Overview: central pontine myelinolysis (central pontine myelinolysis, CPM) symmetry is the base of the pons demyelination as the pathological features of the disease can be fatal. The disease is a rare acute myelin solubility lesions. Now many think that is due to hyponatremia, or rather is due to correct hyponatremia too quickly caused.
Adams et al (1959) first reported. Adams et al in 1950 first observed that a young person with chronic alcoholism, hospitalization due to symptoms of alcohol kick in a few days to become quadriplegic and pseudo-bulbar paralysis, and soon died. pathological examination of the major findings is a large area of the base of the symmetry of the pontine demyelination, then derives its name.[Cause]
early cases, found that most patients with severe or chronic wasting disease patients, especially malnutrition, chronic alcoholism, and more with wernicke encephalopathy exist, so that its incidence and malnutrition. However, in some cases, hard to find the factors of malnutrition there.
now the majority of authors believe that this disease is the cause of hyponatremia with rapid or excessive correction of hyponatremia caused. hyponatremia in the hypotonic state of brain tissue, too quickly added hypertonic saline to correct hyponatremia to the rapid increase in plasma osmolality, caused by dehydration of brain tissue and blood - brain barrier damage, harmful substances through the blood - brain barrier can lead to demyelination.
(B) the pathogenesis
animal experiments on dogs with repeated injection of angiotensin (vasopressin) and cause severe abdominal injection hyponatremia (100 ~ 115meq / L), and injection of hypertonic saline (3%) to correct their hyponatremia and also the serious weakness. The dogs soon thereafter appeared ankylosing quadriplegia, the pathological changes of the human central pontine myelinolysis is almost impossible to distinguish (Laureno, 1983). However, McKee et al (1988) reported that in 139 autopsy cases of severe burn patients, 10 were typical of central pontine demyelination. according to histological features and clinical data analysis, when the 10 with central pontine myelinolysis of pontine lesions in patients with burns, have had any serious clinical, hypertonic persistent viremia without hyponatremia disease; no hypernatremia, hyperglycemia, or high nitrogen hyperlipidemia.
most remarkable feature of this disease is the pathological anatomical changes. Section can be seen in the brain stem level of the base of the central pontine gray, was finely granular lesions. Lesion size from several millimeters in diameter to occupy almost the entire base of the pons, but the surface of the pons between the lesion and normal myelin there is always a circle. Lesion from the back to the medial lemniscus can be very serious, can be extended to cover other structure of the Department. Rare cases the lesion extended to the brain, but never involved in the medulla oblongata. Severe lesions in the pons of cases. Sometimes in the thalamus, hypothalamus, striatum, internal capsule, cerebral cortex and deep white matter close to the pontine lesions found with similar symmetrical distribution of demyelination area, called the pontine myelinolysis outside (extrapontine myelinolysis) .
microscopic lesions characterized by the most basic of all affected areas of myelin destruction and relatively intact axons and nerve cell nuclear bridge. Always start from the pontine lesion center and the site is the most serious disease here, and sometimes develop into obvious tissue necrosis. demyelination seen in the reaction zone phagocytic cells and glial cells, but no oligodendrocytes glial cells, notably the inflammatory response there is no other lesions.
current consensus is that the disease: some cerebral regions, particularly the base of the pons, the special susceptibility of certain metabolic disorders causing central pontine demyelinating disease . This metabolic disorder can be a rapid or excessive correction of hyponatremia, it could be a serious hyperosmolar hyperlipidemia.[Sign]
1. The disease is disseminated, can occur at any age, children's cases are not uncommon. The remarkable feature of this disease in patients with chronic alcoholism or late stage, often accompanied by severe or life-threatening diseases.
majority of cases occur in chronic alcoholism (alcoholism) the late, accompanied by wernicke encephalopathy and multiple peripheral neuritis. Other often associated with central pontine myelinolysis accompanied by disease or clinical symptoms are: The dialysis chronic renal failure; liver failure; advanced lymphoma and cancer; various causes of cachexia; serious bacterial infection; dehydration and electrolyte imbalance; severe burns and hemorrhagic pancreatitis.
2. only a few patients, central pontine myelinolysis was diagnosed in his lifetime. Patients on the basis of the primary disease often sudden limbs flaccid paralysis, chewing, swallowing and speech disorders, nystagmus, and eye gaze disorders, was silent and can be complete or incomplete locked-in syndrome.
3. brainstem auditory evoked potential (BAEP) to help determine the pontine lesion, but can not determine the lesion extent. mri can be found in the pontine base lesions characteristic bat-like wings, is symmetric low signal T1, T2 high signal, no enhancement effect.
chronic alcoholism, severe systemic disease and rapid correction of hyponatremia in patients with clinically within a few days suddenly developed quadriplegia, pseudo-bulbar paralysis and locked-in syndrome should be considered central pontine myelinolysis diagnosis. mri can help confirm the diagnosis.[Aftertreat]
1. still support the current CPM and symptomatic treatment, active treatment of the primary disease. Should be slow to correct hyponatremia without hypertonic saline. Restricted fluid intake, the acute phase can be used mannitol, furosemide (furosemide) and the treatment of cerebral edema.
(b) the prognosis
central pontine myelinolysis a poor prognosis, high mortality rate, in a few days or weeks died, but many patients may actually cause the disease die from other serious systemic diseases. A few survivors left spastic quadriplegia and other serious neurological disorders, sometimes a full recovery of patients.
1. peripheral leukocytosis, ESR.
1. imaging, especially MRI, have greatly increased central pontine myelinolysis a lifetime diagnosis. mri examination revealed a pontine base lesions typical bat-shaped wings with a diagnosis, but this change occurred only in a few days before the clinical symptoms of the formation.
The disease should pontine base infarction, cancer and multiple sclerosis and other identification. CPM mri showed no significant mass effect, symmetrical lesions, vascular distribution does not meet, with the condition improved return to normal.[Disease]
more symptoms of lower extremity
"O"-shaped legs, "X"-type legs, "duck step" gait abnormal Q-angle character footsteps patella fracture patella strain state of flat feet patella chondromalacia patella pain after patellar crepitus pain after patellar patellar patellar pain, patellar emptiness refers to tenderness weeks of restless legs syndrome can not be bent abnormal gait ataxia long or strenuous exercise
GMT+8, 2015-4-26 23:57 , Processed in 0.174827 second(s), 2 queries , Gzip On, Memcache On.