Characteristics and outcomes of unknown onset stroke: The Japan Stroke Data Bank.

BACKGROUND: Clinical outcomes of unknown onset stroke (UOS) are influenced by the enlargement of the therapeutic time window for reperfusion therapy. This study aimed to investigate and describe the characteristics and clinical outcomes of patients with UOS. METHODS: Patients with acute ischemic stroke (AIS) who were admitted within 24 h of their last known well time, from January 2017 to December 2020, were included. Data were obtained from a long-lasting nationwide hospital-based multicenter prospective registry: the Japan Stroke Data Bank. The co-primary outcomes were the National Institutes of Stroke Scale (NIHSS) scores on admission and unfavorable outcomes at discharge, corresponding to modified Rankin Scale (mRS) scores of 3-6. RESULTS: Overall, 26,976 patients with AIS were investigated. Patients with UOS (N = 5783, 78 ± 12 years of age) were older than patients with known onset stroke (KOS) (N = 21,193, 75 ± 13 years of age). Age, female sex, higher premorbid mRS scores, atrial fibrillation, and congestive heart failure were associated with UOS in multivariate analysis. UOS was associated with higher NIHSS scores (median = 8 [interquartile range [IQR]: 3-19] vs. 4 [1-10], adjusted incidence rate ratio = 1.37 [95% CI: 1.35-1.38]) and unfavorable outcomes (52.1 vs. 33.6%, adjusted odds ratio = 1.27 [1.14-1.40]). Intergroup differences in unfavorable outcomes were attenuated among females (1.12 [0.95-1.32] vs. males 1.38 [1.21-1.56], P = 0.040) and in the subgroup that received reperfusion therapy (1.10 [0.92-1.33] vs. those who did not receive therapy 1.23 [1.08-1.39], P = 0.012). CONCLUSIONS: UOS was associated with unfavorable outcomes but to a lesser degree among females and patients receiving reperfusion therapy.

Associations between non-motor symptoms and patient characteristics in Parkinson's disease: a multicenter cross-sectional study.

Objective: Parkinson's disease (PD) is characterized by various non-motor symptoms (NMS), such as constipation, olfactory disturbance, sleep disturbance, mental disorders, and motor symptoms. This study aimed to investigate factors associated with NMS in patients with PD. Methods: Symptoms of PD were evaluated using the Movement Disorder Society Unified Parkinson's Disease Rating Scale (MDS-UPDRS), Parts I-IV. NMS was assessed using the MDS-UPDRS Part I (self-assessment of NMS) and rapid eye movement sleep behavior disorder (RBD) questionnaires. Patients were categorized by age into <70 years and ≥ 70 years (older adults) groups, according to disease duration into early-stage and advanced-stage groups with a cut-off value of 5 years for motor symptoms, and by sex into male and female groups. Results: A total of 431 patients with PD (202 males and 229 females) with a mean age of 67.7 years, a mean disease duration of 6.4 years, and a mean Part I total score of 9.9 participated in this study. The Part I total score was significantly positively correlated (p < 0.01) with disease duration and Part II, III, and IV scores. For Part I sub-item scores, the older group had significantly higher scores for cognitive impairment, hallucinations, sleep problems, urinary problems, and constipation than the <70 years group, whereas the advanced-stage group had significantly higher scores for hallucinations, sleep problems, daytime sleepiness, pain, urinary problems, and constipation (p < 0.05) than the early-stage group. Anxiety was higher in female patients than in male patients, whereas daytime sleepiness, urinary problems, and RBD were higher in male patients than in female patients (p < 0.05). Factors affecting Part I included disease duration, Part II total scores, Part IV total scores, and RBD. Conclusion: According to the self-questionnaire assessment, NMS was highly severe in older adult patients, those with longer illness duration, subjective and objective motor function impairments, and RBD. Sex-based differences were also observed.

Associations between probable REM sleep behavior disorder, olfactory disturbance, and clinical symptoms in Parkinson's disease: A multicenter cross-sectional study.

