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Essential tremor (ET) is one of the most prevalent movement disorders. Because ET is so common, individuals with other neurologic disorders may also have ET. There is evidence, however, that the cooccurrence of ET with Parkinson disease (PD) and/or dystonia is not merely a chance cooccurrence. We have observed combinations of these 3 movement disorders within individuals and across individuals within families containing multiple individuals with ET. This observation has a number of implications. Our objective is to present 4 ET families in whom motor phenomenology was heterogeneous and discuss the implications of this finding.
ET cases and their relatives were enrolled in the Family Study of Essential Tremor (2015-present). Phenotyping was performed by a senior movement disorders neurologist based on neurologic examination.
We present 4 families, including 14 affected individuals, among whom assigned diagnoses were ET, PD, ET + PD, and ET + dystonia. In those with ET and another movement disorder, the pr disorders to manifest within particular families.
To examine sex differences in early stroke deaths according to cause of death.
We investigated 30-day deaths in patients with acute ischemic stroke enrolled in a multicenter stroke registry between 2007 and 2019 in Fukuoka, Japan. We estimated the multivariable-adjusted hazard ratios (HRs) and 95% confidence intervals (CIs) of cause-specific deaths for women vs men using Cox proportional hazards models and competing risk models. The risk of acute infections during hospitalization and the associated case fatality rates were also compared between the sexes.
Among 17,956 patients with acute ischemic stroke (women 41.3%), the crude 30-day death rate after stroke was higher in women than men. However, adjusting for age and stroke severity resulted in a lower risk of death among women (HR [95% CI] 0.76 [0.62-0.92]). Analyses using competing risk models revealed that women were less likely to die of acute infections (subdistribution HR [95% CI] 0.33 [0.20-0.54]). Further analyses showed that women were associated with a lower risk of acute infections during hospitalization (OR [95% CI] 0.62 [0.52-0.74]) and a lower risk of death due to these infections (subdistribution HR [95% CI] 052 [0.33-0.83]).
When adjusting for confounders, the female sex was associated with a lower risk of 30-day death after stroke, which could be explained by a female survival advantage in poststroke infections. Sex-specific strategies are needed to reduce early stroke deaths.
This is a Class I prognostic study because it is a prospective population-based cohort with objective outcomes. Female sex appears to be protective against early stroke deaths and post stroke infections.
This is a Class I prognostic study because it is a prospective population-based cohort with objective outcomes. Female sex appears to be protective against early stroke deaths and post stroke infections.
To assess the risk of recurrence of cervical artery dissection (CAD) during pregnancy and puerperium in women with a history of CAD and then help physicians with providing medical information to women who wish to become pregnant.
Women aged 16-45 years who were admitted to our center for a CAD between 2005 and 2017 were identified from the hospital database, and those with spontaneous and symptomatic CAD were included. They were then contacted to answer a questionnaire that was specifically designed in regard to the recurrence of CAD and pregnancies after the primary CAD.
Ninety-one patients satisfied our inclusion criteria, and 89 were included in the analysis. During a median follow-up of 7.0 years, 4 women (4.4%) had recurrent CAD, although none during pregnancy or puerperium. Eighteen women (20%) had a total of 20 full-term pregnancies, occurring at least 6 months after CAD. Of these 20 pregnancies, 13 (65%) were vaginal deliveries, and 7 (35%) were cesarean sections. The reason for the absence of pregnancies after the initial CAD was unrelated to the vascular event in 89% of cases, but 8% of the women had been advised by a physician to avoid any future pregnancy or they had been recommended to undergo abortion or sterilization.
In this study, there were no CAD recurrences during subsequent pregnancies or postpartum, irrespective of the type of delivery. Thus, pregnancy after a history of CAD appears to be safe.
In this study, there were no CAD recurrences during subsequent pregnancies or postpartum, irrespective of the type of delivery. Thus, pregnancy after a history of CAD appears to be safe.
Etiologic investigations for adults with intellectual disability (ID) pose a special challenge to many adult neurologists. The adaptability of pediatric guidelines for workup of ID to adult populations has not been clearly established. We review the current recommendations on etiologic workup of ID in children and adults and provide initial guidance for adult neurologists who care for individuals with ID of unknown etiology.
Etiologic workup, including genetic testing, is recommended in individuals with ID of unknown origin. Workup should be guided by a thoughtful history and physical examination, which can help identify certain causes of ID.
Specific diagnoses may help guide management and surveillance of comorbid conditions in individuals with ID. Etiologic investigations of adults with ID include genetic and metabolic testing and brain imaging in the appropriate clinical setting.
Specific diagnoses may help guide management and surveillance of comorbid conditions in individuals with ID. Etiologic investigations of adults with ID include genetic and metabolic testing and brain imaging in the appropriate clinical setting.
To determine the prevalence and burden of neurologic comorbidities in hospitalized patients with opioid abuse.
