Other Diseases

Olivopontocerebellar degenerations - atrophy of the cerebellum, bridge and olive brain

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Atactic gait Olivopontocerebellar degeneration( OTSDD), as a hotel type of disease, was described more than 100 years ago. Throughout the period the disease was studied, methods of diagnosis and treatment were developed.

Today it is known that olivopontocerebellar degeneration is inherited. The disease affects the central nervous system. Localization of atrophy - cerebellum, bridge and lower olives( parts of the brain).

The main manifestation is a change in habitual behavior - there are ataxia, extrapyramidal disorders. This type of disease is characterized by the duration of the course, but it is fixed in adulthood. In the process of changes occurring in the human brain, the death of neurons is noted, respectively, the frequency of the signals from them in the central nervous system is violated.

Also degenerative-dystrophic processes that occur throughout the development of the disease affect the cerebellar cortex, bridge and olive brain.

That's why there are characteristic changes in the psyche and the possibilities of man, including physical. In rare cases, during

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Cerebellar Ataxia diagnosis, lesions are noted in such areas as the anterior horn of the spinal cord and its pathways. The disease can also affect the caudal cranial nerves( 9-12 pairs).Violations that are associated with olivopontocerebellar degeneration include:

  • Alzheimer's disease;
  • multiple sclerosis;
  • Pick's disease.

The inheritance of the disease occurs on a recessive and dominant path, and cases of sporadic inheritance are also known.

The patient's condition can be expressed in loss of orientation, memory failure, full or partial sclerosis, abnormalities in the gait, stiffness of movements, decreased motor activity in general and body tone, apathy, loss of ability to concentrate attention.

Cerebellar ataxia

Statistical data

The majority of cases of the disease are reported in elderly people - after 60-65 years - 75% of cases.

However, degeneration can begin to develop at an earlier age - in the period of 30-40 years, and in case of inheritance and in children aged 10-11 years( 2-3%).

That is why the examination is necessary not only for reaching the elderly, but also in 20-40 years, and in the case of congenital disorders - constantly throughout life.

Causes of the development of the disease

An important feature of the disease is the fact that the exact causes of its occurrence and development, if there are no cases of disease in the family, are not known to medicine even a century after the first description. There are violations in the loci of genes, expressed in an increase in the number of trinucleotide repeats.

Among the other possible causes that can lead to the appearance of characteristic disorders, asymmetric atrophic changes in white matter are noted. Atrophy of the cerebellar cortex is noted in the late stages of the disease. In order to find out the exact reasons, it is necessary to undergo a thorough investigation.

Types of

Current There are several main types of olivopontocerebellar degeneration: brain degeneration

  • type Mendel;
  • type of Finkler-Winkler;
  • atrophy with retinal degeneration;
  • type of Shut-Haikman;
  • atrophy with dementia.

Also in a separate category, you can include Shay-Drageer syndrome, which is characterized by the following symptoms of the most frequent manifestation:

  • autonomic disorders;
  • cerebellar disorders and ataxia( varying degrees of severity);
  • is a lesion in the basal ganglia.

Mendel's type has an autosomal dominant inheritance mechanism and the following manifestations:

  • slow and dull development of the disease, which provokes its development and transition to the neglected stage;
  • the age of the manifestation of the first symptoms - from childhood to 60 years;
  • ataxia;
  • decrease in muscle tone( sometimes significant, a person can not perform routine tasks);
  • speech impairment( manifested from weak to strong);
  • trembling and twitching in the hands( tremor);
  • disorder during swallowing;
  • hyperkinesis;
  • disorders associated with eye movement( rarely).

Flicler-Winkler degeneration is manifested by the following features:

  • is inherited by an autosomal recessive method;
  • the age of onset of symptoms and the development of the disease throughout life, starting at age 20;
  • ataxia of the extremities.

If this species is diagnosed, there is no evidence of impaired motor activity.

