Leptomeningeal disease is an inflammation of the soft and arachnoid meninges that occurs when infected with bacterial, viral or fungal agents. The clinical triad includes severe headaches in the forehead and temples, repeated vomiting without nausea, and an increase in body temperature of more than 38 ° C. Diagnosis of pathology involves general and microbiological analysis of cerebrospinal fluid, brain tomography. The treatment program includes etiotropic drugs (antibiotics, antiviral agents, immunoglobulins), pathogenetic and symptomatic therapy. To prevent complications, a comprehensive recovery program is selected.
ICD 10
G00 G03
General information
Leptomeningeal disease is more common than pachymeningitis (inflammation of the dura mater), so it is often referred to in clinical practice by the general term “meningitis”. Meningitis continues to be one of the most common lesions of the nervous system. Leptomeningeal disease is characterized by polymorphism of the clinical picture, a severe course, especially with the development of a purulent process. If medical care is not provided, up to half of the cases end in the death of the patient, which is why the disease remains an urgent problem of clinical neurology.
Causes
Inflammation of the meningeal membranes is caused by infection of the cerebrospinal fluid by pathogenic microorganisms. The etiological structure of the disease is characterized by great variability, it depends on the age of the patient, his immune status, the epidemic situation in the region. In modern infectology , the following groups of pathogens of leptomeningeal disease are distinguished:
- Bacterial. Bacterial meningitis develops when infected with N. meningitidis (50-60%), S. pneumonie (about 30%), H. influenzae (up to 10%). Rare pathogens of the disease include gram-negative enterobacteria, group A and B streptococci, Staphylococcus aureus. In children, in 1-3% of cases, the purulent process is caused by the bacterium Listeria monocytogenes.
- Viral. Primary inflammation occurs in the meningoencephalitic form of the tick-borne encephalitis virus. Secondary viral meningitis is provoked by enteroviruses, the mumps virus, which account for up to 50% of all cases. There are also herpetic, rubella, chickenpox forms of the disease.
- Fungal. This type of disease is extremely rare. Leptomeningeal disease can be observed when infected with fungi of the genus Candida, pathogens Cryptococcus neoformans, Aspergillus. Protozoal meningitis caused by Toxoplasma gondii is registered even less frequently.
Pathogenesis
The entrance gate of infection is the mucous membranes of the upper respiratory tract or digestive tract. First, the infectious agent attaches to them and causes a local inflammatory reaction. At the same time, a large number of microorganisms accumulate in the hearth. Then the pathogen penetrates into the structures of the central nervous system hematogenically, is fixed on the meningeal membranes, causing a serous or purulent inflammatory process.
The literature describes casuistic cases of contact spread of infection with trauma to the bones of the skull or in the presence of a chronic purulent focus near the brain (sinusitis, otitis media, facial furuncle). When infected with the rabies virus, it is possible for the pathogen to penetrate the meninges through the perineural spaces.
The main component of the pathological process of neuroinfections is irritation of the vascular plexuses due to inflammatory changes, which is accompanied by excessive production of cerebrospinal fluid, violation of its reabsorption. As a result, hypertension-hydrocephalus syndrome increases, which causes typical clinical symptoms, provokes swelling of the brain.
In inflamed meningeal membranes, dystrophic damage to neurons and surrounding cells occurs, blood supply to brain tissue worsens, severe hypoxia occurs. Purulent leptomeningeal disease is characterized by the development of fibrous inflammation, severe disorders of cerebrospinal fluid dynamics. In complicated cases, it is possible to involve brain tissue in the process with the formation of meningoencephalitis.
Classification
Specialists in infectious diseases divide leptomeningeal disease into primary, associated with direct penetration of the pathogen into the brain, and secondary, which is caused by the presence in the body of another focus of infection, usually purulent. The second option is much more common. In neurological practice, other diagnostic criteria are also used to classify the disease:
1 Etiology: bacterial, viral, protozoal, fungal.
2 Degree of severity: light, medium-heavy, heavy.
3 Duration: acute, prolonged, chronic.
4 Flow pattern:
- Smooth.
- Non-smooth: with complications, with the addition of secondary infection, with exacerbation of chronic pathology.
Symptoms
Pathology is manifested by a typical triad of clinical signs: febrile fever, severe headache, repeated vomiting. The symptoms of the disease mainly manifest suddenly against the background of full health or existing acute respiratory viral infections, but with tuberculous lesions of the meninges, the symptoms increase gradually. Meningitis is characterized by 3 clinical syndromes: general infectious, cerebral, meningeal.
With a general infectious syndrome, hyperthermia, chills, and severe weakness are typical. The pallor of the skin is noted, the patient refuses to eat and drink. General cerebral signs include severe pain in the frontotemporal zone, which increases with eye movements, loud sounds, bright flashes of light. There are multiple vomiting, unrelated to eating and not bringing relief, convulsions, disturbances of consciousness.
