Funicular myelosis

Funicular myelosis (synonym: combined degeneration, combined sclerosis is a disease of the spinal corddeveloping owing to a prolonged deficiency of vitamin B12 and characterized by motor and sensory disorders. In most cases funicular myelosis combined with pernicious anemia, rarely with other anaemia, leukaemia, ahilia, stomach cancer; sometimes develops after resection of the stomach or invasion wide lentetsom. In funikulyarnom mieloze often affects simultaneously posterior and lateral columns of the spinal cord.
The disease is usually at the age of 30-50 years. The patient complained of General weakness, headache, dizziness, noise in the ears, loss of memory, drowsiness during the day and insomnia at night, fatigue, irritability, decreased appetite, bitter taste in the mouth, tingling in the language, sometimes with the perversion of taste and dry mouth. These phenomena joins legs are weak and unsteady gait. Muscle tone, knee and Achilles reflexes increased, there are pathological reflexes; in the later stages reveals the extinction of tendon reflexes, reduced muscle tone. Characterized by disorders of the muscle and joints and vibration sensitivity (see). All symptoms gradually increase and lead to immobility of the patient. Marked paresthesias - numb and cold in the feet, feeling of crawling, stinging and tingling, characteristic for the defeat of the rear pillars. In the blood indicates the presence of a young forms of erythrocytes, hyperchromic anemia. In a sharply-cut cases funikulyarnogo mieloza when the nervous system is the leading and may even precede the change of blood, the progression of neurological symptoms go relatively quickly and in 2-3 months. can cause paralysis of the limbs, disorders of the functions of pelvic organs, bedsores.
The forecast is determined by the timeliness and effectiveness of the therapy.
Treatment. Certain therapeutic effect achieved with intramuscular injections of vitamin B12. The first two injections a day to 100 - 200 mcg, and then twice a week before the appearance of clinical signs of improvement. Prolonged treatment, for several months. It is recommended to massage, a convalescent-phase - therapeutic exercise.

Funicular myelosis (myelosis funicularis; synonym: combined sclerosis, acute degeneration of the spinal cord) is a combined degeneration of the spinal cord.
In 80-95% of cases funicular myelosis combined with pernicious anemia. In a few cases described the combination funikulyarnogo mieloza with other anaemia, leukaemia, cancer of the stomach. Sometimes funicular myelosis develops after gastrectomy, when invasion wide lentetsom, pellagra. Sometimes observed among members of the same family.
Pathological anatomy. The disease is characterized by the collapse of the axial cylinders and myelin sheaths fibers in the spinal cord with the formation of voids due to insufficient jet expansion glia.
At the beginning of the disease in the posterior and lateral columns of the spinal cord appear small foci of collapse. Gradually the destruction of the white matter with the formation of different sizes of voids is distributed in the spinal cord. Most often affects the thoracic and lumbar areas of the spinal cord, but degeneration can spread throughout clinico spinal cord, never, however, without going to the brain stem. In advanced cases the primary foci of the collapse of attached pockets secondary rebirth. Microscopic examination in the centers degeneration found the collapse of the myelin sheaths and axial cylinders.
Etiology. The relationship between the funicular myelosis and pernicious anemia (see) have clearly. However, the described cases funikulyarnogo mieloza and other listed above debilitating diseases. Pernicious anaemia is causing funikulyarnogo mieloza, but both of these diseases are caused by the same etiological factor.
Over and symptoms. The first signs funikulyarnogo mieloza - paraesthesia in the extremities as numbness, heat, tingling, crawling. Rarely, the disease starts with movement disorders: a weakness of the legs, infringements of gait. In typical cases funikulyarnogo mieloza see different symptoms lesions of the posterior and lateral columns of the spinal cord. The defeat of the rear pillars causes disorder deep sensitivity, ataxia, the disappearance of the tendon reflexes. The defeat of the side columns causes the weakness of the limbs, increase tendon reflexes, pathological pyramid reflexes, increased muscle tone. Further, patients no longer move independently, and spastic paresis of the legs is often replaced sluggish. Disorders of sensitivity become more intense and capture the surface of sensation. When flaccid paralysis of the absence of the tendon reflexes long will pathological pyramid reflexes. This unique combination is pyramidal symptoms and flaccid paresis characteristic funikulyarnogo mieloza. Are mainly affected the lower limbs; upper later and to a much lesser degree. Constipation, an imperative need to pass urine observed with the defeat of the side columns, and stretch the bladder and the presence of residual urine indicate the defeat of the rear pillars. Along with patients who have marked the initial symptoms as paresthesia and lung disorders deep sensitivity, there are patients who have within 2-3 weeks is established pattern of transverse myelitis. Often there are options that have symptoms only the back or only the side pillars. Rarely observed syndrome polinevrita as disorders of sensitivity of peripheral type, atrophy of muscles of the limbs.
Sometimes there are mental disorders in the form of irritability, depression. Patients apathetic, silent, quickly tired during mental stress. Less common are expressed psychotic state in the form of agitation, paranoid, delusional syndrome.
In all cases funikulyarnogo mieloza found ahilia stomach (see).
The diagnosis is not difficult in cases where a patient with pernicious anemia showing signs of nervous system. It is sometimes difficult to differentiate funicular myelosis from multiple sclerosis, tumors of the spinal cord, spinal suchocki. Unlike multiple sclerosis, in funikulyarnom mieloze not observed temporal lobe atrophy half the optic nerves, staccato speech intentsionnogo shake. Against multiple sclerosis and in favor funikulyarnogo mieloza says the disease in old age, permanent ahilia. For tumours of a spinal cord disorders are not limited to the rear and side pillars, in the spinal fluid found protein cellular dissociation. When tabes dorsalis affects only the rear pillars; identify changes pupils, cerebrospinal fluid, there are painful crises, frequent positive serological reactions in the blood and cerebrospinal fluid.
Treatment with vitamin B12 is being recognized more effective than the previously used drugs in the liver. Especially good effect is achieved if treatment is started no later than three months after the onset of symptoms funikulyarnogo mieloza. Therapy with vitamin B12 should be used for a long time.
First daily for 2 weeks intramuscularly 100-200 mcg of vitamin B12, then 50-100 mcg 2 times a week for 6 months, further 50 mg 1 time in 2 weeks. The dose must be individualized and at relapse increased. Also nominated injections of vitamin B1, hydrochloric acid inside. It is recommended to massage in the recovery phase - therapeutic exercise.