MSA of unknown etiology. There are currently involved in lipid peroxidation, enzyme metabolism, slow virus infection, apoptosis of neurons, oligodendrocytes and other cytoplasmic inclusions, leading to progressive multi-system degeneration of the nervous system.
(B) the pathogenesis
1. oligodendrocyte cytoplasmic inclusions
oligodendrocyte cytoplasmic inclusions (oligodendroglial cytoplasmic inclusion) is the histological features of MSA, oligodendrocytes in the pathogenesis play an important role. That in the past few pathological changes in MSA, neuronal degeneration, demyelination is primary, and is the basis of pathological changes, while demyelination is secondary in nature. Since the discovery of oligodendrocyte cytoplasmic inclusions since some authors in the pathogenesis of MSA proposed a new view that oligodendrocytes play in the pathogenesis of neuronal degeneration and an equally important role on the grounds that silver staining and immunohistochemistry showed oligodendrocyte cells within the abnormal changes than the neurons themselves more visible and more features. Nakazato Yoichi other observed oligodendrocyte cytoplasmic inclusions density and distribution of disease location and severity of degeneration consistent. But there are also authors that oligodendrocyte number of cytoplasmic inclusions how much the severity of lesions with MSA no significant correlation. Papp and other observed higher density of oligodendrocytes in the site of the primary motor cortex, pyramidal and extrapyramidal system, cerebellar cortical projection fibers, brainstem autonomic network hub. oligodendrocytes of the main function is to maintain the integrity of myelinated fibers myelin, oligodendrocytes when abnormal internal structure, its function must be affected, which might be an important reason for demyelination.
2. Some people think that neuronal apoptosis in the pathogenesis and neuronal apoptosis. There are two types of nervous system neuronal death: necrosis and apoptosis (apoptosis). apoptosis when the cell membrane to maintain the integrity of, and showed smaller cell size, organelle structure and morphology are present, save the lysosomal components, nuclear chromatin condensation, dna endonuclease endogenous activation of dna degradation of dna fragmentation and apoptotic bodies.
4. pathology gross specimen shows that the cerebellum, brainstem and spinal cord atrophy, thinning; microscope, these particular parts of the nerve cell degeneration and depigmentation, gliosis and myelinated fiber demyelination. The main parts of the pathological changes in the transverse pontine fibers bridge, pontine base core, medullary inferior olivary nucleus, dorsal vagal nucleus, locus coeruleus, cerebellum, offal, and hemispheric cerebellar dentate nucleus, substantia nigra and basal ganglia of the globus pallidus , caudate nucleus, putamen, spinal cord intermediolateral column cells, anterior horn cells and other parts of the neuronal loss and gliosis; corticospinal tract degeneration, sheath depigmentation. Mainly peripheral nerve demyelination.
(1) blue plaques in striatum and substantia nigra lesions: patients with Parkinson's syndrome, leading to the main reason for loss of neurons in substantia nigra with the outer 1 / 3 of the substantia nigra pigmented cells disappear; early stage of striatal neuronal loss, to the dorsal putamen after 2 / 3 of the most serious; extensive involvement of the globus pallidus, locus coeruleus neuronal loss; the pathological changes of idiopathic fully consistent with Parkinson's disease.
(2) Bridge nuclear and cerebellar Purkinje cell disease: clinical with OPCA its prominent symptoms. neurons have lost significant parts of the bridge nuclear, cerebellar Purkinje cells and inferior olivary nucleus, which involved the cerebellum feet more obvious, but the cerebellar granule cells, dentate nucleus and cerebellar peduncle is usually no significant change.
(3) autonomic neuropathy: autonomic failure and pathological changes of the spinal cord are mainly located in the intermediolateral cell column and dorsal vagal nucleus, the lesion involved the sympathetic and parasympathetic systems. Other cells involved in the ventrolateral reticular formation in the brain stem monoamine neurons and the arcuate nucleus cells. ventral section of sacral spinal cord anterior horn cells, 2,3 the Onuf's nuclear system in the bladder and rectal sphincter control nerve center of their own, there are obvious damage. the hypothalamus shows mild neuronal loss.
