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Normotensive hydrocephalus (also known as Hakim-Adams syndrome) is a disorder that clinically presents with disordered gait, impaired cognitive function, and impaired retention.

These symptoms, especially in older patients, are quite common and not always easy to associate with suspicion.

Etiopathogenesis and pathophysiology

In the history of some patients with suspected Hakim-Adams syndrome, it is possible to find, or inflammation of the central nervous system, which over the years led to the expansion of the ventricular system and subsequently to decompensation and clinical manifestations.

Most patients, however, have an idiopathic disorder where no clear cause-and-effect relationship is observed.

From a pathophysiological point of view, the hydrodynamic concept of increased pressure of cerebrospinal fluid pulsations in the ventricles without an increase in intraventricular pressure is recognized, which leads to the formation of hydrocephalus.

There are many reasons for impaired drainage of cerebrospinal fluid. It can be associated with the manifestation of birth defects, be a consequence or, and also develop in the mind of age.

Varieties of the course of the violation

There are two types of Hakim-Adams syndrome - in acute and chronic forms.

Functional classification of normotensive hydrocephalus:

• obstructive;
• communicative;
• hypersecretory;
• hyporesorptive.

In terms of dynamics (important for treatment)

• active;
• passive.

Clinical picture

Symptoms indicating the development of normotensive hydrocephalus:

• walking disorder (frontal): slowing down walking, reducing stride, decreasing stride height;
• impaired cognitive abilitythat resemble signs of dementia - apathy, decreased concentration, loss of interest, memory problems, psychomotor slowdown in general;
• violation of continence - first intermittent disturbance of urinary continence up to incontinence, including fecal incontinence.

Symptoms can be expressed in different ways. Most often, gait impairment appears first, which precedes cognitive impairment, and continental impairment develops last.

Additional symptoms that may be present (eg, psychoaffective disorders) are not typical, but do not preclude the diagnosis of NG. As a rule, they are typical for patients over 60 years of age, therefore, these clinical manifestations can be attributed to aging and their occurrence can be underestimated by the attending physician.

Diagnostic Methods

The following research methods are used to diagnose the disease.

If a disease is suspected, it is necessary to carry out, which will indicate the expansion of the ventricular system.

It is necessary to exclude other pathological changes leading to hydrocephalus (tumors, congenital anomalies, vascular malformations) and possible hydrocephalus "ex vacuo", in which the expansion of the ventricular system develops from brain atrophy and clinical manifestations may be similar.

Based on the CT scan results and clinical suspicions of normotensive hydrocephalus, the patient is examined by a neurologist who, using a structurally more appropriate MRI and clinical assessment, makes a differential diagnosis (other types of dementia and gait disorders - degenerative, intoxication, metabolic,), and, after excluding other causes, directs the patient to the appropriate department for further clinical investigation, walking check and cognitive tests.

If a disease is suspected, a CSF dynamic examination is performed.

Lumbar puncture

The simplest test is a lumbar puncture, in which a needle is inserted into the lumbar spinal canal in the lumbar region and intracranial pressure is measured (a potential exception), after which 30-50 ml of cerebrospinal fluid (CSF) is withdrawn. The sensitivity of the test is about 50-60%.

Lumbar drainage

If the above tests are negative, but with high clinical suspicion, lumbar drainage can be performed with a sensitivity of up to 90%.

Continuous drainage of cerebrospinal fluid 10 ml / h for 3 days mimics the introduction of the EP shunt and leads to clinical improvement.

Therapy options

The main treatment for Hakim-Adams syndrome is the introduction of a ventriculoperitoneal (VP) shunt. The operation takes about an hour, and during it the lateral ventricle is processed, which is connected through a tube to a valve guided by the subcutaneous tissue of the abdominal cavity.

There are also alternatives - ventriculoatrial and lumboperitoneal shunt. In recent years, ventculostromia has also been performed, in which the third ventricle is fenestrated endoscopically.

CT control of the brain is carried out 2-3 days after surgery in order to avoid possible complications. These include bleeding, misalignment of the shunt, etc. In the long term, it can lead to infection, which can lead to a violation of the shunt closure and the need for re-treatment.

Impregnation of the shunts with antibiotics leads to a decrease in the number of infections. The risk of infection during the first year was only up to 5%.

When indicated correctly, there is a relatively rapid and significant improvement in gait disturbances. Improvement in continental disorders is gradual, and patients with significant significant cognitive decline respond less positively to it.

After effective treatment, however, regular clinical monitoring with CT is necessary (once a year).

Complications and prognosis

The incidence of complications after surgery in patients with NG in the literature varies. Mortality does not exceed 2% and, according to some authors, is more associated with concomitant diseases than with shunt implantation.

The formation of subdural disorders is given in the range of 2-17%. The rate of these complications is greatly reduced with the use of modern programmable valves.

The risk of infectious complications is relatively low, and does not exceed 5-6%. Other complications may lie in the malfunction of the shunt, formation after ventricular puncture and even.

The duration of gait deterioration before surgery plays an important role, and patients with gait disorder lasting less than 1 year have the best prognosis.

The positive effect of the above methods of treating the disease is 70-80% of patients. The shunt effect decreases over several years, and the bypass pressure must also be reduced regularly.

In order not to become a victim of the disease, a person must be aware of precautions. It is necessary to always cover your head during risky work, use helmets when riding a motorcycle, detect and treat infectious and inflammatory diseases in time, and prevent hypothermia of the head.

It is characterized by a chronic disorder of CSF dynamics, expansion of the ventricular system without a significant increase in the level of intracranial pressure, clinically manifested by the classic triad of symptoms: gait disturbance, dementia and pelvic disorders.

History

NG was first described as a syndrome by Colombian neurosurgeon S. Hakim in 1957. In the English-language literature, the term "normotensive hydrocephalus" was introduced by the American neurosurgeon R. Adams in an article published in the New England Journal in 1965, where three clinical observations of NG were described - two post-traumatic and one idiopathic.

