There are 2 main types of inflammatory diseases of the central nervous system in dogs: granulomatous meningoencephalomyelitis (GME) and necrotizing encephalitis (NE), which in turn have many subtypes.
Since the classification is based on clinical signs and the specific pattern of changes observed on MRI, the division is largely arbitrary. A single final classification has not yet been accepted - the difficulty is that the final diagnosis and determination of the type of disease can only be established histologically, based on the specific picture of brain tissue postmortem.
Granulomatous meningoencephalomyelitis (GME) is a common disease with acute progressive symptoms. GME has been described in dogs of different breeds and ages, but small and toy breeds (terriers, poodles) of middle age (2-7 years) are most predisposed; Bitches get sick more often than males.
The histological picture is characterized by the presence of perivascular infiltrates (granulomas), consisting mainly of mononuclear cells of the immune system in the tissue of the brain and spinal cord. It may also affect only the brain, or (less commonly) only the spinal cord.
In this type of disease, lesions are concentrated primarily in the white matter.
There are 3 forms of the clinical course of GME:
- focal (localized)
- multifocal (disseminated) - the most common
- ocular (eye) - rare
Symptoms directly depend on the nature of the lesions and the number of lesions.
The disseminated form of GME is characterized by an acute onset of symptoms and a rapid increase in disturbances in the central nervous system; with a localized form, clinical signs develop more slowly.
Typical symptoms of granulomatous meningoencephalomyelitis:
- seizure activity
- focal disorders of the cranial nerves
- pain in the neck, tonic reaction
- Optic neuritis (together with other disorders in the multifocal form or isolated - in the ocular form)
- Sometimes there is an increase in temperature at the onset of the disease
A lifetime diagnosis of GME is made based on clinical signs, medical history (breed, sex, age), results of a neurological examination and data from special studies.
The most informative method of laboratory diagnostics is the study of cerebrospinal fluid.
A cerebrospinal fluid smear reveals mononuclear pleocytosis, with a relatively high content of neutrophils (about 20%). The biochemistry of the cerebrospinal fluid reveals an increased protein content.
In rare cases, changes in the cerebrospinal fluid are not detected.
When performing visual neurodiagnostics (MRI), the results vary. Sometimes the picture may be normal, but in most cases localized or multiple volumetric formations with clear outlines or contrasting zones with blurred boundaries are visualized.
The prognosis for this disease ranges from cautious to unfavorable; life expectancy can range from several days to several years. The use of new treatment protocols can significantly improve patient survival.
Necrotizing encephalitis (NE) is a disorder associated with the formation of multiple foci of necrotic, non-purulent inflammation in the brain, affecting both gray and white matter.
There are necrotizing meningoencephalitis (NME) and necrotizing leukoencephalitis (NLE).
Necrotizing meningoencephalitis is characterized by the presence of foci in the brain; the pathological process also affects the soft and arachnoid membranes. The lesions are usually extensive, and the clear boundary between the gray and white matter is smoothed out.
Necrotizing meningoencephalitis was first described in the 70s in pug dogs, and was known as “pug encephalitis”, however, modern visual neurodiagnostic data show that this type of disease also occurs in other small breed dogs, in particular Maltese dogs, small terriers, Chihuahuas, Shih Tzus, Pekingese, Papillons.
For necrotizing leukoencephalitis, similar lesions are typical, which often, in addition to the cerebrum, also cover the trunk, while lesions of the cerebral cortex and arachnoid membrane may not be observed. Most often the disease is observed in dogs of the breed Yorkshire Terrier.
The course can be acute or chronic (several months). The occurrence and development of clinical symptoms, as with granulomatous meningoencephalomyelitis, is determined by the localization of the lesion. Signs indicating damage to the cerebral cortex predominate - convulsions, maneuvering movements, resting on objects, disturbances of consciousness, visual disturbances with preserved pupillary reflex). There may be soreness in the neck area.
For treatment, drugs with an immunosuppressive effect are used; treatment is long-term, usually lifelong.
