When doctors talk about hydrocephalus, almost always the leading mechanism for 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 such symptoms as a progressive decrease in vision, bursting headaches and vomiting. In the event of a sudden obstruction to the flow of CSF, occlusive hydrocephalus occurs, and then the brain may begin to swell and be infringed.

These types of hydrocephalus are described in detail. But it turns out that there is also a form of the disease that manifests itself with 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 can this disease be cured?

Definition

This is the name of the 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 true: it was not until 1965 that the first generalized data were published in The New England Journal of Medicine. The authors of the material were R. Adams, S. Hakim and C. Fisher. Since no name has yet been coined for this condition, they described "occult hydrocephalus in adults with a chronic course without changes in the fundus and with normal CSF pressure."

Thus, a hydrocephalus syndrome was considered, 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 condition was described, in which not only was the fundus normal, but no characteristic complaints were observed - the flow 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 the diagnosis of normotensive hydrocephalus is made. According to the 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 the mentioned symptoms were detected. The name “Hakim-Adams syndrome” was given to the disease, while for some reason the name of C. Fisher was not indicated, which was between R. Adams and S. Hakim in the list of authors.

Etiology and pathogenesis

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

What is the cause of NH (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 craniocerebral injuries;

• meningitis;

• perinatal lesion of 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;

• operative neurosurgical interventions.

It is clear from this list that nothing specific, leading to pathology, was identified - the list turned out to be too “variegated”, and it must be taken into account that the doctors were specifically looking for the cause. In 50% of cases, no possible factors were found at all. Given this circumstance, they 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 an increase in ICP, it is impossible to "inflate" the ventricles. How does the paradox arise? normal level ICP with dilated ventricles? It turned out that episodes of ICP rise exist, but they occur at the same time as Prinzmetal's spontaneous angina - at night, during REM sleep. The vessels dilate, hyperemia of the brain occurs. The outflow of cerebrospinal fluid, which wears functional character associated with a change in pressure indicators in different parts of the CSF system of the brain. In the daytime, as well as in the phase of slow sleep, this violation does not occur.

Since the episodes of the rise in ICP are temporary, and not permanent, then 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 possible reasons. This is the “Hakim-Adams triad”: first, gait is disturbed, later dementia progresses and its diagnosis is made, and only then pelvic disorders occur, among which urinary incontinence comes to the fore. Symptoms can be expressed with varying intensity, but variability is quite acceptable. What are the characteristics of each "member of the triad"?

Gait disorders

The gait of a patient with NG is uncertain: he overcomes turns poorly, moves in small steps, hardly maintains balance and tries not to raise his legs above the floor plane. Such a 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, step height gradually decreases, it becomes difficult for them to climb stairs. It is just as difficult to start moving, and in order to turn, you need to perform several “preparatory” actions. In this case, the patient often falls. It is noteworthy that the patient can perform stereotypical "walking" movements with his legs, even while lying in bed or sitting.

Muscle tone does not determine the development of gait disturbance and its skills (gait apraxia). It can be diffusely reduced and remain physiological or elevated 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 is given a lumbar puncture and a rather large (20-40 ml) amount of CSF is removed. This test is called "tap-test". As noted, the removal of cerebrospinal fluid significantly improves the ability to keep balance, and at the same time walking is restored.

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 a violation of higher cortical functions, is fully manifested in this disease, usually after gait disorders. But often it precedes them, and in a non-specific form: memory decreases and the rate of mental reaction slows down. Apathy sets in. In the future, complacency arises, aspontaneity appears, or the impulse to any kind of activity decreases. Disorientation in time is observed, and in some patients, cognitive disorders transform into psychotic ones: hallucinations, a manic state appear, and even consciousness is disturbed by the type of delirium.

Others and relatives notice symptoms of "emotional atrophy": patients stop showing any feelings. At severe course disease, drowsiness, a soporous state can begin, and even akinetic mutism can 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 the manifestation of the "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, work is disrupted frontal lobes in depth, their associative and commissural connections between themselves and with the corpus callosum are depleted.

Cognitive impairment in Hakim-Adams disease occurs faster 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 the patient with a complaint of difficulty in gait, then already at the beginning of the disease, phenomena such as nocturia (the predominance of nighttime urination over daytime), as well as dysuric disorders, which are manifested by frequent urination, can be identified. 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 retained.

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

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

Can any symptoms occur in patients 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. This may be pseudobulbar syndrome, grasping and proboscis reflex, some types of tremor.

Diagnostics

It was noted above that the difficulty in 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 anterior horns can be 1/3 of the diameter of the skull, and the picture resembles a "butterfly").

External hydrocephalus, especially isolated, with preservation normal size lateral ventricles, does not allow the diagnosis of "Hakim-Adams syndrome", as well as the presence of thinned and atrophic sulci and convolutions. In normotensive hydrocephalus, the cortex is practically unchanged. On the other hand, in older patients one can hardly see the "ideal" cortex. Usually there are signs of ischemia, the presence of small foci of gliosis, leukoaraiosis. All this indicates chronic cerebrovascular diseases and does not contradict the diagnosis of Hakim-Adams syndrome.

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

• Normal picture on the fundus.

Under the "normal picture" is meant the absence of congestion and swelling 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. Normal cerebrospinal fluid pressure is detected, which does not exceed 180–200 mm of water. Art. This pressure is known to fluctuate normally and is related to pulse, respiration and blood pressure. blood pressure- the amplitude of fluctuations is usually 10% of the level of cerebrospinal fluid pressure (no more than 20 mm of water column). In normotensive hydrocephalus, the amplitude of such fluctuations is much higher.

An important method of confirming the diagnosis is a “fantastic” study for domestic 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, it is possible to make a diagnosis of Hakim-Adams syndrome with full right. Before this study, transcranial dopplerography should be performed. It will allow to evaluate the relationship between the dynamics of CSF pressure pulsation and blood flow in the vessels of the brain. This is necessary for the correct interpretation of the results.

The diagnostics is completed by the implementation of a tap-test and other methods. In particular, the dynamics of the decrease in the level of cerebrospinal fluid pressure after the introduction of endolumbar saline is studied.

In addition, to assess the degree of dementia, it is necessary to use special tests, scales and neuropsychological surveys that are “sensitive” specifically to the frontal psyche. If you use the usual short scale, you can not detect frontal disorders, and this is a standard error of neurologists. But there is simply no single technique that allows 100% to diagnose the frontal psyche.

Treatment

Conservative treatment of Hakim-Adams syndrome is carried out by prescribing Diakarba (acetazolamide) and digoxin cardiac glycoside. But the proven effectiveness of this type of therapy has not been identified. The most difficult 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 conservative treatment diseases are limited: non-surgical methods do not prolong life and do not improve its quality. Therefore, shunting is the world standard in the treatment of NG. After all, it is the decrease in cerebrospinal fluid pressure, despite its normal numbers, that leads to sharp decrease symptoms (improves gait and cognitive function).

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

Complications and prognosis

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

Shunting leads to the fact that in 50-70% of all cases the condition stabilizes, the symptoms regress, and the patient is no longer in danger of disability, especially with an early onset of the disease. The prognosis for normotensive hydrocephalus, which is treated conservatively, is doubtful, 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.