Epidemiology

Normotensive hydrocephalus is considered as a disease of elderly people aged 60-80 years, although cases of the occurrence of this disease in middle and even childhood have been described.

Pathology

Currently, there are primary (idiopathic) and secondary (symptomatic) NG. Secondary NG most often develops after suffering from subarachnoid hemorrhage (23%), meningitis (4.5%), traumatic brain injury (12.5%), but there may be other reasons. 50 When making a diagnosis by a clinician, be it a diagnostician or a neurologist, it is important to measure the typical clinical findings and changes in tomography, as often patients with this pathology are treated as patients with cerebrovascular disease or another form of hydrocephalus.

Clinical manifestations

The Hakim-Adams triad, which includes dementia, impaired walking, and pelvic organ dysfunction, is considered a characteristic manifestation of NG. However, it must be remembered that the classic picture, described in 1965, is observed only in 32–48% of patients. Often there are only two symptoms, usually gait disturbance and dementia (30%), less often one of the 3 symptoms, and usually only a worsening of gait (about 10%). Nevertheless, gait disturbance remains the most common symptom in NG and, as a rule, manifests itself as an imbalance in the stage-by-stage activation of muscle groups, which should be considered as a disorder of subcortical movement control systems, and not as a primary disruption of the pyramidal tract. As NG progresses, the involvement of the corticospinal tract becomes more pronounced. The gait is described as apraxic, bradykinetic, magnetic, parkinsonian. Patients may also have difficulty turning. With NG, there were no changes in the friendliness of hand movements when walking. Cognitive disorders are characterized by a slowdown in psychomotor functions, impaired concentration and mild memory impairment. Such changes are often associated with dysfunction of the anterior regions of the brain and are quite characteristic of the so-called subcortical dementia. Primary disorders of higher cerebral functions (aphasia, apraxia, agnosia, acalculia), as well as psychoses, are rare. However, some patients may experience confabulations, hallucinations, mania, delirium, depression, and epileptic seizures. It is important to remember that for patients with NG, such a well-known classical picture of hydrocephalus, such as headache, nausea, vomiting, visual impairment, is not typical. When examining the fundus, there is no edema of the optic discs. Pelvic dysfunction debuts with an increase in urinary frequency, especially at night, with the gradual development of urinary incontinence. Pelvic disorders in NG are attributed to the frontal type of urination disorders, which is characterized by the absence of urge, the indifferent attitude of the patient to the fact of involuntary urination. Fecal incontinence is rare, usually in patients with advanced disease.

Radiological findings

MRI

• Ventriculomegaly
• Balloon-like expansion of the anterior horns of the lateral ventricles up to 30% or more of the skull diameter. With this pathology, the third ventricle, temporal and frontal horns of the lateral ventricles are especially significantly expanded, which leads to the appearance of a characteristic shape of the ventricular system in the form of a “butterfly” on axial sections.
• Periventricular halo - high signal at T2 and PD. These changes are due to transependymal CSF penetration.
• Deformation (scallop) of the corpus callosum and pericallosa arteries.
• The detection of small ischemic foci or leukoaraiosis does not contradict the diagnosis of NTG, since a combination of NTG and cerebrovascular insufficiency is possible. Signs of microangiopathic cerebrovascular disease correlate with a worse outcome of bypass grafting.
• Irregularity and deformation of subarachnoid spaces and cracks.
• Reduction of the angle of the corpus callosum.

CSF outflow assessment

It is believed that a blood flow velocity at the level of the cerebral aqueduct of more than 24.5 ml / min has 95% specificity for patients with hydrocephalus, and a stroke volume of cerebrospinal fluid of more than 42 μl / s in most cases allows predicting a positive effect of shunting surgery. A decrease in the stroke volume of the cerebrospinal fluid and the hyperdynamic nature of the cerebrospinal fluid in the ventricular system with its normal parameters at the craniospinal level are considered as possible predictors of a positive outcome.

MRI spectroscopy

Increased lactate peak.

PAT

With NTG, there is a decrease in the level of general and regional cerebral blood flow, especially in the frontal and temporal regions of the brain, subcortical white matter. According to the data of positron emission tomography, a decrease in the level of glucose metabolism (general and regional) is revealed, and the more significant the degree of frontal hypometabolism, the more likely the unfavorable outcome of the shunting operation becomes.

Treatment and prognosis

After ventriculoatrial, lumboperitoneal, ventriculoperitoneal shunting, positive dynamics is noted (improvement of regional blood flow, CSF dynamics and cerebral metabolism).

Differential diagnosis

• Normal aging brain
• Alzheimer's disease - atrophy of the hippocampus and temporal lobes.
• Obstructive hydrocephalus - must be the cause of the obstruction (formation in the pineal gland, midbrain, colliculus).
• Lewy body dementia - visual hallucinations and delusions.
• Parkinson's disease is a unilateral symptom.
• HIV-associated encephalopathy - positive serological test for HIV.

Morgagni syndrome occurs due to cerebral ischemia, which occurs with a sharp decrease in cardiac output. This happens when the heart rhythm or heart rate is abnormal.

Often, Adams Stokes' Morgagni attacks are caused by atrioventricular block. An attack occurs when a blockage occurs, followed by the development of sinus rhythm or supraventricular arrhythmia.

Causes provoking diseases and factors

Attacks of the syndrome occur during the following processes in the body:

• atrioventricular block;
• transition of incomplete atrioventricular block to complete;
• violation of the heart rhythm, which is accompanied by a decrease in myocardial contractility (with febrile, ventricular flutter, paroxysmal tachycardia, asystole);
• tachyarrhythmia and tachycardia with a heart rate of more than 200 beats;
• bradyarrhythmia and bradycardia with a heart rate of less than 30 beats.

