Atrophy of the muscles of the hand, hands, forearms develops mainly as a secondary disease against the background of a violation of innervation (nutrition, blood circulation) in a certain area of \u200b\u200bmuscle tissue and less often, as a primary one (usually with myopathy), when the motor function is not impaired.
Causes of atrophy of the muscles of the arm, shoulder and hand
For the development of muscle atrophy, the predisposing causes are: professional factor (constant overstrain during heavy physical labor), wrist joint, endocrine pathology - obesity, and thyroid disease, acromegaly; cicatricial processes after trauma, metabolic and systemic diseases (lupus erythematosus), of various origins, congenital pathologies of the development of the lower limb.
Symptoms of atrophy of the muscles of the arm, shoulder and hand
Muscle atrophy is a serious disease that primarily affects muscle fibers. The main typical sign is the symmetry of the lesion (except for myasthenia gravis) and the slow development of the disease (with the exception of myositis), atrophy of the affected muscles and weakening of tendon reflexes with preserved sensitivity.
Most peripheral nerves have a mixed structure, and when damaged, the work of sensory, motor and autonomic fibers is disturbed. It happens that one of the fibers is affected the most.
If motor fibers are involved in the process, then paresis of the muscles that are innervated by this nerve occurs. The patient complains of muscle weakness, low muscle tone. Atrophy does not develop immediately, but 2-3 months after the lesion. In the absence of proper treatment, after a year and a half, the muscle will completely atrophy.
If sensitive fibers are involved in the process, the clinic is manifested by parasthesia - patients feel a tingling sensation, goosebumps. Neurological symptoms are expressed in the form of hyperesthesia (increased sensitivity), or hypesthesia (reduced sensitivity). A feeling of numbness in the affected limb appears with extensive damage to the nerve fibers.
In most cases, there is a decrease in pain sensitivity while maintaining tactile sensitivity. In the later stages of the development of the disease, deep hypesthesia occurs up to a complete lack of sensitivity. Redness or blanching of the skin, the appearance of a marble pattern indicates vascular disorders that occur when the vegetative fibers are directly damaged.
As a rule, increased or decreased sweating of the affected limb joins. The burning pain of a hyperplastic nature is disturbing, radiating to the entire limb involved in the process. The trophism (cellular nutrition) of tissues is disturbed due to deep vegetative disorders.
Atrophy of the muscles of the hand begins, as a rule, with the most remote or distal parts of the upper limbs. The hand takes the form of a "monkey hand" due to the defeat of the interosseous muscles and fingers. There is a complete loss of tendon reflexes, but sensitivity is preserved in the affected limb. As the disease progresses, the muscles of the neck and trunk are included in the process.
Diagnosis of atrophy of the muscles of the arm, shoulder and hand
The diagnosis does not currently cause any particular difficulties in connection with the introduction of the method of electromyography and biopsy of the affected muscles into the clinical examination. The patient is prescribed mandatory biochemical and general blood tests, urinalysis; in the blood serum, the activity of muscle enzymes is determined (mainly by the CPK method). In the urine, the indicators of creatine and creatinine are calculated. According to the indications, the patient is sent for CT or MRI of the cervicothoracic spine and brain, examination for endocrinological pathology.
Treatment of atrophy of the muscles of the arm, shoulder and hand
When choosing a method of treatment, the following factors are taken into account: the form of the disease, the severity and prevalence of the process, the age of the patient. Along with drug treatment, great importance is attached to proper nutrition, physiotherapy, courses of therapeutic massage and gymnastics, and electrotherapy. In some cases, it is appropriate to prescribe psychotherapy sessions to the patient.
Currently, there is no drug that can completely cure muscle atrophy, but the right choice of treatment method and a timely diagnosis can slow down the pathological process, restore muscle regeneration and return lost abilities to the patient. The main thing is to strictly adhere to the recommendations of the doctor.
Expert editor: Mochalov Pavel Alexandrovich| MD general practitioner
Education: Moscow Medical Institute. I. M. Sechenov, specialty - "Medicine" in 1991, in 1993 "Occupational diseases", in 1996 "Therapy".
