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R Tortelli, S Arcuti, M Copetti, R Barone, C Zecca, R Capozzo, M R Barulli, I L Simone, G Logroscino
OBJECTIVE: To evaluate whether the presence of pseudobulbar affect (PBA) in an early stage of the disease influences survival in a population-based incident cohort of amyotrophic lateral sclerosis (ALS). METHODS: Incident ALS cases, diagnosed according to El Escorial criteria, were enrolled from a prospective population-based registry in Puglia, Southern Italy. The Center for Neurologic Study-Lability Scale (CNS-LS), a self-administered questionnaire, was used to evaluate PBA...
March 11, 2018: Acta Neurologica Scandinavica
Neepa Patel, Hannah Combs, Michele York, Cecile Phan, Joohi Jimenez-Shahed
Pseudobulbar affect (PBA) is a syndrome of affective disturbance associated with inappropriate laughter and crying, independent of mood. PBA is common in amyotrophic lateral sclerosis (ALS) and increasingly recognized in Parkinson's disease (PD) and atypical parkinsonism (aP). Correlates of PBA have not been systematically studied. The purpose of this study was to determine whether cognitive and psychiatric comorbidities correlated with patient-reported symptoms of PBA by using the Center for Neurological Study-Lability Scale among patients with ALS, PD, and aP...
March 5, 2018: Journal of Neuropsychiatry and Clinical Neurosciences
Cornelia Laule, Irene M Vavasour, Elham Shahinfard, Burkhard Mädler, Jing Zhang, David K B Li, Alex L MacKay, Sandra M Sirrs
BACKGROUND AND PURPOSE: Late-onset adult Krabbe disease is a very rare demyelinating leukodystrophy, affecting less than 1 in a million people. Hematopoietic stem cell transplantation (HSCT) strategies can stop the accumulation of toxic metabolites that damage myelin-producing cells. We used quantitative advanced imaging metrics to longitudinally assess the impact of HSCT on brain abnormalities in adult-onset Krabbe disease. METHODS: A 42-year-old female with late-onset Krabbe disease and an age/sex-matched healthy control underwent annual 3T MRI (baseline was immediately prior to HSCT for the Krabbe subject)...
February 26, 2018: Journal of Neuroimaging: Official Journal of the American Society of Neuroimaging
Flora M Hammond, William Sauve, Fred Ledon, Charles Davis, Andrea E Formella
BACKGROUND: Dextromethorphan 20mg /quinidine 10mg (DM/Q) was approved to treat pseudobulbar affect (PBA) based upon phase 3 trials conducted in participants with amyotrophic lateral sclerosis or multiple sclerosis. PRISM II evaluated DM/Q effectiveness, safety and tolerability for PBA following stroke, dementia or traumatic brain injury (TBI). OBJECTIVE: To report results from the TBI cohort of PRISM II, including a TBI-specific functional scale. DESIGN: Open-label trial evaluating twice daily DM/Q over 90 days...
February 22, 2018: PM & R: the Journal of Injury, Function, and Rehabilitation
Regina R Berkovich, Alexey Y Sokolov, Daniel M Togasaki, Aida A Yakupova, Paul-Henry Cesar, Soma Sahai-Srivastava
OBJECTIVE: This study aimed to assess potential efficacy and safety of dextromethorphan/quinidine (DMQ) in prophylactic treatment of migraine in patients with multiple sclerosis (MS) with superimposed pseudobulbar affect (PBA). METHODS: Multiple sclerosis patients with superimposed PBA and comorbid migraine were enrolled into this open-label observational study at the University of Southern California Comprehensive MS Center. The baseline characteristics included, among other data, frequency and severity of acute migraine attacks and use of migraine relievers...
February 22, 2018: Clinical Neuropharmacology
Gokcen Oz Tuncer, Serap Teber, Muhammed Gültekin Kutluk, Pelin Albayrak, Gülhis Deda
INTRODUCTION: Hashimoto's encephalopathy (HE) is an autoimmune condition with varied neurological and psychiatric features. HE is very unusual as a cause of pseudobulbar palsy (PSP). CASE PRESENTATION: A 14-year-old male was admitted with right-sided weakness, dysphagia, speech disorder, and aggressiveness. Brain magnetic resonance imaging showed increased intensity in bilateral temporal, insular cortex, amygdala, and parahippocampal area on T2-weighted and fluid-attenuated inversion recovery images...