Meningeal syndrome is manifested by a forced “cocked trigger” pose: head thrown back, legs bent and tucked to the stomach. They are concerned about photophobia, hyperacusis (excessive susceptibility to sounds), hyperalgesia (increased pain sensitivity). The examination reveals the rigidity of the occipital muscles, symptoms of Kernig, Brudzinsky. Less often, the symptoms of Mondonesi, Bekhterev are determined.
Complications
The most dangerous consequence of leptomeningeal disease is cerebral edema, leading to temporal-tentorial or transtentorial wedging, dislocation at the level of the midbrain. Terminal dislocation, which is fraught with areflexia, total atony, and respiratory arrest, is considered the most prognostically unfavorable.
Also, acute complications include cerebral infarction, ventriculitis, DIC syndrome, multiple organ failure. Untreated purulent leptomeningeal disease results in a cerebral abscess, cystic-adhesive arachnoiditis. Inflammation caused by meningococci can lead to the spread of the purulent process and the onset of meningococcal sepsis.
The residual complications of pathology include a long-lasting neurological deficit, prolonged cerebrogenic asthenia, and a decrease in cognitive abilities. In rare cases, hydrocephalus, deafness, and optic nerve atrophy develop. An atypical complication of the disease is diabetes insipidus.
Diagnostics
The examination is carried out by a neurologist, an infectious disease specialist or an emergency doctor. To make a preliminary diagnosis, it is enough to collect complaints, general and neurological examination of the patient, detection of characteristic meningeal symptoms. Since clinical data do not allow to establish the etiology and type of leptomeningeal disease, the following diagnostic methods are additionally prescribed:
- Neuroimaging. To exclude edema or brain neoplasm, as well as if a complicated course of meningitis is suspected, a CT scan of the brain is indicated. For a more detailed study of the structure of the soft meninges, an MRI of the brain is performed.
- CSF analysis. Purulent meningitis is characterized by an increase in cerebrospinal pressure, turbidity and discoloration of the cerebrospinal fluid, cellular pleiocytosis with an increase in the level of neutrophils. With serous inflammation, lymphocytic pleiocytosis, protein-cell dissociation, transparent or opalescent liquor is observed.
- Microbiological diagnostics. To establish the type of pathogen, quick tests of latex agglutination, antigen detection reactions, bacteriological sowing of CSF for bacteria and fungi are performed. If necessary, PCR diagnostics of the cerebrospinal fluid is performed, blood culture is performed before the start of antibiotic treatment.
Treatment
Therapy should begin as early as possible, especially with the purulent nature of inflammation: even at the prehospital stage, emergency care begins in the form of the introduction of corticosteroids, cardiotonics, anticonvulsants. The patient is immediately hospitalized in an infectious disease hospital or intensive care unit. Treatment of the disease includes the following areas:
- Etiotropic therapy. Purulent leptomeningeal disease requires the use of antibiotics, which are selected empirically at the start, and then the treatment regimen is adjusted after receiving the results of bakposev. In case of viral infection, serum immunoglobulin, specific antiherpetic drugs, high doses of corticosteroids can be used.
- Pathogenetic therapy. The syndromic treatment is selected taking into account the severity of the condition, the period of the disease. Massive detoxification, dehydration and decongestant therapy is prescribed, hypoxia control (including ventilation), anti-shock measures are carried out in the intensive care unit.
- Symptomatic therapy. Nonsteroidal anti-inflammatory drugs (parenterally or rectally), narcotic analgesics are used for debilitating headaches. In case of exacerbation of chronic somatic pathology, its appropriate correction is performed.
To eliminate the residual effects of leptomeningeal disease, comprehensive restorative treatment is necessary. In the acute period, metabolic and neurovegetative protection of the brain is provided, including the intake of vitamins, nootropics, drugs to improve the rheological properties of blood. During convalescence, it is recommended to take adaptogens, angiotropes, and actoprotective drugs.
Prognosis and prevention
The best recovery rate is observed with viral (serous) inflammation of the meninges — the percentage of death is less than 1%. Purulent leptomeningeal disease causes persistent neurological consequences in 9% of cases, mortality in the absence of treatment reaches 50%. Fungal and tuberculous meningitis have an unfavorable prognosis.
Specific prevention includes vaccination against childhood diseases (measles, rubella, mumps), hemophilic and meningococcal infections. After contact with a patient with meningococcal serous or purulent meningitis, post-exposure antibiotic prophylaxis is performed. Non-specific preventive measures involve the prevention of respiratory infections, planned sanitation of existing purulent foci, protection from ticks.