DISEASE OF HUNTINGTON

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	DISEASE OF HUNTINGTON
	*DISEASE OF HUNTINGTON*

DISEASE OF HUNTINGTON

Disease of Huntington

A hereditary degenerative disease of the brain that initially affects mainly the motor coordination.

The disease in general from the group of forty appears in both sexes, pronouncing itself, although it can appear before, being then of faster course.

It affects to between 7 and 10 by 100,000 in Europe and the United States de America.

Complications

The infections and the traumatismos are frequent.

Prognosis

The progression of the disease usually is slow, happening between 15 to 20 years from the beginning of the symptoms to the death.

Against precocious is the beginning of the disease, faster usually it is the progression.

At the moment, although there are very promising open lines of investigation, does not exist treatment for this disease.

Prevention

Genetic advice (to the carriers).

By means of techniques of molecular genetics the prenatal diagnosis can be made.

Diagnosis and treatment

DIAGNOSIS

The diagnosis is made by means of the clinical visit and the neurological exploration.

By means of techniques of molecular genetics the presymptomatic diagnosis can be made.

Sometimes complementary tests will be used, like imaginería cerebral (TAC, RMN or others) to discard other causes of the clinic.

TREATMENT

It does not have effective treatment.

General measures

Aids for the deambulación, safety measures in the surroundings of the patient.

Antidepressants,

Dopaminérgicos antagonists

Activity

All the activities will be made that the state of the patient allows.

Diet

Sometimes it can be necessary to resort to the pastosas or liquid diets.

Warn its doctor if...

It has fever.

It presents/displays new symptoms: the medicines that can prescribe to him have indirect effect.

Signs and symptoms

Appearing on the 35 to 40 years, it begins with motor alterations:

gesticulación,

alterations in the vocalization,

irregular and arrhythmic movements of the legs.

Shortly after it appears a parallel progressive dementia to the motor disorders, being able to predominate:

depression,

alterations of the behavior,

touching instability and

progressive dementia.

Factors of risk

Hereditary in most of the cases.

Causes

It has been discovered that the disease causes a genetic mutation that extends the end of a proteina, turning it toxic for the cerebral cells, the neurons.

Its accumulation produces the progressive death of the neurons, since it causes that they commit suicide.

In general it is a dominant hereditary disease (one pronounces whenever the defect of an ancestor is transmitted), but spontaneous cases occur.

The probability of transmitting the disease to a descendant is of 50%.

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