NEUROLOGY AND NEUROSURGERY - Y. V. ALEKSEEENKO - 2014

AUTONOMIC DYSTONIA SYNDROME. HYPOTHALAMIC SYNDROME

The problem under Structure/133.html">Discussion is so broad, and The Significance of autonomic regulation in human life is so all-encompassing, that it is impossible today to limit ourselves merely to discussing the principles of the Structural and functional Organization OF THE autonomic sphere and the specific signs of its impairment. Understanding the diverse variants of impaired autonomic regulation requires drawing upon a wide range of related clinical disciplines. For many years within the CIS, the dominant scientific school in clinical vegetology has been represented by the specialists of the widely known Autonomic Pathology Center at the Moscow Medical Academy, headed by Professor A. M. Vein. For a more detailed study of this section, we can recommend the manuals on clinical vegetology prepared by representatives of this neurological school.

Clinical vegetology covers issues of causality, Pathogenesis, Clinical presentation, Diagnosis, and Treatment of autonomic disorders across various pathological conditions. Autonomic disorders represent one of the most common groups of pathological states. At the same time, they extremely rarely present as independent clinical entities. There are practically no diseases or diverse pathological conditions in whose clinical picture we could not find certain symptoms of autonomic dysfunction.

Recognizing and treating these disorders quite often requires the participation of a neurologist, psychiatrist, general practitioner, endocrinologist, gynecologist, and other specialists. Unfortunately, the fragmentation of certain clinical fields often leads to the same autonomic disorders being diagnosed by different specialists as entirely different diseases and designated by various terms.

Structural and Functional Organization of the Autonomic Sphere

The Autonomic (vegetative) Nervous system plays a vital role in human life and activity. Its purpose can be viewed in two aspects.

   1. Maintaining the functional parameters of various systems within homeostatic boundaries, i.e., preserving the constancy of the body's internal environment.

   2. Autonomic support for Various Forms of activity and adaptation to changing environmental conditions.

In a healthy individual who is well-adapted to environmental conditions, the support of various forms of mental and physical activity is carried out according to THE PRINCIPLE OF effective sufficiency. That is, the optimal regime is the most efficient activity of various systems and the whole Organism, achieved with minimal Energy Expenditure and autonomic strain.

Levels and Divisions of the Autonomic nervous system. The autonomic nervous system is a complex network of structures traditionally divided into 2 levels. The first is the segmental level, which includes the autonomic nuclei of the Brainstem and Spinal Cord, as well as peripheral autonomic structures. At the segmental level, there are sympathetic and parasympathetic divisions, which differ significantly in Morphology, neurotransmitter mechanisms, and, naturally, have opposite functional purposes. The second level is the suprasegmental level. It includes the structures of the limbic-reticular complex, the boundaries of which are still not clearly defined. The most important links of this system are the brainstem reticular formation, Hypothalamus, thalamus, amygdala, hippocampus, septum, cingulate gyrus, posterior areas of the orbital frontal cortex, etc. There is another designation for this functional-morphological complex: the non-specific Brain system. In a certain sense, these are synonyms. The limbic system, especially its higher divisions, (1) lacks features of segmentality and (2) does not possess strict sympathetic or parasympathetic specialization.

The limbic-reticular complex is an integrative system that ensures the interaction of autonomic, mental, sensorimotor, and endocrine Functions aimed at executing genetically determined and biologically purposeful activities or behaviors, species-preservation processes, and adequate bodily adaptation. The limbic complex is directly involved in regulating emotional sphere, Sleep-wake cycles, and memory. In a state of relative rest, the segmental autonomic system can maintain the automated functioning of many Organs and systems. Adaptation to changing environmental conditions (adaptive behavior) is carried out with varying degrees of involvement from the limbic complex and The Use of segmental mechanisms as the executive apparatus for rational adjustment.

From a functional-biological perspective, the limbic-reticular complex exerts two somewhat opposing types of influence: ergotropic and trophotropic. Ergotropic reactions ensure adaptation to changing environmental conditions and the execution of active physical and mental activity. This system increases the level of mental activation and motor readiness, and provides autonomic mobilization. Meanwhile, at the segmental level, sympathetic effects predominate. Trophotropic reactions are responsible for maintaining Homeostasis and are associated with periods of rest, Digestion, and the accumulation of energy resources. These processes are mediated through the predominance of parasympathetic segmental activity.

The interaction of segmental autonomic mechanisms in normal conditions and various pathological states was long viewed as a balance of antagonistic systems operating on a simple scale principle. However, in recent years, the complex interaction of the sympathetic and parasympathetic divisions has become evident, which in normal conditions is strictly correlated with The Nature of activity and the baseline Background. The balance of these systems can be achieved at different levels of activity for each.

