[Complimentary use of needle electromyography and ultrasonography of tongue is effective for early detection of abnormality in 20 patients with amyotrophic lateral sclerosis].

A group of 20 consecutive patients with amyotrophic lateral sclerosis (ALS) were evaluated using electromyography (EMG) and ultrasonography (US) of the tongue. Their records were reviewed retrospectively for the rates at which abnormalities were detected by these two modalities as well as their clinical features. Visual inspection detected abnormalities in 9 of 20 patients, EMG in 12, and US in 6. However, EMG detected active denervation earlier than did US in 7 of the 12 EMG-diagnosed patients, and US detected fasciculation earlier than did EMG in 1 of the 6 US-diagnosed patients. Thus, we cannot replace EMG completely with US. Indeed, we currently use both methods complementarily at our hospital.

The Sequential Ultrasonographic, Electrophysiological and MRI Findings in a Patient with the Pharyngeal-cervical-brachial Variant of Guillain-Barré Syndrome from the Acute Phase to the Chronic Phase.

Acute progressive weakness in bulbar, neck and limbs is included in several differential diagnoses, including the pharyngeal-cervical-brachial (PCB) variant of Guillain-Barré syndrome (GBS). Patients with the PCB variant of GBS are reported to have localized diagnostic cervical spinal nerve abnormalities that can be examined by nerve ultrasonography (NUS) and magnetic resonance neurography (MRN). We herein report the case of a 77-year-old man with the PCB variant of GBS. Although the nerve conduction study (NCS) findings were indirect indicators for an early diagnosis, the combination of NCS and NUS was a useful complementary measure that facilitated an early diagnosis. MRN did not show any apparent diagnostic abnormalities. After early treatment, the patient was discharged and returned home.

[Clinical manifestations of 5 patients with idiopathic paroxysmal kinesigenic choreoathetosis].

Paroxysmal kinesigenic choreoathetosis (PKC) is a rare disorder characterized by recurrent and brief attacks of choreoathetoid and/or dystonic movements in trunk and limbs triggered by initiation of voluntary movement. Of 5 patients with idiopathic PKC in our hospital, four were men and one was with family history. Age of onset ranged from 8 to 15 years old. They were consistent with previous reports in the characteristics of involuntary movements, normal neurological findings, normal laboratory data, no abnormal findings of standard imaging studies, and good restraining effects on attacks with carbamazepine. Individual body parts where attacks often involved were different among 5 patients. Although previous reports which said the prognosis and outcome of PKC were good, neuropsychological examinations in our study revealed that 2 patients out of 5 had certain cortical dysfunction, one patient was with progressive deterioration, and the other was with underlying mild abnormalities. Detailed and serial neuropsychological examinations might be necessary for some PKC patients.

Seizure Outcomes and Predictors of Recurrent Post-Stroke Seizure: A Retrospective Observational Cohort Study.

BACKGROUND: Seizure is a common complication after stroke (termed "post-stroke seizure," PSS). Although many studies have assessed outcomes and risk factors of PSS, no reliable predictors are currently available to determine PSS recurrence. We compared baseline clinical characteristics and post-stroke treatment regimens between recurrent and non-recurrent PSS patients to identify factors predictive of recurrence. METHODS: Consecutive PSS patients admitted to our stroke center between January 2011 and July 2013 were monitored until February 2014 (median 357 days; IQR, 160-552) and retrospectively evaluated for baseline clinical characteristics and PSS recurrence. Cumulative recurrence rates at 90, 180, and 360 days post-stroke were estimated by Kaplan-Meier analysis. Independent predictors of recurrent PSS were identified by Cox proportional-hazards analysis. RESULTS: A total of 104 patients (71 men; mean age, 72.1 ± 11.2 years) were analyzed. PSS recurred in 31 patients (30%) during the follow-up. Factors significantly associated with PSS recurrence by log-rank analysis included previous PSS, valproic acid (VPA) monotherapy, polytherapy with antiepileptic drugs (AEDs), frontal cortical lesion, and higher modified Rankin Scale score at discharge (all p < 0.05). Independent predictors of recurrent PSS were age <74 years (HR 2.38, 95% CI 1.02-5.90), VPA monotherapy (HR 3.86, 95% CI 1.30-12.62), and convulsions on admission (HR 3.87, 95% CI 1.35-12.76). CONCLUSIONS: Approximately one-third of PSS patients experienced seizure recurrence within one year. The predictors of recurrent PSS were younger age, presence of convulsions and VPA monotherapy. Our findings should be interpreted cautiously in countries where monotherapy with second-generation AEDs has been approved because this study was conducted while second-generation AEDs had not been officially approved for monotherapy in Japan.

