Myelopathy chronic

Myelopathy broadly covers all diseases of the spinal cord.

The main symptoms of myelopathy are the following. Back pain in chronic myelopathy (as opposed to acute myelopathy) is rare and can accompany, for example, spondylosis or syringomyelia. Sensory disorders are more common and may reflect the involvement of the posterior roots, horns, posterior columns and spinotalamic pathways in the lateral columns of the spinal cord. Motor manifestations, as a rule, are leading and slowly progressing. Spastic monoparesis, paraparesis (more often asymmetric) can be observed, for example, with multiple sclerosis, cervical spondylosis, disc herniation, myelopathy in AIDS, funicular myelosis, BAS, radiation myelopathy, spinal forms of spinal-cerebellar degeneration. Progressing myelopathy involving the cells of the anterior horns (ALS, syringomyelia, intraspinal tumor) will be manifested by flaccid paresis with muscular atrophy, fasciculations and hypo- and areflexia at the level of the affected segments. Tendon reflexes in chronic myelopathies (in contrast to acute myelopathies) tend to increase upward, Babinsky's symptom is often observed, as well as imperative urges to urinate and constipation.

At the same time, there are diseases in which there is no spinal lesion, but the clinical manifestations are similar to it and can serve as a source of diagnostic errors. So bilateral involvement of the upper medial parts of the frontal lobe (eg, sagital meningioma) causes spastic paraparesis and apraxia of walking. Thus, lower paraplegia (paraparesis) still does not say anything about the level of the lesion: it can be the result of lesions at many levels, beginning with the parasagital tumor and ending with the lower-thoracic spinal cord. In frontal processes, the search for at least mild dementia, paratonia or a grasping reflex is important.

Normotensive hydrocephalus with characteristic gait disturbances (apraxia walking) and urinary incontinence may resemble myelopathy; but there are no paresis, no spasticity, no disturbances of sensitivity; at the same time, dementia is one of the leading manifestations.

Psychogenic paraplegia (pseudoparaplegia, pseudoparaparez) can proceed chronically, but usually develops sharply in the emotiogenic situation, accompanied by multiple motor disorders (seizures, pseudo-ataxia pseudo-hypocrisy, mutism), sensitive and emotional-personal features with preserved functions of the bladder and intestines in the absence of objective (paraclinical ) confirmation of involvement of the spinal cord.

[1], [2], [3], [4], [5], [6], [7], [8],

The main causes of chronic myelopathy:

1. Multiple sclerosis.
2. Cervical spondylosis, protrusion of the disc.
3. Other diseases of the spine and spinal cord (chronic ischemia, vascular malformation).
4. Subacute combined degeneration of the spinal cord (funicular myelosis).
5. Hereditary spastic paraplegia of the Strympel.
6. Syringomyelia.
7. Poliomyelitis (consequences).
8. Syphilis.
9. Other infections of the spinal cord (including vacuolar myelopathy in AIDS, Lyme disease).
10. Cirrhosis of the liver and port-cavaltic shunt.
11. Myelopathy of unknown etiology (up to 25% of all cases of chronic myelopathy).

Multiple sclerosis

Multiple sclerosis rarely (10-15%) has a primary-progressive form without typical remissions and exacerbations. In such cases it is important to use diagnostic criteria (presumably Poser's criteria are best) suggesting that at least two lesions must be present in a patient aged 10 to 59 years (or one clinical and one paraclinically identified outbreak) and two exacerbations ("reliable" multiple sclerosis). Two exacerbations should affect different areas of the central nervous system, last at least 24 hours and their appearance should be separated by an interval of not less than one month. True, with the primary-progressive form of multiple sclerosis, repeated exacerbations are absent, which creates real diagnostic difficulties. Of course, an active inquiry is required for a history of paresthesia or visual disturbances. MRI and evoked potentials (mainly visual and somatosensory), especially when both of these methods indicate damage to the respective conductors) confirm (or exclude) multiple sclerosis. Another reliable but more difficult method for diagnosing this disease is the detection of oligoclonal IgG groups in the cerebrospinal fluid.

Diagnostic criteria for multiple sclerosis:

I. Schumacher's criteria suggest that there should be a "dissemination in place and time" in the age range from 10 to 50 years:

According to a neurological examination or anamnesis (if examined by a competent neurologist), signs of at least two separate foci should be identified.

