Funicular myelosis

Funicular myelosis is a neurological disease that occurs with severe deficiency of vitamin B12 (cyalcobalamin). The cause of the emerging symptoms is a change in the structure of the spinal cord tissue with the development of gliosis, destruction of the myelin sheaths of nerve fibers in the pathways, spongiform degeneration of tissue mainly in the posterior and lateral columns.

According to ICD-10, funicular myelosis refers to a deficiency of other specified B vitamins (E 53.8).

Possible causes of the disease

The causes of the development of funicular myelosis are virtually all conditions leading to severe hypovitaminosis B12. Most often, this is due to insufficient production of Castle factor by the gastric glands, a substance necessary for the binding and absorption of vitamin B12 in the digestive tract. But a deficiency of cyanocobalamin can also be caused by disturbances in the absorption process in the intestines or a long-term lack of intake from food.

Among the main causes of hypovitaminosis B12 are :

hereditary deficiency of transport proteins;
severe gastritis with atrophic processes in the wall of the stomach and achylia;
autoimmune disorders;
massive helminthic infestations;
condition after operations to remove part of the stomach and intestines;
stomach cancer;
malabsorption syndrome with specific and nonspecific enteritis, Crohn's disease, celiac disease;
sprue;
pregnancy.

B12 deficiency does not always lead to the formation of funicular myelosis; pernicious anemia, polyneuropathy, and glossitis often occur. Damage to the spinal cord occurs with prolonged and severe deficiency of cyanocobalamin.

Funicular myelosis symptoms and treatment

According to ICD-10, funicular myelosis refers to a deficiency of other specified B vitamins (E 53.8).

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.

Which doctor treats funicular myelosis?

When the first symptoms of the disease appear, you should immediately consult a neurologist. You should not delay visiting a medical facility. If your local clinic does not have such a specialist, it is recommended that you see a therapist.

What happens in the spinal cord

Vitamin B12 is involved in the formation of myelin sheaths around nerve fibers, which helps increase the speed of impulses and insulate the nerve pathways in the spinal cord from each other.

With prolonged deficiency of cyanocobalamin, the myelin sheaths and axial cylinders are gradually destroyed, the white matter of the spinal cord becomes thinner and becomes heterogeneous. In its thickness, voids appear that are not filled with gliosis cells. Expanding foci of necrosis and degeneration appear. They are first found in the lower cervical and upper thoracic regions, and then spread up and down, involving adjacent areas of the spinal cord.

The process of destruction of white matter is especially pronounced in the posterior and lateral columns of the spinal cord. In this case, the sensitive pathways of the gentle and sphenoid bundles, as well as the motor pyramidal tract, are affected. With severe atrophy, the spinothalamic and spinocerebellar pathways are also involved.

Clinical picture

Neurological symptoms of funicular myelosis often occur against the background of other signs of chronic hypovitaminosis B12. Due to developing pernicious anemia, the patient is worried about weakness, fatigue, dizziness, absent-mindedness, collapsing and fainting states are possible. Characteristic changes appear on the tongue; its back becomes bright and shiny. When brain fibers are involved, encephalopathy develops, causing cognitive and mental disorders, aphasia, and urinary incontinence.

The clinical picture of funicular myelosis consists of a syndrome of motor and sensory disorders. The patient complains of unpleasant sensations in the extremities in the form of crawling, tingling, burning, and his tactile sensitivity is impaired. Due to a violation of deep sensitivity, a violation of coordination of movements and gait occurs - ataxia.

Weakness appears in the lower extremities with increased muscle tone, revitalized tendon reflexes and pathological pyramidal foot signs (lower central paraparesis).

But due to demyelination of the peripheral nerves, tendon reflexes soon fade away, and paresis can become sluggish. At the same time, pathological reflexes (for example, Babinsky) are preserved. With severe white matter atrophy, pelvic disorders may occur, including disorders of urination, erection and defecation.

