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Maximum Tongue Pressure is Associated with Swallowing Dysfunction in ALS Patients

AbstractMaximum tongue pressure (MTP) measurement is a convenient, less invasive assessment that has been developed to quantify tongue strength; however, it is unclear whether MTP is useful for the detection of swallowing disorders in amyotrophic lateral sclerosis (ALS) patients. The purpose of this study was to clarify the relationship between MTP and the characteristics of swallowing disorders on videofluorography and to determine the usefulness of tongue pressure measurement for the assessment of swallowing function in ALS patients. Twenty-five ALS patients were evaluated according to the ALS functional rating scale-revised (ALSFRS-R), and their ability to swallow yogurt was observed via videofluorography. MTP was measured using a device (TPM-01, JMS, Hiroshima) equipped with a balloon probe. Then, the relationships between the ALSFRS-R score, swallowing function, and MTP were analyzed. MTP was significantly lower in the subjects with reduced tongue function (p = 0.002) or with pharyngeal residue (p = 0.006) than in the subjects with normal characteristics. Bolus formation and oral transit time and pharyngeal transit time were significantly prolonged among those with reduced MTP. MTP at a cut-off value of 21.0 kPa was associated with a full score on the bulbar-related items of the ALSFRS-R. MTP may serve as a new diagnostic tool for the early detection of swallowing dysfunction in ALS patients, because of its good relationship with their swallowing...
Source: Dysphagia - Category: Speech Therapy Source Type: research

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Conclusions: We show that in fibroblasts ofFUS P525L mutation carriers, FUS mislocalized to the cytoplasm where it redistributed into stress granules with likely a dose effect, i.e. a higher number of cells with granules, which persist longer, than in controls and ALS cases. These data represent an early molecular change occurring before ALS onset, suggesting a transient preaggregative state.Neurodegener Dis 2017;17:292-303
Source: Neurodegenerative Diseases - Category: Neurology Source Type: research
Brainstorm Cell Therapeutics (NSDQ:BCLI) said today that the first patients have been enrolled in its Phase III trial evaluating NurOwn as a treatment for amyotrophic lateral sclerosis, or ALS, at Massachusetts General Hospital and UC Irvine Medical Center. The trial, which is slated to enroll 200 patients, is designed to include the pre-specified patient subgroups who saw superior outcomes in the NurOwn Phase II ALS trial. BrainStorm said it expects top-line data from the trial in 2019. Get the full story at our sister site, Drug Delivery Business News. The post BrainStorm enrolls first ALS patient in late-stage NurO...
Source: Mass Device - Category: Medical Devices Authors: Tags: Clinical Trials Neurological Stem Cells Wall Street Beat BrainStorm Cell Therapeutics Inc. Source Type: news
Trial supported by a $16M non-dilutive grant from CIRM Top-line data are expected in 2019 HACKENSACK, N.J. and PETACH TIKVA, Israel, Oct. 16, 2017 -- (Healthcare Sales &Marketing Network) -- BrainStorm Cell Therapeutics Inc. (NASDAQ: BCLI) today an... Regenerative Medicine, Neurology BrainStorm Cell Therapeutics, NurOwn , stem cell, amyotrophic lateral sclerosis
Source: HSMN NewsFeed - Category: Pharmaceuticals Source Type: news
Developments in eye-gaze technology – which converts minute movements of the eye into spoken words – are opening up undreamed of opportunities for people with motor neurone syndromeSteve Thomas and I are talking about brain implants. Bonnie Tyler ’s Holding Out For a Hero is playing in the background and for a moment I almost forget that a disease has robbed Steve of his speech. The conversation breaks briefly; now I see his wheelchair, his ventilator, his hospital bed.Steve, a software engineer, was diagnosed with ALS (amyotrophic lateral sclerosis, a type ofmotor neurone disease) aged 50. He knew i...
Source: Guardian Unlimited Science - Category: Science Authors: Tags: Motor neurone disease Medical research Technology Society Science Health & wellbeing Life and style Source Type: news
TEL AVIV (Reuters) - BrainStorm Cell Therapeutics said the first patients have been enrolled in a late-stage trial of its treatment for amyotrophic lateral sclerosis (ALS) at Massachusetts General Hospital and UC Irvine Medical Center in California.
Source: Reuters: Health - Category: Consumer Health News Tags: healthNews Source Type: news
Source: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration - Category: Neurology Authors: Source Type: research
We report for the first time, two patients with sporadic limb-onset ALS who developed recurrent acute pancreatitis (AP), with portal vein thrombosis as complication, during treatment with riluzole. We suggest that AP should be considered asa probable rare and severe side effect of treatment with riluzole in patients with ALS. We believe that in patients who develop AP during treatment with riluzole, its withdrawn may prevent recurrent AP and should be discussed with patients.
Source: Journal of Clinical Neuroscience - Category: Neuroscience Source Type: research
Frontotemporal dementia (FTD), the second most common form of dementia in people under 65 years of age, is characterized by progressive atrophy of the frontal and/or temporal lobes. FTD overlaps extensively with the motor neuron disease amyotrophic lateral sclerosis (ALS), especially at the genetic level. Both FTD and ALS can be caused by many mutations in the same set of genes; the most prevalent of these mutations is a GGGGCC repeat expansion in the first intron of C9ORF72. As shown by recent intensive studies, some key cellular pathways are dysregulated in the ALS-FTD spectrum disorder, including autophagy, nucleoc...
Source: EMBO Journal - Category: Molecular Biology Authors: Tags: Molecular Biology of Disease, Neuroscience Review Source Type: research
Authors: Kaufman MB Abstract L-glutamine oral powder (Endari) for reducing the acute complications of sickle cell disease; edaravone (Radicava) for the treatment of amyotrophic lateral sclerosis; and midostaurin (Rydapt) for the treatment of acute myeloid leukemia in combination with chemotherapy. PMID: 29018296 [PubMed]
Source: P and T - Category: Drugs & Pharmacology Tags: P T Source Type: research
Identifying large expansions of short tandem repeats (STRs), such as those that cause amyotrophic lateral sclerosis (ALS) and fragile X syndrome, is challenging for short-read whole-genome sequencing (WGS) data. A solution to this problem is an important step toward integrating WGS into precision medicine. We developed a software tool called ExpansionHunter that, using PCR-free WGS short-read data, can genotype repeats at the locus of interest, even if the expanded repeat is larger than the read length. We applied our algorithm to WGS data from 3001 ALS patients who have been tested for the presence of the C9orf72 repeat e...
Source: Genome Research - Category: Genetics & Stem Cells Authors: Tags: METHOD Source Type: research
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