low metabolism
Introduction
Introduction In male patients with craniopharyngioma, residual squamous epithelial cells of the craniopharyngioma with degenerative libido, low blood pressure, low metabolism (up to 35%) and other clinical symptoms may become the origin of craniopharyngioma.
Cause
Cause
The cause is unclear. The predisposing factors may be: genetic factors, physical and chemical factors, and biological factors. The clinical manifestations were mainly increased intracranial pressure, bilateral vision loss, visual field defects, endocrine dysfunction and hypothalamic symptoms. Surgical treatment is the first choice. The younger the age, the easier it is to cut completely, and the fewer complications, so early diagnosis and early treatment are the key. The disease is a congenital disease with slow growth. During normal embryonic development, the elongated tube that connects the Rathke's capsule to the original oral cavity is the craniopharyngioma, which gradually disappears as the embryo develops. The residual part of the anterior wall of the Rathke sac, the anterior lobe nodules, and the residual squamous epithelial cells of the degenerated craniopharyngioma may be the origin of the occurrence of craniopharyngioma. Therefore, craniopharyngioma can occur in the pharynx, sphenoid sinus, saddle, saddle and third ventricle, and some can invade the posterior fossa.
Most craniopharyngioma originate from residual squamous epithelial cells in the cranial buccal tube near the funnel, so the tumor is located on the saddle, forming a so-called "saddle-shaped" craniopharyngioma; a small number of tumors originate from the residual cells in the middle, then The tumor is located in the saddle and forms a so-called "saddle-shaped" craniopharyngioma. Some of the craniopharyngioma are found in the saddle and in the saddle, and the tumor is dumbbell-shaped.
Examine
an examination
Related inspection
Brain CT examination of cerebral angiography
Craniopharyngioma can be seen at any age, but it is most common in 6 to 14 years old. Most craniopharyngioma are intermittently growing, so the overall growth of the tumor is slow, and the symptoms develop slowly. A small number of craniopharyngioma grows rapidly and its disease progresses rapidly. Its clinical manifestations include the following aspects: tumor occupying effect and high intracranial pressure caused by obstruction of interventricular pores; tumor oppression and optic nerve, visual impairment caused by optic nerve; tumor compression hypothalamic and pituitary-induced hypothalamic-pituitary dysfunction The tumor invades other neurological and psychiatric symptoms caused by brain tissue. There are five main aspects:
1. Increased intracranial pressure
The volume of craniopharyngioma is large, and as an intracranial space-occupying lesion, it can directly increase the intracranial pressure through the mass effect. Craniopharyngioma can also compress the third ventricle, block the interventricular pores and increase the intracranial pressure, which may be the main cause of high intracranial pressure. Symptoms of increased intracranial pressure are more common in children. The most common manifestation is headache. It can be light or severe, more than in the morning, accompanied by vomiting, tinnitus, dizziness, photophobia, optic disc edema, nerve palsy, etc. , facial flushing, sweating and other manifestations of autonomic dysfunction. Most of the headaches are located behind the sputum, but also diffuse and radiate to the back neck and back.
Before the child's suture is closed, the suture is separated, the head circumference is enlarged, the sniper is broken, and the scalp vein is angered. Most of the patients who cause intracranial hypertension are larger cysts, and the tumors compress the third ventricle. Blocking the interventricular pores can also cause obstructive hydrocephalus. Because the pressure inside the cyst can change on its own, sometimes the symptoms of intracranial hypertension are automatically relieved. Occasionally, intratumoral cysts rupture, cyst fluid overflow into the subarachnoid space, can cause chemical meningitis and arachnoiditis, manifested as sudden severe headache, vomiting, with meningeal irritation, such as neck resistance, Kening sign positive, cerebrospinal fluid Increased leukocytosis, fever, etc. Advanced intracranial hypertension can cause coma.
