Bell’s disease is a late form of syphilis, occurring in the form of a progressive inflammatory-dystrophic total lesion of cerebral structures. It differs in the stages of development, at first there are symptoms of pseudoneurasthenia, then mental abnormalities against the background of intellectual decline, at the final stage – severe dementia. Diagnosis is carried out on the basis of the conclusions of a neurologist, a psychiatrist, the results of blood and cerebrospinal fluid tests for syphilis, and cerebral MRI data. Treatment is carried out comprehensively with pyrogenic, antitreponemal, psychocorrective, neurometabolic drugs.
ICD 10
A52.1 Neurosyphilis with symptoms
General information
Bell’s disease is a separate nosological form of late neurosyphilis. It is a syphilitic meningoencephalitis — an inflammatory lesion of the membranes and parenchyma of the brain. The disease was described in detail in 1822 by the French psychiatrist Antoine Bayle, after whom the name Bell’s disease was given in neurology. In 1913 , the Japanese pathologist X. Noguchi proved the syphilitic etiology of the disease by detecting pale treponema in the brain tissue of patients. Bell’s disease occurs in 1-5% of syphilis patients. The time range of the development of symptoms lies in the range from 3 to 30 years from the moment of infection, more often it is 10-15 years. The peak incidence occurs in the age range of 35-50 years. In modern neurological practice, Bell’s disease is a fairly rare pathology, which is due to the timely diagnosis and treatment of syphilis.
Causes
The etiofactor of the disease is the causative agent of syphilis — pale treponema. The source of infection is a sick person, the main way of infection is sexual and injection. Transmission of the pathogen through saliva is possible. The introduction of an infectious agent into cerebral tissues occurs by hematogenic and lymphogenic pathways. The cause of the long-term persistence of the pathogen, which is associated with Bell’s disease, is reliably unknown. Probable predisposing factors are the absence or insufficiency of correct treatment of early forms of syphilis, hereditary predisposition, alcoholism. The triggers provoking the activation of infection are considered to be traumatic brain injury, weakening of immunity against the background of acute and chronic somatic diseases.
Pathogenesis
The mechanism of infection activation is unclear. Assume a restraining role of the immune system, blocking the reproduction of the pathogen. Violations of the reactivity of the body, immunosuppressive states lead to the spread of pale treponema in cerebral tissues. In the initial stage, Bell’s disease is characterized by an inflammatory process that covers all brain structures — panencephalitis, then atrophic changes come to replace inflammatory changes.
Macroscopically, there is turbidity of the soft cerebral membrane, its fusion with the underlying cerebral parenchyma. Ependymitis is typical — inflammation of the lining of the ventricles of the brain. There is hydrocephalus, diffuse atrophy of the cortex with the expansion of the furrows between the convolutions. The microscopic picture is represented by vasculitis of small cerebral vessels with plasmocytic perivascular infiltration, shrinking and death of neurons, demyelination and atrophic changes in nerve fibers, glial proliferation, the appearance of amoeboid cells.
Classification
The clinical symptoms are variable, poorly manifested at the onset of the disease, changes and worsens as it progresses. Understanding the stage of the process is necessary to select the most effective therapy. In this regard , in neurological practice, Bell’s disease is divided into three clinical periods:
- Initial. Corresponds to the beginning of clinical symptoms. Symptoms of neurasthenia, sleep disorders, decreased performance are characteristic. The initial stage lasts from several weeks to 2-3 months. By the end of the period, personality changes with a tendency to loss of ethical norms become apparent.
- The development of the disease. Increasing personality changes, behavior disorders, speech, gait and handwriting disorders, intellectual decline prevail. Trophic disorders, dystrophic changes of somatic organs are typical. Without treatment, the stage lasts 2-3 years.
- Dementia. The progressive disintegration of the cognitive sphere is accompanied by apathy, complete loss of interest in the environment. Epileptic seizures and strokes are often observed. Trophic disorders worsen, bone fragility increases. The patient dies from stroke, intercurrent infections.
The following classification is based on the syndromological approach. Taking into account the prevailing symptom complex, a whole list of variants of the disease has been identified. The main ones are the following clinical forms:
- Dementia (simple) — increasing dementia with a predominance of euphoria. The most common. It is not accompanied by severe mental disorders.