Clinical Assessment of Autonomic Nervous System Functions

Methodological approaches and Methods FOR STUDYING autonomic nervous system functions. The Study of the autonomic nervous system must be based on a clinical-experimental approach, The Essence of which lies in analyzing and comparing autonomic tone, autonomic reactivity, and the autonomic support of activity. Autonomic tone and reactivity provide insight into the body's homeostatic capabilities, while the autonomic support of activity reflects its adaptive capabilities or physiological reserves.

By (baseline) autonomic tone, we mean the stable autonomic parameters of a subject during a period of relative rest. The study of autonomic tone is carried out using special questionnaires to record subjective symptoms. In addition, tables of objective indicators of the autonomic sphere are used. Skin color and moisture, vascular pattern, sweating, dermographism, Temperature, pulse, Blood pressure, etc., are recorded.

Autonomic reactivity is characterized by shifts occurring in the autonomic sphere in response to internal or external stimuli. Pharmacological tests, physical or chemical influences, and stimulation of reflexogenic zones (oculocardiac reflex, sinocarotid reflex, etc.) are applied.

Perhaps of greatest interest is the Study of the autonomic support of activity. Under normal conditions, it is strictly correlated with the form, intensity, and duration of human activity. The purpose of these shifts is to ensure the most efficient execution of work with minimal strain on adaptive mechanisms. This is achieved by modeling various experimental activities (physical, such as cycle ergometry, mental activity, etc.). In such cases, indicators of cardiovascular and Respiratory system function are most frequently recorded. The value of this approach lies in The ability to compare work performance quality against the body's energy costs under standardized loads.

This three-stage scheme is used both to study the state of suprasegmental formations and the segmental mechanisms of autonomic regulation. However, a comprehensive Assessment of the autonomic regulation system is impossible without psychological testing and hormonal profiling methods.

Autonomic Dystonia Syndrome

Autonomic dystonia syndrome is one of the most common variants of disintegration within the non-specific system (suprasegmental structures of the autonomic nervous system). Literally, it represents an imbalance of the autonomic regulation systems.

Autonomic dysfunction may exhibit (1) generalized, systemic, and local manifestations. The combination of its manifestations from the most involved organs and systems usually forms a complex clinical picture with individual specific features. The symptom complex of autonomic dystonia often reflects specific constitutional features of The Nervous System's functional organization and reactivity.

Autonomic dystonia syndrome consists of (2) polar symptoms of sympathicotonia and vagotonia. In reality, however, this pathology does not present with pure syndromes of activation or depression of a single autonomic division. Autonomic dysfunction is always mixed and mosaic in nature.

It should be noted that autonomic dysfunction is characterized by (3) both objective and subjective deviations, with the latter overwhelmingly dominating in importance. Finally, autonomic dystonia syndrome typically represents (4) a combination of permanent (prolonged and relatively stable) and paroxysmal (crises) manifestations. The clinical picture of autonomic dysfunction comprises (5) functional-neurological, visceral, and emotional-affective symptoms.

A person with a predominance of sympathetic tone exhibits the following signs:

   ■ pale and dry skin;

   ■ cold extremities;

   ■ bright eyes and mild exophthalmos;

   ■ body temperature instability;

   ■ tendency toward tachycardia, discomfort in The Heart area, tachypnea;

   ■ tendency toward elevated blood pressure;

   ■ tendency toward constipation (decreased intestinal motility);

   ■ Muscle tremors;

   ■ paresthesias, feeling cold, reduced tolerance to sun and heat;

   ■ high working capacity and physical endurance with decreased ability to memorize and concentrate;

   ■ increased performance efficiency toward the evening;

   ■ anxiety, fear, a sense of tension;

   ■ decreased tolerance to noise, bright light, and caffeine-containing products.

In vagotonia, the following signs are characteristic:

   ■ cold, clammy, and pale skin;

   ■ hyperhidrosis and hypersalivation;

   ■ bright red dermographism;

   ■ bradycardia, tendency toward arterial hypotension, respiratory arrhythmia, and a tendency to faint;

   ■ tendency toward weight gain;

   ■ higher performance efficiency in the morning hours, decreased endurance;

   ■ symptoms of apathy, asthenia, low initiative, timidity, sensitivity, and a tendency toward depression.

Systematizing Complaints and individual signs of vegetative dystonia syndrome makes it possible to distinguish several characteristic symptom complexes: cephalagic, cardiovascular, hyperventilatory, neurogastric, thermovascular, motivational-behavioral, etc.

