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Miho Ota,
Noriko Sato,
Yasuhiro Nakata,
Kimiteru Ito,
Kouhei Kamiya,
Norihide Maikusa, Masafumi Ogawa,
Tomoko Okamoto,
Satoko Obu,
Takamasa Noda,
Manabu Araki,
Takashi Yamamura,
Hiroshi Kunugi
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ABSTRACT: Arterial spin labeling (ASL) is a noninvasive technique that can measure cerebral blood flow (CBF). To our knowledge, there is no study that examined regional CBF of multiple sclerosis (MS) patients by using this technique. The present study assessed the relationship between clinical presentations and functional imaging data in MS using pseudocontinuous arterial spin labeling (pCASL). Twenty-seven patients with MS and 24 healthy volunteers underwent magnetic resonance imaging and pCASL to assess CBF. Differences in CBF between the two groups and the relationships of CBF values with the T2-hyperintense volume were evaluated. Compared to the healthy volunteers, reduced CBF was found in the bilateral thalami and right frontal region of the MS patients. The volume of the T2-hyperintense lesion was negatively correlated with regional CBF in some areas, such as both thalami. Our results suggest that demyelinated lesions in MS mainly have a remote effect on the thalamus and that the measurement of CBF using ASL could be an objective marker for monitoring disease activity in MS.
Magnetic Resonance Imaging 04/2013; · 1.99 Impact Factor
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ABSTRACT: Clinical differentiation of parkinsonian syndromes such as the Parkinson variant of multiple system atrophy (MSA-P) and cerebellar subtype (MSA-C) from Parkinson's disease is difficult in the early stage of the disease. To identify the correlative pattern of brain changes for differentiating parkinsonian syndromes, we applied discriminant analysis techniques by magnetic resonance imaging (MRI). T1-weighted volume data and diffusion tensor images were obtained by MRI in eighteen patients with MSA-C, 12 patients with MSA-P, 21 patients with Parkinson's disease, and 21 healthy controls. They were evaluated using voxel-based morphometry and tract-based spatial statistics, respectively. Discriminant functions derived by step wise methods resulted in correct classification rates of 0.89. When differentiating these diseases with the use of three independent variables together, the correct classification rate was the same as that obtained with step wise methods. These findings support the view that each parkinsonian syndrome has structural deviations in multiple brain areas and that a combination of structural brain measures can help to distinguish parkinsonian syndromes.
Computational and Mathematical Methods in Medicine 01/2013; 2013:571289. · 0.68 Impact Factor
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Wakiro Sato,
Atsuko Tomita,
Daijyu Ichikawa,
Youwei Lin,
Hitaru Kishida,
Sachiko Miyake, Masafumi Ogawa,
Tomoko Okamoto,
Miho Murata,
Yoshiyuki Kuroiwa,
Toshimasa Aranami,
Takashi Yamamura
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ABSTRACT: Multiple sclerosis (MS) is a demyelinating disease of the CNS that is presumably mediated by CD4(+) autoimmune T cells. Although both Th1 and Th17 cells have the potential to cause inflammatory CNS pathology in rodents, the identity of pathogenic T cells remains unclear in human MS. Given that each Th cell subset preferentially expresses specific chemokine receptors, we were interested to know whether T cells defined by a particular chemokine receptor profile play an active role in the pathogenesis of MS. In this article, we report that CCR2(+)CCR5(+) T cells constitute a unique population selectively enriched in the cerebrospinal fluid of MS patients during relapse but not in patients with other neurologic diseases. After polyclonal stimulation, the CCR2(+)CCR5(+) T cells exhibited a distinct ability to produce matrix metalloproteinase-9 and osteopontin, which are involved in the CNS pathology of MS. Furthermore, after TCR stimulation, the CCR2(+)CCR5(+) T cells showed a higher invasive potential across an in vitro blood-brain barrier model compared with other T cells. Of note, the CCR2(+)CCR5(+) T cells from MS patients in relapse are reactive to myelin basic protein, as assessed by production of IFN-γ. We also demonstrated that the CCR6(-), but not the CCR6(+), population within CCR2(+)CCR5(+) T cells was highly enriched in the cerebrospinal fluid during MS relapse (p < 0.0005) and expressed higher levels of IFN-γ and matrix metalloproteinase-9. Taken together, we propose that autoimmune CCR2(+)CCR5(+)CCR6(-) Th1 cells play a crucial role in the pathogenesis of MS.
