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Christos Panayi, Nagui Antoun, Richard Sandford
A 44-year-old woman with a history of von Hippel-Lindau (VHL) disease, a rare inherited neoplasia syndrome, presented acutely to hospital with a productive cough, symptoms of respiratory tract infection and odynophagia (painful swallowing). A chest X-ray confirmed right-sided pneumonia. Investigation of the persistent odynophagia using barium swallow revealed aspiration of the contrast into the lungs and suggested a neurological cause for her chest infection. Clinical assessment and speech and language therapy confirmed a pseudobulbar palsy...
October 13, 2016: BMJ Case Reports
N A Trusova, O S Levin, A V Arablinsky
AIM: To study clinical/neuropsychological and neuroimaging characteristics of Alzheimer's disease in the combination with cerebrovascular disease (CVD). MATERIAL AND METHODS: Ninety patients with dementia, including 35 patients with AD, 35 patients with mixed dementia (MD) and 20 patients with vascular dementia, were examined. The character of dementia was established according to NINCDS-ADRDA and NINDS-AIREN criteria. The neuropsychological battery included Addenbrooke's Cognitive Examination (ACE-R), Montreal Cognitive Assessment scale (MoCA), fluency test and the visual memory test (SCT)...
2016: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
Hye Yeon Lee, Min Jeong Kim, Bo-Ram Kim, Seong-Eun Koh, In-Sik Lee, Jongmin Lee
Bilateral paramedian thalamic infarction is a rare subtype of stroke caused by occlusion of the artery of Percheron, an uncommon variant originating from one of the posterior cerebral arteries. This type of stroke has several major clinical presentations: altered mental status, behavioral amnestic impairment, aphasia or dysarthria, ocular movement disorders, motor deficits, cerebellar signs, and others. Few cases of bilateral paramedian thalamic infarction-related pseudobulbar palsy characterized by dysarthria, dysphagia, and facial and tongue weakness have been reported...
August 2016: Annals of Rehabilitation Medicine
Flora M Hammond, David N Alexander, Andrew J Cutler, Stephen D'Amico, Rachelle S Doody, William Sauve, Richard D Zorowitz, Charles S Davis, Paul Shin, Fred Ledon, Charles Yonan, Andrea E Formella, Joao Siffert
No abstract text is available yet for this article.
2016: BMC Neurology
Mary I Butler, David Williams, David R Cotter
No abstract text is available yet for this article.
October 2016: Journal of Clinical Psychopharmacology
Bhavesh Trikamji, Mariam Thomas, Gasser Hathout, Shrikant Mishra
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an autosomal dominant angiopathy caused by a mutation in the notch 3 gene on chromosome 19. Clinically, patients may be asymptomatic or can present with recurrent ischemic episodes and strokes leading to dementia, depression, pseudobulbar palsy, and hemi- or quadraplegia. Additional manifestations that have been described include migraine (mostly with aura), psychiatric disturbances, and epileptic seizures...
April 2016: Annals of Indian Academy of Neurology
Flora M Hammond, David N Alexander, Andrew J Cutler, Stephen D'Amico, Rachelle S Doody, William Sauve, Richard D Zorowitz, Charles S Davis, Paul Shin, Fred Ledon, Charles Yonan, Andrea E Formella, Joao Siffert
BACKGROUND: Phase 3 trials supporting dextromethorphan/quinidine (DM/Q) use as a treatment for pseudobulbar affect (PBA) were conducted in patients with amyotrophic lateral sclerosis (ALS) or multiple sclerosis (MS). The PRISM II study provides additional DM/Q experience with PBA secondary to dementia, stroke, or traumatic brain injury (TBI). METHODS: Participants in this open-label, multicenter, 90-day trial received DM/Q 20/10 mg twice daily. The primary outcome was the Center for Neurologic Study-Lability Scale (CNS-LS), assessing change in PBA episode frequency and severity...
2016: BMC Neurology
Charles P Taylor, Stephen F Traynelis, Joao Siffert, Laura E Pope, Rae R Matsumoto
Dextromethorphan (DM) has been used for more than 50years as an over-the-counter antitussive. Studies have revealed a complex pharmacology of DM with mechanisms beyond blockade of N-methyl-d-aspartate (NMDA) receptors and inhibition of glutamate excitotoxicity, likely contributing to its pharmacological activity and clinical potential. DM is rapidly metabolized to dextrorphan, which has hampered the exploration of DM therapy separate from its metabolites. Coadministration of DM with a low dose of quinidine inhibits DM metabolism, yields greater bioavailability and enables more specific testing of the therapeutic properties of DM apart from its metabolites...
August 2016: Pharmacology & Therapeutics
Laurence P Perotti, Latiba D Cummings, Janyna Mercado
PURPOSE: To determine if it is possible to successfully treat pseudobulbar affect (PBA) using a behavioral approach. DESIGN AND METHODS: Two experiments were conducted, each a double reversal design with the same single subject in both. The first experiment tested the hypothesis that the rate of PBA could be controlled by manipulation of its consequences. The second experiment tested the hypothesis that use of a self-control procedure would control the rate of PBA...
April 2016: Perspectives in Psychiatric Care
Seiji Kaji, Toshitaka Kawarai, Ryosuke Miyamoto, Hiroyuki Nodera, Lucia Pedace, Antonio Orlacchio, Yuishin Izumi, Ryosuke Takahashi, Ryuji Kaji
Pathogenic mutations in the KIF5A-SPG10 gene, encoding the kinesin HC5A, can be associated with autosomal dominant hereditary spastic paraplegia (ADHSP). It accounts for about 10% of the complicated forms of ADHSP. Peripheral neuropathy, distal upper limb amyotrophy, and cognitive decline are the most common additional clinical features. We examined a 66-year-old Japanese woman manifesting gait disturbance and spastic dysarthria for 6years with positive family history. She showed evidence of upper and lower motor neuron involvement and fasciculations, thus mimicking amyotrophic lateral sclerosis (ALS)...