INTERNATIONAL NEUROLOGICAL JOURNAL

INTERNATIONAL NEUROLOGICAL JOURNAL 1

INTERNATIONAL NEUROLOGICAL JOURNAL

UDC 616.831.38-008.811.1(048.8)

IVANOVA M.F12, YEVTUSHENKO S.K.2, SEMENOVA A.V.1, SAVCHENKO E.A.2

1 Institute of Gerontology D.F. Chebotareva NAMS of Ukraine, Kyiv

2 Institute of Emergency and Reconstructive Surgery. VC. Gusak NAMS of Ukraine

NORMOTENSIVE HYDROCEPHALIA OF HAKIM - ADAMS (SCIENTIFIC REVIEW AND PERSONAL OBSERVATION)

Summary. The review reflects the modern interpretation of the term "normotensive hydrocephalus", data on the etiology, pathogenesis, features clinical manifestations, diagnostics. The approaches of neurosurgical and conservative treatment are described. Presented personal observation clinical case verified normotensive hydrocephalus (Hakim-Adams syndrome), which was characterized by the young age of the patient, favorable clinical and neuroradiological course of the disease after neurosurgical and drug treatment. According to the literature, this pathology is most common in the elderly and can be the cause of dementia, impaired walking, and pelvic organ function. The paper substantiates the importance of further study of normotensive hydrocephalus in clinical practice.

Key words: normotensive hydrocephalus, Hakim-Adams syndrome, clinical case, young age, diagnosis, treatment.

Hakim-Adams syndrome, or normotensive hydrocephalus (NH), is characterized by a chronic disorder of liquorodynamics, expansion of the ventricular system without a significant increase in intracranial pressure (ICP), clinically manifested by the classic triad of symptoms: gait disturbance, dementia and pelvic disorders.

NG was first described as a syndrome by the Colombian neurosurgeon S. Hakim in 1957. In the English 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 of post-traumatic and one of idiopathic genesis.

Currently, there are primary (idiopathic) and secondary (symptomatic)

NG. Secondary NG most often develops after subarachnoid hemorrhage (23%), meningitis (4.5%), traumatic brain injury (12.5%), but there may be other causes.

The term "idiopathic" implies that the etiology of NC is definitively unknown. To date, there is no consensus regarding the risk factors for the disease, pathogenesis.

Ivanova Marina Fedorovna

04114, Kyiv, Vyshhorodska, 67

State Institution “Institute of Gerontology named after D.F. Chebotareva

NAMS of Ukraine"

Email: [email protected]

© Ivanova M.F., Evtushenko S.K., Semenova A.V.,

Savchenko E.A., 2016 © International Neurological Journal, 2016 © Zaslavsky A.Yu., 2016

tic mechanisms of development of ventricular dilatation and concomitant damage to the surrounding medulla.

The available epidemiological data remain conflicting, in part because of differences in accepted diagnostic criteria and a paucity of population-based studies. Despite this, it can be assumed that the number of cases of primary and secondary NC will increase, most likely due to an increase in the quality of medical care, a wider introduction of neuroimaging examination methods and an increase in life expectancy. According to modern authors, the ratio between idiopathic and symptomatic forms is approximately 1: 1. Currently, NG is more often considered as a disease of older people aged 60-80 years, although the literature describes cases of its occurrence in middle and even childhood.

A characteristic manifestation of NG is the Hakim-Adams triad, which includes dementia, impaired walking and pelvic organ function. 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%), one of the 3 symptoms is less common, and, as a rule, it is 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 gradual activation of muscle groups, which should be considered as a disorder of the subcortical movement control systems, and not as a primary disorder of the pyramidal tract. As NG progresses, the involvement of the corticospinal pathway becomes more pronounced. The gait is described as apraxic, bradykinetic, magnetic, parkinsonian. Patients may also have difficulty turning. With NG, there are no changes in the friendliness of hand movements when walking.

Cognitive disorders are characterized by a slowdown in psychomotor functions, impaired ability to concentrate, and mild memory impairment. Such changes are often associated with dysfunction of the anterior parts of the brain and are quite characteristic of the so-called subcortical dementia. Primary disorders of higher brain functions (aphasia, apraxia, agnosia, acalculia), as well as psychoses, are rare. However, some patients may experience confabulation, hallucinations, mania, delirium, depression, and epileptic seizures. Important to remember,

that such a well-known classical picture of hydrocephalus as headache, nausea, vomiting, visual impairment is not typical for patients with NG. When examining the fundus, there is no edema of the optic discs.

Pelvic organ dysfunction debuts with an increase in the frequency of urination, especially at night, with the gradual development of urinary incontinence. Pelvic disorders in NG are referred to the frontal type of urination disorders, which is characterized by the absence of urges, the patient's indifferent attitude to the fact of involuntary urination. Fecal incontinence is rare, usually in patients with advanced stages of the disease.

In 2002, an international research group for the study of idiopathic NC (iNC) was established, which in 2005 published a guideline. According to international criteria, based on the history, clinic, neurological examination, physiological criteria, as well as the results of neuroimaging, probable, possible and doubtful iNH are distinguished. However, in 80% of cases, iNH remains undiagnosed in a timely manner; in clinical practice, it is similar to manifestations in a number of neurodegenerative diseases. The above symptoms can also be associated with vascular dementia, other types of hydrocephalus, infectious diseases, etc. Some of these diseases are presented below.

Diseases that require differential diagnosis with normotensive hydrocephalus

Vascular dementia:

cerebrovascular disease.

Stroke.

Multi-infarct condition.

Binswanger's disease.

Cerebral autosomal dominant arteriopathy, subcortical infarcts and leukoencephalopathy.

Vertebrobasilar insufficiency.

Other types of hydrocephalus:

Stenosis of the aqueduct.

Noncommunicating hydrocephalus.

Neurodegenerative diseases:

Alzheimer's disease.

Parkinson's disease.

Dementia with Lewy bodies.

Huntington's disease.

Frontotemporal dementia.

Corticobasal degeneration.

Progressive supranuclear palsy.

Lateral amyotrophic sclerosis.

Multisystem atrophy.

Creutzfeldt-Jakob disease.

Infectious diseases:

Lyme disease.

Syphilis.

Urological disorders:

Infectious diseases of the genitourinary system.

Cancer of the prostate or bladder.

Hyperplasia of the prostate.

Other reasons:

Vitamin B12 deficiency.

Systemic connective tissue diseases.

Epilepsy.

Depression.

Traumatic brain injury.

Spinal stenosis.

Chiari malformation.

Encephalopathy Wernicke.

Carcinomatous meningitis.

Tumors of the spinal cord.

There are a number of additional tests that can improve the accuracy of diagnosis and predict the effectiveness of further neurosurgical treatment. They are recommended for all patients with probable and possible iNH according to international criteria. TO additional methods include: tap-test (tap-test), external lumbar drainage and measurement of resistance to resorption of cerebrospinal fluid, which are usually carried out in specialized neurosurgical clinics. The choice of technique depends on many factors, including predictive value, personal experience, availability of equipment and personnel .

In ICD-10, hydrocephalus of normal pressure has a code - G 91.2. When formulating a detailed diagnosis, one should indicate the course (rapidly progressing, slowly progressing, stationary) or the degree of compensation after bypass surgery (complete, partial).