Reduced estimated glomerular filtration rate affects outcomes 3 months after intracerebral hemorrhage: the stroke acute management with urgent risk-factor assessment and improvement-intracerebral hemorrhage study.

BACKGROUND: The effect of renal dysfunction on intracerebral hemorrhage (ICH) remains unclear. We investigated associations of renal dysfunction assessed by estimated glomerular filtration rate (eGFR) with clinical courses and outcomes in ICH patients. METHODS: From a prospective, multicenter, observational study, 203 patients who had supratentorial ICH within 3 hours of onset were included. Patients were classified into 3 groups based on eGFR: Group 1 (eGFR < 60 mL/minute/m(2)), Group 2 (60-89), and Group 3 (≥ 90). Outcomes included neurologic deterioration within 72 hours, hematoma expansion (> 33% in volume) at 24 hours, and favorable (modified Rankin Scale [mRS] ≤ 2) or unfavorable (mRS ≥ 5) outcome at 3 months. RESULTS: Thirty-seven patients (16 women, 74.6 ± 13.2 years) were assigned to Group 1, 99 (34 women, 65.2 ± 11.4 years) to Group 2, and 67 (30 women, 61.3 ± 9.4 years) to Group 3. Significant differences were found in age (P < .001) and initial systolic blood pressure among the groups (208.4 ± 18.0, 201.9 ± 15.1, and 198.1 ± 14.2 mm Hg for Group 1, 2, and 3, respectively; P = .006). Similar rates of neurologic deterioration (14%, 6%, and 6%) and hematoma expansion (16%, 14%, and 18%) were observed among the groups. However, in Group 1, favorable outcome was less frequent (17%, 48%, and 42%; P = .002) and unfavorable outcome was more frequent (24%, 7%, and 6%; P = .013) than in the other groups. After adjustment for confounders, eGFR < 60 mL/minute/m(2) was independently associated with both favorable outcome (odds ratio [OR], .21; 95% CI, .07-.54) and unfavorable outcome (OR, 5.64; 95% CI, 1.80-18.58). CONCLUSIONS: Renal dysfunction (eGFR < 60 mL/minute/m(2)) was associated with poor clinical outcome after ICH.

Examination timing and lesion patterns in diffusion-weighted magnetic resonance imaging of patients with classically defined transient ischemic attack.