Depending on the prevalence of symptoms, the posterior column form of the disease (with changes in deep and superficial sensitivity), the pyramidal form (with severe movement disorders) and a mixed version are distinguished.

Funicular myelosis: symptoms, treatment and diagnosis

Funicular myelosis is a neurological disease that occurs due to a lack of vitamin B12.
It affects the spinal cord and manifests itself in the form of sensory disturbances and paralysis of the legs. To identify the disease, a special analysis is performed, which shows the level of vitamin B12. Treatment must be used, as there can be serious consequences. You will also need to adhere to a certain diet.

If the correct therapy is prescribed, you can quickly achieve a full recovery.

Main reasons

The main cause of vitamin B12 deficiency is anorexia, when meat, fish and dairy products are excluded. With prolonged fasting or an unbalanced diet. If there is insufficient production of the substance for the absorption of vitamin B12, then funicular myelosis occurs.

There are the following reasons for the development of the disease:

constant drinking of alcoholic beverages;
taking medications that reduce the level of acidity in the stomach;
anemia;
surgery to remove part of the intestine;
viral intestinal diseases;
atrophic gastritis.

Funicular myelosis is most often observed in people after forty years of age. The spinal cord is affected when there is a long-term deficiency of vitamin B12. Vegetarianism leads to the fact that all the necessary elements for the full functioning of the body are not supplied.

Symptoms

Symptoms of funicular myelosis may not occur immediately. If the first signs appear, then in no case should you ignore them. Symptoms may disappear already during the first stage of treatment.

Signs of vitamin B12 deficiency:

rapid and constant fatigue;
discomfort and itching in the fingers;
nausea;
lack of appetite;
changes in the functioning of the gastrointestinal tract;
hair loss;
in women there is a disruption in the menstrual cycle;
redness in the form of small pimples on the tongue;
depression;
fever;
frequent colds.

If treatment is not started on time, serious problems can occur. You may begin to experience tinnitus and severe pain in the brain. Yellow skin color and darkening of palms. Memory loss and hallucinations may occur. Often troubled by insomnia and severe dizziness.

Therapy in this case may take about six months, and complications cannot be avoided. If severe anemia develops, there is a high chance of getting stomach cancer.

Diagnostics

If mental disorders are present along with anemia, the doctor suspects the development of funicular myelosis. To confirm an accurate diagnosis, it is necessary to undergo an examination.

First of all, a special blood test is taken, which shows the level of vitamin B12. Then the patient is sent to a neurologist or gastroenterologist.

A blood test is taken and a complete examination of the stomach and intestines is carried out.

Pathological changes are detected using magnetic resonance imaging. This diagnostic method will help to accurately identify funicular myelosis from malignant and benign tumors. A lumbar puncture is taken to exclude bacterial hematomyelia and myelitis.

An examination of the oral cavity is carried out, this makes it possible to understand changes in the tongue. If the vitamin B12 level is reduced, this indicates the presence of vitamin deficiency. If the doctor sees visual changes, then it is necessary to visit an ophthalmologist . At the first appearance, you need to visit a specialist as soon as possible. If you start the disease, complications can no longer be avoided.

Treatment

In order to eliminate the signs of funicular myelosis, drug therapy is prescribed. First of all, you need to restore the deficiency of vitamin B12. To do this, in the first days, the drug cyanocobalamin is administered every day. After a few days, an injection is given every other day until the patient feels better. After a month, the injection is given once every thirty days.

It is also necessary to use other drugs such as: Pyridoxine, Thiamine . It happens that funicular myelosis is manifested by a lack of folic acid.

This substance is used only after the main therapy, as the mental state may worsen. You must also follow a special diet to restore vitamin B12.

A doctor should prescribe a properly selected diet.

As a rule, with funicular myelosis, it is necessary to consume beef and veal liver. You can also add rabbit meat, eggs, fish, chicken, kefir, and cottage cheese to your diet. This will help quickly eliminate vitamin B12 . Not only drug therapy is prescribed, but also massage procedures and therapeutic exercises.