In Ukraine, by order of the Ministry of Health of Ukraine dated June 13, 2008 No. 317, clinical protocols providing medical care to patients with hydrocephalus in the specialty "neurosurgery".

Neurosurgical treatments for iNH are similar to those for other forms of hydrocephalus, using ventriculoperitoneal (VP), ventriculoatrial (VA), and lumboperitoneal (LP) shunts.

Experimental studies have shown that shunting leads to changes in regional blood flow, liquorodynamics and brain metabolism. Carefully selected patients for

neurosurgical interventions in the future may have positive long-term results of treatment, especially if they are further prophylactically monitored by neurosurgeons who closely monitor the serviceability of the shunt device. To date, various options for shunt devices are available and continue to be improved.

An individual selection of a shunt device for each patient is recommended. The main postoperative complications include: subdural hematoma (from 2 to 17%), infections (from 3 to 6%), seizures (from 3 to 11%), the need for shunt revision (21%).

The clinical effect after neurosurgical treatment in patients with iNH can be stable for 5-7 years. Some patients improve already in the early postoperative period, some require up to 6 months to achieve a significant clinical effect. Two to three postoperative visits are reasonable in the first year after bypass surgery. As a rule, most complications occur during this period. In addition to the routine examination, a CT or MRI examination of the brain must be carried out during the first year. Further choice of the frequency of neuroimaging is determined individually, taking into account the profile of possible risk or with significant clinical deterioration. Patients who develop symptoms some time after shunting deserve close attention, as this may be a sign of shunt obstruction or other causes.

Approaches to drug management of the patient in the preoperative and postoperative periods require further development.

We present a personal observation.

Patient K., 35 years old, an engineer by profession working at a metallurgical plant, on April 3, 2013, applied for medical care for a consultative appointment with a neurologist of the State Institution “Institute of Emergency and Reconstructive Surgery named after A.I. VC. Gusak of the National Academy of Medical Sciences of Ukraine" with complaints of heaviness in the legs, unsteadiness, change in gait. According to the patient, his condition worsened sharply on March 2, 2013, when he noted weakness in his legs, because of which he fell, for some time he could not get up on his own, at the insistence of his parents, who noticed a change in gait, accompanied by his father, he sought medical help. From the addition of the patient's father: a change in gait began to be noticed in his son for the last 6-8 months. On the eve of the deterioration of the condition - a traumatic everyday situation.

In the early history, according to the mother (medical documents are not presented), he was born full-term, with asphyxia, from 1988 to 1993 he was observed in

pediatric neurologist at the place of residence with a diagnosis of "episindrome" (there were three attacks with loss of consciousness, urinary incontinence), took lamictal. At the same time, a spiral computed tomography (SCT) of the brain was performed, which allegedly revealed the expansion of the ventricles of the brain. In 1993 removed from the "D"-registration. Subsequently, the neurologist was not observed, he felt satisfactory. Periodically for the last few years, with excitement, he noted trembling of the fingers.

In the neurological status on examination: consciousness is clear. Attention is somewhat scattered, criticism of one's condition is reduced, associative processes are slowed down. Eye fissures - D > S, pupils - D = S. Photoreactions are saved. There is no nystagmus. Tongue in the midline. Swallowing, phonation, speech are not disturbed. The left corner of the mouth is lowered. Tendon reflexes from the hands are high: D > S, knee reflexes are high: D > S, Achilles reflexes are with a polykinetic response. Abdominal reflexes: upper live, middle and lower are reduced. Convincing disorders of pain sensitivity does not show, vibration sensitivity up to 12 s in the legs. MSC is not violated. In the Romberg position, unsteadiness, intention, dysmetria when performing PNP on both sides. Slight ataxia on walking. The gait is apraxic (some flexion of the upper body forward, combined with flexion of the arms and knees, the automatic swinging of the upper limbs is slightly reduced, the legs are somewhat apart). Symptom Shtryumpelya, Babinsky on both sides. There are no meningeal signs. The tone in the limbs is somewhat reduced, there are no paresis.

In connection with the identified complaints, changes in the neurological status, an examination was prescribed, incl. MRI of the brain and cervical spinal cord. When conducting general clinical analyzes ( general analysis blood, urinalysis, blood sugar), blood test for RW, FLG, ECG, ultrasound thyroid gland no pathology was found. Examined by an ophthalmologist - there is no pathology in the fundus.

On April 4, 2013, MRI of the cervical spine was performed: osteochondrosis of the cervical spine with protrusion of C5-C6, C6-C7 intervertebral discs. Spondylarthrosis.

On April 4, 2013, an MRI of the brain, MR angiography (Philips Panorama, MRI 0.23 T) was performed (Fig. 1) - internal hydrocephalus was detected with a probable block at the level of the Sylvian aqueduct, with the presence of demyelination zones in the white matter of the parietal areas, periventricular to the dorsal parts of the bodies of the lateral ventricles on both sides. An MRI with intravenous contrast was recommended. MRI signs of changes in blood flow were not detected at the time of the study.

On April 8, 2013, MRI of the brain was repeated, MR angiography with intravenous administration

Figure 1. MRI of the brain of patient K. dated April 4, 2013.

tomovista 20 ml. After intravenous administration contrast agent pathological change the intensity of the magnetic resonance signal in the brain tissue was not detected. Recommended MRI control after 1-3 months. according to indications, consultation of a neurosurgeon.

In connection with the revealed changes on MRI of the brain, with a preliminary diagnosis of "normotensive hydrocephalus (Hakim-Adams syndrome)" on 04/09/13, he was consulted by a neurosurgeon of the Donetsk Regional Medical and Clinical Association, hospitalized in the neurosurgical department, where he was from 04/10/13 to 27/04 .13 (case history No. 10250).

On April 10, 2015, the patient was re-examined by an ophthalmologist in the department - the optic disc was not changed, the boundaries were clear, the veins were narrowed, and the arteries were of normal caliber.

On April 11, 2013, a lumbar puncture was performed: a colorless transparent cerebrospinal fluid was obtained under pressure in the sitting position of 450 mm of water column, in the prone position - 130 mm of water column. When conducting liquorodynamic samples, there is no block. Composition of liquo-ra - 1st portion: cytosis - 1 μl, protein - 0.15 g/l, Pandy reaction - positive, 2nd portion: cytosis - 1 μl, protein - 0.11 g/l, Pandey's reaction - posit.

April 16, 2013 - EEG: significant EEG disturbances at rest due to a decrease in the influence of non-specific regulatory systems of the upper brainstem level (removal of the influence of the trunk reticular formation on thalamic and cortical structures), signs of damage to diencephalic formations, mainly the thalamus, with severe hyperactivation of these structures, signs of pronounced violations

Figure 2. CT scan of the brain of patient K. dated April 17, 2013 - control after surgery

Figure 3. CT scan of the brain of patient K. dated April 25, 2013 - control before discharge from the neurosurgical department

intrathalamic internuclear interactions, signs of moderate irritation of the bottom of the third ventricle and a decrease in the threshold of paroxysmal readiness.