January 24, 2018: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Simone Carradori, Daniela Secci, Jacques P Petzer
Monoamine oxidase (MAO) inhibitors, after the initial 'golden age', are currently used as third-line antidepressants (selective MAO-A inhibitors) or clinically enrolled as co-adjuvants for neurodegenerative diseases (selective MAO-B inhibitors). However, the research within this field is always increasing due to their pivotal role in modulating synaptic functions and monoamines metabolism. Areas covered: In this paper, MAO inhibitors (2015-2017) are disclosed ordering all the patents according to their chemical scaffold...
March 2018: Expert Opinion on Therapeutic Patents
Ramnath Santosh Ramanathan, Sandeep Rana
AIM: Primary lateral sclerosis (PLS) is a form of motor neuron disease involving only upper motor neurons. In some patients presenting as PLS, the disease progresses to involve lower motor neurons and thereby converting to amyotrophic lateral sclerosis (ALS). However, pure forms of PLS do exist. Our aim was to study epidemiological and clinical characteristics of pure PLS patients treated at our neuromuscular clinic. METHODS: We retrospectively reviewed 15 patients from July 2011 to October 2014 with PLS treated at the neuromuscular disorder clinic at our hospital...
January 10, 2018: Neurodegenerative Disease Management
Ivan Rektor, Nicolaas I Bohnen, Amos D Korczyn, Viktoria Gryb, Hrishikesh Kumar, Milica G Kramberger, Frank-Erik de Leeuw, Zvezdan Pirtošek, Irena Rektorová, Ilana Schlesinger, Jaroslaw Slawek, Peter Valkovič, Branislav Veselý
This expert working group report proposes an updated approach to subtype definition of vascular parkinsonism (VaP) based on a review of the existing literature. The persistent lack of consensus on clear terminology and inconsistent conceptual definition of VaP formed the impetus for the current expert recommendation report. The updated diagnostic approach intends to provide a comprehensive tool for clinical practice. The preamble for this initiative is that VaP can be diagnosed in individual patients with possible prognostic and therapeutic consequences and therefore should be recognized as a clinical entity...
December 29, 2017: Parkinsonism & related Disorders
Jack J Chen
This activity will update pharmacists and other healthcare professionals on current treatments for pseudobulbar affect (PBA). Points of discussion will focus on the off-label therapies traditionally used to treat PBA, the FDA-approved combination drug product with PBA as an indication, and managed care aspects of treating PBA.
December 2017: American Journal of Managed Care
Tammie Lee Demler
Pseudobulbar affect (PBA), despite its prevalence and distinctive symptoms, is widely underrecognized and undertreated. It is characterized by uncontrollable laughing or crying that can occur in an exaggerated manner or inappropriately to a given situation or stimuli. PBA is thought to center around preexisting neurological conditions, which include Parkinson disease, multiple sclerosis, amyotrophic lateral sclerosis, Alzheimer disease, traumatic brain injury, and stroke. The PBA Registry Series trial was created to measure the prevalence of PBA among patients with these underlying neurological conditions...
December 2017: American Journal of Managed Care
Jiamei Chu, Xiaoping Liu, Feiyu Chen, Feifei Hong, Yehua Bao
OBJECTIVE: To observe the effects of GAO 's neck acupuncture combined with swallowing rehabilitation on swallowing function and quality of life in patients with post-stroke pseudobulbar palsy. METHODS: One hundred patients were randomly assigned in to an observation group and a control group, 50 cases in each one. The patients in the control group were treated with basic pharmaceutical treatment, including neurotrophy medication and free radical scavenging medication as well as swallowing rehabilitation; the patients in the observation group, on the basis of those in the control group, were treated with GAO 's neck acupuncture at Fengchi (GB 20), Yiming (EX-HN 14), Gongxue (Extra), Lianquan (CV 23), Wai Jinjin Yuye (Extra), Tunyan (Extra), Zhiqiang (Extra), Fayin (Extra), once a day, five times a week for continuous eight weeks...
July 12, 2017: Zhongguo Zhen Jiu, Chinese Acupuncture & Moxibustion
Zhixia Ren, Shuai Chen, Yingying Shi, Yuanxing Zhang, Wan Wang, Zuzhi Chen, Mingrong Xia, Xiaohong Shi, Jiewen Zhang
OBJECTIVE: To investigate a cerebral autosomal dominant arteriopathy with the subcortical infarcts and leukoencephalopathy (CADASIL) case with clinical manifestations of baldness, lumbago and Parkinson's symptoms. METHODS: Clinical and imaging data of the patient were analyzed. The patient and his family members were also subjected to genetic testing. RESULTS: The symptoms of the patient included recurrent stroke, dementia, and mood disturbance, in addition with lumbago, baldness and Parkinson's symptoms but no migraine...
