Propriospinal myoclonus: The spectrum of clinical and neurophysiological phenotypes

Antelmi, Elena; Provini, Federica

doi:10.1016/j.smrv.2014.10.007

Cited by 36 publications

(30 citation statements)

References 63 publications

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“…Propriospinal myoclonus (PSM), first described in 1991, has since been increasingly identified as a functional movement disorder in the majority of cases. On the other hand, symptomatic myoclonus has been claimed in about 20% of cases to be due to spinal lesions, neuroinfections, medication or paraneoplastic diseases [ 123 ]. Only in single cases, though, the level of spinal abnormalities corresponded with the start of the myoclonic jerks [ 123 , 124 ].…”

Section: The Anatomical Approach To Categorizationmentioning

confidence: 99%

“…On the other hand, symptomatic myoclonus has been claimed in about 20% of cases to be due to spinal lesions, neuroinfections, medication or paraneoplastic diseases [ 123 ]. Only in single cases, though, the level of spinal abnormalities corresponded with the start of the myoclonic jerks [ 123 , 124 ]. The relevance of spinal cord abnormalities limited to diffusion tensor imaging remains unknown [ 125 ].…”

Section: The Anatomical Approach To Categorizationmentioning

confidence: 99%

“…Jerks may occur spontaneously or stimulus-induced. Myoclonic jerks of 15–5000 ms duration start in the thoracic region and tend to propagate at a rate of 3–15 m/s, producing a repetitive, jerky flexion of the trunk, neck or lower extremities, most often in the supine position [ 123 ].…”

Section: The Anatomical Approach To Categorizationmentioning

confidence: 99%

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Myoclonic Disorders

Eberhardt

Topka

2017

Brain Sciences

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Few movement disorders seem to make a straightforward approach to diagnosis and treatment more difficult and frustrating than myoclonus, due to its plethora of causes and its variable classifications. Nevertheless, in recent years, exciting advances have been made in the elucidation of the pathophysiology and genetic basis of many disorders presenting with myoclonus. Here, we provide a review of all of the important types of myoclonus encountered in pediatric and adult neurology, with an emphasis on the recent developments that have led to a deeper understanding of this intriguing phenomenon. An up-to-date list of the genetic basis of all major myoclonic disorders is presented. Randomized studies are scarce in myoclonus therapy, but helpful pragmatic approaches at diagnosis as well as treatment have been recently suggested.

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Section: The Anatomical Approach To Categorizationmentioning

confidence: 99%

Section: The Anatomical Approach To Categorizationmentioning

confidence: 99%

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Myoclonic Disorders

Eberhardt

Topka

2017

Brain Sciences

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“…Since then, a particular subtype of propriospinal myoclonus has been described in which patients experience jerks exclusively during the transition between wakefulness and sleep, thus causing severe sleep‐onset insomnia (Video 8) . To diagnose propriospinal myoclonus, a specific montage with multichannel EMG is recommended to demonstrate the onset of muscle activation and rostrocaudal propogation …”

Section: Sleep‐related Movement Disordersmentioning

confidence: 99%

“…102 To diagnose propriospinal myoclonus, a specific montage with multichannel EMG is recommended to demonstrate the onset of muscle activation and rostrocaudal propogation. 103 It had been commonly believed that daytime propriospinal myoclonus was linked to various spinal cord pathologies (particularly compressive/ischemic myelopathy or inflammatory lesions), with idiopathic cases also being described. However, it was subsequently found that the EMG pattern of propriospinal myoclonus could be mimicked voluntarily by healthy individuals, 104 and now a significant proportion of patients are believed to have a functional etiology.…”

Section: Propriospinal Myoclonus At Sleep Onsetmentioning

confidence: 99%

Sleep‐related motor and behavioral disorders: Recent advances and new entities

et al. 2018

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Patients with sleep-related motor and behavioral disorders present to a variety of subspecialty clinics (neurology, sleep medicine, respiratory medicine, psychiatry). Diagnosing these disorders can be difficult, and sometimes they have a significant impact on quality of life. Alongside a number of common and well-recognized conditions, several new disease entities have been described in recent years that present with abnormal nocturnal motor phenomena (such as ADCY5-associated disease and anti-IgLON5 disease). Our understanding of the neural basis and prognostic significance of other sleep-related disorders has also grown, particularly rapid eye movement sleep behavior disorder. This review (along with a collection of previously unpublished videos) is intended to aid in the recognition and treatment of these patients. The recent change in terminology from nocturnal frontal lobe epilepsy to sleep-related hypermotor epilepsy is also discussed. © 2018 International Parkinson and Movement Disorder Society.

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Myoclonus: Differential diagnosis and current management

Riva,

D'Onofrio,

Ferlazzo

et al. 2024

Epilepsia Open

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Myoclonus classically presents as a brief (10–50 ms duration), non‐rhythmic jerk movement. The etiology could vary considerably ranging from self‐limited to chronic or even progressive disorders, the latter falling into encephalopathic pictures that need a prompt diagnosis. Beyond the etiological classification, others evaluate myoclonus' body distribution (i.e., clinical classification) or the location of the generator (i.e., neurophysiological classification); particularly, knowing the anatomical source of myoclonus gives inputs on the observable clinical patterns, such as EMG bursts duration or EEG correlate, and guides the therapeutic choices. Among all the chronic disorders, myoclonus often presents itself as a manifestation of epilepsy. In this context, myoclonus has many facets. Myoclonus occurs as one, or the only, seizure manifestation while it can also present as a peculiar type of movement disorder; moreover, its electroclinical features within specific genetically determined epileptic syndromes have seldom been investigated. In this review, following a meeting of recognized experts, we provide an up‐to‐date overview of the neurophysiology and nosology surrounding myoclonus. Through the dedicated exploration of epileptic syndromes, coupled with pragmatic guidance, we aim to furnish clinicians and researchers alike with practical advice for heightened diagnostic management and refined treatment strategies.Plain Language SummaryIn this work, we described myoclonus, a movement characterized by brief, shock‐like jerks. Myoclonus could be present in different diseases and its correct diagnosis helps treatment.

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Propriospinal myoclonus: The spectrum of clinical and neurophysiological phenotypes

Cited by 36 publications

References 63 publications

Myoclonic Disorders

Myoclonic Disorders

Sleep‐related motor and behavioral disorders: Recent advances and new entities

Myoclonus: Differential diagnosis and current management

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