04/16/13 - MRI of the brain (Gyroscan T10, Philips, Holland, MRI 1.0 T): there is a pronounced decrease in differentiation between white and gray matter brain, subshell spaces are not traced throughout. No additional formations were identified. The pituitary gland and brain stem sections are not changed. The cerebellar tonsils are located at the entrance to the foramen magnum. Median structures are not displaced. There is a pronounced expansion of the third ventricle up to 4.5 cm, lateral ventricles - up to 4.5 cm at the level of bodies. The size of the fourth ventricle is not changed. Sylvius aqueduct traced

fragments, narrowed. Around the lateral ventricles there are zones up to 0.5 cm wide with an increase in the MR signal in T2 I/I and FLAIR.

On April 17, 2013, an operation was performed (Ph.D., Associate Professor Korovka S.Ya.): a low-pressure ventriculo-peritoneal shunt (VPS) was installed through the posterior horn of the right lateral ventricle. During the operation, ventricular cerebrospinal fluid was obtained: cytosis - 0, protein - 0.03 g/l, Pandy's reaction - negative.

On April 17, 2013, CT scan of the brain was performed after the operation (Fig. 2): there is a burr hole in the occipital region on the right, in the projection of which a shunt is visualized, which can be traced in the body of the right lateral ventricle. A small amount of air in the cisterns of the basal group, in the subarachnoid spaces under the bone in the temporal and frontal regions, mainly on the right. Median structures are not displaced. Lateral ventricles at the level of bodies - 3.5 cm, anterior horns on the right - 1.2 cm, on the left - 1.4 cm, III ventricle - 1.4 cm, IV ventricle - 1.2 x 0.4 cm. 1.35 cm temporal horns. Subarachnoid spaces are not visible on both sides. The paranasal sinuses are sufficiently pneumatized.

received in the department drug therapy: dexalgin, dexamethasone, medaxon, omez, fluconazole, erius, keltikan, citicoline, actovegin.

During the stay in the department, the patient's condition improved. In the neurological status - the headache does not bother, walking has improved, unsteadiness is less pronounced, heaviness in the legs, there is no nystagmus, the tongue is in the midline, swallowing, phonation, speech is not impaired, the left corner of the mouth is lowered, tendon reflexes from the hands are high - D\u003e S , knee high - D > S, Achilles - with a polykinetic response. There are no meningeal signs. Samples for paresis are negative. Walks on his own. Vital indicators are stable, the sutures are removed, the wound healed by primary intention. On April 25, 2013, the CT scan of the brain was monitored (Fig. 3). On April 27, 2013, the patient was discharged from the department in a satisfactory condition with a diagnosis of normotensive hydrocephalus; surgical treatment April 17, 2013. Further treatment and follow-up by a neurologist, MRI of the brain after 4-6 months were recommended.

After being discharged from the neurosurgical department, he received inpatient treatment at the Department of Reconstructive Angioneurology and Neurorehabilitation of the State Institution “INVKh im. VC. Gusak NAMNU" from 04/29/13 to 05/17/13 (case history No. 6378) with a diagnosis of normotensive hydrocephalus (Hakim-Adams syndrome), condition after surgical treatment (04/17/13 - installation of the VPSH at low pressure), with tetrapyramidal reflex insufficiency, transient paresis in the legs (03/23/13),

static-locomotor ataxia, easy apraxia of walking, stage of regression of symptoms on a residual background (asphyxia during childbirth, episyndrome in childhood according to anamnesis).

In the department he received drug therapy: gliatilin, neurorubin, actovegin, neuromedin, nucleo CMF, citicoline.

Discharged from the department with ongoing subjective and objective (headache does not bother, no apraxia, ataxia when walking) improvement for outpatient treatment under the supervision of a neurologist at the place of residence.

Subsequently, on an outpatient basis, he continued to receive neurometabolic drugs, neuroprotectors, vitamins of group B.

During the period of outpatient treatment (according to the sick leave), the condition remained stable, subjectively continued to notice an improvement, did not complain, refused to register for MSEK, and was discharged for work.

In dynamics, during the follow-up observation of patient K., MRI of the brain was monitored: on December 11, 2013 (Fig. 4) - 10 months after surgical treatment, on December 13, 2014 (photo 5) - after 22 months.

MRI of the brain dated 12/11/13 (0.23 T): condition after VPSH surgery (04/17/13 operation) with the presence of periventricular zones of demyelination. Expansion of subarachnoid spaces. Moderate expansion of the lateral ventricles.

Brain MRI dated December 13, 2014 (Gyroscan T10, Philips, Holland, MRI 1.0T): on a series of MR tomograms of the brain in axial, frontal and sagittal projections in T1- and T2-weighted images in the occipital region on the right, a shunt is visualized, which can be traced to the anterior part of the body of the right lateral ventricle. Periventricularly on both sides, pronounced demyelination zones up to 0.6-0.8 cm wide are visualized, having an increased MR signal in T2 I/I and with FLAIR. In addition, foci with a similar MRI signal up to 0.5 cm in size are determined in the corpus callosum. The pituitary gland and brain stem regions are not changed. Median structures are not displaced. The expansion of the third ventricle is determined up to 0.65 cm. The bodies of the lateral ventricles are 0.9 cm. lateral fissures. On MR angiograms of the cerebral arteries, the tortuosity of both internal carotid arteries is noted, the MR signal from the right vertebral artery is relatively reduced, and the artery itself is somewhat narrower than the left one. No other pathological altered vessels were found: their contours are even, clear, the caliber is not changed.

At the same time, for the period of outpatient observation from May 2013 to December 2014, periodically

(spring, autumn) received neurometabolic drugs, neuroprotectors, B vitamins.

At the time of writing, the patient's condition is stable, he does not complain, he continues to work in his specialty for same place work.

Figure 4. Control MRI of the brain of patient K. dated 12/11/13, 10 months after surgical treatment

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Figure 5. Control MRI of the brain of patient K. dated December 13, 2014, 22 months after surgical treatment

The peculiarity of the presented clinical case is the young age of the patient, timely early diagnosis of NG, positive clinical and neuroradiological dynamics (according to MRI studies of the brain, conducted during follow-up of the patient in follow-up) after surgical neurosurgical and restorative drug treatment.

In this regard, further study of this problem remains relevant, since the true prevalence of NC in the population, especially among young people, is unknown. A multidisciplinary approach, and above all the coordinated interaction of a neurologist, radiologist, neurosurgeon, significantly affects the quality of care for patients with NG, its timely diagnosis, the possible positive effect of neurosurgical and drug treatment, helps prevent the progression of the disease, subsequent disability, maintain professional, social significance of the patient and his quality of life.

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Received 03/13/15 U

Ivanova M.F.1,2, Svtushenko C.K.2, Semyonova O.V.1, Savchenko O.A.2

11Institute of Gerontology A.F. Chebotaryova NAMS of Ukraine, m. Ki!v

21Institute guilty! xipypri "i iM. B.K. Husak NAMS of Ukraine

NORMOTENSIVE PROCEPTION XAKiMA - ADAMSA (SCIENTIFIC REVIEW i SPECIAL CARE)

Summary. In a glance, the current interpretation of the term “normotensive pdrocephalus”, dates about the etiology, pathogenesis, especially the clinical manifestations, and diagnostics are shown. Described are neurosurgical and conservative lies. A special guard has been put in place for a klshchny vipadka verified! normotensive! pdrocephalus (Chaim-Adams syndrome), especially a bully of a young adult patient, a sympathetic klshchniy and a neuroradio- lopical malady for neurosurgical and drug-induced deceit. Zgshchno with false denim, tsya pathology is most often seen in the osb of a frail eye and can be the cause of dementsp, impaired walking and the functioning of the pelvic organs. At the robe, the importance of the submitted vivechennya is primed normotensively! g1drocepha^y in clinical practice.

