Cerebral palsy. Massage for spastic form of cerebral palsy

Official data

Last name, first name: ...

Age: 5 years old.

Home address:

Date of admission to the clinic:

Curation start date: 05/29/2008.

Complaints

The inability to stand, move independently, limited movements in both legs and arms. On mental retardation: he does not speak well.

Disease history.

According to her grandmother, the girl has been ill since she was 6 months old, when in 2003 her parents noticed a delay in physical development: the child did not sit up on his own, he did not hold his head well. From the age of 7 months, there were signs of movement restriction in the arms first, then in the legs. Have addressed to the doctor. At the age of 9 months, an examination was carried out and a diagnosis was made: cerebral palsy, spastic tetraplegia. From the age of 1, every 6 months, the girl undergoes a course of planned treatment.

She is currently undergoing treatment in the inpatient department of the Children's City Hospital No. 5, the neuropsychiatric department.

Anamnesis of life.

Child from first pregnancy. The pregnancy proceeded normally. The mother did not tolerate infectious diseases during pregnancy. Nutrition is satisfactory, vitamin D2 received in the required quantities.

Childbirth I, at term (40 weeks), independent, rapid, without anesthesia. Child at birth m = 3100 g, l = 51 cm, head circumference = 34 cm, chest circumference = 34 cm; she screamed immediately, she was attached to her chest in the delivery room. Apgar score 7 points. The umbilical cord was removed on the 3rd day. She was discharged home on the 5th day. Weight at discharge 3000 g. Artificial feeding.

Development of motor skills: the girl began to hold her head from 5 months. From 6 months he rolls over on his stomach, from 8 months he sits.

Mental development: smiles from 3 months old, started to walk from 5 months old, pronounce separate syllables from 10 months old, utters the first words from 1.5 years old.

The teeth erupted at 6 months, by the year the child has 8 teeth.

Didn't attend kindergarten.

Family history: tuberculosis, alcoholism, sexually transmitted diseases, relatives denies.

Past illnesses.

Chicken pox - 3 years;

SARS - from 3 years old 1-2 times a year in the autumn-winter period;

There were no operations or blood transfusions.

Vaccinations according to an individual schedule.

Allergological anamnesis is not burdened.

Genealogical tree

Conclusion: heredity is not burdened.

The present state of the patient

General inspection.

The general condition of mild severity, the position of the body is natural. Consciousness is clear. The behavior is active. The constitutional type is asthenic.

The physical development of the child is below average, proportional, harmonious.

Skin and PZhK.

The skin is pink. Severe cyanosis and areas of pathological pigmentation are not observed. Moisture and elasticity of the skin are normal. There are no rashes, scratches, scars, visible tumors. Visible mucous membranes are pink, clean, there is no icteric staining of the frenulum of the tongue and sclera. The conjunctiva of the eyes is pink. Correctly shaped fingers. Fungal infection, increased fragility of the nail plates were not noted.

The layer of subcutaneous fat is moderately developed, evenly distributed. The thickness of the skin fold in the umbilical region was 1 cm. No edema was detected. No crepitus was found.

During external examination, the lymph nodes are not visualized. Occipital, parotid, chin, submandibular, cervical, supraclavicular, subclavian, axillary, ulnar, inguinal, popliteal lymph nodes are not palpable.

Bone-articular system.

The joints are not deformed, painless on palpation, seals are not revealed. Mobility restrictions are not observed. There is no crunch or pain when moving.

The physique is correct, there are no deformations and deformities of the trunk, limbs and skull. The shape of the head is oval. Posture is correct. Body halves are symmetrical. There are no chest deformities. The angles of the shoulder blades are directed downward.

The physiological curves of the spine are sufficiently pronounced, there are no pathological curves.

The bones are not deformed, painless on palpation. The terminal phalanges of the fingers are not thickened. "Bracelets", "strings of pearls" - not defined.

Teething is timely, the condition of the teeth is normal.

Respiratory system.

The skin is pale pink, Frank's symptom is negative. The mucosa of the oropharynx is bright pink, without plaque, the tonsils are not enlarged. Nasal breathing is not disturbed, there is no discharge from the nose. The respiratory rate is 20 times per minute. The chest is not deformed, symmetrical, participates in the act of breathing. The type of breathing is mixed.

Palpation of the chest is painless. The chest is moderately rigid. Voice trembling is carried out in the same way in symmetrical areas. Excursion of the chest - 6 cm. The skin folds on the chest are symmetrical.

Percussion.

Percussion sound clear pulmonary over all symmetrical points.

Topographic percussion without features.

The cardiovascular system.

The skin is flesh-colored, no deformation in the chest area is detected. The apex beat is determined in the 5th intercostal space, 1 cm outward from the midclavicular line. Heart hump, cardiac impulse are not defined. Visible pulsation in the area of ​​large vessels is not determined.

Palpation. The apex beat is palpated in the 5th intercostal space 1 cm outward from the midclavicular line; prevalence 1x1 cm; apical impulse of moderate height, moderate strength.

Pulse - correct, firm, full, rhythmic. Heart rate = 90 beats / sec.

Edema is not defined.

Percussion, without features.

Auscultation. Heart tones are crisp, clear, the timbre is soft, normocardia, the rhythm of the tones is correct. The ratio of tones is preserved, no additional tones are heard. Noises are not heard.

Arterial pressure:

Right hand - 110/70 mm Hg. Art.

Digestive organs.

Inspection. Lips pale pink, moist. Cracks, ulcerations, rashes are absent. The tongue is pink, of normal shape and size, the back of the tongue is not lined, the papillae are well defined. The mucous membrane of the tongue is moist, without visible defects. The gums are pink, there is no bleeding or defects. The back wall of the pharynx is not hyperemic, the tonsils are not enlarged. There is no smell from the mouth.

The abdomen is normal, symmetrical. Bloating is not observed. Peristaltic movements are not visible. The umbilicus is retracted. Collaterals on the anterior surface of the abdomen and its lateral surfaces are not expressed. Scars and other changes in the skin are not observed. Hernias are not revealed. The abdominal muscles are involved in breathing.

Palpation.

On superficial palpation: the abdomen is not tense, painless. Hernial ring is not defined. Shchetkin-Blumberg's symptom is negative, Voskresensky's symptom is negative, Dumbadze's symptom is negative. Mendel's sign is negative. The divergence of the abdominal muscles, hernias of the white line was not revealed.

Deep methodical sliding palpation according to Obraztsov-Strazhesko. With deep palpation, pain is noted in the epigastric region and in the navel. A large curvature of the stomach is palpated on both sides of the midline of the body 3 cm above the navel in the form of a roller. The gatekeeper is not determined by palpation. The sigmoid colon is palpated in the left iliac region in the form of a smooth dense cylinder 1.5 cm thick. The caecum is palpated in the form of a moderately tense cylinder 1.5 cm in diameter, painless. The ascending colon and the descending colon are not thickened, 1.5 cm in diameter. The transverse part of the colon is palpated as a cylinder of moderate density 1.5 cm thick one centimeter above the navel, mobile, painless.

On palpation, the liver is soft, smooth, painless, the edge is sharp, located 1 cm below the edge of the costal arch. The spleen is not palpable.

Urinary system.

The skin in the lumbar region is flesh-colored, swelling is not determined. There are no edema.

The kidneys are not palpated.

The bottom of the bladder percussion is not determined. The reduced symptom of Pasternatsky is negative.

Neurological status

The state of the psyche.

Contact with the girl is established with difficulty. Mental development below age. Intelligence is reduced. Speech is difficult, monosyllabic. Vocabulary is poor. Reading, writing, gnosis and praxis cannot be assessed.

Functions of the cranial nerves.

