RESUMEN
[Purpose] The aim of this study was to investigate whether the combination of integrated volitional control functional electrical stimulation and tilt sensor functional electrical stimulation training affected brain activation during the subacute phase following a stroke. [Participant and Methods] The patient was a 60-year-old male with right hemiparesis, secondary to stroke in the left thalamus. Conventional intervention was performed for 60 minutes per day during the first two weeks of treatment (the control condition). Functional electrical stimulation intervention, including integrated volitional control functional electrical stimulation and tilt sensor functional electrical stimulation training, was then performed for 60 minutes per day for two weeks (the experimental condition). These sessions were repeated four times. Brain activity was measured during voluntary right ankle dorsiflexion in both sessions, using functional magnetic resonance imaging. Brain activity measurements were obtained a total of eight times every two weeks (34, 48, 62, 76, 90, 104, 118, and 132 days following the stroke). [Results] There was a significantly higher level of activation in the bilateral cerebellum and the left side of the supplementary motor area in the experimental condition than in the control condition. [Conclusion] The present study demonstrates that the combination of integrated volitional control functional.
RESUMEN
The majority of cases of anti-glutamic acid decarboxylase (GAD)-antibody-positive cerebellar ataxia are reported to have high levels of anti-GAD antibody, and the diagnostic value of low titers of anti-GAD antibody in a patient with cerebellar ataxia is still unknown. The purpose of this study was to verify the characteristics of low-titer-anti-GAD-antibody-positive cerebellar ataxia patients and the diagnostic value of low titers of anti-GAD antibody in patients with cerebellar ataxia. The subjects were six patients positive for low-titer GAD antibody (<100 U/mL). We examined them with MRI, including voxel-based morphometry, and with single-photon emission computed tomography and monitored the GAD antibody index in the cerebrospinal fluid. The levels of antineuronal, antigliadin, anti-SS-A, antithyroid antibodies, and of vitamins E, B1, and B12 were determined. Thoracic and abdominal CT scans were performed to exclude a paraneoplastic origin. We treated three patients with immunotherapy. All cases showed cortical cerebellar atrophy. The GAD antibody index in three of the five patients reviewed was >1.0. Two of the six patients were thyroid antibody-positive, and one was both antinuclear- and anti-SS-A antibody-positive. After the administration of immunotherapy to three patients, two showed clear effectiveness, and one, transient effectiveness. Effectiveness was greatest in the two patients with familial occurrence of the disease. In cerebellar ataxia, regardless of family history or isolated illness, it is critical to measure the GAD antibody level, and, even with a low titer level, if the result is positive, immunotherapy should be considered.
Asunto(s)
Autoanticuerpos/metabolismo , Ataxia Cerebelosa , Glutamato Descarboxilasa/inmunología , Anciano , Ataxia Cerebelosa/sangre , Ataxia Cerebelosa/inmunología , Ataxia Cerebelosa/terapia , Corteza Cerebelosa/patología , Femenino , Humanos , Inmunoterapia , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Tomografía Computarizada de Emisión de Fotón ÚnicoRESUMEN
OBJECTIVE: It has been reported that autoimmune cerebellar ataxias, such as anti-glutamic acid decarboxylase (GAD)-antibody-positive cerebellar ataxia and gluten ataxia, are treatable. Here, we examined the therapeutic efficacy of intravenous immunoglobulin (IVIg) on autoantibody-positive cerebellar ataxia. PATIENTS AND METHODS: IVIg therapy was administered in seven autoantibody-positive cerebellar ataxia patients. Therapeutic efficacy was examined in terms of its effects on clinical symptoms and changes in brain perfusion using single photon emission computed tomography (SPECT). RESULTS: Treatment was effective in four cerebellar cortical atrophy patients (two anti-GAD antibody-positive and two anti-gliadin antibody-positive) and in one anti-thyroid antibody-positive spinocerebellar ataxia type 3 (SCA3) patient, but not in two multiple system atrophy (MSA) patients. All four IVIg effective patients who underwent SPECT showed apparent increases in cerebellar perfusion. CONCLUSION: If cerebellar ataxia with an autoimmune mechanism is suspected and radiological findings do not reveal MSA, it is worth considering immunotherapy including IVIg.
Asunto(s)
Autoanticuerpos/sangre , Ataxia Cerebelosa/inmunología , Ataxia Cerebelosa/terapia , Inmunoglobulinas Intravenosas/uso terapéutico , Anciano , Anciano de 80 o más Años , Ataxia Cerebelosa/diagnóstico por imagen , Cisteína/análogos & derivados , Femenino , Gliadina/inmunología , Glutamato Descarboxilasa/inmunología , Humanos , Yoduro Peroxidasa/inmunología , Masculino , Persona de Mediana Edad , Compuestos de Organotecnecio , Radiofármacos , Tomografía Computarizada de Emisión de Fotón Único , Resultado del TratamientoRESUMEN
In slowly progressive cerebellar atrophy, it has been difficult to suppress the progression of cerebellar symptoms because no effective therapeutic agents are available when the diagnosis of secondary cerebellar atrophy, such as drug-induced cerebellar atrophy or paraneoplastic syndrome, is denied. However, amongst the different forms of slowly progressive cerebellar atrophy, some may be associated with treatable immune abnormalities. Therefore, we investigated the therapeutic efficacy of intravenous immunoglobulin (IVIg) in 9 patients with slowly progressive cerebellar atrophy (4 sporadic atrophy; 5 hereditary atrophy). The results were as follows. With regard to the 4 cases of sporadic atrophy, gait ataxia and imbalance were markedly improved in 1 patient who had positive anti-GAD antibody. Moderate improvement was seen in 1 patient and slight improvement in 2. With regard to the 5 cases of hereditary atrophy, gait ataxia and imbalance were moderately improved in 2 patients with SCA3, although there were 3 non-responders. In conclusion, our study results suggested that not only patients with sporadic atrophy but also some with hereditary atrophy may respond to therapy. In cases of slowly progressive cerebellar atrophy in which the cause may be due to immune abnormality, we should consider instituting active immunotherapy when a pathological state caused by immune abnormality is suspected after extensive evaluations of autoantibodies, including anti-GAD, anti-thyroid and anti-gliadin antibody, malignancy, and so on.