Several corticosteroids (prednisolone, methylprednisolone), cyclosporine and cytosine arabinose (cytosar) can be used as therapy. The latter are preferred due to both less severe side effects and increased median survival.
As a rule, animals have a favorable short-term response to steroid monotherapy at immunosuppressive doses (from 3 mg/kg/day with a gradual dose reduction to 0.5 mg/kg/day for a long period), but the clinical effect may be short-lived. After the first 4-6 weeks of prednisone therapy, cytosine arabinoside may be recommended (SC administration at a dose of 50 mg/m2 every 12 hours for 2 days, interval between courses 3-6 weeks).
The client should be informed that there is no definitive treatment for this group of diseases in dogs; most outcomes result in the death of the animal or euthanasia due to unsatisfactory quality of life. Therapy is aimed at suppressing the pathological immune response in the central nervous system for as long as possible. Symptomatic anticonvulsant therapy may be indicated in some cases.
The long-term prognosis is cautious. With a good response to therapy, survival ranges from 6 months to, rarely, several years.
Kurganskaya Natalia Ivanovna,
veterinary neurologist
20.04.2019
Leukoencephalitis is an inflammation of the brain of viral origin. Pathological changes affect the white matter, and degenerative processes also occur in neurons (gray matter).
Schilder's disease is a rare but very dangerous disease for which any therapy is ineffective. The causes of its occurrence are still being studied by scientists.
Leukoencephalitis of the brain: what is it?
The disease is classified as a pathology. The pathogen provokes loss of myelin, which leads to severe neurological disorders. For the first time, a disease called “diffuse periaxial sclerosis” was described by a neurologist from the USA P. Schilder in 1912. Later, other forms of leukoencephalitis were described, which made it possible to combine them into a group.
This disease was identified as an independent disease relatively recently - in 1912. The description was compiled by, after whom the pathology was named. It is characterized by degenerative demyelinating lesions of the brain, with the formation of large confluent segments of demyelination (a pathological process that represents damage to the myelin sheaths).
The disease develops slowly but steadily, while general infectious symptoms are rarely observed. Signs consist of progressive dementia (dementia), disorders of the autonomic system, hyperkinesis (involuntary movements), psychotic state disorders, and epileptic seizures. There is no typical picture of Schilder's disease.
Sometimes an illness can resemble a mental illness, sometimes it “disguises” itself as a tumor in the brain tissue or. Such signs are explained by the diffuse, mixed characteristics of the processes, the size and number of lesions, the level of severity of edema, which are localized around the vessels of the brain tissue.
A common form of the disease is pseudotumor, that is, pseudotumor. Symptoms of high intracranial pressure (nausea with vomiting, blurred vision) accompany progressive unifocal symptoms (epileptic seizures, blindness in one half of the visual field, weakening of muscle tissue).
The pseudotumorous form of encephalitis differs from tumors in a number of features:
- Multifocality of lesions.
- Regular changes in Lange reactions.
- The intensity of symptoms tends to fluctuate (from pronounced negative signs to the complete absence of symptoms during periods of remission).
- EEG reveals early significant lesions in some segments of brain tissue.
- During remissions, a decrease in the concentration of protein-cellular breakdown is observed (determined by taking samples of cerebrospinal fluid - puncture). Focal changes also decrease.
The age at which the disease often manifests remains controversial. Foreign medical scientists believe that Schilder's leukoencephalitis affects people aged 6-13 years, while observations by Russian specialists indicate that the number of cases of different categories is equal.
Etiology and pathogenesis
Despite the fact that the viral nature of the pathology has been established, a specific virus could not be isolated. The disease is believed to be caused by viruses:
- measles;
- herpes;
- rabies.
The disease is associated with the activation of slow infections that remain in the human body for many months or years.
Penetrating the blood-brain barrier, viruses begin replication in nerve cells, causing the destruction of myelin. Demyelination occurs diffusely or its individual foci appear, and the process affects all parts of the brain.
