Abnormal gating of axonal slow potassium current in cramp-fasciculation syndrome

Shimatani, Yoshimitsu; Nodera, Hiroyuki; Shibuta, Yoshiko; Miyazaki, Yoshitsugu; Misawa, Sonoko; Kuwabara, Satoshi; Kaji, Ryuji

doi:10.1016/j.clinph.2014.09.013

Cited by 12 publications

(8 citation statements)

References 44 publications

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“…A previous study by Kiernan and Bostock [34] did not detect these alterations, probably because they did not use the extended stimulation protocol with prolonged and stronger hyperpolarizing conditioning pulses used by Czesnik and coworkers [35]. Shimatani et al [37] reported a change in slow potassium channel activity in four patients with frequent cramps, but since motor unit multiplets were illustrated in their electromyography (EMG) recordings it is suggested that their patients had neuromyotonia, an autoimmune disorder, rather than ICFS.…”

Section: Idiopathic Cramp-fasciculation Syndromementioning

confidence: 97%

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Muscular cramp: causes and management

Swash

Czesnik

Carvalho

2018

Euro J of Neurology

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Muscular cramp is a common symptom in healthy people, especially among the elderly and in young people after vigorous or peak exercise. It is prominent in a number of benign neurological syndromes. It is a particular feature of chronic neurogenic disorders, especially amyotrophic lateral sclerosis. A literature review was undertaken to understand the diverse clinical associations of cramp and its neurophysiological basis, taking into account recent developments in membrane physiology and modulation of motor neuronal excitability. Many aspects of cramping remain incompletely understood and require further study. Current treatment options are correspondingly limited.

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Section: Idiopathic Cramp-fasciculation Syndromementioning

confidence: 97%

“…Shimatani et al . reported a change in slow potassium channel activity in four patients with frequent cramps, but since motor unit multiplets were illustrated in their electromyography (EMG) recordings it is suggested that their patients had neuromyotonia, an autoimmune disorder, rather than ICFS.…”

Section: Idiopathic Cramp‐fasciculation Syndromementioning

confidence: 99%

Muscular cramp: causes and management

Swash

Czesnik

Carvalho

2018

Euro J of Neurology

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“…Cramps may also develop during high-rate repetitive nerve stimulation 66. Benign fasciculations and cramp-fasciculation syndrome can be regarded as part of a larger spectrum of disease that also incorporates acquired auto-immune neuromyotonia 67. Indeed, antibodies to the voltage-gated potassium channel complex have been identified in 32% of patients with cramp-fasciculation syndrome,68 including leucine-rich glioma-inactivated-1 antibodies in a small, tested subset of patients.…”

Section: Benign Fasciculation and Cramp Fasciculation Syndromesmentioning

confidence: 99%

Fasciculation in amyotrophic lateral sclerosis: origin and pathophysiological relevance

Carvalho

Kiernan

Swash

2017

J Neurol Neurosurg Psychiatry

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This review considers the origin and significance of fasciculations in neurological practice, with an emphasis on fasciculations in amyotrophic lateral sclerosis (ALS), and in benign fasciculation syndromes. Fasciculation represents a brief spontaneous contraction that affects a small number of muscle fibres, causing a flicker of movement under the skin. While an understanding of the role of fasciculation in ALS remains incomplete, fasciculations derive from ectopic activity generated in the motor system. A proximal origin seems likely to contribute to the generation of fasciculation in the early stages of ALS, while distal sites of origin become more prominent later in the disease, associated with distal motor axonal sprouting as part of the reinnervation response that develops secondary to loss of motor neurons. Fasciculations are distinct from the recurrent trains of axonal firing described in neuromyotonia. Fasciculation without weakness, muscle atrophy or increased tendon reflexes suggests a benign fasciculation syndrome, even when of sudden onset. Regardless of origin, fasciculations often present as the initial abnormality in ALS, an early harbinger of dysfunction and aberrant firing of motor neurons.

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“…The threshold is related to membrane potential, as membrane hyperpolarization increases and depolarization decreases the threshold. Additional factors such as nerve ischemia may also impact on threshold, and, consequently, assessment of axonal [136,137] I/V = current threshold relationship; TEh = threshold electrotonus (hyperpolarizing direction); TEd = threshold electrotonus (depolarizing direction); RRP = relative refractory period excitability parameters (discussed later) is required to provide a clear depiction of membrane excitability [21][22][23][24][25].…”

Section: The Clinical Assessment Of Axonal Excitabilitymentioning

confidence: 99%

Axonal Excitability in Amyotrophic Lateral Sclerosis

2017

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Axonal excitability testing provides in vivo assessment of axonal ion channel function and membrane potential. Excitability techniques have provided insights into the pathophysiological mechanisms underlying the development of neurodegeneration and clinical features of amyotrophic lateral sclerosis (ALS) and related neuromuscular disorders. Specifically, abnormalities of Na and K conductances contribute to development of membrane hyperexcitability in ALS, thereby leading to symptom generation of muscle cramps and fasciculations, in addition to promoting a neurodegenerative cascade via Ca-mediated processes. Modulation of axonal ion channel function in ALS has resulted in significant symptomatic improvement that has been accompanied by stabilization of axonal excitability parameters. Separately, axonal ion channel dysfunction evolves with disease progression and correlates with survival, thereby serving as a potential therapeutic biomarker in ALS. The present review provides an overview of axonal excitability techniques and the physiological mechanisms underlying membrane excitability, with a focus on the role of axonal ion channel dysfunction in motor neuron disease and related neuromuscular diseases.

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Abnormal gating of axonal slow potassium current in cramp-fasciculation syndrome

Cited by 12 publications

References 44 publications

Muscular cramp: causes and management

Muscular cramp: causes and management

Fasciculation in amyotrophic lateral sclerosis: origin and pathophysiological relevance

Axonal Excitability in Amyotrophic Lateral Sclerosis

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