Muscular atrophy is a process that develops in the muscles and leads to a progressive decrease in their volume and degeneration. Muscle fibers gradually become thinner, in severe cases their number decreases sharply, sometimes they completely disappear. There are primary, or simple, and secondary, or neurogenic, muscle.
Primary muscle atrophy depends on the damage to the muscle itself. It is apparently based on hereditary metabolic disorders in the form of a congenital defect in muscle or an increase in muscle membranes. However, environmental factors that contribute to the development of the disease (physical overstrain, infections, injuries) also play a significant role. Primary muscular atrophy is most clearly expressed in myopathy (see).
Secondary, or neurogenic, muscle atrophy develops when cells of the anterior horns, roots, or peripheral nerves are damaged. In the latter case, the atrophic syndrome is accompanied by a sensitivity disorder. Secondary muscular atrophy develops after an injury to the nerve trunks, with infections that affect the motor cells of the anterior horns of the spinal cord (and). In some cases, the process is hereditary. In this case, the predominantly distal location of atrophy is characteristic (muscles of the feet, legs, hands, etc.) with a slower and more benign course of the process. There are several forms of neurogenic atrophy:
With neural amyotrophy, or Charcot-Marie amyotrophy, the muscles of the feet and legs are affected (Fig.), especially the extensor and abductor muscles. The feet are deformed. The gait takes on the character of the so-called steppage: when walking, patients raise their knees high so that the hanging feet do not cling to the floor. Reflexes fade, superficial sensitivity in the distal extremities decreases. A few years after the onset of the disease, atrophy extends to the hands and forearms.
Progressive muscular atrophy of Werdnig-Hoffmann is the most severe. The disease usually begins in early childhood, often in several children in a family of apparently healthy parents. Atrophy is accompanied by loss of tendon reflexes, sharp and fibrillar twitches.
Atrophic syndrome is also observed in progressive adult muscular atrophy - Aran-Duchenne atrophy. Initially, the process is localized in the distal parts of the upper limbs. The eminences of the thumb and little finger and the interosseous muscles atrophy. A very characteristic posture of the hand in the form of a “monkey hand” is formed. Tendon reflexes disappear, sensitivity is preserved. The process is steadily progressing, spreading to the muscles of the neck and torso.
Treatment. In the treatment of muscle atrophy, the following are used: disodium salt of adenosine triphosphoric acid 1% solution of 1-2 ml intramuscularly, for a course of 30 injections; 0.5-1 ml of 5% solution intramuscularly; 0.5-1 ml of 0.01% solution every other day intramuscularly, for a course of 15-20 injections; 1 teaspoon 1-2 times a day; 0.25% solution 0.3 - 1 ml subcutaneously every other day, for a course of 10-15 injections; inside dibazol or oksazil at the rate of 0.001 g per 1 year of a child's life 1 time per day; 0.001 g for 1 year of a child's life 1 time per day (adults - 0.015 g 2-3 times a day) orally or subcutaneously 0.05% solution of 0.3-1 ml, depending on age, every other day, 10-15 injections. Transfusion of single-group blood (150-200 ml) is also used, and. Such treatment, carried out in separate courses over the course of months, each time brings some improvement, and sometimes can help stabilize the process.
Muscular atrophy is a pathological process that develops in the muscles and leads to a decrease in their volume and degeneration. An early sign of the development of an atrophic process in the muscle is the homogenization of the muscle fiber and the formation of vacuoles as a result of a violation of the colloidal structure and changes in the water exchange of muscle tissue. As the process progresses, the number of muscle fibers in the muscle decreases and their contractile part can be replaced by connective and adipose tissues, forming pseudohypertrophies. In the final stage, a sclerotic process develops in a number of muscles, leading to the development of retractions and contractures.
There are two types of muscle atrophy: primary, or simple, and secondary, or neurogenic.
primary or simple, muscle atrophy occurs due to damage to the muscle itself and develops while maintaining the structure and function of the peripheral motor neuron. It is characterized by quantitative changes in electrical excitability, the absence of fibrillar twitches. The mechanical excitability of the muscles is preserved. With this form, there are often retractions.
