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...
Abstract Hirayama's disease is a rare clinical variant of amyotrophic lateral sclerosis where distal muscles are involved more compared to proximal muscles and vice-versa occurs only in 10% cases and so it is differentiated from O'Sullivan McLeod syndrome which involves only small muscles of single limb. Here, we present a case of Hirayama's disease where disease achieved a plateau after 3 years with no further progression. His electrophysiological studies, and clinical picture, and magnetic resonance imaging findings were consistent with a diagnosis of Hirayama's disease. PMID: 28828346 [PubMed]
AbstractEndoplasmic reticulum (ER) and mitochondria are tubular organelles with a characteristic “network structure” that facilitates the formation of inter-organellar connections. As a result, mitochondria-associated ER membranes (MAMs), a subdomain of the ER that is tightly linked to and communicates with mitochondria, serve multiple physiological functions including lipid synthesis and e xchange, calcium signaling, bioenergetics, and apoptosis. Importantly, emerging evidence suggests that the abnormality and dysfunction of MAMs have been involved in various neurodegenerative disorders including Alzheimer&rsq...
Conclusion: Combined analysis of TDP-43, τT, and τP-181 in CSF may be useful for the antemortem diagnosis of ALS and FTD.Dement Geriatr Cogn Disord 2017;44:144-152
Contrary to conventional wisdom, more people with white-collar jobs like systems analysts or engineers die of amyotrophic lateral sclerosis (ALS) or Parkinson disease than farmers or construction workers who are exposed to toxins linked with both diseases.
Jia Liu, Fei Wang
Evaluating the cognitive and behavioral features in amyotrophic lateral sclerosis (ALS) patients is important for therapy and care. Fifty-seven ALS, 5 ALS with the behavioral variant of frontotemporal dementia (FTD) (ALS-FTD), 12 FTD patients, and 35 control subjects were evaluated by 10 different tests for cognitive and behavioral (mini-mental state examination (MMSE), Hasegawa dementia rating scale – revised (HDS-R), frontal assessment battery (FAB), Montreal cognitive assessment (MoCA), ALS-frontotemporal dementia-Questionnaire (ALS-FTD-Q), and anosognosia scale), affective (depression, apathy, and behavioral and ...
The objective of this study is to assess the changes in CMAP scan from abductor pollicis brevis (APB) and abductor digiti minimi (ADM) muscles during the diagnosis and follow up period in amyotrophic lateral sclerosis (ALS) patients.
To study inter- and intra-rater agreement on diagnostic criteria for amyotrophic lateral sclerosis (ALS).
Loss of motor units (MUs) is the primary disease process in degenerative motor neuron diseases like amyotrophic lateral sclerosis (ALS). Reinnervation can compensate for MU loss. The net result is a reduced number of MUs, and increased MU size. The assessment of the number and size of MUs will be essential to study disease progression, and to study the efficacy of treatment.
To compare the sensitivity of MUNE methods to quantitative motor unit potential (MUP) analysis in amyotrophic lateral sclerosis (ALS).