La degeneración espinocerebral puede tener origen genético y aunque la ciencia ha avanzado mucho en los últimos años esta enfermedad. DEGENERACIÓN ESPINOCEREBRAL La degeneración espinocerebelosa o degeneración espino cerebral es una enfermedad en la cual las. Review Article. Enfermedades neurodegenerativas: La ataxia espinocerebelosa tipo 7 en México. Rolando Garcia-Martinez1*, Elizabeth Hernandez2, Rebeca.

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Physical therapy approach to spinocerebellar ataxia: The spinocerebellar ataxia SCA is an inherited disorder that leads to progressive degeneration of the cerebellum and its pathways with impairments of balance and other functions. Physical therapy studies for SCA treatment and their methodological quality were examined. We also investigated if the benefits achieved with treatment can be retained.

The interventions identified included balance, gait and coordination training; strengthening; weights around the limbs during exercise and transcranial magnetic stimulation. The long-term improvements were related to the degree of SCA evolution and the continuity of exercise practice. Nevertheless, further studies with higher scientific accuracy are necessary to elect the best espinocerebeloosa therapy approaches for SCA patients.

eNeurobiología – Revista electrónica

Since then, several classifications for these ataxias have been proposed by different authors. The evolution of genetic research enabled the connection between the clinical disorders of ataxia and the affected chromosomal locus.

The current classification based on genetic changes comprehends 31 types of SCA 4. Its prevalence is of 0. SCA 1, 3 and 6 are the most common ones throughout the world 1. All kinds of SCA cause progressive cerebellar degeneration, but each type has additional clinical characteristics related to the other affected regions, especially by type brain stem, cortex, bone marrow, cranial nerves and diencephalon 3.

The degeneration caused by SCA, among other problems, leads to balance and gait changes, which increase the risk of falls. In fact, Van de Warrenburg et al. These falls can induce a vicious circle of immobility and the fear of falling The progressive character of SCA, added to this vicious circle, leads to the accumulation of motor loss, thus producing an inexorable scenario of immobility and wheelchair dependency around the 15 th year of disease evolution 9.

There is evidence concerning an important contribution of the cerebellum in the motor learning process Several studies have demonstrated that patients with cerebellar lesions may present impairment in the learning of motor skills Therefore, some authors question if the rehabilitation of patients with SCA could have any positive effect on their motor condition. The validity of rehabilitation in such cases becomes even more controversial, given the progressive aspect of the disease: In this context, the objectives of this review are: Each search was performed with at least two combined descriptors so that one of them would be related to the pathology ex.: The search was complemented by related references cited by the found publications.

Clinical trials published in Portuguese, English and Spanish from on were included. There was no restriction as to the type of used physical therapy approach.

Identification, selection and the posterior quality assessment of the article by means of the physiotherapy evidence database scale PEDro 19 was made by two researchers, independently.

The articles were included when they received at least five out of the total of ten points in the PEDro scale. In the conducted search, studies were found. Out of these, were excluded for not meeting the inclusion criteria.

Among the six eligible studies, one of them did not reach the minimum score for inclusion Figure 1 and Table 1. Therefore, five articles were included in this review. After four weeks, patients were training at home for eight weeks. Scales assessing the staging of ataxia were used impairment severity: After practice, the staging of the disease improved for all of the patients. Only the group with cerebellar ataxia demonstrated significant balance improvement, increased velocity and step length, maintaining the benefits after eight weeks.

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This retention was not correlated with the staging of ataxia. To assess the long term efficacy of this protocol, Ilg et al. After this period, the staging of ataxia remained significantly better in relation to pre-intervention values.

The improvement in GAS was also maintained. The benefits for balance and gait speed, however, did not remain significant. The deyeneracion concluded that the treatment was efficient in the long term. Ten simple TMS pulses were applied once a day for 21 days. The stimulation group presented significant cadence improvement, as well as in relation to the time spent in the 10 m walking espinocerebeloda, besides the increased number of possible steps in tandem and the ability to stay standing.

After the 21 days of stimulation, 1 group of patients continued to receive TMS pulses once or twice a week. They retained the improvement obtained in the study for at least six months. Another group received TMS pulses only every two weeks.

In this group, the scores of the used assessment methods returned to baseline two weeks after the end of applications. Only the immediate group performed 1 hour daily trainings with conditioning exercises, amplitude of movement, muscle strengthening, stairs, static balance and gait for four weeks. In order to assess both groups, some tools were used: After practice, the immediate group presented improvements in all of the evaluations, except for cadence, when compared to the control group.

