Encephalomyelopathy

Encephalomyelopathy is a collective term that can mean etiologically different combined lesions of the brain and spinal cord. For example, the disease can be associated with tumor and degenerative processes, vascular compression, radiation exposure, metabolic disorders, trauma, etc.

The course is usually slowly progressive. Treatment depends on the underlying cause of the pathologic encephalomyelopathy syndrome. [1]

Epidemiology

Encephalomyelopathy as a result of pathology or trauma affects people of any age, regardless of gender and race. The severity of dysfunction depends on the extent of damage to the brain and spinal cord.

The disease due to primary tumor processes is more often registered in patients over 30 years of age. Posttraumatic encephalomyelopathy is more common among patients aged 16-35 years. There is an increased risk of developing this pathology in people with repeated traumas - for example, when regularly carrying heavy objects, gymnastics, etc.

Precise statistics on the incidence of encephalomyelopathy are not available. However, it is noted that men get the disease slightly more often (by about 30%).

Causes of the encephalomyelopathies

The appearance of encephalomyelopathy is often attributed to the following causes:

• Elevated blood pressure, associated with increased tone of the vascular wall, causes a significant narrowing of the vascular lumen. This negatively affects the blood supply to the brain and spinal cord.
• Atherosclerosis, accompanied by lipoprotein imbalance and impaired vascular circulation, leads to loss of the ability of vessels to respond adequately with necessary changes in wall tone.
• Hypertension in combination with atherosclerotic changes cause the appearance of microvascular damage and multiple cholesterol deposits on the vascular walls.
• Hypotension is accompanied by an inadequate supply of blood and nutrients to brain structures.
• Cardiac pathologies associated with improper conduction of the muscle impulse, failure of myocardial contractile function, and disorder of valve function lead to decreased cardiac output, deficit of systemic circulation, insufficient cardiac cavity contraction, formation of residual blood volumes within cavities, and wall thrombosis.
• Diabetes mellitus is accompanied by a prolonged increase in blood glucose levels and elevated glycosylated hemoglobin, which negatively affects the permeability and structure of capillary walls. As a consequence, the trophism of neurons and nerve fibers is disturbed, hypoxia and energy deficiency in cells develop. Oxygen-free metabolic reactions, which entail the accumulation of toxins in the blood, are compensatory involved. As a result, the functions of the spinal cord and brain are impaired.
• Autonomic vascular dystonia manifests itself in an imbalance of sympathetic and parasympathetic activity of the nervous system, which entails qualitative functional failures.
• Vascular injuries and compressions lead to mechanical cessation of blood flow in the vessel and disruption of blood supply to brain structures.
• Vasculitides cause infiltration, proliferation and edema of vessel walls, which slows down and worsens the course of metabolic processes.
• Toxic effects of exogenous and endogenous character are accompanied by accumulation of toxins in the blood, changes in the permeability of the blood-brain barrier, disorder of electrolyte and acid-base metabolic reactions.
• Developmental defects of the cardiovascular apparatus - in particular, anomalies and abnormal vessel connections - can provoke wall ruptures and hemorrhages into brain structures.
• Hematological pathologies are accompanied by a violation of the quality and composition of blood, lead to a violation of the transportation of nutrients to tissues and metabolic reactions.

Risk factors

The provoking factors for the development of encephalomyelopathy are as follows:

• high blood pressure;
• Vascular atherosclerosis (and especially its combination with hypertension);
• heart disease;
• endocrine, metabolic pathologies (diabetes mellitus);
• autonomic vascular dystonia;
• Vascular injuries, crush injuries, head injuries, spinal column injuries;
• tumor growths;
• vasculitis;
• exo- and endogenous toxic exposures;
• developmental defects of the cardiovascular apparatus;
• hematogenous pathologies.

Pathogenesis

Encephalomyelopathy refers to nonspecific syndromes and reflects a diffuse disorder of the spinal cord and brain. Both acute and chronic diseases can be complicated by encephalomyelopathy. Pathology often develops against the background of hypoxic conditions, and is also observed in patients with severe somatic diseases leading to the development of multi-organ failure.

The appearance of encephalomyelopathy may indicate that the pathologic reaction has gone beyond a single primary focus.

