ABSTRACT
Objective：We aimed to determine the impact of the COVID-19 pandemic on the “body function”, “activity”, and “participation” domains of functioning as defined in the International Classification of Functioning (ICF) for individuals with spinocerebellar degeneration (SCD) and multiple system atrophy (MSA).Methods：A questionnaire survey was administered to 1,000 members of the SCD/MSA patient association in Japan between November and December 2020. Questions related to COVID-19 were extracted from the multipurpose data, and the degree of COVID-19-related effects on each life function was assessed using a 7-point scale. Results were stratified into two groups based on age, care needs based on long-term care insurance coverage, and limited opportunities for rehabilitation. The χ2 test was used to examine differences between pairs of groups regarding impacts on functioning.Results：Data from 460 participants with SCD and MSA were analyzed. Participation restriction was reported in 54％ of participants with SCD and 46％ of participants with MSA, and impairment and activity limitation in approximately 20％ of participants with both conditions resulting from the COVID-19 pandemic. Participants with low care needs reported more impairment in SCD and more participation restriction in MSA. About 24％ of subjects reported limited opportunities for rehabilitation. MSA participants with these limitations reported greater impairment.Conclusion：The COVID-19 pandemic has had a particular impact on the participation domain of functioning in participants with SCD and MSA. The domains of body function and participation were found to be affected in participants with low care needs or limited opportunities for rehabilitation.
ABSTRACT
Objective：The purpose of this study was to evaluate the effectiveness of a four-week short-term intensive rehabilitation on ataxia in patients with ambulatory spinocerebellar degeneration (SCD), using the Scale for the Assessment and Rating of Ataxia (SARA) total score and sub-score.Methods：This study included 23 SCD patients；we considered those with a SARA gait score of＜3 points. Our program included a one-hour training session with individualized instructions and a one-hour self-balance training session. Additionally, occupational or speech-language-hearing therapy was performed for one hour daily. Our program was conducted five days weekly for four weeks. We assessed the SARA total score and sub-score findings immediately, before the first and after the last interventions.Results：The SARA total, gait, stance, and heel-shin scores showed more significant improvement after intervention than before intervention (p＜0.05). The other SARA sub-scores were not significantly different between “before and after” interventions.Conclusion：The four-week short-term intensive rehabilitation on ataxia in patients with ambulatory spinocerebellar degeneration improved the SARA total score and ataxia of the trunk and lower limbs.
ABSTRACT
Objective：The purpose of this study was to evaluate the effectiveness of a four-week short-term intensive rehabilitation on ataxia in patients with ambulatory spinocerebellar degeneration (SCD), using the Scale for the Assessment and Rating of Ataxia (SARA) total score and sub-score.Methods：This study included 23 SCD patients；we considered those with a SARA gait score of＜3 points. Our program included a one-hour training session with individualized instructions and a one-hour self-balance training session. Additionally, occupational or speech-language-hearing therapy was performed for one hour daily. Our program was conducted five days weekly for four weeks. We assessed the SARA total score and sub-score findings immediately, before the first and after the last interventions.Results：The SARA total, gait, stance, and heel-shin scores showed more significant improvement after intervention than before intervention (p＜0.05). The other SARA sub-scores were not significantly different between “before and after” interventions.Conclusion：The four-week short-term intensive rehabilitation on ataxia in patients with ambulatory spinocerebellar degeneration improved the SARA total score and ataxia of the trunk and lower limbs.
ABSTRACT
Objective：This study aimed to identify the characteristics of daily rehabilitation in patients with spinocerebellar degeneration (SCD), including those with multiple system atrophy, with a focus on physical status and social background.Methods：We analyzed questionnaire responses from 914 patients in Japan and conducted inter-group comparisons of backgrounds and rehabilitation-related information by dividing patients into a rehabilitation group, a self-directed exercise group, and a non-rehabilitation group.Results：Among all subjects, the rehabilitation group accounted for 67.9％, the self-directed exercise group for 17.7％, and the non-rehabilitation group for 14.3％．In the rehabilitation group, the percentage receiving services increased with a decline in the level of independent living and increase in coverage by long-term care insurance. The frequency and duration of rehabilitation showed a maximum distribution of 2-3 sessions/week for 40 minutes per session in the rehabilitation group and 7 sessions/week for 20 minutes per session in the self-directed exercise group. The intent to continue in the rehabilitation group was significantly greater than in the self-directed exercise group (p＝0.018), although subjective changes with rehabilitation showed no difference between groups.Conclusion：The percentage of SCD patients who participated in rehabilitation programs including self-directed exercise was high. However, the subjective effect was not clear. We consider it necessary to develop an environment in which experts can provide rehabilitation to all SCD patients, irrespective of the disease stage.