There must be at least two episodes of functionally significant symptoms lasting more than 24 hours, separated by a period of at least one month. Remission is not a mandatory requirement. The existing neurological disorders can not be adequately explained by another pathological process.

Schumacher's criteria (1965) still refer to the "gold standard" for the diagnosis of multiple sclerosis.

II. McAlpin's criteria ( McAlpin , 1972) suggest the allocation of reliable, probable and possible multiple sclerosis:

Reliable multiple sclerosis: in anamnesis there should be indications of retrobulbar neuritis, double vision, paresthesia, weakness in the extremities, which eventually decreased or disappeared altogether; presence of one or more exacerbations. On examination, signs of pyramidal tract lesions and other symptoms that indicate the presence of several foci in the central nervous system (gradual development of paraparesis with periods of deterioration and signs of lesion of the brainstem, cerebellum or optic nerve) should be revealed.

Probable multiple sclerosis: anamnesis data on two or more retrobulbar neuritis in combination with symptoms of pyramidal tract involvement. During this exacerbation there should be signs of multi-focal CNS lesion with good recovery. With prolonged observation, nystagmus, tremor, blanching of the temporal halves of the optic nerve disk are attached to the symptoms of the defeat of the pyramidal tract. There may not be clear exacerbations.

Possible multiple sclerosis: progressive paraparesis at a young age without signs of exacerbation and remission. With the exclusion of other causes of progressive paraparesis.

The McDonald and Halliday criteria (1977) and the Bauer criteria (1980) are also known , which are now used less often and we do not give them here.

The most widely used criteria in Europe, North America and Russia are Poser. They are designed for practical neurologists and include, in addition to clinical data, the results of additional research methods (MRI, brain potentials, detection of oligoclonal antibodies in CSF). Poseur's criteria have only two categories: "reliable" and "probable" multiple sclerosis. We have already mentioned them above.

Differential diagnosis of multiple sclerosis includes such diseases as autoimmune inflammatory diseases (granulomatous angiitis, systemic lupus erythematosus, Sjogren's disease, Behcet's disease, nodular periarteritis, paraneoplastic syndromes, acute disseminated encephalomyelitis, post-infection encephalomyelitis); infectious diseases (borreliosis, HIV infection, neurosyphilis); sarcoidosis; metachromatic leukodystrophy (juvenile and adult types); spino-cerebellar degeneration; Arnold-Chiari malformation; insufficiency of vitamin B12.

Cervical spondylosis

Cervical spondylosis (a combination of degenerative changes in intervertebral discs, facet joints and yellow ligament) is the most common cause of myelopathy in patients of mature and advanced age (in the Russian-language literature the term "osteochondrosis" is used synonymously). Cervical myelopathy develops in about 5-10% of patients with clinically manifested spondylosis. It is easier and faster to develop in the presence of congenital narrowing (stenosis) of the spinal canal (12 mm or less) and is caused by external compression of the spinal cord and its vessels (mainly lateral and posterior columns). The degenerative process usually begins in the disk with secondary changes in the adjacent bone and soft tissues. The spinal cord is compressed by a hernia of the intervertebral disc, by protrusion (hypertrophy) of the yellow bundle into the canal or by osteophytes. Pain in the neck is usually the first symptom; further there are numbness in the hands and slight disabilities of the gait, which gradually increase; possible (infrequently) gross violations of the bladder.

There are several clinical variants of cervical myelopathy:

1. Spinal cord involvement involving corticospinal (pyramidal), spinal-thalamic tracts and conductors in the posterior columns of the spinal cord (tetraparesis with weakness mainly in the legs, spasticity, sensory ataxia, sphincter disturbances and Lermuth's symptom).
2. Primary involvement of the cells of the anterior cornea corticospinal tract (syndrome of ALS without sensory disorders).
3. Syndrome of pronounced motor and sensory disorders with weakness in the arms and spasticity in the legs.
4. Syndrome Brown-Sekar (typical contralateral sensory deficiency and ipsilateral - motor).
5. Atrophy, prolapse of reflexes (defeat of spinal cord motoneurons) and radicular pain in the hands. Weakness predominantly in the V and IV fingers.

Hyperreflexia is detected in about 90% of cases; a symptom of Babinsky - in 50%; The symptom of Hoffmann (on the hands) is about 20%.

