RESUMEN
Neuromyelitis optica (NMO) is a severe inflammatory demyelinating disease with exacerbations involving recurrent or bilateral optic neuritis and longitudinally extensive transverse myelitis. Pulse steroid therapy is recommended as the initial, acute-phase treatment for NMO. If ineffective, treatment with plasma exchange (PE) should commence. However, no evidence exists to support the effectiveness of PE long after the acute phase. Immunoadsorption therapy (IA) eliminates pathogenic antibodies while sparing other plasma proteins. With IA, side effects of PE resulting from protein substitution can be avoided. However, whether IA is effective for NMO remains unclear. We describe a patient with anti-aquaporin-4-positive myelitis who responded to IA using a tryptophan polyvinyl alcohol gel column that was begun 52 days after disease onset following the acute phase. Even long after the acute phase when symptoms appear to be stable, IA may be effective and should not be excluded as a treatment choice.
Asunto(s)
Técnicas de Inmunoadsorción , Neuromielitis Óptica/inmunología , Neuromielitis Óptica/terapia , Enfermedad Aguda , Adulto , Acuaporina 4/inmunología , Autoanticuerpos/sangre , Autoanticuerpos/aislamiento & purificación , Enfermedad Crónica , Humanos , Masculino , Fuerza Muscular , Neuromielitis Óptica/fisiopatología , Intercambio Plasmático , Plasmaféresis , Esteroides/uso terapéutico , Resultado del TratamientoRESUMEN
Gluten ataxia, a type of cerebellar ataxia caused by exposure to gluten in sensitive patients, has been considered common in the USA and Europe, and rare in Asia. We measured anti-deamidated gliadin peptide (DGP) antibody levels in 49 patients with cerebellar ataxia, excluding those with multiple system atrophy, hereditary spinocerebellar ataxia, or cancer, as well as those who were receiving oral administration of phenytoin. Anti-DGP antibody was positive in eight (16.3 %) patients, five of these patients were positive only for IgA, one was positive for both IgG and IgA, and two were positive only for IgG antibody. Intravenous immunoglobulin was administered to five of the eight patients, and was markedly effective in one, moderately effective in two, and ineffective in two. Steroid therapy was administered to four patients, but none had an apparent response. Ataxia symptoms improved in one patient treated with a gluten-free diet only. Although it had been thought to be extremely rare in Asia, we speculate that more than 10 % of cerebellar ataxia patients in Japan currently have gluten ataxia; therefore, measuring anti-DGP antibody or anti-gliadin antibody in cerebellar ataxia patients in Asia is important.
Asunto(s)
Ataxia Cerebelosa/inmunología , Ataxia Cerebelosa/terapia , Gliadina/inmunología , Glútenes/efectos adversos , Enfermedades Metabólicas/inmunología , Anciano , Anciano de 80 o más Años , Autoanticuerpos/sangre , Encéfalo/patología , Ataxia Cerebelosa/epidemiología , Ataxia Cerebelosa/patología , Dieta Sin Gluten , Femenino , Humanos , Inmunoglobulina A/sangre , Inmunoglobulinas Intravenosas/uso terapéutico , Factores Inmunológicos/uso terapéutico , Inmunoterapia , Japón/epidemiología , Imagen por Resonancia Magnética , Masculino , Enfermedades Metabólicas/epidemiología , Enfermedades Metabólicas/patología , Enfermedades Metabólicas/terapia , Persona de Mediana Edad , Esteroides/uso terapéutico , Resultado del TratamientoRESUMEN
OBJECTIVE: With conventional MRI and single-photon emission computed tomography (SPECT), accurate diagnosis and precise classification of cerebellar atrophy are often difficult. The objective was to verify the utility of MRI voxel-based morphometry (VBM) in combination with SPECT using easy Z-score imaging (eZIS) for diagnosing and classifying cerebellar atrophy. PATIENTS AND METHODS: We assessed gray matter atrophy using VBM and blood perfusion using SPECT with eZIS in fifteen patients with different types of cerebellar atrophy, such as the cerebellar variant of multiple system atrophy (MSA-C), spinocerebellar ataxia type 3 (SCA3), SCA6, and autoimmune cerebellar ataxia (AICA). RESULTS: In all five MSA-C patients, VBM imaging showed atrophy of the brainstem, the entire cerebellar vermis, and the cerebellar hemispheres, while SPECT using eZIS showed reduced perfusion in the same regions. Regarding SCA3, brainstem atrophy and reduced perfusion were recognized in two of the four patients, but none exhibited abnormal findings in the posterior lobe of the cerebellar vermis. SPECT showed that all four patients had obviously reduced perfusion in the anterior lobe of the vermis, but VBM demonstrated that there was no obvious atrophy of gray matter in any patient, meaning that the results of SPECT and VBM contradicted each other completely. All SCA6 and AICA patients exhibited atrophy and reduced perfusion in the cerebellar hemispheres but not in the brainstem. Only one AICA patient exhibited atrophy and reduced perfusion of the entire cerebellar vermis. CONCLUSION: VBM clearly showed characteristic gray matter atrophy in the cerebellum and brainstem in different pathological conditions, thus indicating its high degree of utility in diagnosing and classifying cerebellar atrophy in combination with SPECT using eZIS.
