Publications by authors named "Takeshi Taguchi"

25 Publications

  • Page 1 of 1

Persistent Hemichorea as a Preceding Symptom of Cerebral Infarction due to Middle Cerebral Artery Stenosis.

Intern Med 2021 Jun 12. Epub 2021 Jun 12.

Department of Neurology, Tokyo Medical University, Japan.

We herein report an 84-year-old woman with right middle cerebral artery (MCA) stenosis who presented with persistent left hemichorea preceding cerebral infarction. She visited our hospital on day 9 after the hemichorea onset. Magnetic resonance imaging (MRI) showed no acute cerebral infarction. Magnetic resonance angiography revealed right MCA stenosis. Her hemichorea persisted for 19 days and subsequently disappeared. On day 21, she developed left hemiplegia. Repeat MRI revealed a cerebral infarction in the right putamen. MCA stenosis can present with persistent hemichorea, even in the absence of cerebral infarction. Persistent hemichorea with MCA stenosis may presage cerebral infarction.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.2169/internalmedicine.7191-21DOI Listing
June 2021

Relationship between I-FP-CIT-SPECT and motor severity in drug-naive patients with Parkinson's disease.

J Neurol Sci 2021 Jul 6;426:117476. Epub 2021 May 6.

Department of Neurology, Tokyo Medical University, 6-7-1 Nishishinjuku, Shinjuku-ku, Tokyo 160-0023, Japan.

Introduction: Although functional imaging is useful for the diagnosis and pathophysiological evaluation of Parkinson's disease (PD), little is known about the relationship between functional imaging findings and PD clinical features. The objective of this study was to determine the relationship between I-FP-CIT-SPECT findings and motor symptoms, in particular gait disturbance.

Methods: The study included 46 drug-naive patients with early-stage PD. The specific binding ratios (SBRs) in the striatum and its subregions, namely anterior/posterior putamen and caudate nucleus, were calculated in patients who underwent I-FP-CIT-SPECT. Motor symptoms were evaluated using the modified Hoehn and Yahr (HY) stage and the Unified Parkinson's Disease Rating Scale (UPDRS) part III. Gait disturbance was evaluated by the mean gait cycle duration and the mean gait acceleration amplitude measured with a wearable sensor.

Results: The mean SBRs of the striatum and anterior putamen were significantly associated with the modified HY stage and UPDRS part III score. The mean SBR of the caudate nucleus was significantly associated with the UPDRS part III score. The mean striatal SBR was also significantly associated with the mean gait cycle duration and mean gait acceleration amplitude.

Conclusion: The mean striatal SBR, as determined by I-FP-CIT-SPECT, was significantly associated with motor severity and gait severity in drug-naive patients with PD.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jns.2021.117476DOI Listing
July 2021

Cerebral Infarction and Myalgia in a 75-year-old Man with Eosinophilic Granulomatosis with Polyangiitis.

Intern Med 2020 Dec 4;59(23):3089-3092. Epub 2020 Aug 4.

Department of Neurology, Tokyo Medical University, Japan.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare condition of systemic vasculitis of small to medium-sized blood vessels. We herein report the case of a 75-year-old man who presented with hemiplegia on his right side due to cerebral infarction following myalgia and a high fever. He had no history of asthma or allergic rhinitis. He was diagnosed with EGPA based on the presence of eosinophilia, sinusitis suggested by magnetic resonance imaging, and muscle pathology. His hemiplegia improved rapidly after corticosteroid therapy. This case suggests that EGPA should be a differential diagnosis of cerebral infarction with myalgia and eosinophilia.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.2169/internalmedicine.5099-20DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7759697PMC
December 2020

Analysis of non-invasive gait recording under free-living conditions in patients with Parkinson's disease: relationship with global cognitive function and motor abnormalities.

BMC Neurol 2020 Apr 29;20(1):161. Epub 2020 Apr 29.

