Pedro Nascimento Alves

Ultrasound for assessment of diaphragm in ALS

OBJECTIVE To evaluate the correlation between diaphragm thickness assessed by ultrasound (US) with respiratory function tests and the diaphragm motor responses, in patients with amyotrophic lateral sclerosis (ALS). METHODS 42 consecutive ALS patients were studied (11 with bulbar-onset), excluding patients with marked orofacial paresis. Investigation included: revised ALS functional rating scale (ALSFRS-R), forced vital capacity (FVC), maximal voluntary ventilation (MVV), maximal inspiratory (MIP) and expiratory (MEP) pressures, nasal inspiratory pressure during sniff (SNIP); peak-to-peak amplitude of the diaphragmatic motor response to phrenic nerve stimulation (Diaphragm-CMAP), diaphragmatic thickness measured by ultrasound during maximal inspiration and during maximal expiration. Patients were analysed in bulbar or spinal subgroups. Correlations and multiple linear regression models were studied. RESULTS The mean age at disease onset was 58.4 ± 11.1 years and with a mean disease duration of 17.8 ± 13.6 months. Ultrasound studies of diaphragm thickness in full inspiration correlated with diaphragm CMAP in the whole population and in spinal-onset patients; and were similar in the two groups. Multiple linear modelling showed that FVC, SNIP and MVV were dependent on the change of thickness (p=0.001, 0.001 and 0.020, respectively) and that MIP and MEP were related to diaphragm CMAP p=0.003 and p=0.025, respectively). CONCLUSION Diaphragm thickness correlates with Diaphragm-CMAP, except in bulbar-onset patients. Respiratory tests are dependent on both diaphragm thickness and Diaphragm-CMAP. SIGNIFICANCE US thickness of the diaphragm correlates with the number of functional motor units as assessed by the phrenic nerve motor amplitude.

Phrenic nerve stimulation is more sensitive than ultrasound measurement of diaphragm thickness in assessing early ALS progression.

AIMS OF THE STUDY To compare the assessment of respiratory decline with conventional tests, measurement of diaphragm compound muscle action potential (CMAP) to phrenic nerve stimulation and diaphragm thickness to ultrasound (US) investigation in amyotrophic lateral sclerosis (ALS) patients followed for a short period of time. PATIENTS AND METHODS We evaluated in 40 consecutive ALS patients, the clinical functional scale (ALSFRS-R), forced vital capacity (FVC), maximal voluntary ventilation (MVV), maximal inspiratory (MIP) and expiratory (MEP) pressures, sniff nasal inspiratory pressure (SNIP), Diaphragm-CMAP (latency and amplitude) and diaphragm US (maximal and minimal diaphragm thickness during full inspiration and expiration, respectively). Patients were evaluated at study entry and 4 months later. RESULTS Clinical functional assessment (ALSFRS-R), MEP, SNIP, and Diaphragm-CMAP amplitude declined significantly. Conversely, FVC, MVV, MIP, and diaphragm thickness showed a non-significant decline. We found significant correlations between all variables measured at both evaluation times. CONCLUSION In this study, we included patients who might be potentially eligible for a clinical trial and re-evaluated them within 4 months. In this short time, diaphragm thickness as assessed by US is not sensitive to detect changes. However, Diaphragm-CMAP amplitude was confirmed to be a sensitive non-volitional method to measure respiratory function in ALS.

Patients With Undetermined Stroke Have Increased Atrial Fibrosis: A Cardiac Magnetic Resonance Imaging Study

Background and Purpose— Some patients with ischemic strokes that are currently classified as having an undetermined cause may have structural or functional changes of the left atrium (LA) and left atrial appendage, which increase their risk of thromboembolism. We compared the LA and left atrial appendage of patients with different ischemic stroke causes using cardiac magnetic resonance imaging. Methods— We prospectively included a consecutive sample of ischemic stroke patients. Patients with structural changes on echocardiography currently considered as causal for stroke in the Trial of ORG 10172 in Acute Stroke Treatment (TOAST) classification were excluded. A 3-T cardiac magnetic resonance imaging was performed. Results— One hundred and eleven patients were evaluated. Patients with an undetermined cause had a higher percentage of LA fibrosis (P=0.03) than patients with other stroke causes and lower, although not statistically significant, values of LA ejection fraction. Patients with atrial fibrillation and undetermined stroke cause showed a similar value of atrial fibrosis. Conclusions— The LA phenotype that was found in patients with undetermined cause supports the hypothesis that an atrial disease may be associated with stroke.

Axial myoclonus after ischemic stroke

Myoclonus is an unusual manifestation of stroke.1 We report a 67-year-old woman admitted with an acute ischemic stroke in the right thalamo-capsular and hippocampal areas (figure). Becoming upright produced marked postural instability, due to an apparent reduction in cervical and dorsal axial muscle tone. EMG disclosed negative myoclonus in left upper limb and right paraspinal muscles, and both negative and positive myoclonus in left paraspinal muscles (video on the Neurology® Web site at Neurology.org). No epileptic activity was observed on EEG.

