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1 the VGKCC syndrome, had been misdiagnosed as myoclonus.
2 gressive encephalomyelitis with rigidity and myoclonus.
3 gressive encephalomyelitis with rigidity and myoclonus.
4 gressive encephalomyelitis with rigidity and myoclonus.
5 lear envelopes, alteration in locomotion, or myoclonus.
6 mice exhibit motor deficits and spontaneous myoclonus.
7 es not produce abnormal nuclear envelopes or myoclonus.
8 otentials indicated a cortical origin of the myoclonus.
9 t, slowly progressive, disabling, multifocal myoclonus.
10 xia, cortical sensory deficits, dystonia and myoclonus.
11 gressive encephalomyelitis with rigidity and myoclonus.
12 ellum is the underlying cause of posthypoxic myoclonus.
13 n of myoclonus in a rat model of posthypoxic myoclonus.
14 were evaluated in a rat model of posthypoxic myoclonus.
15 inds a precedent in the syndrome of cortical myoclonus.
16 d in an established rat model of posthypoxic myoclonus.
17 ain nuclei possibly involved in post-hypoxic myoclonus.
18 at underwent 4 min of cardiac arrest without myoclonus.
19 LF plays a contributing role in post-hypoxic myoclonus.
20 ma-hydroxybutyric acid for alcohol-sensitive myoclonus.
21 t may have contributed to the development of myoclonus.
22 oherence evident between signals in cortical myoclonus.
23 may be exaggerated in patients with cortical myoclonus.
24 nge of frequencies in patients with cortical myoclonus.
25 analyzed movement behavior in a patient with myoclonus.
26 e motor areas producing the typical regional myoclonus.
27 wever, with intact RRN and vMPJ never showed myoclonus.
28 ctivation of NMDA receptors in NMC can block myoclonus.
29 atients (1.2%) had isolated segmental spinal myoclonus.
30 of genetic progressive encephalopathies with myoclonus.
31 ism and dystonia with cognitive decline, and myoclonus.
32 excitability that eventually causes cortical myoclonus.
33 f 157 patients (62%) with available data had myoclonus.
34 dically refractory, progressive epilepsy and myoclonus.
35 ts were included, of whom 69 (16%) had early myoclonus.
36 gressive encephalomyelitis with rigidity and myoclonus.
37 tor cortex excitability, as seen in cortical myoclonus.
38 rapeutic hypothermia and 471 (18%) exhibited myoclonus.
39 limbic involvement), and two had opsoclonus-myoclonus.
40 gressive encephalomyelitis with rigidity and myoclonus.
41 ms were more common at younger age of onset (myoclonus 0.93, 0.90-0.97, p=0.0007; seizures 0.95, 0.92
43 nt early, but quickly became overshadowed by myoclonus; 10 patients were wheelchair-bound by their la
44 onal neurological features; individuals with myoclonus (40 [47%] with PSEN1 mutations and 12 [33%] wi
45 ment disorders, including tremor (15 [94%]), myoclonus (5 [31%]), and parkinsonism (11 [69%]), were c
46 of 1-2 was reported in 9.8% of patients with myoclonus, 5.8% with MyS/SM, 5.7% with MSE, and 82.0% wi
47 verify that the lesions were responsible for myoclonus, 6 additional cats received N-methyl-D-asparta
50 ine percent of cardiac arrest survivors with myoclonus after cardiac arrest had good functional outco
51 alongside, a trend towards worsening of the myoclonus after the cerebellar stimulation was observed.
