Weakness of the Neck Muscles

2018 ◽  
Author(s):  
Aziz Shaibani
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Movement Disorders ◽  
Cervical Dystonia ◽  
Neuromuscular Disorders ◽  
The Elderly ◽  
Central Control ◽  
Lateral Bending ◽  
Flexion Extension ◽  
Differential Involvement ◽  
Lateral Sclerosis

The neck is furnished by tens of muscles for flexion, extension, lateral bending, and rotation. It carries a 10-pound head for at least two-thirds of every day. These muscles are under delicate central control, and they are subject to different central and peripheral malfunctions. Differential involvement of the neck flexors’ Vs extensors helps with the diagnosis of various neuromuscular disorders. Weakness of the cervical extensors leads to head drop, a troubling condition that is caused by many neuromuscular disorders. Movement disorders such as cervical dystonia and Parkinson disease lead to head drop, causing confusion with neuromuscular causes such as myasthenia gravis (MG) and amyotrophic lateral sclerosis (ALS). Head protrusion of the elderly (camptocormia) is a different entity.

Weakness of the Neck Muscles

2015 ◽  
Author(s):  
Aziz Shaibani
Keyword(s):  
Myasthenia Gravis ◽  
Parkinson Disease ◽  
Movement Disorders ◽  
Cervical Dystonia ◽  
Neuromuscular Disorders ◽  
The Elderly ◽  
Central Control ◽  
Lateral Bending ◽  
Flexion Extension ◽  
Differential Involvement

The neck is furnished by dozens ofmuscles for flexion, extension, lateral bending, and rotation. It carries a 10-pound head at least two-thirds of every day. These muscles are under delicate central control, and they are subject to different central and peripheral malfunctions. Differential involvement of the neck flexors versus extensors helps in diagnosing different neuromuscular disorders. Weakness of the cervical extensors leads to head drop, a troubling condition that is caused by many neuromuscular disorders. Movement disorders such as cervical dystonia and Parkinson disease may also lead to head drop, causing confusion with neuromuscular causes such as myasthenia gravis and ALS. Head protrusion of the elderly (camptocormia) is a different entity.

Amyotrophic lateral sclerosis diagnostic index

Neurology ◽  
2019 ◽  
Vol 92 (6) ◽  
pp. e536-e547 ◽  
Author(s):  
Nimeshan Geevasinga ◽  
James Howells ◽  
Parvathi Menon ◽  
Mehdi van den Bos ◽  
Kazumoto Shibuya ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Confidence Interval ◽  
Diagnostic Accuracy ◽  
Neuromuscular Disorders ◽  
Area Under The Curve ◽  
Disease Process ◽  
Diagnostic Utility ◽  
Training Cohort ◽  
Diagnostic Index ◽  
Lateral Sclerosis

ObjectiveThe aim of the study was to assess the utility of a novel amyotrophic lateral sclerosis (ALS) diagnostic index (ALSDI).MethodsA prospective multicenter study was undertaken on patients presenting with suspected ALS. The reference standard (Awaji criteria) was applied to all patients at recruitment. Patients were randomly assigned to a training (75%) and a test (25%) cohort. The ALSDI was developed in the training cohort and its diagnostic utility was subsequently assessed in the test cohort.ResultsA total of 407 patients were recruited, with 305 patients subsequently diagnosed with ALS and 102 with a non-ALS mimicking disorder. The ALSDI reliably differentiated ALS from neuromuscular disorders in the training cohort (area under the curve 0.92, 95% confidence interval 0.89–0.95), with ALSDI ≥4 exhibiting 81.6% sensitivity, 89.6% specificity, and 83.5% diagnostic accuracy. The ALSDI diagnostic utility was confirmed in the test cohort (area under the curve 0.90, 95% confidence interval 0.84–0.97), with ALSDI ≥4 exhibiting 83.3% sensitivity, 84% specificity, and 83.5% diagnostic accuracy. In addition, the diagnostic utility of the ALSDI was confirmed in patients who were Awaji negative at recruitment and in those exhibiting a predominantly lower motor neuron phenotype.ConclusionThe ALSDI reliably differentiates ALS from mimicking disorders at an early stage in the disease process.Classification of evidenceThis study provides Class I evidence that for patients with suspected ALS, the ALSDI distinguished ALS from neuromuscular mimicking disorders.