The risk of developing the syndrome exists if the following conditions are present in the history:

• chagas disease;
• inflammatory processes localized in the heart muscle, and which spread to the conducting system;
• diffuse growth of scar tissue, and subsequent damage to the heart in Leva-Legener disease, rheumatoid arthritis, Liebman-Sachs disease, systemic scleroderma;
• diseases with general neuromuscular changes (genetic diseases, myotonia);
• intoxication with medications (beta-blockers, calcium antagonists, cardiac glycosides, amiodarone, lidocaine);
• ischemia of the heart muscle with cardiomyopathies, myocardioclerosis, heart attack;
• increased iron deposition in hemochromatosis and hemosiderosis;
• systemic amyloidosis;
• functional disturbances of conduction in the atrioventricular node.

Features of the clinical picture

The syndrome is observed in 25-60% of patients with complete atrioventricular block. The frequency and number of seizures varies from case to case. Attacks of Morgagni Edems Stokes can occur at intervals of every few years, and can occur several times during one day.

An attack can be provoked by sudden movements, abrupt changes in body position, nervous overload, worries, emotional stress.

The attack is preceded by the following signs:

After some time (about 1 minute), the patient has an attack, and he loses consciousness. Fainting occurs when the heart rate is less than 30.

Fainting is often short-lived and does not last more than a few seconds. During this time, compensatory mechanisms are activated, which make it possible to eliminate the arrhythmia. After recovering from this state, the patient has retrograde amnesia, and he does not remember what happened.

With an attack of Morgagni Adams Stokes syndrome, the following symptoms are characteristic:

• pallor of the skin;
• swelling of the neck veins;
• blue lips and fingertips;
• spontaneous bowel movements and urination;
• cold sweat (sticky);
• weak muscle tone, cramps;
• inability to determine the pulse;
• low blood pressure;
• deaf and arrhythmic heart sounds;
• dilated pupils;
• rare and deep breathing.

Depending on the degree of intensity of the manifestation of symptoms, several forms of an attack are distinguished:

1. Light - no loss of consciousness occurs, the patient experiences dizziness, impaired sensitivity, noise in the ears and head.
2. Moderate - the patient experiences loss of consciousness, but there are no signs such as voluntary urination and defecation, convulsions are also not observed.
3. Severe - the entire symptom complex is present.

Emergency first aid

With an attack of Morgagni-Adams-Stokes syndrome, the patient needs emergency medical care, on which the duration of the attack itself and the patient's life will depend.

The first step is mechanical defibrillation, also called precordial shock. It is necessary to strike with a fist in the chest, namely in its lower part. Do not hit the heart area. After mechanical defibrillation, the heart reflexively begins to contract.

If there is no effect, electrical defibrillation is performed. To do this, electrodes are applied to the patient's chest and a shock is produced with a current discharge. After that, the correct rhythm of the heartbeat should return.

In the absence of breathing, artificial lung ventilation is performed. To do this, air is blown into the patient's mouth using a special apparatus, or by the mouth-to-mouth technique.

Cardiac arrest is an indication for the injection of Epinephrine (intracardiac) or Atropine (subcutaneous).

If the patient remains conscious, then he must be given the drug Isadrin under the tongue (the effect is similar to Adrenaline, Ephedrine, Norepinephrine, but there is no increase in blood pressure).

The patient must be taken to the intensive care unit of the hospital. In the hospital, emergency care is accompanied by monitoring on an ECG machine. The patient is injected subcutaneously with the drugs Atropine sulfate and Ephedrine several times a day, and Izadrin is given under the tongue. Electrical stimulation is performed if necessary.

Establishing diagnosis

Loss of consciousness is possible with various diseases. Therefore, when carrying out diagnostics, the Adams-Stokes-Morgagni syndrome must be differentiated with the following conditions:

To determine the syndrome, the following diagnostic methods are used:

• electrocardiogram (ECG) in dynamics;
• monitoring of the cardiogram on the Holter apparatus (allows you to identify temporary changes, a combination of atrial flutter and atrial fibrillation);
• long-term monitoring of the electroencephalogram;
• contrast coronography of vessels;
• myocardial biopsy.

Syndrome treatment

Initiation of treatment implies emergency treatment for an attack. This is followed by therapy, the purpose of which is to prevent the recurrence of attacks of Morgagni-Adams-Stokes syndrome. Therapeutic measures are carried out in the cardiology department.

Initially, the causes of seizures are identified, a detailed examination of the heart is carried out, the diagnosis is clarified and a set of therapeutic measures is prescribed. Such methods of therapy of the syndrome are used.

Drug treatment

After the patient is admitted to the intensive care unit, the patient is treated with medications. Droppers are used with the introduction of Ephedrine, Orciprenaline. Every 4 hours, the patient is given Izadrin. Injections of Ephedrine, Atropine are made.

Inflammatory processes are relieved with corticosteroids. Since bradycardia is accompanied by tissue acidosis and hyperkalemia, it is necessary to take diuretics, an alkaline solution. This helps to remove potassium from the body and normalize blood pressure.

After the cessation of the attack, prophylactic therapy with the use of antiarrhythmic drugs is prescribed, and also therapeutic measures are directed to get rid of the main cause of the syndrome (ischemia, intoxication, inflammatory process).

Surgery

If there is a risk of sudden cardiac arrest and recurrence of seizures, implantation of a pacemaker is a necessary measure. It is possible to use two types of pacemakers: with a complete blockade - an apparatus that provides constant heart stimulation, with incomplete blockade - an apparatus that is triggered when violations occur.

During surgery, an electrode is inserted through a vein and fixed in the right ventricle of the heart. The body of the stimulator is fixed in the rectus abdominis muscle (for men) or in the retromammary space (for women).

The pacemaker must be checked for operability every 3-4 months.

Prevention of seizures and relapses

The use of preventive measures is possible with attacks that are caused by paroxysms of tachyarrhythmias or tachycardia. In this case, patients are prescribed various antiarrhythmic drugs.

You should also exclude the factors that lead to the onset of an attack - abrupt movements, sudden changes in body position, experiences, nervous overload, emotional stress, intoxication.