52 edominantly distal, semi-continuous rhythmic myoclonus (all patients), generalized tonic-clonic seizu
55 sive cortical dementia with pyramidal signs, myoclonus and cerebellar abnormalities that closely rese
60 mental ability as well as the development of myoclonus and extrapyramidal signs are consistent manife
61 racetam is especially useful for posthypoxic myoclonus and gamma-hydroxybutyric acid for alcohol-sens
63 is considered a rhythmic variant of cortical myoclonus and is part of the 'spectrum of cortical myocl
64 to exhibit periods of spontaneous or sensory myoclonus and locomotion beginning 24 h after decerebrat
65 l had cerebellar signs, and the majority had myoclonus and lower limb pyramidal signs, with relativel
67 rx plus 7") pups display abnormal spasm-like myoclonus and other key EEG features, including multifoc
68 withdrawn in 330 of 427 patients (78%) with myoclonus and poor outcome, due to neurological futility
71 18 and 15 (83%) of 18 patients had improved myoclonus and sedation after hydration versus eight (47%
72 resented as a progressive cognitive decline, myoclonus and seizures developing later in the disease,
75 of abnormal neurotransmission, manifested by myoclonus and seizures, the molecular mechanisms by whic
76 was higher in the published data cohort for myoclonus and spasticity (19.4%, 16.6-22.2 and 15.0%, 12
77 ns in the DIAN-OBS cohort was low, including myoclonus and spasticity (9.3%, 95% CI 3.8-15.0), and se
78 c studies demonstrated a cortical origin for myoclonus and striking coactivation of agonist and antag
80 s consists of: rapidly progressive dementia, myoclonus and typical electroencephalographic findings (
82 argic (lh) is characterized by ataxia, focal myoclonus, and absence epilepsy due to a loss-of-functio
85 oplastic cerebellar degeneration, opsoclonus-myoclonus, and encephalitides affecting the limbic syste
87 gressive encephalomyelitis with rigidity and myoclonus, and less frequently in other variants of stif
89 t pupillary or corneal reflexes, presence of myoclonus, and neuron-specific enolase greater than 75 m
91 nt our approach for evaluating patients with myoclonus, and suggest practical guidelines for treating
92 gressive encephalomyelitis with rigidity and myoclonus, and two as stiff person syndrome; five had a
94 ations which are being used in management of myoclonus are levetiracetam and gamma-hydroxybutyric aci
99 gressive encephalomyelitis with rigidity and myoclonus associated with glycine receptor alpha 1 antib
100 5-phosphonopentanoic acid (AP5, 20 mM) block myoclonus at a latency of 0.6-3 min with the block lasti
103 em reflexes, motor response, and presence of myoclonus) at Day 3 after cardiac arrest remains an accu
106 toimmune target in paraneoplastic opsoclonus myoclonus ataxia (POMA) patients with latent cancer, red
107 an autoantigen in paraneoplastic opsoclonus myoclonus ataxia (POMA), a disorder associated with brea
108 tigens targeted in paraneoplastic opsoclonus myoclonus ataxia (POMA), an autoimmune neurologic diseas
109 nts suffering from paraneoplastic opsoclonus myoclonus ataxia (POMA), Nova-1 and Nova-2 proteins are
113 utoimmune disorder paraneoplastic opsoclonus-myoclonus ataxia and contain K-homology (KH)-type RNA bi
114 ith the possible exception of the opsoclonus-myoclonus ataxia and Lambert-Eaton myasthenic syndromes.
115 utoimmune disorder paraneoplastic opsoclonus-myoclonus ataxia, which is characterized by failure of i
117 ruses (n=31) by the neurological findings of myoclonus, ataxia, weakness, and autonomic instability.