Articulatory Range of Movement in Individuals With Dysarthria Secondary to Amyotrophic Lateral Sclerosis

2018 ◽  
Vol 27 (3) ◽  
pp. 996-1009 ◽  
Author(s):  
Jimin Lee ◽  
Michael Bell
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Convex Hull ◽  
Speaking Rate ◽  
Range Of Movement ◽  
Lower Lip ◽  
Working Space ◽  
Electromagnetic Articulography ◽  
Differential Involvement ◽  
Lateral Sclerosis ◽  
Anterior Posterior

Purpose The current study examined overall articulatory range of movement (ROM) in individuals with amyotrophic lateral sclerosis (ALS). Differential involvement of articulators was also tested using articulatory working space in individuals with varying degrees of dysarthria severity and in typically aging individuals. A strong association between overall articulatory ROM and severity measures among individuals with ALS was hypothesized. In addition, it was hypothesized that differential involvement of articulators would be detected using overall articulatory ROM measures. Method Twenty-two speakers with dysarthria secondary to ALS and 22 typically aging speakers participated. Speech intelligibility and speaking rate were used as indices of severity. Movement range and overall articulatory working space area (convex hull) of the tongue, lower lip, and jaw were each measured by electromagnetic articulography while the speakers produced the Rainbow Passage. Results Tongue convex hull size was significantly correlated with both indices of severity. A significant interaction between articulators and groups was observed. Individuals with severe dysarthria had reduced tongue convex hull size and exaggerated lower lip convex hull size. ROM in the anterior–posterior dimension showed a more notable differential involvement pattern than ROM in the superior–inferior dimension. Results in the area under a receiver operating characteristic curve analysis revealed group-specific ROM sensitivity. Conclusions The findings indicate that tongue ROM is reduced in individuals with more severe dysarthria when estimated using a standardized paragraph containing all American English phonemes. The articulatory working space measure could be useful for estimating speech dysfunction in ALS. ROM of the tongue decreases, but ROM of the lower lip and jaw each increase in individuals with severe dysarthria. Differential involvement of the articulators in the anterior–posterior dimension needs to be further investigated.

scholarly journals Teaching an Old Dog New Tricks: Serum Troponin T as a Biomarker in Amyotrophic Lateral Sclerosis

2021 ◽  
Author(s):  
Sergio Castro-Gomez ◽  
Barbara Radermacher ◽  
Pawel Tacik ◽  
Sandra R. Mirandola ◽  
Michael T. Heneka ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Troponin I ◽  
Troponin T ◽  
Case Reports ◽  
Neuromuscular Disorders ◽  
University Hospital ◽  
Biological Origin ◽  
Muscle Involvement ◽  
Als Patients ◽  
Lateral Sclerosis

AbstractAmyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease characterized by progressive loss of upper and lower motor neurons. Diagnosis, management and therapeutic trials are hampered by a lack of informative biomarkers. Troponins (Tn) are components of skeletal and cardiac muscles. Acute elevation of cardiac isoforms of troponin I (cTnI) and T (cTnT) in serum indicates myocardial injury. Case reports suggested that serum levels of cTnT, but not cTnI are chronically elevated in ALS and other neuromuscular disorders.Using standard clinical laboratory methodologies we studied serum troponin levels in a multicentric cross-sectional cohort of 75 ALS patients and sixty controls (DESCRIBE-ALS cohort) and in a real-world cohort of 179 consecutive patients from our ALS clinic at the University Hospital Bonn.We found that serum cTnT, is elevated in >60% of ALS patients while cTnI is always normal. Serum cTnT levels increase over time and correlate with disease severity as measured with the revised ALS FRS score. There was no correlation with the phosphorylated neurofilament heavy chain (pNfH) levels in the cerebrospinal fluid. We propose that cTnT elevations in ALS are of non-cardiac origin and may serve as a proxy of lower motor neuron or skeletal muscle involvement. They potentially help to stratify patients according to lower motoneuron involvement. Further research will determine the biological origin of the cTnT elevation and its validity as a diagnostic and/or prognostic marker. Our finding also serves as a reminder to interpret cTnT with caution elevations in patients with neuromuscular diseases.

