Myoclonus

2016 ◽  
Author(s):  
Hiroshi Shibasaki ◽  
Marina A J Koning-Tijssen
Keyword(s):  
Spinal Cord ◽  
Key Words ◽  
Loss Of Consciousness ◽  
Voluntary Movements ◽  
Involuntary Movements ◽  
Cortical Myoclonus ◽  
Progressive Myoclonus Epilepsy ◽  
Myoclonus Epilepsy ◽  
Jakob Disease

Myoclonus is defined as sudden, brief, jerky, shocklike, involuntary movements involving the extremities, face, and trunk, without loss of consciousness. Myoclonus is one of the most commonly encountered involuntary movements, and it can be seen in association with a variety of conditions. This review covers  the epidemiology, classification, and diagnosis of myoclonus, as well as a thorough discussion of cortical myoclonus, myoclonus of brainstem origin, myoclonus of spinal cord origin, and myoclonus of undetermined origin. Figures show the principle of jerk-locked back-averaging, example data of jerk-lock averaging, schematic diagrams of cortical reflex myoclonus and spontaneous cortical myoclonus, a polygraphic electromyogram (EMG) recorded from a patient with postanoxic, reticular reflex myoclonus, surface negative slow electroencephalographic (EEG) potentials recorded before psychogenic jerks and voluntary movements mimicking the jerks in a patient with a diagnosis of psychogenic truncal movements, and an EEG-EMG polygraph in a patient with Creutzfeldt-Jakob disease. Tables list the classification of myoclonus based on the estimated site of origin, causes of cortical myoclonus, and causes of progressive myoclonus epilepsy and gene abnormalities.   Key words: cortical myoclonus, EEG-EMG polygraph, epilepsy syndromes, involuntary movements, myoclonus   This review contains 6 highly rendered figures, 3 tables, and 63 references.

Myoclonus

2016 ◽  
Author(s):  
Hiroshi Shibasaki ◽  
Marina A J Koning-Tijssen
Keyword(s):  
Spinal Cord ◽  
Key Words ◽  
Loss Of Consciousness ◽  
Voluntary Movements ◽  
Involuntary Movements ◽  
Cortical Myoclonus ◽  
Progressive Myoclonus Epilepsy ◽  
Myoclonus Epilepsy ◽  
Jakob Disease

Myoclonus is defined as sudden, brief, jerky, shocklike, involuntary movements involving the extremities, face, and trunk, without loss of consciousness. Myoclonus is one of the most commonly encountered involuntary movements, and it can be seen in association with a variety of conditions. This review covers  the epidemiology, classification, and diagnosis of myoclonus, as well as a thorough discussion of cortical myoclonus, myoclonus of brainstem origin, myoclonus of spinal cord origin, and myoclonus of undetermined origin. Figures show the principle of jerk-locked back-averaging, example data of jerk-lock averaging, schematic diagrams of cortical reflex myoclonus and spontaneous cortical myoclonus, a polygraphic electromyogram (EMG) recorded from a patient with postanoxic, reticular reflex myoclonus, surface negative slow electroencephalographic (EEG) potentials recorded before psychogenic jerks and voluntary movements mimicking the jerks in a patient with a diagnosis of psychogenic truncal movements, and an EEG-EMG polygraph in a patient with Creutzfeldt-Jakob disease. Tables list the classification of myoclonus based on the estimated site of origin, causes of cortical myoclonus, and causes of progressive myoclonus epilepsy and gene abnormalities. Key words: cortical myoclonus, EEG-EMG polygraph, epilepsy syndromes, involuntary movements, myoclonus This review contains 6 highly rendered figures, 3 tables, and 63 references.

FAMILIAL PAROXYSMAL CHOREA-ATHETOSIS

PEDIATRICS ◽  
1963 ◽  
Vol 31 (4) ◽  
pp. 656-659
Author(s):  
Jonathan Williams ◽  
Harold Stevens
Keyword(s):  
Loss Of Consciousness ◽  
Voluntary Movements ◽  
Involuntary Movements ◽  
Neurological Findings ◽  
Metabolic Defect ◽  
Leg Movements

A patient with familial paroxysmal chorea-athetosis is described. Analysis of the sequential evolution and pattern of the involuntary movements was facilitated by study of movies taken during induced attacks. Attacks were evoked by having the patient sit immobile and then suddenly rise and walk briskly, and also by hyperventilation. Crossing of hen legs or other voluntary movements minimized the likelihood of an attack. Typically, there was no loss of consciousness, neurological findings were negative, and intellectual faculties were unimpaired. Brief, single, and limited movements of the legs were observed in our patient between attacks. This feature had not been described in the two prior reports of this rare hereditable syndrome. It is suggested that a metabolic defect may underlie the pathogenesis. Diphenylhydantoin (Dilantin) reduced the attacks, but not the leg movements.