Histological studies indicate the presence of degenerative and inflammatory changes in the medulla, accumulation of cellular elements of blood and lymph, destruction of axons, and proliferation of glial cells. The resulting pathomorphological changes entail, often with hemorrhages.
Subacute forms are characterized by sequential destruction of conduction systems, and in more rare cases, diffuse lesions of the white cerebral matter. The lesions affect the associative zones responsible for storing information, thought processes, and learning ability.
In parallel with the destruction of myelin sheaths, proliferative processes occur in glia, and the number of fibrillar astrocytes increases.
Causes
The causes and mechanisms of development of the disease are still under study. Judging by the name, we can conclude that the cause was considered to be inflammation of the brain tissue. A viral etiology has also been suggested.
Microviruses that trigger inflammatory processes can provoke development. However, attempts to isolate infectious agents remained unsuccessful. Therefore, another theory was put forward, which suggests a connection between the occurrence and development of the disease with dysfunction of the regulatory mechanism of lipid metabolism, which brings leukoencephalitis closer to leukodystrophy.
Morphological changes consist in the fact that significant lesion zones appear in the white matter of brain tissue, which are most often located asymmetrically and have clear, pointed features. Cases have been recorded where such changes are formed in the brain stem and/or cerebellum.
In adolescent and adult patients, round lesions were observed that resembled the plaques that occur in multiple sclerosis.
Symptoms
Leukoencephalitis is characterized by polymorphic, nonspecific symptoms. They appear:
- Decrease in intelligence, up to dementia.
- Mental instability: apathy gives way to strong agitation.
- Hallucinations.
- Loss of acquired skills: writing, reading, ability to perceive speech.
- Deterioration or loss of vision and hearing.
- Involuntary trembling.
- Convulsive seizures.
There are a lot of variants of combinations of these signs, so it is impossible to identify a classic variation in the course of the disease. In some cases, the clinical picture is similar to a variant of multiple sclerosis, sometimes it is pseudotumor in nature or has symptoms of a psychiatric disease.
Classification and features of forms of leukoencephalitis
Acute hemorrhagic leukoencephalitis
The reasons for its development are not fully understood, however, cases of the development of the clinical picture after preventive vaccinations have been recorded.
It has a very severe course, characterized by rapid loss of myelin sheaths of brain axons and inflammation of vascular structures. Inflammation of the blood vessels is necrotic in nature, resulting in multiple hemorrhages. Fibrin accumulates in the brain structures, and tissue infiltration by white blood cells (leukocytes, macrophages, lymphocytes) occurs.
The death of neurons provokes the formation of necrotic foci, which merge to form large areas of necrosis. The rapid course of the pathology leads to death within a few days after the appearance of the first clinical signs.
The cause of the development of the pathology is considered to be a slow viral infection (presumably measles or herpes). The pathogen provokes the launch of autoimmune mechanisms, which leads to demyelinating processes in the neurons of the central nervous system. Some scientists attribute the disease to hereditary leukodystrophies.
Schilder's disease is characterized by the appearance in the white matter of chaotically located large foci of demyelination with clear outlines. The process affects not only the cerebral hemispheres, but also the brain stem and cerebellum. When the disease develops in adolescence, plaque-like structures are revealed, which are similar in structure to the plaques of multiple sclerosis.
There is still debate about the age groups that are most susceptible to the disease. According to Western neurologists, the first clinical signs develop in school-age children under twelve years of age.
Domestic scientists adhere to the theory that the disease develops in people of different age categories.
Acute hemorrhagic leukoencephalitis of Hurst
By its nature, the disease resembles acute hemorrhagic leukoencephalitis, however, upon careful examination, some differences are revealed. The disease is rare and is characterized by high degree lethality. Destructive processes in the white cerebral matter lead to its “melting,” which is associated with fibrinoid vascular necrosis. Various hemorrhages form in areas with fibrin effusion. A picture of diffuse cerebral edema is observed.