The type with retinal degeneration has the following symptoms: ataxic movements

  • is an autosomal dominant transmission principle to the new generation;
  • affects young people and children;
  • ataxia;
  • extrapyramidal disorders;
  • changes in visual acuity( significant drop), there is a violation due to retinal pigmentation.

The appearance of the Jester-Haikman is marked by the following symptoms and manifestations:

  • is an autosomal dominant - the main method of inheritance;
  • manifestations and development in young( 20-30 years) or childhood;
  • ataxia;
  • characteristic for this type of symptom - facial paralysis;
  • swallowing disorders( sometimes severe);
  • speech defects;
  • vibration disturbance.

Type with dementia characterized by the following features:

  • autosomal dominant inheritance;
  • manifestation and development - in middle age, but not later than 40 years;
  • infringement of intelligence( sometimes very strong);
  • extrapyramidal disorders;
  • ataxia.

In general, the symptoms are similar, but the differences present enable doctors to put the right diagnosis and prescribe the most effective treatment.

Common Symptoms

A common symptom for all types of olivopontocerebellar degenerations is ataxia. In the beginning, a slight instability is observed in a person, then when walking fast, the patient experiences discomfort, expressed in awkward movements. Additional visual disturbances of gait:

  • frequent falls;
  • widely apart during foot walking;
  • fluctuations of the body from side to side( disruption of the cerebellum);
  • unstable position while sitting on a chair.

Late symptoms( disease progresses): tremor of hands

  • large handwriting;
  • dysmetry;
  • trembling in the extremities( tremor);
  • head shake;
  • decreased or increased reflexes;
  • speech impairment.

Also common symptoms include:

  • swallowing disorder( noted in most cases);
  • pyramidal failure.

Violation and ataxia Facial paresis is not a common symptom for all types, in contrast to incontinence.

A person almost always becomes listless and apathetic, loses interest in the events around him. It is often fixed retardation and even stupidity. Also, the disease is accompanied by dementia in varying degrees of severity, depressive states, fears and phobias appear that were previously absent. Hallucinations and confusion are the characteristic symptoms of this disease.

If the disease is in a neglected state, then the person gradually loses the ability to walk first, and then ceases to perform the simplest acts of caring for themselves. Often during this period, the patients weakened immunity, which leads to the development of concomitant diseases.

Diagnostic Criteria

In order to diagnose the disease, and then to know exactly its type, it will take several examinations. This is necessary to obtain objective and reliable information. One of the examinations is a neurological status. Neuropsychological data are also examined.

All results give the doctor an opportunity to find out if the patient has cerebellar degeneration, whether multiple sclerosis and other symptoms of olivopontocerebellar degeneration are present.

Objective information can be obtained by going through a computer tomography scan of the brain, and then a brain MRT.To determine the type of disease, you will need to consult a specialist in genetics.

Additionally, DNA diagnostics may be required. If there is a significant deterioration in vision, then the diagnosis is added to the consultation of the ophthalmologist.

Complex of measures - what can be done?

No special effective treatment for this disease has been created. Today, measures are being taken to maintain the indicators in averaged and close to normal values. In most cases, symptomatic treatment with a bias in neurology is carried out.

It also emphasizes the symptomatology present at the time of diagnosis.

Essential medicines - neurometabolites, anticholinergics, and also fortifying agents. To maintain optimal muscle tone, therapeutic massage and exercise therapy are provided. Such medications are also used: Cerebrolysin

  • glutamic acid;
  • Cerebrolysin( injections);
  • Aminalon;
  • Proserin.

Active outdoor activities and simple walks are part of the complex of measures for the treatment of the disease. If there are visual impairments, then the therapy includes appropriate drugs.

There will be no significant improvement in the state of human health. However, the current therapy will allow maintaining the indicators within the permissible limits for a long time, so the duration of the disease on average is 12 years, there are cases when patients could fight with such diseases for 20 years. The main cause of death is pneumonia or sepsis, as concomitant diseases.

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