Key words: normotensive hydrocephalus, Hayum-Adams syndrome, clinical depression, young adults, diagnostics, exuberance.

Ivanovo M.F.12, Yevtushenko S.K.2, Semionova O.V.1, Savchenko O.A.2

11nstitute of Gerontology named after D.F. Chebotariov of National Academy of Medical Sciences of Ukraine, Kyiv

2 Institute of Urgent and Reconstructive Surgery named after V.K. Husak of National Academy of Medical Sciences of Ukraine, Ukraine

NORMAL PRESSURE HYDROCEPHALUS OR HAKIM-ADAMS SYNDROME (SCIENTIFIC REVIEW AND CLINICAL CASE REPORT)

summary. The review presents the modern interpretation of the term "normal pressure hydrocephalus", data on the etiology, pathogenesis, clinical manifestations, diagnosis. The approaches to neurosurgical and conservative treatment are described. A clinical observation of verified normal pressure hydrocephalus (Hakim-Adams syndrome) is presented, its feature was the young age of a patient, favorable clinical and neuroradiological course of the disease after neurosurgical and medical treatment. According to the literature data, this pathology is most common in the elderly and may be a cause of dementia, walking disorders and pelvic organ function. The work proved the importance offurther study ofnormal pressure hydrocephalus in clinical practice.

Key words: normal pressure hydrocephalus, Hakim-Adams syndrome, clinical case, young age, diagnosis, treatment.

Normotensive hydrocephalus (NTH, Hakim-Adams syndrome) is a syndrome characterized by a combination of dementia, walking disorders and urinary incontinence with marked expansion of the ventricular system and normal CSF pressure. The prevalence of NTH is low, it is detected in 1-5% of patients with dementia. For the first time NTG as an independent disease was described by S. Hakim and R.D. Adams. In 1965, they published articles on "symptomatic occult chronic hydrocephalus in adults with normal fundus", or "hydrocephalus with normal CSF pressure". The authors paid special attention to the possible reversibility of the clinical manifestations of this syndrome with adequate treatment.

Causes of normotensive hydrocephalus The development of the disease is based on an imbalance between the secretion and resorption of CSF, as well as a violation of liquorodynamics. NTG in adults can be induced by various causes:

• hemorrhage in the brain
• purulent-inflammatory process in the cranial cavity,
• perinatal lesions of the brain and meninges,
• volumetric intracranial formations,
• anomalies in the development of the brain (the most common cause is atresia of the Sylvian aqueduct),
• undergone brain surgery.

Approximately 40-60% of cases in the anamnesis of patients with Hakim-Adams syndrome do not indicate any clear cause underlying the development of the disease (the so-called idiopathic IGT).

Clinical manifestations of Hakim-Adams syndrome

NTG is characterized by the gradual development of the Hakim-Adams triad - dementia, walking disorders and urinary incontinence. In most cases, gait disturbance is the first symptom, followed by dementia and later pelvic disorders follow. Possible fluctuation in the severity of symptoms, but this is not typical for IGT. The main complaint of patients with dacha pathology at the appointment with a neurologist is dizziness, which they describe as a feeling of instability during movement, sharp turns of the torso. In this case, the basis of dizziness is postural instability and dysbasia. characteristic of the disease. Walking disorders include elements of apraxia gait in the form of a shuffling gait with short, widely spaced steps and loss of balance control. With normotensive hydrocephalus, there are no changes in hand movements when walking, which distinguishes it from Parkinson's disease. In the early stages, with minimal support, there may be little change in gait in patients with normotensive hydrocephalus.

As the disease progresses, the step height decreases, it becomes difficult for patients to take their feet off the ground, difficulties appear in initiating the act of walking, turns are made in several stages. falls are frequent, postural instability may occur. Need to carry out differential diagnosis with Parkinson's disease and other extrapyramidal disorders. At the same time, patients with normotensive hydrocephalus can imitate the movements of the legs, which they must perform when walking, lying down or sitting. In idiopathic normotensive hydrocephalus, there is a relationship between the presence of arterial hypertension and the severity of clinical symptoms, especially walking disorders. Muscle tone in the legs, as a rule, is increased according to the plastic type or the type of resistance. In more severe cases of normotensive hydrocephalus, spasticity, hyperreflexia occur in the lower extremities, and pathological foot signs are detected. The presence of symptoms predominantly in the legs in this pathology may be due to the fact that the motor pathways connecting the cerebral cortex with lower limbs, are located more medially - near the walls of the lateral ventricles, and the paths leading to the upper limbs are more lateral. Changes in gait in patients may also be due to dissociation of the basal ganglia from the frontal regions, dysfunction of the frontal cortex, and impaired sensorimotor integration.

Another important manifestation of Hakim-Adams syndrome is dementia. Patients are characterized by the presence of disorientation more in time than in place. It is often difficult for patients to state the history of their disease. Some may develop hallucinations, mania, depression. A characteristic symptom of normotensive hydrocephalus is also the development of emotional dullness. In general, cognitive impairments are manifested by a decrease in memory, a slowdown in the rate of mental processes and psychomotor reactions, a decrease in the ability to use acquired knowledge, apathy, which is associated with dysfunction of the anterior parts of the brain and is characteristic of the so-called subcortical dementia.

Cognitive impairment in IGT is not the dominant syndrome, especially at the onset of the disease, when gnosis and other cortical functions are usually not impaired. Unlike Alzheimer's disease, memory impairment in normotensive hydrocephalus is not so pronounced and is caused by an ocular image of a decrease in the functional integration of the frontal lobes. Severe dementia in patients with normotensive hydrocephalus implies either an irreparable morphological defect (due to TBI, stroke, etc.) or the presence of concomitant Alzheimer's disease or vascular dementia. To detect cognitive disorders, especially on early stages diseases, neuropsychological methods sensitive to frontal disorders are used. The frontal nature of cognitive disorders in normotensive hydrocephalus may be due to the predominant expansion of the anterior horns of the lateral ventricles, accompanied by a more significant dysfunction of the deep compartments of the frontal lobes and anterior sections. corpus callosum. In contrast to Alzheimer's disease, the cognitive defect in normotensive hydrocephalus develops more rapidly - within 3-12 months. The severity of cognitive disorders may decrease after administration of 20-50 ml of CSF (tap-test). It is believed that the basis of cognitive disorders in normotensive hydrocephalus is compression of the brain capillaries by increased intraparenchymal pressure, especially since, according to positron emission tomography, a diffuse decrease in metabolism is detected both in the cortical and 8 subcortical regions.