In some pathologies, lesions of the spinal cord and brain indicate a disorder of brain metabolic processes. Such a disorder is possible as a primary failure in brain structures (in hereditary, degenerative diseases), or secondary (with pathology of parenchymatous organs, endocrine system, water-electrolyte balance, as well as exogenous toxic processes).

As a rule, encephlomyelopathy has a chronic progressive course, which is characterized by the development of multiple foci or diffuse lesions of the brain and spinal cord and is manifested by a range of neuropsychiatric and neurological disorders.

Symptoms of the encephalomyelopathies

Physicians speak of these leading clinical aggregates characteristic of encephalomyelopathy:

• cognitive disorders, such as impairment of mainly short-term memory and poor concentration, intellectual impairment and mental retardation, difficulty remembering and planning, rapid thought fatigue and personal disorientation;
• motor disorders, which include pyramidal, extrapyramidal, cerebellar disorders, changes in statics and gait, monoparesis and hemiparesis, parkinson-like syndrome with predominance of mild rigidity and bradykinesia, seizure-like dizziness with nausea and occipital headaches;
• psychopathology with violation of the emotional-volitional sphere, which is manifested by emotional scarcity, reduction of vital interests, asthenia, depression;
• pseudobulbar disorders such as violent emotional displays, oral automatisms, dysarthria, dysphonia and dysphagia;
• pelvic pathologies (urinary disorders, including incontinence, followed by complete loss of control over pelvic organs).

The first signs of encephalomyelopathy may vary depending on the underlying cause of encephalomyelopathy, the severity of the pathology and its form (acute, chronic). If the causes are tumor processes, compression and trauma, the initial symptoms may be pain - more often with irradiation to the extremities, as well as disorders of sensitivity or motor skills, contractures. If the problem of encephalomyelopathy was provoked by osteoarthritis, then complaints of worsening mobility, weakness, spinal deformities, numbness may be voiced. With infectious processes, an increase in temperature, swelling, hypersensitivity is noted.

Perinatal encephalomyelopathy in a newborn infant

If pregnancy or childbirth was characterized by an unfavorable course, there was insufficient oxygen supply to the structures of the brain and spinal cord of the fetus, then there may be disorders at the metabolic and microcirculatory level. In small vessels that provide the exchange of oxygen and carbon dioxide, the corresponding processes are disturbed, and brain tissue suffers from a lack of vital oxygen.

The main causes of perinatal encephalomyelopathy:

• violation of fetal passage through the birth canal (narrow pelvis of the mother, large fetus, incorrect position of the fetus, etc.), birth trauma;
• rapid or prolonged labor, emergency cesarean section;
• asphyxia due to tight cord wrapping, aspiration of amniotic fluid;
• infectious processes (meningitis, meningoencephalitis).

In severe encephalomyelopathy in children, symptoms such as depression or excitation of the central nervous system, increased intracranial pressure, seizures, and coma may be noted. However, most children with moderate to severe course of the pathology show symptoms only after some time (months and even years).

Taking into account the individual characteristics of development, minor disorders can be managed independently. However, if the lag in psychomotor development of a premature baby for 2 or more months requires consultation with a pediatric neurologist. Only a specialist can determine whether the child needs treatment and prescribe comprehensive rehabilitation.

Stages

In the clinical course of encephalomyelopathy it is customary to distinguish three stages:

1. Subjective symptoms are noted in the form of pain and heaviness in the head and body, severe fatigue, impaired memory and concentration, sleep and gait disorders. Examination reveals a poorly expressed neurologic picture that does not correspond to any specific neurologic syndrome. The patient can independently serve himself, the ability to work is present.
2. Basic signs become more pronounced, pyramidal, vestibulocerebellar, pseudobulbar, pyramidal, amyostatic, cognitive, personality-emotional disorders are detected. There may be increased frequency of urination at night. Work capacity is slightly reduced, self-care is preserved, but in some cases outside help may be required.
3. There is a combination of neurological symptoms, pelvic organ dysfunction (urinary incontinence), periodically recorded paroxysmal states (falls, epi-seizures, syncope, etc.), intellectual and mental disorders. Self-care is sharply impaired, assistance is required in almost everything.