Van Bograart disease
Occurs in infants under six months of age. The disease is characterized by predominant damage to the cerebral trunk and spinal cord. The disease begins with extrapyramidal structures, gradually moving to pyramidal ones. Most children die from complications before reaching the age of ten.
Pathological anatomy
A pathoanatomical autopsy of the skull reveals signs of cerebral edema, widening of the grooves, and atrophic changes in the cortical layer. On a section of the hemispheres, foci of necrosis of asymmetric localization are visible. Some areas acquire a spongy structure, and a slight expansion of the ventricles of the brain is observed.
Histological preparations show signs of acute inflammation. An accumulation of white blood cells - lymphocytes and plasma cells, and focal demyelination is detected. The inflammatory process affects the white matter, its foci are located in the cortex and meninges.
Leukoencephalitis of the brain: clinical picture
The characteristic signs of this disease are mild, and the symptoms are similar to other forms of encephalitis:
- weakness, headache, vomiting;
- irritability;
- cognitive disorders;
- movement disorders;
- fainting, coma.
An early symptom of the disease is neuropsychic abnormalities. Patients complain of weakness and fatigue, they become irritable. Over time, the symptoms worsen, unmotivated aggression appears, thinking abilities decrease, and dementia may develop. Patients experience auditory or visual hallucinations.
Behavioral disorders are accompanied by movement disorders. Muscle rigidity, convulsive contractions of the facial muscles, tonic spasms of the muscle groups of the body and other types of hyperkinesis are observed.
After damage to the pyramidal system, in later stages there is a decrease in motor activity, paralysis and paresis. A person experiences depression and becomes apathetic. In this case, convulsive muscle contractions can persist at all stages of the disease.
The last stages of the disease are characterized by trophic and vegetative disorders. Patients lose the ability to move, thermoregulation disorders, exhaustion, and hyperhidrosis are observed.
Diagnosis of encephalitis
Accurate data on how to diagnose Schilder's encephalitis have not yet been developed. The diagnosis is made after the death of the patient, during an autopsy. To determine the disease during life, a careful comparison of the anamnesis, tomographic studies, and the general clinical picture will be required.
Other specialists are also involved:
- Ophthalmologist.
- ENT doctor.
- Psychiatrist.
Electroencephalography allows you to identify symptoms of brain tissue damage:
- Decreased alpha activity.
- Increased epileptic activity.
- Alpha rhythm disturbance.
A puncture is prescribed, that is, cerebrospinal fluid is taken for analysis. The presence of the disease is indicated by an increase in gamma globulin, while the specific gravity of the albumin fraction decreases significantly. The most informative method for identifying Schilder’s leukoencephalitis is. The syndrome is confirmed by the presence of several confluent or one significant focus of demyelination in the brain tissue.
In order to finally make a diagnosis, most neurologists are guided by the criteria of S.M. Poser:
- According to MRI, there are round lesions, the size of which cannot be less than two by three centimeters.
- Pathology of the adrenal glands is excluded.
- Absence of other cerebral disorders (neoplasms, encephalomyelitis or others).
- The concentration of fatty acids is normal.
If the patient dies, an autopsy (post-mortem autopsy) is performed. The presence of the disease is indicated by the presence of segments in which abnormal tissue growth has occurred.
The disease can be distinguished from leukodystrophy only by histological examination of the cerebral brain tissue of the lesion segments.
Treatment of leukoencephalitis
The approach to treatment must be comprehensive. Pathogenetic therapy involves the use of anti-inflammatory, decongestant and. Diuretics are used to eliminate cerebral edema, and glucocorticoids (Prednisolone) help reduce the intensity of the inflammatory process.
In parallel with hormonal anti-inflammatory drugs, antihistamine therapy (Diphenhydramine, Suprastin, Diazolin) is carried out.
To restore metabolic processes in the body, infusion of solutions (physiological, Ringer-Locke) is indicated. Magnesium and potassium supplements and B vitamins help strengthen the nervous system.