With a complete atrioventricular blockade, the main preventive method is the installation of a pacemaker.

What is the consequence?

The severity of the consequences directly depends on the frequency of seizures and their duration. Frequent and prolonged hypoxia of the brain entails a negative prognosis of the disease.

Hypoxia for more than 4 minutes causes irreversible brain damage. The lack of resuscitation measures (chest compressions, artificial respiration) can lead to the cessation of cardiac activity, the disappearance of bioelectrical activity and death.

During surgery, the prognosis is positive. Implantation of a pacemaker allows you to restore the quality of life, work capacity and health of the patient.

This section was created to take care of those who need a qualified specialist, without breaking the usual rhythm of their own life.

Biology and medicine

Normotensive hydrocephalus (Hakim-Adams syndrome)

Hakim-Adams syndrome (normotensive hydrocephalus) is a condition that is often talked about but is difficult to diagnose. Normotensive hydrocephalus is a syndrome with characteristic clinical and pathophysiological signs, as well as typical changes on CT or MRI. The classic triad is gait disturbance as a result of ataxia or apraxia (see Table: Combination of frontal dysbasia-type gait disorders (gait apraxia) with dementia and urinary incontinence).

CT or MRI detects enlargement of the lateral ventricles (hydrocephalus) in the absence or slight severity of cerebral atrophy. Hydrocephalus - communicating, we pass the sylvian aqueduct (Fig. 367.3). CSF pressure during lumbar puncture corresponds to the upper limit of the norm. The content of protein and glucose in CSF is not changed. It is believed that normotensive hydrocephalus develops due to disruption of CSF flow along the upper-lateral surface of the hemispheres and CSF absorption into the venous system. As the disease progresses slowly, the lateral ventricles dilate, but the CSF pressure does not increase significantly.

The cause of the clinical manifestations of normotensive hydrocephalus is unknown. Perhaps they are due to the stretching of the fibers of the radiant crown of the brain. Sometimes normotensive hydrocephalus develops after diseases leading to the formation of adhesions on the membranes of the basal surface of the brain, for example, meningitis, subarachnoid hemorrhage, or traumatic brain injury. However, in most cases, normotensive hydrocephalus develops for no apparent reason.

In contrast to Alzheimer's disease, in normotensive hydrocephalus, gait disturbances develop early, and cortical atrophy is not found on CT or MRI. Attempts have been made to find research methods that will confirm the diagnosis of normotensive hydrocephalus and predict the outcome of bypass surgery. Among them - cisternography (allows you to diagnose a slowdown in CSF absorption on the upper lateral surface of the hemispheres) and various CSF dynamic studies. None of these studies have sufficient specificity and are not used in everyday practice.

In some cases, gait and cognitive function temporarily improve after removal of the CSF ml, however, this cannot serve as a criterion for the effectiveness of the forthcoming bypass surgery. According to one study, no more than 1–2% of dementia cases are associated with normotensive hydrocephalus.

Sometimes this disease is misdiagnosed instead of Alzheimer's disease, since the latter can be accompanied by a violation of gait, and atrophy of the cortex at an early stage of the disease is not always visible on CT or MRI. A characteristic feature of Alzheimer's disease in this case may be atrophy of the hippocampus on MRI.

Bypass grafting with proven normotensive hydrocephalus is effective in 30-50% of cases. After surgery, gait sometimes recovers better than memory. Often, the improvement is short-lived. Patients for surgery are carefully selected, since it is often accompanied by subdural hematoma and secondary infection.

A clinical triad is characteristic: memory loss, gait disturbance and urinary incontinence. The first symptom is often gait disturbance, and the dementia is usually mild. CT and MRI of the brain reveal enlargement of the lateral ventricles, but atrophy of the cortex is absent or minimal. With lumbar puncture, the CSF pressure is normal or slightly increased, the composition of the CSF is not changed.

Normotensive hydrocephalus can be both idiopathic and a consequence of prior meningitis or subarachnoid hemorrhage (due to ruptured aneurysm or traumatic brain injury).

The probable pathogenetic mechanism is blockade of CSF outflow along the upper lateral surface of the brain, as well as difficulty in absorption of CSF into the blood, which leads to stretching and twisting of the pathways in the radiant crown. Ventricular shunting sometimes helps.

Differential diagnosis of normotensive hydrocephalus with Alzheimer's disease is difficult.

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Normotensive hydrocephalus, or Hakim-Adams disease

1. Definition 2. Etiology and pathogenesis 3. Symptoms of idiopathic hydrocephalus 4. Gait disorders 5. Dementia 6. About pelvic disorders 7. Diagnostics 8. Treatment 9. Complications and prognosis

When doctors talk about hydrocephalus, then almost always the leading mechanism of its development is an increase in intracranial pressure (increased ICP syndrome), or intracranial hypertension. It is she who is to blame for the appearance of symptoms such as progressive decrease in vision, bursting headaches and vomiting. In the event of a sudden obstruction to the flow of cerebrospinal fluid, occlusive hydrocephalus occurs, and then the brain may begin to swell and be impaired.

These types of hydrocephalus are described in detail here. But it turns out that there is also a form of the disease, which manifests itself in completely different symptoms and often goes unnoticed. We are talking about the so-called normotensive hydrocephalus, or Hakim-Adams syndrome. What is this pathology and is it possible to cure this disease?

Definition

This is the name of a condition in which the pressure of the cerebrospinal fluid almost always does not exceed the permissible values, but at the same time there is dropsy of the ventricular system of the brain. Since the pressure is within the normal range, the symptoms of such a pathological condition should be completely different. And this is so: it was only in 1965 that the first summarized data were published in The New England Journal of Medicine. The authors of the material were R. Adams, S. Hakim and C. Fisher. Since the name for this condition has not yet been invented, they described "latent hydrocephalus in adults with chronic course without changes in the fundus and with normal cerebrospinal fluid pressure."