EFFICACY OF ENDOSCOPIC HEMOCLIPS AND INTERMITTENT INTRAVENOUS PPI IN PEPTIC ULCER BLEEDING

2019 ◽  
pp. 40-44
Author(s):  
Van Huy Tran ◽  
Duy Lieu Dinh
Keyword(s):  
Peptic Ulcer ◽  
Key Words ◽  
Proton Pump Inhibitors ◽  
Proton Pump ◽  
Ulcer Bleeding ◽  
Peptic Ulcer Bleeding ◽  
Forrest Classification

Background: Efficacy of continuous intravenous proton- pump inhibitors (IV PPI) and hemoclips alone was proved, but data about combination of an application of endoscopy clips and intermittent IV PPI in Vietnam was still limited. This study aimed to assess the efficacy of endoscopy hemoclip combined with intermittent IV PPI in the patients of peptic ulcer bleeding. Patients and methods: 34 patients diagnosed as peptic ulcer bleeding, having Forrest classification of Ia, Ib, IIa and IIb, were enrolled. Esomeprazole was administered as 80 mg IV bolus followed by intermittent IV injection of 40 mg/8h during 72h. Results: Immediate hemostasis was achieved in all 34 patients. Only 1 patient (2.9%) had early rebleeding. No severe complications was found in this study. Conclusion: Combination of endoscopy hemoclips and intermittent PPI showed effective, safe in patients of peptic ulcer bleeding. Key words: Peptic ulcer bleeding, intermittent PPI, endoscopy hemoclip

FEATURES OF OPHTHALMIC CARE FOR PATIENTS WITH COMPLICATED CATARACTS

2020 ◽  
pp. 84-89
Author(s):  
Inna Ivanovna Lapkina
Keyword(s):  
Health Care ◽  
Surgical Treatment ◽  
Postoperative Complications ◽  
Key Words ◽  
Outpatient Treatment ◽  
Practical Application ◽  
Unified Approach ◽  
Case Histories ◽  
High Prevalence

Today, around 50 million people worldwide suffer from cataracts, more than a half of them need surgical treatment. High prevalence of this pathology in Ukraine, the need to improve the provision of ophthalmic care to patients, and the reform of the health care system have made the research relevant. Concomitant diseases and special conditions of the eye increase the risk of intra− and postoperative complications, worsen the functional parameters of patients after surgery. In order to develop a unified approach to the treatment of complicated cataracts based on diagnostically related groups of patients, a retrospective analysis of case histories of patients with different variants of complications related to the condition of the lens itself, its ligament apparatus and other structures of the eye was conducted. In each case, the surgeon has to choose the appropriate modification of cataract phacoemulsification surgery. The study proposed the classification of cataract phacoemulsification modifications on the basis of the techniques and the sequence of operation stages, taking into account the classification of the degrees of turbidity of the lens, proposed by L. Buratto. It has been noted that in complicated cases, according to the indications of the patient, surgery may be performed on several modifications of cataract phacoemulsification. The developed classification made it possible to generalize the various variants of pathology and greatly facilitate the choice of tactics of surgical treatment in complicated cataracts. It can be used not only for practical application, but also for improving the qualification of trained professionals. The prospect of further research is to identify contraindications for outpatient treatment of the patients with complicated cataracts. Key words: cataract complication, classification of phacoemulsification modifications, diagnostically related groups.

scholarly journals Sensitive protein misfolding cyclic amplification of sporadic Creutzfeldt–Jakob disease prions is strongly seed and substrate dependent

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Maxime Bélondrade ◽  
Simon Nicot ◽  
Charly Mayran ◽  
Lilian Bruyere-Ostells ◽  
Florian Almela ◽  
...  
Keyword(s):  
Prion Protein ◽  
Protein Misfolding ◽  
Protein Misfolding Cyclic Amplification ◽  
Bank Voles ◽  
Substrate Dependence ◽  
Brain Extracts ◽  
Jakob Disease ◽  
Human Prion Protein

AbstractUnlike variant Creutzfeldt–Jakob disease prions, sporadic Creutzfeldt–Jakob disease prions have been shown to be difficult to amplify in vitro by protein misfolding cyclic amplification (PMCA). We assessed PMCA of pathological prion protein (PrPTSE) from 14 human sCJD brain samples in 3 substrates: 2 from transgenic mice expressing human prion protein (PrP) with either methionine (M) or valine (V) at position 129, and 1 from bank voles. Brain extracts representing the 5 major clinicopathological sCJD subtypes (MM1/MV1, MM2, MV2, VV1, and VV2) all triggered seeded PrPTSE amplification during serial PMCA with strong seed- and substrate-dependence. Remarkably, bank vole PrP substrate allowed the propagation of all sCJD subtypes with preservation of the initial molecular PrPTSE type. In contrast, PMCA in human PrP substrates was accompanied by a PrPTSE molecular shift during heterologous (M/V129) PMCA reactions, with increased permissiveness of V129 PrP substrate to in vitro sCJD prion amplification compared to M129 PrP substrate. Combining PMCA amplification sensitivities with PrPTSE electrophoretic profiles obtained in the different substrates confirmed the classification of 4 distinct major sCJD prion strains (M1, M2, V1, and V2). Finally, the level of sensitivity required to detect VV2 sCJD prions in cerebrospinal fluid was achieved.