1st pair - olfactory nerves, 2nd pair - optic nerve: the functions could not be investigated.

3rd, 4th, 6th pairs - oculomotor, trochlear, abducens nerves: the width of the palpebral fissures is normal. The size of the pupil is about 4 mm, correct, round; preserved direct reaction to light, friendly reaction from the other eye. The reaction to convergence and accommodation is preserved.

5th pair - trigeminal nerve: paresthesia and pain in the area of ​​innervation of the trigeminal nerve were not detected. The sensitivity of the skin of the face is not changed. Pressure sensitivity of nerve exit points (Valle's points) is normal. The condition of the chewing muscles (movement of the lower jaw, tone, trophism and strength of the chewing muscles) is satisfactory.

7th pair - facial nerve: the symmetry of the face at rest and during movement is preserved. Lagophthalmos, hyperacusis are absent. Lacrimal function is not broken.

8th pair - vestibulocochlear nerve: no tinnitus. Auditory hallucinations were not identified.

9-10th pair - glossopharyngeal and vagus nerves: no pain in the throat, tonsils, ear. Phonation, swallowing, salivary function, pharyngeal and palatine reflexes were within normal limits.

11th pair - accessory nerve: raising the shoulder girdle, turning the head, approaching the shoulder blades, raising the arm above the horizontal are impaired due to the presence of spastic paralysis of the arms.

12th pair - hypoglossal nerve: the tongue is clean, moist, mobile; the mucous membrane is not thinned, normal folding; fibrillar twitchings are absent.

motor functions.

Active and passive movements are limited. Identified hypertonicity in all limbs. Tendon reflexes from the biceps, triceps muscles, carporadial reflexes from both hands are enhanced. Patellar, Achilles, plantar reflexes from both legs are enhanced.

Skin reflexes: abdominal upper, middle, lower - positive.

Pathological reflexes: Babinsky, Oppenheim, Gordon, Schaeffer, Rossolimo, Bekhterev, Zhukovsky are positive on all limbs.

Symptoms of oral automatism: labial, nasolabial, palmar-oral, negative.

Coordination of movements is difficult to assess due to the condition of the child.

Sensitivity.

Pain, tactile sensitivity is approximately not violated. Anesthesia, hypoesthesia are absent. Segmental and conductive type of sensory impairment were not identified.

meningeal symptoms.

Rigidity of the muscles of the neck - muscle tone is not increased, Kernig's symptom, Brudzinsky's (upper, lower, middle) are absent.

Vegetative-trophic sphere: temperature is normal, sweating is intense on the palms and feet. The subcutaneous fat layer is moderately developed.

Preliminary diagnosis and its justification

Based on complaints about the inability to stand and move independently, limited movements in both legs and arms, mental retardation and neurological examination data, it can be assumed that the nervous system is involved in the pathological process.

Syndromes identified:

Syndrome of spastic tetraplegia: based on complaints of the inability to stand, move independently, limited movements in both legs and arms and on the basis of objective data (active and passive movements are limited. Hypertonicity was detected in all limbs. Tendon reflexes from the biceps, triceps muscles, carporadial from both Strengthened knee, Achilles, plantar reflexes from both legs are strengthened Pathological reflexes: Babinsky, Oppenheim, Gordon, Schaeffer, Rossolimo, Bekhterev, Zhukovsky are positive on all limbs).

Syndrome of mental retardation: based on the history (the girl began to hold her head from 5 months. From 6 months she rolls over on her stomach, from 8 months she sits, she smiles from 3 months, she began to walk from 5 months, to pronounce separate syllables from 10 months, from 1.5 years she pronounces the first words) and objective data (contact with the girl is difficult to establish, mental development is below age, intelligence is reduced, speech is difficult, monosyllabic, vocabulary is poor).

Based on the identified syndromes, a diagnosis can be assumed: infantile cerebral palsy, spastic tetraplegia, severe course, stage of rehabilitation.

Topical diagnosis and its rationale

Considering the data of neurological examination (hyperreflexia, hypertension, positive pathological reflexes on all limbs - spastic (central) tetraplegia), it can be assumed that the pathological focus is located at the level of the brain.

The presence of central paralysis, together with mental disorders (low mental development, reduced intelligence) suggests the presence of a pathological focus in the frontal lobe of the cerebral cortex, and on both sides, since the disorders were detected symmetrically on both sides.

Plan of additional research methods

Laboratory methods:

General blood analysis;

Blood chemistry;

General urine analysis;

Feces on eggs of worms;

Instrumental methods:

Results of additional research methods.

General blood analysis:

Leukocytes - 5.2 G/l

Segmented - 56%

Eosinophils - 2%

Lymphocytes - 38%

Monocytes - 4%

ESR - 4 mm/h

General urine analysis:

Specific gravity - 1023

Color straw yellow

The reaction is sour

Protein - negative

Sugar - negative

Leukocytes - 3-4 in the field of view

Conclusion: urine parameters without pathological abnormalities.

Feces on the eggs of worms - "negative".

Blood chemistry:

Total protein - 72.0

β-lipoproteins - 44 units

ALT - 16 Ukat/1

ASAT - 36 Ukat/1

Bilirubin - 11.4 µmol/l

Alkaline phosphatase - 532 U/l

GGTP - 28 U/l

Whey sugar - 4.4

Conclusion: the biochemical composition of the blood without pathological abnormalities.

Differential Diagnosis

Volumetric formation of the brain.

Common signs of the formation of the brain and cerebral palsy is the presence of focal neurological symptoms, mental disorders that are present in our case.

But brain tumors are characterized by shell symptoms: impaired consciousness, headaches; symptoms of increased intracranial pressure, which was not detected in our case. Also, the formations of the central nervous system are characterized by a progressive course of the disease, with a gradual increase in the clinic, the absence of signs of regression. In our case, the girl's condition is stable, without progression of neurological symptoms.

In addition, for the development of such a clinic (spastic tetraplegia), there must be a bilateral lesion of the cerebral cortex, which is extremely rare.

Treatment

Medical treatment of cerebral palsy

Medicines are used in the treatment of an acute period of brain damage in a newborn child, mainly in the first half of life.

In the formation of cerebral palsy, drug treatment is mainly prescribed for those patients in whom cerebral palsy is accompanied by convulsions, and is also sometimes used to reduce muscle spasticity and spontaneous movements.

To combat seizures, two groups of medicines are used:

Anticonvulsants, which quickly stop seizure activity and prevent its recurrence. There are a large number of drugs in this group, which differ in the mechanism of action and require long-term treatment.

Drugs of the benzodiazepine group are used in emergency cases to stop frequent convulsions or status epilepticus. They act on the chemical processes in the brain. The most common of these is diazepam.

In the treatment of cerebral palsy, medications are also sometimes used to reduce muscle spasticity, especially after orthopedic interventions.

For this purpose, the following medicines are most often used: diazepam, which acts as a general relaxant of the brain and body; baclofen (lioresal), which blocks signals (commands to contract) from the spinal cord to the muscles; and dantrolene, which affects the process of muscle contraction. When taken in pill form, these medications can reduce muscle tone for only a short period of time. Their benefits for a long-term decrease in muscle tone have not yet been proven by anyone. These drugs can cause significant side effects, such as drowsiness or allergic reactions, and their effect on the child's nervous system is not yet fully understood.

The introduction of botulinum toxin A can also be attributed to drug treatment.

Physical rehabilitation.

One of the most important methods of treating cerebral palsy is physical rehabilitation, which begins in the first months of a child's life, immediately after the diagnosis is made. At the same time, sets of exercises are used aimed at two important goals - to prevent weakening and atrophy of the muscles due to their insufficient use, and also to avoid the development of contractures, in which spastically tense muscles become inactive and fix the patient's limbs in a pathological position.