The etiology and pathogenesis of simple muscular atrophy has not yet been resolved. It is based, apparently, metabolic disorders. Some enzymatic metabolic defects leading to muscular dystrophies are already known. The factors leading to the development of the disease are varied: alimentary dystrophy, trauma, monotonous tension of certain muscle groups during work, long-term infections, and many chronic intoxications. The development of muscle atrophy is known in cases of hypothyroidism, after thyroidectomy and in other thyroid dysfunctions, as well as in pituitary disorders, Simmonds cachexia, Addison's disease and other endocrine disorders. Muscle atrophy can also develop as a result of prolonged immobilization of the limbs after the application of plaster for fractures, orthopedic operations, as well as with prolonged inactivity of the diseased limb, with hemiplegia and monoplegia of central origin. Undoubtedly, severe neuropsychic overstrains are also important, resulting in dynamic disruptions to somatic disorders.
In the pathogenesis of muscle atrophy, one should also take into account violations of sympathetic innervation associated with dysfunction of the central and peripheral structures of the autonomic nervous system, which also affect changes in the metabolism of muscle tissue. This is evidenced by muscle atrophy of reflex origin, which develops after surgery on the cervical sympathetic nerve or after cervical sympathectomy, as well as as a result of prolonged pain irritation during bruises, bone fractures, with painful scars and inflammatory processes that cause pathological centripetal impulses. In these cases, along with a violation of muscle trophism, other vegetative and animal symptoms develop - hypesthesia, hyperreflexia, cyanosis and coldness of the extremities, sweating, osteoporosis, etc. It is also known that when sympathetic innervation is lost, the proximal muscles, which are richer in sympathetic fibers, suffer first of all. .
The group of simple muscle atrophy also includes the so-called arthritic (arthrogenic) muscle atrophy, which develops in diseases of the joints. In this case, the muscles lying near the affected joint atrophy (for example, interosseous - for diseases of the joints of the hand, deltoid - for diseases of the shoulder joint, etc.) (see).
, final task.docx , TOR-Tests. with tasks.....doc , Lena E KR OPTION 2 Task 2 27112019.doc , 118 task.docx .
Answers:
Stamping gait, steppage, sensitive ataxia
- Main Causes: Sensitive ataxia is characteristic of lesions of the posterior columns and other levels of deep sensitivity (peripheral nerve, posterior root, brainstem, etc.).
MRI, spondylogram
Therefore, sensitive ataxia is observed in the picture of diseases such as polyneuropathy ("peripheral pseudotabes"), funicular myelosis, dorsal tabes, complications of vincristine treatment; paraproteinemia; paranesplastic syndrome, etc.)
Problem #50
The patient complains of seizures, manifested by palpitations, increased blood pressure, headache, chill-like trembling of the body, a feeling of fear, swelling of the intestines, lasts 20-30 minutes. At the height of the attack, consciousness is darkened, short tonic convulsions of the limbs occur, the pupils are dilated, the face is pale.Questions about the task:
What is the name of the described syndrome?
What structures are affected?
What are the etiopathogenetic factors of its development?
Assign an examination to the patient to clarify the nosological diagnosis.
Determine the treatment for such attacks.
Task number 51
Active movements of the arms and legs of the patient are absent. Muscle tone is increased selectively: on the arms, mainly in the flexors of the forearms and pronators of the hands, on the legs - in the extensors of the legs. Tendon and periosteal reflexes from the arms and legs are high, clonuses of the patella and feet, skin abdominal reflexes are absent. Pathological reflexes of the Babinsky and Rossolimo groups, both carpal and foot, are caused from both sides. Protective reflexes (reflexes of spinal automatism) are noted. All types of sensitivity are lost in the region of the occiput, neck, trunk and extremities (according to the conduction type). Retention of urine and stool. Severe respiratory disorders (dyspnea, difficulty coughing, sneezing) and hiccups. Chest X-ray revealed a sharp limitation of diaphragm mobility.Questions about the task:
Determine where the lesion is located.
Substantiate the topical diagnosis.
Describe the conductive type of sensitivity disorder.
How are protective reflexes studied and what is their clinical significance?