Thus, we considered hydrocephalus syndrome, devoid of its basic and supporting diagnostic features. Everyone knows that at the first "classic" complaints associated with a presumptive increase in ICP, the doctor assesses the condition of the patient's fundus. In the event that there are no changes on it, then the diagnosis of hydrocephalus is considered doubtful. And here a mysterious state was described, in which not only the fundus of the eye was normal, but also there were no characteristic complaints - the course was hidden.

Nevertheless, this condition turned out to have its own "markers" - dementia, gait disorders and urinary incontinence. If these symptoms are accompanied by a pronounced expansion of the ventricles of the brain, then a diagnosis of "normotensive hydrocephalus" is made. According to ICD-10, he was assigned the code G 91.2, or “normal pressure hydrocephalus”.

The great difficulty in recognizing this condition is that such signs are often found in elderly patients with various forms of dementia, and no one knew about the presence of hydrocephalus when these symptoms were detected. The disease was given the name "Hakim-Adams syndrome", while for some reason the name of Charles Fisher, who was in the list of authors between R. Adams and S. Hakim, was not indicated.

Etiology and pathogenesis

One should not think that this is a common disease and that normotensive hydrocephalus should be suspected in every elderly patient with pelvic disorders: the frequency of its spread does not exceed 4% among people suffering from various forms of dementia. The situation is complicated by the fact that the diagnosis of dementia itself has different criteria, and so far there is no common approach among scientists from different countries.

What is the cause of NG (normotensive hydrocephalus)? In about half of all cases, it was possible to prove that the patient had a history of:

• hemorrhages in the brain (both intraventricular and subarachnoid);

• various traumatic brain injuries;

• meningitis;

• perinatal damage to the central nervous system;

• volumetric formations in the cranial cavity (from castes and aneurysms to tumors);

• congenital anomalies of the cerebrospinal fluid system - atresia, or underdevelopment of the Sylvian aqueduct;

• surgical neurosurgical interventions.

From this list, it is clear that nothing concrete, leading to pathology, was identified - the list turned out to be too "motley", and it should be borne in mind that the doctors were specifically looking for the cause. In 50% of cases, no possible factors were found at all. Given this circumstance, it was agreed to call this condition “idiopathic hydrocephalus”. The situation was saved, and the cause of the Hakim-Adams syndrome could "officially" remain unknown.

Interestingly, without increasing ICP it is impossible to "inflate" the ventricles. How does the paradox of the normal ICP level arise with dilated ventricles? It turned out that episodes of ICP rise exist, but they occur at the same time as Prinzmetal's spontaneous angina pectoris - at night, during REM sleep. The vessels dilate, there is a hyperemia of the brain. The outflow of cerebrospinal fluid is also disturbed, which is of a functional nature, associated with changes in pressure indicators in different parts of the cerebrospinal fluid system. In the daytime, as well as in the phase of slow sleep, this disorder does not occur.

Since the episodes of ICP rise are temporary, and not permanent, the symptoms of "stagnation" are not determined - it simply does not have time to develop.

How is this disease detected?

Symptoms of idiopathic hydrocephalus

Fortunately, the clinical manifestations of the disease are much more definite than the possible causes. This is the "Hakim-Adams triad": at first, the gait is disturbed, later dementia progresses and is diagnosed, and only then do pelvic disorders occur, among which urinary incontinence comes to the fore. Symptoms can be of varying intensity, but variability is acceptable. What are the characteristics of each "triad member"?

Gait disorders

The gait of a patient with NG is uncertain: he overcomes turns poorly, moves in small steps, has difficulty maintaining balance and tries not to raise his legs above the floor plane. This gait is also called "stuck" or "magnetic". Only the movements of the legs are striking, with the hands of the patients everything is in order.

In patients, the step height gradually decreases, it becomes difficult for them to climb the stairs. It is just as difficult to start moving, and to turn, you need to perform several "preparatory" steps. In this case, the patient often falls. Attention is drawn to the fact that the patient can make stereotypical "walking" leg movements, even lying in bed or sitting.

Muscle tone does not determine the development of gait disorders and gait skills (gait apraxia). It can be diffusely decreased and remain physiological or increased in a pyramidal or spastic type.

A characteristic feature that makes it possible to determine the Hakim-Adams syndrome is a sharp improvement in the patient's gait after he undergoes a lumbar puncture and a rather large (20-40 ml) amount of cerebrospinal fluid is removed. This test is called "tap-test". As noted, the removal of cerebrospinal fluid significantly improves the ability to maintain balance while restoring walking.

Some scientists believe that the functions of the hands remain normal because their motor fibers go further from the walls of the lateral ventricles of the brain than the fibers of the legs, and undergo minimal morphological changes.

Dementia

Dementia, or violation of higher cortical functions, manifests itself in this disease in full, usually after gait disorders. But often it precedes them, and in a non-specific form: memory decreases and the speed of mental reaction slows down. Apathy begins. In the future, complacency arises, spontaneity appears, or the urge to any kind of activity decreases. Disorientation in time is observed, and in some patients, cognitive disorders are transformed into psychotic: hallucinations, manic states appear, and even consciousness is impaired by the type of delirium.

Others and relatives notice the symptoms of "emotional atrophy": patients cease to show any feelings. In a severe course of the disease, drowsiness, soporosis and even akinetic mutism may develop. All this leads to the occurrence of bedsores, the addition of a secondary infection and the death of the patient.

There is also a tendency to manifest a "frontal" psyche. The "frontal" nature of the disorders consists in a decrease in self-criticism, the appearance of foolishness, a tendency to flat and "greasy" jokes, as well as a violation of the sequence of actions. So, the patient can first urinate, and then unbutton his pants. Many researchers believe that this is due to the predominant lesion of the anterior horns in the ventricles. As a result, the work of the frontal lobes in depth is disrupted, their associative and commissural connections between themselves and with the corpus callosum are depleted.