(4) oligodendrocyte cytoplasmic inclusions: the most common and characteristic pathological changes is Gallyas staining and immunohistochemical staining or modified Bielschowsky silver stain method in the white matter oligodendrocytes have extensive diffuse cytoplasmic inclusions, also called "oligodendrocytes cell tangles like inclusions (oligodendroglial tangle-like inclusion)", or "glial cytoplasmic inclusions (glial cytoplasmic inclusion, GCI) "," oligodendrocyte microtubule tangles (oligodendroglial microtubular tangle) ". Mainly in the small circle around the nucleus of the half-moon / sickle-shaped, or close to the flame nucleus argyrophilic structures, which mainly changes the composition of microtubules. Electron microscope, the inclusion of diameter 10 ~ 25nm, the fine granular material consisting of pipe network structure, mainly in the brain stem, basal ganglia, cerebellum and cerebral cortex of the white matter, and their number varies. Application of multi-polyacrylamide gel electrophoresis can be oligodendrocytes isolated cytoplasmic inclusions with a variety of proteins, which mainly contain α-synuclein (α-synuclein), αB-crystallin (& alpha ; B-crystallin), tubulin (tubulin) and ubiquitin. These proteins are cytoskeletal proteins. There are authors that oligodendrocytes may represent cytoplasmic inclusions synchronous degeneration of neurons, neuronal degeneration may be a phenomenon before, as a special sign of pathology diagnosis of MSA, because these inclusion bodies found in almost pathologically confirmed All the MSA patients but not in the control group. These structures support the OPCA, SDS and SND is the same disease process variation concept. At present, most report that oligodendrocyte cytoplasm inclusions seen only in sporadic cases of MSA brain and spinal cord white matter, but not in simple or hereditary spinocerebellar degeneration of the central nervous system white matter. This will help MSA and pathological features of hereditary spinocerebellar degeneration disease identification. There are authors that oligodendrocyte cytoplasm inclusions and other neurodegenerative diseases in the inclusion bodies, is an α-synuclein gene with mutations that led to α-synuclein coding and structure biochemical abnormalities synuclein nerve disease.
in the pathological diagnosis of MSA, at least the putamen and the substantia nigra and other three lesions can be determined. SDS, SND and OPCA pathology often overlap, such as SDS cases showed lateral horn cells of spinal cord demyelination evident, but the latter with clinical autonomic dysfunction is not entirely consistent, but there are some cases of OPCA has the same damage. Characteristic pathological manifestations of SND putamen neuronal loss and heme (haematin) and lipofuscin (1ipofuscin) deposition and a qualitative change of the black. OPCA cerebellar cortex and the basic lesions of pontine, inferior olivary nucleus atrophy, neurons and transverse fibers decreased, olive severe neuron loss and marked gliosis. depigmentation of cerebellar Purkinje cells, granular layer thinning, especially the vermis.
Mcleod and bennet et al reported pathological changes in peripheral nerve myelinated fibers decreased root ganglia after axonal degeneration, myelinated fibers did not see no change. But Guo Yupu other on the MSA with peripheral neuropathy in 7 patients, sural nerve biopsy showed myelinated fibers were mild to moderate depigmentation, seen to form a thin myelinated and myelin-based, but also changes in hypertrophy and regeneration of nerve fibers, not See axonal degeneration. Electron microscopy in some cases to reduce the non-medullated fibers, and schwann cells and collagen fibers, and collagen capsule formation, consistent with chronic myelogenous off disease. MSA failed to confirm root ganglion cells after primary degeneration of evidence.[Sign]
MSA age of onset in middle age or early old age (32 to 74 years), of which 90% was 40 to 64 years earlier than idiopathic Parkinson's disease, duration 3 to 9 years.
There are three main clinical symptoms: the cerebellar symptoms, extrapyramidal symptoms, autonomic symptoms. 89% had Parkinson's syndrome; 78% of the autonomic nervous system failure occurs; 50% of cerebellar ataxia appear. The most common combination of parkinsonism and autonomic failure or cerebellar ataxia and autonomic failure. In addition, a considerable part may have pyramidal tract signs, brain stem damage (paralysis of eye muscles), cognitive function disorders.
1. latent onset, slowly progressive, gradually increased.
2. from a single system to a multi-system development, the groups of symptoms may have appeared, with overlap and mix.
SND and OPCA easily evolve into MSA. xu Xiaoxiang reported first symptoms have appeared after an average of 3 years other parts of the nervous system involvement of clinical symptoms, compare the extent of damage: the autonomic nervous symptoms SDS> OPCA> SND, cerebellar symptoms of OPCA> SDS> SND, extrapyramidal symptoms SND> SDS > OPCA, pyramidal tract signs SND ≥ SDS> OPCA, brain stem damage OPCA> SDS.