The Journal of Immunology 10/2012; · 5.79 Impact Factor
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ABSTRACT: A 60-year-old woman developed type 1 diabetes mellitus and anti-aquaporin-4 antibody positive optic neuritis during type 1 interferon therapies for chronic hepatitis C. The diabetes mellitus was elicited by interferon-α plus ribavirin therapy, while the optic neuritis was induced after interferon-β treatment, followed by interferon-α and ribavirin therapy. It is possible that type 1 interferons lead to the onset of the two autoimmune diseases by inducing disease-specific autoantibodies. Autoimmune disease is an infrequent complication of type 1 interferon treatment; however, once it has occurred, it may result in severe impairments. Patients undergoing type 1 interferon therapy should therefore be carefully monitored for any manifestations of autoimmune diseases.
Internal Medicine 01/2012; 51(18):2625-9. · 0.94 Impact Factor
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ABSTRACT: Interferon-β (IFN-β) is known to expand regulatory CD56(bright) natural killer (NK) cells in multiple sclerosis (MS). In this cross-sectional study we show that MS patients treated with IFN-β alone or in combination with low-dose prednisolone displayed increased proportion of all NK cell subsets in the active phase of the cell cycle (Ki-67+). There was no difference in NK cell apoptosis markers. In vitro experiments showed that both IFN-β and IFN-β in combination with corticosteroids increased the proportion of Ki-67(+) NK cells. This study, although limited, shows that treatment with IFN-β affects NK cell cycle without altering NK cell apoptosis in MS patients.
Journal of neuroimmunology 06/2011; 236(1-2):111-7. · 2.84 Impact Factor
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ABSTRACT: Neuromyelitis optica (NMO) is an inflammatory disease affecting the optic nerve and spinal cord, in which autoantibodies against aquaporin 4 (AQP4) water channel protein probably play a pathogenic role. Here we show that a B-cell subpopulation, exhibiting the CD19(int)CD27(high)CD38(high)CD180(-) phenotype, is selectively increased in the peripheral blood of NMO patients and that anti-AQP4 antibodies (AQP4-Abs) are mainly produced by these cells in the blood of these patients. These B cells showed the morphological as well as the phenotypical characteristics of plasmablasts (PB) and were further expanded during NMO relapse. We also demonstrate that interleukin 6 (IL-6), shown to be increased in NMO, enhanced the survival of PB as well as their AQP4-Ab secretion, whereas the blockade of IL-6 receptor (IL-6R) signaling by anti-IL-6R antibody reduced the survival of PB in vitro. These results indicate that the IL-6-dependent B-cell subpopulation is involved in the pathogenesis of NMO, thereby providing a therapeutic strategy for targeting IL-6R signaling.
Proceedings of the National Academy of Sciences 02/2011; 108(9):3701-6. · 9.68 Impact Factor
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ABSTRACT: An 82 year-old woman with Parkinson's disease complained of a tendency to fall. She has had an extensive kyphosis since she was 66 years old. Over the last 6 months, she has repeatedly fallen. Even though she took anti-parkisonian drugs, she had also developed camptocormia. Her plasma levodopa concentration was analyzed for 4 hrs after administrating an oral dose of levodopa (200 mg) plus carbidopa (20 mg) at the time of fasting. The change in the plasma levodopa concentration showed bimodal peaks. The physical symptoms depended on the plasma concentration and improved twice. Esophageal tortuosity and esophageal hiatal hernia were detected by esophagography and upper gastric endoscopy. Such physical symptoms were speculated to have been caused by the transit disturbance of the drug in the gastrointestinal duct. During a second analysis of the plasma levodopa concentration, the patient was instructed to keep extending her back after consuming the same dose of drugs but with a greater amount of water than in the first analysis. A single and a higher peak were observed for the plasma levodopa concentration, and the physical symptoms, including camptocormia and parkinsonism, were improved. Hunched posture could influence the absorption of antiparkinsonian drugs.
Rinsho shinkeigaku = Clinical neurology 08/2009; 49(8):493-6.