Cognitive impairment in Hakim-Adams disease occurs more rapidly than in "classic" dementia, such as Alzheimer's syndrome. So, after 9-12 months from the onset of cognitive disorders, you can get a patient who will constantly need the help of others.

About pelvic disorders

If you carefully question a patient with a complaint of difficulty walking, then already at the onset of the disease, you can identify such phenomena as nocturia (the predominance of nocturnal urination over daytime), as well as dysuric disorders, which are manifested by frequent urination. Then there are imperative urges: the patient urgently needs to go to the toilet, and if there is no suitable place, the urine is simply not kept.

In the future, with the progression of the "frontal psyche", the patient becomes indifferent to both the imperative urge and the later developing urinary incontinence even without any urge. As a rule, patients with normotensive hydrocephalus do not suffer from fecal incontinence. However, this can occur at a later stage, in bedridden patients.

During the "tap-test", it is possible to restore the function of the pelvic organs for a while: the patient begins to retain urine. Such a reaction does not occur with other types of PTO disorders (pelvic organ function).

Can any patient experience symptoms other than the Hakim-Adams triad? The answer is yes, but taken separately, they have no independent diagnostic value, since they speak of a general involution of the central nervous system. It can be pseudobulbar syndrome, grasping and proboscis reflex, some types of tremor.

Diagnostics

It was noted above that the difficulty of detecting the disease is due to the wide spread of the main symptoms in the elderly in general, as well as the insufficient level of medical care for this category of the population.

It is useless to carry out all kinds of routine analyzes, their information content is zero. The main diagnostic criteria are:

• the presence of a supporting "triad";

• detection of signs of internal hydrocephalus on MRI with an increase in the lateral ventricles (their front horns can be 1/3 of the diameter of the skull, and the picture resembles a "butterfly").

External hydrocephalus, especially isolated, with the preservation of the normal size of the lateral ventricles, does not allow the diagnosis of Hakim-Adams syndrome, as well as the presence of thinned and atrophic grooves and convolutions. In normotensive hydrocephalus, the cortex is practically unchanged. On the other hand, in elderly patients it is unlikely that a “perfect” bark can be seen. Usually there are signs of ischemia, the presence of small foci of gliosis, leukoaraiosis. All this speaks of chronic cerebrovascular diseases and does not contradict the diagnosis of Hakim-Adams syndrome.

During the MRI scan, it is necessary to carefully assess the state of the Sylvian water supply system and the outflow of cerebrospinal fluid along it. This will allow you to decide on further treatment tactics if bypass surgery is needed. Therefore, it is preferable to do MRI, rather than CT, since it is on MRI that the structures of the brain are clearly visible.

By "normal picture" is meant the absence of congestion and edema of the optic discs. Everything else may be present according to age and concomitant diseases.

After that, they begin to conduct a lumbar puncture - the main test. The normal pressure of the cerebrospinal fluid is revealed, which does not exceed 180-200 mm of water. Art. It is known that this pressure fluctuates in the norm and is associated with pulse, respiration and blood pressure level - the amplitude of fluctuations is usually 10% of the level of CSF pressure (no more than 20 mm of water column). With normotensive hydrocephalus, the amplitude of such fluctuations is much higher.

An important method of confirming the diagnosis is a "fantastic" study for Russian pensioners - night monitoring of intracranial pressure, carried out in conjunction with polysomnography. If its increase coincides with the phase of REM sleep and regression during wakefulness, the diagnosis of Hakim-Adams syndrome can be justified. Before this study, transcranial Doppler sonography should be performed. It will allow assessing the relationship between the dynamics of pulsation of cerebrospinal fluid pressure and blood flow in the vessels of the brain. This is necessary for the correct interpretation of the results obtained.

The diagnosis is completed by the implementation of the tap-test and other techniques. In particular, the dynamics of the decrease in the level of cerebrospinal fluid pressure after the introduction of endolumbar saline is investigated.

In addition, to assess the degree of dementia, it is necessary to use special tests, scales and neuropsychological surveys, which are "sensitive" to the frontal psyche. If you use the usual short scale, then you may not detect frontal disorders, and this is a standard mistake of neurologists. But there is simply no single methodology that allows one to diagnose the frontal psyche by 100%.

Treatment

Conservative treatment of Hakim-Adams syndrome is carried out by the appointment of "Diacarb" (acetazolamide) and cardiac glycoside digoxin. But the proven effectiveness of this type of therapy has not been identified. The most challenging task is the treatment of urinary disorders in patients. For this, the use of anticholinergic drugs is recommended, in order to at least develop in patients the skill of emptying the bladder at the right time.

But the possibilities of conservative treatment of the disease are limited: non-surgical techniques do not extend the life span and do not lead to an improvement in its quality. Therefore, shunting is the world standard in the treatment of NG. After all, it is the decrease in CSF pressure, despite its normal numbers, that leads to a sharp decrease in symptoms (gait and cognitive functions improve).

Most often, ventriculoperitoneal shunting is used, with a stable positive trend of 60% or more, especially with early surgery after several months of illness.

Complications and prognosis

In the event that a timely surgical intervention is carried out, the risk of complications in elderly patients is 30%. This is a high number, but, as a rule, there is a postoperative hematoma, "sticking" of the ventricles due to a sharp drop in pressure and cerebrospinal fluid hypotension. To eliminate these consequences, it is required to choose the right shunt and calculate the optimal pressure.

Bypass surgery leads to the fact that in 50–70% of all cases, the condition stabilizes, symptoms regress, and the patient is no longer threatened with disability, especially with an early onset of the disease. The prognosis for normotensive hydrocephalus, which is treated conservatively, is dubious, and even unfavorable: within a year, the development of pelvic disorders and the progression of dementia are possible.

In conclusion, it must be said that such a pathology as the Hakim-Adams syndrome is a "litmus test" of the state of health care in the country and attitudes towards old age. If the problem of an elderly person is treated with attention, doctors do not just diagnose dementia, but look for the cause - this is a step towards recovery.