Department of Neurology, Tokyo Medical University, 6-7-1 Nishishinjuku, Shinjuku-ku, Tokyo, 160-0023, Japan.

Background: We investigated the gait characteristics of patients with Parkinson's disease (PD), under free-living conditions, using a wearable device, and assessed their relationships with global cognitive function and motor abnormalities.

Methods: The study subjects comprised patients with PD aged < 80 years, with a Mini-Mental State Examination (MMSE) score of ≥20, free of any motor complications. A wearable sensor with a built-in tri-axial accelerometer was waist-mounted on each patient, and continuous, 24-h records were obtained. The mean gait cycle duration and mean gait acceleration amplitude, under free-living conditions, were computed and analyzed to determine their relationship with disease duration, MMSE score, Unified Parkinson's Disease Rating Scale (UPDRS) Part III score, and postural instability and gait difficulty (PIGD) score.

Results: The study included 106 consecutive patients with PD. The mean gait cycle duration was 1.18 ± 0.12 s, which was similar to that of the normal controls. However, the mean gait acceleration amplitude of PD patients (1.83 ± 0.36 m/s) was significantly lower than that of the control (p < 0.001). In PD patients, the mean gait acceleration amplitude correlated with the MMSE (β = 0.197, p = 0.028), UPDRS Part III (β = - 0.327, p < 0.001), and PIGD (β = - 0.235, p = 0.008) scores.

Conclusions: The gait rhythm of PD patients is preserved at levels similar to those of normal subjects. However, the mean gait acceleration amplitude was significantly reduced in patients with PD. The results indicate that gait acceleration amplitude correlates with the severity of motor disorders and global cognitive function.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1186/s12883-020-01729-wDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7189597PMC
April 2020

Assessment and Rating of Motor Cerebellar Ataxias With the Kinect v2 Depth Sensor: Extending Our Appraisal.

Front Neurol 2020 11;11:179. Epub 2020 Mar 11.

Movement Disorders Project, Tokyo Metropolitan Institute of Medical Science, Tokyo, Japan.

Current assessment of patients with cerebellar disorders is based on conventional neurological examination that is dependent on subjective judgements. Quantitative measurement of cerebellar ataxias (CAs) is essential for assessment of evidence-based treatments and the monitoring of the progress or recovery of diseases. It may provide us a useful tool to navigate future treatments for ataxia. We developed a Kinect v2. sensor system with a novel algorithm to measure and evaluate movements for two tests of Scale for the Assessment and Rating of Ataxia (SARA): the nose-finger test and gait. For the nose-finger test, we evaluated and compared accuracy, regularities and smoothness in the movements of the index finger and the proximal limbs between cerebellar patients and control subjects. For the task of walking, we evaluated and compared stability between the two groups. The precision of the system for evaluation of movements was smaller than 2 mm. For the nose-finger test, the mildly affected patients tended to show more instability than the control subjects. For a severely affected patient, our system quantified the instability of movements of the index finger using kinematic parameters, such as fluctuations and average speed. The average speed appears to be the most sensitive parameter that contrasts between patients with CAs and control subjects. Furthermore, our system also detected the adventitious movements of more proximal body parts, such as the elbow, shoulder and head. Assessment of walking was possible only in patients with mild CAs. They demonstrated large sways and compensatory wide stances. These parameters appeared to show higher accuracy than SARA. This examiner-independent device measures movements of the points of interest of SARA more accurately than eye and further provides additional information about the ataxic movements (e.g., the adventitious movements of the elbow, shoulder and head in the nose-finger test and the wide-based walking with large oscillation in the gait task), which is out of the scope of SARA. Our new system enables more accurate scoring of SARA and further provides additional information that is not currently evaluated with SARA. Therefore, it provides an easier, more accurate and more systematic description of CAs.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3389/fneur.2020.00179DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7078683PMC
March 2020

Association of daily physical activity with cognition and mood disorders in treatment-naive patients with early-stage Parkinson's disease.