Surgery.

One of the additional methods of treatment of cerebral palsy are surgical operations. The most common of these are orthopedic interventions aimed at eliminating muscle shortening and bone deformities. The purpose of these operations in a child with the potential to walk is to improve his ability to move. For children who do not have the prospect of independent walking, the goal of surgical intervention may be to improve the ability to sit, facilitate the performance of hygienic functions, and, in some cases, eliminate pain syndromes.

RCHD (Republican Center for Health Development of the Ministry of Health of the Republic of Kazakhstan)
Version: Archive - Clinical Protocols of the Ministry of Health of the Republic of Kazakhstan - 2010 (Order No. 239)

Other type of cerebral palsy (G80.8)

general information

Short description

Infantile cerebral palsy (CP)- a group of syndromes that are the result of brain damage that occurred in the perinatal period.

A characteristic feature of cerebral palsy is a variety of motor disorders that are manifested by paralysis, discoordination of movements, often combined with various disorders of speech, mentality, and sometimes epileptic seizures.

Cerebral palsy is a polyetiological disease of the brain that can occur during its intrauterine formation, during childbirth and the newborn, as well as in the postnatal period. Cerebral palsy is the most common cause of childhood disability (30-70%), among which diseases of the nervous system are in the first place. In most cases, the physical condition of patients improves, but activity restrictions remain: reduced mobility, speech disorders, and others.

According to the literature, the incidence of cerebral palsy in the population is 2 per 1000 newborns; according to L. O. Badalyan (1987) - from 3 to 6 cases per 1000 births. The main causes of cerebral palsy are associated with the development of pregnancy and childbirth (perinatal period): chronic diseases of the mother, infectious diseases, medication during pregnancy, premature birth, protracted labor, hemolytic disease of the newborn, entanglement of the umbilical cord and others.

Protocol"Cerebral palsy"

ICD-10 code:

G 80.0 Spastic cerebral palsy

Congenital spastic (cerebral)

G 80.1 Spasmodic diplegia

G 80.2 Childhood hemiplegia

Classification

Classification

G 80 Infantile cerebral palsy.

Includes: Little's disease

Excludes: hereditary spastic paraplegia

G 80.0 Spastic cerebral palsy.

Congenital spastic palsy (cerebral)

G 80.1 Spasmodic diplegia.

G 80.2 Children's hemiplegia.

G 80.3 Dyskinetic cerebral palsy.

G 80.8 Another type of cerebral palsy.

Mixed syndromes of cerebral palsy.

G 80.9 Cerebral palsy, unspecified.

Classification of cerebral palsy(K. A. Semenova et al. 1974)

1. Double spastic hemiplegia.

2. Spastic diplegia.

3. Hemiparesis.

4. Hyperkinetic form with subforms: double athetosis, athetous ballism, choreatetous form, choreic hyperkinesis.

5. Atonic-astatic form.

By the severity of the injury: mild, moderate, severe form.

Period:

Early recovery;

late recovery;

residual period.

Diagnostics

Diagnostic criteria

Complaints to a delay in psychoverbal and motor development, movement disorders, involuntary movements in the limbs, seizures.

History- perinatal pathology (prematurity, birth asphyxia, birth trauma, prolonged conjugation jaundice, intrauterine infections, etc.), previous neuroinfections, stroke, TBI in early childhood.

Physical examinations: movement disorders - paresis, paralysis, contractures and stiffness in the joints, hyperkinesis, delayed psycho-speech development, intellectual disability, epileptic seizures, pseudobulbar disorders (impaired swallowing, chewing), pathology of the organs of vision (strabismus, atrophy of the optic nerves, nystagmus ).

The spastic form of cerebral palsy is characterized by:

Increased muscle tone in the central type;

High tendon reflexes with extended reflexogenic zones, clonuses of the feet and patella;

Pathological reflexes: Babinsky, Rossolimo, Mendel-Bekhterev, Oppenheim, Gordon, Schaeffer. Among them, the Babinsky reflex is most constantly detected.

The hyperkinetic form of cerebral palsy is characterized by: hyperkinesis - choreic, athetoid, choreoathetoid, double athetosis, torsion dystonia.

At the age of 2-3 months, "dystonic attacks" appear, characterized by a sudden increase in muscle tone during movements, with positive and negative emotions, loud sounds, bright light; accompanied by a sharp cry, pronounced vegetative reactions - sweating, redness of the skin and tachycardia.

Tendon reflexes in most patients of normal living creatures or somewhat brisk, pathological reflexes are not detected.

Hyperkinesis in the muscles of the tongue appears at the age of 2-3 months of life, earlier than hyperkinesis in the muscles of the body - they appear at 4-6 months and become more pronounced with age. Hyperkinesis is minimal at rest, disappears during sleep, increases with voluntary movements, is provoked by emotions, and is more pronounced in the supine and standing position. Speech disorders are hyperkinetic in nature - dysarthria. Mental development is disturbed less than in other forms of cerebral palsy.

The atonic-astatic form of cerebral palsy is characterized by:

1. Muscle tone is sharply reduced. Against the background of general muscular hypotension, the tone in the upper limbs is higher than in the lower limbs, and movements in the upper part of the body are more active than in the lower.

2. Tendon reflexes are high, pathological reflexes are absent.

3. Recurvation in the knee joints, flat-valgus feet.

4. In the mental sphere, 87-90% of patients have a pronounced decrease in intelligence, speech disorders are of a cerebellar nature.

Laboratory research:

1. Complete blood count.

2. General analysis of urine.

3. Feces on the eggs of the worm.

4. ELISA for toxoplasmosis, cytomegalovirus - according to indications.

5. Definition of TTG - according to indications.

Instrumental research:

1. Computed tomography (CT) of the brain: there is a different atrophic process - expansion of the lateral ventricles, subarachnoid spaces, cortical atrophy, porencephalic cysts and other organic pathology.

2. Electroencephalography (EEG) - general disorganization of the rhythm, low-voltage EEG, peaks, hypsarrhythmia, generalized paroxysmal activity are detected.

3. EMG - according to indications.

4. MRI of the brain - according to indications.

5. Neurosonography - to exclude internal hydrocephalus.

6. Optometrist - examination of the fundus reveals the expansion of veins, narrowing of the arteries. In some cases, congenital atrophy of the optic nerve, strabismus are found.

Indications for specialist consultations:

1. Speech therapist - detection of speech disorders and their correction.

2. Psychologist - to clarify mental disorders and their correction.

3. Orthopedist - identifying contractures, resolving the issue of surgical treatment.

4. Prosthetist - to provide orthopedic care.

5. Optometrist - examination of the fundus, detection and correction of eye pathology.

6. Neurosurgeon - in order to exclude neurosurgical pathology.

7. Physical therapy doctor - appointment of individual lessons, styling.

8. Physiotherapist - for the appointment of physiotherapy procedures.

Minimum examination when referring to a hospital:

1. Complete blood count.

2. General analysis of urine.

3. Feces on the eggs of the worm.

The main diagnostic measures:

1. Complete blood count.

2. General analysis of urine.

3. Speech therapist.

4. Psychologist.

5. Optometrist.

6. Orthopedist.

7. Prosthetist.

9. Physical therapy doctor.

10. Physiotherapist.

11. Computed tomography of the brain.

List of additional diagnostic measures:

1. Neurosonography.

2. Neurosurgeon.

3. MRI of the brain.

4. ELISA for toxoplasmosis.

5. ELISA for cytomegalovirus.

6. Infectionist.

8. Cardiologist.

9. Ultrasound of the abdominal organs.

10. Geneticist.

11. Endocrinologist.

Differential Diagnosis

Nosology

The onset of the disease

Characteristic symptoms

Biochemical blood tests, instrumental data, diagnostic criteria

congenital myopathy

Since birth

Diffuse muscle hypotension, hyporeflexia. Myopathic symptom complex, delayed motor development, osteoarticular deformities