3. clinical and pathological findings of phase separation, pathological findings of lesions involving the scope of clinical findings is often more extensive than this separation of complex compensatory mechanisms may also Clinical examination and clinical manifestations of lagging behind the careless or pathological prejudice.
in a group of 188 cases of pathologically confirmed MSA patients, 28% of patients exist cerebellar, extrapyramidal, autonomic nervous system and the system of four kinds of cones and signs; another 29 % of patients also have Parkinson's syndrome, autonomic dysfunction and cerebellar signs, or pyramidal signs three kinds of symptoms and signs; 11% of patients with Parkinson's syndrome and autonomic nerve damage symptoms; 10% of the patients showed only Parkinson's comprehensive levy.
Sakakibara of 121 cases of MSA patients (OPCA 48 例, SND l7 cases, SDS 56 cases) conducted a questionnaire survey showed that MSA patients with urinary symptoms (96%) was common in orthostatic hypotension symptoms (43%) (P <0.01), especially in OPCA and SND. 53 patients, and urinary symptoms were orthostatic hypotension, urinary system, the first symptom of 48%, compared to orthostatic hypotension as the first symptom (29%) more common, 23% of patients while the existence of two symptoms. Thus the authors concluded: MSA urinary dysfunction in patients with orthostatic hypotension than the more common, and often appeared earlier.
as disease incidence and prevalence rates lower course of the disease in different parts of the nervous system involved in the clinical manifestations as the first symptom, often a system damage To highlight the performance of other systems of clinical symptoms are relatively mild damage, or to appear late stage, so early a clear clinical diagnosis more difficult.
1999 in the United States such as Michigan Gilman made a MSA of four clinical features and diagnostic criteria
1. Clinical features
(1) autonomic failure and (or) voiding dysfunction.
(2) Parkinson's syndrome.
(3) cerebellar ataxia.
(4) corticospinal tract dysfunction.
2. Diagnostic criteria
(1) may MSA: the first clinical features plus two other features.
(3) diagnosis of MSA: nerve biopsy confirmed.[Aftertreat]
ate multiple system atrophy?
above information is for reference only, please consult a doctor.[Prevent]
hypoproteinemia should be how to prevent?
should first treatment-induced protein intake, excessive loss, the decomposition of hyperthyroidism primary disease. If the primary disease without contraindications, may be given a high protein, high calorie diet, the daily protein intake of up to 60 ~ 80g, to ensure an adequate supply of calories (2500 kcal / day or more), and appropriate use of drugs to promote protein synthesis. poor digestion, could be the flow of food or semi-liquid food, and enough vitamin. Severe cases may enter the plasma or albumin.[Treat]
(1) SND to type the whole duration of extrapyramidal symptoms, small amplitude finger twitch muscle spasm or irregular beating of the hand tremor, often bilateral symmetry of onset, poor efficacy of levodopa or invalid.
(2) SDS model to autonomic dysfunction is prominent, from dizziness, loss of libido, urinary frequency and other progressive development of impotence can occur, no sweat, fever, orthostatic the performance of such failure, syncope, orthostatic hypotension, which is most prominent.
(1) kidney yin and yang deficiency, marrow dystrophy and lack of blood, mainly from the dizziness, exercise less, retardation, ataxia, or tremor, less sweat or no sweat half-length, pale, impotence, urine, weakness, pale, tongue white, pulse was weak. (2) phlegm in the resistance, did not rise or Qing yang yu and heat, mainly dizziness and lethargy, insomnia, tongue strong words Jian, exercise less, slow, numbness, cough, sputum, loss of appetite, abdominal distension, frequent urination, constipation, impotence, or see impatient interference restless, Fan, sputum yellow, pale or red tongue, greasy or yellow thick greasy tongue coating, slippery pulse or slide. dialectical therapy using marrow soup former, the latter through the use of marrow soup. Also for orthostatic hypotension, dry stool, frequent urination or incontinence, sleep talking and sleep behavior disorder, etc. have corresponding dialectical therapy.
treatment had no effective therapy, usually with support and symptomatic treatment.