For severe manifestations of the disease, symptomatic therapy is prescribed. They use anticonvulsants, tranquilizers, antidepressants, painkillers and antispasmodics.
Due to the fact that there is no data on the exact causes of leukoencephalitis, effective treatment methods have not yet been developed. Glucocorticosteroid therapy has some effect. Therefore, many patients are prescribed Methylprednisolone. Initially, it is administered by injection (in increased dosages), then taken orally, with a gradual reduction in doses.
Therapy is also prescribed:
- Neuroprotective, that is, protecting neurons from death (Cerebrolysin, Ceraxon, Piracetam, etc.).
- Vascular (Cinnarizine, Vinpocetine).
- Antioxidant (Mexidol, Glutamic acid and others).
- Decongestant (Acetazolamide, Furasemide, etc.).
- Anti-convulsant drugs are prescribed (Diazepam, Cabamazepine, etc.).
If necessary, psychotropic medications, polypeptides, and a special vitamin course are used. Of course, the patient needs constant care, he should be fed, his skin should be monitored, wiped with specialized products to prevent the appearance of bedsores.
Disease prognosis
Leukoencephalitis of the brain is a severe pathology with a progressive course. Depending on the form of the disease, patients die within the first few days after the appearance of the first clinical symptoms, or several months later.
With a favorable remitting course of the disease and adequate treatment, patients can live for several years. During this period, the symptoms of the disease may be erased or completely absent.
Schilder's leukoencephalitis inevitably progresses, pathological changes are irreversible. Therefore, death always occurs as a result of the disease. The duration of development of the disease ranges from five months to four years. Cases have been recorded where the disease lasted longer than this period, becoming protracted and chronic. In this case, periods of remission may be observed when the symptoms of encephalitis are completely absent.
Schilder's leukoencephalitis is an incurable disease. All therapeutic measures can only somewhat alleviate the condition of the sick person and for some time restrain the progression of this syndrome. However, scientists are actively working on developments aimed at identifying the causes and creating effective means to combat the disease.
Necrotizing meningoencephalitis (NME) is an inflammatory disease of the central nervous system (CNS) that occurs more commonly in young and typically miniature breed dogs. Necrotizing meningoencephalitis has been described in many small dog breeds, including the pug, maltese, and chihuahua.
Without supportive aggressive immunosuppressive treatment, necrotizing meningoencephalitis is quite rapidly fatal and often fatal, even despite adequate therapy. Similar to granulomatous menigoencephalitis, it is difficult to diagnose and only histological analysis of the affected tissue can definitively make a diagnosis.
Clinical picture of necrotizing meningoencephalitis in the Yorkshire terrier and possibilities of supportive treatment
NME is characterized by multiple, often asymmetric, necrotizing, nonexudative meningoencephalitis and is mainly localized in the cortex and subcortical white matter of the brain. Necrotizing menigoencephalitis is identified as a separate nosology. The most commonly affected animals are: Yorkshire Terrier, French Bulldog, Spitz, Miniature Pinscher, Pekingese. Unlike granulomatous meningoencephalitis, when the accumulation of cells in the affected areas prevails, in necrotizing meningoencephalitis the processes of alteration (destruction) prevail. These changes can be detected using research methods such as MRI and CT. Intense inflammation and softening of areas are detected, usually limited to the subcortical white matter of the brain and most often in the region of the brain stem and cerebellum.
For maintenance treatment, immunosuppressive therapy is used. Treatment is basically the same.
Leukoencephalitis(leukoencephalitis; Greek leukos white + encephalitis[s]) is a progressive inflammatory and degenerative lesion of the white matter of the brain. Refers to demyelinating diseases. With leukoencephalitis, the gray matter of the brain is usually affected, so the more correct term is “panencephalitis”.There are several forms of the disease: subacute sclerosing panencephalitis of Van Bogaert, periaxial leukoencephalitis of Schilder, acute hemorrhagic leukoencephalitis. It is assumed that Van Bogart panencephalitis is caused by the measles virus, which can persist in brain neurons for a long time and, under certain conditions, become activated. Some researchers consider Schilder's leukoencephalitis as a variant of multiple sclerosis in children. Acute hemorrhagic leukoencephalitis often occurs after preventive vaccinations. The pathogenesis is not clear. A hyperergic autoimmune process is assumed, in which viruses play the role of a trigger.