2. Optic nerve compression performance
It manifests as vision, visual field changes and fundus changes. The saddle-type tumor has different compression points due to its non-regular growth direction, which makes the visual field defect very variable, which can be quadrant defect, hemianopia, dark spot and so on. Tumor oppression can cause visual field defects. Commonly, the two sides are hemianopia. For example, see the lower quadrant of the lower quadrant, which indicates that the compression is from top to bottom, and the degree of damage on both sides can be inconsistent. If the tumor only oppresses one side of the beam, it produces homonymous hemianopia. If the tumor is severely oppressive, the primary optic atrophy may occur; if the tumor invades the third ventricle, causing hydrocephalus and increased intracranial pressure, secondary optic atrophy may occur. The motor nerves of the eye can be affected and produce symptoms such as diplopia. The intra-saddle tumor is compressed from the bottom to the top, and the visual field defect is the same as that of the pituitary tumor. The vision loss is related to optic atrophy. Sometimes blindness can occur due to bleeding infarction at the intersection of the optic chiasm and blood circulation disorder. Optic disc edema is rare in people with primary optic atrophy. Foster-Kennedy syndrome can be produced when tumors grow to one side. Children do not pay attention to early visual field defects until they are severely impaired.
3. Hypothalamus symptoms
Craniopharyngioma compression of the hypothalamus and pituitary can also cause a variety of endocrine and metabolic disorders and hypothalamic dysfunction: tumor destruction of the supraoptic nucleus or neurohypophysis, can cause diabetes insipidus, the incidence of about 20%; tumor invasion of the hypothalamus The thirst center can cause the patient to have polydipsia and polydipsia or thirst; tumor invasion and satiety center can cause polyphagia or anorexia; tumor invasion and thermoregulatory center can cause fever; tumor damage and pituitary portal system or direct Invasion of the pituitary gland can cause hypopituitarism, tumor destruction of the hypothalamic TRH, CRH, GnRH neurons can cause TSH, ACTH and gonadotropin deficiency; tumor damage and hypothalamic inhibitory neurons can cause pituitary function Hyperthyroidism, common manifestations of sexual precocity, acromegaly, deepening of skin pigmentation, hypercortisolism, etc.; some patients have obesity, lethargy, mental disorders, vasomotor dysfunction and other symptoms.
(1) Obesity reproductive incompetence syndrome: the management function and reproductive activity of the nodules in the hypothalamus, and through the gonadotropin in the anterior pituitary; the funnel and gray nodules are related to fat metabolism. The compression and destruction of the above-mentioned parts can cause obesity, undeveloped sexual organs in children, disappearance of adult sexual desire, menopause, lactation disorders, and disappearance of secondary sexual characteristics.
(2) abnormal body temperature regulation: the clinical damage of the posterior hypothalamus is characterized by low body temperature (35 ~ 36 ° C), a small number of patients may have chills; the front of the hypothalamus can cause central hyperthermia (39 ~ 40 ° C ).
(3) Diabetes insipidus: It shows an increase in urine output, which can reach several thousand milliliters or even 10,000 ml per day. Therefore, a large amount of drinking water is available, and children can easily trampoline at night. The cause of diabetes insipidus is the reduction of or deficiency of vasopressin (ADH) secretion in the supraoptic nucleus, paraventricular nucleus, hypothalamic-pituitary tract or neurohypophysis, but polyuria is associated with normal secretion of ACTH, such as anterior pituitary Impaired, ACTH secretion is reduced, it will not cause urine collapse. Sometimes due to the hypothalamic thirst center and destruction at the same time, it can produce diabetes insipidus with thirst sensation syndrome. Although the patient has diabetes collapse and high plasma permeability, there is no thirst. In the case of banned drinking, the osmolality does not rise or rise slightly, blood volume decreases, and hypernatremia. Patients can develop headaches, tachycardia, irritability, confusion, paralysis and even coma, sometimes producing paroxysmal hypotension.
(4) lethargy: seen in advanced cases, light can still wake up, heavy people sleep all day long.
(5) Psychiatric symptoms: such as forgetfulness, inattention, fiction, etc., related to the damage of the hypothalamic-marginal system or the hypothalamic frontal lobe, more common in adults.
(6) Bulimia or refusal to eat: The saphenous nucleus of the hypothalamic nucleus may have bulimia (patient obesity), and the eclipse of the ventrolateral nucleus may have anorexia or refusal (patient wasting). Less clinically seen.