(1) physical therapy: in a variety of measures, should be the first to use physical therapy, because these treatments can be simple, practical and often control symptoms, such as lying to the head and trunk should be higher than the lower limb 15 ° ~ 20 °, to wearing tights and stockings, tilt table exercise every day (daily tilt- exercise) and so on.
peripheral α1 adrenergic agonist midodrine (midodrine hydrochloride) is an effective drug, can increase orthostatic hypotension in patients with peripheral vascular resistance, improve the patient's systolic blood pressure, improve circulation of blood volume due to lack of emergence of dizziness and orthostatic hypotension; give 2.5mg, 2 times / d; with good physiological tolerance. The main side effects of heart rate, vertical hair reactions, urinary retention, and supine blood pressure.
fludrocortisone (9α-fludrocortisone), began a few days every 0.1mg, and gradually increased to 0.3mg ~ 1.0mg, based on changes in blood pressure and plasma volume change to adjust the dose. Should carefully monitor patients to avoid water retention and high blood pressure. L - Su -3,4 - Dihydro-phenyl serine (DOPS) on orthostatic hypotension may also be effective.
Diamond, etc. to give tyramine-rich foods (such as dry yeast 9 ~ 18g / d and beer 500ml / d) and oral administration of monoamine oxidase inhibitors (such as isoniazid, 0.6 g / d, orally or by intravenous infusion; or furazolidone 0.3g / d), to promote peripheral sympathetic norepinephrine release and inhibition of sympathetic nerve endings of the re-absorption to treat the disease.
recent years the use of Zhamo Castro (Xamoterol) treatment, 67% reduction in episodes of orthostatic hypotension, in particular, can increase diastolic blood pressure, it is worth a try. indomethacin (indomethacin) and β-adrenergic blockers such as propranolol (Inderal) and other drugs can also try.
(4) Recent studies suggest that intake of water available and effective vasoconstriction, increased blood pressure effect, and this can not currently understood physiology and pathophysiology learning mechanism to explain. In some patients, blood pressure can increase the water intake of 50%, and vasoconstriction than the application of existing drugs more effective. Vasoconstrictor effect of the use of water and carbohydrate-rich foods combined inhibition of caution, many MSA patients can now control blood pressure without medication interference (Robertson, 2001).
(b) the prognosis
As of 1995, a total of 300 cases of foreign literature neuropathological examination confirmed the MSA patients , of which 200 cases of survival time of 5 to 6 years, the longest survival of 10 years.
3. terminally ill because of throat muscle paralysis caused by drinking cough and difficulty in swallowing or aspiration occurred aspiration pneumonia, prolonged bed rest to merge pressure ulcers, lung infections and urinary system infection.
4. late disease throat, muscle weakness occurs due to sleep apnea, night wheezing and cyanosis can occur at any time, airway obstruction, central apnea can also be sudden, heart arrest stop, or chronic central respiratory failure, involving life.
hypoproteinemia should be how?
mainly malnutrition. Mainly proteins in the blood plasma and red blood cells contain hemoglobin protein. plasma proteins including albumin, various globulin, fibrinogen, and a small amount of binding proteins such as glycoproteins, lipoproteins, etc., the total amount of 6.5 ~ 7.8g%. If the plasma total protein less than 6.0g%, can be diagnosed with hypoproteinemia.[Diff]
hypoproteinemia need to do the following identification.
① protein intake or malabsorption. Various causes of loss of appetite and anorexia, such as severe heart, lung, liver, kidney disease, gastrointestinal tract congestion, brain lesions; gastrointestinal obstruction, feeding difficulties, such as esophageal cancer, gastric cancer; chronic pancreatitis, biliary tract disease, gastrointestinal anastomosis due to malabsorption syndrome.
③ large quantities of protein loss. Gastrointestinal ulcers, hemorrhoids, hookworm, menorrhagia, wound exudate such a large area can lead to loss of a large number of plasma proteins. Repeated abdominal paracentesis, peritoneal dialysis treatment with end-stage renal disease by peritoneal loss of protein can be. Nephrotic syndrome, lupus nephritis, malignant hypertension, diabetes, kidney disease may have proteinuria, protein from urine loss. gastrointestinal cancer and giant hypertrophic gastritis, protein leakage of gastrointestinal disease, ulcerative colitis, enteritis, also lost a large number of proteins by the digestive tract.
mainly malnutrition. Mainly proteins in the blood plasma and red blood cells contain hemoglobin protein. plasma proteins including albumin, various globulin, fibrinogen, and a small amount of binding proteins such as glycoproteins, lipoproteins, etc., the total amount of 6.5 ~ 7.8g%. If the plasma total protein less than 6.0g%, can be diagnosed with hypoproteinemia.[Disease]
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