Neurorheumatism is a rheumatic process in the tissues of the central and peripheral nervous system. It is manifested by symptoms of encephalitis, meningitis, myelitis, arachnoiditis, sciatica, neuritis. The leading clinical form is small chorea. Neurorheumatism is diagnosed by a neurologist and a rheumatologist taking into account laboratory data, electroencephalography, CT/MRI of cerebral structures, and cerebrospinal fluid studies. Treatment includes rheumatism therapy, decongestants, psychotropic, antiepileptic, antihyperkinetic drugs.
General information
Rheumatism is a systemic lesion of connective tissue of infectious and allergic genesis (collagenosis). It mainly affects the heart, blood vessels, joints with the development of rheumatic endocarditis, vasculitis, polyarthritis, respectively. The name “cerebral rheumatism” was proposed in 1845. The term “neurorheumatism” has become widely used among specialists in the field of neurology and rheumatology since the middle of the twentieth century, thanks to the works of Professor V. V. Mikheev. The defeat of the nervous system in rheumatism occurs both against the background of the acute phase of the disease (rheumatic neurosis, psychosis, ACVA due to thromboembolism) and as a result of the inflammatory process in the connective tissue elements of the NS (encephalitis, myelitis, neuritis). The concept of neurorheumatism includes lesions directly caused by the rheumatic process in the structures of the NS.
Causes
An etiological factor in the occurrence of rheumatism is an infectious process caused by beta-hemolytic streptococcus (angina, acute pyelonephritis, pharyngitis). In its structure, the pathogen has antigens similar to individual elements of the connective tissue of the human body. In some cases, the production of antibodies to streptococcus is accompanied by a malfunction of the immune system, leading to the production of autoantibodies to the components of its own connective tissue. Autoimmune inflammation develops involving the walls of blood vessels, heart valves, intra-articular and periarticular tissues, and nerve structures.
Rheumatism occurs in 0.3-3% of patients with streptococcal infection. Predisposing factors are:
- Young age — the peak of morbidity falls on the period of 7-15 years.
- Dysfunction of the immune system with a tendency to autoimmune processes.
- Burdened heredity — the presence of rheumatism in the closest
- Female relatives — women get sick 3 times more often than men.
Pathogenesis
Neurorheumatism with peripheral NS lesion is associated with the production of antibodies to connective tissue elements of spinal roots and nerve trunks. Rheumatic damage to the central nervous system is caused by cerebral vasculitis, inflammatory changes in brain tissues. Vasculitis is characterized by thickening of the vascular walls, perivascular edema, diapedesis hemorrhages, microthrombosis. Vascular damage leads to obstruction of venous outflow, ischemia of brain tissues. At the same time, inflammatory changes are observed in the meninges (thickening, increased blood filling, hemorrhages), subcortical structures (gliosis, degenerative processes), the medulla, and the diencephalic region. The features of the clinical picture depend on the predominant involvement of one or another part of the brain. In the overwhelming majority of cases, neurorheumatism occurs with damage to subcortical nodes (striatum, caudate nucleus), clinically manifested by symptoms of minor chorea.
Classification
Classification of rheumatic lesions of the NS is based on their clinicomorphological features. Sometimes there are combinations of different variants of neurorheumatism with the predominance of one of the components. According to the classification, there are two forms of the disease – with damage to the peripheral and central nervous system.
Neurorheumatism with central nervous system damage:
- Encephalitis is a diffuse small—focal inflammation of cerebral tissues combined with softening areas. With the predominant localization of the process in the striatum, the most common form of neurorheumatism develops — small chorea. The predominant lesion of the diencephalic region causes the occurrence of rheumatic diencephalitis.
- Meningitis is rheumatic inflammation of the cerebral membranes.
- Arachnoiditis is inflammatory changes in the subarachnoid membrane with the formation of adhesions, arachnoid cysts.
- Myelitis is an inflammatory process in the substance of the spinal cord.
- Epileptiform syndrome — epilepsy attacks developing against the background of rheumatism.
Neurorheumatism with peripheral NS lesion:
- Sciatica is an autoimmune inflammation in the area of the spinal root. It can be multiple in nature.
- Neuritis — inflammation of the trunk of one (mononeuritis) or several (polyneuritis) peripheral nerves.
Symptoms
Clinical manifestations are polymorphic, depending on the form of pathology. They occur mainly after an attack of rheumatism. With the latent course of the rheumatic process, neurorheumatism can act as the debut of the disease.
Rheumatic encephalitis manifests psychoemotional symptoms: irritability, tearfulness, touchiness. Headache comes to the fore from the general cerebral manifestations. Then hyperkinesis joins, which can be of the nature of tics, chorea, myoclonia, torsion dystonia. In the classical version, the disease is characterized by symptoms of minor chorea: rapid choreic hyperkinesis in the muscles of the extremities, facial muscles with muscular dystonia, mental disorders, vegetative symptoms, discoordination. Diencephalitis occurs with hyperthermic syndrome, polydipsia, hypertension, hyperhidrosis, sleep/wake rhythm disorder, emotional disorders (fears, hypochondria, hysteria).
