Imbalance in multiple sclerosis and neuromyelitis optica: association with deep sensation disturbance.

Abnormality in balance is one of the most important causes of gait disturbance which has a direct impact to disability and medical cost in multiple sclerosis (MS) and neuromyelitis optica (NMO). However, characteristics of imbalance in these two diseases have not been fully elucidated. The aim of this study was to evaluate the degree and features of imbalance using stabilography, the degree of deep sensation disturbance using tibial nerve somatosensory evoked potentials (SEP), and their association with clinical impairment, in patients with MS and NMO. Seven NMO patients and seven MS patients with balance disturbance were examined. The relationship among stabilography measurements representing the degree and features of imbalance, height-adjusted P38 peak latency of SEP, and neurological functional disability, were analyzed. Stabilography evaluation showed a significantly severer degree of imbalance in NMO than in MS. Romberg quotient of the patients with brainstem lesions was significantly larger than those without them. In all patients, length of excursion per second significantly correlated positively with anterio-posterior-axis power spectra at intermediate frequency band. In all patients and in NMO, P38 peak latency adjusted by height significantly correlated positively with anterio-posterior-axis power spectra at intermediate frequency band. These findings suggest that the degree of imbalance of MS and NMO possibly correlate with deep sensation disturbance, which could be evaluated by anterio-posterior-axis power spectra at intermediate frequency band by stabilography. Severer imbalance in NMO than MS may be associated with the severe longitudinally extensive spinal cord lesions.

Usefulness of exercise stress echocardiography for predicting cardiovascular events and atrial fibrillation in hypertrophic cardiomyopathy.

BACKGROUND: In hypertrophic cardiomyopathy (HCM), the determinants of exercise tolerance and the usefulness of exercise stress echocardiography (ESE) for predicting hard endpoints have not been fully investigated. We aimed to assess the key parameters of ESE for exercise tolerance and the factors predictive of cardiovascular events and new-onset atrial fibrillation (AF) in patients with HCM. METHODS: Seventy-four consecutive patients with HCM who underwent ESE and with an ejection fraction ≥50 % were enrolled. The primary endpoint was a composite of cardiovascular death, heart failure hospitalization, ventricular fibrillation or tachycardia, and ventricular assist device implantation. The secondary endpoint was new-onset AF. RESULTS: The primary endpoint occurred in 13 patients. The left and right ventricular functions during exercise were responsible for decreased exercise tolerance. Peak exercise e' and tricuspid annular plane systolic excursion (TAPSE) significantly predicted increased primary outcome risk (hazard ratio 1.35, 95 % confidence interval 1.10-1.76, p = 0.003; hazard ratio 1.19, 95 % confidence interval 1.07-1.32, p = 0.002, respectively), and the results were consistent even after adjustment by maximum workload. These ESE parameters improved the prognostic model containing estimated glomerular filtration rate (eGFR) and left atrial (LA) volume index. In AF-naive patients (n = 58), LA volume, peak exercise LA reservoir strain, and left ventricular outflow tract (LVOT) pressure gradient predicted new-onset AF. CONCLUSIONS: In patients with HCM, ESE parameters related to left and right ventricular function were responsible for low exercise tolerance. Furthermore, e' and TAPSE at peak workload could be useful for predicting cardiovascular events in addition to eGFR and LA volume index. LVOT pressure gradient and LA function during exercise predicted new-onset AF.

Amplitude of Somatosensory Evoked Potentials (SEPs) Recorded in Short-Latency SEP Condition Is 80% of That in Giant SEP Condition.

PURPOSE: Giant somatosensory evoked potentials (SEPs) with enhanced long-loop reflex (C-reflex) are useful to detect cortical motor hyperexcitability in patients with myoclonic epilepsy. The recording conditions of giant SEPs are different from those of short-latency SEPs (SSEPs). We investigated the waveform characteristics obtained for each condition. METHODS: Forty-eight upper limbs of 24 adult normal subjects (12 men, age 35.5 ± 9.7 years [mean ± SD]) were investigated. Somatosensory evoked potentials of each subject were recorded in both conditions on the same day. The main differences in recording conditions were reference electrodes (SSEP: Fz vs. giant SEP: the earlobe electrode ipsilateral to the stimulated limb), stimulus rate (5 vs. 1 Hz), and bandpass filter (20 Hz-3 kHz vs. 1 Hz-1 kHz). Somatosensory evoked potentials were elicited by unilateral percutaneous electrical stimulation of the median nerve at the wrist with intensity of 110% of the movement threshold and recoded at C3'/C4'. RESULTS: The amplitudes of N20 onset-N20 and N20-P25 were significantly larger in giant SEP condition than in SSEP condition (p < 0.001). The mean + 3SD of N20-P25 amplitude was 10.0 µV in giant SEP condition and 7.8 µV in SSEP condition. The N20-P25 amplitude was significantly correlated between giant SEP condition and SSEP condition (R = 0.64, p < 0.001). C-reflex was not elicited. CONCLUSIONS: The amplitude of SEPs in SSEP condition is equivalent to 80% of that in giant SEP condition. The information is useful for detecting cortical hyperexcitability in various neurological disorders including myoclonic epilepsy.

