The Experts below are selected from a list of 321 Experts worldwide ranked by ideXlab platform
Yoshihiro Maegaki - One of the best experts on this subject based on the ideXlab platform.
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Case Report Stimulus-induced reflex epileptic Spasms in 5p syndrome
2016Co-Authors: Kentaro Shirai, Yoshiaki Saito, Atushi Yokoyama, Yoko Nishimura, Akiko Tamasaki, Yoshihiro MaegakiAbstract:Here we describe two patients with 5p syndrome who suffered from epilepsy characterised by stimulus-induced epileptic Spasms manifesting as head nodding. In patient 1, a series of Spasms were exclusively triggered by eating, and were associated with diffuse high-voltage slow waves on ictal EEG, particularly presenting as a positive slow potential at the left mid-temporal area. Clusters of sharp waves with negative polarity emerged in the same area during the inter-Spasm periods during eating. In patient 2, Spasms were provoked by either eating or micturition. Ictal EEG of clustered Spasms after micturition showed positive slow or triphasic waves, which correlated with each Spasm, over the bifrontal and vertex areas. These findings suggest that the focal cortical areas act as trigger regions in reflex epilepsies, and that a Spasm-generator responsible for the execution of reflex Spasms exists either in other cortical areas or in the subcortical structures. Although epilepsy is an unusual complication of 5p syndrome, this syndrome may have a propensity to develop reflex epilepsy, particularly epileptic Spasms. However, identification of responsible genes and their roles in this phenotype requires further investigations.
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Stimulus-induced reflex epileptic Spasms in 5p- syndrome.
Brain & development, 2015Co-Authors: Kentaro Shirai, Yoshiaki Saito, Atushi Yokoyama, Yoko Nishimura, Akiko Tamasaki, Yoshihiro MaegakiAbstract:Abstract Here we describe two patients with 5p− syndrome who suffered from epilepsy characterised by stimulus-induced epileptic Spasms manifesting as head nodding. In patient 1, a series of Spasms were exclusively triggered by eating, and were associated with diffuse high-voltage slow waves on ictal EEG, particularly presenting as a positive slow potential at the left mid-temporal area. Clusters of sharp waves with negative polarity emerged in the same area during the inter-Spasm periods during eating. In patient 2, Spasms were provoked by either eating or micturition. Ictal EEG of clustered Spasms after micturition showed positive slow or triphasic waves, which correlated with each Spasm, over the bifrontal and vertex areas. These findings suggest that the focal cortical areas act as trigger regions in reflex epilepsies, and that a Spasm-generator responsible for the execution of reflex Spasms exists either in other cortical areas or in the subcortical structures. Although epilepsy is an unusual complication of 5p− syndrome, this syndrome may have a propensity to develop reflex epilepsy, particularly epileptic Spasms. However, identification of responsible genes and their roles in this phenotype requires further investigations.
Giovanni Fabbrini - One of the best experts on this subject based on the ideXlab platform.
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Spread of Muscle Spasms in Hemifacial Spasm.
Movement Disorders Clinical Practice, 2014Co-Authors: Antonella Conte, Marika Falla, Maria Concetta Diana, Matteo Bologna, Antonio Suppa, Andrea Fabbrini, Carlo Colosimo, Alfredo Berardelli, Giovanni FabbriniAbstract:Hemifacial Spasm (HFS) is a clinical condition characterized by involuntary contractions in facial muscles. The aim of the study was to investigate, systematically in 178 patients with HFS, the frequency of spread from the site of origin to other facial muscles. Patients enrolled underwent a complete neurological examination and a face-to-face interview. Spread of the Spasm to other facial muscles was considered to be present in those patients whose Spasms onset in a single site and involved both upper and lower facial muscles at the time of examination. We also collected information about gender, age, age at HFS onset, symptom duration, muscles involved by the Spasm at the time of onset, and spread of Spasm to other facial muscles. Spread of Spasms to the other facial muscles of the same side of the face was present in 93.4% of patients with HFS, and latency of spread was related to disease duration and age at onset. In patients with HFS, spread of muscle Spasms represents the natural history of HFS.