118 in the Americas, was characterised by fever, myoclonus, ataxia, weakness, autonomic instability, and
122 induced episodes of generalized rigidity and myoclonus, axial hyperextension, and trismus, without im
126 ric limb rigidity and apraxia with dystonia, myoclonus, cortical sensory loss and alien limb phenomen
128 (supranuclear vertical gaze palsy, rhythmic myoclonus, dementia with psychiatric symptoms, or hypoth
130 confirmed shorter survival in patients with myoclonus (difference, -125.9 [95% CI, -236.3 to -15.5]
137 We established a cohort of patients with myoclonus dystonia syndrome and SGCE mutations to determ
139 n all, 89 patients with clinically suspected myoclonus dystonia syndrome were recruited from the UK a
141 dystonia, writer's cramp, cranial dystonia, myoclonus dystonia, and off-state dystonia associated wi
142 (DYT6 and DYT13), dopa-responsive dystonia, myoclonus dystonia, rapid-onset dystonia parkinsonism, F
149 port a large kindred with essential familial myoclonus-dystonia and map a locus for the disorder to a
150 rophysiological subcortical abnormalities in myoclonus-dystonia and their modulation by alcohol admin
154 rainstem interaction were investigated in 17 myoclonus-dystonia patients with epsilon-sarcoglycan (SG
155 generated iPSC-derived cortical neurons from myoclonus-dystonia patients with mutations (W100G and R1
156 ved cortical neurons with SGCE mutations for myoclonus-dystonia research and, in more general terms,
159 solated dystonia, good treatment response in myoclonus-dystonia, and suggest that early treatment cor
160 Clinical and genetic heterogeneity underlie myoclonus-dystonia, dopa-responsive dystonia and deafnes
161 erexcitability characteristic of this unique myoclonus-dystonia-like syndrome associated with cardiac
162 ree-generation family with a unique dominant myoclonus-dystonia-like syndrome with cardiac arrhythmia
164 nResponsiveness [FOUR] score at 72 hr, early myoclonus), electroencephalography (EEG) (reactivity, co
165 clinical examination (brainstem reflexes and myoclonus), electroencephalography reactivity during the
168 el for human Unverricht-Lundborg progressive myoclonus epilepsy (EPM1) on 21q22.3 nor for spinocerebe
170 ickle 1 (Pk1) is associated with progressive myoclonus epilepsy (PME) in humans, and its reduced gene
173 comprehensively describe the new syndrome of myoclonus epilepsy and ataxia due to potassium channel m
175 n this study CNS tissues from a patient with myoclonus epilepsy and ragged red fibers (MERRF) syndrom
177 disease (LD) is a fatal form of progressive myoclonus epilepsy caused by recessive mutations in eith
179 fora disease is a rare and fatal progressive myoclonus epilepsy characterized by the accumulation of
180 LD) is a teenage-onset inherited progressive myoclonus epilepsy characterized by the accumulations of
181 is an autosomal recessive, fatal progressive myoclonus epilepsy characterized by the intracellular bu
182 Lafora disease (LD) is a fatal progressive myoclonus epilepsy characterized neuropathologically by
183 CBD of laforin likely result in progressive myoclonus epilepsy due to mis-localization of phosphatas
188 with several features akin to of progressive myoclonus epilepsy such as neuronal ceroid lipofuscinosi
190 The typical presentation was of progressive myoclonus epilepsy with debilitating myoclonic seizures
193 afora disease (LD) is an autosomal recessive myoclonus epilepsy with onset in the teenage years leadi
196 Clinical distinction of type A (progressive myoclonus epilepsy) and type B (dementia with motor dist
197 on but may also be seen in Lafora's disease (myoclonus epilepsy), cyanamide alcohol aversion therapy,
198 a disease, a fatal teenage-onset progressive myoclonus epilepsy, are caused by mutations in either th
199 E) resulted, at age 13 years, in progressive myoclonus epilepsy, with many inclusions present in almo
205 aluated for target symptoms (hallucinations, myoclonus, fatigue, and sedation), global well-being, an
206 ation, clumsy useless limb, limb apraxia and myoclonus, four had cortical sensory impairment and foca
209 cal analysis showed that the cats generating myoclonus hemorrhagic lesions in the retrorubral nucleus
210 enotype that includes severe infantile onset myoclonus, hypotonia, optic nerve abnormalities, dysphag
211 nus and is part of the 'spectrum of cortical myoclonus', i.e. a wide range of clinical motor phenomen
215 with encephalopathy and developed prolonged myoclonus in the absence of clear systemic provoking fac