Rheumatic meningitis is manifested by severe headache, increased susceptibility to external stimuli, meningeal syndrome. Arachnoiditis is accompanied by a violation of the outflow of cerebrospinal fluid, an increase in intracranial pressure with a bursting headache, nausea, a feeling of pressure on the eyeballs, visual disturbances, cerebellar ataxia. The clinic of myelitis depends on the location of the spinal cord lesion, includes central and peripheral paresis, sensitivity disorders, pelvic dysfunction.
Epileptiform syndrome is characterized by atypical epileptic paroxysms, the predominance of small seizures (absences, akinetic paroxysms). Characteristic changes of the psyche (excitement, twilight state of consciousness, hallucinatory syndrome), automatism. Unlike true epilepsy, some patients are able to feel an approaching attack, which allows them to avoid sudden falls, injuries. Epileptic episodes are rare, occur once every 2-3 months, an increase in frequency is observed against the background of deterioration of the somatic condition.
Neurorheumatism with lesions of the spinal roots manifests itself as a radicular syndrome. There are pains in the spine and along the spine, loss of sensitivity, weakness of muscle groups, the innervation of which is provided by the affected spinal nerve. Involvement of the peripheral nerve in the rheumatic process is manifested by pain, paresthesia, numbness, muscle weakness and hypotension. These symptoms are localized in the innervation zone of the nerve affected by rheumatism, with the multiple nature of the process they cover several areas.
Complications
In severe cases, neurorheumatism is complicated by cerebral edema. Inflammation in encephalitis, meningoencephalitis, encephalomyelitis is accompanied by an exudative process leading to swelling and swelling of the medulla. The leading symptom is a progressive disorder of consciousness leading to coma. Without urgent medical care, compression of the cerebral trunk with vital centers located in it responsible for the regulation of respiration and cardiovascular activity occurs. There is paradoxical breathing, sharp hypotension, cardiac arrhythmias. Possible fatal outcome.
Diagnostics
Polymorphism of symptoms makes neurorheumatism a difficult disease in diagnostic terms. The diagnosis is simplified by indicating in the anamnesis the presence of rheumatism in the patient. Diagnostic measures include:
- Neurologist’s examination. In 90% of cases, neurorheumatism is accompanied by violations of voluntary movements, reflex sphere, muscle tone. There may be sensory disorders, signs of autonomic dysfunction, mental changes. With neuritis and sciatica, pain points and areas of hypesthesia are determined.
- Lumbar puncture. Half of the patients have increased cerebrospinal fluid pressure. Examination of cerebrospinal fluid (CSF) does not detect obvious deviations, which makes it possible to exclude acute neuroinfection.
- Electroencephalography. Reveals diffuse changes in the form of rhythm disorganization, decrease / increase in the amplitude of the alpha rhythm. If neurorheumatism occurs with epileptiform syndrome, there is increased epileptic activity.
- MRI/CT of the brain. They may not detect pathological changes, they allow to exclude volumetric formations (cysts, abscesses, cerebral tumors). MRI of the brain with contrast is more informative, visualizing inflammatory foci by contrast accumulation.
- Consultation of a rheumatologist. It is necessary for the diagnosis of the rheumatic process, its stage, degree of activity. Includes an assessment of the state of the heart.
- Laboratory diagnostics of rheumatism. A clinical blood test is carried out, determination of rheumatoid factor, C-reactive protein, ASL-O, antistreptokinase, and a cupping sample in the blood.
Depending on the clinical form, neurorheumatism has to be differentiated from bacterial meningitis, tick-borne encephalitis, infectious myelitis, tumor processes in the central nervous system, Huntington’s chorea, idiopathic epilepsy.
Treatment
Therapy is carried out mainly in a hospital. The patient is shown bed rest with a lot of sleep. Recommended food rich in proteins and vitamins with a restriction of fluid intake, salt. The basis of drug treatment is anti-rheumatic therapy, against the background of which measures are taken to relieve the neurological symptoms that have arisen, mental disorders. Pharmacotherapy includes:
- Antirheumatic treatment: nonsteroidal anti-inflammatory drugs, glucocorticosteroids, antibacterial drugs (bicillin). Appointments depend on the activity and stage of rheumatism.
- Dehydration therapy. It is performed with diuretics (acetazolamide, furosemide). It is aimed at reducing intracranial pressure, preventing brain edema.
- Relief of hyperkinesis with minor chorea is carried out by chlorpromazine, with other hyperkinesis — benzodiazepines, cholinolytics. The drug and dose are selected individually.
- Vitamin therapy: B vitamins, ascorbic acid, rutin.
- Psychotropic drugs: neuroleptics (haloperidol), tranquilizers (diazepam), antidepressants (amitriptyline), sedatives. They are indicated for mental disorders.
- Anticonvulsants: valproic acid preparations, carbamazepine, phenobarbital, clonazepam. They are prescribed for epileptiform syndrome.
Prognosis and prevention
The outcome depends on the timeliness and success of treatment of the underlying disease — rheumatism. Against the background of therapy, the duration of minor chorea usually varies within 1-3 months. With angina, acute respiratory viral infections, pregnancy, relapses are possible. The absence of specific treatment is fraught with aggravation of CNS lesions, has an unfavorable prognosis. Measures that can prevent neurorheumatism include earlier detection and correct treatment of primary forms of rheumatism, and drug prevention of relapses. For secondary prevention, the patient should be monitored by a rheumatologist.