Panencephalitis is characterized by diffuse or focal demyelination in all parts of the brain, atrophy of the gyri, and widening of the sulci. Histologically, diffuse inflammatory and degenerative changes in the white and gray matter of the brain, perivascular infiltrates, degeneration of neurons with neuronophagic nodules and inclusions in the nuclei and cytoplasm are noted. Normally formed myelin is destroyed (myelinoclastic type of demyelination, as opposed to dysmyelination observed in leukodystrophies and some intracellular lipidoses, for example amaurotic idiocy). There is intense proliferation of astrocytes and a proliferative reaction of glia. Gliosis can be small-focal or large-focal. Diffuse gliosis leads to thickening of the brain substance. Neurons of the cerebral cortex contain spherical inclusions with a diameter of 30-40 microns or smaller, tubular in shape.
In acute hemorrhagic leukoencephalitis, cerebral edema is found, on a section of the hemispheres - foci of a soft pink-gray color with numerous pinpoint hemorrhages, and histologically - fibrinoid necrosis of the walls of small vessels with ring-shaped hemorrhagic zones, perivascular demyelination with axonal destruction.
Subacute sclerosing Van Bogaert panencephalitis develops mainly in children aged 5 to 14 years (usually in boys); after 17 years, the disease is extremely rare. A connection with measles is revealed (as the incidence of measles decreases due to vaccination, the incidence of Van Bogart panencephalitis decreases). The onset is gradual, with the development of asthenoneurotic syndrome: increased fatigue, irritability, lethargy, and mood instability appear. Then efficiency, anger, aggressiveness, egocentrism, inertia of thinking, headaches, fears develop, and children become withdrawn. Against this background, focal neurological symptoms arise and progress: hyperkinesis (myoclonus, twitching of the head, limbs, torso); muscle tone increases; spastic paralysis of the tetraparesis type, pseudobulbar, less often bulbar, syndrome develops. Cerebellar disorders are added - dynamic ataxia, dyskinesia. Convulsive seizures are characteristic. They appear at different stages of the disease. There may be minor, abortive convulsive, generalized convulsive seizures. In advanced stages of the disease, trophic and autonomic disorders, cachexia, thermoregulation disorders, immobility, and decerebrate rigidity are noted. Sometimes a pseudotumorous course with characteristic cerebral and local symptoms is observed. Primary optic atrophy often develops,
Characteristic changes in the EEG: periodic flashes of high-amplitude slow waves against the background of reduced bioelectrical activity. An increased protein content is sometimes detected in the cerebrospinal fluid. During electrophoretic separation of protein fractions, a decrease in albumin content and an increase in IgG fractions are observed. A pronounced increase in the titer of anti-measles antibodies is observed in the blood and cerebrospinal fluid. Computed tomograms of the brain reveal atrophy of the cerebral hemispheres, enlargement of the ventricles of the brain, and foci of low density of varying sizes in the white matter of the hemispheres.
The course of Van Bogaert panencephalitis is steadily progressive, sometimes spontaneous remissions are observed. Duration from 1-3 months. up to 3 years or more.
The diagnosis is made on the basis of a progressive course, an increase in symptoms of damage to the extrapyramidal and pyramidal systems, cerebellar symptoms against the background of the development of dementia, optic nerve atrophy, seizures, and an increase in the titer of anti-measles antibodies. Computed tomography data are also important.
Hormonal and symptomatic therapy is used (muscle relaxants - mydocalm, cyclodol, baclofen, anticonvulsants, B vitamins). The prognosis is unfavorable.