J Neural Transm (Vienna) 2019 12 30;126(12):1617-1624. Epub 2019 Sep 30.

Department of Neurology, Tokyo Medical University, 6-7-1 Nishishinjuku, Shinjuku-ku, Tokyo, 160-0023, Japan.

To determine the association of daily physical activity with cognition, mood disorders, and olfactory function in treatment-naive patients with early-stage Parkinson's disease (PD). The study subjects were 52 treatment-naive patients with early-stage PD (< 80 years). Daily physical activity was measured using a wearable sensor with a built-in triaxial accelerometer, and its association with cognition [mini-mental state examination (MMSE), clock-drawing test (CDT), frontal assessment battery (FAB), and behavioral assessment of the dysexecutive syndrome (BADS)], depressive symptoms [Beck Depression Inventory-Second Edition (BDI-II)], apathy [Starkstein Apathy Scale (AS)], and olfactory function [Odor Stick Identification Test for the Japanese (OSIT-J)] was analyzed using multiple linear regression after adjustment for age, sex, and education status. The daily physical activity (0.42 ± 0.11 m/s) of the PD group was significantly lower than that of healthy controls (p < 0.001). Moreover, the daily physical activity of the PD group was significantly associated with FAB (β = 0.337, p = 0.027) and BADS (β = 0.374, p = 0.017) scores, but not with MMSE, CDT, BDI-II, AS, and OSIT-J scores. The daily physical activity is significantly reduced in treatment-naive patients with early-stage PD, and the low activity correlates with frontal/executive function.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00702-019-02085-xDOI Listing
December 2019

[A case of meningeal carcinomatosis mimicking subarachnoid hemorrhage on MRI].

Rinsho Shinkeigaku 2018 Jun 1;58(6):403-406. Epub 2018 Jun 1.

Department of Neurology, Tokyo Medical University.

We report a case of meningeal carcinomatosis that needed to be distinguished from subarachnoid hemorrhage. A 67-year-old female with acute severe headache was admitted to a previous hospital. Since high intensity signal was detected within the parietal cerebral sulci on the right side on brain FLAIR MRI, cerebral angiography was performed due to suspicion of subarachnoid hemorrhage. However, no vascular abnormality was observed. Then, cerebral spinal fluid was collected, which showed an increase in cell count, suggesting meningitis. She was transferred to our hospital for evaluation of neurological disease. After admission to our hospital, there was an episode of hematemesis. Upper gastrointestinal endoscopy was performed, and advanced gastric cancer was found. She was diagnosed as having meningeal carcinomatosis due to gastric cancer. Meningeal carcinomatosis should be considered in addition to subarachnoid hemorrhage when a patient with acute headache shows high intensity signal within the cerebral sulci on brain FLAIR MRI.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.5692/clinicalneurol.cn-001151DOI Listing
June 2018

Secondary parkinsonism.

Nihon Rinsho 2017 Jan;75(1):63-70

Although many disorders are included in secondary parkinsonism, the mechanisms underlying parkinsonism vary and have yet to be elucidated. Herein, we introduced a group of diseases included among the forms of secondary parkinsonism and provide overviews of clinically significant drug-induced parkinsonism (DIP), vascular parkinson- ism (VP), and idiopathic normal pressure hydrocephalus (iNPH) with a focus on pathophysiology and symptoms. Although DIP has the highest frequency among the forms of secondary parkinsonism, it is overlooked in many patients due to lack of knowledge about drugs by the prescribing physicians. Both VP and iNPH present with "lower body parkinsonism, " showing the characteristic gait disturbance. DIP and iNPH are treatable, highlighting the importance of early diagnosis and treatment intervention.
View Article and Find Full Text PDF

Download full-text PDF

Source
January 2017

Prevalence of Autoantibodies and the Efficacy of Immunotherapy for Autoimmune Cerebellar Ataxia.

Intern Med 2016 1;55(5):449-54. Epub 2016 Mar 1.