Autonomic crises (paroxysms) are the most striking and dramatic manifestation of vegetative dystonia syndrome. They represent an episodic flare-up of the aforementioned emotional-affective, functional neurological, and visceral disorders over a relatively short period of time. This is a turbulent and concentrated manifestation of the disease. Crises can be sympathoadrenal or vagoinsular, although mixed paroxysms are more frequently observed. In English-language literature, a close equivalent to autonomic crises is «panic attacks.» This term emphasizes the dominant role of emotional-Affective Disorders within the symptom complex. Diagnostic criteria include fear or anxiety plus 4 or more autonomic symptoms.

Autonomic crises occur more frequently in women (approximately twice as often). Most commonly, this happens between the ages of 20 and 45. The frequency of crises varies significantly. The duration of each crisis can range from 20 minutes to several days. Crises may be spontaneous («out of the blue»), but they more often develop against the background of triggering factors (psychogenic, physiogenic, biological, etc.).

The most common symptoms during a crisis include:

   ■ shortness of breath, difficulty breathing, or suffocation;

   ■ palpitations, pain in the left side of the chest, skipped beats, or fluttering sensations;

   ■ excessive sweating;

   ■ numbness and/or paresthesia in the extremities and face;

   ■ sensation of a lump in the throat;

   ■ hot or cold flashes;

   ■ chills or shivering;

   ■ dizziness, unsteadiness, or lightheadedness;

   ■ visual or Hearing impairments;

   ■ faintness, presyncope, or sudden weakness;

   ■ syncopal state (a variant of vasovagal crisis);

   ■ muscle cramps in the extremities;

   ■ discomfort in the epigastric region and abdomen;

   ■ nausea, vomiting;

   ■ frequent urination, diarrhea;

   ■ loss of speech or voice;

   ■ gait disorders;

   ■ fear of death;

   ■ feelings of unreality or detachment (derealization/depersonalization);

   ■ fear of losing one's mind or doing something uncontrollable;

■ anxiety, inner tension;

   ■ dysphoria, depression, hopelessness;

   ■ aggression.

Thus, There is a combination of somatic, emotional-affective, and functional-neurological symptoms. The latter include the sensation of a "lump in the throat," manifestations resembling aphonia, amaurosis, mutism, numbness and weakness in the limbs, tonic muscle spasms, elements of psychogenic ataxia, chills, and others.

Currently, the following etiopathogenetic variants of autonomic dystonia syndrome are distinguished.

1. Autonomic dystonia syndrome of constitutional origin. In this case, signs of autonomic instability appear in early childhood and accompany a person throughout almost their entire life, reaching a degree of decompensation under certain circumstances. These disorders frequently have a familial and hereditary character. Most commonly, one observes ARTERIAL BLOOD PRESSURE instability, heart rate Variability, a tendency toward syncope, dyskinesia of various PARTS OF THE gastrointestinal tract, rapid changes in skin coloration, sweating, a tendency toward low-grade fever (subfebrile temperature), poor tolerance to physical and intellectual stress, and meteosensitivity. Such individuals are often referred to as "invalids of the autonomic nervous system," meaning they possess noticeably limited adaptive reserves—not yet patients, but prone to an intensification of all the aforementioned manifestations under adverse environmental conditions. This often coincides with periods of hormonal adjustment and so forth. With age, provided there is proper career guidance, The Development of optimal life strategies, and the creation of relative comfort conditions, such individuals usually achieve a certain degree of compensation.

2. Autonomic dystonia syndrome arising against the background of the body's endocrine shifts. It manifests during the periods of Puberty and menopause.

3. Autonomic dystonia syndrome associated with primary lesions of Internal Organs. This refers to a wide range of diseases in which the neurogenic factor is not the leading link in pathogenesis: urolithiasis, Chronic Pancreatitis, cholelithiasis, chronic tonsillitis, infectious colds, and so on. Cure or regression of the underlying disease leads to the disappearance or significant alleviation of signs of autonomic dysfunction.

4. Autonomic dystonia syndrome in primary Diseases of the peripheral Endocrine glands.

5. Allergy. In this regard, The Immune System must be viewed as one of the most vital elements of homeostasis and adaptive regulation.

6. Autonomic dystonia syndrome associated with Pathology of the segmental autonomic nervous system. It occurs in vertebral osteoarthrosis (osteochondrosis), plexus lesions, ganglionitis, neuralgia, and the like.

