Rippling is not always electrically silent in rippling muscle disease

Takakuni Maki1, Riki Matsumoto, Nobuo Kohara

  • 1Department of Neurology, Graduate School of Medicine, Kyoto University, 54 Kawaharacho, Sakyo-ku, Kyoto 606-8507, Japan.

Muscle & Nerve
|March 16, 2011
PubMed

Insights

Rippling muscle disease (RMD) is a rare myopathy. Electromyography in one patient revealed unique muscle fiber hyperexcitability, differing from previous RMD findings.

Area of Science:

  • Neurology
  • Muscle Physiology
  • Electromyography

Background:

  • Rippling muscle disease (RMD) is a myopathy characterized by muscle hyperirritability.
  • The underlying pathophysiology of RMD remains uncertain.
  • Previous electromyographic findings in RMD cases have varied, including electrical silence or motor unit potentials.

Related Concept Videos

Alterations in Muscle Tone lll01:11

Alterations in Muscle Tone lll

Rigidity and myotonia are distinct abnormalities of muscle tone that affect resistance and relaxation during movement. Although both involve altered muscle contraction, they arise from different neurological and muscular mechanisms.CharacteristicsRigidity is characterized by uniform resistance to passive movement across the entire range, independent of speed, affecting flexors and extensors equally. It may appear as lead-pipe rigidity (smooth, constant resistance) or cogwheel rigidity...
Muscle Stimulation Frequency01:22

Muscle Stimulation Frequency

The contraction strength of muscles is regulated by motor neurons, which modulate the frequency of action potentials dispatched to the motor units based on the body's requirements. This process of varying the muscle stimulation frequency allows muscles to contract with a force that is precisely tailored to the needs of the moment, whether lifting a feather or a heavy box.
Wave summation
At low firing rates, motor neurons induce individual twitch contractions in muscle fibers. These twitches...
REM Sleep Behavior Disorder01:15

REM Sleep Behavior Disorder

REM Sleep Behavior Disorder (RBD) is a sleep disorder characterized by the absence of muscle paralysis that normally occurs during the REM phase of sleep. This absence allows individuals to physically act out their dreams, which are often vivid and disturbing. Common behaviors exhibited during episodes include kicking, punching, and yelling. These actions can be dangerous, potentially leading to injuries for the person with RBD or their bed partner.
RBD is significantly associated with...
Cardiomyopathy IV: Restrictive Cardiomyopathy01:29

Cardiomyopathy IV: Restrictive Cardiomyopathy

Restrictive cardiomyopathy (RCM) is a rare heart muscle disease characterized by impaired ventricular filling due to stiffened ventricular walls, leading to significant diastolic dysfunction.EtiologyRestrictive cardiomyopathy can arise from both inherited and acquired diseases, many of which are systemic. It is categorized into four main types: infiltrative, storage, non-infiltrative, and endomyocardial diseases.Infiltrative diseases, such as amyloidosis, lead to RCM by depositing amyloid...
Relaxation of Skeletal Muscles01:29

Relaxation of Skeletal Muscles

The period of muscle contraction primarily influences the duration of stimulation at the neuromuscular junction (NMJ), the presence of free calcium ions in the sarcoplasm, and the availability of energy or ATP to support contractions.
When an action potential reaches the axon terminal, it depolarizes the membrane and opens voltage-gated sodium channels. Sodium ions enter the cell, further depolarizing the presynaptic membrane. This depolarization causes voltage-gated calcium channels to open.
Pulse rhythm01:30

Pulse rhythm

Pulse rhythm refers to the pattern of pulsations within specific intervals, offering valuable insights into the regularity or irregularity of the heart's beats as observed through the pattern of pulsation within specific intervals. A regular pulse exhibits a consistent heart rate with uniform waveforms and pulsation force, variations of which can be classified as normal, weak, or bounding.
Conversely, an irregular pulse pattern is termed dysrhythmia, stemming from disruptions in cardiac muscle...