Forms

According to the etiologic factor, the following types of encephalomyelopathy are distinguished:

• Post-traumatic encephalomyelopathy develops as a distant consequence of a brain injury. The problem can appear several months or years after the injury. The first signs of the disorder consist in behavioral disorders, depressive states, changes in thought processes. Pathology is prone to progression, over time can provoke the development of dementia.
• Intoxication variant of encephalomyelopathy is found mainly in persons who abuse alcohol, have drug addiction, prolonged contact with chemical compounds and heavy metals, natural toxic substances and toluene. The basic signs are memory impairment up to its loss, visual impairment and mental status.
• Dysmetabolic encephalomyelopathy develops due to chronic pathological processes - for example, hepatic cirrhosis, diabetes mellitus, uremia, pancreatitis, hypoxia and other diseases that negatively affect the body's metabolism.
• Dyscirculatory encephalomyelopathy, in turn, is subdivided into atherosclerotic, hypertensive, venous. The pathology is characterized by a gradual increasing course, with the formation of multiple foci or in the form of diffuse vascular lesions of the brain and spinal cord.
• Radiation encephalomyelopathy occurs as a result of radiation exposure. The intensity of the clinical picture depends on the radiation dose received. Possible symptoms: neurotic disorders, psychosis, vegetative-vascular dystonia, hemorrhages, cerebral edema.

Complications and consequences

The development of complications in encephalomyelopathy is most often associated with pronounced and irreversible damage to brain structures. The following adverse effects may occur:

• loss of self-care, which is associated with the appearance of motor and cognitive impairment;
• pelvic organ dysfunction;
• immobilization, paralysis and paresis;
• development of dementia with loss of previously acquired knowledge and skills, with further complete personal disintegration;
• deterioration and loss of visual and auditory function;
• severe mental disorders;
• seizure syndrome;
• disturbance of consciousness to the point of coma;
• lethal.

Encephalomyelopathy is a complex pathology. However, compliance with all doctor's prescriptions and recommendations can prevent the development of complications and slow down the progression of the pathological process.

Diagnostics of the encephalomyelopathies

If encephalomyelopathy is suspected, the diagnosis consists of the following steps:

• listening to complaints, taking a history;
• a neurologist's exam;
• assessment of the state of the trunk vascular network, with possible application of ultrasound technology (ultrasound Dopplerography, duplex vascular scanning, rheoencephalography).

The specialist detects atherosclerotic vascular change or complete stenosis, kinks, bends, arterial dissections, and defects in vascular development.

Next, neuroimaging methods are connected - in particular, magnetic resonance or computed tomography, after which the state of higher functions (neuropsychological testing), assess the quality of fat and carbohydrate metabolism, analyze the rheological properties of blood. If necessary, an electrocardiogram and echocardiogram are performed.

The tests for encephalomyelopathy are nonspecific, but as part of the general diagnostic measures provide an opportunity to assess kidney, liver, pancreas function, and in case of intoxication help to detect toxic substances in the bloodstream.

Instrumental diagnosis in suspected encephalomyelopathy is usually represented by such investigations:

• ultrasound Doppler ultrasonography of cerebral and cervical vessels;
• electroencephalography with the evaluation of electrical impulses that occur during brain activity;
• Rheoencephalography (as an adjunct to Doppler);
• CT or magnetic resonance imaging.

At the discretion of the doctor, it is possible to prescribe other examinations and specialist consultations - to clarify the root cause of encephalomyelopathy.

Differential diagnosis

The initial stage of encephalomyelopathy should be differentiated with the syndrome of vegeto-vascular dystonia, which is a functional imbalance in nervous activity.

The clinical picture noted in patients with encephalomyelopathy is also found in malignant tumors, somatic pathologies affecting the brain and spinal cord, and often becomes the first manifestation of the prodromal stage of infectious diseases, or is a symptom complex of borderline psychopathologies (neuroses, psychopathies), depressive states, schizophrenia.

Symptomatology in encephalomyelopathy is nonspecific, may contain signs of posthypoxic, posttraumatic, intoxication, infectious-allergic, metabolic, paraneoplastic pathologies. In turn, disorders of brain metabolic processes are often the result of congenital or acquired abnormalities of neuronal metabolism, or extracerebral disease.

Difficulties arise in differential diagnosis with neurodegenerative pathologies that are accompanied by focal neurologic symptoms and cognitive impairment, such as:

• multisystem atrophy;
• supranuclear paralysis;
• Parkinson's disease and Alzheimer's;
• cortico-basal degeneration;
• frontal temporal dementia and several others.

