What is ataxia?
Consistent disorder in the lack of muscular coordination, which makes it difficult to perform voluntary movements : this is the definition of ataxia specified by the AISA, the Italian Association for the fight against Ataxic Syndromes.
To give a better idea, let's take an example. The ataxia in mild form is comparable to the walk of a drunk; in fact, a subject in the throes of alcoholic intoxication has an obvious uncertain bearing: he advances in different steps, with a different width from one another, the gait is not straight and the state of intoxication makes it difficult to maintaining a standing position.
Other articles on ataxia:
- Ataxia: classification
- Ataxia: symptoms and causes
- Ataxia: diagnosis and therapy
- Friedreich's ataxia
- Cerebellar ataxia
- Charcot-Marie Tooth Ataxia
- Ataxia in brief: Summary on ataxia
Generality
An ataxic subject presents movements of reduced or excessive amplitude, the gait is not stable, but uncertain and shaky; moreover, the uncoordination between movements is established between the trunk and the head, between the trunk and the arms or, again, between the legs and the trunk: due to these characteristics, an ataxia patient tends to fall. In ataxic syndromes, there is simultaneous spasms and contraction of antagonistic muscle bundles.
Considering that the muscular movements are controlled by the cerebellum, directly connected to the spinal cord and to the peripheral nerves, the possible lesions at the level of these sites can generate ataxia: the cerebellum represents, in fact, the center of regulation and coordination of voluntary muscular movements; a hypothetical damage located in the center of the cerebellum is able to block the interconnection between the other sections of the CNS, generating mild ataxia, a typical walk of the state of intoxication. When, on the other hand, a lateral site of the cerebellum is injured, it is observed that there is only half of the body, corresponding to the compromised hemicetellum.
There are also genetic forms of ataxia, that is transmitted from the parent to the children.
Degeneration of ataxia
Ataxias, and the corresponding ataxic syndromes, are subtle disorders that begin with few signs, but degenerate slowly and progressively. In general, the first manifestations involve the legs, then the arms, to then also affect the voice, compromising the articulation of the word (ataxia of the word), the musculature, the hearing and the sight. [taken from www.atassia.it]
Ataxia and ataxic syndromes
By convention, the terms ataxia and ataxic syndrome are used without distinction, since they refer to the same disorders. In reality, it would not be entirely correct, since "ataxic syndrome" indicates a set of invalidating genetic diseases, while "ataxia" represents the main symptom, as well as the progressive loss of the ability to perform a voluntary and controlled movement, directed towards the correct direction. With the ineluctable evolution in a negative sense of ataxia, the subject shows a clear aggravation of motility, accompanied by a pain of variable intensity but always present, responsible for the overall worsening of the ataxic syndrome.
Incidence
Ataxic syndromes are quite rare: in Italy, it is estimated that approximately 5, 000 subjects are affected. Unfortunately, however, the prognosis is not good, since ataxic syndromes are often heavily debilitating pathologies of the CNS, which are characterized by a slow but progressive loss of muscular control and motor coordination.
The incidence of ataxic syndromes is not well known: however, it appears that 4.5-6.4 subjects are affected per 100, 000 healthy individuals [from pediatric neurology, by Lorenzo Pavone, Martino Ruggieri].
The age of onset of the very first ataxic manifestations varies according to the type of ataxia: for example, the target of Friedreich's ataxia is given by infants.
Ataxia and associated diseases
It is not uncommon for ataxia to be accompanied by other disorders, of more or less severe intensity, such as: incontinence, difficulty swallowing, uncoordination and slowness of eye movements, and other uncontrolled and involuntary gestures of the head, trunk and lower limbs or above. In some cases, ataxia can also lead to memory loss, and neurological damage could lead to cardiac disorders and bronchopulmonary complications [from www.atassia.it].
Deepening: AISA onlus
In order to encourage scientific research of immunological-genetic-molecular type on ataxias, in 1982, in Lombardy, AISA was born, the Italian Association for the fight against Ataxic Syndromes, legally recognized by the Ministry of Health only in 1995: this association, made up of volunteers, aims to promote the forms of prevention of ataxia, to inform and sensitize the population, and to support the ataxic patients, powerless in the face of this debilitating disease.