BACKGROUND: This study investigated factors associated with the presence of acute ischemic lesions after transient ischemic attack (TIA), using diffusion-weighted imaging (DWI) data from a multicenter retrospective, observational study. METHODS: Of the 464 patients admitted to 13 stroke centers in Japan within 7 days after TIA onset, 458 patients underwent a DWI examination in this registry. Patients were divided into those with acute ischemic lesions and those without. We analyzed associations between DWI lesions and baseline characteristics, including age, sex, comorbidities, large artery atherosclerosis (LAA), type and duration of symptoms, the presence of multiple occurrences of TIA within 90 days before hospital visits (multiple TIAs) and the time from symptom onset to DWI examination (time-to-DWI). RESULTS: Among the 458 patients (291 men, 68.4±13.2 years old), 374 (81.7%) underwent a DWI examination within the initial 24 hours after the symptom onset. DWI lesions were found in 96 patients (21.0%), and divided into a single lesion (56 patients, 12.2%) or multiple lesions (40 patients, 8.7%). The presence of DWI lesions had an association with male sex (odds ratio [OR] 1.84; 95% confidence interval [CI] 1.07-3.29), time-to-DWI longer than 24 hours (OR 2.96; CI 1.57-5.52), and intracranial LAA (OR 1.99; CI 1.02-3.79). The presence of a single DWI lesion had an association with atrial fibrillation (OR 2.70; CI 1.41-5.03), and multiple DWI lesions did with time-to-DWI longer than 24 hours (OR 6.20; CI 2.60-15.20), multiple TIAs (OR 3.04; CI 1.35-6.76), intracranial LAA (OR 3.63; CI 1.44-8.89), and extracranial LAA (OR 3.53; CI 1.08-10.78). CONCLUSIONS: Acute ischemic lesions on DWI were associated with time-to-DWI and LAA in patients with classically defined TIA. Additionally, we identified some differences in relating factors between patients with single and multiple DWI lesions. These results indicate that time-to-DWI and DWI lesion pattern may be important for the diagnosis and management of TIA.

Impact of early blood pressure variability on stroke outcomes after thrombolysis: the SAMURAI rt-PA Registry.

BACKGROUND AND PURPOSE: The present study determines associations between early blood pressure (BP) variability and stroke outcomes after intravenous thrombolysis. METHODS: In 527 stroke patients receiving intravenous alteplase (0.6 mg/kg), BP was measured 8 times within the first 25 hours. BP variability was determined as ΔBP (maximum-minimum), standard deviation (SD), coefficient of variation, and successive variation. RESULTS: The systolic BP course was lower among patients with modified Rankin Scale (mRS) 0 to 1 than those without (P<0.001). Most of systolic BP variability profiles were significantly associated with outcomes. Adjusted odds ratios (95% confidence interval) per 10 mm Hg (or 10% for coefficient of variation) on symptomatic intracerebral hemorrhage were as follows: ΔBP, 1.33 (1.08-1.66); SD, 2.52 (1.26-5.12); coefficient of variation, 3.15 (1.12-8.84); and successive variation, 1.82 (1.04-3.10). The respective values were 0.88 (0.77-0.99), 0.73 (0.48-1.09), 0.77 (0.43-1.34), and 0.76 (0.56-1.03) for 3-month mRS 0 to 1; and 1.40 (1.14-1.75), 2.85 (1.47-5.65), 4.67 (1.78-12.6), and 1.99 (1.20-3.25) for death. Initial BP values before thrombolysis were not associated with any outcomes. CONCLUSIONS: Early systolic BP variability was positively associated with symptomatic intracerebral hemorrhage and death after intravenous thrombolysis.

Intravenous alteplase at 0.6 mg/kg for acute stroke patients with basilar artery occlusion: the stroke acute management with urgent risk factor assessment and improvement (SAMURAI) Recombinant tissue plasminogen activator registry.

BACKGROUND: The therapeutic efficacy of low-dose intravenous alteplase (0.6 mg/kg) for basilar artery occlusion (BAO) remains unknown. METHODS: BAO patients enrolled from the Japanese multicenter registry involving 600 stroke patients treated with the low-dose intravenous alteplase were studied. RESULTS: Twenty-five patients had BAO (8 women ranging from 32-92 years of age; mean baseline National Institutes of Health Stroke Scale [NIHSS] score 16). The stroke subtype was cardioembolic in 15 patients and atherothrombotic in 4 patients. BAO was recanalized during hospitalization in 18 (78%) of 23 patients undergoing follow-up angiography. Within the initial 24 hours, 14 patients (56%) had a ≥ 8-point decrease in the NIHSS score, being more common than 267 patients with middle cerebral artery occlusion (MCO) from the same registry (odds ratio [OR] 2.50; 95% confidence interval [CI] 1.06-5.97) after adjustment by sex, age, and baseline NIHSS score. In addition, 4 patients (16%) had a ≥ 4-point increase in the score, being marginally more common than MCO patients (OR 3.13; 95% CI 0.81-10.25). Symptomatic intracranial hemorrhage within the initial 36 hours (8% v 5%), independence at 3 months (modified Rankin Scale score ≤ 2, 48% v 52%), and mortality at 3 months (4% v 6%) were similar when comparing BAO and MCO patients. When compared with previous studies of BAO, vital and functional outcomes at 3 months were relatively better in our study. CONCLUSIONS: The use of low-dose alteplase resulted in similar outcomes when comparing acute BAO and MCO patients.