Department of Neurology, Tokyo Medical University Hachioji Medical Center, Japan.

Objective: Autoimmune cerebellar ataxias were recently reported to be treatable. However, the proportion of patients with cortical cerebellar atrophy of unknown etiology with autoimmune-associated cerebellar ataxia and the actual effectiveness of immunotherapy in these diseases remain unknown.

Methods: We measured the level of autoantibodies (including anti-gliadin antibody, anti-glutamic acid decarboxylase (GAD) antibody, and anti-thyroid antibody) in 58 Japanese patients with cerebellar ataxia, excluding those with multiple system atrophy, hereditary spinocerebellar ataxia, cancer, or those who were receiving phenytoin, and the efficacy of immunotherapy was assessed.

Results: Thirty-one of 58 (53%) patients were positive for anti-GAD antibody, anti-gliadin antibody, or anti-thyroid antibody. Seven of the 12 anti-gliadin antibody-positive patients, three of the four anti-GAD antibody-positive patients, and three of the six anti-thyroid antibody-positive patients responded well to immunotherapy, indicating that 59% of patients with ataxia-associated antibody-positive cerebellar ataxia undergoing immunotherapy responded well.

Conclusion: Some patients with cerebellar ataxia have autoimmune conditions and diagnosing autoimmune cerebellar ataxia is therefore an important component in the care of patients with this disease entity.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.2169/internalmedicine.55.5156DOI Listing
August 2016

[Autoimmune cerebellar ataxia].

Nihon Rinsho 2015 Sep;73 Suppl 7:632-7

View Article and Find Full Text PDF

Download full-text PDF

Source
September 2015

Storage of washed platelets in BRS-A platelet additive solutions based on two types of clinically available bicarbonated Ringer's solutions with different electrolyte concentrations.

Transfus Apher Sci 2015 Oct 12;53(2):233-7. Epub 2015 May 12.

Japanese Red Cross Tohoku Block Blood Center, Sendai, Miyagi, Japan.

Background: In Japan, no platelet (PLT) additive solutions (PASs) are officially approved for clinical use although blood centers often receive requests for washed PLTs to reduce adverse reactions. Recently, we developed a novel PAS called BRS-A based on clinically available bicarbonated Ringer's solution (BRS), Bicanate and acid-citrate-dextrose formula A (ACD-A), which has been shown to maintain the in vitro properties of PLTs in the condition of <5% residual plasma during 7-day storage. The aim of this study was to evaluate whether another clinically available BRS, Bicarbon with different electrolyte concentrations can be used as a PAS.

Study Design And Methods: Two types of BRS-As were prepared by adding 25 mL of ACD-A to 500 mL of Bicanate or Bicarbon BRSs. Bicanate-based BRS-A and Bicarbon-based BRS-A contain 0.9 or 0.5 mmol/L of magnesium chloride, 95.2 or 100.1 mmol/L of sodium chloride, 4.2 or 5.1 mmol/L of trisodium citrate, and 26.6 or 23.8 mmol/L of sodium bicarbonate, respectively; the other components were identical. Apheresis PLTs stored in these solutions with less than 5% plasma for 7-day storage were compared with regard to their in vitro properties.

Results: The pH levels of all units were above 7 throughout storage. The mean PLT volume, hypotonic shock response, glucose consumption, lactate production, swirling, and CD62P and CD42b expression were similar during 7-day storage. The bicarbonate levels in Bicarbon-based BRS-A were lower than those in Bicanate-based BRS-A.

Conclusion: Differences in concentrations of electrolytes such as magnesium, sodium, citrate, and bicarbonate salts in BRS-A do not affect the in vitro properties of PLTs during 7-day storage. These results indicate that the use of another type of BRS-A based on Bicarbon as a PAS is feasible. Thus, BRS-A can be used in hospitals that do not stock Bicanate but have Bicarbon.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.transci.2015.05.002DOI Listing
October 2015

Gluten ataxia in Japan.