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ABSTRACT: We investigated the relationship between nasal flaring and SpO2 in 19 patients with Duchenne muscular dystrophy (DMD) and 26 patients with myotonic dystrophy (DM1). In DMD patients, nasal flaring was observed when SpO2 was lower than 96%, while it was not seen even at 82% of SpO2 in DM1. None of the DM1 patients could perform voluntary nasal flaring. Nasal flaring is a useful indicator of hypoxemia in DMD but not in DM1. It remains to be elucidated whether the lack of nasal flaring in DM1 patients is due to abnormal respiratory central mechanism or nasal muscle weakness.
Rinsho shinkeigaku = Clinical neurology 06/2009; 49(5):278-80.
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Satoshi Kamei,
Shigeki Kuzuhara,
Masaki Ishihara,
Akihiko Morita,
Naoto Taira,
Masaki Togo,
Makoto Matsui, Masafumi Ogawa,
Kinya Hisanaga,
Tomohiko Mizutani,
Sadako Kuno
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ABSTRACT: To study the incidence and clinical features of acute juvenile female non-herpetic encephalitis (AJFNHE) in Japan.
A nationwide questionnaire on patients with severe non-herpetic encephalitis of unknown etiology with a prolonged clinical course or death was sent to the departments of Internal Medicine, Neurology, Pediatrics, and Emergency and Critical Care at all hospitals with 200 beds or more in Japan.
The recovery rate was 25% (1,279 out of 5,030 departments) and 90 patients were enrolled in this study. The annual incidence was 0.33/10(6) population. 85% of patients were female. The means and standard deviations of age at onset and hospital stay were 26+/-10 years and 180+/-228 days. As first symptoms, fever and psychosis were presented in 90%. Among the neurological symptoms, disturbance of consciousness was presented in 92%, convulsions in 65%, and involuntary movements in 55%. Respiratory failure during hospitalization was observed in 71% and required care with mechanical ventilation. The detection rate of anti-GluR epsilon2 and/or delta1 antibodies was 67% of patients. Anti-N-methyl-D-aspartate receptor NR1/NR2 antibody was detected in all four examined patients with anti-GluR epsilon2 antibody, and also detected in both of the two examined patients without anti-GluR epsilon2 antibody. As for outcome, 46% returned to work and 37% returned home, but 7% died. Associated tumors were demonstrated in 39%. All reported patients had ovarian tumors, among which teratoma was the most frequent.
A nationwide survey provided data for the annual incidence and clinical features of AJFNHE in Japan.
Internal Medicine 02/2009; 48(9):673-9. · 0.94 Impact Factor
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ABSTRACT: Dementia with Lewy bodies (DLB) is the second most common form of dementia. It is thought to involve impairment of the visual association area. In this study, we applied diffusion tensor imaging (DTI) to examine the microstructural interruption of visual association areas in patients with DLB. The DTI metrics of three visual association fibres - the inferior longitudinal fasciculus (ILF), visual pathway, and splenial fibres - were compared between 14 patients with DLB and 13 healthy subjects. The fractional anisotropy value of the ILF was significantly lower in patients with DLB than in healthy subjects. The difference in the mean diffusivity value of ILF was at trend level. The lambda(2),(3) of ILF were significantly lower in patients with DLB; however, there was no difference in lambda(1). DTI metrics of the visual pathway and splenial fibres showed no differences between the groups. Our results showed degeneration of the ILF, which is responsible for visuospatial cognition. ILF dysfunction may influence the clinical features in DLB.
NMR in Biomedicine 12/2008; 22(3):280-4. · 3.21 Impact Factor
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ABSTRACT: Plaque-type deposition of prion protein (PrP) in the brain has been extremely rare in sporadic Creutzfeldt-Jakob disease patients with methionine homozygosity at polymorphic codon 129 of the PrP gene and type 1 abnormal isoform of PrP (sCJD-MM1). Here we report three sCJD-MM1 patients who showed prominent PrP-positive amyloid plaques in the cerebral and cerebellar white matter. All three patients showed clinical courses of long duration (2 years < or =), particularly at the end-stage. The white matter of these patients was severely damaged because of the prolonged disease duration. Furthermore, Alzheimer's amyloid precursor protein, which accumulates within the axonal swellings under pathological conditions, co-accumulated with the PrP-amyloid plaques. These findings suggest that the axonal damage reflecting the prolonged disease duration causes the deposition of PrP-amyloid plaques in the white matter. The present study shows that PrP-amyloid plaques can occur in the white matter of sCJD-MM1 cases.