Systolic blood pressure lowering to 160 mmHg or less using nicardipine in acute intracerebral hemorrhage: a prospective, multicenter, observational study (the Stroke Acute Management with Urgent Risk-factor Assessment and Improvement-Intracerebral Hemorrhage study).

OBJECTIVE: Optimal blood pressure (BP) control in acute intracerebral hemorrhage (ICH) remains controversial. We determined the effects of SBP lowering to 160 mmHg or more using intravenous nicardipine for acute ICH patients. METHODS: This is a prospective, multicenter, observational study conducted in Japan, with the lack of control groups. Patients with supratentorial ICH within 3 h of onset, admission SBP 180 mmHg or more, Glasgow Coma Scale (GCS) 5 or more, and hematoma volume less than 60 ml were initially treated with intravenous nicardipine to maintain SBP between 120 and 160 mmHg with 24-h frequent BP monitoring. The primary endpoints were neurological deterioration within 72 h [GCS decrement ≥ 2 points or National Institutes of Health Stroke Scale (NIHSS) increment ≥ 4 points; estimated 90% confidence interval (CI) on the basis of previous studies: 15.2-25.9%] and serious adverse effects (SAE) to stopping intravenous nicardipine within 24 h (1.8-8.9%). The secondary endpoints included hematoma expansion more than 33% at 24 h (17.1-28.3%), modified Rankin Scale (mRS) 4 or more (54.5-67.9%) and death at 3 months (6.0-13.5%). RESULTS: We enrolled 211 Japanese patients (81 women, 65.6 ± 12.0 years old). At baseline, BP was 201.8 ± 15.7/107.9 ± 15.0 mmHg. Median hematoma volume was 10.2 ml (interquartile range 5.6-19.2), and NIHSS score was 13 (8-17). Neurological deterioration was identified in 17 patients (8.1%), SAE in two (0.9%), hematoma expansion in 36 (17.1%), mRS 4 or more in 87 (41.2%), and death in four (1.9%). All the results were equal to or below the estimated lower 90% CI. CONCLUSION: SBP lowering to 160 mmHg or less using nicardipine appears to be well tolerated and feasible for acute ICH.

Low-dose intravenous recombinant tissue-type plasminogen activator therapy for patients with stroke outside European indications: Stroke Acute Management with Urgent Risk-factor Assessment and Improvement (SAMURAI) rtPA Registry.

BACKGROUND AND PURPOSE: The purpose of this study was to determine the safety and efficacy of intravenous recombinant tissue-type plasminogen activator (0.6 mg/kg alteplase) within 3 hours of stroke onset in Japanese patients outside the indications in the European license. METHODS: Of the 600 patients who were treated with recombinant tissue-type plasminogen activator, 422 met the inclusion criteria of the European license (IN group) and 178 did not (OUT group). RESULTS: The OUT group was inversely associated with any intracerebral hemorrhage (adjusted OR, 0.50; 95% CI, 0.29-0.84), positively associated with an unfavorable outcome (2.48; 1.55-3.94) and mortality (2.04; 1.02-4.04), and not associated with symptomatic intracerebral hemorrhage (0.53; 0.11-1.79) or complete independency (0.65; 0.40-1.03) after multivariate adjustment. CONCLUSIONS: Functional and vital outcomes 3 months after low-dose recombinant tissue-type plasminogen activator in patients outside the European indications were less favorable compared with those included in the indications; however, the risk of intracerebral hemorrhage was not.