Other diseases of the spine and spinal cord

Chronic myelopathy can develop in other diseases of the spine (rheumatoid arthritis, ankylosing spondylitis) and vascular diseases of the spinal cord. Slowly progressive paraparesis (with or without sensory disturbances) in a mature or elderly patient with a vascular disease (arteriosclerosis, arterial hypertension, vasculitis) may be associated with chronic spinal circulatory failure; but first you need to exclude other possible causes of myelopathy: tumors, degenerative motor neuron diseases, funicular myelosis (subacute combined degeneration of the spinal cord), cervical spondylosis and, sometimes, multiple sclerosis. Vascular malformation is sometimes manifested in a picture of chronic myelopathy.

Subacute combined degeneration of the spinal cord

Funicular myelosis develops in the absence of vitamin B12 or folic acid, which leads to the defeat of the lateral and posterior columns of the spinal cord at the cervical and upper-thoracic level of the spinal cord. Causes: Achilles gastritis, gastrectomy, intestinal surgery, AIDS, strict vegetarian diet, introduction of nitric oxide. The disease begins gradually with paresthesia in the hands and feet, weakness, walking disorders. Sensitive ataxia, spastic paraparesis is revealed. Possible reduction of visual acuity, symptoms of involvement of the brainstem and the cerebellum. The diagnosis is confirmed by the study of the level of vitamin B12 in the serum and the positive Shilling test (it can be abnormal even at a normal B12 level in the serum). Homocysteine and methylmalonic acid (precursors of vitamin B12) are increased in 90% of patients with vitamin B12 deficiency. Typical symptoms of anemia.

Folate deficiency leads to a similar syndrome and develops with malabsorption, alcoholism, in elderly people, intestinal diseases, Crohn's disease, ulcerative colitis and in patients receiving anticonvulsants. A certain risk of developing a deficiency of folic acid is in pregnant women.

Hereditary spastic paraplegia of the Strumpel

Spastic paraplegia Strumpell refers to diseases of the upper motoneuron and begins in childhood or early adulthood with complaints of stiffness in the muscles of the legs and instability in walking, which is based on progressive paraplegia with high tendon reflexes and pathological stop signs. Characteristic increase in the tone in the leading muscles of the thigh, which leads to a characteristic dysbasia with half-bent legs and a rigid "crossing" step. Less "pure" forms have a variety of additional neurological syndromes (dementia, optical atrophy, retinal degeneration, parkinsonism, dystonia, epilepsy, muscular atrophy, heart disease). Family history and typical clinical manifestations are the basis of diagnosis.

Syringomyelia

Syringomyelia usually manifests as a cavity in the central gray matter, but the latter can spread to the region of the anterior or posterior horns. The most frequent localization is the cervical or upper thoracic part of the spinal cord (less often can be observed in the lumbar region and in the trunk region). In adults, Arnold-Chiari type I malformation is often found; in children, a coarser malformation. Postural syringomyelia is detected in 1-3% of patients with severe spinal cord trauma. Tumors of the spinal cord and inflammatory processes can also lead to the formation of syringomyelic cavities. Pain, weakness and muscle atrophy more often than one hand, scoliosis and dissociated sensitivity disorders (pain and temperature reduction with tactile and deep sensitivity preservation) refer to cardinal manifestations of syringomyelia. With a large cavity, the posterior and lateral columns are involved (sensitized ataxia in the legs and lower spastic paraparesis, violation of pelvic functions), as well as vegetative conductors (Horner's syndrome, orthostatic hypotension). Syringobulbia is manifested by such typical symptoms as one-sided atrophy of the tongue, trigeminal pains or hypesisesia in the zones of the Selder, paralysis of the muscles of the soft palate and larynx, dizziness and nystagmus. MRI helps in diagnosis.

Polio

Poliomyelitis is a viral disease that begins acutely after an incubation period of 2-10 days in the form of general infectious symptoms. After 2-5 days, asymmetric progressing flaccid paralysis develops, often affecting the proximal parts of the lower extremities. After about a week, atrophy begins to appear in the paralyzed muscles. In 10-15% of patients, the muscles of the pharynx, larynx or facial muscles are involved. The diagnosis is confirmed by sowing the poliovirus from the smear (separated from the nasopharynx, feces) and rarely from the CSF or blood. It is also useful to take into account the epidemiological situation.

In 10-70 years after acute poliomyelitis, in 20-60% of patients new symptoms may appear in the form of fatigue and increase in weakness in those muscles that were previously affected by poliomyelitis; but there may be weakness and atrophy in the muscles that are not affected in the acute period - the so-called post-polyposis progressive muscular atrophy. Its cause is unclear.