EMG - the primary muscular nature of the changes. Increased content of CPK

Leukodystrophy

No abnormalities in the neonatal period

Movement disorders, muscular hypo-, then hypertension, ataxia. Progressive course, convulsions, spastic paralysis, paresis, progressive decrease in intelligence, hyperkinesis

CT scan of the brain: extensive bilateral foci of low density

Mucopolysaccharidoses

No abnormalities in the neonatal period

Characteristic appearance of the patient: grotesque facial features, depressed bridge of the nose, thick lips, short stature. Delay in the development of neuropsychic static functions, underdevelopment of speech

The presence of acidic mucopolysaccharides in the urine

Spinal muscular amyotrophy of Werdnig-Hoffmann

From birth or from 5-6 months of age

Generalized hypotension, hypoareflexia, fasciculations in the muscles of the back, proximal limbs.

progressive course. EMG from limb muscles - denervation type

congenital hypothyroidism

The first symptoms may appear already in the maternity hospital (attacks of respiratory arrest, jaundice), but more often develop in the first months of life

The specific appearance of the patient: small stature, short limbs, wide hands and feet, sunken bridge of the nose, swollen eyelids, large tongue, dry skin, brittle hair. Delayed psychomotor development, diffuse muscular hypotension. As a rule, constipation, bradycardia are noted. Very characteristic yellowish-earthy skin color

Aplasia or insufficiency of thyroid function. Delayed emergence of ossification nuclei

Ataxia-telangiectasia Louis Bar

No abnormalities in the neonatal period. Onset 1-2 years

Ataxia, hyperkinesis, decreased intelligence, telangiectasia, age spots, often lung pathology

Atrophy of the vermis and cerebellar hemispheres, high levels of serum α-phenoprotein

Phenylketouria

Children are born healthy, the disease manifests itself from 3-6 months of age: suspension in psycho-speech and motor development

The disease manifests itself from 3-6 months of age by lightening of the hair, iris, musty specific smell of urine and sweat, cessation or slowing of motor and mental development; convulsions appear - up to 1 year more often infantile spasms, later they are replaced by tonic-clonic seizures

An increase in blood phenylalanine, a positive Fehling test

Rett syndrome

Up to 1 year, psychoverbal development corresponds to age, then a gradual loss of previously acquired skills. In the ante- and perinatal period of development, as well as in the first half of life, the development of children is often regarded as normal.

The age at which deviations in the development of children are first noted ranges from 4 months to 2.5 years; most often from 6 months to 1.5 years. The first signs of the disease include a slowdown in the child's psychomotor development and head growth, loss of interest in games, and diffuse muscle hypotension. An important symptom is the loss of contact with others, which is often misinterpreted as autism. Diagnosis is considered provisional until two or five years of age.

Diagnostic criteria for Rett syndrome (according to Trevathan et al., 1998) include the necessary criteria, among which are normal prenatal and perinatal periods, normal head circumference at birth followed by slowing of head growth between 5 months and 4 years; loss of acquired purposeful hand movements between the ages of 6 and 30 months, associated in time with impaired communication; deep damage to expressive and impressive speech and gross delay in psychomotor development; stereotyped hand movements, reminiscent of squeezing, squeezing, clapping, “washing hands”, rubbing, appearing after the loss of purposeful hand movements; gait disorders (apraxia and ataxia) that appear at the age of 1-4 years


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Treatment

Treatment tactics

It is extremely important to suspect or make a diagnosis in the first year of life, since the child's brain has a huge potential to compensate for the damage received. The child's brain is extremely plastic, so it is necessary to try to stimulate its recovery capabilities. The complex treatment of cerebral palsy includes medications, exercise therapy, orthopedic care, various types of massage, reflexology, physiotherapy, sessions with a speech therapist and psychologist. Rehabilitation therapy is most effective in patients in the early recovery period, the sooner you start preventing or reducing the development of pathological phenomena, the greater the effect of therapeutic measures.

Basic principles of treatment of cerebral palsy in the recovery period:

1. With the help of medications, a decrease in the zone of excitation and a decrease in muscle tone. Fight against delayed tonic reflexes and pathological muscle tone.

2. To achieve the formation of the vertical position of the child, his movements and manual manipulations, the cultivation of the skill of standing, moving initially with outside help, and then independently or with the help of crutches, sticks, orthopedic shoes, splints, devices.

3. Improvement of general mobility and coordination of movements.

4. Promoting the normalization of mobility in the joints and muscle performance, elimination of joint contractures.

5. Education and training of support and balance.

6. Stimulation of cognitive and psycho-speech development, mental development and education of correct speech.

7. Teaching vital, applied, labor skills, self-service skills education.

Purpose of treatment:

Improving motor and psycho-speech activity;

Prevention of pathological postures and contractures;

Acquisition of self-service skills;

Social adaptation, the emergence of motivations;

Relief of seizures.

Non-drug treatment:

1. General massage.

2. Exercise therapy - individual and group classes.

3. Physiotherapy - ozocerite applications, SMT, magnetotherapy.

4. Conductive pedagogy.

5. Classes with a speech therapist, psychologist.

6. Acupuncture.

7. Adele suit.

8. Hippotherapy.

Medical treatment

Recently, drugs of the nootropic series - neuroprotectors, have been widely used in order to improve metabolic processes in the brain. Most nootropic drugs, due to their psychostimulating effect, are prescribed in the first half of the day. The duration of nootropic treatment courses is from one to two to three months.

Cerebrolysin, ampoules 1 ml / m, piracetam, 5 ml ampoules 20%, tablets 0.2 and 0.4, ginkgo biloba (tanakan), tablets 40 mg, pyritinol hydrochloride (encephabol), dragee 100 mg, suspension - 5 ml contain 80.5 mg pyritinol (corresponding to 100 mg pyritinol hydrochloride).
Encephabol - a minimum of contraindications, approved for use from the first year of life. Dosage of the suspension (with a content of 20 mg of encephabol in 1 ml) for children 3-5 years old, a daily dose of 200-300 mg (12-15 mg of body weight) is prescribed in 2 doses - in the morning (after breakfast) and in the afternoon (after daytime sleep and afternoon tea) . The duration of the course is 6-12 weeks, long-term use is advisable, which increases working capacity and learning ability, improves higher mental functions.

Actovegin, ampoules 2 ml 80 mg, dragee-forte 200 mg of the active substance. Neurometabolic drug containing exclusively physiological components. Children are prescribed in dragee-forte, taking before meals ½ -1 dragee 2-3 times a day (depending on age and severity of symptoms of the disease), up to 17 hours. The duration of therapy is 1-2 months. Instenon tablets (1 tablet contains etamivan 50 mg, hexobendine 20 mg, etophylline 60 mg). Multicomponent neurometabolic drug. The daily dose is 1.5-2 tablets, administered in 2 divided doses (morning and afternoon) after meals. To avoid side effects, a gradual dose increase over 5-8 days is recommended. The duration of treatment is 4-6 weeks.