In order to achieve a speedy recovery, it is necessary to take medications and follow a special diet. If treatment is started within a few weeks after the development of funicular myelosis, recovery can be achieved quickly.

If the disease is advanced, the patient’s condition can be temporarily improved. Signs may disappear after at least six months of drug therapy. It is necessary to remember that the sooner treatment is started, the less consequences there will be.

Complications

If funicular myelosis is not treated promptly, a number of serious complications may occur. First of all, in order to prevent unpleasant consequences, it is necessary to consult a doctor at the first symptoms.

The following complications can develop with funicular myelosis:

encephalopathy;
memory and attention impairment;
aggressive behavior;
auditory and visual hallucinations;
slurred speech;
impotence;
urinary incontinence;
optic neuropathy.

If funicular myelosis is combined with peripheral neuropathy, then there may be sensory disturbances. Often the patient complains of constant numbness of the feet and headaches. If you want to cure funicular myelosis without any consequences, then at the first sign it is recommended to contact a medical facility.

Prevention

The first step is to prevent funicular myelosis, but for this you need to follow some rules. It is necessary to eat properly and include dairy and meat products in your diet. If you are pregnant, you need to take vitamin B12, but only as prescribed by your doctor. In order to avoid illness, you must first take care of your health.

Quit alcohol and tobacco products. If treatment is provided on time, there is a high chance of curing the disease completely. If the disease is advanced, then therapy will be long. It is important to adhere to proper nutrition and engage in moderate physical activity. If viral or infectious diseases appear, they must be treated promptly.

Walk more in the fresh air and ride a bike. Try to avoid hypothermia and stressful situations. Combine work and rest, because this makes it possible not to overexert yourself. If all recommendations are followed, it will be possible to prevent funicular myelosis.

Also read about Erb's myopathy, Bell's palsy and the causes of neuralgia in children.

Vitamin B12 deficiency has a number of serious complications, and to avoid them you need to contact a medical facility in time. Treatment is carried out using special drugs.

The doctor first conducts an examination and only then applies drug therapy.

Treatment of funicular myelosis can take up to six months, so the sooner you contact a specialist, the greater your chance of recovering faster.

Source:

Funicular myelosis: what it is, symptoms, treatment

With insufficient intake of vitamin B12 and folic acid into the body, a pathology such as funicular myelosis develops. Vitamin deficiency not only provokes the development of various diseases of the spine, but also paralysis of the legs.

In most cases, the pathology manifests itself as a combined type of sclerosis. Myelosis of the funicular type usually affects mature people over the age of forty, but in certain cases the disease is diagnosed at a young age.

Reasons for the development of myelosis

One of the main causes of vitamin deficiency is an incorrect, unbalanced diet, leading to anorexia. Patients with a deficiency of the above vitamin, as a rule, do not eat dishes that include meat, seafood and milk.

Vitamin deficiency is associated with impaired absorption of vital substances in the small intestine. For the body to function fully, cyanocobalamin (B12) must be converted into a form that can be absorbed by the intestines.

The process occurs under the influence of enzymes produced by the stomach (Castle factor). In case of cancerous tumors or gastric resection, its synthesis is disrupted and vitamin B12 is not absorbed.

This is the main reason for the development of funicular type myelosis.

In addition, B12 deficiency can result from:

celiac disease;
Crohn's disease;
gastroenteritis;
rehabilitation period after surgery;
pellagra.

Bacteria living in the gastrointestinal tract can absorb cyanocobalamin, hence the lack of it in the body.

To date, scientists have not fully determined the mechanism of funicular myelosis, as well as the etiology of the disease. The pathology provokes demyelination of the tissues of the spinal canal. In severe cases, damage occurs to the nerves responsible for vision.

The disease is very severe, and if immediate treatment is not started, it can lead to the death of the patient. Therefore, it is so important to diagnose the disease in a timely manner and begin treatment for the disease.