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tactile temporal discrimination in patients with blepharoSpasm
Journal of Neurology Neurosurgery and Psychiatry, 2008Co-Authors: Mirta Fiorio, Giovanni Fabbrini, Michele Tinazzi, Alessandra Scontrini, Clementina Stanzani, Mattia Gambarin, Antonio Fiaschi, Giuseppe Moretto, Alfredo BerardelliAbstract:Background: BlepharoSpasm is an adult-onset focal dystonia that causes involuntary blinking and eyelid Spasms. Studies have shown the presence of sensory deficits associated with dystonia. Aim: To rule out any confounding effect of muscle Spasms on sensory performance in affected and unaffected body regions of patients with blepharoSpasm and with hemifacial Spasm. Methods: Participants (19 patients with blepharoSpasm, 19 patients with hemifacial Spasm and 19 control subjects) were asked to discriminate between two stimuli that were either simultaneous or sequential (temporal discrimination threshold, TDT). Pairs of tactile stimuli were delivered with increasing or decreasing inter-stimulus intervals from 0 to 400 ms (in 10-ms steps) to the hands or on the skin over the orbicularis oculi muscle. Results: Tactile stimuli elicited similar TDTs in control subjects and patients with hemifacial Spasm, but significantly higher TDTs in patients with blepharoSpasm, regardless of whether stimuli were applied to the orbicularis muscle or the hand. Conclusions: As TDT was abnormal in unaffected body regions of patients with blepharoSpasm, and patients with hemifacial Spasm processed tactile stimuli normally, TDT deficits in blepharoSpasm depend on central rather than peripheral factors. This study further supports the link between focal dystonia and impaired temporal processing of somatosensory inputs.
Kentaro Shirai - One of the best experts on this subject based on the ideXlab platform.
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Case Report Stimulus-induced reflex epileptic Spasms in 5p syndrome
2016Co-Authors: Kentaro Shirai, Yoshiaki Saito, Atushi Yokoyama, Yoko Nishimura, Akiko Tamasaki, Yoshihiro MaegakiAbstract:Here we describe two patients with 5p syndrome who suffered from epilepsy characterised by stimulus-induced epileptic Spasms manifesting as head nodding. In patient 1, a series of Spasms were exclusively triggered by eating, and were associated with diffuse high-voltage slow waves on ictal EEG, particularly presenting as a positive slow potential at the left mid-temporal area. Clusters of sharp waves with negative polarity emerged in the same area during the inter-Spasm periods during eating. In patient 2, Spasms were provoked by either eating or micturition. Ictal EEG of clustered Spasms after micturition showed positive slow or triphasic waves, which correlated with each Spasm, over the bifrontal and vertex areas. These findings suggest that the focal cortical areas act as trigger regions in reflex epilepsies, and that a Spasm-generator responsible for the execution of reflex Spasms exists either in other cortical areas or in the subcortical structures. Although epilepsy is an unusual complication of 5p syndrome, this syndrome may have a propensity to develop reflex epilepsy, particularly epileptic Spasms. However, identification of responsible genes and their roles in this phenotype requires further investigations.
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Stimulus-induced reflex epileptic Spasms in 5p- syndrome.
Brain & development, 2015Co-Authors: Kentaro Shirai, Yoshiaki Saito, Atushi Yokoyama, Yoko Nishimura, Akiko Tamasaki, Yoshihiro MaegakiAbstract:Abstract Here we describe two patients with 5p− syndrome who suffered from epilepsy characterised by stimulus-induced epileptic Spasms manifesting as head nodding. In patient 1, a series of Spasms were exclusively triggered by eating, and were associated with diffuse high-voltage slow waves on ictal EEG, particularly presenting as a positive slow potential at the left mid-temporal area. Clusters of sharp waves with negative polarity emerged in the same area during the inter-Spasm periods during eating. In patient 2, Spasms were provoked by either eating or micturition. Ictal EEG of clustered Spasms after micturition showed positive slow or triphasic waves, which correlated with each Spasm, over the bifrontal and vertex areas. These findings suggest that the focal cortical areas act as trigger regions in reflex epilepsies, and that a Spasm-generator responsible for the execution of reflex Spasms exists either in other cortical areas or in the subcortical structures. Although epilepsy is an unusual complication of 5p− syndrome, this syndrome may have a propensity to develop reflex epilepsy, particularly epileptic Spasms. However, identification of responsible genes and their roles in this phenotype requires further investigations.
Joseph Jankovic - One of the best experts on this subject based on the ideXlab platform.
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the many faces of hemifacial Spasm differential diagnosis of unilateral facial Spasms
Movement Disorders, 2011Co-Authors: Toby C Yaltho, Joseph JankovicAbstract:Hemifacial Spasm is defined as unilateral, involuntary, irregular clonic or tonic movement of muscles innervated by the seventh cranial nerve. Most frequently attributed to vascular loop compression at the root exit zone of the facial nerve, there are many other etiologies of unilateral facial movements that must be considered in the differential diagnosis of hemifacial Spasm. The primary purpose of this review is to draw attention to the marked heterogeneity of unilateral facial Spasms and to focus on clinical characteristics of mimickers of hemifacial Spasm and on atypical presentations of nonvascular cases. In addition to a comprehensive review of the literature on hemifacial Spasm, medical records and videos of consecutive patients referred to the Movement Disorders Clinic at Baylor College of Medicine for hemifacial Spasm between 2000 and 2010 were reviewed, and videos of illustrative cases were edited. Among 215 patients referred for evaluation of hemifacial Spasm, 133 (62%) were classified as primary or idiopathic hemifacial Spasm (presumably caused by vascular compression of the ipsilateral facial nerve), and 4 (2%) had hereditary hemifacial Spasm. Secondary causes were found in 40 patients (19%) and included Bell's palsy (n = 23, 11%), facial nerve injury (n = 13, 6%), demyelination (n = 2), and brain vascular insults (n = 2). There were an additional 38 patients (18%) with hemifacial Spasm mimickers classified as psychogenic, tics, dystonia, myoclonus, and hemimasticatory Spasm. We concluded that although most cases of hemifacial Spasm are idiopathic and probably caused by vascular compression of the facial nerve, other etiologies should be considered in the differential diagnosis, particularly if there are atypical features. © 2011 Movement Disorder Society
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Psychogenic hemifacial Spasm.