With spastic forms of cerebral palsy Myospasmolytics are widely used in practice: tolperisone, tizanidine, baclofen.
Tolperisone (midocalm) is a centrally acting muscle relaxant, has membrane stabilizing activity, due to which it suppresses the formation and conduction of action potentials in hyperstimulated motor neurons of the brain stem and in peripheral nerves.
The mechanism of action is associated with a depressing effect on the caudal part of the reticular formation, suppression of pathologically increased spinal reflex activity and effects on peripheral nerve endings, as well as central n-anticholinergic properties. It also has a vasodilatory effect. This leads to a decrease in abnormally high muscle tone, reduces pathologically increased muscle tone, muscle rigidity, and improves voluntary active movements.
Mydocalm suppresses the functions of activating and inhibitory reticulospinal tracts and inhibits the conduction of mono- and polysynaptic reflexes in the spinal cord.
Dosage regimen: children from 3 months. up to 6 years, mydocalm is prescribed orally at a daily dose of 5-10 mg / kg (in 3 doses during the day); at the age of 7-14 years - at a daily dose of 2-4 mg / kg; adults 50 mg - 150 mg (1-3 tablets) 3 times a day.

Tizanidin (sirdalud) is a centrally acting myospasmolytic. The main point of application of its action is in the spinal cord. It selectively suppresses the polysynaptic mechanisms responsible for increasing muscle tone, mainly by reducing the release of excitatory amino acids from interneurons. The drug does not affect neuromuscular transmission.
Sirdalud is well tolerated and effective for cerebral and spinal spasticity. It reduces resistance to passive movements, reduces spasms and clonic convulsions, and increases the strength of voluntary contractions.

Baclofen is a centrally acting muscle relaxant; GABA receptor agonist. It inhibits mono- and polysynaptic reflexes, apparently due to a decrease in the release of excitatory amino acids (glutamate and aspartate) and terminals, which occurs as a result of stimulation of presynaptic GABA receptors. Against the background of the use of the drug, the motor and functional activity of patients increases.

Angioprotectors: vinpocetine, cinnarizine.

B vitamins: thiamine bromide, pyridoxine hydrochloride, cyanocobalamid; neuromultivit - a special complex of B vitamins with directed neurotropic action; neurobex.

Vitamins: folic acid, tocopherol, retinol, ergocalciferrol.

Anticonvulsants in epileptic syndrome: valproic acid, carbamzazepine, diazepam, clonazepam, topamax, lamotrigine.

With a combination of cerebral palsy with hydrocephalus, hypertensive-hydrocephalic syndrome, the appointment of dehydration agents is indicated: acetozolamide, furosemide, and potassium preparations at the same time: panangin, asparkam, potassium orotate.

With severe anxiety, a syndrome of neuro-reflex excitability, sedatives are prescribed: novo-passit, noofen, a mixture with citral.

In recent years, in spastic forms of cerebral palsy, botulinum toxin has been used to reduce the spasticity of individual muscle groups. The mechanism of action of botulinum toxin (Dysport) is to inhibit the release of acetylcholine at neuromuscular synapses. Injection of the drug leads to relaxation of the spastic muscle.
The main indication for prescribing dysport in children with various forms of cerebral palsy is equinovarus deformity of the foot. The introduction of Dysport is technically simple and is not accompanied by significant adverse reactions.
The standard dose of Dysport per procedure is 20-30 mg per 1 kg of body weight. The maximum allowable dose in children is 1000 units. the average dose for each head of the gastrocnemius muscle is 100-150 units, for the soleus and posterior tibial muscles - 200 units.
Injection technique: 500 units Dysport is diluted in 2.5 ml of physiological saline (i.e. 1 ml of solution contains 200 units of the drug). The solution is injected into the muscle at one or two points. The effect of the drug (relaxation of spastic muscles) begins to appear on the 5-7th day after the administration of the drug, with the maximum effect achieved by 10-14 days.
The duration of the muscle relaxant is individual and varies from 3 to 6 months. after the introduction. Dysport should be introduced into the complex scheme as early as possible, before the formation of joint contractures.

Preventive actions:

Prevention of contractures, pathological postures;

Prevention of viral and bacterial infections.

Further management: dispensary registration with a neurologist at the place of residence, regular physical therapy classes, training parents in massage skills, exercise therapy, orthopedic styling, caring for children with cerebral palsy.

List of essential medicines:

1. Actovegin ampoules 80 mg, 2 ml

2. Vinpocetine, (Cavinton), tablets, 5 mg

3. Piracetam in 5 ml ampoules 20%

4. Pyridoxine hydrochloride ampoules 1 ml 5%

5. Folic acid tablets 0.001

6. Cerebrolysin, ampoules 1 ml

7. Cyanocobalamin, ampoules 200 mcg and 500 mcg

Additional medicines:

1. Aevit, capsules

2. Asparkam, tablets

3. Acetozolamide, tablets 250 mg

4. Baclofen 10 mg and 25 mg tablets

5. Valproic acid, syrup

6. Valproic acid tablets 300 mg and 500 mg

7. Gingo-Biloba 40 mg tablets

8. Glycine, tablets 0.1

9. Hopantenic acid, (Pantocalcin), tablets 0.25

10. Diazepam, ampoules 2 ml 0.5%

11. Dysport, ampoules 500 IU

12. Carbamazepine tablets 200 mg

13. Clonazepam, tablets 2 mg

14. Konvuleks, capsules 150 mg, 300 mg, 500 mg, solution in drops

15. Lamotrigine 25 mg and 50 mg tablets

16. Magne B6 tablets

17. Neuromidin tablets 20 mg

18. Novo-Passit, tablets, solution 100 ml

19. Noofen, tablets 0.25

20. Panangin, tablets

21. Piracetam tablets 0.2

22. Pyritinol, suspension or tablets 0.1

23. Thiamine bromide ampoules, 1 ml 5%

24. Tizanidine (Sirdalud), tablets 2.4 and 6 mg

25. Tolpirisone, (mydocalm) ampoules 1 ml, 100 mg

26. Tolpirisone, dragee 50 mg and 150 mg

27. Topamax, capsules 15 mg and 25 mg, tablets 25 mg

28. Cinnarizine tablets 25 mg

Treatment effectiveness indicators:

1. Improving motor and speech activity.

2. Decreased muscle tone in spastic forms of cerebral palsy.

3. An increase in the volume of active and passive movements in the paretic limbs.

4. Relief of seizures with concomitant epilepsy.

5. Reduction of hyperkinesis.

6. Acquisition of self-service skills.

7. Replenishment of active and passive vocabulary.

8. Improving the emotional and mental tone of the child.

Hospitalization

Indications for hospitalization: movement disorders, paresis, paralysis, delayed psychomotor and speech development from their peers, seizures, hyperkinesis, contractures, joint stiffness

Information

Sources and literature

  1. Protocols for the diagnosis and treatment of diseases of the Ministry of Health of the Republic of Kazakhstan (Order No. 239 of 04/07/2010)
    1. L.O. Badalyan. Children's neurology. 1975 Moscow Handbook of a child psychiatrist and neuropathologist, edited by L.A. Bulakhova. Kyiv 1997 L.Z. Kazantsev. Rett syndrome in children. Moscow 1998 Clinic and diagnosis of cerebral palsy. Teaching aid. Zhukabaeva S.S. 2005

Information

List of developers:

Developer

Place of work

Job title

Kadyrzhanova Galiya Baekenovna

RCCH "Aksay" psycho-neurological department №3

Head of department

Serova Tatyana Konstantinovna

RCCH "Aksay" psycho-neurological department No. 1

Head of department

Mukhambetova Gulnara Amerzaevna

Department of Nervous Diseases Kaz.NMU

Assistant, Candidate of Medical Sciences

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  • The concept of cerebral palsy (cerebral palsy). History of study

    Cerebral palsy is a severe brain disease that manifests itself in various psychomotor disorders with a leading motor defect.

    The term cerebral palsy (CP) refers to a group of movement disorders that occur when the motor systems of the brain are damaged and manifested in a lack or absence of control by the nervous system over muscle functions.

    With cerebral palsy, there is early, usually intrauterine damage or underdevelopment of the brain. The reasons for these violations can be different:

    • - various chronic diseases of the expectant mother;
    • - transferred by the mother infectious, especially viral diseases, intoxication;
    • - incompatibility of mother and fetus by Rh factor or group affiliation;
    • - injury during pregnancy, etc.