BACKGROUND: Rapid eye movement sleep behavior disorder (RBD) and olfactory dysfunction are useful for early diagnosis of Parkinson's disease (PD). RBD and severe olfactory dysfunction are also regarded as risk factors for cognitive impairment in PD. This study aimed to assess the associations between RBD, olfactory function, and clinical symptoms in patients with PD. METHODS: The participants were 404 patients with non-demented PD. Probable RBD (pRBD) was determined using the Japanese version of the RBD screening questionnaire (RBDSQ-J) and the RBD Single-Question Screen (RBD1Q). Olfactory function was evaluated using the odor identification test for Japanese. Clinical symptoms were evaluated using the Movement Disorder Society Revision of the Unified PD Rating Scale (MDS-UPDRS) parts I-IV. RESULTS: In total, 134 (33.2%) patients indicated a history of pRBD as determined by the RBD1Q and 136 (33.7%) by the RBDSQ-J based on a cutoff value of 6 points. Moreover, 101 patients were diagnosed as pRBD by both questionnaires, 35 by the RBDSQ-J only, and 33 by the RBD1Q only. The MDS-UPDRS parts I-III scores were significantly higher and disease duration significantly longer in the pRBD group. pRBD was significantly associated with male gender and the MDS-UPDRS part I score. The olfactory identification function was significantly reduced in the pRBD group. CONCLUSIONS: About 33% of the patients with PD had pRBD based on the questionnaires, and both motor and non-motor functions were significantly decreased in these patients. These results suggest that more extensive degeneration occurred in patients with non-demented PD with RBD.

Comparisons of Characteristics and Outcomes after Mechanical Thrombectomy for Vertebrobasilar Occlusion with Cardioembolism or Atherosclerotic Brain Infarction: Data from the Tokyo-Tama-Registry of Acute Endovascular Thrombectomy (TREAT).

BACKGROUND: Some reports suggest the efficacy of mechanical thrombectomy (MT) for acute vertebrobasilar artery (VBA) occlusion. The major causes of VBA occlusion include cardioembolism (CE) and large-artery atherosclerosis (LAA). However, the clinical characteristics of each cause remain unclear, and they might be important for decision making related to the indications and strategy of MT. OBJECTIVE: This study aimed to compare functional outcomes and factors affecting outcomes between patients with CE and LAA with acute VBA occlusion. METHODS: This was a retrospective and prospective observational study using data from TREAT (Tokyo-Tama-Registry of Acute Endovascular Thrombectomy), a multicenter registry of MT for acute large-vessel occlusion in the Tokyo metropolitan area. Patients with VBA occlusion classified into CE and LAA groups were analyzed. The primary outcome was a modified Rankin Scale score of 0-2 at 90 days. RESULTS: Seventy-nine patients (57 with CE and 22 with LAA) were eligible from January 2015 to March 2020. Despite significantly shorter puncture-to-recanalization and onset-or-last-well-known-to-recanalization times in the CE group, the primary outcome was not significantly different between the 2 groups (CE, 31.6% vs. LAA, 45.5%; P = 0.248). In the subgroup analysis, patients with CE had worse clinical outcomes in the onset-or-last-well-known-to-door time ≥180 minutes, onset-or-last-well-known-to-door time ≥300 minutes, and low posterior circulation Alberta Stroke Program Early CT Score (≤7) subgroups. CONCLUSIONS: Functional outcomes of VBA occlusion were not significantly different between CE and LAA. Based on the subgroup analysis, patients with CE might have poorer collateral status than do patients with LAA, and earlier recanalization might therefore be desired.

A Case of Endovascular Treatment for In-Hospital Stroke with COVID-19 under Protected Code Stroke.

Objective: Coronavirus disease 2019 (COVID-19) is characterized by severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) and presents with respiratory symptoms. Overall, 5.7% of COVID-19 patients with severe respiratory status have been reported to develop acute cerebrovascular diseases (CVDs), and 41.3% of COVID-19 cases were considered nosocomial infections. Therefore, Protected Code Stroke, which is a guideline for acute stroke management that takes into account the safety of healthcare workers, has been developed. We created an operational manual for COVID-19 in the endovascular treatment center of our hospital and report our experience treating acute stroke in a COVID-19 patient. Case Presentation: A 67-year-old man presented with a 5-day history of fever. Chest CT showed ground glass opacity (GGO) on admission, and the polymerase chain reaction (PCR) test for COVID-19 was positive. Dysarthria, right-sided hemiparesis, and aphasia were discovered on the morning of the third day after hospitalization. MRI showed an acute ischemic stroke at the left corona radiata and occlusion of the left middle cerebral artery (MCA). Progression of right-sided hemiparesis and exacerbation of respiratory status developed after the MRI. Tracheal intubation was performed, and the patient was treated with intravenous alteplase and mechanical thrombectomy (MT). Recanalization of blood flow was not obtained, and the neurological deficits remained. Conclusion: MT was performed for large-vessel occlusion (LVO) in a COVID-19 patient during the COVID-19 pandemic. Safety for healthcare workers and appropriate rapid treatment for acute stroke patients are both vital in the current environment.