The Journal of neuropsychiatry and clinical neurosciences, 2001Co-Authors: Eng-king Tan, Joseph JankovicAbstract:Facial Spasms that distort facial expression are typically due to facial dystonia, tics, and hemifacial Spasm (HFS). Psychogenic facial Spasms, however, have not been well characterized. The authors sought to 1) determine prevalence of psychogenic facial Spasm in patients referred for evaluation of HFS and 2) draw attention to clinical characteristics and potential diagnostic pitfalls. Among 210 consecutive patients referred for evaluation of HFS, 5 (2.4%) received diagnoses of psychogenic facial Spasm. All patients were female; mean age was 34.6 years (range 26-45) and mean symptom duration 1.1 years (range 2 wk-2 yr). Onset was left-sided in 3 patients, and the lid was the initial site affected in 2 patients. This series of patients shows that facial Spasms, although usually of neurovascular etiology, may be the initial or only manifestation of a psychogenic movement disorder, often associated with an underlying depression.
Hiroaki Kohno - One of the best experts on this subject based on the ideXlab platform.
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Differential incidence and morphology of provoked Spasm between intracoronary acetylcholine and ergonovine testing: recommendation of supplementary use
Heart and Vessels, 2019Co-Authors: Shozo Sueda, Yasuhiro Sasaki, Tomoki Sakaue, Hirokazu Habara, Kaori Fujimoto, Toyofumi Yoshii, Hiroaki KohnoAbstract:When cardiologists diagnose patients with coronary spastic angina, Japanese Circulation Society (JCS) guidelines recommend the intracoronary injection of acetylcholine (ACh) and ergonovine (ER) as class I. However, the pharmacological difference between ACh and ER is controversial in the clinic. We performed both ACh and ER tests in the same 528 patients during 26 years. We investigated the provoked Spasm configuration, Spasm site, and clinical characteristics of provoked Spasm between ACh and ER, retrospectively. We defined positive Spasm as ≥90% luminal narrowing. Provoked positive Spasm was observed in 161 right coronary arteries (RCA) including 83 ACh just positive, 35 ER just positive, and 43 both positive. In contrast, positive Spasm was documented in 172 left coronary arteries (LCA) including 94 ACh just positive, 28 ER just positive, and 50 both positive. ACh provoked Spasm more distally and diffusely, while ER induced Spasm more proximally and totally or focally in the RCA. In the LCA, ACh provoked Spasm more proximally, whereas ER induced Spasm more distally. ER testing after the negative ACh tests of RCA and LCA documented new positive Spasms in 10.3% (35/340) and 7.4% (28/376), respectively. Coronary artery trees may each have a sensitive receptor on each segment. We recommend the supplementary use of ACh and ER to document coronary artery Spasm in the cardiac catheterization laboratory.
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Differential Incidence and Morphology of Spasm According to Coronary Arterial Location by Intracoronary Ergonovine Spasm Provocation Testing.