Treatment of the encephalomyelopathies

The basis of therapeutic measures in encephalomyelopathy are such directions as restoration of functional disorders, activation of compensatory schemes. It is mandatory to combine drug therapy with physiotherapy, massage, physical therapy. Social and psychological support of patients, specifics of care, application of additional rehabilitation techniques to optimize the quality of life of patients are separately specified.

When selecting an appropriate treatment, the doctor takes into account, among other things, etiologic and pathogenetic features of encephalomyelopathy development in a particular patient. Specialists of narrow profile are invited for consultation: cardiologists, pulmonologists, urologists, etc.

Medication treatment for encephalomyelopathy may include the use of such medications:

• vasoactive drugs (Vinpocetine, Bilobil, Cavinton, Cinnarizine, Nicotinic acid);
• Nootropic drugs (Nootropil, Phenotropil, Piracetam, Cytoflavin, Cerebrolysin, Neuropeptide, γ-aminobutyric acid preparations, Glycine);
• complex drugs (Actovegin, Vasobral, Tanakan);
• antioxidants (vitamin C, E, Mexidol).

Concomitant therapy is supplemented with symptomatic medications, including:

• with extrapyramidal and pyramidal pattern correctors;
• anticonvulsants;
• with dehydration solutions;
• as correctors of psycho-vegetative activity and insomniac disorders;
• pain medication;
• non-steroidal anti-inflammatory drugs;
• of the B vitamins.

Central inhibitors of acetylcholinesterase - in particular, Reminyl, Rivastigmine, Aricept - should be used to inhibit the development of degeneration processes in encephalomyelopathy. Reminyl is administered in the initial dose of 4 mg twice a day for a month. Then switch to a maintenance dose of 8 mg twice a day for two months to six months. At the same time, the maximum recommended amount is 12 mg twice a day, for a long time.

Memantine (Acatinol) is a common drug for encephalomyelopathy. It improves the course of cognitive reactions and memory, promotes daily activity, inhibits the progression of the disease. The dosage is increased gradually, bringing it to the required amount. During the first week take 1/2 tablet in the morning, then switch to taking 1 tablet per day. From the third week, the dose is increased by 10 mg weekly. On average, the established daily dose is 10-30 mg.

Surgical treatment

A radical measure in the form of surgery can be used when the lumen of the carotid or vertebral arterial trunks is narrowed. The aim of the operation is to prosthetically repair or reconstruct the arterial vessel, stenting in order to restore normal blood flow in the brain structures.

The type of surgery for encephalomyelopathy is selected and performed by a specialist vascular surgeon on an individual basis.

Prevention

To minimize the risk of encephalomyelopathy and avoid the development of complications, the following recommendations should be remembered:

• minimize any stress and anxiety;
• eat a healthy and balanced diet;
• take multivitamin and mineral complexes regularly;
• keep a full day's routine, get enough rest;
• avoid injury;
• adhere to an active lifestyle, walk a lot in the fresh air;
• give up all bad habits, lead a healthy lifestyle;
• Seek medical attention in a timely manner if necessary.

To prevent perinatal encephalomyelopathy, risk factors during pregnancy and delivery should be minimized. It is important to take measures to prevent intrauterine oxygen deprivation of the fetus. It is necessary to adequately and timely treat any acute and chronic diseases, carefully observe and correct a complicated pregnancy.

Forecast

Encephalomyelopathy of moderate to severe course requires a comprehensive drug and physiotherapeutic approach, with courses of massage and physical therapy. With organized competent therapy, it is possible to significantly restore impaired functions. Some patients may require lifelong supportive therapy.

Success in the treatment of the disease depends on the effectiveness of targeting the underlying cause of encephalomyelopathy and the dynamics of cerebrospinal and cerebral changes.

At the third stage of pathology, we are more often talking about irreversible disorders, serious neurological and mental disorders, loss of ability to work, disability and even lethal outcome.

More detailed prognosis of encephalomyelopathy can only be voiced by the attending doctor, taking into account that the initial conclusion is usually not final. It changes during a certain period of treatment - for example, when the pathology worsens, or when the positive dynamics of therapeutic measures appear.

If detected early, encephalomyelopathy may have a more favorable prognosis.