- Expansive — expressed excitement with a predominance of delusional thoughts about their own greatness, wealth. Euphoric-complacent mood is abruptly replaced by anger. Typically verbose, criticism is completely lost.
- Euphoric — progressive dementia is accompanied by fragmentary ideas of greatness. In contrast to the expansive form, it proceeds without pronounced neuropsychic excitement.
- Depressive — dementia progresses against the background of ridiculous hypochondriac complaints. It seems to patients that they have no insides, that they have already died. Elements of depersonalization, derealization up to the Cotard syndrome are characteristic.
- Agitated — arousal is pronounced, there is a tendency to aggression. Visual and auditory hallucinations are observed. Consciousness is confused, there is a rapid disintegration of personality.
- Circulatory — similar to the manifestations of bipolar disorder. The period of manic excitement is replaced by severe depression. There is an opinion that this form is realized against the background of a genetic predisposition to mental illness.
- Hallucinatory-paranoid — psychosis with a predominance of hallucinatory syndrome, delusions of persecution, catatonia. It is suggested that this variant of the disease occurs in patients with schizoid psychopathy.
The most typical onset of Bell’s disease is in middle age. Bell’s disease, manifesting in childhood and old age, differs in a number of features. Accordingly, there are 2 age-related forms of the disease:
- Juvenile. Debuts at the age of 6-16 years after suffering from congenital syphilis resulting from transplacental infection. More often it occurs in the form of a dementia form with the Getchinson triad (keratitis, hearing loss, lesion of the upper incisors). The manifestation of symptoms may be preceded by a delay in mental development.
- Senile. The debut falls on the period after 60 years. Typically a long asymptomatic course. Korsakov syndrome may develop. The senile form is extremely difficult to differentiate from senile dementia.
Bell’s disease symptoms
The disease begins with nonspecific, slowly increasing symptoms, similar to neurasthenic manifestations. Patients complain of decreased performance, unusual fatigue, irritability, headaches, memory impairment, sleep disorders. Over time, the patient’s relatives notice changes in his personality: indifference to the problems of others, loss of a sense of tact, behavior going beyond ethical norms. Patients begin to make gross mistakes in their usual professional activities, with the progression of symptoms they lose the ability to realize them. Sleep disorders are aggravated: hypersomnia is noted during the day, insomnia at night. There are eating disorders (anorexia, bulimia). Speech is excessively accelerated or slowed down. Criticism of their behavior is decreasing.
At the stage of development of the disease, Bell’s disease is accompanied by an aggravation of personal and behavioral disorders. Inappropriate behavior, ridiculous actions, flat jokes are revealed. Mental disorders differ in polymorphism, euphoria, depression, delusions, hallucinations, depersonalization are detected. There are difficulties in pronouncing complex words in speech, then dysarthria is formed — speech becomes slurred, blurred with the omission of individual sounds.
The gait is uncertain, loose, the handwriting is uneven, dysgraphy with missing letters is noted. There are fainting spells, epileptic seizures (more often by the type of Jackson’s epilepsy). Appetite disorders cause weight loss or weight gain. Trophic disorders appear: reduction of skin turgor, brittle nails, mild infection with the development of boils, abscesses. It is characterized by increased bone fragility, dystrophic changes of the heart (cardiomyopathy) and liver (hepatosis).
During the period of dementia, there is an increase in dementia up to senility. The patient ceases to serve himself, interact with others, arbitrarily control the function of the pelvic organs. Refusal to eat leads to pronounced weight loss. Sometimes dysphagia is noted — a swallowing disorder. Numerous trophic ulcers are formed, the increasing fragility of bones causes fractures.
Complications
Accompanying Bell’s disease, cerebral vasculitis is the cause of frequent ACVA. As a result of a stroke, a persistent neurological deficit is formed: paresis, aphasia, apraxia. Trophic changes lead to a decrease in the barrier function of the skin with the development of pyoderma, abscesses, and bedsores at later stages. Possible pneumonia, inflammation of the urinary tract (cystitis, pyelonephritis). The penetration of infectious agents into the blood causes the occurrence of sepsis. Strokes and infectious complications are the most common cause of death.