Case series of mobile structures detected vividly by using superb microvascular imaging.

Background: Superb microvascular imaging (SMI) is a new imaging technique that can reveal low-velocity blood flow without use of a contrast agent. SMI is based on an original algorithm and effectively removes tissue motion artifacts (clutter motion) from the background, thereby preserving visibility of low-velocity blood flow. SMI is expected to be useful for the evaluation of heart diseases, as well as blood vessels. Case summary: Here, we report three cases in which a mobile structure in the heart or a blood vessel was detected easily by strong enhancement on SMI. In the heart, the entire mass was strongly enhanced by colour-SMI and had the appearance of 'a fire ball'. In the abdominal aorta and carotid artery, SMI captured a strongly enhanced echo image of a mass and revealed hyperechoic mobile plaque. It was hard to detect with the conventional echocardiography. Discussion: It is important to detect mobile intravascular and intracardiac structures as they are risk factors of thrombosis. Echo images are often strongly affected by the skill of the examiner, the patient's body habitus, and the presence of intestinal gas; thus, it is often difficult to detect a small mass with conventional echocardiography. With the use of SMI, even small mobile structures can be displayed at high intensity in comparison with the surrounding blood flow. Therefore, the non-invasive SMI was useful for the detection of mobile intravascular and intracardiac structures. Our findings of the current report may lead to new developments in SMI for imaging in the cardiac region.

Substitute parameters of exercise-induced pulmonary hypertension and usefulness of low workload exercise stress echocardiography in mitral regurgitation.

In asymptomatic patients with mitral regurgitation (MR), data of exercise-induced pulmonary hypertension (EIPH) are limited, and feasibility of evaluating EIPH is not high. We aimed to investigate prognostic impact of EIPH and its substitute parameters. Exercise stress echocardiography (ESE) were performed in 123 consecutive patients with moderate to severe degenerative MR. The endpoint was a composite of death, hospitalization for heart failure, and worsening of symptoms. EIPH [tricuspid regurgitation peak gradient (TRPG) at peak workload ≥ 50 mmHg] was shown in 57 patients (46%). TRPG at low workload was independently associated with TRPG at peak workload (ß = 0.67, p < 0.001). Early surgical intervention (within 6 months after ESE) was performed in 65 patients. Of the remaining 58 patients with the watchful waiting strategy, the event free survival was lower in patients with EIPH than in patients without EIPH (48.1 vs. 97.0% at 1-year, p < 0.001). TRPG at low workload ≥ 35.0 mmHg as well as EIPH were associated with poor prognosis in patients with the watchful waiting strategy. In conclusion, the importance of ESE and evaluating EIPH in patients with MR was re-acknowledged. TRPG at peak workload can be predicted by TRPG at low workload, and TRPG at low workload may be useful in real-world clinical settings.

Utility of real-time three-dimensional echocardiography for Duchenne muscular dystrophy with echocardiographic limitations.

Duchenne muscular dystrophy (DMD) is strongly associated with a unique form of dilated cardiomyopathy. Cardiac complications are the leading cause of death in DMD; thus, longitudinal assessments and early intervention for cardiac dysfunction are necessary to improve prognosis. Two-dimensional echocardiography, which is routinely used for cardiac assessment, has some limitations for quantitative analyses in DMD patients with thoracic deformities and regional wall motion abnormalities in the left ventricle. Recently, real-time three-dimensional echocardiography has emerged as a feasible tool for cardiac assessment in various cardiac diseases. The aim of this study was to examine the utility of this technology in DMD. We evaluated left ventricular ejection fraction (LVEF), a major parameter of left ventricular function, in 17 male DMD patients. LVEF values measured by real-time three-dimensional echocardiography were compared with those determined by two established nuclear cardiology methods: "the first-pass method of radionuclide angiocardiography" and "quantitative electrocardiogram-gated single-photon emission computed tomography". A good correlation was observed for LVEF values, particularly between real-time three-dimensional echocardiography and "the first-pass method of radionuclide angiocardiography" (r=0.90, p<0.05). Thus, real-time three-dimensional echocardiography can provide an accurate measurement of LVEF in DMD patients with echocardiographic limitations.