Any interruption in neural pathways of the central nervous system such as brain or spinal cord can cause spinal myoclonus. It is characterized by painless, repetitive jerking of the trunk, neck, hips, and knees. Myoclonus is a hyperkinetic movement disorder defined as brief, shocklike jerks caused by muscular contractions or inhibitions. Myoclonus is defined as a sudden, brief, shocklike, involuntary movement due to either active muscular contraction positive myoclonus or inhibition of muscle activity negative myoclonus 1. Periodic limb movement disorder what is periodic limb movement disorder plmd. The jerking may be spontaneous but may also occur in response to a stimulus. An elderly gentleman presented with distressing jerks of both lower limbs which caused him much social. In this issue we report a rare case of spinal myoclo spinal myoclonus in subacute combined degeneration caused by nitrous oxide intoxication meishian wu1,2, yawdon hsu1, jiannchyun lin1, shihcheng chen1,3, and jiunntay lee1 abstract a 26yearold patient developed ascending weakness and paresthesias. In the course of examining spinal motor function in many hundreds of people with traumatic spinal cord injury, we encountered 6 individuals who developed involuntary and rhythmic contractions in muscles of their legs. Spinal segmental myoclonus in both legs associated with antibodies to glycine receptors neurology clinical practice. Potential publication bias was evaluated through a funnel map. Spinal myoclonus can be caused by trauma, spondylosis, tumors, infections, myelitis, or ischemia 1,2. Appropriate therapy for myoclonus depends on the etiology, and in some cases, myoclonus can improve when the provoking cause is eliminated.
The contractions are nearly rhythmic, and may be synchronous in several muscles. Segmental myoclonus hyperkinetic movement disorders. When the underlying cause for the movements is not. In the fourth, a segment of thoracic spinal cord is narrow and probably atrophic. The latter is usually due to a thoracic generator producing truncal flexion jerk. The myoclonus is often stimulussensitive and rhythmic hz and may occur during sleep as well as wakefulness. Periodic limb movement disorder plmd is a condition that was formerly called sleep myoclonus or nocturnal myoclonus. Spinal myoclonus following combined spinalepidural. This is something i really wanted to do as when i was suffering with it there really wasnt much. Propriospinal myoclonus psm is a rare hyperkinetic movement disorder involving the axial muscles.
Progressive myoclonus epilepsy pme is a group of conditions involving the central nervous system and representing more than a dozen different diseases. These diseases share certain features, including a worsening of symptoms over time and the presence of both muscle contractions myoclonus and seizures epilepsy. We describe a patient with spinal segmental myoclonus as a rare presentation of multiple sclerosis ms. For example, reaching for an object or taking a step may trigger jerks that disrupt the movement. It is a reaction to a stimulus on a specific segment of the spinal cord. The mechanism does not appear to be mediated through opioid receptors 15 although the spinal cord may be involved 16.
In our patient, a focal morphologic change that consisted of vacuolar degeneration and chromatolysis of anterior horn cells was found at the levels of the spinal cord corresponding to the involved muscles. Myoclonus describes a movement disorder characterised by brief, abrupt and involuntary contractions of muscles or groups of muscles, usually associated with intracranial lesions, with limited evidence linking it to spinal pathologies. Spinal segmental myoclonus masquerading as a psychogenic. Spinal myoclonus is described as a rare condition but can result from any spinal trauma, spinal tumor, peripheral neuropathy, even an epidural for labor and delivery.
Myoclonus is a brief less than half a second contraction involving agonist and antagonist muscles, leading to a sudden jerk. Spinal segmental myoclonus ssm is characterized by involuntary, semirhythmic contractions of skeletal muscle groups innervated by a limited spinal cord region. Only 3 cases have been reported in children so far. Spinal myoclonus in subacute combined degeneration. Recent advances in medications and surgical therapy for neurological disorders may offer new therapeutic options for the treatment of myoclonus. The neuroanatomical localization of myoclonus spans the cns with origins in the cortex, brainstem, or spinal cord. A novel diagnostic approach to patients with myoclonus. In this video i explain to you what segmental spinal myoclonus is from my experience.
Segmental myoclonus is a type of myoclonus which is based on anatomical distribution. Myoclonus is a hyperkinetic movement disorder characterized by brief, involuntary muscular jerks. The pathophysiology of spinal myoclonus is extensive and multifactorial. Case report open access spinal segmental myoclonus as an unusual presentation of multiple sclerosis raed abdullah alroughani1,2, samar farouk ahmed3,4, riyadh ahmed khan3 and jasem yousef alhashel3,5 abstract background. Spinal segmental myoclonus in both legs associated with. Spinal segmental myoclonus masquerading as a psychogenic movement disorder laxmi khanna1, anuradha batra1 and ankita sharma2 1 department of neurology and neurophysiology, sir gangaram hospital, new delhi, india 2department of neurophysiology, sir gangaram hospital, new delhi, india corresponding author. Electrophysiologic studies for example, eeg, multichannel surface emg, longlatency emg responses to nerve stimulation, eegemg backaveraging, and. Propriospinal myoclonus psm is a rare movement disorder first described in 1991. It may be a normal phenomenon, as in the socalled sleep starts. Propriospinal myoclonus is idiopathic and has occurred following spinal cord lesions, spinal trauma, drug use, tumor, or infections. Response of spinal myoclonus to a combination therapy of.