(7) Hyperprolactin (PRL): In a small number of cases, the tumor affects the hypothalamus or pituitary stalk, resulting in decreased secretion of prolactin inhibitor (PIF), increased secretion of PRL cells in the anterior pituitary, and clinically produced galactorrhea-menopausal syndrome.
(8) Loss of pituitary hormone secretion: The hypothalamus may cause loss of GHRH, TRH, CRH secretion, and clinical manifestations affect growth and thyroid and adrenal dysfunction.
4. Pituitary dysfunction symptoms
Pituitary dysfunction is more common than pituitary hyperfunction, especially in LH/FSH and GH deficiency. It is reported that about 50% of children have delayed growth, and about 10% have obvious short stature with sexual dysplasia. The performance of GH deficiency in adult patients is not prominent, but there are more than 30% of sexual dysfunction. Secondary hypothyroidism caused by insufficient TSH is seen in about one-fourth of patients, and secondary adrenal insufficiency caused by insufficient ACTH is not uncommon.
Early manifestations of dysfunction of pituitary in children are physiologic retardation, short stature, thinness, fatigue, fatigue, decreased activity, smooth and pale skin, yellow complexion, wrinkles, and looks like old age. The teeth and bones stop developing, the bones do not unite or delay the joint, the sexual organs are infant-type, there is no secondary sexual characteristics, and there are also those with no testicular syndrome. A few may have cold, mild mucous edema, low blood pressure, and even Simmond cachexia. Adult women have menstrual disorders or menopause, infertility and premature aging. Men have decreased libido, hair loss, low blood pressure, low metabolism (up to 35%).
5. Adjacent symptoms
Tumors can grow around, such as growing to the sides, invading the sacral leaves, can cause temporal lobe epilepsy. The tumor expands downward and invades the brain and feet, which can produce spasmodic hemiplegia and even go to the state of brain toughness. Some patients may have mental disorders, manifested as memory loss or even loss, emotional apathy, severe confusion or dementia. Such as the growth of the saddle can produce cavernous sinus syndrome, causing III, IV, VI on the cranial nerve disorders; to the sphenoid sinus, ethmoid sinus growth can cause nosebleeds, cerebrospinal fluid rhinorrhea, etc.; Produce psychiatric symptoms, such as memory loss, poor orientation, inability to take care of themselves, as well as epilepsy, olfactory disorders, etc.; growth to the mid-cranial fossa can produce temporal symptoms such as temporal lobe epilepsy and illusion, illusion; Backward growth produces brainstem symptoms, even to the cranial fossa causing cerebellar symptoms. A small number of patients can also be affected by the olfactory and facial nerves, which are characterized by loss of olfactory and facial paralysis.
The above symptoms are slightly different in children and young patients and adult patients. The first symptoms are more common in intracranial hypertension, and the latter are more common in optic nerve compression. All patients may have endocrine changes, but adults find Earlier.
Laboratory inspection:
(1) In patients with decreased pituitary function, gonadotropin and growth hormone decreased significantly, thyroid hormone and TSH decreased; basal metabolic rate decreased; glucose tolerance was often reduced.
(2) The pressure of cerebrospinal fluid is increased, and the number of white blood cells and protein can be slightly increased.
(3) Skull X flat film: calcification in the saddle area, enlargement and destruction of the sella.
(4) CT and MRI scans
Tumors can be seen in the saddle area.
(5) Cerebral angiography: showing the tumor on the saddle.
Patients with any age, such as high intracranial pressure, neurological ophthalmology and hypothalamic-pituitary dysfunction should consider the possibility of craniopharyngioma. It is not difficult to diagnose craniopharyngioma based on the location, clinical manifestations and auxiliary examination. All adolescents and children with endocrine dysfunction, such as stunting, polydipsia, obesity, genital dysplasia, etc., should first consider the disease; if there are saddle or saddle calcification, it is more helpful for diagnosis. If an adult has sexual dysfunction or headache, vision and visual impairment, this disease should also be considered.
A small number of patients with atypical clinical manifestations and mild clinical symptoms are not easy to diagnose. The key is to improve the vigilance of this disease. It is of great significance for diagnosis through laboratory examination, CT and MRI. Such examination should be done in time for suspected cases to avoid delay in diagnosis.