Already in the early stages, with active, purposeful questioning, it is possible to identify patients' complaints of frequent urination and nocturia. As the disease progresses, imperative urges and periodic urinary incontinence join. Patients stop feeling the urge to urinate and are indifferent to the fact of involuntary urination, which is typical for the frontal type of pelvic disorders. Fecal incontinence is rare, usually in patients at an advanced stage of the disease.

Results of additional surveys

It is important to note that during ophthalmoscopy, patients do not have congestion in the fundus. According to the EEG, in normotensive hydrocephalus, nonspecific changes in the bioelectrical activity of the brain are revealed, characterized by the predominance of slow frequency characteristics.

The results of CT and MRI make it possible to detect sharply dilated cerebral ventricles, while the cortical sulci remain within the normal range or slightly dilated. Using these techniques, other causes of hydrocephalus can be ruled out. The detection of small ischemic foci or areas of leukoaraiosis does not contradict the diagnosis of normotensive hydrocephalus, since a combination of Hakim-Adams syndrome and cerebrovascular insufficiency is possible. With normotensive hydrocephalus, the 111 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. The expansion of the anterior horns of the lateral ventricles in normotensive hydrocephalus reaches 30% or more of the diameter of the skull.

Surgical treatment of NTG syndrome

The basis of treatment is shunt operations of ventriculoperitoneal and lumboperitoneal shunting, in which a positive effect is achieved.
in 60% of patients. In advanced stages of the disease, when there are already irreversible changes in the brain, the prognosis of surgical treatment worsens. In some patients, among whom no
significant improvement after lumbar puncture, bypass surgery may also be effective. Complications after ventriculoperitoneal shunting in 30-40% of patients:

• subdural hematomas;
• liquor hypotension syndrome.

Mortality after ventriculoperitoneal shunting is about 6-7%. To prevent complications, an individual selection of a shunt is recommended.

Normotensive hydrocephalus conservative treatment

Purpose: to reduce the production of cerebrospinal fluid

Prescribed diacarb (acetazolamide) ...

Treatment of urination disorders is an extremely difficult task: the available medications ineffective.

Some patients are helped, at least temporarily, by learning to void. bladder"by the hour".

Normotensive hydrocephalus (G91.0) is hydrocephalus that develops as a result of traumatic brain injury, subarachnoid hemorrhage, purulent meningitis, and there is no intracranial hypertension.

Normotensive Hakim-Adams hydrocephalus is characterized by a triad of symptoms: gait disturbance due to ataxia or frontal dysbasia due to apraxia, dementia, urinary disorders.

Normal pressure hydrocephalus occurs in 0.4-6% of patients with dementia. Prevalence: 2.5-5 per 100 thousand people. In 50% of cases, it is combined with anomalies in the development of the ventricular system.

Symptoms of normotensive hydrocephalus

Initially, there is a violation of walking (“legs stick to the floor”, slowing down of walking, difficulty in starting to move, imbalance / falling) for several months, years. Cognitive impairments (difficulty in thinking, memory loss) appear within 6-12 months. IN early period diseases are associated with urination disorders.

An objective examination of the patient can reveal ataxia, apraxia of walking (85%), a slow gait with widely spaced legs (70%), acheirokinesia (10%), hypertonicity in the legs of the pyramidal type (70%), paratonic rigidity (20%), hyperreflexia (30%), apathy/depression (50%), cognitive decline (40%). There are disorientation in place and time, hallucinations, episodes of delirium (30%); epileptic seizures (20%); pollakiuria, nocturia, urge to urinate, urinary incontinence (15-50%).

Diagnosis of normotensive hydrocephalus

• Ophthalmoscopy (absence of indirect signs of intracranial hypertension).
• Computed / magnetic resonance imaging of the brain (severe hydrocephalus; scalloped corpus callosum).

• Lumbar puncture with the removal of 30-50 ml of CSF (leads to a short-term improvement in gait), performing neuropsychological tests, pelvic functions (tap-test).

• Long-term monitoring of intracranial pressure using intraventricular sensors.

Differential Diagnosis:

• spinocerebellar degeneration.
• Cerebellar degeneration as a result of alcoholism.
• chorionepithelioma.

Treatment of normotensive hydrocephalus

Treatment is prescribed only after confirmation of the diagnosis by a specialist doctor. Neurosurgical operations are performed with the imposition of a ventriculoperitoneal, ventriculoatrial or lumboperitoneal shunt. Symptomatic therapy is shown.

For citation: Damulin I.V., Oryshich N.A. Normotensive hydrocephalus: clinic, diagnosis, treatment // BC. 2000. No. 13. S. 589

MMA named after I.M. Sechenov

Normotensive hydrocephalus (NTH) is a syndrome characterized by a combination of dementia, walking disorders and urinary incontinence with marked expansion of the ventricular system and normal cerebrospinal fluid (CSF) pressure.

Neurological disorders in IGT can completely or significantly regress after a timely bypass surgery, however, experience shows that operations are effective in 50-70% of cases.

Prevalence of NTG small - according to different authors, it is detected in 0.4-6% of patients with dementia . Frequency variability is associated with different diagnostic criteria to assess dementia.

The primacy of describing NTG as an independent disease belongs to S.Hakim and R.D.Adams. In 1965 they published articles on "symptomatic occult chronic hydrocephalus in adults with normal fundus" or "hydrocephalus with normal CSF pressure". The authors focused on the potential curability of this syndrome, which is clinically manifested by peculiar gait disorders such as walking apraxia, dementia, and pelvic disorders. Later this clinical symptom complex received the eponymous name Hakim-Adams triads . In modern literature, the term “normotensive hydrocephalus” is most widely used.

Etiology

The development of NTG is due to an imbalance in the secretion and resorption of CSF and a violation of liquorodynamics. NTH in adults can be caused by various reasons: subarachnoid and intraventricular hemorrhage, traumatic brain injury, inflammation (meningitis), perinatal damage to the brain and meninges, volumetric intracranial formations (tumors, aneurysms of cerebral vessels), anomalies in the development of the brain (the most common - atresia of the Sylvian aqueduct), undergone operations on the brain and in other situations that create mechanical obstacles to the normal circulation of the CSF. In this case, there may be some (sometimes quite a long) period during which hydrocephalus does not manifest itself in any way. To the factors associated with the occurrence of hydrocephalus in adults, N.L. Graff-Radford et al. include congenital anomalies of the ventricular system that appear at a later age, impaired CSF absorption, age, and arterial hypertension. However, in about 30-50% of cases in the anamnesis of patients with IGT, there is no indication of any cause, in this situation the diagnosis becomes competent. adult idiopathic normotensive hydrocephalus ”.

Clinical features

NTG is characterized by the gradual development of the Hakim-Adams triad, in most cases, walking disorders are the first symptom, then dementia occurs and later pelvic disorders join. There may be fluctuation in the severity of symptoms, but this symptom is not considered characteristic of IGT.