7. Autonomic dystonia syndrome in organic brain damage. It is most vividly manifested in tumor, traumatic, vascular, infectious, or toxic lesions of deep-seated structures that form the Links of the limbic-reticular complex. At the same time, a distinction should be made between autonomic dystonia syndrome and the complex of manifestations of hypothalamic syndrome, the diagnostic criteria of which will be discussed later.

8. Autonomic dystonia syndrome as a manifestation of neurosis. Neuroses are arguably the most common diseases and simultaneously the most frequent cause of autonomic disorders. Autonomic dystonia syndrome is an obligate manifestation of neuroses. The special relationship between the emotional and autonomic spheres is reflected in the designation of psychophysiological and psychautonomic disorders. Symptoms caused by impaired regulation of internal organ functions create a situation where the complex of psycho-autonomic-visceral disorders becomes the reason for determining their primary cause among three categories of conditions: neurosis, autonomic dystonia syndrome of another origin, and primary somatic pathology. A variant of autonomic dystonia syndrome in this series is of a psychophysiological nature, arising against the background of acute or chronic stress.

Thus, establishing the leading etiopathogenetic factors of autonomic dystonia syndrome is a rather complex and responsible task. Solving it allows for outlining adequate therapeutic tactics. Based on this, autonomic dystonia syndrome cannot figure as the primary clinical diagnosis.

MODERN CONCEPTS OF hypothalamic syndrome and its clinical manifestations

The previous section described autonomic dystonia syndrome caused by organic brain damage. The hypothalamus is one of the key links of the limbic-reticular complex. Therefore, its lesion typically causes not only autonomic disorders but also severe disturbances of neuroendocrine functions. However, in medical practice, there is still a tendency toward overdiagnosis of this pathological condition, which was previously more often referred to as diencephalic syndrome.

Among the complex of main manifestations of hypothalamic syndrome is vasovagal/cardiovascular dysfunction in the form of crisis states and permanent disorders of autonomic regulation. The next mandatory component is Neuroendocrine Syndromes, in which primary lesions of the peripheral endocrine glands are absent (Cushing's syndrome, diabetes insipidus, etc.) and multiglandular insufficiency is characteristic. Motivational disorders (hunger, thirst, libido changes) are observed. Neurogenic thermoregulation disorders are documented. Another regular manifestation is sleep-wake cycle disorders. It is important to emphasize the mandatory complex nature of hypothalamic disorders.

It must be emphasized that the so-called "diencephalic Epilepsy," previously classified among the manifestations of hypothalamic syndrome, is not such. It is necessary to distinguish chills and shivering, tonic cramping of the limb Muscles, a certain stupor or fainting as symptoms of an autonomic crisis from the autonomic aura of a true temporal lobe epilepsy attack. Such a form of epileptic disease is usually accompanied by diverse autonomic disorders due to the specific localization of the epileptic activity focus in the limbic structures, posing a challenge in differentiating the Nature of the paroxysms.

What is hypothalamic syndrome? It is a symptom complex of neuro-metabolic-endocrine disorders resulting from dysfunction of the hypothalamo-pituitary region of the brain, the clinical picture of which consists of metabolic-endocrine, autonomic-vascular, and thermoregulatory disorders, as well as disturbances in sleep, motivations, and drives.

Hypothalamic syndrome is merely a topical diagnosis that does not reflect the nosological affiliation of pathological phenomena. Direct traumatic, infectious, tumor, or vascular lesions of these structures are extremely rare. In the vast majority of cases, clinicians deal with a constitutionally conditioned (neurochemical) deficiency of the hypothalamus and its associated structures of the limbic-reticular complex, decompensated under the Influence of External or internal environmental impacts. Among the most obvious predispositions and decompensating factors, the following can be noted:

   ■ advanced parental age;

   ■ history of occupational hazards in parents;

   ■ parental bad habits;

   ■ presence of allergies and serious somatic diseases in them;

   ■ pathological course of Pregnancy;

   ■ adverse influences during the perinatal period of the child's development,

   ■ periods of hormonal shifts in the body;

   ■ stress impacts;

   ■ infections;

   ■ injuries;

   ■ allergic conditions;

   ■ somatic diseases, etc.

