 to 2 hours. After the attack, the body feels 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, acromegaly occurs.
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 organ development, 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 field disorder: Hypothalamic syndrome occurs in the anterior tumor of the hypothalamus, which can cause vision loss, visual field disorder (temporal hemianopia, unilateral hemianopia, tubular vision) diplopia, optic nerve atrophy, optic disc edema Even blind.
4. Other diseases with hypothalamic symptoms have been recognized as obesity, sexual dysplasia, mental retardation, abnormal bone development, impaired glucose tolerance (or diabetes) and hypothalamic disease. Such as: 1 Prader-Willi syndrome (Prader-Willi syndrome) also known as low muscle tension - low intelligence - gonadal development under the - obesity syndrome; 2 Albright syndrome, also known as Albright bone Malnutrition sign, abnormal bone fiber proliferation, multiple bone fiber dystrophy; 3 periodic somnolence and morbid syndrome (also known as Klein-Levin syndrome); 4 obesity-reproductive syndrome (adiposogenital syndrome) is also known as Frö-lich syndrome.
A major feature of hypothalamic syndrome is the diversity and complexity of symptoms, which makes it difficult to diagnose. The complete diagnosis of the 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 the patient has other symptoms (such as nervous system performance, mental disorders, headache, fever), the disease should be highly suspected. Conversely, when a patient seeks symptoms with the above symptoms, he should check whether his endocrine function is abnormal. Thin slices of the saddle 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 there are symptoms of increased intracranial pressure such as headache, accompanied by abnormal vision or visual field, and progressive diabetes insipidus and sexual dysfunction, the tumor factor should be considered first. MRI and CT sometimes help a lot.
(3) Some congenital lesions may have chain symptoms: such as loss of smell, malformation, developmental delay, may be Kallmann syndrome.
(4) Diagnosis of trauma, drugs, and radiation factors requires detailed medical history. Despite this, many patients still can't find out the cause, and the doctor's experience is very important.
3. The clinical manifestations of patients with injury sites can sometimes reflect the location of hypothalamic lesions, which can be used as follows:
Damage to the anterior region: autonomic dysfunction.
Damage to the anterior region of the hypothalamus: high fever.
Anterior hypothalamus: eating disorders.
The anterior hypothalamic and supraoptic nucleus, paraventricular nucleus: diabetes insipidus, idiopathic hypernatremia.
Abdominal lateral area damage: anorexia, weight loss.
Impaired ventral region: bulimia, obesity, personality changes.
The ventral medial aspect of the hypothalamus was impaired in the median bulge: low sexual function, ACTH, GH, abnormal secretion of 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 of tumor surgery or autopsy should have a clear pathological diagnosis.
Diagnosis
Differential diagnosis
1. Hypothalamus syndrome (HTS) is a clinical feature characterized by endocrine and metabolic dysfunction, accompanied by autonomic dysfunction. Its etiology and clinical manifestations vary widely. Symptoms and signs that appear clinically cannot be explained by damage to a single pituitary or single-targeted gland or other causes. Therefore, it is necessary to identify the specific symptoms and the results of the examination.
2. The standard test of hypothalamic thyroid releasing hormone and luteinizing hormone releasing hormone helps to identify pituitary diseases.
3. In pheochromocytoma, the blood urinary catecholamine and its metabolites were increased, the histamine challenge test was positive, and the phentolamine inhibition test was positive. Can be identified with this sign.
4. Some symptoms of hypothalamic syndrome can also be differentiated from neurosis, hypoglycemia, and temporal lobe epilepsy.
5. Primary pituitary disease: no symptoms of hypothalamic autonomic dysfunction, insomnia, no appetite and special abnormalities in body temperature, and no diabetes insipidus.
6. Hypothalamic functional disorders: traumatic, environmental changes can also be associated with anorexia, weight loss and glandular hypofunction, such as amenorrhea, impotence, etc., but no organic damage, change the environment can restore it. Acute hypofunction of the hypothalamus is seen in low-temperature, anesthesia, burns, trauma, sepsis and other emergency stress states. At this time, hyperglycemia is often accompanied by stress. The severity and duration of hyperglycemia are related to prognosis. Occasionally There are also 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: fever must be excluded from 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.
