Funicular myelosis

Causes

The main cause of funicular myelosis is considered to be hypovitaminosis B12, less commonly a combined deficiency of cyanocobalamin and folic acid or vitamin B9 alone. The mechanism of the onset of the disease has not yet been established.

In most cases, funicular myelosis is accompanied by pernicious anemia, but this is only a secondary cause of spinal cord damage. Another culprit of combined degeneration is considered to be the Castle factor. Thanks to this gastromucoprotein, cyanocobalamin is transformed into an accessible form. With a deficiency of its synthesis, the full absorption of vitamin B12 is impaired.

The following pathologies can provoke the appearance of funicular myelosis:

Achilles gastritis;
malignant tumors of the stomach;
tuberculous enteritis;
ahilia;
pellagra;
Crohn's disease;
intestinal diverticulosis;
gastrectomy or short bowel resection;
dibotriocephalosis.

Very often, people who adhere to strict vegetarianism or are constantly on diets suffer from funicular degeneration.

Causes of the disease

The main reasons for the development of funicular myelosis are a decrease in the absorption of vitamin B 12, less often the cause is a sharp decrease in the level of vitamin consumed in food (applies only to vegetarians). There are several factors that can lead to malabsorption of cyanocobalamin: dysfunction of the thyroid gland and adrenal glands, especially due to autoimmune damage, the presence of worms that compete for the absorption of the vitamin (especially tapeworms), the presence of blind loop syndrome - when vitamin B 12 is used active bacteria. Also, reasons leading to a decrease in the absorption of vitamin B 12 include gastric resection, atrophic gastritis, hypoplasia or absence of parts of the small intestine (congenital pathology), inflammatory processes of the small intestine, and surgical interventions on the small intestine.

Much less frequently, the absorption of vitamin B 12 is impaired due to the presence of malabsorption syndrome, with chronic pancreatitis, after the use of a number of medications (biguanides, calcium salt chelating agents, etc.). Sometimes funicular myelosis is provoked by increased consumption of vitamin B 12 during current hyperthyroidism and subsequent disruption of its absorption.

It is quite difficult to explain the neurological manifestations of myelosis pathogenetically; the mechanism of development of the disease is not completely clear. Apparently, we are talking about a violation of the composition of the myelin sheath of the nerve and, as a result, Reduced rate of excitation along the nerve.

It should also be taken into account that the reserves of vitamin B 12 in the body are quite large; it accumulates in the liver. In this case, daily expenses are equal to five thousandths of a milligram, and total reserves are about 4-5 milligrams, therefore, the onset of the disease usually occurs no earlier than 2.5-3 years after the development of the causes that led to a decrease in the absorption of cyanocobalamin.

Symptoms

In most cases, the disease begins with flaccid paresis of the lower, less often upper, extremities and severe weakness. Deterioration in motor function is accompanied by a feeling of numbness or burning, tingling, crawling sensations, and cold feet.

In the first stages of funicular myelosis, the cervical and thoracic segments of the spinal cord are affected, then the degeneration process descends to the lumbosacral region. The pathological condition is accompanied by neurological and movement disorders.

Symptoms of funicular myelosis:

unsteadiness of gait;
deterioration in coordination of movements;
pelvic disorders;
pallor;
tachycardia with shortness of breath;
bright crimson color of the tongue.

Neurological disorders are multiple in nature. Weakness, fatigue, depression, irritability, drowsiness, and sometimes acute psychosis appear. In some cases, funicular myelosis is accompanied by ophthalmological manifestations: optic neuropathy, blurred vision, and scotoma of the eye.

FUNICULAR MYELOSIS (combined sclerosis) - subacute combined degeneration of the spinal cord with damage to the posterior and lateral cords. The cause of the disease (is a deficiency of vitamin B12. It is observed with pernicious anemia and some other blood diseases, sometimes with vitamin deficiency, intoxication, hypokalemia of renal origin, portacaval anastomosis.

Symptoms, course . Gradually, paresthesia appears in the feet, followed by disturbances of deep sensitivity, sensory ataxia and weakness in the legs. Depending on the degree of disorder of the joint-muscular sense, paresis in the legs can be either spastic or flaccid. Tendon reflexes are initially increased, but fade as the disease progresses. Pathological foot reflexes are constantly detected (Babinsky and others). The combination of pyramidal symptoms with flaccid paresis is a characteristic feature of the disease. Sensory and motor disorders are accompanied by dysfunction of the pelvic organs. Often, funicular myelosis is combined with B12-deficiency polyneuropathy, when peripheral nerves are involved in the process. Typical forms are characterized by a combination of sensitive ataxia and paraplegia (atactic paraplegia). In some cases, apathy, drowsiness, emotional lability, and depression develop. Along with the usual, gradual development of the disease, sometimes there is an acute development of neurological disorders, in which within 2-3 weeks a picture of transverse damage to the spinal cord with lower paraplegia, paraanesthesia, and pelvic disorders appears. Currently, due to the widespread use of vitamin B12, at least half of cases of funicular myelosis are atypical.