Early detection of cognitive impairment in Parkinson's disease with the use of the Wisconsin Card Sorting Test: correlations with Montreal Cognitive Assessment and smell identification test.

Cognitive function is often impaired in early Parkinson's disease (PD). The Wisconsin Card Sorting Test (WCST) is a neuropsychological test of "set-shifting" ability. To see whether WCST is useful for detecting early changes of cognitive function in PD, we examined the correlations of WCST with the Montreal Cognitive Assessment (MoCA) and the Odor Stick Identification Test (OSIT). Subjects were 48 PD patients (age 66 ± 10 years; Hoehn & Yahr stage 2.3 ± 0.8; mean duration 3.1 ± 2.5 years). WCST sub-scores for categories achieved (CA), perseverative errors of Nelson type (PEN), and difficulties of maintaining set (DMS) were evaluated. MoCA-J (Japanese version) and OSIT-J (Japanese version) were done in that order, followed by the WCST. In PD patients, CA was 2.2 ± 2.0, PEN was 7.0 ± 6.4, and DMS was 2.3 ± 2.0, and all were worse than those of age-matched normal subjects. MoCA-J scores significantly correlated with PEN. OSIT-J scores were also significantly correlated with CA and DMS. As MoCA-J and OSIT-J show high sensitivity and specificity for detecting mild cognitive impairment in PD, WCST may also be a useful supplementary diagnostic tool for early and mild cognitive impairment in PD patients.

Postural Abnormality in Parkinson's Disease: A Large Comparative Study With General Population.

BACKGROUND: Postural abnormalities in Parkinson's disease (PD) patients and unimpaired elderly are not well differentiated. Factors related to postural abnormality associated with PD are controversial. OBJECTIVE: We assessed differences in postural change between PD patients and unimpaired elderly and elucidated factors related to abnormal posture in PD patients. METHODS: We measured the dropped head angle (DHA), anterior flexion angle (AFA), and lateral flexion angle (LFA) of the thoracolumbar spine of an unprecedented 1,117 PD patients and 2,732 general population participants (GPPs) using digital photographs. Two statistical analyses were used for elucidating factors related to these angles. RESULTS: In GPPs, age was correlated with DHA, AFA, and LFA. DHAs, AFAs, and LFAs of PD patients and age-matched GPPs were 21.70° ± 14.40° and 13.13° ± 10.79°, 5.98° ± 12.67,°and - 3.82° ± 4.04°, and 0.86° ± 4.25° and 1.33° ± 2.16°, respectively. In PD patients, factors related to DHA were age, male sex, and H & Y stage during ON time. Factors related to AFA were age, duration of disease, H & Y stage during ON and OFF times, pain, vertebral disease, and bending to the right. A factor related to LFA was AFA. CONCLUSIONS: DHA and AFA of GGPs correlated with age and were larger in PD patients than those with in GPPs. Some PD patients showed angles far beyond the normal distribution. Thus, factors associated with disease aggravation affected postural abnormality in PD patients.

Effectiveness of Nonvitamin K Antagonist Oral Anticoagulants and Warfarin for Preventing Further Cerebral Microbleeds in Acute Ischemic Stroke Patients with Nonvalvular Atrial Fibrillation and At Least One Microbleed: CMB-NOW Multisite Pilot Trial.

BACKGROUND: Nonvitamin K antagonist oral anticoagulants (NOACs) are considered superior, or at least noninferior, to warfarin in preventing stroke or systemic embolism in patients with nonvalvular atrial fibrillation. Here, we recruited acute ischemic stroke patients with nonvalvular atrial fibrillation and at least one cerebral microbleed (CMB), and evaluated the proportion of patients who had an increased number of CMBs (%) after receiving anticoagulant therapy with NOACs or with warfarin for 12 months. METHODS: This was a multicenter, prospective, observational cohort study at 20 centers, conducted between 2015 and 2017, in which we recruited 85 patients with at least one CMB detected by 1.5T magnetic resonance imaging (T2*WI) at baseline, who received NOACs or warfarin for at least 12 months. We compared the proportions of patients with increased numbers of CMBs in the NOACs and warfarin treatment groups. RESULTS: The proportions of patients with increased numbers of CMBs at month 12 of treatment were 28.6% and 66.7% in the NOACs and warfarin groups, respectively. The new CMBs showed no specific regional localization in either group. In the NOACs and warfarin groups, physicians prescribed lower-than-standard dosing in 13.3% and 50% of the cases, respectively. The administration of reduced doses at physicians' discretion did not appear to alter the incidence of new CMBs. DISCUSSION: This is the first evidence to suggest efficacy of NOACs for preventing further CMBs in patients with at least one CMB, although no statistical evaluation was carried out.