Diagnosis
Differential diagnosis
Differential diagnosis of low metabolism:
1, systemic metabolism is low: the main manifestation is that the systemic metabolic rate slows down. It is a systemic manifestation of endocrine and metabolic diseases. Lack of tissue specificity and organ specificity in endocrine and metabolic diseases. The hormone secreted by the endocrine glands enters the blood circulation and exerts its physiological effects as it reaches the various organs and tissues of the body. A hormone can act on multiple sites, and multiple hormones can also act on the same organ tissue. Therefore, the clinical manifestations of a certain endocrine gland dysfunction will involve multiple systems and organs throughout the body, unlike other systems with more common symptoms associated with this system.
2, glucose metabolism disorder: regulation of glucose, fructose, galactose and other metabolic hormones or enzyme structure, function, concentration abnormalities or pathological changes of tissues and organs.
3, water and salt metabolism disorders: water and inorganic salts (electrolytes) is an important component of the body, but also an important component of body fluids. Body fluid is the internal environment of cell life activity, and its constant capacity, osmotic pressure, pH and appropriate ion concentration play an important role in ensuring the normal metabolism of cells. For example, hypotonic water shortage is the simultaneous loss of water and sodium, but the lack of water is less than the loss of sodium. This situation is the metabolic imbalance between water and inorganic salts.
4. Reduction of bone metabolism: Systemic bone disease caused directly or indirectly by metabolic disorders such as calcium and phosphorus, but may also be highlighted as a bone change in a certain part of the body. Bone metabolic diseases mainly manifest as transitional disorders or abnormalities between bone formation and bone resorption. Bone formation or bone resorption can be reduced or increased, bone matrix formation can be lacked or increased, mineralization can be lacked, insufficient or excessively deposited. As a result, bone can show looseness, softening, hardening or excessive calcification. More than two performances.
Craniopharyngioma can be seen at any age, but it is most common in 6 to 14 years old. Most craniopharyngioma are intermittently growing, so the overall growth of the tumor is slow, and the symptoms develop slowly. A small number of craniopharyngioma grows rapidly and its disease progresses rapidly. Its clinical manifestations include the following aspects: tumor occupying effect and high intracranial pressure caused by obstruction of interventricular pores; tumor oppression and optic nerve, visual impairment caused by optic nerve; tumor compression hypothalamic and pituitary-induced hypothalamic-pituitary dysfunction The tumor invades other neurological and psychiatric symptoms caused by brain tissue. There are five main aspects:
1. Increased intracranial pressure: The volume of craniopharyngioma is larger. As an intracranial space-occupying lesion, it can directly increase the intracranial pressure through the mass effect. Craniopharyngioma can also compress the third ventricle, block the interventricular pores and increase the intracranial pressure, which may be the main cause of high intracranial pressure. Symptoms of increased intracranial pressure are more common in children. The most common manifestation is headache. It can be light or severe, more than in the morning, accompanied by vomiting, tinnitus, dizziness, photophobia, optic disc edema, nerve palsy, etc. , facial flushing, sweating and other manifestations of autonomic dysfunction. Most of the headaches are located behind the sputum, but also diffuse and radiate to the back neck and back.
Before the child's suture is closed, the suture is separated, the head circumference is enlarged, the sniper is broken, and the scalp vein is angered. Most of the patients who cause intracranial hypertension are larger cysts, and the tumors compress the third ventricle. Blocking the interventricular pores can also cause obstructive hydrocephalus. Because the pressure inside the cyst can change on its own, sometimes the symptoms of intracranial hypertension are automatically relieved. Occasionally, intratumoral cysts rupture, cyst fluid overflow into the subarachnoid space, can cause chemical meningitis and arachnoiditis, manifested as sudden severe headache, vomiting, with meningeal irritation, such as neck resistance, Kening sign positive, cerebrospinal fluid Increased leukocytosis, fever, etc. Advanced intracranial hypertension can cause coma.
