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Query: UMLS:C0002736 (amyotrophic lateral sclerosis)
 19,048 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The concurrence of amyotrophic lateral sclerosis (ALS) and multiple sclerosis (MS) is extremely rare. We report on a 55-year-old female patient presenting with a progressive gait disorder for 6 months and a speech disorder for 3 months. Neurological examination revealed a spastic paraparesis and mild dysarthria and dysphagia. Technical and laboratory investigations met the diagnostic criteria for MS: magnetic resonance imaging showed multiple periventricular white matter and cervical lesions; cerebrospinal fluid showed a typical autoimmune response. Within the following 3 months generalized fasciculations, atrophy of the small hand muscles and bulbar signs were noticed. Nerve conduction studies revealed acute and chronic signs of denervation in all limbs without nerve conduction block. Hence clinical and paraclinical examination met the El Escorial criteria for ALS. Although myelitic lesions in the anterior horn cells may lead to peripheral segmental denervation, the generalized denervation suggested the unusual coincident combination of ALS and MS in this patient. In clinical praxis motoneuron diseases should also be considered in patients with pronounced peripheral denervations once "definite" MS has been diagnosed.
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PMID:[Simultaneous presentation of amyotrophic lateral sclerosis and multiple sclerosis]. 1787 78

Almost all people with amyotrophic lateral sclerosis (ALS) experience a motor speech disorder, such as dysarthria, as the disease progresses. At some point, 80 to 95% of people with ALS are unable to meet their daily communication needs using natural speech. Unfortunately, once intelligibility begins to decrease, speech performance often deteriorates so rapidly that there is little time to implement an appropriate augmentative and alternative communication (AAC) intervention; therefore, appropriate timing of referral for AAC assessment and intervention continues to be a most important clinical decision-making issue. AAC acceptance and use have increased considerably during the past decade. Many people use AAC until within a few weeks of their deaths.
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PMID:Communication Support for People with ALS. 2160 29

Amyotrophic lateral sclerosis (ALS) is a progressive debilitating neurological disease. ALS disturbs the quality of life by affecting speech, swallowing and free mobility of the arms without affecting intellectual function. It is therefore of significance to improve intelligibility and quality of speech sounds, especially for ALS patients with slowly progressive courses. Currently, however, there is no effective or established approach to improve speech disorder caused by ALS. We investigated a surgical procedure to improve speech disorder for some patients with neuromuscular diseases with velopharyngeal closure incompetence. In this study, we performed the surgical procedure for two patients suffering from severe speech disorder caused by slowly progressing ALS. The patients suffered from speech disorder with hypernasality and imprecise and weak articulation during a 6-year course (patient 1) and a 3-year course (patient 2) of slowly progressing ALS. We narrowed bilateral lateral palatopharyngeal wall at velopharyngeal port, and performed this surgery under general anesthesia without muscle relaxant for the two patients. Postoperatively, intelligibility and quality of their speech sounds were greatly improved within one month without any speech therapy. The patients were also able to generate longer speech phrases after the surgery. Importantly, there was no serious complication during or after the surgery. In summary, we performed bilateral narrowing of lateral palatopharyngeal wall as a speech surgery for two patients suffering from severe speech disorder associated with ALS. With this technique, improved intelligibility and quality of speech can be maintained for longer duration for the patients with slowly progressing ALS.
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PMID:Surgical improvement of speech disorder caused by amyotrophic lateral sclerosis. 2317 42

We report an autopsy case of globular glial tauopathy (GGT) presenting clinically with amyotrophic lateral sclerosis (ALS) with dementia. A 79-year-old female developed weakness in the right upper limb, which progressed gradually. She developed apathy and speech disorder at 80 years of age. On neurological examination, she showed signs of upper and lower motor neuron disorder and dementia, but no extrapyramidal signs. The clinical diagnosis was ALS with dementia. The autopsy revealed left predominant marked atrophy of the frontal lobe due to severe neuronal loss and Gliosis. Immunohistochemistry using anti-4-repeat tau antibody revealed numerous globular glial inclusions. Severe neurodegeneration in the primary motor cortex and corticospinal tract was observed. There were distinctive tau-positive inclusions in both Betz and anterior horn cells. TDP-43-positive inclusions in motor neurons were not detected. Sequence analysis of the tau gene revealed no mutations in exons 1-5, 7, 9-13, or the adjacent intronic sequences. GGT can cause a clinical phenotype of ALS with dementia. (Received December 28, 2015; Accepted February 23, 2016; Published August 1, 2016).
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PMID:[An Autopsy Case of Globular Glial Tauopathy Presenting with Amyotrophic Lateral Sclerosis with Dementia]. 2750 23

Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurological disease that affects the speech motor functions, resulting in dysarthria, a motor speech disorder. Speech and articulation deterioration is an indicator of the disease progression of ALS; timely monitoring of the disease progression is critical for clinical management of these patients. This paper investigated machine prediction of intelligible speaking rate of nine individuals with ALS based on a small number of speech acoustic and articulatory samples. Two feature selection techniques - decision tree and gradient boosting - were used with support vector regression for predicting the intelligible speaking rate. Experimental results demonstrated the feasibility of predicting intelligible speaking rate from only a small number of speech samples. Furthermore, adding articulatory features to acoustic features improved prediction performance, when decision tree was used as the feature selection technique.
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PMID:Predicting Intelligible Speaking Rate in Individuals with Amyotrophic Lateral Sclerosis from a Small Number of Speech Acoustic and Articulatory Samples. 2942 54

Dysarthria is commonly understood as a motor speech disorder characterized by symptoms that are framed as physiologically or acoustically measurable. The effects of dysarthria on social interaction through conversation have been reported but, in comparison with physical measures, remain relatively unexplored. Other-initiated repair sequences are particularly common in dysarthria-in-interaction, drawing attention to the actions of both participants in managing (un)intelligibility, rather than the behavior(s) of the person with dysarthric speech in isolation. These sequences merit detailed and ongoing investigation as they enable us to understand how dysarthria impacts on conversation and, critically, how participants attempt to manage difficulties when they arise. This study explores the organization of other-initiated repair sequences in a dyad where one participant has severe dysarthria arising from amyotrophic lateral sclerosis (ALS) also known as MND. Two hours of recordings were collected on four dates over a 12-month period with the data presented here from recording two. The evidence shows that the participants were able to resolve their troubles, but it required extensive work to both identify the trouble sources and to unravel the problems to reach a satisfactory understanding. The interactions presented in this paper reveal an important limitation of other initiation of repair. Physical restrictions were seen to play an important part in the dysarthric speaker's ability to position his talk in sequential context and successfully accomplish self-repair; particularly, third-turn repair. The present study has offered a depiction of layered conversational problems that other-initiation of repair may not completely resolve or, in some cases, multiply.
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PMID:Dysarthria and other-initiated repair in everyday conversation. 3189 71