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Morgagni-Adams-Stokes syndrome: causes, signs, diagnosis, help and treatment

Syndrome Morgagni-Adams-Stokes (Morgagni-Edems-Stokes, MAS, MES) is a sudden violation of the heart rhythm, which leads to cardiac arrest, disruption of blood transport to organs and, above all, the brain. Pathology is characterized by sudden fainting, leading to a malfunction of the central nervous system, which manifests itself within seconds after cardiac arrest. An attack of the MAC syndrome can result in clinical death.

According to statistics, up to 70% of patients with permanent complete atrioventricular block have manifestations of the MAC syndrome. In pediatric practice, this syndrome is usually observed in children with atrioventricular block of 2-3 degrees and sick sinus syndrome.

The severity of the manifestations of the MAS syndrome and the frequency of the attack depend on its cause, the initial state of the heart and blood vessels, metabolic changes in the myocardium. In some cases, attacks can be short-lived and go away on their own, but severe arrhythmias and cardiac arrest require urgent resuscitation measures, so such patients need increased attention from cardiologists.

Causes of the MAS syndrome

The conducting system of the heart is represented by nerve fibers, the impulses along which move in a strictly specified direction - from the atria to the ventricles. This ensures the synchronous operation of all chambers of the heart. When there are obstacles in the myocardium (scars, for example), additional conduction beams formed in utero, the mechanisms of contractility are disrupted, and the prerequisites for arrhythmia appear.

example of MAC syndrome due to bradycardia

In children, among the causes of conduction disturbances, there are congenital malformations, disturbances in the intrauterine anlage of the conducting system, in adults - acquired pathology (diffuse or focal cardiosclerosis, electrolyte disturbances, intoxication).

An attack of the MAS syndrome is usually triggered by various factors, including:

• Complete AV block when the impulse from the atria does not reach the ventricles;
• Transformation of an incomplete blockade into a complete one;
• Paroxysmal tachycardia, ventricular fibrillation, when the contractility of the heart muscle drops sharply;
• Tachycardia over 200 and bradycardia below 30 beats per minute.

It is clear that such severe arrhythmias do not arise by themselves, they need a substrate that appears when the myocardium is damaged due to ischemic disease, after a heart attack, or inflammatory processes (myocarditis). A certain role can be played by intoxication with drugs from the group of beta-blockers, cardiac glycosides. Patients with rheumatic diseases (systemic scleroderma, rheumatoid arthritis), when it is likely to involve the heart with inflammation and sclerosis, deserve increased attention.

Depending on the prevailing symptomatology, it is customary to distinguish several variants of the course of the MAS syndrome:

1. Tachyarrhythmic, when the heart rate reaches, the function of ejection of blood into the aorta sharply suffers, the organs experience hypoxia and ischemia.
2. Bradyarrhythmic form - the pulse decreases before a minute, and the cause is usually a complete atrioventricular block, weakness of the sinus node and its complete stop.
3. Mixed type with alternating paroxysms of asystole and tachycardia.

Features of symptoms

With the MAS syndrome, seizures occur suddenly, and they may be preceded by stress, severe nervous tension, fright, and excessive physical activity. A sharp change in body position, when the patient gets up quickly, can also contribute to the manifestation of heart pathology.

Typically, among full health, a characteristic symptom complex of MAC appears, including cardiac disorders and brain dysfunction with loss of consciousness, convulsions, involuntary defecation and urinary excretion.

The main symptom of the disease is loss of consciousness, but in front of it, the patient feels some changes, which he can later tell about. Darkness in the eyes, severe weakness, dizziness and noise in the head indicate an impending fainting. Cold sticky sweat appears on the forehead, a feeling of nausea or lightheadedness appears, possibly a feeling of palpitations or a sinking in the chest.

Seconds after a paroxysm of arrhythmia, the patient loses consciousness, and the signs of the disease are recorded by those around him:

• Lack of consciousness;
• The skin turns pale, cyanosis is possible;
• Breathing is shallow and may stop altogether;
• Blood pressure drops;
• The pulse is threadlike and often cannot be felt at all;
• Convulsive muscle twitching is possible;
• Involuntary emptying of the bladder and rectum.

If the attack lasts for a short time, and the rhythmic contractions of the heart are restored themselves, then consciousness returns, but the patient does not remember what exactly happened to him. With prolonged asystoles lasting up to five minutes or longer, clinical death occurs, acute cerebral ischemia, and emergency measures cannot be dispensed with.

The disease can proceed without loss of consciousness. This is typical of young patients in whom the vascular walls of the cerebral and coronary arteries are not damaged, and the tissues are relatively resistant to hypoxia. The syndrome is manifested by severe weakness, nausea, dizziness with preservation of consciousness.

Elderly patients with atherosclerosis of the arteries of the brain have a worse prognosis, and their seizures are more severe, with a rapid increase in symptoms and a high risk of clinical death, when there is no heartbeat and breathing, pulse and pressure are not detected, the pupils are dilated and do not respond to light.

How to make a correct diagnosis?

In the diagnosis of MAC syndrome, the main importance is given to electrocardiographic techniques - ECG at rest, daily monitoring. To clarify the nature of the pathology of the heart, ultrasound examination, coronary angiography can be prescribed. Of no small importance is auscultation, when the doctor can listen to peculiar noises, an increase in the first tone, the so-called three-member rhythm, etc., but all auscultatory signs are necessarily correlated with the data of electrocardiography.

Since the Morgagni-Adams-Stokes syndrome is a consequence of various kinds of conduction disorders, it does not have electrocardiographic diagnostic criteria as such, and the phenomena on the ECG are associated with the type of arrhythmia that is provoked in a particular patient.

In case of disturbance of conduction from the atrial node on the ECG, first of all, the duration of the PQ interval is assessed, which reflects the time of passage of the pulse along the conducting system from the sinus node to the ventricles of the heart.

