scholarly journals Treatment of progressive paralysis associated with cervical myelopathy and suspected amyotrophic lateral sclerosis: A case report

2021 ◽  
Vol 12 ◽  
pp. 550
Author(s):  
Yoshinori Ishikawa ◽  
Naohisa Miyakoshi ◽  
Takashi Kobayashi ◽  
Toshihiko Kikuchi
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Cervical Myelopathy ◽  
Progressive Disease ◽  
Definitive Diagnosis ◽  
Motor Deficit ◽  
Case Description ◽  
Posterior Decompression ◽  
Needle Electromyography ◽  
Characteristic Features ◽  
Lateral Sclerosis

Background: Amyotrophic lateral sclerosis (ALS) is an intractable progressive disease, with an incidence of 2.2– 2.3 per 100,000 individuals, which is not extremely low. ALS symptoms are accompanied by spinal myeloradicular motor deficit; its differential diagnosis is must because progressive paralysis needs emergency surgery. Case Description: A 64-year-old man with suspected ALS showing progressive paralysis with cervical myelopathy was diagnosed as normal after performing a nerve conduction study preoperatively. Postoperative diffuse fasciculation after posterior decompression allowed the diagnosis of ALS through needle electromyography (EMG). Thereafter, the patient’s condition slowly deteriorated and he died after 16 months. Conclusion: Surgery might aggravate ALS symptoms; however, surgery for progressive paralysis in patients with suspected ALS is required for distinguishing patients with non-ALS paralysis. Approximately 70% of cases have spinal-onset ALS lacking typical cranial nerve symptoms; thus, to prevent unnecessary surgery, surgeons should at least know the characteristic features of ALS and should be aware that early diagnosis requires needle EMG for definitive diagnosis of ALS.

Needle electromyography of bulbar muscles in patients with amyotrophic lateral sclerosis: Evidence of subclinical involvement

Neurology ◽  
1998 ◽  
Vol 51 (5) ◽  
pp. 1417-1422 ◽  
Author(s):  
J. Finsterer ◽  
M. Erdorf ◽  
B. Mamoli ◽  
A. Fuglsang-Frederiksen
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Needle Electromyography ◽  
Lateral Sclerosis

Needle electromyography of the rectus abdominis in patients with amyotrophic lateral sclerosis

2007 ◽  
Vol 35 (3) ◽  
pp. 383-385 ◽  
Author(s):  
Yingsheng Xu ◽  
Juyang Zheng ◽  
Shuo Zhang ◽  
Dexuan Kang ◽  
Jun Zhang ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Rectus Abdominis ◽  
Needle Electromyography ◽  
Lateral Sclerosis

scholarly journals Oral motor functions, speech and communication before a definitive diagnosis of amyotrophic lateral sclerosis

2016 ◽  
Vol 61 ◽  
pp. 97-105 ◽  
Author(s):  
Tanja Makkonen ◽  
Anna-Maija Korpijaakko-Huuhka ◽  
Hanna Ruottinen ◽  
Riitta Puhto ◽  
Kirsi Hollo ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Definitive Diagnosis ◽  
Motor Functions ◽  
Oral Motor ◽  
Lateral Sclerosis

Needle electromyography of the frontalis muscle in patients with amyotrophic lateral sclerosis

2016 ◽  
Vol 54 (6) ◽  
pp. 1093-1096 ◽  
Author(s):  
Hua Pan ◽  
Fan Jian ◽  
Jinxi Lin ◽  
Na Chen ◽  
Zaiqiang Zhang ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Frontalis Muscle ◽  
Needle Electromyography ◽  
Lateral Sclerosis

scholarly journals Amyotrophic lateral sclerosis and the innate immune system: protocol for establishing a biobank and statistical analysis plan