Acta Neuropathologica 09/2008; 116(5):561-6. · 9.32 Impact Factor
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ABSTRACT: Neuromyelitis optica (NMO) is an inflammatory demyelinating disease that largely affects optic nerves and spinal cord. Recent studies have identified an elevation of serum anti-aquaporin 4 antibody as a hallmark of NMO. Typical cases of NMO significantly differ from multiple sclerosis (MS) in immunological markers, histopathology, and responses to therapy. In fact, plasma exchange may be more efficacious for NMO than MS, whereas interferon-ß is recommended for MS but not for NMO. An emerging idea that pathogenesis of NMO may involve an interaction of the newly identified helper T cell subset, Th17, with B cells offers potential targets of therapy.
Therapeutic Advances in Neurological Disorders 07/2008; 1(1):5-12.
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ABSTRACT: A 26-year-old woman noticed gradually progressive, right lower leg weakness over a 1.5-month period. Neurological examination revealed right hemiparesis with slightly increased deep tendon reflexes, Babinski's sign on the right side, loss of position sense in the right leg, and slight loss of superficial sensation in the right toes. MR FLAIR images showed a high intensity area measuring 5 x 2 x 3 cm in the left frontal lobe, extending to the outer surface of the body of the corpus callosum and the adjacent right cingulate gyrus. Gadolinium enhancement was seen along the cortex and the outer surface of the body of the corpus callosum. CSF findings showed no pleocytosis, a protein content of 32 mg/dl, a sugar level of 85 mg/dl, and an IgG index of 0.46. The biopsy specimen obtained from the superior frontal gyrus showed perivascular cuffing of T-lymphocytes and some B-lymphocytes, as well as multiple small foci of demyelination. Starting on the second day of admission, the patient was treated with methylprednisolone pulse therapy (1,000 mg/day for 3 days); she was then switched to oral prednisolone (20 mg/day). Thereafter, the patient had two clinical relapses: one was due to a lesion in the dorsal part of the medulla oblongata associated with a disturbance of deep sensation in both hands, and the other was due to a lesion involving the right internal capsule, the globus pallidus, and the caudate nucleus associated with left facial nerve palsy. Visual evoked potentials suggested a demyelinating lesion in the right optic nerve. We suspected a diagnosis of multiple sclerosis based on the presence of more than two clinical episodes of neurological deficits with identifiable lesions on MRI. Multiple sclerosis should be considered in the differential diagnosis of lesions located in the outer part of the corpus callosum and transcallosal bilateral hemispheres on MRI, even though inner callosal lesions are common in multiple sclerosis.
Rinsho shinkeigaku = Clinical neurology 06/2008; 48(5):321-7.
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ABSTRACT: A 64-year-old woman with myasthenia gravis (MG) presented with isolated bulbar symptoms. Two years earlier, she had developed neck weakness, diplopia, and ptosis and was diagnosed with MG. Extensive thymectomy was performed, and she was treated with predonisolone (PSL). The neck weakness, diplopia, and ptosis improved over a 2-year period. However, dysphagia developed, and her voice took on a nasal tone that did not improve subjectively even after administration of 10 mg of edrophonium chloride (EC). We then performed videofluorography (VF). After consumption of 10 ml of liquid barium and 8 g of corned beef hash, she attempted to swallow, but the residue remained in the valleculae and the piriform fossa. After the EC injection, her dysphagia on ingestion of corned beef hash improved; however, there was slight subjective improvement in swallowing. Drinking of liquid barium resulted in some residue with slight improvement of dysphagia. After treatment with 70 mg of PSL for 4 weeks, VF showed improvement of dysphagia. Thus, VF, particularly during consumption of solid food, with EC administration is helpful in evaluating bulbar symptoms in patients with MG.
Rinsho shinkeigaku = Clinical neurology 11/2007; 47(10):669-71.