scholarly journals Automatic amyotrophic lateral sclerosis detection using tunable Q-factor wavelet transform

2020 ◽  
Vol 9 (4) ◽  
pp. 744
Author(s):  
Abdelouahad Achmamad ◽  
Abdelali Belkhou ◽  
Atman Jbari
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Wavelet Transform ◽  
Nearest Neighbor ◽  
Neuromuscular Disorders ◽  
Computation Time ◽  
Support Vector ◽  
Q Factor ◽  
K Nearest Neighbor ◽  
Emg Signal ◽  
Lateral Sclerosis

Early diagnosis of amyotrophic lateral sclerosis (ALS) based on electromyography (EMG) is crucial. The processing of a non-stationary EMG signal requires powerful multi-resolution methods. Our study analyzes and classifies the EMG signals. In the present work, we introduce a novel flexible method for classification of EMG signals using tunable Q-factor wavelet transform (TQWT). Different sub-bands generated by the TQWT technique were served to extract useful information related to energy and then the calculated features were selected using a filter selection (FS) method. The effectiveness of the feature selection step resulted not only in the improvement of classification performance but also in reducing the computation time of the classification algorithm. The selected feature subsets were used as inputs to multiple classifier algorithms, namely, k-nearest neighbor (k-NN), least squares support vector machine (LS-SVM) and random forest (RF) for automated diagnosis. The experimental results show better classification measures with k-NN classifier compared with LS-SVM and RF. The robustness of the classification task was tested using a ten-fold cross-validation method. The outcomes of our proposed approach can be exploited to aid clinicians in neuromuscular disorders detection.

scholarly journals Expanding the Spectrum of Movement Disorders Associated With C9orf72 Hexanucleotide Expansions

2021 ◽  
Vol 7 (2) ◽  
pp. e575
Author(s):  
Carlos Estevez-Fraga ◽  
Francesca Magrinelli ◽  
Davina Hensman Moss ◽  
Eoin Mulroy ◽  
Giulia Di Lazzaro ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Movement Disorders ◽  
Medical Records ◽  
Multiple Comparisons ◽  
Upper Limbs ◽  
Hexanucleotide Repeat ◽  
Repeat Expansions ◽  
Clinical Records ◽  
Lateral Sclerosis

ObjectiveHexanucleotide repeat expansions (HREs) in C9orf72 are a major cause of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS). We aimed to determine the frequency and phenomenology of movement disorders (MD) in carriers of HRE in C9orf72 through a retrospective review of patients' medical records.MethodsWe retrospectively reviewed the clinical records of patients carrying a C9orf72 HRE in the pathogenic range and compared the characteristics of patients with and without MD.ResultsSeventeen of 40 patients with a C9orf72 HRE had a documented MD. In 6 of 17, MD were the presenting symptom, and in 2 of 17, MD were the sole manifestation of the disease. FTD was present in 13 of 17 patients, ALS in 5 of 17 patients, and 2 of 17 patients did not develop FTD or ALS. Thirteen of 17 patients had more than one MD. The most common MD were parkinsonism and tremor (resembling essential tremor syndrome), each one present in 11 of 17 patients. Distal, stimulus-sensitive upper limbs myoclonus was present in 6 of 17 patients and cervical dystonia in 5 of 17 patients. Chorea was present in 5 of 17 patients, 4 of whom showed marked orofacial dyskinesias. The most frequent MD combination was tremor and parkinsonism, observed in 8 of 17 patients, 5 of whom also had myoclonus. C9orf72 patients without MD had shorter follow-up times and higher proportion of ALS, although these results did not survive the correction for multiple comparisons.ConclusionsMD are frequent in C9orf72. They may precede signs of ALS or FTD, or even be present in isolation. Parkinsonism, tremor, and myoclonus are most commonly observed.

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