Cerebellum 2014 Oct;13(5):623-7

Department of Neurology, Hachioji Medical Center, Tokyo Medical University, 1163 Tatemachi, Hachioji, Tokyo, 193-0998, Japan,

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.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s12311-014-0582-3DOI Listing
October 2014

[The utility of voxel-based morphometry in the diagnosis of spinocerebellar degeneration].

Brain Nerve 2014 Jun;66(6):699-704

Department of Neurology, Tokyo Medical University Hachioji Medical Center.

We evaluated atrophic sites in the brainstem and cerebellum in the patients with spinocerebellar degeneration by using voxel-based morphometry (VBM). Gray matter atrophy was found extensively in both the cerebellar hemispheres and vermis of subjects presenting the cerebellar variant of multiple system atrophy (MSA-C; n=9). In addition, remarkable white matter atrophy was observed in the middle cerebellar peduncle, brainstem, and cerebellar hemispheres. In contrast, gray matter atrophy was not apparent in the cerebellar hemispheres or vermis of subjects in the SCA3 group (n=6), whereas intense white matter atrophy was visible in the middle cerebellar peduncle, brainstem, and cerebellar hemispheres. White matter atrophy was also observed in the brainstem and surrounding the dentate nucleus in both cases of dentatorubral-pallidoluysian atrophy (DRPLA) (n=2), whereas gray matter atrophy of the cerebellum was not remarkable. In both the SCA6 group (n=3) and the SCA31 group (n=2), gray matter atrophy was prominent in the cerebellar hemispheres and vermis; however, white matter atrophy was not found in the middle cerebellar peduncle and brainstem, whereas symmetric atrophy of white matter was found in the vicinity of the dentate nucleus. In each of these diseases, VBM findings were consistent with the pathological findings; therefore, VBM can be considered a useful tool for the diagnosis of spinocerebellar degeneration.
View Article and Find Full Text PDF

Download full-text PDF

Source
June 2014

Immunoadsorption therapy for neuromyelitis optica spectrum disorders long after the acute phase.

J Clin Apher 2015 Feb 6;30(1):43-5. Epub 2014 May 6.

Department of Cardiology, Tokyo Medical University, Hachioji Medical Center, Tokyo, Japan.

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.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1002/jca.21324DOI Listing
February 2015

Low-titer anti-GAD-antibody-positive cerebellar ataxia.

Cerebellum 2013 Apr;12(2):171-5

Department of Neurology, Tokyo Medical University Hachioji Medical Center, 1163 Tatemachi, Hachioji, Tokyo, 193-0998, Japan.

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.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s12311-012-0411-5DOI Listing
April 2013

Selective loss of Purkinje cells in a patient with anti-gliadin-antibody-positive autoimmune cerebellar ataxia.

Diagn Pathol 2011 Feb 4;6:14. Epub 2011 Feb 4.

Department of Neurology, Tokyo Medical University Hachioji Medical Center 1163 Tatemachi, Hachioji, Tokyo, 193-0998, Japan.

The patient was an 84-year-old woman who had the onset of truncal ataxia at age 77 and a history of Basedow's disease. Her ataxic gait gradually deteriorated. She could not walk without support at age 81 and she was admitted to our hospital at age 83. Gaze-evoked nystagmus and dysarthria were observed. Mild ataxia was observed in all limbs. Her deep tendon reflex and sense of position were normal. IgA anti-gliadin antibody, IgG anti-gliadin antibody, anti-SS-A/Ro antibody, anti-SS-B/La antibody and anti-TPO antibody were positive. A conventional brain MRI did not show obvious cerebellar atrophy. However, MRI voxel based morphometry (VBM) and SPECT-eZIS revealed cortical cerebellar atrophy and reduced cerebellar blood flow. IVIg treatment was performed and was moderately effective. After her death at age 85, the patient was autopsied. Neuropathological findings were as follows: selective loss of Purkinje cells; no apparent degenerative change in the efferent pathways, such as the dentate nuclei or vestibular nuclei; no prominent inflammatory reaction. From these findings, we diagnosed this case as autoimmune cerebellar atrophy associated with gluten ataxia. All 3 autopsies previously reported on gluten ataxia have noted infiltration of inflammatory cells in the cerebellum.In this case, we postulated that the infiltration of inflammatory cells was not found because the patient's condition was based on humoral immunity. The clinical conditions of gluten ataxia have not yet been properly elucidated, but are expected to be revealed as the number of autopsied cases increases.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1186/1746-1596-6-14DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3042899PMC
February 2011