Diagnostics
The disease is most difficult to diagnose in the initial period. Individual patients do not have anamnestic data on the presence of syphilis in the past, which also makes it difficult to determine the nosology. The diagnosis is established with laboratory confirmation of syphilitic etiology. The list of necessary examinations includes:
- Neurological examination. At the stage of the debut, attention is paid to slight anisocoria, paresis of the oculomotor muscles, asymmetry of reflexes, instability, unsteadiness of gait, monotony of speech. In the midst of symptoms, the neurologist reveals a decrease in pupil photoreaction, dysarthria, and pronounced cognitive dysfunction. Paresis, aphasia and other focal symptoms caused by the transferred ACVA are possible.
- Neuropsychological testing. Performed by a psychiatrist, neuropsychologist. Includes assessment of cognitive abilities and mental status. Dementia, lack of criticism, psychomotor agitation, depressive-phobic, hallucinatory, delusional syndromes are characterized by varying severity. Twilight consciousness is possible.
- Laboratory diagnostics. Treponemal blood tests (Wasserman reaction, RPR test) are positive in almost 100% of patients. In doubtful cases, repeated analyses are additionally carried out in dynamics, RIF, RIT.
- Examination of cerebrospinal fluid. Liquor is obtained by lumbar puncture. The analysis reveals an increased amount of protein, positive globulin reactions, cytosis due to lymphocytes. The Lange reaction gives a specific shape of the curve in the form of a tooth. Most patients have positive reactions of RIF and RIBT with cerebrospinal fluid.
- MRI of the brain. Depending on the phase of the disease, it reveals diffuse inflammatory or atrophic changes affecting the membranes, brain matter and ventricular ependyma. Scanning makes it possible to exclude the presence of a volumetric formation (intracerebral tumor, brain abscess), to conduct a differential diagnosis with progressive degenerative diseases.
The initial stage of Bell’s disease should be differentiated from neurasthenia. Features of the neurological status, the presence of epileptic episodes, early changes in the patient’s personality noticed by relatives can help. In the future, differential diagnosis is carried out with mental disorders, degenerative cerebral processes (vascular dementia, Alzheimer’s disease, multisystem atrophy, Pick disease). The most reliable criterion confirming Bell’s disease is positive treponemal reactions of blood and cerebrospinal fluid.
Bell’s disease treatment
Patients need etiotropic and psychocorrective therapy. Experimentally, the therapeutic effect of high body temperature was discovered. Modern treatment includes four main components:
- Pyrotherapy. Previously, it was carried out by introducing malaria pathogens followed by antimalarial therapy. Pyrogenic preparations are currently being used.
- Antibacterial therapy. Penicillin antibiotics, ceftriaxone, quinine iodosmutate, bismuth nitrate have an anti-treponemic effect. Etiotropic therapy begins with large doses, continues for 2-3 weeks. In parallel, probiotics, multivitamin preparations are prescribed, if necessary, hepatoprotectors.
- Psychotropic treatment. It is shown in the second phase of Bell’s disease. Individual selection of pharmaceuticals (sedatives, neuroleptics, antipsychotics, antidepressants) is carried out in accordance with the symptoms.
- Neurotropic therapy. It is aimed at improving metabolic processes in the brain and restoring its functions. It includes pharmaceuticals of vascular, nootropic, neurometabolic action. In the stage of dementia, neurotropic treatment does not have a significant effect.
Prognosis and prevention
The results of treatment depend on the timeliness of its start. In 20% of cases, correct therapy makes it possible to achieve almost complete restoration of health. In 30-40% of patients, it is possible to achieve stable remission with moderate mental abnormalities that allow them to adapt in society. Persistent organic dementia persists in 40% of cases. The process of liquor sanitation takes from six months to 3-4 years. It is possible to prevent Bell’s disease by observing personal hygiene, refusing casual sexual relations, unprotected sexual acts. When syphilis is first established, correct treatment is necessary until complete sanitation of biological media. Subsequently, the patient is recommended to follow up and periodic examination for early detection of late forms of syphilis.