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ABSTRACT: We report beneficial effects of pioglitazone on insulin resistance in diabetes mellitus accompanied with myotonic dystrophy (DM1). We studied eight DM1 patients with diabetes mellitus aged 32 to 60 (mean age 52.1 +/- 8.6 years). Three of them were under glibenclamide treatment, but their plasma glucose control was poor because of occasional hypoglycemia; others had not been treated with any hypoglycemic drugs. We administered a daily dose of 15 mg pioglitazone for 6-36 months (mean period 14.8 +/- 9.1 months). Plasma glucose control improved in all patients. In a 75 g oral glucose tolerance test, plasma glucose level at 120 min dropped from 203.3 +/- 41.7 mg/dl to 153.9 +/- 39.5 mg/dl (p = 0.04); the area under the insulin curve up to 120 min (sigma IRI) dropped from 236.9 +/- 170.2 microU x hr/ml to 169.6 +/- 81.3 microU x hr/ml (p = 0.12). Sigma IRI decreased in four patients with pretreatment sigma IRI > or = 250 microU x hr/ml; it slightly increased in other patients with pretreatment sigma IRI < or = 150 microU x hr/ml. The homeostasis model assessment-insulin resistance (HOMA-IR) improved from 2.1 +/- 1.0 to 1.1 +/- 0.4 (p = 0.04). Impairment of liver functions, cardiac failure, or hypoglycemia was not observed. Pioglitazone treatment is useful to improve insulin resistance and glucose control in DM1 patients with diabetes mellitus, especially patients with reactive hyperinsulinemia to glucose loading.
Rinsho shinkeigaku = Clinical neurology 04/2005; 45(4):287-92.
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ABSTRACT: Patients with myotonic dystrophy (DM1) rarely complain of dyspnea despite of severe hypoxemia. We studied the perception of dyspnea caused by breath-holding in 9 DM1 patients and 8 healthy control subjects. The patients, as well as the control subjects, complained of dyspnea and showed decrease in SpO2. In none of the patients but one, however, the bottom SpO2 became lower than the minimal SpO2 recorded in 24-hour monitoring. DM1 patients were able to realize hypoxia caused by apnea, although they had not realized hypoxia that already existed. Consequently, the breath-holding test does not uncover a blunted perception of dyspnea in DM1; an afferent system contributable to air hunger sensation in breath-holding is preserved in DM1. Breath-holding test may be useful for a DM1 patient to recognize the significance of sleep apnea.
Rinsho shinkeigaku = Clinical neurology 03/2005; 45(2):117-20.
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ABSTRACT: We examined the relation of maximum phonation time (MPT) and vital capacity (VC) and reviewed the usefulness of MPT as a respiratory function screening.
18 healthy adult subjects (8 men and 10 women), and 32 myopathic patients (24 men and 8 women).
MPT and VC were measured in sitting position. Six patients were tested with and without air stacking by glossopharyngeal respiration.
In healthy subjects, MPT was 29.9 +/- 11.8 seconds in men and 21.7 +/- 7.8 seconds in women. Second trials showed good reproducibility. The healthy group had no correlation between MPT and VC. The patient group showed a significant positive correlation between MPT and VC (r2= 0.25, p=0.003). All patients with MPT less than 15 seconds showed VC less than 1.5 l and %VC less than 50%. Air-stacking by glossopharyngeal respiration significantly increased the MPT.
MPT is a useful screening test for respiratory muscle weakness. The patients are easily aware of the effect of air-stacking by glossopharyngeal respiration.
Nō to shinkei = Brain and nerve 11/2004; 56(10):873-6.
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ABSTRACT: The Multiple Sclerosis Quality of Life-54 instrument (MSQOL-54) is a specific quality of life (QOL) scale in English for multiple sclerosis (MS). It is composed of 54 items, and is a combination of the 36-item short form health survey (SF-36) and 18 disease-specific questions, such as fatigue, mental sexual and cognitive dysfunction. We developed the Japanese-translated version of MSQOL-54. The SF-36 has been previously validated and published in Japanese; therefore the translation work was performed mainly on the 18 MS specific items. The Japanese-translated version MSQOL-54 was examined in 62 Japanese patients with MS. The mean age of the patients was 42.8 years; mean expanded disability status scale (EDSS) score was 3.0. The ratio of respondents was almost complete for all scales except for those within the sexual scales. Internal consistency reliability estimates for the 11 multi-item scales ranged from 0.65 to 0.93 in 62 patients. Test-retest intraclass correlation coefficients ranged from 0.61 to 0.95 in 20 patients. Compared to the previous reported mean scores of general Japanese population of SF-36, the mean scores of patients with MS had lower scores in all scales. In comparison with an original article in English, the validation of the Japanese-translated version MSQOL-54 may be acceptable. There were no correlations between the results of the Japanese-translated version MSQOL-54 and EDSS except for physical function and physical health composite score. The Japanese-translated version of MSQOL-54 may provide unique information not readily evaluated by EDSS, and may be useful as clinical outcome measures in patients with MS.