In what diseases can such symptoms be observed?
Answer:
1.2. The entire diameter of the spinal cord at the level of the 1st-4th cervical vertebrae is affected. The defeat of the pyramidal tracts at this level is evidenced by central paralysis of the arms and legs, accompanied by a sharp restriction of the mobility of the diaphragm, respiratory distress and hiccups. The lower level of the lesion corresponds to the 4th cervical segment. It cannot descend to the 5th cervical segment because reflexes from the tendons of the biceps muscles are high. Confirmation of the upper level of damage to the spinal cord is the loss of all types of sensitivity from the back of the head down due to the blockade of the conductors of sensitivity at the level of the specified segment.3Conductor type of sensory impairment (sensitivity disorder throughout below the level of the lesion of the conduction path), occurs when: the posterior and lateral cords of the spinal cord are damaged; brain stem; visual hillock (thalamic type); posterior third of the leg of the internal capsule; white subcortical matter. The conductor type of sensitivity disorder is characterized by a decrease or loss of its various types from any level down.
4 Technique of inducing protective reflexes: when pinching, pricking or applying a drop of ethyl ether to a straightened paralyzed leg, the latter involuntarily bends. Protective reflexes are one of the symptoms of damage to the pyramidal tract.
Problem #52
The patient has Horner's triad on both sides. There are no active movements of the arms and legs. Atony and atrophy of the muscles of the shoulder girdle and arms are observed. The tone of the muscles of the legs is increased. Bicipital, tricipital and carporadial reflexes are absent. High knee and Achilles reflexes, clonuses of the patella and feet. Skin abdominal reflexes are depressed. Pathological reflexes of the Babinsky and Rossolimo groups are evoked on both sides. Lost all kinds of sensitivity from the area of the shoulder girdle downwards (according to the conductive type). Retention of urine and stool.Questions about the task:
Determine where the defeat is.
Substantiate the topical diagnosis.
What are the symptoms of Horner's triad?
What diseases can cause this syndrome?
Assign an examination to the patient and conduct a differential diagnosis of diseases accompanied by these disorders.
1. The entire diameter of the spinal cord is affected at the level of the 5-8th cervical and 1-2nd thoracic segments.
2. Peripheral paralysis of the arms indicates damage to the anterior horns of the 5-8th cervical and 1-2nd thoracic segments, since the motor cells of the anterior horns of these segments innervate the muscles of the upper limbs. At the same level in the spinal cord (with the preservation of the function of the cranial nerves), the pyramidal tracts were affected, which is manifested by central paralysis of the legs, as well as urinary and stool retention. The loss of all types of sensitivity from the level of the shoulder girdle down is the result of the defeat of all conductors of sensitivity at the level of the 5th cervical segment. The presence of Horner's triad on both sides is the result of damage to the lateral horns of the 8th cervical and 1st thoracic segments (ciliospinal center), which innervate three smooth muscles: the orbital, dilating pupil, and cartilage muscle of the upper eyelid. The arc of the bicipital reflex closes at the level of the 5th-6th cervical segments.
Nervous system
(systema nervosum)
The nervous system performs the following functions:
- provides communication of the organism with the external environment;
- regulates and coordinates the functions of all organs,
ensuring functional unity and integrity
organism. Conventionally, the nervous system is divided into sections
(according to functional and topographical principles):
Functionally:
Nervous system
Sympathetic Parasympathetic
part part
Topographically:Nervous system
Central nervous Peripheral nervous
system system
The central nervous system (systema nervosum entral) includes the brain
brain (encephalon) and spinal cord (medulla spinalis).
The brain and spinal cord are composed of nervous tissue, in which nervous
cells and neuroglia. A specific function is performed by a nerve cell - a neuron
(neuronum, neurocytus)
brain stem
(truncus cerebri)
Sylvius aqueduct - A
3rd ventricle – b
4th ventricle - V
diencephalon- G
1.- thalamus, 2- epiphysis, 3- handles
4- cranked bodies, 5- decussation
optic nerves, 6- pituitary gland,
7 - mastoid bodies, 8 - gray
tubercle with funnel
midbrain– d
9 - interpeduncular fossa, 10 - legs of the brain,
11-plate of the quadrigemina
Bridge–e
12- bridge, basilar sulcus, 13- legs of the bridge,
14 - cavity of the 4th ventricle, 15 - lower legs of the cerebellum,
21- superior cerebellar peduncles
Medulla- and
(medulla oblongata)
16 - pyramids, 17 - olives, 18 - cross of pyramids,
19- wedge-shaped bundle, 20- gentle bundle
H NERP NERVES
Name
plexus
Location
Main
Kopchikov.