[A case of autoimmune polyglandular syndrome-related Parkinsonian syndrome that required differentiation from multiple system atrophy].

Rinsho Shinkeigaku 2010 Oct;50(10):704-9

Tokyo Medical University Hachioji Medical Center.

A 76-year-old woman experienced unsteadiness in walking in 1996. On the basis of clinical and imaging findings, the patient was diagnosed multiple system atrophy. During follow-up, her gait disturbance became aggravated leaving her unable to walk unaided. She was referred to our department in 2003. T2-weighted images on brain magnetic resonance imaging (MRI) revealed low signal intensity in both putamina and a linear high-signal-intensity area on their outsides. Single photon emission computed tomography (SPECT) disclosed a reduced blood flow in both corpora striata. These findings were consistent with the diagnosis of Parkinsonian-type multiple system atrophy. The patient had anti-glutamic acid decarboxylase (GAD) antibody-positive type 1 diabetes mellitus and a normal thyroid function, and was positive for antithyroid antibodies. She was not found to have anemia on blood tests, but was positive for intrinsic factor antibodies. Vitamin B12 was markedly reduced to below the detection limit. The findings suggested that the patient's condition was autoimmune polyglandular syndrome type 3. In 2004, treatment with intramuscular injection of vitamin B12 was initiated, after which the patient's gait disturbance was improved and she was able to walk unaided. In 2009, her unsteady gait returned and was again unable to walk unaided. Autoimmune encephalopathy was suspected, and thus high-dose intravenous immunoglobulin therapy was performed. Following treatment she was able to walk steadily. This case suggests the importance of detailed tests for autoantibodies, including endocrine autoantibodies, and the measurement of vitamin B12 and total homocysteine levels in view of the possibility of autoimmune polyglandular syndrome-related neurological disorders in diabetic patients with intractable neurological disorders that are difficult to diagnose.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.5692/clinicalneurol.50.704DOI Listing
October 2010

[A case of lung adenocarcinoma presenting with chorea with bilateral basal ganglial lesions on MRI].

Rinsho Shinkeigaku 2010 Aug;50(8):556-60

Department of Neurology, Tokyo Medical University, Hachioji Medical Center.

The patient, a 63-year-old man, experienced the subacute onset of chorea, for which his family doctor prescribed oral haloperidol. However, the involuntary movements gradually worsened, and the patient was referred and admitted. High-signal lesions were seen in the caudate nucleus, putamen and globus pallidus bilaterally on MRI T2-weighted and FLAIR images. Chest CT, FDG-PET and tissue biopsies also revealed that the patient had lung adenocarcinoma with multiple lymph node metastases. The patient was diagnosed as having paraneoplastic chorea associated with primary lung adenocarcinoma. Antineuronal antibodies, such as anti-CRMP-5 and anti-Yo antibodies, were absent. The patient received steroid pulse therapy, oral prednisolone therapy, and concurrent radiochemotherapy. Chorea and high-signal lesions in the corpus striatum bilaterally on MRI improved quickly, and the mediastinal lymph node swelling also improved. The patient has been stable for 3 years since the onset of his symptoms. As the prognosis of paraneoplastic chorea is relatively favorable in some patients, it should be considered in the differential diagnosis of patients with chorea.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.5692/clinicalneurol.50.556DOI Listing
August 2010

Classification of cerebellar atrophy using voxel-based morphometry and SPECT with an easy Z-score imaging system.