BMJ Open ◽  
2020 ◽  
Vol 10 (8) ◽  
pp. e037753
Author(s):  
Anne-Lene Kjældgaard ◽  
Katrine Pilely ◽  
Karsten Skovgaard Olsen ◽  
Anne Øberg Lauritsen ◽  
Stephen Wørlich Pedersen ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Neurons ◽  
Complement System ◽  
Progressive Disease ◽  
Statistical Analysis Plan ◽  
Skeletal Musculature ◽  
Registration Number ◽  
The Complement System ◽  
Control Study ◽  
Lateral Sclerosis

IntroductionAmyotrophic lateral sclerosis (ALS) is a devastating, progressive disease that causes degeneration of the motor neurons leading to paresis of the bulbar and the skeletal musculature. The pathogenesis of ALS remains unknown. We will test the hypothesis that the complement system is involved in the pathophysiology of ALS. This protocol article describes our efforts to establish a national Danish ALS biobank. The primary aim is to obtain biological material from patients with ALS for the current study as well as for future studies.Methods and analysisWe intend to establish an observational ALS biobank; some of the material from this biobank will be used for a prospective, observational case–control study. The participants are patients with ALS, neurologically healthy controls and non-ALS neurological controls. Each participant consents to be interviewed and to donate blood and cerebrospinal fluid to the biobank. Analysis of the complement system will be carried out on the three groups of patients and compared.Ethics and disseminationThe project has been approved by the Committees on Health Research Ethics in the Capital Region of Denmark (Approval number H-16017145) and the Danish Data Protection Agency (file number 2012-58-0004). All results will be published in peer-reviewed, medical journals and presented at scientific conferences.Trial registration numberNCT02869048

Myopathy and Motor Neuron Disorders

2019 ◽  
pp. 171-210
Author(s):  
Christopher H. Hawkes ◽  
Kapil D. Sethi ◽  
Thomas R. Swift
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Differential Diagnosis ◽  
Muscular Dystrophy ◽  
Motor Neuron ◽  
Hereditary Spastic Paraplegia ◽  
Periodic Paralysis ◽  
Myasthenic Syndrome ◽  
Mcardle's Syndrome ◽  
Characteristic Features ◽  
Lateral Sclerosis

The first part of this chapter deals with the main varieties of muscular dystrophy and their differential diagnosis, many of which are instantly recognizable. Handles are given for the main neuromuscular junction disorders including, myasthenia gravis and Lambert-Eaton myasthenic syndrome. Inflammatory and dysthyroid myopathies are evaluated. Also appraised are periodic paralysis, McArdle’s syndrome, and mitochondrial, distal, and medication-induced myopathy. In the second section, motor neuron disorders are discussed, in particular the characteristic features of amyotrophic lateral sclerosis and its mimics as well as various forms of bulbar palsy. Hereditary spastic paraplegia and its variants are discussed and how they can simulate other diseases.

scholarly journals Aβ1-42 and Tau as Potential Biomarkers for Diagnosis and Prognosis of Amyotrophic Lateral Sclerosis