Botulinum toxin A is effective to treat tension-type headache caused by hemifacial spasm.

OBJECTIVE: We examined the relationship between hemifacial spasm (HFS; a form of cranio-cervical dystonia) and chronic primary headache, including tension-type headache (TTH). We also examined whether botulinum toxin A (BoNT/A) therapy for HFS ameliorates concomitant TTH. METHODS: Fifty-one HFS patients receiving BoNT/A therapy were recruited. Patients' characteristics (including age, gender, chronic headache history, exercise habits, stiff neck, cervical spondylolysis history), stress factors, worsening/new onset of headache associated with HFS, and dose of BoNT/A were examined. We diagnosed headache types according to The International Classification of Headache Disorders, 3rd edition, beta. Numerical Rating Scale (NRS) and Headache Impact Test-6 (HIT-6) scores for headache severity were compared between the 6-week baseline before BoNT/A therapy and 6-week follow-up after BoNT/A therapy. RESULTS: Of 51 patients with HFS, 17 (33.3%) reported worsening or new onset of headache (especially TTH) associated with HFS (Group-S), and 34 were not aware of headache (Group-N). Twelve patients (70.6%) in group-S reported improvement of headache after BoNT/A therapy. NRS (from 7 [5-9] to 0 [0-5], p<0.01) and HIT-6 (from 55 [54-64] to 44 [36-52], p<0.001) scores were significantly improved after BoNT/A therapy. Logistic regression analysis revealed significant interaction between TTH associated with HFS and the presence of stress factors (odds ratio 43.11: 2.95-629.39, p<0.001) and history of chronic headache (odds ratio 28.53: 2.96-275.10, p<0.001). CONCLUSIONS: Primary headache, especially TTH, is associated with HFS. BoNT/A therapy for HFS may also be indirectly effective for treatment of TTH.

Characteristics of Cerebral White Matter Lesions on MRI in Juvenile Patients with Migraine.

OBJECTIVE: Cerebral white matter lesions (WMLs) have been frequently observed on MRI in patients with migraine. We investigated characteristics of WMLs in migraine and tried to determine the relationship between its causal mechanism and arteriosclerosis. METHODS: A head MRI was performed in juvenile migraine patients. The distributions of deep and periventricular WMLs were separately studied in the anterior and posterior circulation. Grading was conducted according to the Fazekas classification. Arteriosclerotic risk factors were identified, and their effects on WMLs were investigated. RESULTS: WMLs were observed in 85 (40.5%) of 210 patients in our hospital. This is significantly higher than the 10 (19.2%) of 63 patients in the control group (p < 0.01). WMLs were significantly observed on the anterior territory of the deep white matter (p < 0.01) and the posterior territory of the periventricular white matter (p < 0.05). Multivariable analysis revealed that the occurrence of WMLs was not related to arteriosclerotic risk factors, while migraine (p < 0.01) and aging (p < 0.05) were significant risk factors. CONCLUSION: While migraine was a risk factor of WMLs, its relationship with arteriosclerotic factors was weak. Accordingly, a mechanism other than arteriosclerosis may be involved.

[Successful thrombolysis without hemorrhage in a patient with cardioembolic stroke under dabigatran treatment-a case report and review of literature].

A 72-year-old male with heart failure was admitted to our hospital. Treatment with dabigatran (220 mg per day) was initiated because of atrial fibrillation. On the third day, the patient developed left-sided hemiparesis and dysarthria at 4.5 hr after the last dose of dabigatran. The activated partial thromboplastin time (APTT) was 39.1 sec, and the NIHSS (NIH Stroke Scale) was 11. An intravenous infusion of rt-PA was administered at 160 min after the onset of hemiparesis (at 7 hr after the last dose of dabigatran). Although diffusion weighted MRI revealed a minor infarction in the right lower frontal gyrus, the patient was discharged without hemorrhage (NIHSS 0), and the score on the modified Rankin scale assessed 3 months later was 0. The outcomes have been good in 8 out of 9 reported cases, including the present case; the remaining severe case developed complicating intracranial hemorrhage. Thrombolytic therapy could be safe, if it is performed more than 7 hr after the last dose of dabigatran and the APTT is less than 40 sec.