2. Optic nerve compression performance
It manifests as vision, visual field changes and fundus changes. The saddle-type tumor has different compression points due to its non-regular growth direction, which makes the visual field defect very variable, which can be quadrant defect, hemianopia, dark spot and so on. Tumor oppression can cause visual field defects. Commonly, the two sides are hemianopia. For example, see the lower quadrant of the lower quadrant, which indicates that the compression is from top to bottom, and the degree of damage on both sides can be inconsistent. If the tumor only oppresses one side of the beam, it produces homonymous hemianopia. If the tumor is severely oppressive, the primary optic atrophy may occur; if the tumor invades the third ventricle, causing hydrocephalus and increased intracranial pressure, secondary optic atrophy may occur. The motor nerves of the eye can be affected and produce symptoms such as diplopia. The intra-saddle tumor is compressed from the bottom to the top, and the visual field defect is the same as that of the pituitary tumor. The vision loss is related to optic atrophy. Sometimes blindness can occur due to bleeding infarction at the intersection of the optic chiasm and blood circulation disorder. Optic disc edema is rare in people with primary optic atrophy. Foster-Kennedy syndrome can be produced when tumors grow to one side. Children do not pay attention to early visual field defects until they are severely impaired.
3. Hypothalamus symptoms
Craniopharyngioma compression of the hypothalamus and pituitary can also cause a variety of endocrine and metabolic disorders and hypothalamic dysfunction: tumor destruction of the supraoptic nucleus or neurohypophysis, can cause diabetes insipidus, the incidence of about 20%; tumor invasion of the hypothalamus The thirst center can cause the patient to have polydipsia and polydipsia or thirst; tumor invasion and satiety center can cause polyphagia or anorexia; tumor invasion and thermoregulatory center can cause fever; tumor damage and pituitary portal system or direct Invasion of the pituitary gland can cause hypopituitarism, tumor destruction of the hypothalamic TRH, CRH, GnRH neurons can cause TSH, ACTH and gonadotropin deficiency; tumor damage and hypothalamic inhibitory neurons can cause pituitary function Hyperthyroidism, common manifestations of sexual precocity, acromegaly, deepening of skin pigmentation, hypercortisolism, etc.; some patients have obesity, lethargy, mental disorders, vasomotor dysfunction and other symptoms.
(1) Obesity reproductive incompetence syndrome: the management function and reproductive activity of the nodules in the hypothalamus, and through the gonadotropin in the anterior pituitary; the funnel and gray nodules are related to fat metabolism. The compression and destruction of the above-mentioned parts can cause obesity, undeveloped sexual organs in children, disappearance of adult sexual desire, menopause, lactation disorders, and disappearance of secondary sexual characteristics.
(2) abnormal body temperature regulation: the clinical damage of the posterior hypothalamus is characterized by low body temperature (35 ~ 36 ° C), a small number of patients may have chills; the front of the hypothalamus can cause central hyperthermia (39 ~ 40 ° C ).
(3) Diabetes insipidus: It shows an increase in urine output, which can reach several thousand milliliters or even 10,000 ml per day. Therefore, a large amount of drinking water is available, and children can easily trampoline at night. The cause of diabetes insipidus is the reduction of or deficiency of vasopressin (ADH) secretion in the supraoptic nucleus, paraventricular nucleus, hypothalamic-pituitary tract or neurohypophysis, but polyuria is associated with normal secretion of ACTH, such as anterior pituitary Impaired, ACTH secretion is reduced, it will not cause urine collapse. Sometimes due to the hypothalamic thirst center and destruction at the same time, it can produce diabetes insipidus with thirst sensation syndrome. Although the patient has diabetes collapse and high plasma permeability, there is no thirst. In the case of banned drinking, the osmolality does not rise or rise slightly, blood volume decreases, and hypernatremia. Patients can develop headaches, tachycardia, irritability, confusion, paralysis and even coma, sometimes producing paroxysmal hypotension.
(4) lethargy: seen in advanced cases, light can still wake up, heavy people sleep all day long.
(5) Psychiatric symptoms: such as forgetfulness, inattention, fiction, etc., related to the damage of the hypothalamic-marginal system or the hypothalamic frontal lobe, more common in adults.
(6) Bulimia or refusal to eat: The saphenous nucleus of the hypothalamic nucleus may have bulimia (patient obesity), and the eclipse of the ventrolateral nucleus may have anorexia or refusal (patient wasting). Less clinically seen.