218 sessment of patients with suspected cortical myoclonus in whom myoclonic EMG bursts repeat rhythmical
219 spiratory failure (fatal in 1 case), startle myoclonus (in 2 unrelated cases), axial rigidity, and Pa
223 PRETATION: We propose that familial cortical myoclonus is a novel movement disorder that may be cause
224 gressive encephalomyelitis with rigidity and myoclonus is characterized by rigidity, painful muscle s
228 l discharge was noted in 9% of patients with myoclonus, less frequently in myoclonus with epileptifor
229 episodes), dryness of mouth (two episodes), myoclonus like activity of eyelids (four episodes), ocul
230 ve, motor, and sensory examinations, tremor, myoclonus, local sensory symptoms, symptoms at the expos
231 cohorts to determine whether the presence of myoclonus may aid in the assessment of disease severity,
232 The sudden, shock-like jerks which define myoclonus may be highly disabling, and when they persist
236 (n = 14), or extrapyramidal (n = 12) signs, myoclonus (n = 12), visual disturbance (n = 9), and akin
239 y of the movement disorders was complex with myoclonus observed in 68.3% of cases (28/41), either in
240 signs was decreased, and the development of myoclonus occurred later among patients with APOE epsilo
241 ld a higher percentage of good outcomes, but myoclonus of itself should not be considered a sign of f
245 pite circumstantial evidence that opsoclonus-myoclonus (OM) is often immune mediated, no specific aut
247 gressive encephalomyelitis with rigidity and myoclonus or stiff person syndrome, and glycine receptor
248 re brainstem syndrome (including opsoclonus, myoclonus, or both), cerebellar syndrome, myelopathy, pe
249 No patients with Pattern 1, subcortical myoclonus, or other patterns survived with favorable out
250 althy participants had excessive fragmentary myoclonus (p=0.039), and nine (38%) patients, but no hea
251 G) phenotypes of early postanoxic multifocal myoclonus (PAMM) that develop after cardiac arrest.
254 g Delirium Screening Scale (NuDESC), Unified Myoclonus Rating Scale (UMRS), Functional Assessment of
255 evaluation of patients included the Unified Myoclonus Rating Scale and Burke-Fahn-Marsden Dystonia R
256 clinical rating scales including the Unified Myoclonus Rating Scale and the Burke-Fahn-Marsden Dyston
258 Variants in the LIMP-2 gene cause action myoclonus-renal failure syndrome and also have been link
261 e sum of four dehydration symptoms (fatigue, myoclonus, sedation and hallucinations, 0 = best and 40
262 ra bodies and rescues the neurodegeneration, myoclonus, seizure susceptibility, and behavioral abnorm
263 ear gaze palsy, hypothalamic manifestations, myoclonus, seizures, ataxia, or OMM, especially when tis
264 ive or behavioural symptoms, and presence of myoclonus, seizures, pyramidal signs, extrapyramidal sig
265 sented with subacute cerebellar syndrome and myoclonus several days after general infectious symptoms
266 es such as limbic encephalitis or opsoclonus-myoclonus should always raise suspicion of a paraneoplas
267 The motor cortex is the most commonly shown myoclonus source, but origins from subcortical areas, br
268 rating the distinct elements of the cortical myoclonus spectrum, and how cortical tremor fits into it
270 ers include tremors, dystonia, chorea, tics, myoclonus, stereotypies, restless legs syndrome, and var
271 rvations establish the etiology of sustained myoclonus (sudden involuntary muscle movements) and earl
275 2 patients recovering from viral opsoclonus-myoclonus syndrome, comparing saccadic-vergence response
278 t for Parkinson tremor and rhythmic cortical myoclonus, the discovery of drugs for tremor disorders h
279 nge of clinical motor phenomena, from reflex myoclonus to myoclonic epilepsy, caused by abnormal sens
280 mands only and abnormal movements, including myoclonus, tongue and orofacial dyskinesias, and opsoclo
283 bability including seizures, encephalopathy, myoclonus, tremor and spasticity, with immunotherapy res
285 Symptoms included agitation, confusion, myoclonus, tremor, and seizures (1 case with prominent s
286 nervous system hyperexcitability (agitation, myoclonus, tremor, seizures), pleocytosis, and frequent
288 large, 4-generation family with a history of myoclonus underwent careful questioning, examination, an
291 bnormal nuclear envelopes, motor deficits or myoclonus, we produced paternally inherited striatum-spe
293 tient had severe axial and limb rigidity and myoclonus, which partially improved after chemotherapy a
296 patients with myoclonus, less frequently in myoclonus with epileptiform activity (2% vs 15%; p < 0.0