With the first degree of blockade, this interval exceeds 0.2 seconds, with the second degree, the interval gradually lengthens or exceeds the norm in all cardiac complexes, while the QRST periodically drops out, which indicates that the next impulse simply did not reach the ventricular myocardium. At the third, most severe degree of blockade, the atria and ventricles contract by themselves, the number of ventricular complexes does not correspond to the P waves, that is, the impulses from the sinus node do not reach the end point in the conductive fibers of the ventricles.

variety of arrhythmias that cause MAC syndrome

Tachycardia and bradycardia are established based on counting the number of cardiac contractions, and ventricular fibrillation is accompanied by a complete absence of normal teeth, intervals and ventricular complex on the ECG.

Treatment of the MAC syndrome

Since the MAC syndrome is manifested by sudden attacks of loss of consciousness and cerebral dysfunction, the patient may require emergency care. It often happens that a person falls down and loses consciousness in a public place or at home in the presence of relatives, then the latter should immediately call an ambulance and try to provide first aid.

Of course, others may be confused, not know where to start resuscitation, how to properly carry it out, but in case of sudden cardiac arrest, the count goes for minutes, and the patient may die right in front of eyewitnesses, so in such cases it is better to do at least something for saving a person's life, because delay and inaction cost lives.

1. Precardiac beat.
2. Indirect cardiac massage.
3. Artificial respiration.

Most of us have heard of cardiopulmonary resuscitation techniques in one way or another, but not everyone knows these skills. When you are not confident in your skills, you can limit yourself to pressing on the chest (about 2 times a second) before the ambulance arrives. If the resuscitator has already encountered such manipulations and knows how to do them correctly, then for every 30 clicks he performs 2 mouth-to-mouth exhalations.

A precordial blow is an intense fist thrust into the lower third of the sternum that often helps restore electrical activity to the heart. A person who has never done this should be careful, because a strong blow of a fist, especially a man's, can cause rib fractures and soft tissue bruises. Also, this technique is not recommended for young children.

Indirect cardiac massage and artificial respiration can be done alone or with a partner, the latter is easier and more effective. In the first case, there are 2 exhalations for 30 pressings, in the second - one exhalation of pressing on the chest.

The ambulance team in cardiac arrest will continue emergency care, supplementing it with medication support. To restore the heart rhythm, electrocardiostimulation is performed, and if it is impossible to implement it, adrenaline is injected intracardiacally or into the trachea.

In order to restore the conduction of impulses from the atria to the ventricles, atropine is indicated intravenously or subcutaneously, the administration of which is repeated every 1-2 hours due to the short duration of the drug. As the patient's condition improves, he is given izadrin under the tongue and transported to a cardiological hospital. If atropine and izadrin do not have the expected result, then orciprenaline or ephedrine is administered intravenously under strict control of the heart rate.

In the bradyarrhythmic form of MAC, treatment includes temporary cardiac stimulation and the introduction of atropine, in the absence of the effect of which aminophylline is indicated. If after these drugs the result is negative, dopamine, adrenaline are administered. After stabilization of the patient's condition, the issue of continuous cardiac stimulation is considered.

The tachyarrhythmic form requires the elimination of ventricular fibrillation by means of electrical pulse therapy. If tachycardia is associated with the presence of additional conduction pathways in the myocardium, then the patient will need further surgery to cross them. With ventricular tachycardia, a cardioverter pacemaker is installed.

In order to avoid attacks of cardiac arrest, patients with MAC syndrome are prescribed prophylactic antiarrhythmic therapy, including drugs such as flecainide, propranolol, verapamil, amiodarone, etc. (prescribed by a cardiologist!).

If conservative treatment with antiarrhythmics does not work, there is a high risk of complete atrioventricular blockade and cardiac arrest, then pacing is shown with the installation of a special device that supports the heart and at the right time gives it the necessary impulse to contract.

The pacemaker can work continuously or "on demand", and its type is selected individually based on the characteristics of the course of the disease. With a complete blockade of impulses from the atria to the ventricles, it is advisable to use a pacemaker that works continuously, and if the heart is relatively safe, it is possible to recommend a device operating in the "on demand" mode.

Morgagni-Adams-Stokes syndrome is a dangerous pathology. Sudden attacks of loss of consciousness and the likelihood of clinical death require timely diagnosis, treatment and observation. Patients with MAC syndrome should regularly come for examination to a cardiologist and follow all his recommendations. The prognosis depends on the type of arrhythmia and the frequency of cardiac arrest, and timely implantation of a pacemaker significantly improves it and allows the patient to prolong life and relieve asystole attacks.

The classic triad of normotensive hydrocephalus: dementia, urinary incontinence and gait disturbances. The disease is accompanied by expansion of the ventricles of the brain, but atrophy of the cortex is absent or minimal.

Normotensive hydrocephalus develops due to impaired circulation or absorption of CSF. CSF pressure during lumbar puncture does not exceed the upper limit of normal. Inflammatory disease, such as meningitis or encephalitis, or subarachnoid hemorrhage, may be the cause. However, in most cases, the etiology is not known.

Dementia in normotensive hydrocephalus has no characteristic features. CT or MRI detects ventricular enlargement in the absence of significant cortical atrophy, signs of CSF leakage into the periventricular white matter, balloon-like expansion of the lateral ventricular horns. MRI describes the disappearance of the signal from the third ventricle due to the movement of the CSF. Isotope cisternography does not register CSF flow in the subarachnoid space of the upper lateral surface of the hemispheres. Removing 20-30 ml of CSF results in a temporary improvement in cognitive function and gait. After a lumbar puncture, cognitive function and gait are examined every few days. Improvement after extraction of large amounts of CSF is a favorable prognostic sign that suggests a good effect of lumboperitoneal shunting. However, it is impossible to accurately predict the outcome of this operation. In this regard, the decision on the operation is made jointly by the neurosurgeon, the patient and his family members.

Prof. D. Nobel