(7) Hyperprolactin (PRL): In a small number of cases, the tumor affects the hypothalamus or pituitary stalk, resulting in decreased secretion of prolactin inhibitor (PIF), increased secretion of PRL cells in the anterior pituitary, and clinically produced galactorrhea-menopausal syndrome.
(8) Loss of pituitary hormone secretion: The hypothalamus may cause loss of GHRH, TRH, CRH secretion, and clinical manifestations affect growth and thyroid and adrenal dysfunction.
4. Pituitary dysfunction symptoms
Pituitary dysfunction is more common than pituitary hyperfunction, especially in LH/FSH and GH deficiency. It is reported that about 50% of children have delayed growth, and about 10% have obvious short stature with sexual dysplasia. The performance of GH deficiency in adult patients is not prominent, but there are more than 30% of sexual dysfunction. Secondary hypothyroidism caused by insufficient TSH is seen in about one-fourth of patients, and secondary adrenal insufficiency caused by insufficient ACTH is not uncommon.
Early manifestations of dysfunction of pituitary in children are physiologic retardation, short stature, thinness, fatigue, fatigue, decreased activity, smooth and pale skin, yellow complexion, wrinkles, and looks like old age. The teeth and bones stop developing, the bones do not unite or delay the joint, the sexual organs are infant-type, there is no secondary sexual characteristics, and there are also those with no testicular syndrome. A few may have cold, mild mucous edema, low blood pressure, and even Simmond cachexia. Adult women have menstrual disorders or menopause, infertility and premature aging. Men have decreased libido, hair loss, low blood pressure, low metabolism (up to 35%).
5. Adjacent symptoms
Tumors can grow around, such as growing to the sides, invading the sacral leaves, can cause temporal lobe epilepsy. The tumor expands downward and invades the brain and feet, which can produce spasmodic hemiplegia and even go to the state of brain toughness. Some patients may have mental disorders, manifested as memory loss or even loss, emotional apathy, severe confusion or dementia. Such as the growth of the saddle can produce cavernous sinus syndrome, causing III, IV, VI on the cranial nerve disorders; to the sphenoid sinus, ethmoid sinus growth can cause nosebleeds, cerebrospinal fluid rhinorrhea, etc.; Produce psychiatric symptoms, such as memory loss, poor orientation, inability to take care of themselves, as well as epilepsy, olfactory disorders, etc.; growth to the mid-cranial fossa can produce temporal symptoms such as temporal lobe epilepsy and illusion, illusion; Backward growth produces brainstem symptoms, even to the cranial fossa causing cerebellar symptoms. A small number of patients can also be affected by the olfactory and facial nerves, which are characterized by loss of olfactory and facial paralysis.
The above symptoms are slightly different in children and young patients and adult patients. The first symptoms are more common in intracranial hypertension, and the latter are more common in optic nerve compression. All patients may have endocrine changes, but adults find Earlier.
Laboratory inspection:
(1) In patients with decreased pituitary function, gonadotropin and growth hormone decreased significantly, thyroid hormone and TSH decreased; basal metabolic rate decreased; glucose tolerance was often reduced;
(2) The pressure of cerebrospinal fluid is increased, and the number of white blood cells and protein can be slightly increased;
(3) Skull X flat film: calcification in the saddle area, enlargement and destruction of the sella;
(D) CT, MRI scan: visible tumor in the saddle area.
(5) Cerebral angiography: showing the tumor on the saddle.
Patients with any age, such as high intracranial pressure, neurological ophthalmology and hypothalamic-pituitary dysfunction should consider the possibility of craniopharyngioma. It is not difficult to diagnose craniopharyngioma based on the location, clinical manifestations and auxiliary examination. All adolescents and children with endocrine dysfunction, such as stunting, polydipsia, obesity, genital dysplasia, etc., should first consider the disease; if there are saddle or saddle calcification, it is more helpful for diagnosis. If an adult has sexual dysfunction or headache, vision and visual impairment, this disease should also be considered.
A small number of patients with atypical clinical manifestations and mild clinical symptoms are not easy to diagnose. The key is to improve the vigilance of this disease. It is of great significance for diagnosis through laboratory examination, CT and MRI. Such examination should be done in time for suspected cases to avoid delay in diagnosis.
