scholarly journals Progressive Neurologic Disorder

1985 ◽  
Vol 60 (3) ◽  
pp. 211
Author(s):  
David G. Cogan
Keyword(s):  
2021 ◽  
pp. 153575972199832
Author(s):  
Karnig Kazazian ◽  
Marissa Kellogg ◽  
Nora Wong ◽  
Krista Eschbach ◽  
Raquel Farias Moeller ◽  
...  

New-onset refractory status epilepticus (NORSE) is a rare clinical presentation of refractory status epilepticus (RSE) that occurs in people without active epilepsy or preexisting neurologic disorder. Febrile infection-related epilepsy syndrome (FIRES) is a subcategory of NORSE. New-onset refractory status epilepticus/FIRES are becoming increasingly recognized; however, information pertaining to disease course, clinical outcomes, and survivorship remains limited, and mortality and morbidity are variable, but often high. The objective of the NORSE/FIRES Family Registry is to (1) provide an easily accessible and internationally available multilingual registry into which survivors or NORSE/FIRES surrogates or family members of people affected by NORSE/FIRES or their physicians can enter data in a systematic and rigorous research study from anywhere in the world where internet is available; and (2) to examine past medical history, outcomes, and quality of life for people affected by NORSE/FIRES.


2015 ◽  
Vol 2015 ◽  
pp. 1-3
Author(s):  
Christian Grønhøj Larsen ◽  
Mette Gyldenløve ◽  
Aia Elise Jønch ◽  
Birgitte Charabi ◽  
Zeynep Tümer

Idiopathic facial palsy (IFP), also known as Bell’s palsy, is a common neurologic disorder, but recurrent and familial forms are rare. This case series presents a three-generation family with idiopathic facial palsy. The mode of inheritance of IFP has previously been suggested as autosomal dominant with low or variable penetrance, but the present family indicates an autosomal dominant trait with high or complete penetrance. Chromosome microarray studies did not reveal a pathogenic copy number variation, which could enable identification of a candidate gene.


1976 ◽  
Vol 2 (2) ◽  
pp. 107-111 ◽  
Author(s):  
Yoshitaka Nagai ◽  
Takashi Momoi ◽  
Masaki Saito ◽  
Eizo Mitsuzawa ◽  
Sugishi Ohtani

2018 ◽  
Vol 38 (05) ◽  
pp. 539-547 ◽  
Author(s):  
Neil Vaishnav ◽  
Winston Chiong

AbstractThe doctrine of informed consent sits at the intersection of law, ethics, and neuroscience, posing unique challenges for human subject research involving neurological patients. These challenges are compounded by the variegated nature of both neurological injury and the law governing research consent. This article provides a framework for investigators likely to encounter subjects with some degree of neurological impairment, whose capacity to consent requires scrupulous assessment prior to enrollment in research trials. We consider several researches and disease contexts—from emergency epilepsy research to long-term dementia research—and clarify the ethical and legal principles governing consent for participation in each. We additionally explore empirical research on consent capacity and survey several areas of emerging ethical import that will require the attention of investigators in decades to come.


2010 ◽  
Vol 10 (1) ◽  
Author(s):  
Abdulrahman M El-Sayed ◽  
Craig Hadley ◽  
Fasil Tessema ◽  
Ayelew Tegegn ◽  
John A Cowan ◽  
...  

2021 ◽  
Vol 12 ◽  
Author(s):  
Thomas Hassa ◽  
Stefan Spiteri ◽  
Roger Schmidt ◽  
Christian Merkel ◽  
Mircea Ariel Schoenfeld

Cognitive reappraisal is an emotion regulation strategy to reduce the impact of affective stimuli. This regulation could be incomplete in patients with functional neurologic disorder (FND) resulting in an overflowing emotional stimulation perpetuating symptoms in FND patients. Here we employed functional MRI to study cognitive reappraisal in FND. A total of 24 FND patients and 24 healthy controls employed cognitive reappraisal while seeing emotional visual stimuli in the scanner. The Symptom Checklist-90-R (SCL-90-R) was used to evaluate concomitant psychopathologies of the patients. During cognitive reappraisal of negative IAPS images FND patients show an increased activation of the right amygdala compared to normal controls. We found no evidence of downregulation in the amygdala during reappraisal neither in the patients nor in the control group. The valence and arousal ratings of the IAPS images were similar across groups. However, a subgroup of patients showed a significant higher account of extreme low ratings for arousal for negative images. These low ratings correlated inversely with the item “anxiety” of the SCL-90-R. The increased activation of the amygdala during cognitive reappraisal suggests altered processing of emotional stimuli in this region in FND patients.