May 15, 2016: Journal of the Neurological Sciences
Chun-Hong Zhang, Jin-Ling Bian, Zhi-Hong Meng, Li-Na Meng, Xue-Song Ren, Zhi-Lin Wang, Xiao-Yan Guo, Xue-Min Shi
Tongguan Liqiao acupuncture therapy has been shown to effectively treat dysphagia after stroke-based pseudobulbar paralysis. We presumed that this therapy would be effective for dysphagia after bulbar paralysis in patients with brainstem infarction. Sixty-four patients with dysphagia following brainstem infarction were recruited and divided into a medulla oblongata infarction group (n = 22), a midbrain and pons infarction group (n = 16), and a multiple cerebral infarction group (n = 26) according to their magnetic resonance imaging results...
February 2016: Neural Regeneration Research
Dynela Garcia-Baran, Thomas M Johnson, Joyce Wagner, Joann Shen, Michelle Geers
Pathological laughing and crying, or pseudobulbar affect (PBA), has been described in patients with neurological disorders such as multiple sclerosis, amyotrophic lateral sclerosis, Alzheimer's disease, stroke, and traumatic brain injury (TBI) since the 19th century (Schiffer 2005). The syndrome is characterized by inappropriate episodes of laughing or crying after minor stimuli. It was first coined a disinhibition of cortical control by Kinnier Wilson in 1924. It was observed in brain disease and seen with mild TBI...
March 2016: Medicine (Baltimore)
Eman Bahrani, Chloe E Nunneley, Sylvia Hsu, Joseph S Kass
Life-threatening and benign drug reactions occur frequently in the skin, affecting 8 % of the general population and 2-3 % of all hospitalized patients, emphasizing the need for physicians to effectively recognize and manage patients with drug-induced eruptions. Neurologic medications represent a vast array of drug classes with cutaneous side effects. Approximately 7 % of the United States (US) adult population is affected by adult-onset neurological disorders, reflecting a large number of patients on neurologic drug therapies...
March 2016: CNS Drugs
Katarzyna Szacka, Anna Potulska-Chromik, Katarzyna Fronczewska-Wieniawska, Andrzej Spychała, Leszek Kròlicki, Magdalena Kuźma-Kozakiewicz
PURPOSE OF THE REPORT: Approximately 30% of patients with motor neuron disease (MND) present swallowing difficulties even in early disease stages. The aim of this study was to examine the usefulness of esophageal scintigraphy in detecting early stage of dysphagia in MND. METHODS: Esophageal scintigraphy (ES) including mean transit time (MTT) estimation was performed in 121 MND patients presenting various levels of upper (UMN) and lower motor neuron (LMN) degeneration...
April 2016: Clinical Nuclear Medicine
Elias D Granadillo, Mario F Mendez
Humor, or the perception or elicitation of mirth and funniness, is distinguishable from laughter and can be differentially disturbed by neuropsychiatric disease. The authors describe two patients with constant joking, or Witzelsucht, in the absence of pseudobulbar affect and review the literature on pathological humor. These patients had involvement of frontal structures, impaired appreciation of nonsimple humor, and a compulsion for disinhibited joking. Current neuroscience suggests that impaired humor integration from right lateral frontal injury and disinhibition from orbitofrontal damage results in disinhibited humor, preferentially activating limbic and subcortical reward centers...
2016: Journal of Neuropsychiatry and Clinical Neurosciences
Young D Chang, Mellar P Davis, Joshua Smith, Terrence Gutgsell
No abstract text is available yet for this article.
May 2016: Journal of Pain and Symptom Management
Nimish J Thakore, Erik P Pioro
OBJECTIVE: To report an observational study of depression in a large cohort of patients with amyotrophic lateral sclerosis (ALS), including its prevalence, associations, longitudinal course, and effect on survival. METHODS: The Patient Health Questionnaire-9 (PHQ-9) (a validated depression instrument) and other self-reported measures were obtained from patients with ALS as part of routine clinical care via tablet devices using a software system (Knowledge Program)...
March 15, 2016: Neurology
O S Cohen, I Kimiagar, A D Korczyn, Z Nitsan, S Appel, C Hoffmann, H Rosenmann, E Kahana, J Chapman
BACKGROUND AND PROPOSE: Familial Creutzfeldt-Jakob disease (fCJD) in Jews of Libyan ancestry is caused by an E200K mutation in the PRNP gene. The typical presenting symptoms include cognitive decline, behavioral changes and gait disturbances; however, some patients may have an unusual presentation such as a stroke-like presentation, alien hand syndrome or visual disturbances. The aim of this paper is to describe uncommon presentations in our series of consecutive patients with E200K fCJD...
May 2016: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
David C Gillespie, Amy P Cadden, Rosalind Lees, Robert M West, Niall M Broomfield
BACKGROUND: Several studies have reported that emotional lability is a common consequence of stroke. However, there is uncertainty about the "true" prevalence of the condition because, across these studies, patients have been recruited at different stages of recovery, from different settings, and using different diagnostic methods. There have been no systematic reviews of the published evidence to ascertain how the prevalence of poststroke pseudobulbar affect (PBA) might vary according to these factors...
March 2016: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
Marian W Roman
No abstract text is available yet for this article.
2015: Issues in Mental Health Nursing
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