Divisions of the autonomic nervous system.
Nervous system
(Latin names)
| medulla spinalis | - spinal cord |
| dura manner spinalis | - hard shell |
| arachnoidea spinalis | - spider web |
| pia mater spinalis | - soft shell |
| encephalon | - brain |
| corpus callosum | - corpus callosum |
| bulbi olfactorii | - olfactory bulbs |
| rhombencephalon | - rhomboid brain |
| myelencephalon | - medulla |
| metencephalon | - back brain |
| mesencephalon | - midbrain |
| prosencephalon | - forebrain |
| diencephalon | - diencephalon |
| telencephalon | - telencephalon |
| pons | - bridge |
| cerebellum | - cerebellum |
| nn.olfactorii | - olfactory nerves |
| n.opticus | - optic nerve |
| n.oculomotorius | - oculomotor |
| n.trochlearis | - trochlear nerve |
| n.trigeminus | - trigeminal nerve |
| n.abducens | - abducens nerve |
| n.facialis | - facial nerve |
| n.vestibulocochlearis | - vestibulocochlear nerve |
| n.glossopharyngeus | - glossopharyngeal nerve |
| n.vagus | - nervus vagus |
| n.accessorius | - accessory nerve |
| n.hypoglossus | - hypoglossal nerve |
| plexus cervicalis | - cervical plexus |
| pl. brachialis | - brachial plexus |
| pl.lumbosacralis | - lumbosacral plexus |
| pl.lumbalis | - lumbar plexus |
| pl. sacralis | - sacral plexus |
| n.ishiadicus | - sciatic nerve |
| n.femoralis | - femoral nerve |
| n.axillaris | - axillary nerve |
| n.ulnaris | - ulnar nerve |
| n.radialis | - radial nerve |
| n.medianus | - median nerve |
cranial nerves
 |
check yourself
(work in pairs)
Solve problems on the spinal nerves:
1. The patient has partial atrophy of the deltoid muscle, the impossibility of abducting the arm to a horizontal position. Which SMN is affected?
2. The patient cannot put one leg on the other. Which SMN is affected?
3. When squeezing the hand into a fist 11 and 111, the fingers do not bend. Which SMN is damaged?
4. The patient has lost the knee reflex, the anterior thigh muscle group is partially atrophied. Which SMN is damaged?
5. When squeezing the hand into a fist, it is not possible to bend fingers 2 and 3. Which SMN is damaged?
6. The patient has a "hanging" foot, a disorder of sensitivity on the back of the foot. Which SMN is damaged?
7. The patient has a disorder of skin sensitivity of a part of the back surface of the hand and fingers 1.2 parts 3. Which SMN is damaged?
8. The patient has a disorder of skin sensitivity on the lateral surface of the thigh. Which SMN is damaged?
Solve problems on cranial nerves:
1. The patient has dizziness, a feeling of instability, falling in the Romberg position. The branch of which CG is damaged?
2. The patient has impaired sensitivity of the forehead skin. What FMN is damaged?
3. The patient's head is lowered on the chest, lifting and turning it is difficult. What FMN is damaged?
4. The patient has pain in all the upper teeth of one half, as well as in the middle parts of the face. What FMN is damaged?
5. The patient has ptosis / drooping of the upper eyelid /. What FMN is damaged?
6. After otitis, the child has partially lost his taste. What FMN is damaged?
7. The patient has internal strabismus. What FMN is damaged?
8. After inflammation of the parotid gland, the child's face was distorted. What FMN is damaged?
9. U. As a result of an osteoma/tumor of the bone tissue/base of the skull, the movements of the eyeball were disturbed in a patient. What CNs are affected? Where is the osteoma located?