Walking disorders

Walking disorders in IGT include elements of apraxia gait with short, magnetic gait, poor balance control, and difficulty turning. Patients are characterized by a shuffling gait on widely spaced legs, instability when turning. With NTG, there are no changes in hand movements when walking. In the early stages, with minimal support, the gait of patients with IGT may become intact. As the disease progresses, the step height decreases, it is difficult for patients to take their feet off the ground, difficulties are noted at the beginning of the act of walking, turns are made in several stages, falls are frequent. At the same time, patients with IGT can imitate the movements of their legs, which they must perform when walking, lying down or sitting. It should be noted that similar walking disorders are also observed in vascular lesions of the brain. In idiopathic IGT, there is a relationship between the presence of arterial hypertension and the severity of clinical symptoms, especially walking disorders . Walking disorders can largely regress immediately after the removal of a large amount (20-50 ml) of CSF during a lumbar puncture (“tap-test”). According to E.Blomsterwall et al. , the “tap-test” improves balance more than patient walking and does not depend on the etiology of IGT. Muscle tone in the legs, as a rule, is increased according to the plastic type, paratonic rigidity is noted. In more severe cases of NTG in the lower extremities, spasticity, hyperreflexia occur, and a pathological Babinski reflex is detected. The presence of symptoms predominantly in the legs with NTH may be due to the fact that the motor pathways connecting the cerebral cortex with the lower extremities are located more medially - near the walls of the lateral ventricles, and the paths leading to the upper extremities are more laterally. Changes in gait in patients with IGT may be due to dissociation of the basal ganglia from the frontal regions, dysfunction of the frontal cortex, and impaired sensorimotor integration.

Violations of higher brain functions

Patients with NTG are characterized by the presence of spontaneity, complacency, disorientation, more in time than in place . Patients cannot state the history of their disease. Some patients may develop hallucinations, mania, delirium, depression. A characteristic symptom of NTG is also emotional dullness . The aspontaneity of patients as the disease progresses can turn into akinetic mutism, increased drowsiness, stupor, and a vegetative state.

Cognitive impairment occur in the vast majority of patients at the onset of the disease. These disorders are manifested by a decrease in memory, a slowdown in the speed of mental processes and psychomotor reactions, a decrease in the ability to use acquired knowledge, apathy, which is associated with dysfunction of the anterior parts of the brain and is characteristic of the so-called subcortical dementia. Cognitive impairment in IGT is not the dominant symptom; in the early stages, gnosis and other cortical functions are usually not impaired. Unlike Alzheimer's disease, memory impairment in IGT is not as pronounced and is mainly due to a decrease in the functional integration of the frontal lobes. Severe dementia in patients with IGT implies either an irreparable morphological defect or the presence of Alzheimer's disease or vascular dementia.

To date, there is no specific neuropsychological technique that can unequivocally differentiate cognitive impairment in Alzheimer's disease and IGT. It should be emphasized that the distinction between cortical and subcortical dementia is very relative. This is evidenced by the results of the study by J.Kramer and J.Duffy. The authors did not note significant differences in the incidence of praxis and gnosis disorders between patients with cortical and subcortical dementia (the latter group included patients with IGT and Parkinson's disease).

To detect cognitive impairment in IGT, especially in the early stages of the disease, neuropsychological scales sensitive to frontal impairment are used. The use of tests commonly used in dementia (such as the Mini-Mental Assessment Scale) often gives false-negative results, since these methods are not very informative for a frontal-type cognitive defect. Therefore, with NTG, tests aimed at assessing the patient's ability to form and change an action program depending on conditions (such as the Wisconsin card sorting test), as well as tests that assess the mobility of mental processes, the level of exhaustion and attention (Schulte tables, red-black tables) are more informative. or Stroop color test).

The frontal nature of cognitive disorders in IGT may be due to the predominant expansion of the anterior horns of the lateral ventricles, accompanied by a more significant dysfunction of the deep sections of the frontal lobes and anterior corpus callosum. Unlike Alzheimer's disease, the cognitive defect in IGT develops more rapidly - within 3-12 months. The severity of cognitive disorders may decrease after the removal of 20-50 ml of CSF. It has been suggested that cognitive disorders are based on microcirculatory cerebral disorders due to compression of capillaries by elevated intraparenchymal pressure, especially since, according to positron emission tomography, NTG reveals a diffuse decrease in metabolism both in the cortical and subcortical regions.

Pelvic Disorders

Already in the early stages of NTG, with active, purposeful questioning, it is possible to identify Patient complaints of frequent urination and nocturia . Subsequently join urge and urinary incontinence . Patients are no longer aware of the urge to urinate and are indifferent to the fact of involuntary urination, which is typical for the frontal type of pelvic disorders. Fecal incontinence is rare, usually in patients with advanced IGT. From patients with other causes of dementia, patients with IGT are distinguished by the presence of pelvic disorders in the early stages of the disease and the partial restoration of control over the pelvic organs after the “tap-test”.

Patients with NTG usually do not complain of headache. On neurological examination, in addition to the Hakim-Adams triad, patients with NTG may experience postural tremor, a kind of akinetic-rigid syndrome characterized by the “freezing phenomenon”, the absence of acheirokinesis, paratonic rigidity in the axial muscles and in the muscles of the extremities , pseudobulbar syndrome, grasping reflex.

Diagnosis of normotensive hydrocephalus

The complexity of diagnosing IGT is due to the fact that the symptoms characteristic of this disease - dementia, pelvic disorders and walking disorders - are often observed in the elderly.

Routine examination, as a rule, does not reveal any pathology, craniograms are not changed . It is especially important to emphasize the absence of congestion in the fundus. According to the EEG, NTG reveals nonspecific changes characterized by an increase in slow-wave activity.

Lumbar puncture remains one of the main methods for diagnosing IGT. CSF pressure usually does not exceed 200 mm of water. If a manometer is attached to the puncture needle, then, as is well known, the cerebrospinal fluid column detects pressure fluctuations depending on the pulse, blood pressure, respiration. Normally, CSF pulsation does not exceed 15-20 mm. However, with NTG, it significantly exceeds this value, and the recording of oscillations reveals a change in the waveform - it becomes steeper. Laboratory analysis of CSF usually does not reveal any abnormalities.

Monitoring of intracranial pressure (ICP) It is the most modern method diagnosing NTG . When registering ICP for 24-48 hours in patients with IGT, pathologically high ICP values ​​are noted, especially in the REM sleep phase, which is associated with vasodilation and an increase in brain blood supply during this period. NTG is characterized by the presence of a large number of secondary b-waves and "plateau"-waves. Changes in ICP fluctuations are associated with the existing functional obstacle to the outflow of CSF from the ventricular system into the subarachnoid spaces due to the difficulty of CSF reabsorption and a decrease in the gradient between CSF pressure in the ventricles and on the convexital surface of the brain. To increase the accuracy of the technique, ICP monitoring should be carried out synchronously with polysomnography, since it has been shown that the relative frequency, amplitude, length and shape of b-waves depend on the sleep phase, and their quantitative representation during sleep is considered by some authors as a diagnostic and prognostic sign of NTG . Before monitoring ICP, it is proposed to use transcranial Doppler ultrasound, since there is a non-linear relationship between ICP b-waves and blood flow velocity in the intracranial arteries, described as the transcranial Doppler equivalent of b-waves.

"Tap-test" indirectly reflects the violation of CSF resorption underlying the pathogenesis of IGT . For a more accurate assessment of the resistance to CSF ​​resorption, we use infusion test , which consists in the simultaneous endolumbar administration of saline and recording the rate of decrease in CSF pressure after its initial increase in response to the administration of the solution. There is another test method - long-term infusion of 0.9% NaCl solution under constant pressure. It should be noted that the reliability of the infusion test itself and the methodology for its implementation are the subject of debate. Various modifications of the infusion test are used primarily for research purposes. Currently, the “tap-test” is used all over the world as the simplest, fastest, cheapest and fairly reliable method.