The diagnosis of pernicious anemia is not difficult. First of all, it should be differentiated from B12-deficiency polyneuropathy, which is observed in approximately 40% of patients with pernicious anemia. The decisive differential diagnostic feature is damage to the pyramidal system, indicating funicular myelosis. The main diagnostic difficulties arise in cases where neurological symptoms develop before the onset of anemia. The diagnosis of funicular myelosis is confirmed by the detection of achilic gastritis and megaloblasts in the sternum punctate. The disease may resemble tabes dorsalis, but the latter almost necessarily requires Argyll Robertson syndrome and positive serological tests for syphilis in the blood and cerebrospinal fluid. With multiple sclerosis, as a rule, there is a multifocality of symptoms, as well as a remitting course of the disease.

Combined damage to the posterior and lateral cords is observed with compression lesions of the spinal cord (tumors, vertebrogenic cervical myelopathy). The diagnosis is made by detecting protein-cell dissociation with lumbar puncture and block with myelography. An important criterion for diagnosing funicular myelosis is the symmetry of neurological defects.

Treatment . Begin with intramuscular administration of 500-1000 mcg of vitamin B12 daily. After 5 injections, the dose is reduced to 100 mcg once a week for six months. Maintenance therapy - 100 mcg of vitamin B121 once a month. The administration of folic acid in a dose of 5-15 mg/day is permissible only for a very rarely detected form of funicular myelosis - folic deficiency, since otherwise folic acid causes an exacerbation of the disease.

Treatment started a few weeks after the onset of spinal symptoms usually leads to recovery; with delayed treatment, the process improves or stabilizes. The main regression of symptoms is observed in the first 3-6 months of treatment.

Diagnostics

The simultaneous manifestation of symptoms of pernicious anemia and neurological disorders significantly facilitates diagnosis. Difficulties arise when damage to the nervous system precedes the onset of symptoms of B12 deficiency anemia.

To confirm the diagnosis, the following activities are carried out:

MRI of the spine;
gastroscopy;
X-ray of the intestine;
study of gastric juice;
complete blood test with markers of pernicious anemia and hypovitaminosis B12.

In case of visual disturbances, the patient is referred for consultation to an ophthalmologist for ophthalmoscopy, perimetry and visometry.

Diagnosis of funicular myelosis

The list of mandatory methods includes endoscopy

The primary diagnosis is made already in the presence of neurological disorders and anemia. To confirm the disease, a special examination is carried out, which includes:

blood test for the presence of vitamin B12;
studies to determine the secretory function of the stomach;
analysis for the presence of antibodies to intrinsic Castle factor;
general examination of the intestines;
magnetic resonance imaging of the spine;
lumbar puncture;
sternal puncture;
ophthalmological examination - carried out if the patient has disorders in the functioning of the visual system.

Also, in some cases, severe nervous disorders may require consultation with a psychiatrist.

Treatment

Treatment of funicular myelosis is based on replenishing vitamin B12 deficiency. On the first day of compensatory therapy, 500–1000 mcg of cyanocobalamin is administered intramuscularly daily. After 2 days, the drug is started to be injected every other day, then according to indications until signs of improvement in health appear. The maintenance dosage is 100–200 mcg of cyanocobalamin once a month.

After compensation for the lack of vitamin B12, treatment with folic acid (10-15 mg/day) begins. Therapy is carried out only if a folate deficiency lesion is detected. Otherwise, vitamin B9 can cause severe aggravation.

In parallel with the prescription of cyanocobalamin, the patient is prescribed injections of pyridoxine and thiamine. In addition to injections, massage, a course of therapeutic exercises, and physical therapy are performed.

It must be remembered that funicular myelosis responds well to treatment only in the first weeks after signs of spinal cord damage are detected. It is much more difficult to cure the disease at a later date. You can only stabilize the pathological process.

Treatment of the disorder

Therapy for the disease must be general and consist of conservative treatment to compensate for the lack of vitamin B12.

If a person experiences folate deficiency, he is required to drink folic acid up to 6 times a day. If you take this remedy for another illness, it often causes some complications.

Funicular myelosis is characterized by increased muscle tone. To combat this phenomenon, a professional set of physical exercises, relaxation in a sanatorium, massage and manual therapy has been developed. The following medications should be taken:

Mydocalm;
Baclofen;
Sibazon;
Nootropics.

To quickly treat myelitis, it is very important to take vitamins and adhere to a special diet. A person needs to be prescribed vitamins B1 and B6 and the drug Phosphaden 3 times a day for 1.5 months. You should eat a lot of sea fish, meat, soy, beans, cereals, nuts, pistachios and offal.

If you start therapy a few weeks after the onset of the disease, this guarantees the patient’s complete recovery. If treatment is started later, the condition may temporarily improve and stabilize. Symptoms should go away after six months of drug treatment.

Complications

With chronic cyanocobalamin deficiency, the development of various complications is observed:

encephalopathy;
deterioration of memory and attention;
aggression;
auditory and visual hallucinations;
speech disorder;
urinary incontinence;
impotence;
Optic neuritis.

If funicular myelosis is combined with peripheral neuropathy, vibration sensitivity is impaired, persistent numbness of the feet and loss of the Achilles reflex are noted.