    Predisposing factors may be - prematurity or postmaturity of the fetus.

    In some cases, the cause of cerebral palsy can be:

    • - obstetric traumatism;
    • - prolonged labor with entanglement of the umbilical cord around the fetal neck, which leads to damage to the nerve cells of the child's brain due to a lack of oxygen;
    • - sometimes cerebral palsy occurs after birth at the age of up to one year as a result of infectious diseases complicated by encephalitis (inflammation of the brain substance), after severe bruises of the head. Cerebral palsy, as a rule, is not a hereditary disease.

    The term cerebral palsy has been around for over a century, and the disease itself has probably existed without a name throughout human history. However, despite its long history, there is still no unity in views on this problem.

    Along with the generalized term cerebral palsy, the term Little's disease is sometimes used in clinical practice. This name was proposed in honor of the British orthopedic surgeon William John Little (William John Little), who in the middle of the 19th century was the first to establish a causal relationship between complications during childbirth and impaired mental and physical development of children after birth. His views were summarized in the article "On the influence of pathological and difficult births, prematurity and asphyxia of newborns on the mental and physical condition of children, especially with regard to deformities." This article, addressed to the Obstetrical Society of Great Britain, is most often cited in books and articles on cerebral palsy.

    William John Little (1810-1894), British orthopedist, first established a causal relationship between complications during childbirth and impaired mental and physical development of the child. Little's work attracted the attention of his contemporaries. In response to his opponents, Little did not claim his primacy in describing the neurological consequences of pathological childbirth. Finding no information on this subject in the English medical literature, he quoted William Shakespeare. According to Little, in the description of Richard III, deformations that arose as a result of prematurity and, possibly, complications during childbirth, are clearly guessed. Shakespeare puts these words into the mouth of the tragic personality of the English king:

    “I, who have neither height nor posture, To whom in return the swindler nature handed over lameness and lopsidedness; I, made carelessly, somehow And sent to the world of the living ahead of time, So ugly, so crippled, That dogs bark when I pass ... "

    Such movement disorders were called Little's disease until the Canadian physician William Osler proposed the use of the term cerebral palsy in 1889. In an extensive monograph "Cerebral Palsy in Children", he also noted the relationship between difficult childbirth and lesions of the nervous system in children.

    William Osler (1849-1919), famous Canadian orthopedist. Working at the University Johns Hopkins, wrote the book "Cerebral Palsy in Children" Cerebral palsy as a separate nosological form, combining a variety of motor disorders of cerebral origin, was first identified by the famous Viennese neuropathologist, and later by the outstanding psychiatrist and psychologist Sigmund Freud.

    Sigmund Freud (Sigmund Freud‚ 1856-1939) full name Sigismund Shlomo Freud, Austrian physician, founder of psychoanalysis

    In all previous publications of the 19th century, devoted to motor disorders in children, the term "cerebral palsy" was used, if at all, only in combination with other terms (for example, "cerebral birth palsy"). Nevertheless, the daily clinical practice of the second half of the 19th century needed to specify the terminology. In his monograph, Freud writes that the term "infantile cerebral palsy" combines "those pathological conditions that have long been known, and in which muscle rigidity or spontaneous muscle twitches predominate over paralysis." Freud's classification and interpretation of cerebral palsy was broader than subsequent formulations by other authors. He proposed to use this term even in cases of complete absence of paralysis, such as epilepsy or mental retardation. This interpretation of cerebral palsy is much closer to the concept of early "brain damage", formulated much later. Perhaps Freud proposed to unite various motor disorders in children into one nosological group because he could not find another way to streamline this area of ​​​​child neurology. At first he studied cerebral hemiplegia. Then he combined all other motor lesions into one group, which he called cerebral diplegia, meaning by this term the defeat of both halves of the body. Four varieties were distinguished in this group: 1) general cerebral rigidity; 2) paraplegic rigidity; 3) bilateral hemiplegia; and 4) general chorea and bilateral athetosis. Later, Freud combined all these different motor disorders into one nosological unit - cerebral palsy. In the twentieth century, the lack of consensus in the definition of nosology made it difficult to conduct scientific research. The need to form a common view on cerebral palsy became more and more obvious to specialists. Some researchers interpreted cerebral palsy as a single clinical nosology, others as a list of similar syndromes.

    One of the initiatives aimed at generalizing and further developing modern views on cerebral palsy was the creation of the Little Club on the initiative of Ronald MacKeith and Paul Polani in 1957. After two years of their work, they published a Memorandum on the terminology and classification of cerebral palsy. According to the Little Club definition, cerebral palsy is a non-progressive brain lesion that appears in the early years of life with disturbances in movement and body position. These disturbances resulting from impaired brain development‚ are non-progressive but modifiable.

    In the future, scientists from different countries of the world offered various interpretations of this concept. Leading Soviet specialist in the problem of cerebral palsy, head of the largest center for the treatment of patients with cerebral palsy in Moscow, Prof. K.A. Semenova offers such a definition. Cerebral palsy unites a group of syndromes with different clinical manifestations that arise as a result of brain underdevelopment and damage at various stages of ontogenesis and are characterized by an inability to maintain a normal posture and perform voluntary movements. The definition of cerebral palsy proposed by Academician Levon Badalyan was consonant. In his opinion, the term "cerebral palsy" combines a group of syndromes resulting from underdevelopment or damage to the brain in the prenatal, intranatal and early postnatal period. Brain damage is manifested by a violation of muscle tone and coordination of movements, the inability to maintain a normal posture and perform voluntary movements. Movement disorders are often combined with sensory disorders, delayed speech and mental development, and convulsions.

    An important milestone in the development of views on cerebral palsy was the holding in July 2004 in Maryland (USA) of the International Seminar on the definition and classification of cerebral palsy. The workshop participants confirmed the importance of this nosological form and emphasized that cerebral palsy is not an etiological diagnosis, but a clinical descriptive term. The results of the workshop were published in the article "Proposal for the definition and classification of cerebral palsy". The authors proposed the following definition: “The term cerebral palsy (CP) refers to a group of movement and posture developmental disorders causing activity limitations that are caused by non-progressive damage to the developing brain of the fetus or child. Motor disturbances in cerebral palsy are often accompanied by defects in sensory, cognitive and communication functions, perception and/or behavioral and/or seizure disorders.”

    BASHKIR STATE MEDICAL UNIVERSITY

    Department of Nervous Diseases with a course of medical genetics.

    Head department: prof.

    Teacher:

    DISEASE HISTORY

    Curator: student gr. L-405B

    1. General information about the patient

    2. Age 11 years (21.11.88.)

    3. Gender female

    4. Bashkir nationality

    5. Place of residence

    6. Mother controller

    7. Father operator, KGDU

    8. Visited d / y does not attend

    9. Date of admission to the clinic 10.04.2000, 1330

    10. Clinical diagnosis:

    ICP, hemiparetic form, residual stage, I degree, central left-sided hemiparesis.

    2. Complaints of the patient (by the moment of admission)

    Complaints of weakness in the left arm and leg, gait disturbance, general weakness.

    3. History of present illness.

    The perinatal anamnesis is burdened (rapid delivery). Child from the 6th pregnancy, 3 births. Pregnancy: high blood pressure, uterine bleeding. She was born at term, birth weight 2850 g. The cry is weak. Breastfeeding on the 12th day. He holds his head from 3 months. Sitting since 8 months. She started walking on her own at 18 months. Speech developed after 2 years. Motor activity in the arms and legs is reduced from birth.

