Propriospinal myoclonus associated with fragile-X-premutation and response to selective serotonin reuptake inhibitor

Wong, Sui; Selvan, Arul; White, Richard P.

doi:10.1016/j.parkreldis.2011.04.009

Cited by 4 publications

(5 citation statements)

References 4 publications

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“…In around 80% of cases, PSM appears to be idiopathic, the remainder comprising patients with a broad range of medical conditions, including spinal lesions, namely cervical hemangioblastoma or cervical herniations, syringomyelia and dural arteriovenous fistula [1, 20,21], paraneoplastic condition [18], paraproteinemic neuropathy [22], demyelinating lesions [23,24], thoracic herpes zoster [2], HIV infection [25], Lyme neuroborreliosis [26], fragile X permutation [27], celiac disease [28] and as a possible complication of an enteropathogenic toxin [29]. Different pharmacological treatments have also been associated with PSM onset, i.e., interferon-alpha [30], intrathecal bupivacaine [31,32], ciprofloxacin [33] and cannabis [34].…”

Section: Clinical Featuresmentioning

confidence: 99%

“…1a and 2) or less frequently in cervical muscles such as the sternocleidomastoideus (SCM). Cranial muscles are usually not involved, even though a few electromyographic recordings have shown a slight activation of the masseter [22,27,36,39]. Sometimes the jerks remain localized in the single segmental muscle where the propagation usually starts [3,4,35].…”

Section: Clinical Featuresmentioning

confidence: 99%

“…However, many of these reports did not check the data with the subject's state of mental activation. A few individual cases have been reported in which the movement seemed to be elicited by standing or changing position [27,30,41,42]. 14,15].…”

Section: Clinical Featuresmentioning

confidence: 99%

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Propriospinal myoclonus: The spectrum of clinical and neurophysiological phenotypes

Antelmi¹,

Provini²

2015

Sleep Medicine Reviews

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Section: Clinical Featuresmentioning

confidence: 99%

Section: Clinical Featuresmentioning

confidence: 99%

See 1 more Smart Citation

Propriospinal myoclonus: The spectrum of clinical and neurophysiological phenotypes

Antelmi¹,

Provini²

2015

Sleep Medicine Reviews

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“…Selective serotonin reuptake inhibitors seem to be effective sporadically. 52 Valproate was reported to be effective in 6 but ineffective in 10 cases. Additional administered drugs included carbamazepine, prednisone, and baclofen, and were mostly reported as ineffective.…”

Section: Follow-up and Therapy Follow-up Was Reported In 126 (70%) Cmentioning

confidence: 99%

“…17 An associated back trauma was reported in 10 cases. 2,18,23,40 Single secondary cases were reported in herpes zoster, 4 Lyme neuroborreliosis, 21 hepatitis C, 48 gluten sensitivity, 55 a fragile X permutation, 52 anti-MAG antibodies, 47 myasthenia gravis, 2 breast cancer, 41 and inflammation. 42 Furthermore, 2 PSM cases were related to drug abuse, 1 involving cannabis 29 and 1 with N 2 0 and vitamin B 12 deficiency.…”

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confidence: 99%

Propriospinal myoclonus

et al. 2014

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Objective: Propriospinal myoclonus (PSM) is a rare disorder with repetitive, usually flexor arrhythmic brief jerks of the trunk, hips, and knees in a fixed pattern. It has a presumed generation in the spinal cord and diagnosis depends on characteristic features at polymyography. Recently, a historical paradigm shift took place as PSM has been reported to be a functional (or psychogenic) movement disorder (FMD) in most patients. This review aims to characterize the clinical features, etiology, electrophysiologic features, and treatment outcomes of PSM.Methods: Re-evaluation of all published PSM cases and systematic scoring of clinical and electrophysiologic characteristics in all published cases since 1991.Results: Of the 179 identified patients with PSM (55% male), the mean age at onset was 43 years (range 6-88 years). FMD was diagnosed in 104 (58%) cases. In 12 cases (26% of reported secondary cases, 7% of total cases), a structural spinal cord lesion was found. Clonazepam and botulinum toxin may be effective in reducing jerks.Conclusions: FMD is more frequent than previously assumed. Structural lesions reported to underlie PSM are scarce. Based on our clinical experience and the reviewed literature, we recommend polymyography to assess recruitment variability combined with a Bereitschaftspotential recording in all cases. Propriospinal myoclonus (PSM) is a rare hyperkinetic movement disorder involving the axial muscles.1 Brown et al. 1 were the first to describe the clinical phenomenology based on 3 cases. PSM is characterized by painless, usually flexor arrhythmic jerks of the trunk, hips, and knees, which are often stimulus sensitive and typically increase when supine.1-3 Based on the initial publications, PSM had a presumed generation in the spinal cord and diagnosis depended on characteristic features at polymyography. [1][2][3][4] The myoclonic activity in PSM was assumed to originate from a spinal generator and spread up and down the spinal cord via intrinsic propriospinal pathways, and these axial jerks were therefore termed propriospinal myoclonus.1 Electrophysiologic features of PSM were published after the first clinical descriptions and included a fixed pattern of muscle activation, a slow conduction velocity (5-15 m/s) consistent with spread within the propriospinal tract, EMG burst duration of less than 1,000 ms, synchronous activation of agonist and antagonist muscles, and no facial involvement. 4 Until recently, patients were subdivided into idiopathic PSM (i.e., primary, no underlying cause found) and secondary PSM. However, the diverse etiology of this disorder is currently highlighted by several movement disorders specialists, including our centres.5-7 A functional etiology was reported to underlie several PSM cases. Psychogenic or functional movement disorders (FMD) are common and characterized by abnormal movements inconsistent with a known organic etiology. 8

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Movement Disorders

Zeineddine

Undevia

2022

Respiratory Medicine

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Propriospinal myoclonus associated with fragile-X-premutation and response to selective serotonin reuptake inhibitor

Cited by 4 publications

References 4 publications

Propriospinal myoclonus: The spectrum of clinical and neurophysiological phenotypes

Propriospinal myoclonus: The spectrum of clinical and neurophysiological phenotypes

Propriospinal myoclonus

Movement Disorders

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