After the four weeks, the control group was also trained and their data were analyzed together espinoceregelosa the immediate group, accounting for 42 assessed patients before, right after and 4, 12 and 24 weeks after intervention. After two years, only the improvement in gait speed was maintained. However, it was observed that 22 out of the 42 patients had retained improvement in at least one of the assessed parameters during the period.

By analyzing the correlation between staging and the assessed parameters, it was concluded that patients who retained some sort of improvement were those with less degenracion degrees of ataxia. The practice consisted of lateral gait, in a straight line, with obstacles and movements of the upper limbs. After 20 sessions of 30 min, there was significant balance improvement, as well as in the risk of falls, in FIM and in the staging of ataxia, only for the weight group.

These results were maintained 30 days after the end of the intervention.

The authors defend gait improvement even if there were no differences in Dynamic Gait Index scores. Out of the five studies analyzed in this review Table 2none of them investigated only patients with SCA. Individuals with ataxia of other etiologies were also included. Even so, the studies report the positive impact of physical therapy for patients with SCA.

This suggests that the interventions used for ataxias of other etiologies can also be useful for the SCA treatment, even though this fact is not completely clear. Only three studies compared the performance of a trained group and a control group. This limitation does not make it possible to rule out the placebo effect, nor to assess if the benefits reported by them are only due to the proposed training.

Only Dias et al. This is essential to observe the improvement in the quality of their daily lives. Out of degenerzcion five studies in this review, four of them 20,21,23,24 espinocerfbelosa the staging of SCA. This is necessary to investigate if, after treatment, the clinical condition of the patient, in short and long terms, became worsen, better or stable.


Such description can also help identify the adequate treatments for the different stages degeneracioj the disease. In the long term, Miyai et al. Apparently, maintaining the improvement obtained with interventions depends on continuing to practice exercises.

Shiga 22 also maintained the application of TMS pulses with better results in the group that continued to receive frequent applications. On the other hand, Dias et al.

autosomal dominant cerebellar ataxia – Wikidata

The gain retention described in some studies may indicate that patients with cerebellar lesions are able to retain learning, even though their lesions are located in the cerebellum, which is such an important area for motor learning. Still, it is hard to distinguish if the lack of benefit retention after an exercise program is owed to the progressive degeneration, characteristic of the disease, to the inability to retain the learned movement patterns or to the need to elect more appropriate exercises for these patients.

Most of the found studies employed balance, gait, muscle strengthening, stretching and coordination exercises. However, none determined which the most efficient one was. Therefore, the good results found in this study indicate that different types of exercise could be equally beneficial for these patients.

Indeed, Fryer et al. These findings may corroborate the idea that immobility leads to motor loss, which goes beyond those promoted by the characteristic degeneration of SCA itself.

Therefore, it seems that keeping patients active alone can already being some sort of benefit.

Additionally, Dias et al. Most studies, except for the one of Ilg et al. Such details are important to ensure replicability in new studies, including multicenter clinical trials, and to enable the reproduction of the protocol in clinical practice.

In this review, studies demonstrating the positive effects of interventions to treat the sequels of SCA were identified: Treatment seems to be more efficient less impaired patients. Besides, retaining the improvements obtained in the long term was related to the degree of disease evolution and to the eapinocerebelosa practice of exercises, even if at home.

Future studies including only patients with SCA, which would describe the staging of the disease, including a proper control group, blind evaluators, description of the impact of treatment on activities and participation and containing a detailed description of the intervention protocol are necessary to select the most adequate approaches for the treatment of espinoccerebelosa with SCA. These studies may contribute with the elaboration of protocols based on important evidence to face the problems caused by SCA properly.

The authors would like to thank Anke Bergman for reviewing the methodology of this article. Brain pathology of spinocerebellar ataxias. The clinical features and classification of the late onset autosomal dominant cerebellar ataxias. A study of 11 families, including descendants of degenetacion “the Drew family of Espinoderebelosa. Klockgether T, Paulson H. Autosomal dominant cerebellar ataxias: Marsden J, Harris C. Frequency of spinocerebellar ataxia type 1, dentatorubropallidoluysian atrophy, and Machado-Joseph disease mutations in a large group of spinocerebellar ataxia patients.

Falls in degenerative cerebellar ataxias.

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