Rinsho shinkeigaku = Clinical neurology 08/2004; 44(7):417-21.
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ABSTRACT: A 60-year-old right-handed man showed dysprosody and agnosia for environmental sounds. His mother tongue was Japanese, and he could not speak foreign languages. He gradually developed difficulty in speaking from the age of 57 years, speaking non-native Japanese. In addition, he often complained of difficulty in hearing sounds, but audiometry showed no abnormalities. At the age of 60 years, the standard language test of aphasia showed no abnormalities in repetition, verbal comprehension, or reading, suggesting the absence of aphasia. However, in speaking, marked abnormality in rhythm, and occasional lack of postpositional particles and syllable-stumblings were observed. Writing was almost accurate, but a few grammatical errors were observed in speaking were observed. There were no cerebellar symptoms, pyramidal signs, pathologic reflexes, or abnormalities in phonation-related organs. Though the recognition of verbal sounds was maintained, impairment in the recognition of non-verbal sounds was observed. An environmental sound perception test showed correct answers only in 8 of 21 non-verbal sound sources (such as a car starting, glass breaking and so on), suggesting agnosia for environmental sounds. He insisted that the difficulty in perception was due to hearing impairment. However, re-examination with an increase in the sound volume showed similar results. He had no inconvenience in daily life and was not aware of agnosia for environmental sounds. He could recognize and differentiate sounds he heard once. His intelligence was normal, and neither apraxia nor frontal lobe symptoms were observed. MRI of the brain revealed slight atrophy of the right temporal lobe. Cerebral blood flow SPECT showed decreased blood flow from the superior temporal gyrus to the area around the arcuate fasciculi in the right temporal lobe. We considered that the lesion responsible for environmental auditory sound agnosia was present in the area around the secondary auditory area of the right temporal lobe and this patient differed from slowly progressive aphasia characterized by decreased blood flow in the left temporal lobe. Although the pathological process occurring in the area of hypoperfusion remained unclear, early stage of some degenerative disorders was more likely than cerebrovascular disease.
Rinsho shinkeigaku = Clinical neurology 02/2004; 44(1):28-33.
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Masafumi Ogawa
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ABSTRACT: The confirmed pharmacological treatment of cerebellar ataxia is still lacking. In a recent preliminary trial, we showed that D-cycloserine, a partial NMDA allosteric agonist, may relieve the symptoms. In this paper, major clinical trials to relieve ataxic symptoms are reviewed. Previous studies showed some efficacy of physostigmine in ataxic patients. However, physostigmine did not improve the ataxia in a recent double-blind crossover study. The replacement therapy of the deficient cholinergic system with choline or choline derivatives was tried in patients with Friedreich's ataxia and other ataxic patients, but the result was not definitive. A levorotatory form of hydroxytryptophan (a serotonin precursor), a serotoninergic 5-HT1A agonist, a serotoninergic 5-HT3 antagonist, and a serotonin reuptake inhibitor were also used for the therapy for ataxia. In a double-blind randomized study, buspirone, a 5-HT1A agonist was active in cerebellar ataxia, but the effect is partial and not major. The effects of the studies with the other serotoninergic drugs were not consistent. The effect of sulfamethoxazole-trimethoprim therapy in spinocerebellar ataxia type3/Machado-Joseph disease (MJD) was reported, although the therapy improved spasticity or rigidity, rather than ataxia. In contrast to previous studies, sulfamethoxazole-trimethoprim therapy in MJD had no effect in a 2001 double-blind crossover study. The thyrotropin-releasing hormone, D-cycloserine, and acetazolamide for SCA6 may have some efficacy. However, a well-designed double-blind crossover trial is needed to confirm the effect.
The Cerebellum 02/2004; 3(2):107-11. · 3.21 Impact Factor