Syphilis

Syphilis with spinal cord injury (myelopathy) can be manifested by a pattern of meningovasculitis (meningomyelitis), hypertrophic spinal pachymeningitis (more often at the cervical level) and spinal cord gum; all of them are quite rare. The late form of the neurosyphilis on the spinal level is the dorsal (tabes dorsalis). It represents progressive degeneration, affecting primarily the posterior columns and posterior roots of the spinal cord. Usually it develops late, 15-20 years after infection, progresses slowly, more often affects men than women. There are shooting pains, more often in the legs, lasting from several minutes to several hours, sometimes they are grouped into "bundles". 20% of patients report periodic abdominal pain (tabetic crises). Later, a sensitive ataxia develops with a characteristic "stamping" (tabetic) gait, areflexia. Typical repetitive injuries due to disruption of walking with the formation of a typical "Sharco joint" in the area of the knee joint. There is a symptom of Argyle-Robertson; the atrophy of the optic nerve is possible, more rarely other symptoms.

Other infections

Among the other infections, the most relevant is HIV infection, which can also lead to myelopathy. Vacuolar myelopathy is observed in approximately 20% of AIDS patients and is characterized by damage to the posterior and lateral columns of the spinal cord, mainly at the cervical level. Clinical manifestations develop slowly and vary from mild lower paraparesis with sensitive ataxia to paraplegia with gross pelvic disorders. MRI detects hyperintensive signals in T2-weighted images in the area of corticospinal tracts and posterior columns of the spinal cord. Microscopically (autopsy) is a picture of vacuolar myelopathy.

Lyme disease (borreliosis) has three stages of flow. The first is characterized by characteristic erythema; the second - after months after the first it proceeds as a meningitis or meningoencephalitis. One third of patients are represented by a polyneuropathic syndrome, which is called Banwarth syndrome or Garin-Bujadoux syndrome. The third stage may appear months and even years after infection and is manifested by arthritis and symptoms of damage to the brain and spinal cord, cranial and peripheral nerves. Myelitis develops in about 50% of patients and manifests itself as progressive para- or tetraparesis with sensory disorders and impaired pelvic organs. Transverse myelitis develops on the thoracic and lumbar spinal cord. It remains unclear whether the third stage is caused by a direct damaging effect of the spirochete or is associated with parainfection-immune immune disorders. In cerebrospinal fluid, pleocytosis (200-300 cells and above), high protein content, normal or low sugar levels, increased synthesis of IgG. In blood and in liquor - the raised maintenance of antibodies. MRI reveals a focal or diffuse increase in signal intensity in the cervical spinal cord in some patients.

Cirrhosis of the liver, port-cavaltic shunt

Cirrhosis of the liver and port-cavaltic shunt can lead not only to encephalopathy, but also to myelopathy with a slowly progressing lower paraparesis. In some patients (rarely) this is the main neurological syndrome of liver failure. Hyperammonemia is characteristic.

Myelopathy of unknown etiology

Myelopathy of unknown etiology is common (up to 27% of all cases of chronic myelopathy), despite the use of modern diagnostic methods (MRI, myelography, study of cerebrospinal fluid, the use of evoked potentials and EMG). Her neurological portrait has been studied quite well. The most common symptom is paresis (or paralysis). They are observed in 74% of cases and are more often detected in the legs (72%) than in the hands (26%). In 71% of cases, these pareses are asymmetric. Hyperreflexia predominates (65%), more often asymmetric (68%); a symptom of Babinsky takes place in 63%. Muscle tone was increased by spasticity in 74%. Sensory disorders are present in 63% of cases; sphincter disorders - in 63%. Myelopathy of unknown etiology is the "diagnosis of an exception".

Diagnostic studies in patients with chronic myelopathy

General somatic examination (to exclude systemic diseases, neurofibromatosis, infection, malignant tumor, liver, stomach, aorta, etc.), neurological examination to exclude cerebral disease and to clarify the level of spinal lesion; CT or MRI to measure the width of the spinal canal, exclude intramedullary processes; myelography to exclude extramedullary compression of the spinal cord; evoked potentials for assessing afferentation from the peripheral nerves to the spinal cord and further to the brain; lumbar puncture (for the exclusion of infectious myelitis, carcinomatous meningitis or multiple sclerosis); EMG is also necessary (for example, to exclude multifocal motor neuropathy or (encephalo) myelopolyneuropathy).

[9], [10], [11], [12]