Acute hemorrhagic leukoencephalitis is characterized by an acute onset, a lightning-fast increase in the pattern of brain damage. This is preceded by a period of catarrhal phenomena in the pharynx (for 2-4 days), followed by severe headaches, meningeal symptoms, disturbances of consciousness, convulsions, hemi- or tetraparesis, pseudobulbar syndrome. In the cerebrospinal fluid there is polymorphonuclear pleocytosis and an increase in protein concentration. The duration of the disease is from 2 days to 2 weeks. The outcome is fatal.
19. The metabolic and molecular bases of inherited disorders / Ch. Scriver et al. (Eds). - New York et al.: McGraw-Hill Inc., 1995. - Vol. 2, -P. 3011-3013.
ACUTE HEMORRHAGIC LEUCOENCEPHALITIS OF HURST T.V. Maratkanova, G.A. Denisova, I.L. Vostrikova
Moscow Regional Research Clinical Institute
Acute hemorrhagic leukoencephalitis (AHLE) of Hurst is a rare, fatal post-infectious or allergic demyelinating disease of the central nervous system with a rapid increase in symptoms, observed mainly in young people.
This disease was first described as an independent nosological unit by E.W. Hurst in 1941.
Pathoanatomical changes in OGLE are characterized by destruction of the white matter of the cerebral hemispheres to the extent of melting, widespread fibrinoid necrosis of the vessel walls, leading to the formation of multiple small hemorrhages in the affected areas with exudation of fibrin into the surrounding space, as well as cellular infiltration of areas of necrosis.
Clinically, the disease occurs in the form of an acute infection with high fever and a rapid increase in neurological symptoms in the form of pronounced cerebral and focal symptoms. Hemi- or tetraparesis, epileptic seizures and disturbances of consciousness are often observed.
Sometimes OGLE develops immediately, without prodromal phenomena.
In general, the clinical picture of OGLE has the character of severe encephalitis without signs pathognomonic specifically for this form.
Most patients die 2-4 days after the onset of neurological symptoms.
Diagnosis of OHLE due to its rarity and rapid lethal outcome presents significant difficulties.
Patient G-nov., 21 years old, was transferred to the neurological department of MONIKI on November 24, 1999 from the Lyubertsy Central District Hospital in serious condition. Ill for about a week: according to relatives, he became drowsy, apathetic, and inadequate. On November 20, the patient developed a generalized epileptic seizure, after which disorientation and lack of reaction to the environment appeared. He was hospitalized in a hospital at his place of residence.
On examination: the patient is lethargic and adynamic. The patient has no complaints. Conscious, but uncommunicative, inadequate, foolish. Doesn't answer questions, only carries out basic commands after repeated repetitions.
The general condition is relatively satisfactory, pulse - 88 beats/min., rhythmic, blood pressure - 115/80 mmHg. There were no significant changes in the internal organs.
Neurological status: no clear meningeal symptoms.
Cranial nerves - without pathology. Diffuse muscle hypotonia. Tendon reflexes are low and symmetrical. No pathological foot signs were identified. There are no paresis. Sensitivity due to the patient’s negativism is not possible to check. Pelvic functions are not impaired.
On the 2nd day after admission, an RCT examination of the brain was performed - there were signs of diffuse cerebral edema, predominantly of the white matter, with compression of the basal cisterns, both Sylvian fissures, the 3rd ventricle and predominantly the upper parts of the left lateral ventricle, and the external subarachnoid space. Against this background, supratentorially - in both hemispheres of the brain, foci of increased and decreased density are vaguely defined with a predominant localization in the basal parts of the right temporal, both frontoparietal lobes (including parasagittal) and outward from the head of the caudate nucleus on the left. The sizes of hyperdense lesions are up to 1 cm in diameter, their density increases imperceptibly after intravenous contrast enhancement. The 4th ventricle is located in the middle, of normal shape, size and position. The conclusion was given: RCT picture of diffuse focal changes in both hemispheres of the brain. It is necessary to differentiate between the inflammatory process (viral encephalitis?) and metastatic lesions. Clinical further examination is recommended.
