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Pathophysiology
Published in Ibrahim Natalwala, Ammar Natalwala, E Glucksman, MCQs in Neurology and Neurosurgery for Medical Students, 2022
Ibrahim Natalwala, Ammar Natalwala, E Glucksman
Regarding common clinical examination signs, which of the following statements are true and which are false? Testing the bicep reflex tests the C5, C6 nerve root.The Achilles reflex tests the S4 nerve root.The Moro reflex normally disappears within the first year of life.A normal Babinski response is extensor plantar response and fanning of toes.Fasciculations are a sign of an upper motor neurone lesion.
Progressive Neurological Diseases
Published in Amy J. Litterini, Christopher M. Wilson, Physical Activity and Rehabilitation in Life-threatening Illness, 2021
Christopher M. Wilson, Amy J. Litterini
The typical presentation of ALS symptoms includes persistent weakness and/or spasticity in one or more extremities, with initial presentation often unilateral. Difficulty with swallowing and/or speech may accompany the extremity symptoms. Bowel and bladder dysfunction may also be noted by individuals with ALS, and chronic constipation leads to increased risk for bowel obstruction. Prolonged immobility can also contribute to symptoms of pain for these individuals. Fasciculations are also seen, as well as cognitive and behavioral issues for some people. Risk factors for ALS which are consistently reported include older age, male gender, and tobacco use disorder.33
Diseases of the Peripheral Nerve and Mononeuropathies
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
Diana Mnatsakanova, Charles K. Abrams
Fasciculations are random and spontaneous twitching of a group of muscle fibers or a motor unit that is often grossly visible in a limb or the tongue. A fasciculation potential is a recording often associated with clinical fasciculations. This has the configuration of a motor unit action potential (MUP) but occurs spontaneously. Fasciculation potentials can occur in any nerve disorder, but are most notoriously associated with motor neuron disease.
Adolf Kussmaul (1822–1902), and the naming of “poliomyelitis”
Published in Journal of the History of the Neurosciences, 2022
Nadeem Toodayan, Eric Matteson
In paralysis générale spinale antérieure subaiguë, [I]mmediately after the symptoms of paralysis have appeared, we find a very considerable reduction in the electromotoric excitability, indeed in some muscles the excitability for the strongest currents has disappeared, without the atrophy in them being particularly high. … Not so with progressive muscular atrophy [however, in which] we almost always see the decrease in electrical excitability keep pace with the atrophy of the affected muscle. … It is difficult to say in how far the fibrillar twitchings [referring to fasciculations], which are very frequently observed in progressive muscular atrophy and are hardly observed in paral. général, spin. antér. subaiguë can be used for the differential diagnosis.
Muscle ultrasonography in the diagnosis of amyotrophic lateral sclerosis
Published in Neurological Research, 2020
Wang Juan, Liu Fang, Wen Qi, Ma Jing, Huang Shan, Zhang Jing, Chang Xueli, Zhang Wei, Guo Junhong
Fasciculations are clinically defined as random, spontaneous, and intermittent contractions of a portion of a muscle that does not result in movement of a joint, which can be presented in various neurogenic disorders such as AMAN, SMA, and ALS [15]. Fasciculations also occur in healthy individuals, especially after exercise [16]. However, the distributions of fasciculations vary among different conditions, which is useful for differential diagnosis. Widespread fasciculations are a clinical hallmark of ALS. Several studies indicated that more than 40% of examined muscles showed fasciculations detected by MUS [9,10,17]. Consistent with the previous studies, our results also revealed a high proportion (58.5%). Furthermore, in the present study, two or more anatomic regions with fasciculations were more common in patients with ALS (15/16). Compared to the ALS patients, individuals with ALS mimics only had local fasciculations. Of the 10 patients with ALS mimics in this study, only one had widespread distribution of fasciculations involving 2 or more anatomic regions. Similarly, fasciculations were only sporadically encountered in healthy adults as verified by our data [15]. Furthermore, fasciculation rates in patients with ALS were not affected by the onset sites, subgroups according to the Awaji ALS criteria or disease duration. A previous study had revealed similar results [9]. Therefore, fasciculations detected by MUS can add valuable information in diagnosing ALS.
SOD1-related ALS with anticipation in a large family from Martinique
Published in Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 2021
Anna-Gaelle Giguet-Valard, Rémi Bellance, Séverine Jeannin, Sophie Duclos, Pascale Olive, Oriane Allard-Saint-Albin, Cécile Cazeneuve, Fabienne Clot, Pittion-Vouyovitch Sophie, Thomas Barnetche, Juliette Smith-Ravin, Cyril Goizet
The pedigree showed clear autosomal dominant inheritance of ALS without FTD expression (Figure 1). Onset was predominantly associated with motor expression in lower limbs (5/9), except for 3 cases in whom pseudo-polyneuritic (2/9) and upper limbs signs (2/9) were first observed (Table 1). Initial symptoms were mixed bulbar and motor symptoms in one case. Among initials signs, cramps were frequent (4/9), as muscle atrophy (4/9), fasciculations (3/9) and muscular weakness (3/9). All documented patients shared classical clinical signs of ALS: cramps, fasciculations, and muscle atrophy. Interestingly, survival delay and age at onset of the disease decreases were observed during transmission through generations and clinical course impaired more rapidly in younger generations.