Manifestation of the disease

Clinical signs of the disease include sensory and motor disturbances. At a doctor’s appointment, the patient complains of unpleasant sensations in the legs in the form of burning, pinching, numbness, and “pins and needles.” Changes in tactile sensitivity gradually occur.

As sensitivity decreases, problems with movement coordination develop. The patient is diagnosed with ataxia.

Symptoms of funicular myelosis include the following disorders:

The main signs of the disease are muscle weakness and paresthesia. The latter represent a change in the sensitivity of the legs, with numbness gradually spreading to all parts of the lower extremities.
If timely treatment is not started, the patient’s coordination of movements changes, and the gait becomes “sloppy.” In later stages, paraparesis is diagnosed.
Tendon reflexes and muscle tone are enhanced. As the pathological process spreads, the pyramidal tract is affected, but involuntary movements of the feet remain. At the last stage of myelosis, the patient can no longer move independently.
Problems with potency, involuntary bowel movements and urinary incontinence develop.
Mental disorders of varying degrees of intensity appear: depression, sleep problems, psychosis.
In rare cases, damage to the optic nerve is diagnosed, which is manifested by deterioration of vision.
Nonspecific symptoms of the disease - breathing problems, gum disease, change in the shade of the tongue, etc.

Source: //cmiac.ru/nevralgiya/funikulyarnyj-mieloz-simptomy-lechenie-i-diagnostika.html

Diagnostic issues

When a patient is suspected of having funicular myelosis, the following examinations are performed:

examination by a neurologist (identifying signs of symmetrical damage to the posterior and lateral columns of the spinal cord);
CBC to detect hyperchromic pernicious anemia;
examination of the oral cavity, which allows us to identify characteristic changes in the tongue - glossitis with atrophy of the papillae (Gunter's or varnished tongue);
consultation with a therapist or gastroenterologist followed by an examination to identify pathologies of the gastrointestinal tract;
myelography;
EMG showing axonal degeneration
if necessary, sternal puncture as prescribed by a hematologist, revealing megaloblastic changes in the bone marrow;
spinal puncture with general analysis of cerebrospinal fluid, which does not change with funicular myelosis;
serological examination of blood and cerebrospinal fluid for syphilis;
MRI or CT of the spinal cord to exclude a space-occupying process, multiple sclerosis and other diseases.

The combination of signs of B12 deficiency, sensory disorders and signs of pyramidal insufficiency in movement disorders are key signs in the diagnosis of funicular myelosis.

Read about: Baker's cyst and treatment methods.

Treatment

The main direction in the treatment of funicular myelosis is the elimination of vitamin B12 deficiency. To do this, the body is saturated with cyanocobalamin, injecting it intramuscularly. Therapy consists of several stages:

the first 5 days are done daily at a daily dosage of 500–1000 mcg;
cyanocobalamin in a dose of 1 mg is administered once a week for several months (up to six months);
Maintenance treatment is carried out for life, for this purpose 500–1000 mcg of cyanocobalamin is administered once a month.

If a folic deficiency state is detected, an additional 5–15 mg of folic acid per day is prescribed; such a measure is rarely required. It is unacceptable to use this drug without indications, so as not to provoke the development of the fulminant form of funicular myelosis.

A course of treatment with vitamins B1 and B6 and phosphaden is required. In case of severe mental disorders, the use of psychotropic drugs may be required; they are prescribed by a psychiatrist. To reduce spasticity in paralyzed limbs, muscle relaxants (baclofen, mydocalm), as well as seduxen, are prescribed.

If possible, correct the disease that caused the impaired absorption of vitamin B12; this usually requires complex treatment from a gastroenterologist. Be sure to conduct an examination of the urinary system, treatment and prevention of urogenital infections.

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Funicular myelosis

- spinal cord damage that develops due to vitamin B12 deficiency. As a rule, it is combined with pernicious anemia.