From 1 year of hospital discharges, 2,182 patients with opioid abuse were identified (prevalence 6.3%), with abuse greater among younger patients (
< 0.0001), women (
< 0.0001), Whites (
< 0.0001), and urban population (
= 0.028). Matching for age, sex, race, and urban-rural residence, 347 patients were reviewed, and 179 (52%) had a neurologic comorbidity. The comorbidities frequently overlapped and included encephalopathy (130), neuromuscular disorders (42), seizures (23), spine disorders (23), strokes (20), CNS infections (3), and movement disorders (2). Abuse patients with neurologic comorbidities experienced substantially greater number of hospital and intensive care unit days and mortality, independent of overdose.
Neurologic comorbidities are a frequent and heretofore underappreciated contributor to the disease burden of hospitalized patients with opioid abuse. The importance of neurologic comorbidities should be included in the public health discussions surrounding the opioid epidemic.
Neurologic comorbidities are a frequent and heretofore underappreciated contributor to the disease burden of hospitalized patients with opioid abuse. The importance of neurologic comorbidities should be included in the public health discussions surrounding the opioid epidemic.
Physician communication skills are a critical part of care for amyotrophic lateral sclerosis (ALS) patients and caregivers. check details They shape the development of autonomy and quality of life, and they mitigate emotional trauma. Communication skills are especially critical at 2 different time points in the course of the disease early when delivering and establishing the diagnosis, and later when clarifying goals of care.
Several techniques for physician communication of difficult information are available, including SPIKES (Setting up the interview, assessing the patient's Perception, obtaining the patient's Invitation, giving Knowledge and information to the patient, addressing the patient's Emotions with Empathetic responses, and Strategy and Summary), ABCDE (Advance preparation, Build a therapeutic environment/relationship, Communicate well, Deal with patient and family reactions, Encourage and validate emotions), and BREAKS (Background, Rapport, Exploring, Announce, Kindling, Summarize). These emphasize the physician's accountability and responsibility for communicating effectively. Formal training in these techniques is limited, and their applicability specifically to ALS is inexact.
We propose an ALS-specific technique which we call ALS ALLOW to guide physicians in conducting difficult communications with ALS patients and caregivers to develop their understanding, establish autonomy, set goals, and mitigate emotional trauma. The techniques are useful in discussions both early and late stages in the disease.
We propose an ALS-specific technique which we call ALS ALLOW to guide physicians in conducting difficult communications with ALS patients and caregivers to develop their understanding, establish autonomy, set goals, and mitigate emotional trauma. The techniques are useful in discussions both early and late stages in the disease.
To determine the impact of percutaneous endoscopic gastrostomy (PEG) tubes in patients with advanced Huntington disease (HD).
A retrospective chart review of patients with HD was conducted to assess the rate of pneumonia and pressure ulcer, length of life, changes in weight, and serologic nutritional measures. Surviving and deceased patients with and without PEG tubes were compared using descriptive statistical analysis.
One hundred forty-eight records were reviewed (39 patients with PEG tubes). link2 The mean age of patients still alive and diagnosed with HD was 58.3 ± 12.7 years and age at death (n = 62) 57.7 ± 10.3 years. At the time of analysis, the mean duration of HD was 14.2 ± 7 years. Groups were similar in sex, age, and weight at admission. In those deceased, insertion of a PEG tube increased the length of life with HD by 3.6 years (16.2 ± 6.7 vs 13.2 ± 4.9 years). PEG tube placement significantly reduced cholesterol levels, increased the prevalence of skin ulcers and the rate of pneumonia. Insertion of a PEG tube did not significantly change weight or albumin levels.
PEG tube placement in advanced HD provided benefit in the length of life, but weight, other nutritional measures, and the rate of pneumonia were either not impacted or worsened with the insertion of a PEG tube. Impact on quality of life needs further study, but providers, patients, and families should consider all options when discussing preferences for interventions.
This study provides Class IV evidence that for patients with advanced HD, PEG tube placement increases the length of life but has no or negative impacts on nutritional measures.
This study provides Class IV evidence that for patients with advanced HD, PEG tube placement increases the length of life but has no or negative impacts on nutritional measures.
We sought to explore current perspectives and attitudes of general neurologists and movement disorder specialists toward deep brain stimulation (DBS) for Parkinson disease (PD), focusing on perspectives on its earlier use in the clinical course of the disease.
We designed a 30-question online survey comprised of Likert-type, multiple choice, and rank-order questions, which was distributed to 932 neurologist members of the American Academy of Neurology. We analyzed clinicians' sociodemographic information, treatment patterns used for patients with PD, reasons for and against patient referral for DBS, and general attitudes toward DBS. link3 Data were analyzed using descriptive and inferential statistics.
We received 164/930 completed surveys (completion rate of 18%). Overall, most respondents agreed that DBS was more useful after the appearance of motor complications and that DBS utilization offered better management of PD than medication alone. However, respondents were divided on issues like minimum duration of disease needed to consider DBS as a treatment option and timing of DBS referral relative to disease progression.
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