Cerebellar ataxia: Species and Classification, including Pierre Marie, Causes, Symptoms and Treatment

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Cerebellar ataxia( MA) is characterized by impaired ability to move and coordinate due to pathological processes in the cerebellum. The term "ataxia" is taken to denote any coordination disorders.

Description and history of the disease

The cerebellum is part of the human brain located at its base. In the structure of the body, the two hemispheres responsible for the accuracy of movements are separated by a worm that provides stability and balance.

One type of ataxia is the cerebellar( G 11.1-11.3 according to ICD-10), in which these problems are caused by the pathology of the cerebellum. To describe the characteristic complex of symptoms( gait disorders, nystagmus, finger tremors, speech disorders and handwriting, etc.), the term "cerebellar ataxia syndrome" is used.

Depending on what part of the cerebellum is affected, the ataxia is divided into static-locomotor( the worm is affected, which is manifested by the disorder of gait, stability) and dynamic( hemispheres are affected and the ability to perform arbitrary limb movements is impaired).

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Similar problems occur in a standing position for a static shape and when walking for a dynamic shape. According to different data, the frequency of such pathology can reach 23 people per 100 thousand.

Causes of cerebellar ataxia

In most cases, cerebellar ataxia is a genetic disease, less commonly acquired.

In the latter version, the pathology is a consequence:

  • head injury;
  • of tumor processes;
  • viral infections( chickenpox, measles caused by the Coxsackie virus);
  • of bacterial infections( encephalitis, meningitis);
  • stroke and minijnsultov( transient ischemic attack);
  • cerebral palsy;
  • intoxications( lead, mercury, pesticides, solvents);
  • multiple sclerosis;
  • deficiency of vitamin B12;
  • of paraneoplastic syndrome.

A sudden strong sense of fear, accompanied by tachycardia, chills, increased sweating and a whole host of unpleasant symptoms is usually called a vegetative crisis or panic attacks. From this article you will learn how to remove the vegetative crisis and what measures can be taken to prevent it.

In addition to the vegetative crisis, the sympathetic-adrenal is also isolated. What symptoms are typical for him, and how he differs from other crises, you will find by reference.

Hereditary forms of the pathology

Hereditary forms are associated with gene mutations and are classified into:

  • congenital non-progressing AI due to the lack of, underdevelopment of the cerebellar structures;
  • is autosomal recessive( Friedreich's ataxia), the symptoms of which appear in children from an early age( or up to 25 years), characterized by abnormal skeleton formation( this form develops when a defective gene is obtained from both parents);
  • recessive, X-chromosomal with progressive cerebellar insufficiency, low prevalence predominantly in men;
  • Betten's disease, congenital AI with delayed child development, but with the patient's adaptation to his condition as he grows up;
  • , the hereditary ataxia of Pierre Marie, refers to late autosomal dominant cerebellar ataxia, the signs of which( "chopped" speech, trembling, decreased mental abilities, changes in tendon reflexes, vision problems, strabismus, etc.) appear after 25 yearsforms of ataxia is enough to obtain a gene from only one parent).

A lecture on the cerebellar ataxia of Friedreich:

According to the nature of the current, MA is divided into:

  • acute( acute cerebellar ataxia develops, usually as a result of stroke, encephalitis and other causes);
  • subacute( caused by the effects of tumor processes, multiple sclerosis, etc.);
  • chronically progressing, lasting for many months and even years;
  • paroxysmal episodic( children, adults).

Symptoms and manifestations of

Static abnormalities of MA are characterized by a specific posture: the patient stands, legs wide apart, balancing with his hands, trying not to turn or tilt the body and head.

When you try to move your legs( Romberg's posture - the feet are shifted, the arms are stretched forward, the head is raised), a slight push( pushing symptom), the patient falls, not realizing it.

Disturbances of dynamics are manifested in the "cerebellar gait", reminiscent of the movements of a drunk person: the patient moves, as on stilts, with wide apart strained straight legs.

Its body is also straightened( Tom's symptom), slightly reclined. It is difficult for the patient to make a turn, he brings it to the sides, he may even fall.