ABSTRACT
Objective：This study aimed to identify the characteristics of daily rehabilitation in patients with spinocerebellar degeneration (SCD), including those with multiple system atrophy, with a focus on physical status and social background.Methods：We analyzed questionnaire responses from 914 patients in Japan and conducted inter-group comparisons of backgrounds and rehabilitation-related information by dividing patients into a rehabilitation group, a self-directed exercise group, and a non-rehabilitation group.Results：Among all subjects, the rehabilitation group accounted for 67.9％, the self-directed exercise group for 17.7％, and the non-rehabilitation group for 14.3％．In the rehabilitation group, the percentage receiving services increased with a decline in the level of independent living and increase in coverage by long-term care insurance. The frequency and duration of rehabilitation showed a maximum distribution of 2-3 sessions/week for 40 minutes per session in the rehabilitation group and 7 sessions/week for 20 minutes per session in the self-directed exercise group. The intent to continue in the rehabilitation group was significantly greater than in the self-directed exercise group (p＝0.018), although subjective changes with rehabilitation showed no difference between groups.Conclusion：The percentage of SCD patients who participated in rehabilitation programs including self-directed exercise was high. However, the subjective effect was not clear. We consider it necessary to develop an environment in which experts can provide rehabilitation to all SCD patients, irrespective of the disease stage.
ABSTRACT
Ryoukeigomikanzoto is a Kampo formulation originally described in the text <i>Kinkiyouryaku</i>. This formulation has also been described as an indication for respiratory disease in some texts, because it was classified under respiratory diseases in the <i>Kinkiyouryaku</i>.<br>The authors, however, considered that indications for ryoukeigomikanzoto could be wider than previously thought. The composition of this formulation is very similar that of ryokeimikanto and ryokeijutsukanto. And we have prescribed ryokeigomikanzoto for patients with a reddish face and coldness of the legs, whose chief complaints were a reddish nose, coldness of the leg, dysuria, spioncerebellar degeneration and ringing of the ears. Moreover, we have obtained good results from the current trial. This report is intended to elucidate indications for this formulation by means of past clinical results and our own experience.
ABSTRACT
The specific effect of underwater exercise in a 61-year old woman with cerebellar ataxia induced by spinocerebellar degeneration was observed. She was treated through home visit rehabilitation. She was admitted to this hospital in 2004 and started underwater exercise. She received 40 minutes of physical therapy three times a week as well as 40 minutes of occupational therapy and 30 minutes of underwater exercise three times a week. After that training, her cerebellar ataxia was relieved, and she could walk indoors with good stability using a T cane. During 5 hospitalizations in 3.5 years, her cerebellar ataxia improved and her walking was stabilized.<br>Some differences in her rehabilitation during hospitalization compared to when she was at home was that occupational therapy was added and that the training frequency was increased five times by physical therapy and occupational therapy for one week in total, and that underwater exercise was added. In regards to the improvement of her dysfunction in terms of daily living, a trunk and an inferior limb were important factors. Therefore, we think that intervention through physical therapy and underwater exercise was significant. Also, the difference between her physical therapy during hospitalization and when at-home was three times a week during hospitalization and twice a week when at home. Therefore, we determined there was a large difference of her rehabilitation effected by the underwater exercise. There is a possibility the underwater exercise improved her intension tremor and dysmetria, stabilized her indoor walking, and extended the distance she walked outdoors.<br>Underwater exercise is a plural classical training method that is reasonably safe while at the same time providing rehabilitation for cerebellar ataxia, and this exercise is expected to provide addition due to the warm temperature of the water.<br>The authors propose that new rehabilitation using underwater exercise for cerebellar ataxia be considered.