Intern Med 2010 15;49(6):535-41. Epub 2010 Mar 15.

Department of Neurology, Tokyo Medical University Hachioji Medical Center.

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.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.2169/internalmedicine.49.2785DOI Listing
June 2010

[Case of Wernicke's encephalopathy and subacute combined degeneration of the spinal cord due to vitamin deficiency showing changes in the bilateral corpus striatum and cardiac arrest due to beriberi heart disease].

Brain Nerve 2009 Sep;61(9):1069-73

Department of Neurology, Tokyo Medical University Hachioji Medical Center, 1163 Tate-machi, Hachioji-shi, Tokyo 193-0998, Japan.

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.
View Article and Find Full Text PDF

Download full-text PDF

Source
September 2009

Intravenous immunoglobulin therapy for autoantibody-positive cerebellar ataxia.

Intern Med 2009 15;48(10):783-90. Epub 2009 May 15.

Department of Neurology, Tokyo Medical University Hachioji Medical Center, Tokyo.

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.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.2169/internalmedicine.48.1802DOI Listing
August 2009

[A case of anti-gliadin-antibody-positive cerebellar ataxia effectively treated with intravenous immunoglobulin in which voxel-based morphometry and FineSRT were diagnostically useful].

Rinsho Shinkeigaku 2009 Jan;49(1):37-42

Department of Neurology, Tokyo Medical University Hachioji Medical Center.

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
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.5692/clinicalneurol.49.37DOI Listing
January 2009

Transient hemiballism caused by a small lesion of the subthalamic nucleus.

J Clin Neurosci 2008 Dec 7;15(12):1416-8. Epub 2008 Oct 7.

Department of Neurosurgery, Hachioji Medical Center, Tokyo Medical University, Tokyo, Japan.

Hemiballism is most commonly caused by ischemic stroke and most cases have a favorable prognosis. Lesions directly involving the subthalamic nucleus (STN) are the cause of a minority of cases but are usually associated with poor prognosis. We report two patients with a small STN lesion who presented with transient hemiballism. A small lesion confined to and only focally affecting the STN may cause hemiballism yet may have excellent outcome. Precise evaluation of the affected region with MRI is useful in predicting the prognosis.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jocn.2007.05.024DOI Listing
December 2008

[Efficacy of intravenous immunoglobulin for slowly progressive cerebellar atrophy].

Rinsho Shinkeigaku 2006 Jul;46(7):467-74

Department of Neurology, Tokyo Medical University Hachioji Medical Center.

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.
View Article and Find Full Text PDF

Download full-text PDF

Source
July 2006

Huge seminoma developed in a patient with testicular feminization.

J Obstet Gynaecol Res 2004 Apr;30(2):109-12

Department of Obstetrics and Gynecology, Juntendo University, Juntendo Urayasu Hospital, Chiba, Japan.

We report a 36-year-old patient who presented with primary amenorrhea and was found to have a giant abdominal tumor. There was a family history of primary amenorrhea in her two aunts. Physical and hormonal examinations as well as chromosomal analysis led to a diagnosis of testicular feminization. The patient underwent tumorectomy along with pelvic and para-aortic lymphadectomy. The pathological diagnosis was bilateral seminoma of the testis with metastasis to para-aortic lymph nodes. Nowadays, cases of undiagnosed seminoma developing into a huge abdominal mass in patients with testicular feminization are rarely encountered, since surgical castration is generally recommended as early as possible after puberty. In testicular feminization, the risk of malignant transformation of the dysgenetic male gonads increases substantially after puberty. Early and correct diagnosis together with careful follow-up are critically important in managing testicular feminization, a rare congenital disorder.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/j.1447-0756.2003.00168.xDOI Listing
April 2004