    From the age of 3 to 5, she had febrile convulsions, received an age-appropriate dose of phenobarbital. The last attack was in 1992. Phenobarbital stopped in 1996.

    The diagnosis of cerebral palsy was established at the age of 11 months, since then, 2 times a year, he undergoes a course of rehabilitation treatment at the RCSC. At the moment, she entered the next planned course of treatment.

    4. General history.

    The third child in the family. Has two healthy brothers. There are no mental and nervous diseases, malignant neoplasms in the family.

    Past illnesses: SARS, bronchitis, chicken pox, enuresis, catarrhal tonsillitis, epilepsy.

    Difficult to train. Homeschooled in 3rd grade. I studied in the 1st grade for 2 years.

    Family tree (pedigree)

    Proband : xxxxxx xxxxxx Borisovna,

    11 years old (11/21/1988), Bashkir. Diagnosis: cerebral palsy, hemiparetic form, residual stage, I degree, psychomotor retardation.

    I generation 1 2

    II generation 3 4 5 6 7 8

    III generation 9 10 11 12 13 14

    Legend:

    1. Ivanov I.S., 82 years old, Bashkirin, pensioner.

    2. Ivanova T.S., 1922, Bashkir. She died in 1996 from MI.

    3. Ivanova S.I., 62 years old, Bashkir, pensioner

    4. Ivanov S.I., 63 years old, Bashkirin, pensioner

    5. Ivanov K.I., 54 years old, Bashkirin, driver,

    6. Ivanova Ch.K., 54 years old, confectioner,

    7. Petrova L.I., 51 years old, counter-Bashkir, Oler,

    8. Petrov B.S., 53 years old, Bashkirin, operator,

    9. Ivanov K.S., 31 years old, Bashkirin, tractor driver,

    10. Ivanov I.S., 29 years old, Bashkir, Bashkirin, engineer,

    11. Ivanova I.K., 25 years old, Bashkir, accountant,

    12. Petrov D.B., 20 years old, Bashkirin, student,

    13. Petrov N.B., aged 16, Bashkirin, student,

    5. General condition.

    General examination

    The patient is of medium build, normosthenic. The position is active, consciousness is clear. Facial expression is normal, facial expressions are lively.

    The skin and visible mucous membranes are of normal color, without areas of de- and hyperpigmentation.

    The hair is thick, shiny, there are no areas of baldness. The nails are pale pink in color, without deformities and fungal infections. Subcutaneous tissue is moderately developed. There are no edema.

    Lymph nodes are not palpable.

    Respiratory system

    The thorax is conical in shape, without deformities, supraclavicular and subclavian fossae are weakly expressed. The type of breathing is abdominal, breathing is deep, rhythmic, with a frequency of 20 per minute. There is no shortness of breath.

    With comparative percussion of the chest above the symmetrical areas of the lungs, a clear pulmonary sound is heard.

    On auscultation, breathing is vesicular, there are no side breath sounds, bronchophony is the same over symmetrical areas on both sides.

    No pathology was found in the respiratory organs.

    Circulatory organs

    When examining a visible protrusion in the region of the heart is not observed, the pulsation is not visually determined.

    Epigastric pulsation is not observed. There is no trembling in the region of the heart.

    On auscultation, the heart sounds are rhythmic, slightly muffled. Heart rate - 85 beats / min. The magnitude of the pulse waves on both hands is the same, the pulse is rhythmic, frequent, firm, of medium filling, uniform.

    Neck veins are not expressed. Venous pulse is negative. There are no dilated veins on the surface of the chest, anterior abdominal wall, and extremities. There are no seals and pain along the veins.

    Digestive organs

    When examining the oral cavity, the tongue is moist, pink, lined with a white coating, papillae are pronounced, ulcers and cracks are absent. The gums and palate are pale pink in color, without plaque and ulceration. The patient does not notice increased bleeding of the gums and loosening of the teeth. Zev pink, clean, without swelling and plaque. There is no unpleasant odor from the oral cavity.

    The abdomen is correctly convex, participates in the act of breathing, the peristalsis of the stomach and intestines is not visually noticeable, venous collaterals are not expressed. The navel is retracted. With percussion, the sound over the hollow organs is tympanic.

    Ortner's symptom is negative.

    On superficial palpation, the abdomen is not tense, the symptoms of Shchetkin-Blumberg, Mendel, Kivul and Volkovich are negative.

    With deep sliding palpation according to Obraztsov-Strazhesko, the internal organs of the abdominal cavity are painless, the lower border of the stomach is elastic, convex; the sigmoid colon is not palpable, the caecum is palpable in the form of a moderately tense cylinder with a rounded bottom, 3 cm in diameter, parallel to the umbilical-iliac line, 1 cm below the interosseous line. The ileum is not palpable. The transverse colon is palpated 3 cm below the border of the stomach in the form of an arcuate and transverse cylinder of moderate density, 2-2.5 cm thick, easily moved and not rumbling. Other parts of the intestine are not palpable.

    The spleen is not palpable, its borders cannot be determined by percussion. The pancreas and liver are not palpated. Symptoms of Mayo-Robson, Carte, Courvoisier are negative. Auscultatory noise of peritoneal friction and vascular noise is absent. There is a sound of intestinal peristalsis.

    genitourinary system

    There is no swelling in the lumbar region. Pasternatsky's symptom is negative on both sides. The kidneys and bladder are not palpable. Pathology from the external genitalia is not detected.

    6. Neurological and mental state.

    Functions of the cranial nerves.

    I pair - the phenomena of hypo-, ano-, dysosmia are absent;

    II pair - visual fields, color perception and visual acuity are not disturbed, the fundus of the eye is unchanged;

    III, IV, VI pairs - pupils of the usual form, the same size, the reaction to light is expressed, Argyll Robertson's symptom is negative, the movements of the eyeballs are in full;

    V pair - the sensitivity of the skin and mucous membranes, the trophism of the chewing muscles are not disturbed, the movements of the lower jaw are performed in full, the trigeminal points are painless, the corneal, conjunctival, superciliary and mandibular reflexes are alive;

    VII pair - palpebral fissures - S>D, the face at rest is symmetrical, the left nasolabial fold is smoothed, facial expressions are lively, the strength of facial muscles is 5 points, taste sensitivity of the anterior 2/3 of the tongue is preserved;

    VIII pair - no pathology of the hearing organs and vestibular apparatus was detected, dizziness and nytagmus were absent;

    IX, X pairs - dysphonia, dysarthria is not observed, swallowing is not difficult, taste sensitivity of the posterior 1/3 of the tongue is preserved;

    XI pair - the head position is normal, head turns, shoulder lifting are carried out without difficulty, there is no hyperkinesis of the sternocleidomastoid and trapezius muscles, strength - 4 points;

    XII pair - there are no fibrillar twitchings of the tongue, the movements of the tongue are in full, the trophism is not disturbed, the position is along the midline.

    Motor functions:

    The muscles of the left limbs are somewhat hypotrophic. Fibrillar and fascicular twitches are absent, the strength of the muscles of the left limbs is 4 points, the right ones are 5 points. The range of active movements is somewhat limited in the left limbs. Pathological synkinesis and hyperkinesis are absent

    The gait is paretic. The finger-nose test on the left is difficult to perform. In the complicated position of Romberg it is unstable. Adiadochokinesis on the left.

    The expression is expressed. Speech is intelligible.

    Reflexes: high tendon reflexes are noted, D

    Sensitive Features:

    tension symptoms are absent, pain and paresthesia are absent. Pain, temperature and other types of both superficial and deep sensitivity are not disturbed.

    Meningeal symptoms:

    Rigidity of the occipital muscles, the symptom of Kernig, Brudzinsky, Danzeg, Mendel are absent.