Asunto(s)
Enfermedades Cerebelosas/clasificación , Enfermedades Cerebelosas/diagnóstico por imagen , Cerebelo/diagnóstico por imagen , Cerebelo/patología , Diagnóstico por Imagen/métodos , Tomografía Computarizada de Emisión de Fotón Único/métodos , Atrofia/diagnóstico por imagen , Atrofia/patología , Tronco Encefálico/diagnóstico por imagen , Tronco Encefálico/patología , Ataxia Cerebelosa/diagnóstico por imagen , Ataxia Cerebelosa/patología , Humanos , Imagen por Resonancia Magnética , Ataxias Espinocerebelosas/diagnóstico por imagen , Ataxias Espinocerebelosas/patologíaRESUMEN
A 52-year-old woman was admitted to the hospital because of appetite loss, unsteadiness, psychogenic symptoms, ataxia, and consciousness disturbance as a result of the ingestion of a diet restricted to only carbohydrates for a long term. Laboratory examination indicated the presence of pancytopenia with macrocytic anemia; further, decreased vitamin B1 and B12 levels were detected in her serum. Magnetic resonance imaging fluid attenuated inversion recovery (FLAIR), revealed high-signal intensity in the bilateral corpus striatum, third ventricle circumference, and cerebellar cortex. Thereafter, she received drip infusion that did not include vitamin B1 or B12 and subsequently suffered a cardiac arrest due to the aggravation of cardiac insufficiency; consequently, she was transferred to our hospital. Upon admission the patient was diagnosed to have obvious cardiomegaly with pleural effusion; further, a negative T-wave was obtained on the electrocardiogram. A diagnosis of beriberi heart disease was made because of thiamine deficiency. She was treated by thiamine administration, following which the cardiac symptoms improved immediately. Various neurological symptoms caused by encephalopathy, peripheral neuropathy and subacute combined spinal cord degeneration improved by treatment with thiamine and cyanocobalamine administration; however, some of these symptoms still remained. General awareness of the fact that neurological symptoms can be caused by vitamin deficiency is essential.
Asunto(s)
Beriberi/etiología , Cardiomegalia/etiología , Cuerpo Estriado/patología , Deficiencia de Ácido Fólico/complicaciones , Paro Cardíaco/etiología , Degeneración Combinada Subaguda/etiología , Deficiencia de Tiamina/complicaciones , Deficiencia de Vitamina B 12/complicaciones , Encefalopatía de Wernicke/etiología , Beriberi/diagnóstico , Beriberi/tratamiento farmacológico , Cardiomegalia/diagnóstico , Cardiomegalia/tratamiento farmacológico , Femenino , Ácido Fólico/administración & dosificación , Paro Cardíaco/diagnóstico , Paro Cardíaco/tratamiento farmacológico , Humanos , Persona de Mediana Edad , Degeneración Combinada Subaguda/diagnóstico , Degeneración Combinada Subaguda/tratamiento farmacológico , Tiamina/administración & dosificación , Deficiencia de Tiamina/diagnóstico , Deficiencia de Tiamina/tratamiento farmacológico , Resultado del Tratamiento , Vitamina B 12/administración & dosificación , Deficiencia de Vitamina B 12/diagnóstico , Deficiencia de Vitamina B 12/tratamiento farmacológico , Encefalopatía de Wernicke/diagnóstico , Encefalopatía de Wernicke/tratamiento farmacológicoRESUMEN
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
We present the case of a 51-year-old man with a 5-year history of slowly progressive gait ataxia and dysarthria who showed a wide-based gait requiring assistance. The patient's score on the Revised Hasegawa Dementia Scale (HDS-R) was 22/30 and constructional apraxia was also evident. Cerebrospinal fluid analysis showed 3 cells/microl, and the protein concentration was 58 mg/dl. Brain MRI showed no evidence of cerebellar atrophy, and SPECT-eZIS showed no decrease in cerebellar blood flow. However, voxel based morphometry (VBM) and FineSRT revealed cortical cerebellar atrophy and reduced cerebellar blood flow. In addition, the patient tested positive for anti-gliadin (IgA) and anti-SS-A/Ro antibodies, and was thus diagnosed as having autoimmune cerebellar ataxia. The patient showed positive response to intravenous immunoglobulins (IVIg) and regained the ability to walk unassisted. The HDS-R score also improved to 27/30. If cortical cerebellar atrophy can be diagnosed in the early stages in patients with progressive cerebellar ataxia by imaging techniques such as MRI-VBM and FineSRT, and if such patients test positive for anti-gliadin, anti-GAD or anti-thyroid antibodies, it is possible that they have autoimmune cerebellar ataxia. The commencement of immunotherapy including IVIg should be considered in such
Asunto(s)
Autoanticuerpos/análisis , Enfermedades Autoinmunes/diagnóstico , Enfermedades Autoinmunes/tratamiento farmacológico , Ataxia Cerebelosa/diagnóstico , Ataxia Cerebelosa/tratamiento farmacológico , Gliadina/inmunología , Inmunoglobulinas Intravenosas/uso terapéutico , Enfermedades Autoinmunes/inmunología , Ataxia Cerebelosa/inmunología , Humanos , Masculino , Persona de Mediana Edad , Tamaño de los Órganos , Programas InformáticosRESUMEN
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.