Clinical features and varieties of non-motor fluctuations in Parkinson's disease: a Japanese multicenter study.

OBJECTIVE: This multicenter cross-sectional study aimed to investigate the clinical features and varieties of non-motor fluctuation in Parkinson's disease (PD). METHODS: To identify motor and non-motor fluctuation, we employed the wearing-off questionnaire of 19 symptoms (WOQ-19) in 464 PD patients. We compared the frequency of levodopa-related fluctuation as identified by the WOQ-19 with recognition by neurologists. We compared patients with both motor and non-motor fluctuations with those who only had motor fluctuations. Non-motor fluctuations were separated into psychiatric, autonomic, and sensory categories for further analysis. RESULTS: The patients' average age was 70.8 ± 8.4 years (mean ± SD) and disease duration was 6.6 ± 5.0 years. The frequency of motor fluctuations was 69% and for non-motor fluctuation 40%. Fifty-three percent of patients with motor fluctuations also had non-motor fluctuations, whereas 93% of patients with non-motor fluctuations also had motor fluctuations. The WOQ-19 showed a sensitivity of 82% but a specificity of only 40%. The patients with both non-motor and motor fluctuations exhibited more severe motor symptoms, more non-motor symptoms and higher levodopa daily doses (p < 0.05). Patients had significantly higher fluctuation rates if they had psychiatric (49%) and sensory (45%) symptoms than patients with autonomic symptoms (32%, p < 0.01). Forty-eight percent of patients with non-motor fluctuations exhibited more than one type of non-motor fluctuation. CONCLUSION: Forty percent of PD patients presented with non-motor fluctuations, and almost half of these exhibited more than one type. Appropriate recognition of levodopa-related fluctuations, both motor and non-motor, can lead to treatment modifications in PD patients.

Pramipexole reduces the prevalence of fatigue in patients with Parkinson's disease.

OBJECTIVE: The aim of this multicenter cross-sectional study was to assess the relation between fatigue in a large number of Japanese patients with Parkinson's disease (PD) and drugs taken to treat PD. METHOD: We used the 16-item Parkinson Fatigue Scale (PFS-16), which was designed to assess fatigue exclusively associated with PD. Multiple logistic regression analyses were used to assess the relation between antiparkinson drugs and fatigue in PD. RESULTS: A total of 350 non-demented PD patients were enrolled. Fatigue (PFS score of ≥4) was revealed in 319 patients (91%). Pramipexole was administered to 24% of PD patients. Multiple logistic regression analysis revealed that the administration of Pramipexole was significantly related to low rates of fatigue in PD patients with Hoehn and Yahr stage <3 (p=0.011, odds ratio=5.23, 95% confidence interval; 1.47-18.63). CONCLUSION: The reduced fatigue in PD patients was observed in taking Pramipexole.

[The development and validation of a new comprehensive self-completing questionnaire for symptoms in Parkinson's disease (MASAC-PD 31)].

BACKGROUND: Patients with Parkinson's disease (PD) suffer from various symptoms. In order to identify untreated symptoms within the limited time of a clinical interview, we developed a new self-completing questionnaire (MASAC-PD 31). The questionnaire consists of two parts (5 domains, 31 items); Part I intended at rating the motor symptoms and activities of daily living (ADL) during both "on" and "off" periods, and Part II aimed at screening and assessing mainly the non-motor symptoms, such as sleep-related difficulties, autonomic symptoms, cognition, mood and others. The purpose of this study was to evaluate the validity, reliability, and clinical usefulness of the questionnaire. SUBJECTS AND METHODS: Based on the number of valid answers in a pilot trial, MASAC-PD 31 was refined by improving the expression and layout. Of the initially enrolled 107 patients attending three hospitals, 102 patients were included in the final analysis. Correlations of the scores on the MASAC-PD 31 with other clinical scales were evaluated. A second trial consisting of 57 participants was conducted a month later to assess the test-retest reproducibility of the questionnaire. RESULTS: The average time needed to complete MASAC-PD 31 was 17 min (range: 3-90 min). Each of the domains in Part I showed high internal consistency (Cronbach's alpha: 0.663 for "on" motor) and strong correlations with preexisting indices (Spearman's correlation coefficient: 0.547, 0.544, and 0.571 for "on" motor against "on" UPDRS, PDQ-39, and Schwab & England ADL scale, respectively). The questions in the Part II domains also showed strong correlations with preexisting scales. Most of the items showed high reproducibility (weighted kappa coefficient) and consistency. CONCLUSION: This new comprehensive questionnaire was shown to be valid and reliable for assessing the motor disability in patients with PD. Moreover, it may be useful in clinical management for identifying clinically unrecognized symptoms, especially non-motor problems.

