Funicular myelosis - causes, symptoms, diagnosis and treatment
Funicular myelosis - Spinal cord lesion that develops due to vitamin B12 deficiency. As a rule, combined with pernicious anemia. Disorder manifested deep sensitivity, movement disorders in the form of flaccid paralysis with lower pyramidal signs, mental health disabilities. Diagnosed funicular myelosis by determining the levels of B12 in the blood of the neurological, gastrointestinal and hematological examinations. Treatment consists in filling the deficit of vitamin B12 vitamin B12 compliance rich diet, the introduction of other B vitamins Weather favorable.
Funicular myelosis
pernicious anemia, it is almost always accompanied by a funicular myelosis. The first description of this comorbidity gave Lihtgeym in 1887
Funicular myelosis can develop at any age, but more often (about 90%) occurs after the age of 40. The cause of the disease may act as an exogenous vitamin deficiency B12 t. E. Insufficient intake of dietary cyanocobalamin and endogenous B12 vitamin deficiency caused by malabsorption at different gastrointestinal pathology. In addition, more than 50% of the funicular myelosis accompanied by the formation of antibodies to intrinsic factor Castle and the formation of atrophic gastritis, which indicates the existing immune disorders. In connection with the above, myelosis funicular is in the range of interests of several clinical disciplines: neurology, gastroenterology, immunology.
anorexia, complete exclusion from the diet of cyanocobalamin-rich meat, fish and dairy products, long starvation or malnutrition, leading to malnutrition. Endogenous B12 vitamin deficiency is associated with impaired absorption of cyanocobalamin. For the successful absorption of vitamin B12 is necessary transformation in his digestible form, which is carried out through the stomach sinteziruemomu biermerin - biermerin. Sintez latter is reduced significantly under ahilii, tumors of the stomach, gastric resection or gastrectomy, which leads to disruption of cyanocobalamin absorption. This is the most common cause of causing funicular myelosis.
Various bowel disease, accompanied by malabsorption syndrome can also be the cause of inadequate intake of B12. These include:. Crohn's disease, tuberculous enteritis, sprue, celiac disease, Whipple's disease, pellagra, post-resection syndrome short bowel and etc. Reduced admission cyanocobalamin in the body is due to its excessive absorption of intestinal flora, an excess of which is observed during the formation of stagnant "blind" bowel loops after forming enteroanastomoza when bowel diverticulosis and diphyllobothriasis.
Pathogenesis funicular myelosis determining pathological changes in the spinal cord is not completely installed. The main dysfunction believe methionine synthase and methylmalonyl-CoA mutase - enzymes involved in the processes of formation of myelin and the formation of the myelin sheath of nerve fibers. Morphologically funicular myelosis accompanied by demyelination of fibers extending in the rear and side of the spinal columns. And demyelination process is most pronounced in the rear pillars, where the way of deep sensitivity (paths and Burdach Gaulle). The side pillars demyelinating lesions affecting the pathways of motor activity (pyramidal, spinocerebellar, spinothalamic and others.). In some cases, there is demyelination of optic nerve fibers.
sensory ataxia, accompanied discoordination movements, instability, shaky gait. Then there is a weakness in the limbs, especially in the legs (lower paraparesis). Initially palsy is spastic character with increased tendon reflexes, muscle hypertonia, clonus feet. Then, reduced muscle tone, reflexes fade. Paresis acquires the features of the peripheral. It remains, however stopnye pathological reflexes, indicating lesion pyramidal pathway. With the patient loses the ability to walk over time. Join a pelvic disorders (faecal incontinence, urinary incontinence, impotence).
Mental disorders have a character of variability. Can depression, apathy, irritability, hypersomnia, sometimes - acute psychosis. In some cases, developing neuropathy of the optic nerve, manifests a progressive decrease in visual acuity with visual field loss of the central portion (central scotoma). Along with neurological and ophthalmologic manifestations, funicular myelosis accompanied by symptoms of pernicious anemia: pallor, crimson tongue, glossitis and /or stomatitis, tachycardia, shortness of breath, etc.
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B12 in the blood. Further diagnosis is carried out together with the neurologist and a gastroenterologist hematologist. To confirm the diagnosis and determine the cause of beriberi needed: CBC, the study of gastric secretory function (gastroscopy, gastric intubation, gastric analysis), analysis on antibodies to intrinsic factor Castle, bowel examination (plain radiography, X-ray barium passage).
Visualization spinal morphological changes (foci of demyelination) is possible with the help of MRI of the spine. It also allows you to differentiate from the funicular myelosis compressive myelopathy with spinal tumors, vertebral myelopathy, spinal tumors and cysts. Lumbar puncture and examination of cerebrospinal fluid to rule out infection and hemorrhachis myelitis. If you want to exclude neurosyphilis appointed RPR-test. With a view to the diagnosis of hematological disorders sternal puncture is performed followed by the study of myelogram. When visual disorder showed an ophthalmologist consultation with carrying out visometry, ophthalmoscopy and perimetry.
massage, physical therapy and exercise therapy.
As a rule, a funicular myelosis subacute or slow flow. Prior to establishing its causes disease for 2 years resulted in death. With timely treatment with vitamin B12 favorable prognosis. At the beginning of treatment after a few months of onset of the disease can be achieved stabilization, but we can not count on the complete regression of neurological symptoms.