December 10, 2017: Zhonghua Yi Xue Yi Chuan Xue za Zhi, Zhonghua Yixue Yichuanxue Zazhi, Chinese Journal of Medical Genetics
Chenguang Zhou, Yuanhong He, Xiaorui Tian, Zhiwen Chao, Yinghui Zhu, Du Cheng, Kui Li
Isolated bilateral cerebral peduncular infarctions (BCPI) presenting as acute pseudobulbar palsy are rarely reported and, to the best of our knowledge, most of the previous reports of BCPI were related to locked-in syndrome and disturbance of consciousness. Herein, we described a case of a 55-year-old man who presented with acute pseudobulbar palsy and mild tetraparesis, but preserved eye movements, with no consciousness disturbance. DWI revealed an acute infarction involving the central portion of the cerebral peduncle with a characteristic "traditional Chinese eight character" sign...
2017: Case Reports in Neurological Medicine
Jennifer M Gass, Anvir Cheema, Jessica Jackson, Patrick R Blackburn, Jay Van Gerpen, Paldeep S Atwal
INTRODUCTION: Alexander disease is a rare neurodegenerative disease caused by variants in the glial fibrillary acidic protein gene (GFAP). This disorder can develop as an infantile, juvenile or adult-onset form and is characterized by several clinical features, including macrocephaly, seizures, ataxia, and bulbar/pseudobulbar signs. While the majority of these patients have the more progressive infantile form which causes severe leukodystrophy and early death; the less common adult form is more variable (ie, onset age, symptoms), with bulbar dysfunction as the primary feature...
November 2017: Neurologist
Peter Bede, Eoin Finegan
No abstract text is available yet for this article.
November 2, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
Jiaping Zheng, Guoqiang Chen, Qing Xiao, Yiyang Huang, Yupeng Guo
The present study aimed to investigate the efficacy of endoscopy in the treatment of post-shunt placement for slit ventricle syndrome (SVS). Endoscopic surgery was performed on 18 patients with SVS between October 2004 and December 2012. Sex, age, causes of the hydrocephalus, ventricular size and imaging data were collected and analyzed. All patients were divided into two groups according to ventricular size and underwent endoscopic surgeries, including endoscopic third ventriculostomy (ETV), endoscopic aqueductoplasty and cystocisternostomy...
October 2017: Experimental and Therapeutic Medicine
Mai Tsuchiya, Ryusuke Takaki, Fumikazu Kobayashi, Takamura Nagasaka, Kazumasa Shindo, Yoshihisa Takiyama
We report a 40-year-old man who presented with multiple bone pseudofractures after about 20 years from the onset of Wilson's disease (WD). At age 36, he first noticed pain in his left shoulder. At age 39, he had multiple chest pain. On neurologic examinations, dysarthria and dysphagia due to pseudobulbar palsy, rigidity and tremor on right upper lim were observed. WD was confirmed because of low levels of plasma cupper and ceruloplasmin in addition to ATP7B gene mutation. The chest X-ray revealed multiple fractures of the several ribs...
September 30, 2017: Rinshō Shinkeigaku, Clinical Neurology
Carrie Allen, Barbara Zarowitz, Terrence O'Shea, Edward Peterson, Charles Yonan, Fanta Waterman
Pseudobulbar Affect (PBA) is a neurologic condition characterized by involuntary outbursts of crying and/or laughing disproportionate to patient mood or social context. Although an estimated 9% of nursing home residents have symptoms suggestive of PBA, they are not routinely screened. Our goal was to develop an electronic screening tool based upon characteristics common to nursing home residents with PBA identified through medical record data. Nursing home residents with PBA treated with dextromethorphan hydrobromide/quinidine sulfate (n = 140) were compared to age-, gender-, and dementia-diagnosis-matched controls without PBA or treatment (n = 140)...
August 11, 2017: Geriatric Nursing
M B Mironov, M Yu Bobylova, I V Nekrasova, T M Krasilschikova, M M Gunchenko, M N Sarzhina, A S Petrukhin, S G Burd, T T Batisheva
AIM: To study neurologic status, results of video-EEG monitoring and magnetic resonance imaging in children under 3 years old with paroxysms of tonic muscle tension. MATERIAL AND METHODS: One hundred and forty-six infants and young children with motor disturbances and different variants of clinically similar epileptic seizures, hyperkinesis and stereotypes were examined. RESULTS AND CONCLUSION: Cerebral palsy (91%), genetic and chromosomal abnormalities (6%), brain malformations (2%) were identified...
2017: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
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