It manifests itself as a disorder of deep sensitivity, motor disorders in the form of flaccid lower paralysis with pyramidal signs, and deviations in the mental sphere.

Funicular myelosis is diagnosed by determining the level of B12 in the blood, conducting neurological, hematological and gastroenterological examinations. Treatment consists of replenishing vitamin B12 deficiency, following a diet rich in vitamin B12, and introducing other B vitamins. The prognosis is favorable.

Funicular myelosis develops as a result of degenerative processes occurring in the posterior and lateral spinal cords against the background of vitamin B12 deficiency (cyanocobalamin deficiency).

Since B12 deficiency also leads to pernicious anemia, it almost always accompanies funicular myelosis.

The first description of this combined pathology was given by Lichtheim in 1887.

Funicular myelosis can develop at any age, but more often (about 90% of cases) occurs after 40 years of age. The cause of the disease can be both exogenous vitamin B12 deficiency, i.e., insufficient intake of cyanocobalamin from food, and endogenous B12 vitamin deficiency, caused by absorption disorders in various gastrointestinal pathologies.

In addition, in more than 50% of cases, funicular myelosis is accompanied by the formation of antibodies to the internal Castle factor and the formation of atrophic gastritis, which indicates existing immune disorders.

In connection with the above, funicular myelosis is in the range of interests of several clinical disciplines: neurology, gastroenterology, immunology.

Funicular myelosis

Exogenous vitamin B12 deficiency occurs with anorexia, complete exclusion of cyanocobalamin-rich meat, fish and dairy products from the diet, prolonged fasting or malnutrition, leading to nutritional dystrophy. Endogenous vitamin B12 deficiency is associated with impaired absorption of cyanocobalamin.

For successful absorption, the transformation of vitamin B12 into its digestible form is necessary, which is carried out thanks to the gastromucoprotein synthesized by the stomach - intrinsic Castle factor. The synthesis of the latter is significantly reduced by achylia, gastric tumors, gastric resection or gastrectomy, which leads to impaired absorption of cyanocobalamin.

This is the most common cause of funicular myelosis.

Various intestinal diseases accompanied by malabsorption syndrome can also cause insufficient absorption of B12. These include: Crohn's disease, tuberculous enteritis, sprue, celiac disease, Whipple's disease, pellagra, post-resection short bowel syndrome, etc.

A reduced intake of cyanocobalamin into the body is due to its excessive absorption by intestinal flora, the excess of which is observed during the formation of a congestive “blind” intestinal loop after the formation of enteroanastomosis, with intestinal diverticulosis and diphyllobothriasis.

The pathogenesis of the pathological changes in the spinal cord that determine funicular myelosis has not been fully established. The main one is the dysfunction of methionine synthase and methylmalonyl-CoA mutase - enzymes involved in the processes of myelin formation and the formation of the myelin sheath of nerve fibers.

Morphologically, funicular myelosis is accompanied by demyelination of fibers passing in the posterior and lateral spinal columns. Moreover, the process of demyelination is most pronounced in the posterior columns, where the paths of deep sensitivity pass (Gaull and Burdach paths).

In the lateral columns, foci of demyelination affect the pathways of motor activity (pyramidal, spinocerebellar, spinothalamic, etc.). In some cases, demyelination of the optic nerve fibers is observed.

The clinical picture consists of sensory-motor disorders and mental disorders. The disease debuts with the appearance of paresthesia and general weakness. Paresthesia in the form of tingling, transient numbness, crawling, and heat appear in the fingers of the limbs, then cover the shoulders, chest and abdomen.

Musculo-articular sensitivity is affected, then vibration sensitivity. As a result, sensitive ataxia develops, accompanied by incoordination of movements, instability, and unsteady gait. Then weakness occurs in the limbs, mainly in the legs (lower paraparesis).