    Vegetative-trophic functions:

    Local dermographism is white, unstable, disappears after 45 seconds. Reflex dermographism without features. Skin temperature is normal. The solar plexus and cervical sympathetic nodes are painless. Salo-, salivation is not disturbed. Dyshidrosis of the palms and feet. Trophic disorders are absent. The Dagnini-Ashner test causes a decrease in heart rate by 7 beats.

    Functions of the pelvic organs: not violated.

    Higher cortical functions:

    a) receptive speech - understands the meaning of words, shows the called familiar objects, understands the meaning of whole phrases, including ridiculous ones, a story.

    b) expressive speech - clear, pronounces all sounds. Can repeat short phrases, name shown objects, actions with a hint.

    c) written speech - can write off printed and capital letters. Slowly draws letters.

    d) reading - reads, can tell a poem learned by ear, retell what he heard.

    f) praxis - performs actions on request, both simple movements and actions with real and imaginary objects. Can imitate the actions of a doctor.

    Mental functions:

    Consciousness is clear, recognizes the mother, relatives, medical staff; oriented in relation to place and time. Approach to the examination is adequate. The mood is satisfactory. Gets tired quickly and has difficulty concentrating. Memory and attention are reduced. Sleep deep, restful.

    7. Laboratory and other additional studies

    12.04.2000 OAM

    color - light yellow

    reaction is sour

    density - 1010

    L - 1-2-3 in sight

    squamous epithelium - single cells

    protein - neg.

    04/12/2000 UAC

    Hb - 119 g / l

    col. indicator - 0.99

    Er - 4.03x10 12 /l

    L - 7.3x10 9 /l

    ESR - 5 mm/hour

    11.04.2000 REG

    FM leads FM leads

    left right

    rheographic

    index, UE – 2.38 2.15

    diastolic

    index, UE – 1.12 1.04

    fast time

    filling of vessels, s – 0.06 0.07

    slow time

    vessel filling, s – 0.06 0.06

    anacrotic duration, s – 0.12 0.13

    catacrotic duration, s – 0.35 0.40

    dicratic index, % – 56.0 48.8

    rheographic coefficient, % – 18.8 27.0

    venous ratio – 172.8 135.7

    systole-diastolic

    indicator, % – 47.2 48.6

    Heart rate, beats / min - 96 93

    coefficient asymmetries – – 10.8

    Conclusion: on the left and on the right, the pulse blood filling of the vessels is increased, elasticity is not changed, the tone of the main arteries is preserved, the venous outflow is not disturbed, the tone of the veins is preserved.

    04/14/2000 ECG

    R II >R I =R III

    Conclusion: lower atrial rhythm, EOS without deviations, heart rate 100. Partial phenomenon of premature excitation of the ventricles.

    04/14/2000. Psychologist consultation.

    Conclusion: The child is mentally immature, intellectually passive. Sustainability does not last long. Detects insufficiency of ideas and lack of formation of concepts, accessible to her age.

    8. Syndromic diagnosis

    Central left-sided hemiparesis (restriction of movements in the muscles of the left limbs, adiadochokinesis, increased tendon reflexes on the left, the presence of pathological reflexes of Babinsky, Rossolimo, Bekhterev on the left limbs).

    Delayed psychomotor development (inconsistency of the required knowledge, skills and abilities with age).

    9. Topical diagnosis

    The defeat is predominantly the upper and middle third of the precentral gyrus of the cerebral cortex on the right (responsible for the motor activity of the left limbs); cortical structures, possibly the frontal lobe (delayed psychomotor development).

    10. Clinical diagnosis

    Main disease:

    ICP, hemiparetic form, residual stage, I degree, central left-sided hemiparesis. Delayed psychomotor development

    Diagnosis is based on:

    Complaints (weakness in the left river and leg, gait disturbance, delayed psychomotor development, limitation of active movements of the left limbs);

    History of the disease (rapid delivery, lag in psychomotor development, fibril convulsions from 3 to 5 years);

    Data of examination and physical examination (limitation of active movements in the left limbs, adiadochokinesis on the left, increased tendon reflexes on the left, the presence of pathological reflexes of Babinsky, Rossolimo, Bekhterev on the left limbs; delayed psychomotor development);

    Data from instrumental studies and expert advice

    (REG, EEG).

    11. Differential diagnosis

    Cerebral palsy should be differentiated from tumors of the nervous system, disorders of the spinal circulation, chromosomal syndromes.

    Unlike tumors of the upper lumbar segments of the spinal cord, there are no radicular pains in the zone of innervation of the femoral nerves, in addition, there is a lesion of the upper extremities.

    Unlike tumors of the thoracic part of the spinal cord, there are no disturbances in the sensitivity and functions of the pelvic organs, in addition, there is a lesion of the upper limbs, and there are no radicular pains.

    In addition, the age of the patient is uncharacteristic - spinal cord tumors are observed mainly at the age of 20 to 60 years.

    Unlike tumors of the cerebellum, often found in children, as well as tumors of the precentral gyrus, there are no headaches, vomiting and other signs of increased intracranial pressure, dizziness, convulsions (inherent in tumors of the precentral gyrus).

    Unlike disorders of the spinal circulation, there are no pain symptoms, etiological factors that cause vascular damage (pathology of the aorta, anomaly of the spinal vessels, trauma).

    Unlike chromosomal diseases, the patient's heredity is not burdened (among relatives on the maternal and paternal lines, there are no patients with similar diseases).

    12. Treatment

    1) drugs that improve metabolism and microcirculation in the brain - nootropics (piracetam, aminalon, glutamic acid), cavinton, cerebrolysin

    2) drugs that improve the metabolism of muscle tissue - nerabol, methandrostenolone

    3) vitamins - B 1, B 6, B 12, C, nicotinic acid

    4) transcerebral electrophoresis according to Bourguignon with potassium iodide

    5) therapeutic limb massage

    6) physiotherapy exercises

    7) hydrogen sulfide baths, sea bathing, hydromassage, physical education in the water.

    8) educational role-playing games

    Rep.: Tab. Aminaloni 0.25 obd. N. 200

    D.S. 2 tablets 3 times a day

    Rp.: Sol. Cyanocobalamini 0.01% 1ml

    D.t.d. N. 10 ampull.

    S. 1 ml intramuscularly

    Rep.: Tab. Methandrostenoloni 0.005 N 100

    D.S. 1/2 tablet 2 times a day

    Rp.: Cerebrolisini 1 ml

    D.t.d. N. 20 ampull.

    S. Intramuscularly 1 ampoule every other day

    13. Stage epicrisis

    An 11-year-old patient has been undergoing planned inpatient treatment at the RCSC since April 10, 2000 for cerebral palsy, hemiparetic form, residual stage, I degree, psychomotor developmental delay.

    While in the hospital, the patient was consulted by specialists, instrumental studies were carried out to study the dynamics of the disease (REG, EEG). Receives conservative treatment.

    Some positive dynamics is noted.

    Forecast

    a) labor– disability due to delayed psychomotor development and possible future mild mental retardation, as well as a reduced range of active movements (it is difficult to predict the possibility of learning, presumably will be able to engage in light unskilled work)

    b) vital- favorable (the disease is not fatal)

    in) social- doubtful (possible violations of the adaptive function to a social lifestyle due to a delay in psychomotor development).

    Literature

    1) Badalyan L. O. "Infantile cerebral palsy", M. 1985.

    2) Badalyan L.O. "Children's neurology", M. 1984.

    3) Vasilenko V.Kh. "Propaedeutics of internal diseases", 1984

    4) Gusev E.I. "Nervous diseases", M. 1988

    5) Makolkin V.I. "Internal Diseases", M. 1987

    6) Mashkovsky M.D. "Medicines", M. 1988

    7) Scheme of medical history for BSMU students.

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