Circulation journal : official journal of the Japanese Circulation Society, 2017Co-Authors: Shozo Sueda, Hiroaki KohnoAbstract:BACKGROUND We reported less provoked Spasm in the left circumflex artery (LCX) by acetylcholine testing compared with the left anterior descending artery (LAD) and right coronary artery (RCA), so we investigated the clinical characteristics of provoked Spasm in the LCX by ergonovine (ER) testing.Methods and Results:We retrospectively analyzed 1,185 consecutive cases of intracoronary ER testing during 25 years. Maximal ER dose was 64 μg into the left coronary artery (LCA) and 40 μg into the RCA. Positive Spasm was defined as a transient ≥90% narrowing and usual chest symptoms or ischemic ECG changes. Positive provoked Spasm was recognized in 347 patients (29.3%), including 207 RCA Spasms, 166 LAD Spasms, and 79 LCX Spasms. Spasm was provoked in the LCX significantly less than in the other vessels (P
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overview of the pharmacological Spasm provocation test comparisons between acetylcholine and ergonovine
Journal of Cardiology, 2017Co-Authors: Shozo Sueda, Tadao Uraoka, Hiroaki Kohno, Takaaki Ochi, Kensuke TsunemitsuAbstract:The Spasm provocation tests of ergonovine and acetylcholine have been employed in the cardiac catheterization laboratory. Ergonovine acts through the serotogenic receptors, while acetylcholine acts through the muscarinic cholinergic receptors. Different mediators may have the potential to cause different coronary responses. However, there are few reports concerning the coronary response between ergonovine and acetylcholine in the same patients. Acetylcholine is supersensitive for females; Spasm provoked by ergonovine is focal and proximal, whereas provoked Spasm by acetylcholine is diffuse and distal. We should use both tests as supplementary in the clinic because ergonovine and acetylcholine have self-limitations to induce coronary Spasms during daily life. The maximal pharmacological doses, administration methods, and the angiographical positive definition are remarkably different for each institution in the world. We recommend the pharmacological Spasm provocation tests as Class I in the guidelines in patients with vasospastic angina throughout the world.
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safety and optimal protocol of provocation test for diagnosis of multivessel coronary Spasm
Heart and Vessels, 2016Co-Authors: Shozo Sueda, Toru Miyoshi, Yasuhiro Sasaki, Tomoki Sakaue, Hirokazu Habara, Hiroaki KohnoAbstract:We examined the safety of acetylcholine (ACh) and ergonovine (ER) tests retrospectively and investigated the optimal protocol of provocation test for diagnosis of multivessel coronary Spasm. We performed 1546 ACh tests and 1114 ER tests during 23 years. ACh was injected in incremental doses of 20/50/80 μg into the right coronary artery (RCA) and of 20/50/100/200 μg into the left coronary artery (LCA) over 20 s. ER was administered in total doses of 40 μg into the RCA and of 64 μg into the LCA over 2–4 min. When a coronary Spasm was induced and did not resolve spontaneously within 3 min after the completion of ACh/ER injection, or when hemodynamic instability due to coronary Spasms occurred, 2.5–5.0 mg of nitrate was administered into the responsible vessel. To relive provoked Spasm, it is necessary to administer nitrate in 31 cases by ACh and in 76 cases by ER (2.0 vs. 6.8 %, p < 0.01) before another vessel attempts. Multivessel Spasms were often observed in LCA testing than in RCA testing on both agents [ACh: 78.6 % (11/14) vs. 11.8 % (2/17), p < 0.001, ER: 37.8 % (14/37) vs. 20.5 % (8/39), ns]. Even after the administration of nitrates, positive coronary Spasm was obtained in 21.1 % by ACh and 52.9 % by ER tests on another coronary artery. No irreversible complications were recognized on both tests. We should firstly perform Spasm provocation tests in the LCA and we may be able to diagnose another vessel Spasm by performing the complete Spasm provocation tests after the administration of nitrates to relieve provoked Spasm in the first attempt.
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Coronary flow reserve in patients with vasospastic angina: correlation between coronary flow reserve and age or duration of angina
Coronary Artery Disease, 2003Co-Authors: Shozo Sueda, Hiroaki Kohno, Hiroshi Fukuda, Tadao UraokaAbstract:Objectives This study sought to assess the coronary flow reserve (CFR) in patients with pure vasospastic angina (VSA). Methods and results The phasic flow velocities of both Spasm-positive and Spasm-negative coronary arteries of the left anterior descending artery (LAD) were recorded at rest and during hyperaemia (50 μg of adenosine triphosphate infusion intracoronary) using a 0.014 inch, 15 MHz Doppler guide wire in 42 patients with pure VSA and acetylcholine (ACh)-induced coronary artery Spasms (20-100 μg), and 23 controls with normal coronary arteries without ACh-induced vasoSpasm. These 42 patients had 16 vessels with focal Spasms (>99%), 17 vessels with diffuse Spasms (>90%) in the LAD, and nine vessels with ACh-induced Spasms In the right coronary artery, but not the LAD. Coronary flow reserve was obtained from the ratio of the hyperaemic/baseline time-averaged peak velocity. Coronary flow reserve did not differ between patients with VSA and the controls (2.9 ′ 0.8 versus 3.2 ′ 0.7, NS). Moreover, CFR did not differ among the four cases (focal: 2.8 ′ 0.7; diffuse: 3.0 ′ 0.9; non Spasm: 2.9 ′ 0.7 versus controls: 3.2 + 0.7, respectively, NS). Coronary flow reserve in vessels with proximal Spasms was significantly higher than that in vessels with mid or distal Spasms (3.4 ′ 0.8 versus 2.6 ′ 0.6, 2.6 ′ 0.9, p