2020 ◽  
Vol 21 (8) ◽  
pp. 2911 ◽  
Author(s):  
Débora Lanznaster ◽  
Rudolf C. Hergesheimer ◽  
Salah Eddine Bakkouche ◽  
Stephane Beltran ◽  
Patrick Vourc’h ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Definitive Diagnosis ◽  
Clinical Markers ◽  
Biomarker Identification ◽  
Total Tau ◽  
Diagnosis And Prognosis ◽  
Highly Active ◽  
Csf Levels ◽  
Als Patients ◽  
Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) is the most common motor neuron disease, but its definitive diagnosis delays around 12 months. Although the research is highly active in the biomarker field, the absence of specific biomarkers for diagnosis contributes to this long delay. Another strategy of biomarker identification based on less specific but sensitive molecules may be of interest in clinical practice. For example, markers related to other neurodegenerative diseases such as Alzheimer’s disease (AD) could be fully explored. Here, we compared baseline levels of amyloidβ1-42 (Aβ1-42), total Tau, and phosphorylated-Tau (phospho-Tau) protein in the cerebrospinal fluid (CSF) of ALS patients to controls and correlated it with clinical parameters of ALS progression collected over 12 months. We observed increased levels of Aβ1-42 (controls: 992.9 ± 358.3 ng/L; ALS: 1277.0 ± 296.6 ng/L; p < 0.0001) and increased Aβ1-42/phospho-Tau ratio and Innotest Amyloid Tau Index (IATI) (both p < 0.0001). IATI and the phospho-Tau/total Tau ratio correlated positively with ALSFRS-R and weight at baseline. Multivariate analysis revealed that baseline ALSFRS-R was associated with Aβ1-42 and phospho-Tau/total Tau ratio (p = 0.0109 and p = 0.0013, respectively). Total Tau and phospho-Tau levels correlated negatively with ALSFRS-R variation at months 6 and 9, respectively (p = 0.02 and p = 0.04, respectively). Phospho-Tau/total Tau ratio correlated positively with ALSFRS-R variation at month 9 (p = 0.04). CSF levels of Aβ1-42 could be used as a complementary tool to ALS diagnosis, and total Tau and phospho-Tau levels may help establishing the prognosis of ALS. Further studies merit exploring the pathophysiological mechanisms associated with these markers. Despite their lack of specificity, phospho-Tau/total Tau and Aβ1-42 should be combined to other biological and clinical markers in order to improve ALS management.

Automated Acoustic Analysis of Oral Diadochokinesis to Assess Bulbar Motor Involvement in Amyotrophic Lateral Sclerosis

2020 ◽  
Vol 63 (1) ◽  
pp. 59-73 ◽  
Author(s):  
Panying Rong
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Acoustic Analysis ◽  
Speaking Rate ◽  
Disease Stage ◽  
Healthy Controls ◽  
Tongue Movement ◽  
Major Barrier ◽  
Syllable Repetition ◽  
Oral Diadochokinesis ◽  
Lateral Sclerosis

Purpose The purpose of this article was to validate a novel acoustic analysis of oral diadochokinesis (DDK) in assessing bulbar motor involvement in amyotrophic lateral sclerosis (ALS). Method An automated acoustic DDK analysis was developed, which filtered out the voice features and extracted the envelope of the acoustic waveform reflecting the temporal pattern of syllable repetitions during an oral DDK task (i.e., repetitions of /tɑ/ at the maximum rate on 1 breath). Cycle-to-cycle temporal variability (cTV) of envelope fluctuations and syllable repetition rate (sylRate) were derived from the envelope and validated against 2 kinematic measures, which are tongue movement jitter (movJitter) and alternating tongue movement rate (AMR) during the DDK task, in 16 individuals with bulbar ALS and 18 healthy controls. After the validation, cTV, sylRate, movJitter, and AMR, along with an established clinical speech measure, that is, speaking rate (SR), were compared in their ability to (a) differentiate individuals with ALS from healthy controls and (b) detect early-stage bulbar declines in ALS. Results cTV and sylRate were significantly correlated with movJitter and AMR, respectively, across individuals with ALS and healthy controls, confirming the validity of the acoustic DDK analysis in extracting the temporal DDK pattern. Among all the acoustic and kinematic DDK measures, cTV showed the highest diagnostic accuracy (i.e., 0.87) with 80% sensitivity and 94% specificity in differentiating individuals with ALS from healthy controls, which outperformed the SR measure. Moreover, cTV showed a large increase during the early disease stage, which preceded the decline of SR. Conclusions This study provided preliminary validation of a novel automated acoustic DDK analysis in extracting a useful measure, namely, cTV, for early detection of bulbar ALS. This analysis overcame a major barrier in the existing acoustic DDK analysis, which is continuous voicing between syllables that interferes with syllable structures. This approach has potential clinical applications as a novel bulbar assessment.

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