Initially, the paresis is spastic in nature with increased tendon reflexes, muscle hypertension, and foot clonus. Then muscle tone decreases and reflexes fade. Paresis acquires peripheral features. However, pathological foot reflexes remain, indicating damage to the pyramidal tract.

Over time, the patient loses the ability to walk. Pelvic disorders are added (encopresis, urinary incontinence, impotence).

Mental disorders are variable in nature. Possible depression, apathy, irritability, hypersomnia, and sometimes acute psychosis.

In some cases, optic neuropathy develops, manifesting itself as a progressive decrease in visual acuity with loss of the central part of the visual field (central scotoma).

Along with neurological and ophthalmological manifestations, funicular myelosis is accompanied by symptoms of pernicious anemia: pallor, crimson tongue, glossitis and/or stomatitis, tachycardia, shortness of breath, etc.

The combination of neurological symptoms with manifestations of anemia immediately raises the suspicion of the existence of cyanocobalamin deficiency. In order to confirm this assumption, an analysis of the B12 content in the blood is performed. Further diagnostics are carried out by a neurologist together with a gastroenterologist and hematologist.

To confirm the diagnosis and establish the cause of vitamin deficiency, the following are necessary: a clinical blood test, a study of the secretory function of the stomach (gastroscopy, gastric intubation, gastric juice analysis), an analysis for antibodies to the internal Castle factor, an examination of the intestines (general radiography, barium passage radiography).

Visualization of spinal morphological changes (foci of demyelination) is possible using MRI of the spine. It also makes it possible to differentiate funicular myelosis from compression myelopathy in spinal tumors, vertebrogenic myelopathy, spinal tumors and cysts.

Lumbar puncture and cerebrospinal fluid examination are performed to exclude infectious myelitis and hematomyelia. If it is necessary to exclude neurosyphilis, an RPR test is prescribed. To diagnose hematological disorders, a sternal puncture is performed followed by a myelogram study.

In case of visual disorders, consultation with an ophthalmologist with visometry, perimetry and ophthalmoscopy is indicated.

Therapy consists mainly of correcting cyanocobalamin deficiency. At the beginning of treatment, cyanocobalamin preparations are administered intramuscularly daily. After 5-10 days, the drug is administered once a week, after 4 weeks - once a month.

If indicated, monthly vitamin B12 injections may be prescribed for life for life. A diet containing nutrients rich in B vitamins (different types of fish, beef, cottage cheese, cheese, chicken eggs, crabs) is recommended.

In parallel with treatment with vitamin B12, a course of administration of other drugs of this group is carried out: thiamine (B1) and pyridoxine (B6).

The presence of folic acid deficiency is an indication for its use. However, it is better to start treatment with folic acid after replenishing the cyanocobalamin deficiency, since otherwise it may aggravate the severity of neurological symptoms.

According to indications, correction of the secretory function of the stomach and malabsorption in the intestine, prevention of urogenital infection is carried out. For the fastest and most complete restoration of sensorimotor functions, massage, physiotherapy and exercise therapy are performed.

As a rule, funicular myelosis has a subacute or slow course. Before the cause of its occurrence was identified, the disease was fatal within 2 years.

With timely treatment with vitamin B12, the prognosis is favorable.

When starting treatment several months after the onset of the disease, it is possible to achieve stabilization of the condition, but one cannot count on complete regression of neurological symptoms.

Source: //www.KrasotaiMedicina.ru/diseases/zabolevanija_neurology/funicular-myelosis

Forecast

If specific therapy is started in the first few weeks after the first signs of funicular myelosis appear, complete restoration of the myelin sheaths and white matter of the spinal cord is possible. This will lead to healing and restoration of all functions.

With a late start of treatment or rapid development of severe myelosis, injections of vitamin B12 do not lead to complete relief of symptoms. During the first 3–6 months, the condition improves, after which a stabilization stage begins. If persistent paraparesis leads to severe impairment of motor functions, the issue of the patient’s ability to work can be resolved by determining the disability group.