ABSTRACT
PURPOSE: To report a patient with dentatorubropallidoluysian atrophy (DRPLA) accompanied by a corneal endothelial change. METHODS: The ophthalmic findings in a 37-year-old man with cerebellar ataxia, showing cerebellar atrophy on magnetic resonance imaging and was diagnosed with DRPLA based on DNA analysis, which revealed an expansion of the CAG repeats at the DRPLA locus, were compared with those of healthy control subjects. This study examined the best corrected visual acuity, the light reflex, the slit lamp examination, the topography, the fundus findings, the fluorescein angiography (FAG), the visual field, the electroretinography (ERG), and the specular microscopy. RESULTS: The best-corrected visual acuity was 1.0 on both eyes by the Han visual chart, and the other ocular findings were within the normal limits. The only abnormal finding was significantly lower corneal endothelial cell density (876 and 941 cells/mm2 respectively) compared to the normal level. CONCLUSIONS: Corneal endothelial cell loss is the only an important finding of DRPLA, and the corneas of DRPLA patients should be examined specifically by specular microscopy.
Subject(s)
Adult , Humans , Atrophy , Cerebellar Ataxia , Cornea , Corneal Endothelial Cell Loss , DNA , Electroretinography , Endothelial Cells , Endothelium, Corneal , Fluorescein Angiography , Magnetic Resonance Imaging , Microscopy , Reflex , Visual Acuity , Visual FieldsABSTRACT
Studies on spinocerebellar ataxias (SCA) have been hampered by a lack of disease markers. Clinical and pathological heterogeneity also made the classification unreliable. Linkage studies established that there are multiple subtypes of SCA. Five types are found to have unstable CAG expansion; the diagnosis can be established by molecular genetic study. Therefore, we systemically screened degenerative ataxia patients for these five SCA types, and identified eight patients with SCA2 (seven from six families and one sporadic case). This paper presents the clinical information on the seven patients, whose clinical information was available in detail. CAG repeat expansion in the patients ranged from 38 to 47 (normal control, 19 to 27). The onset ages ranged from 16 to 41 with 27.1 years as the mean, which correlated inversely with repeat lengths. All patients presented dysarthria and gait ataxia. Upper limb dysmetria or dysdiadochokinesia appeared later but progressed, causing severe disability. Slow saccade (4 patients in 7) and decreased DTR (4 in 7) were common. MRIs showed severe atrophy of the brainstem and cerebellum in all patients. We conclude that SCA2 is the most frequent type in Korea and carries rather pure cerebellar syndrome, slow saccade, and hyporeflexia.
Subject(s)
Adult , Female , Humans , Male , Adolescent , Age of Onset , Brain/pathology , DNA Mutational Analysis , Korea , Lymphocytes , Magnetic Resonance Imaging , Spinocerebellar Ataxias/genetics , Spinocerebellar Ataxias/diagnosis , Spinocerebellar Ataxias/blood , Trinucleotide Repeats/geneticsABSTRACT
To investigate the effects of Kampo and a rehabilitation approach on patients with spinocerebellar degeneration (SCD), a 65-year-old female patient with SCD was treated with Ogi-kenchu-to and therapeutic exercise based on the Bobath concept.<br>She had a gait disturbance (unable to walk) with slightly increased muscle tonus and deep sensory disturbance in the left leg before therapy. In Kampo confirmations, there was a generalized cold feeling and fatigue due to decreased physical fitness. In the electrophysiological study, the amplitude of Auditory Brainstem Responses (ABR) and Somatosensory Evoked Potentials (SEP) in the left side showed a greater decrease than those in the right side. The amplitude of the F-wave in the left side showed a greater increase than that of the right side.<br>After two months of this therapy, the patient was able to walk unassisted. Improvement in the neurological and general findings, along with normalization of the ABR, SEP and F-wave were also observed.<br>It was suggested that Kampo therapy using Ogi-kenchu-to and therapeutic exercise based on the Bobath concept were effective in the treatment of this patient with SCD.