2019 ◽  
Vol 6 (4) ◽  
pp. 1592
Author(s):  
Sheeraz A. Dar ◽  
Wasim A. Wani ◽  
Mudasir Nazir ◽  
Zul Eidain Hussan

Background: Seizures are the most common pediatric neurologic disorder, with 4% to 10% of children suffering at least one seizure in the first 16 years of life objectives to compare efficacy of IV phenytoin, IV valproate, and IV levetiracetam in childhood seizures between 2months to 16 years of age.Methods: This prospective, randomized, study was done on pediatric patients in the age group of 2 months to 16 years who present actively convulsing to the emergency department of pediatrics.Results: At 24 hours seizures were controlled in 44 (88%) patients out of 50 patients in phenytoin group, 39 (78%) out of 50 patients in levetiracetam group and 46 (92%) out of 50 patients in valproate group (p-value 0.115). The relative risk of seizure recurrence for levetiracetam and phenytoin groups when compared to valproate was 2.75 and 1.5, respectively.Conclusions: Present study demonstrates that IV levetiracetam and IV valproate were comparable to IV phenytoin in terms of seizure control in acute setting. All the three are safe and efficacious. Time to regain consciousness was less in valproate group and long-term seizure control too was also better.


2014 ◽  
Vol 21 (1) ◽  
pp. 31-34 ◽  
Author(s):  
Joana Machado ◽  
Paula Ministro ◽  
Eugénia Cancela ◽  
Ricardo Araújo ◽  
António Castanheira ◽  
...  

PEDIATRICS ◽  
1991 ◽  
Vol 88 (6) ◽  
pp. 1204-1210
Author(s):  
Claudine De Praeter ◽  
Piet Vanhaesebrouck ◽  
Paul Govaert ◽  
Joris Delanghe ◽  
Jules Leroy

Creatine kinase brain isoenzyme (CK-BB) was determined in cerebrospinal fluid of 150 neonates by a newly developed immunoenzymatic assay. Newborns with a documented neurologic disorder (intraventricular hemorrhage, postasphyxial encephalopathy, central nervous system infection, or persistent periventricular intraparenchymal echodensities) showed markedly higher concentrations of immunoreactive CK-BB than did the normal newborns or those with subarachnoid hemorrhage. In neonates with seizures the data suggest that the underlying neurologic disorder accounts for the higher CK-BB values and not the seizures per se. High concentrations of CK-BB in the neonatal period were followed by poor short-term outcome.


PEDIATRICS ◽  
1989 ◽  
Vol 83 (3) ◽  
pp. 323-331
Author(s):  
Joseph Maytal ◽  
Shlomo Shinnar ◽  
Solomon L. Moshé ◽  
Luis A. Alvarez

In an ongoing study of status epilepticus, 193 children with status epilepticus of varying causes have been followed up for a mean period of 13.2 months. Of these, 97 patients were recruited prospectively. The patients' ages ranged from 1 month to 18 years (mean, 5.0 years). The cause of the status epilepticus was classified as idiopathic in 46 cases, remote symptomatic in 45, febrile in 46, acute symptomatic in 45, and progressive neurologic in 11. The mortality and incidence of sequelae following status epilepticus was low and primarily a function of etiology. Seven children died within 3 months of having the seizure. New neurologic deficits were found in 17 (9.1%) of the 186 survivors. All of the deaths and 15 of the 17 sequelae occurred in the 56 children with acute or progressive neurologic insults. Only two of the 137 children with other causes sustained any new deficits (P < .001). Duration of the status epilepticus affected outcome only within the acute symptomatic group (P < .05). Neurologic sequelae occurred in 29% of infants younger than 1 year of age, 11% of children 1 to 3 years of age, and 6% of children older than 3 years of age. However, this was a reflection of the greater incidence of acute neurologic disease in the younger age groups. Within each cause, age did not affect outcome. Of the 193 children, 61 (32%) had a history of prior unprovoked seizures. Of the 125 surviving children with no history of prior unprovoked seizures, 37 (30%) had subsequent unprovoked seizures. It is concluded that the morbidity of aggressively treated status epilepticus in children, in the absence of an acute neurologic insult or progressive neurologic disorder, is low.


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