Radioisotope cisternography NTG reveals the accumulation of the radiopharmaceutical in the ventricular system in the absence of its circulation over the fornix of the brain even 48 hours after administration. However, these findings are not considered highly specific for IGT.

Important for the diagnosis of NTG are the results of neuroimaging research methods (computer and magnetic resonance imaging - CT / MRI), which allow to detect sharply dilated ventricles of the brain, while the cortical grooves remain within the normal range or are slightly expanded. Using these techniques, other causes of hydrocephalus can be ruled out. The detection of small ischemic foci or leukoaraiosis does not contradict the diagnosis of IGT, since a combination of IGT and cerebrovascular insufficiency is possible. There is significant agreement between CT and MRI findings in normal aging, IGT, and dementia of degenerative origin. With IGT, 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. The expansion of the anterior horns of the lateral ventricles with IGT reaches 30% or more of the diameter of the skull.

It is believed that although hydrocephalus can be diagnosed quite easily using CT, MRI is recommended for all patients to obtain a more detailed image of the brain structures. . The dimensions of the ventricular system using CT and MRI are estimated with the same accuracy, however, MRI is better at visualizing the presence of transependymal CSF penetration. The presence of periventricular transependymal CSF penetration (increased signal in T2- and proton density mode - according to MRI, hypodense zones on CT) in combination with dilated ventricles is a characteristic neuroimaging sign of NTH in elderly patients in the absence of an intracranial occlusive process. The thickness of the periventricular “halo” correlates with the best outcome of shunting, the presence of signs of microangiopathic cerebrovascular disease (lacunae and foci of increased signal intensity in the deep sections of the white matter in T2 and proton density MRI modes) - with an unsatisfactory outcome of shunting. C.Jack et al. consider that the best outcome of the operation in patients with more pronounced periventricular changes is associated with more significant liquorodynamic disorders inherent in this category of patients, the reduction of which after shunting is accompanied by a significant clinical improvement. Particular importance is attached to the methods of MRI-assessment of liquor outflow through the water supply system. , both for diagnosing IGT and for evaluating the success of subsequent bypass surgery.

With IGT, 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 positron emission tomography, a decrease in the level of glucose metabolism (general and regional) is detected, and the greater the degree of frontal hypometabolism, the more likely the adverse outcome of bypass surgery becomes.

The prognosis of shunting with IGT is better in patients who have experienced an increase in regional cerebral blood flow after the administration of glycerol. According to J.-L.Moretti, after the removal of 30-50 ml of CSF in patients with IGT, an increase in regional cerebral blood flow is noted. This contradicts the data previously reported in the literature and obtained using the 133Xe inhalation method. M.Kushner et al. did not reveal an increase in total cerebral blood flow in IGT after lumbar puncture and after successful shunting.

L.Ketonen and M.Berg emphasize that so far there is no clarity regarding the best test or combination of tests, the results of which could predict the success of bypass surgery in IGT.

Differential Diagnosis

Differential diagnosis of IGT should be carried out with other types of hydrocephalus, ventriculomegaly in neurodegenerative and vascular diseases, with volumetric intracranial processes, etc. In the literature, there is a description of cases of the occurrence of the IGT syndrome in rheumatoid arthritis, improvement in the condition (decrease in the severity of cognitive and pelvic disorders, walking disorders) was noted after a course of corticosteroid therapy. Lyme disease can lead to IGT syndrome due to impaired CSF flow. A. Danek et al. describe a 74-year-old woman whose symptoms of IGT regressed after ceftriaxone therapy. NTH cases have been described in myotonic dystrophy, which is associated with a cellular membrane defect leading to impaired CSF absorption. A case of NTG is described in an elderly patient with chronic inflammatory demyelinating polyneuropathy, vitamin B1 and folate deficiency. The authors attribute the occurrence of hydrocephalus in this patient to impaired CSF resorption due to high protein levels. Ventricular dilatation occurs in cancer patients, especially those with malnutrition or chemotherapy. In chronic neuroinfectious processes (especially of a mycotic nature), metabolic and endocrine disorders (vitamin B12 deficiency, hypothyroidism, etc.), so-called pseudoatrophy may occur, which, with successful therapy, can regress. With the Hakim-Adams triad, meningovascular syphilis can occur.

Treatment

Bypass surgery with ventriculoperitoneal and lumboperitoneal shunts is the treatment of choice. With the right selection of patients, the positive effect reaches 60%. Of course, in the advanced stages of the disease, when there are already irreversible changes in the brain, the prognosis of surgical treatment worsens. Mortality during this operation is about 6-7%. The most likely positive effect of shunting is in cases where clinical picture NTH develops during the first months after a subarachnoid hemorrhage, meningitis or traumatic brain injury, there is an improvement after the removal of a large amount of CSF during a lumbar puncture, and according to the neuroradiological study, a pronounced ventricular CSF flow (pulsation) is detected. A good outcome of shunting is noted in patients with hyperdynamic type of CSF flow in the third ventricle and aqueduct, which is characterized by the absence of a signal from the CSF on the sagittal midline MRI. At the same time, according to the data obtained by J. Malm et al. , the results of the “tap-test” do not help in the selection of patients for bypass surgery. In some patients who do not improve after lumbar puncture, bypass surgery may also be effective.

The simplest and most reliable for predicting the prognosis of bypass surgery can be single removal of 20-50 ml of cerebrospinal fluid with a lumbar puncture - “tap-test” . Another version of the “tap-test” is the withdrawal of 30 ml for 3 days. A lumbar puncture is performed on the patient in the supine position, the initial CSF pressure, the amount of CSF excreted and the final CSF pressure are recorded, if possible, CSF pressure fluctuations are recorded. It is especially important to evaluate the dynamics of the main symptoms of the Hakim-Adams triad after this test. Even short-term clinical improvement may indicate a possibly favorable prognosis for bypass surgery.

We observed two patients 59 and 63 years old with the classical Hakim-Adams triad, in whom, after the removal of 40-50 ml of CSF, the restoration of the correct orientation in place and time, an increase in speech expression and the level of attention were noted; independent walking became possible and control over the pelvic organs was restored. The “tap-test” effect persisted for 10 days. Both patients had a significant positive effect of bypass surgery.

As a conservative therapy to reduce CSF production, patients with IGT are prescribed acetazolamide And digoxin However, the effectiveness of this therapy has not yet been proven. Treatment of urinary disorders in IGT presents a challenge , anticholinergics are used very rarely for this type of urination disorder, some patients, at least temporarily, are helped by teaching them to empty the bladder “by the clock”.

Complications after bypass surgery observed in 31-38% of patients. The main complications include the following:

In rare cases, subdural hematoma due to a rapid decrease in the size of the ventricles, which requires a second operation;

CSF hypotension syndrome, manifested by headaches when standing up, the prevention of which is the individual selection of a low, medium and high pressure shunt.

References can be found at http://www.site

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Appendices to the article