Voluntary control of a plegic limb during yawning

Yawning is a phylogenetically ancient phenomenon coordinated by a network of supratentorial and infratentorial centres located in brainstem, hypothalamus, and limbic regions [1]. Movements of plegic limbs during yawning in patients with stroke are common [2]. The first descriptions come from the 18th and 19th centuries by Darwin and by Abercrombie, respectively [3, 4]. These movements have always been described as involuntary and stereotyped, however [5]. We report a novel phenomenon: a stroke patient who could voluntarily control the movements of his plegic limb during yawning. A 59-year-old right-handed male was admitted in the emergency department presenting right facial palsy, dysarthria, right hemiplegia, and right pain anesthesia of sudden onset. The relevant medical antecedents were diabetes mellitus and arterial hypertension. The presentation suggested a sensorimotor lacunar syndrome due to a subcortical stroke. The diagnosis of acute left Middle Cerebral Artery infarct was made and he was submitted to endovenous thrombolysis. The inferior limb deficit improved; however, the right superior limb remained plegic—grade 0 (MRC scale) in all segments, including abduction/adduction, flexion/extension, and medial/lateral rotation of the shoulder and flexion/extension and supination/pronation of the elbow. The only exception was the elevation of the shoulder, in which the patient could perform a full range of motion if the force gravity was eliminated (grade 2). Since the first hours of stroke onset, the patient presented a reflexive, stereotyped movement of flexion of the right elbow while yawing. Remarkably, at day 6, the patient started to have voluntary control over that movement. Ability to touch the chin or the left arm with the right hand according to the request of the observer was documented (Online Resource 1). The patient noticed an association between the duration of yawning and the range of movements that he was able to do, such that during more sustained yawning, he could even perform more complex movements of distal joints—grabbing objects purposely, for instance. These movements could also be volitionally suppressed. They were impossible without yawning or when he simulated yawning. He did not notice an increase in the frequency of yawning, fatigue of the movement during multiple episodes of yawning, and his limb was not spastic. MRI showed an infarct involving lenticular nucleus, anterior and posterior limbs of internal capsule, body of caudate nucleus, and corona radiata (Fig. 1a). Tractography and transcranial magnetic stimulation confirmed the disruption on the left pyramidal tract (Fig. 2). The topography of his lesion was compared to controls. The controls were selected prospectively and had: (a) an acute ischemic lesion on brain MRI of the left lenticulocapsulo-radiate region; (b) an upper limb paresis ≤ 2; (c) yawned during admission. Of the four controls selected, two did not present any movement during yawning and two presented involuntary movements exclusively. Brain images were normalized to MNI152 space. The volume of lesion of the case was 8461 mm3 and that of the controls ranged from 3747 to 9858 mm3. The overlay of lesions of case and controls showed that some areas of Electronic supplementary material The online version of this article (http s://doi.org/10.1007 /s004 15-017-8729 -z) contains supplementary material, which is available to authorized users.

Loss of object ownership feeling following a left hemisphere infarct.

Pedro Nascimento Alves , Sofia Reim~ ao , Catarina Campos , Carolina Maruta , Luz Rocha , Graça S a , Ana Catarina Fonseca a and Isabel Pav~ ao Martins c a Department of Neurosciences and Mental Health, Neurology, Hospital de Santa Maria, CHLN, Lisbon, Portugal b Department of Neuroradiology, Hospital de Santa Maria, CHLN, Lisbon, Portugal c Lisbon Faculty of Medicine, Language Research Laboratory, Lisbon, Portugal

Laterality of Pneumonia in Acute Stroke.

Background/Aims: Previous studies demonstrated an alteration of diaphragmatic excursion on the paretic side after stroke; however, it is unclear if this change has clinical repercussions. We aimed to determine if there was an association between the paretic side and the laterality of pneumonia after stroke. Methods: A retrospective analysis of a consecutive cohort of patients admitted to a stroke unit from 2008 to May 2016 was performed. Patients with the diagnosis of acute stroke and pneumonia were included. The laterality of pneumonia was determined through the blinded observation of chest X-rays. Fishers exact test was applied to study the association between the side of paresis and pneumonia. Results: One hundred and five patients were included. Sixty one percent (n = 64) had an ischemic stroke, 39% (n = 41) had brain hemorrhage, and 49.5% (n = 52) had right side paresis. We did not find in general an association between the side of paresis and the side of pneumonia (p = 1.00); however, we found a statistically significant association in patients with severe lower limb paresis (Medical Research Council, MRC ≤2; p = 0.035). Conclusion: We found an association between severe paresis of the lower limb (MRC ≤2) and ipsilateral pneumonia. We hypothesize that the proximity between the diaphragmatic and inferior limb corticospinal pathways could be the reason for this association.