scholarly journals Altered Expression of Peroxiredoxins in Mouse Model of Progressive Myoclonus Epilepsy upon LPS-Induced Neuroinflammation

Antioxidants ◽  
2021 ◽  
Vol 10 (3) ◽  
pp. 357
Author(s):  
Mojca Trstenjak Prebanda ◽  
Petra Matjan-Štefin ◽  
Boris Turk ◽  
Nataša Kopitar-Jerala
Keyword(s):  
Mouse Model ◽  
Redox Homeostasis ◽  
Underlying Mechanism ◽  
Loss Of Function ◽  
Altered Expression ◽  
Lps Challenge ◽  
Progressive Myoclonus Epilepsy ◽  
Myoclonus Epilepsy ◽  
The Brain ◽  
Deficient Mice

Stefin B (cystatin B) is an inhibitor of endo-lysosomal cysteine cathepsin, and the loss-of-function mutations in the stefin B gene were reported in patients with Unverricht–Lundborg disease (EPM1), a form of progressive myoclonus epilepsy. Stefin B-deficient mice, a mouse model of the disease, display key features of EPM1, including myoclonic seizures. Although the underlying mechanism is not yet completely clear, it was reported that the impaired redox homeostasis and inflammation in the brain contribute to the progression of the disease. In the present study, we investigated if lipopolysaccharide (LPS)-triggered neuroinflammation affected the protein levels of redox-sensitive proteins: thioredoxin (Trx1), thioredoxin reductase (TrxR), peroxiredoxins (Prxs) in brain and cerebella of stefin B-deficient mice. LPS challenge was found to result in a marked elevation of Trx1 and TrxR in the brain and cerebella of stefin B deficient mice, while Prx1 was upregulated only in cerebella after LPS challenge. Mitochondrial peroxiredoxin 3 (Prx3), was upregulated also in the cerebellar tissue lysates prepared from unchallenged stefin B deficient mice, while after LPS challenge Prx3 was upregulated in stefin B deficient brain and cerebella. Our results imply the role of oxidative stress in the progression of the disease.

Classification of involuntary movements in dogs: Tremors and twitches

2016 ◽  
Vol 214 ◽  
pp. 109-116 ◽  
Author(s):  
Mark Lowrie ◽  
Laurent Garosi
Keyword(s):  
Involuntary Movements

Split spinal cord malformations in children

1998 ◽  
Vol 88 (1) ◽  
pp. 57-65 ◽  
Author(s):  
Yusuf Ersşahin ◽  
Saffet Mutluer ◽  
Sevgül Kocaman ◽  
Eren Demirtasş
Keyword(s):  
Spinal Cord ◽  
Neurological Deficits ◽  
Single Type ◽  
Type I ◽  
Type Ii ◽  
Content Type ◽  
Spinal Lesion ◽  
The Mean ◽  
Fine Print

Object. The authors reviewed and analyzed information on 74 patients with split spinal cord malformations (SSCMs) treated between January 1, 1980 and December 31, 1996 at their institution with the aim of defining and classifying the malformations according to the method of Pang, et al. Methods. Computerized tomography myelography was superior to other radiological tools in defining the type of SSCM. There were 46 girls (62%) and 28 boys (38%) ranging in age from less than 1 day to 12 years (mean 33.08 months). The mean age (43.2 months) of the patients who exhibited neurological deficits and orthopedic deformities was significantly older than those (8.2 months) without deficits (p = 0.003). Fifty-two patients had a single Type I and 18 patients a single Type II SSCM; four patients had composite SSCMs. Sixty-two patients had at least one associated spinal lesion that could lead to spinal cord tethering. After surgery, the majority of the patients remained stable and clinical improvement was observed in 18 patients. Conclusions. The classification of SSCMs proposed by Pang, et al., will eliminate the current chaos in terminology. In all SSCMs, either a rigid or a fibrous septum was found to transfix the spinal cord. There was at least one unrelated lesion that caused tethering of the spinal cord in 85% of the patients. The risk of neurological deficits resulting from SSCMs increases with the age of the patient; therefore, all patients should be surgically treated when diagnosed, especially before the development of orthopedic and neurological manifestations.

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