Consultation with an ophthalmologist: the disc is pink, the nasal border is blurred. The veins are wide. Phenomena of initial stagnant disk.
Ultrasound of the abdominal organs and retroperitoneal space, clinical and biochemical blood tests revealed no pathology.
Treatment with corticosteroids and cycloferon was started with some positive dynamics - a decrease in the phenomena of congestion in the fundus, the patient became more active, adequate, followed simple commands, which made it possible to conduct an MRI study of the brain on the 8th day.
MRI examination of the brain in sagittal and axial projections, in T1 and T2 VI modes - in both hemispheres of the cerebrum and cerebellum, multiple small (maximum up to 6 mm) lesions are detected, having a high signal on both T1 and T2 VI. Their structure on T2 is relatively homogeneous, and on T1 it is heterogeneous due to the presence of a low-intensity zone in the center of each of the foci, which gives
they have a ring shape. The lesions are predominantly localized in the cortex, including the subcortical nuclei and adjacent white matter. There are also in the thalamus on both sides. No perifocal changes were noted. The ventricles of the brain were of normal size in the present study. The basal cisterns are differentiated. The external subarachnoid spaces are smoothed. The conclusion was given: Multiple focal brain lesions of hemorrhagic density, most likely of inflammatory origin. Further dynamic monitoring is recommended.
28.11.01. The patient's condition began to deteriorate: lethargy increased again with periodic attacks of psychomotor agitation, and bulimia developed. In the neurological status, pronounced meningeal symptoms appeared (stiff neck muscles, severe Kernig's sign on both sides), failure of the eyeballs to reach the external commissures.
Despite the therapy (cycloferon, corticosteroids, Lasix, mannitol), the patient’s condition progressively worsened; on December 26, 1999, due to severe respiratory disorders (tachypnea up to 40-42 per minute, cyanosis, arterial hypotension up to 80/40), the patient was transferred to the intensive care unit.
Intensive therapy was carried out, the patient was transferred to mechanical ventilation.
01/5/99 - the patient’s condition is extremely serious. FMN - anisocoria, D>S, mydriasis. Diffuse muscle hypotonia. Tendon reflexes are sharply depressed and symmetrical. There are no pathological symptoms. rigidity of the occipital muscles - 3 fingers, Kernig's sign on both sides. Coma III-IV.
On January 6, 1999, despite infusion therapy and the administration of sympathomimetics, cardiac arrest occurred.
Clinical diagnosis: Subacute progressive panencephalitis. Swelling of the substance and membranes of the brain.
A pathological examination confirmed the nature of the brain damage - hemorrhagic leukoencephalitis, subacute course, productive meningitis. The disease occurred against the background of suppression of both humoral and cellular immunity.
Thus, the interest of this observation lies in the fact that it demonstrates the capabilities and importance of MRI studies in identifying hemorrhagic-like changes in the brain, which can be observed, according to the literature, in AIDS patients. And only taking into account the clinic, the diagnosis was made during the patient’s lifetime and confirmed by the result of an autopsy.
LITERATURE
1. Dekonemko E.P. “On the issue of the modern definition of the term “leukoencephalitis” “Materials for the republican working meeting” Issues of diagnosis and treatment of demyelinating diseases of the nervous system.”, Stupino, February 23-24, 1999, pp. 25-28.
2. Buravtseva V.P., Smirnova G.G. Acute hemorrhagic leukoencephalitis of Hurst\\Journal of Neuropathology and Psychiatry. - 1965.- No. 3. - P.328-333.
3. I.A.Kachkov., M.F. Makarenko, S.V. Kotov, A.M. Kiselev, G.A. Stashuk. Clinical and computed tomographic differential diagnosis of acute necrotizing encephalitis and glial brain tumor \\Bulletin of Practical Neurology.1998. -No. 4. - P.158-160.