Spinal segmental myoclonus as an unusual presentation of multiple sclerosis. Contrast induced spinal myoclonus after percutaneous. Opioid induced neuroexcitation has been reproduced and studied extensively in animals 1418. It may occur spontaneously or be triggered by a stimulus, such as a sudden noise, light, or a movement. The topological correlation between the emg muscle activities and the mri findings was consistent with spinal myoclonus arising from the thoracic spinal cord. Concerning the mechanism of propriospinal myoclonus, they suggested that damage to the more rostral spinal cord, particular the cervical cord, may be an important factor to release the myoclonic generators in the thoracic level6. Although there are many reports of unusual muscle activation patterns associated with different forms of myoclonus, we believe that. If treatment of the underlying disorder is not possible, treatment of symptoms is worthwhile, although limited by sideeffects and a lack of controlled evidence. The final diagnosis was combination of spinal segmental myoclonus and propriospinal myoclonus accompanied by features of psychosomatic disorders. Infection, intramedullary and extramedullary spaceoccupying lesions, trauma, vascular. Spinal myoclonus associated with intrathecal bupivacaine. Unusual presentations of multiple sclerosis ms at onset may post a diagnostic dilemma to the treating neurologists.
Spinal myoclonus is further divided into segmental and propriospinal. Subcortical myoclonus is generated between the cortex and spinal cord, a part of these cases. Spinal myoclonus sometimes arises from a certain spinal segment and slowly spreads rostrally as well as caudally, probably being conducted through the proprio spinal tract propriospinal myoclonus. Unusual presentations of multiple sclerosis ms at onset may post a diagnostic dilemma to the. Antibodies to glycine receptors were associated with synchronized myoclonus in both legs and the myoclonus derived from not only the brainstem but also the spine. Myoclonus brain, spinal cord, and nerve disorders msd. Nervous system an unusual case of spinal myoclonus has been reported after subarachnoid anesthesia with bupivacaine 28 a. It is characterized by repetitive limb movements that occur during sleep and cause sleep disruption. Spinal myoclonus with vacuolar degeneration of anterior. Coherent cortical and muscle discharge in cortical myoclonus brain. Spinal myoclonus may pose a diagnostic challenge when it presented at the disease onset and especially in patients with progressive course at onset. Spinal myoclonus is rare in ms and may lead to perform extensive investigations to rule out other etiologies affecting the spinal cord.
Segmental myoclonus is a rare movement disorder characterized by myoclonic involvement of a muscle or a group of muscles supplied by a few contiguous segments of the brain stem or spinal. There are few previously reported cases of spinal myoclonus, all different in various respects fromthe present one, which is reminiscent ofthe results ofexperimental inoculation ofvirus into feline spinal cord. Spinal segmental myoclonus as an unusual presentation of. The condition is characterized by involvement of areas of the body that are near or next to each other. Jerks affect muscles innervated by one or two neighbouring spinal segments myotomes. Spinal myoclonus appears as stimulussensitive, repetitive jerks in a group of muscles supplied by one or several contiguous segments of the brainstem or spinal cord, and unlike other forms of myoclonus, is unaffected by sleep, anesthesia, or coma. Two patients are described who developed action, reflex myoclonus during acute renal failure. Pdf segmental spinal myoclonus and metastatic cervical. Fourchannel needle electromyography shows rhythmic bursts of 256 to 400 ms of muscle activity in the intercostal muscles of the 3 rd space channel 2. Pdf spinal myoclonus following combined spinal and. Spinal myoclonus is a rare entity in which myoclonic movements occur in muscles originating from few segmental, or many adjacent spinal motor roots propriospinal. This movement is caused by a central nervous system generator which arises from circumscribed interneuron pools along the craniospinal neuraxis that are normally concerned with segmental.
Pdf segmental spinal myoclonus rarely occurs in association with spinal cord tumor. Segmental myoclonus is defined as rhythmic or semi. Recognition of myoclonus and determination of the underlying aetiology remains challenging given. Propriospinal myoclonus psm is a rare hyperkinetic movement disorder involving the axial. Myoclonus fact sheet national institute of neurological. This suggests that the pyramidal tract and spinal motor neurons transmit information faithfully at a. To our knowledge, previous studies of patients with spinal myoclonus have not reported a pathologic correlation.
However, a specific cause is identified in only 20% of patients. Stimulus sensitive myoclonus is a type of myoclonus triggered by outside stimuli such as lights, noise, or movement. A 63yearold man underwent spinal anesthesia with bupivacaine 15 mg for transurethral resection of the prostate and soon after complained of severe perineal pain and involuntary tonicclonic movements of the legs. We report a case of spinal myoclonus induced by the tip of an intrathecal catheter in a 35yearold patient with severe, adultonset, generalized dystonia of unknown cause, treated for 2 years.
Although considered not to be a case of psychogenic myoclonus, a psychogenic factor was definitely involved in the induction of the organic myoclonus. Spinal myoclonus spinal segmental myoclonus spinal segmental myoclonus originates in the spinal cord, and is generated by pacemaker circuits table 2 causes of cortical, subcortical and mixed myoclonus. The most common cause of segmental myoclonus is damage to the spinal. Propriospinal myoclonus psm is a rare disorder with repetitive, usually flexor arrhythmic brief jerks of the trunk, hips, and knees in a fixed pattern. Spinal generated movement disorders sgmds include spinal segmental myoclonus, propriospinal myoclonus, orthostatic tremor, secondary paroxysmal dyskinesias, stiff person syndrome and its variants, movements in brain death, and painful legsmoving toes syndrome.
In two, the myoclonus is associated with severe cervical spondylitis. When pathological, myoclonus is a symptom of a broad range of neurological and systemic diseases. Spinal segmental myoclonus originates in the spinal cord, and is generated by pacemaker circuits involved in locomotion. We described a 31yearold male who presented with involuntary brief jerky movements of the left shoulder and. Patients with cortical myoclonus have involuntary muscle jerks due to the synchronous discharge of many motor units. Myoclonus may develop in response to infection, head or spinal cord injury, stroke, brain tumors, kidney or liver failure, lipid storage disease, chemical or drug poisoning, or other disorders.
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