10. As a result of a hemorrhage in the brain stem, a patient developed ptosis /drooping of the upper eyelid/. What FMN is damaged? In what part of the brainstem did the hemorrhage occur?
check yourself
THE LENGTH OF THE SPINAL CORD IS
a) 35 - 40 si
b) 40 - 45 cm
c) 45 - 50 cm
INTERCUTIVE SENSITIVE NEURONS OF THE SPINAL CORD ARE IN THE GRAY SUBSTANCE
a) lateral horns
b) frequent horns
c) back horns
d) spinal nodes
IN THE WHITE MATTER OF THE posterior cords of the spinal cord pass
a) descending pathways
b) ascending pathways
c) ascending and descending paths
d) neither one nor the other.
DURING BILATERAL TRANSACTION OF THE ANTERIOR ROOTS OF THE SPINAL CORD IN THE DOG, SENSITIVITY
a) disappears
b) goes down
c) does not change
d) rises
THE MIDDLE BRAIN IS CONNECTED WITH THE CERENELS
a) brain sails
b) upper legs
c) middle legs
d) lower legs
IN THE GRAY MATTER OF THE UPPER COLLICLES THERE ARE:
a) subcortical visual centers
b) subcortical auditory centers
c) red nuclei
d) black matter
RETICULAR FORMATION IS A STRUCTURE
a) executive (motor)
b) sensory (perceiving)
c) executive and sensory
d) tuning
THE FRONTAL LOBE IS LIMITED FROM THE PARIETAL, sulcus
a) central (Roland)
b) lateral (silviaoy)
c) top: frontal
d) precentral
THE ZONE OF SKIN SENSITIVITY IN THE BRAIN CORTEX IS IN
a) precentral gyrus
b) postcentral gyrus
c) superior temporal gyrus
d) occipital zone
THE COMPOSITION OF THE BASAL NUCLEI OF THE LARGE BRAIN IS NOT INCLUDED
a) striatum
b) amygdala
c) internal capsule.
d) fence
CEREBRAL SPINAL FLUID FORM
a) hard and choroid
b) arachnoid
c) sinuses of the dura mater
d) choroid plexus of the ventricles
THE EMISSIONS ON ITS SURFACE DO NOT APPLY TO THE BRAIN
a) pyramids
c) tubercles of thin and wedge-shaped nuclei
d) tubercles of the quadrigemina
CARDIOVASCULAR REFLEXES ARE CARRIED OUT MOSTLY
a) medulla oblongata
b) spinal cord
c) thalamus
DOES NOT APPLY TO THE HYPOTHALAMUS
a) cranked bodies
b) a gray tubercle with a funnel
c) mastoid bodies optic chiasm and optic tract
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 |
Higher nervous activity (HNI)- the activity of the higher parts of the central nervous system, providing the most perfect adaptation of animals and humans to the external environment.
Psyche- a subjective image of the objective world, a reflection of reality in the brain.
Thinking, or rational activity is the most complex type of brain activity of the body in the process of adapting to new conditions and solving new life problems.
Reasoning activity allows you to capture patterns that connect objects and phenomena of the environment, and use them in new conditions in your behavior.
The adaptive nature of behavior is determined by the conditioned reflex activity of the organism, formed on the basis of unconditioned reflexes (according to I.M. Sechenov and I.P. Pavlov).
Emotions- experiences in which a person's attitude to the world around him and to himself is manifested.
Emotions.
For a more specific description of the picture of the disease, we present data on the patient observed in our clinic.
Patient A., 37 years old, was admitted to the clinic with complaints of significant weakness, fatigue, especially when walking, difficulty in unclenching a clenched fist. The patient over the past 10 years has lost 18 kg in weight, despite normal nutrition. In addition, she complained of bouts of intense pain in the epigastric region.
Family members, according to the patient and her relatives, did not have any myotonic phenomena, no muscle atrophy, no cataracts, as well as severe endocrine pathology. The patient at the age of 21 got married; did not have a pregnancy after the first abortion.