As the AI ​​progresses: the

  • loses the ability to alternate movements, touch the tip of the nose alternately with the index fingers of different hands;
  • handwriting and speech are broken;
  • impoverished mimicry( masklike face);
  • develop pain in the lower back, extremities, neck;
  • increases muscle tone;
  • appears convulsive twitching;
  • develop ptosis, strabismus, nystagmus, impaired vision;
  • noted difficulties with food intake and ingestion;
  • hearing is impaired;
  • develops depression, the psyche changes.
All types of AI have a disruption of the coordinated work of the muscles in the performance of the motor act.

For congenital forms are typical:

  • disproportionality of effort to the action performed;
  • impaired coordination of individual muscles;
  • shake;
  • the rhythmic deviation from a correct trajectory of movement increasing at approach to a destination destination;
  • vertical instability;
  • jerky speech, with a postal accent;
  • nystagmus;
  • later on the beginning of sitting, walking, uncertain gait in children;
  • delayed mental and speech development( compensation is reached by about 10 years).

Diagnosis of cerebellar ataxia

  • Evaluation of tendon reflexes.
  • Functional tests.
  • Spinal puncture and analysis of cerebrospinal fluid.
  • Blood tests, urine tests.
  • MRI / CT.
  • Dopplerography of the brain.
  • ultrasound.
  • DNA research.
  • Collect information about diseases of close relatives.

Any tremor is characterized by the fact that a person can not himself control these arbitrary movements. Certainly, such pathology brings not a small discomfort in ordinary life. Is it possible to completely get rid of it, and whether there is a treatment of essential tremor with folk remedies.

Abuse of alcohol can also lead to shivering in the hands or feet. About the remaining symptoms of alcoholic epilepsy can be read here.

For posttraumatic encephalopathy, a separation into several degrees is characteristic. What is the feature of the 2nd degree and how dangerous is it to human life http: //gidmed.com/ bolezni-nevrologii /jencefalopatija/ posttravmaticheskaya.html.

Treatment of

Treatment of cerebellar ataxia depends on its cause. If it is possible to eliminate the cause, then the disease is curable.

So, the virus-associated MA can by itself pass with time, bacterial AI - when taking antibiotics.

With MA caused, for example, by multiple sclerosis, as well as hereditary, there is no specific treatment.

Therefore, for most forms of pathology, symptomatic therapy is used:

  • preparations that improve cerebral blood supply( cinnarizine, pyracetam, etc.);
  • nootropics;
  • muscle relaxants;
  • beta-histidine preparations( betaserk);
  • anticonvulsants;
  • physiotherapy;
  • massage;
  • occupational therapy;
  • logopedic exercises;
  • adaptive devices( canes, modified utensils, etc.)

In the treatment of Pierre Marie's disease, meliktin, baclofen, kondelfin, B vitamins are used, that is, a means of reducing muscle tone.

Homeopathic treatment is prescribed by a homeopathic physician based on a subjective description of the patient's feelings, so there is no specific drug for all forms of MA.

Physiotherapy in MA involves performing exercises with jerks, sharp strokes, as well as repeated repetition of household skills( pouring liquid, turning pages, zipping "fastening").

Consequences and prognosis of

Cerebellar ataxia is incurable, except for the form caused by the infectious process.

Rapid progression, impaired function of many organs, deterioration of quality of life are unfavorable prognostic signs.

The following are the main consequences of cerebellar ataxia:

Prevention measures

The only preventive measure is the vaccination of children against viral diseases that increase the risk of developing AI.

Families in whom there are patients with hereditary AI, it is recommended to refrain from procreation. Also, any closely related marriages should be excluded.

Thus, cerebellar ataxia, disrupting coordination of movements and stability, creates a lot of intractable problems for the patient.

Therefore, if you have the smallest suspicion of such a pathology, you should immediately consult with your doctor and take all possible measures to make life easier with this diagnosis.

In the video, the neurologist M.M.Sperling will tell you a few more words about cerebellar ataxia:

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