Clinical Cases

Case 1

A 19-year-old male consulted a doctor complaining of malaise that developed after catching a cold. He reports persistent feelings of heaviness in the HEAD, pressing pain in the frontal and temporal regions—typically occurring In the second half of the day after university classes—along with weakness, fatigue, and sweating triggered by minor exertion or emotional stress. He has noticed a decline in mental performance and memory impairment, leading to difficulties in preparing for exams and anxiety regarding his academic performance. He reports difficulty waking up and a lack of refreshing sleep. When sustaining prolonged attention or staying in a stuffy room or a crowded place, he experiences palpitations, chest pressure, shortness of breath, tinnitus, lightheadedness, and fear of fainting. Physical examination reveals moderate hyperhidrosis of the hands and feet. Blood pressure is 105/60 mm Hg. Pulse is 58 bpm, with pronounced sinus arrhythmia. Other evaluations of the internal organs and blood tests revealed no abnormalities. Signs of chronic pharyngitis are present.

   • List the main neurological disorders.

   • Characterize the cephalgic syndrome.

   • How should these episodic deteriorations in well-being be interpreted?

   • How can the Objective Examination findings be explained (pulse, blood pressure, limited objective abnormalities)?

   • Formulate a preliminary diagnosis.

   • What is the most probable etiopathogenetic mechanism of this condition?

   • Propose the main directions for Differential diagnosis.

   • Outline the protocol and define the objectives of instrumental neurological examination.

   • List the methods and objectives of somatic examination.

   • Name the principal forms and symptom complexes of autonomic dystonia.

   • Formulate an explanation of this condition and the causes of malaise in a manner accessible to the patient.

   • Describe the MAIN DIRECTIONS OF comprehensive therapy.

   • Formulate preventive recommendations.

Case 2

A 44-year-old female patient has experienced an increasing frequency of headaches over the past two years, along with unstable blood pressure with a hypertensive tendency, decreased performance, emotional lability, and marked weather sensitivity. Over the past few weeks, she has experienced excessive stress and troubles at work. She sought medical evaluation after an attack that occurred without apparent cause in the morning while she was feeling otherwise well. The episode began with generalized chills, throbbing head pain, and tinnitus, which were interpreted as signs of elevated blood pressure. This was followed by a feeling of tension, hot flashes, palpitations, pressing chest pain, shortness of breath, epigastric discomfort, skin pallor, numbness and tingling in the Lips and Tongue, loss of speech and voice, limb stiffness, and a fear of impending death. Blood pressure rose to 150/90 mm Hg, and pulse increased to 110 bpm. The attack subsided a few minutes after the on-site paramedic administered antispasmodics, followed by frequent urination, lethargy, fatigue, and drowsiness. She is frightened by the incident and fears a recurrence.

   • Classify the nature of the experienced attack.

   • Group its MAIN CLINICAL MANIFESTATIONS.

   • Formulate a preliminary clinical diagnosis.

   • Determine the most probable etiopathogenetic variants of this disease.

   • What conditions must be considered in the Cytology/practical/136.html">DIFFERENTIAL DIAGNOSIS OF the described episode?

   • Outline the protocol and define the objectives of the instrumental neurological examination.

   • List the methods and objectives of the somatic examination.

   • List the MAIN TYPES OF autonomic crises.

   • Specify the characteristic symptom progression patterns typical of autonomic crises.

   • Formulate an explanation of this condition and the causes of malaise in a way that is easily understood by the female patient.

   • Describe the treatment methods for an autonomic crisis.

   • Formulate preventive recommendations.

Option 3

A 21-year-old man on a bus experienced progressive generalized weakness, lightheadedness, heaviness and ringing in the head, blurred Vision, chest discomfort, profuse sweating, and lost consciousness. According to witnesses, the patient turned pale, fell, and remained unconscious for about 1 minute. Upon regaining consciousness, he complained of weakness and lightheadedness. Since childhood, he has noted poor tolerance for stuffy, enclosed spaces and motion sickness in transport. On several occasions in a vertical position under similar circumstances, he experienced progressive feelings of weakness, lightheadedness, and ringing in the head. However, the attack was averted by breathing fresh air or changing body position. No focal neurological symptoms were detected. BP 115/60 mmHg, pulse 64. There are no abnormalities on the part of internal organs.

   • Name the main types of paroxysmal conditions accompanied by brief alterations of consciousness.

   • What circumstances and clinical parameters of the experienced episode need to be further clarified to determine its nature?

   • How can the Features of the remote medical history be interpreted?

   • Formulate a preliminary diagnosis.

   • Propose the main directions for differential diagnosis.

   • Outline the protocol and define the objectives of the instrumental neurological examination.

   • List the methods and objectives of the required somatic examination.

   • Determine the most likely cause of the developed condition.

   • List the diagnostic criteria for simple syncope.

   • Explain The Nature and pathophysiological mechanisms of the syncopal state.

   • Describe the features and content of therapeutic management.

   • Formulate an explanation of what happened and provide preventive recommendations for the patient.