At the age of twenty, she noticed that when shaking hands she held someone else's hand, because she could not unclench her fingers; later she noticed that after a long silence she could not immediately open her mouth; after a long stay in a sitting position, it was difficult to get up from a chair. At first, the patient did not pay much attention to all these phenomena and considered herself practically healthy.
At the age of 27, attacks of pain appeared in the epigastric region, spreading to the entire abdomen. Each such attack lasted about 6 hours. Over the next 7 years, she had 4 similar attacks of extremely intense pain. During one of them, she experienced collapse phenomena.
The patient is extremely asthenic, with a long thin neck, sunken cheeks; the face therefore appears sharply flattened; the upper jaw is somewhat prognathic; the skin is pale, there is a slight bilateral exophthalmos. The hair on the head is thin, very sparse, with many gray hairs. The upper eyelids are somewhat drooping. The area of the temporal muscles sunk, they are very weakly palpable with a tight closure of the mouth; masseters are noticeably atrophic, when teeth are bared, the lips barely move apart, a smile is impossible due to atrophy of the muscles of the face.
The patient's voice is weak, hoarse, aphonic. There is a sharp atrophy of the sternocleidomastoid muscles, they are barely palpable; also atrophic muscles - extensors of the neck. When tilting the head back, the patient can bring her out of this position only with the help of her hands. There is also atrophy of the muscles of the shoulder girdle, to a lesser extent of the legs and pelvic girdle. Tendon and periosteal reflexes on the hands are not evoked, Achilles reflexes are absent, knee reflexes are very low. When struck with a hammer on the tongue, a depression and a sharp roller are formed; when hitting the tenar, the thumb is adducted and opposed to the hand for a few seconds. Fingers clenched into a fist with difficulty, slowly unbend. When irritated by galvanic and faradic current of the muscles of the hand and forearm, a myotonic reaction is observed. A chronaximetric study of the orbicular muscle of the mouth and the superficial flexor of the fingers shows a slight increase in the threshold of excitability in normal chronaxia. With myographic recording: contraction of the flexor of the III finger in response to stimulation by induction current sometimes lasts a certain time after the cessation of irritation, forming a small plateau on the myogram.
The study of sensitivity did not reveal noticeable deviations from the norm. The sharp cooling of the hands attracted attention; dermographism - pale pink, unstable, Ashner's test was accompanied by a decrease in the pulse by 10 beats per minute, on the right side, the Khvostek phenomenon was intermittently evoked. Injections of pilocarpine and epinephrine produced no noticeable reaction.
Menstruation is scanty, the uterus is smaller than normal. The thyroid gland is slightly enlarged. The Turkish saddle is within the normal range. The thymus gland, judging by the X-ray data, is not enlarged.
The main metabolism is minus 9%, the specific dynamic effect of the protein is + 9%, i.e., it is noticeably reduced. In urine: specific gravity 1013, traces of protein, 50-60 leukocytes and 1-2 leached erythrocytes per field of view. Blood pressure 110/50. Blood test: hemoglobin 48%, erythrocytes 3,600,000, color index 0.6. Leukocytes - 4800; basophils - 0, eosinophils - 5%, young - 3%, stab - 5%, segmented - 47%. lymphocytes - 40%. The Wasserman reaction in the blood is negative. Residual nitrogen - 47.7 mg%. In the blood, potassium - 22.36 mg%, calcium - 14 mg%.
On palpation, pain in the duodenal region was determined, but X-ray examination did not reveal an organic pathology of the abdominal organs. Examination of gastric juice: total acidity - 78, free acid - 56, bound - 16, reaction to the presence of blood - negative.
The patient was discharged without noticeable improvement (treatment: strychnine injections, vitamins).
After 3 years, she was again in the clinic, and an increase in the process was noted. Three years later, the patient died of pneumonia.
In the above observation, there were almost all the main symptoms of myotonic dystrophy: widespread dystrophic changes in many muscle groups, areflexia, the presence of myotonic phenomena during active movements, as well as myotonic reaction with mechanical and electrical irritation of the tongue and thenar muscles, ptosis of the eyelids, speech changes, hair thinning , vasomotor disorders, pronounced general asthenia, emaciation, reduced basal metabolism, some enlargement of the thyroid gland. Attacks of pain in the epigastric region can also be regarded as vegetative crises, given the increased acidity of gastric contents. (In one patient, Kurschmann also noted long-term disorders of the gastrointestinal tract.)