Fatigue in Japanese patients with Parkinson's disease: a study using Parkinson fatigue scale.

The objective of this multicenter cross-sectional study was to determine the prevalence of fatigue and factors contributing to it in a large sample of Japanese patients with Parkinson's disease (PD). We used the 16-item Parkinson Fatigue Scale (PFS-16), which was designed to assess fatigue exclusively associated with PD. We carried out this study using PFS-16, the Unified Parkinson's Disease Rating Scale, Zung's Self-Rating Depression Scale, Parkinson's Disease Sleep Scale (PDSS), and the PD quality of life (QOL) scale (PDQ-39) by interview using questionnaires and physical examination by neurologists in 361 nondemented PD patients. Fatigue (an average PFS score of 3.3 or greater) was revealed in 151 patients (41.8%). Multiple logistic regression analysis indicated that the significant independent variables related to the presence of fatigue were the scores of PDSS and PDQ-39. Depression score was not a significant contributing factor. Our study revealed that the prevalence of fatigue in Japanese PD patients is as high as that in Western countries, and that fatigue is a relatively independent symptom, although sleep disturbance may be associated with fatigue. Since fatigue is significantly related to QOL reduction, therapeutic interventions including treatment of sleep disturbance are important.

Cerebral pachyleptomeningitis associated with MPO-ANCA induced by PTU therapy.

A 34-year-old woman with hyperthyroidism, who had been previously treated with propylthiouracil (PTU) is reported. She was admitted because of clumsiness in her left hand and abnormal behavior. A neurological examination demonstrated impairment of higher cortical function, and weakness and hyperreflexia of the left leg. An MRI scan with gadolinium enhancement showed pachyleptomeningeal thickening in the right frontoparietal lobe. Blood tests revealed a high MPO-ANCA titer of 122 EU (normal:<10 EU). After steroid administration, the neurological symptoms and the MRI findings improved markedly. This is the first report of PTU-induced cerebral pachyleptomeningitis associated with a high serum MPO-ANCA titer.

Co-morbidity of moyamoya disease with Graves' disease. report of three cases and a review of the literature.

Moyamoya disease is a cerebrovascular disorder characterized by bilateral stenosis or occlusion of the terminal portions of the internal carotid arteries accompanied by typical net-like collateral vessels in the basal ganglia. Although the etiology of moyamoya disease remains unknown, hereditary and immunogenic as well as hemodynamic factors have been implicated in the underlying mechanism of moyamoya disease. We report two patients with confirmed moyamoya disease and a patient with probable moyamoya disease complicated with Graves' disease. We reviewed the literature and summarized 23 cases of moyamoya disease or probable moyamoya, coexisting with Graves' disease.

[A case of Good syndrome accompanied by myasthenia gravis: immunological evaluations].

Good syndrome, characterized by both thymoma and hypogammaglobulinemia, is a rare immunodeficient disorder. We experienced a case of Good syndrome accompanied by myasthenia gravis (MG). A 58-year-old man was admitted to our hospital because of muscle weakness and fatigability. Based on the presence of anti-acetylcholine receptor (AChR) antibody and thymoma, he was diagnosed as having MG. Peripheral blood lymphocyte count was normal, but gammaglobulin levels were markedly decreased (IgG 283 mg/dl, IgA 17 mg/dl, IgM 1 mg/dl). Clinical remission of MG was achieved by thymectomy followed by high-dose corticosteroids. Despite monthly intravenous immunoglobulin supplementation, he suffered from repeated respiratory tract infections and candidiasis. Body CT revealed adrenal tumor and pancreatic cancer with liver metastasis, and he died of bacterial pneumonia. Immunological evaluations showed complete lack of CD19+ B cell in the peripheral blood and responses of peripheral blood mononuclear cells to mitogens. Peripheral blood T cells responded to a suboptimal concentration of a recombinant AChR fragment: this pattern of AChR-induced T cell response was typical of MG patients. We failed to detect IgG autoantibodies reactive with B cells in his serum. Patients with Good syndrome represent imbalance of immune responses, leading to both immunodeficiency and autoimmunity.