Rohrer gave a detailed analysis of 82 cases of myotonic dystrophy. The tables compiled by him make it possible to analyze a number of basic symptoms characterizing the clinical picture and the course of the disease.
Based on his data, the number of women accounted for only a quarter of the total number of patients. 59 cases were sporadic, and 23 had familial disease. (Rohrer admits that the latter number should have been slightly increased due to the inaccuracy of the anamnestic data; but, in any case, the number of family diseases did not exceed half of the total number of patients.)
In 72 cases, there was atrophy of the muscles of the forearm as an initial symptom of the disease, and only in 7 cases the disease began with damage to the muscles of the face. Subsequently, the muscles of the face and neck atrophied almost simultaneously and approximately to the same extent. Noticeable atrophy of the sternocleidomastoid muscles and masticatory muscles was detected in 30 cases. There were indications of tongue atrophy in 8 cases. Changes in speech to one degree or another were noted in 39 patients. The muscles of the legs were atrophic to a greater or lesser extent in 39 patients, while the peroneal muscle group was affected earlier and more intensively. The muscles of the shoulder girdle were involved in the process only in isolated cases.
Myotonic phenomena in the muscles were noted as the primary symptom in 32 patients, and dystrophic phenomena in 33 patients. Myotonic phenomena were caused by mechanical irritation on the muscles of the hand in 47 cases, and on the tongue in 37 cases.
Testicular atrophy was found in 16 male patients (out of a total of 55).
Decrease or disappearance of tendon or periosteal reflexes was noted in 40 cases. Vasomotor phenomena in the form of acrocyanosis, cold extremities - in 15 cases. In 18 patients, early cataract was detected. Khvostek's phenomenon was found in 12 patients, a slight enlargement of the thyroid gland - in 1/6 of the total number of patients.
From the data given by Rohrer, it can be judged that myotonic dystrophy is obviously not such a rare disease.
Hoffmann believes that 9-10% of patients with myotonia have symptoms of myotonic dystrophy. According to more accurate statistics, myotonic dystrophy is much more common than myotonia.
Of interest are the data of Walton and Nattras, who also provide, on the basis of their own clinical observations, some data regarding the features of the course of myotonic dystrophy. Of the 15 cases (8 men, 7 women), the onset of the disease between 20-50 years was noted in 11 patients. In 3 families there was a hereditary transmission of the disease (according to the dominant type).
All 15 patients had varying degrees and sequences of damage to the muscles of the extremities. Usually, weakness and atrophy first occurs in the extensors and flexors of the forearm, and then in the tibial and peroneal muscles. In some cases, deltoid muscles, pectoral and anterior serratus muscles, small muscles of the hand are later involved in the process.
Myotonic phenomena in the extremities occurred in 13 cases; in one case only in cold weather.
Indications of a decrease in libido were found in 12 cases; testicular atrophy was found in 6 sick men (out of 8). Cataract was found in 6 patients, and in the remaining 9 people, changes in the lens were established during the study using a slit lamp. Tendon reflexes were completely absent in 4 patients, partially in 9. Ptosis was noted in 14 cases, fades myopatica in all cases, speech disorders in the form of dysarthria or aphonia in 14 cases. Often there is early baldness.
Unsharply pronounced mental changes were observed in most cases.
Flow
Interesting data on the course of the disease. Most authors note a significant preservation of motor functions for a relatively long time; patients walk, maintain a certain working capacity. The course of the disease, as a rule, is slowly progressive, without noticeable suspensions of the process or remissions. So, according to Walton and Nattras, after 10 years of illness, 10 patients moved well, after 20 years - 1. Of the patients observed by them, four died: one at 58 years old, others at the age of 38-49 years.
All researchers note a relatively frequent disease of persons suffering from myotonic dystrophy, pulmonary tuberculosis and pneumonia. These infections in some cases are the cause of death of patients.