Propriospinal myoclonus associated with gluten sensitivity in a young woman

Zhang, Yan; Menkes, Daniel L.; Silvers, David

doi:10.1016/j.jns.2011.12.004

Cited by 10 publications

(8 citation statements)

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“…PSM at sleep onset PSM worsening at sleep onset was first reported by sleep medicine experts [3,4,22,24,35,39], but also in other case-reports and cohort studies [19,28,36,37,43,44]. Indeed, it seems to be a peculiar feature of PSM even if it is not always reported, probably because clinicians do not always ask specifically about this feature [37].…”

Section: Bpmentioning

confidence: 96%

“…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%

“…The movement seems to be position-dependent, recurring more frequently when the patient is lying down [1,16,22,24,28,31,35,40] and disappearing or subsiding when standing or walking. However, many of these reports did not check the data with the subject's state of mental activation.…”

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: Bpmentioning

confidence: 96%

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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“…Other therapies included surgery of spinal lesions or discectomy, for instance Shprecher et al 43 recently described the almost complete resolution of PSM after anterior cervical discectomy with fusion. 1,20,24 In isolated cases, removal of surgical screws, 13 plasmapheresis, 47 continuous positive airway pressure, 37 and a gluten-free diet 55 were reported helpful. In FMD, the transient therapeutic response to electrical nerve stimulation 51 and effect of cognitive behavioral therapy 44 prompted the diagnosis of FMD.…”

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.…”

mentioning

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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Diagnostic Criteria, Differential Diagnosis, and Treatment of Minor Motor Activity and Less Well-Known Movement Disorders of Sleep

Stefani

Högl

2019

Curr Treat Options Neurol

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Purpose of reviewSleep-related movement disorders (SRMD) include several different motor activities during sleep. Few of them are well known and well classified, whereas others are minor motor disorders of sleep which are neither thoroughly characterized and classified nor have been extensively investigated to clarify their pathogenesis and clinical relevance. This review will focus on those minor sleep-related movement disorders.Recent findingsBefore diagnosing periodic limb movement (PLM) disorder in patients with PLM during polysomnography, other disorders associated with PLM need to be excluded, namely restless legs syndrome (RLS), narcolepsy, REM sleep behavior disorder (RBD), and sleep-related breathing disorder. For the diagnosis of propriospinal myoclonus at sleep-onset, multi-channel surface electromyography recording during polysomnography is required and a possible psychogenic origin of the movement disorder has to be considered. Excessive fragmentary myoclonus (EFM) does not require symptomatic treatment, but further evaluation is suggested as electrophysiological abnormalities are present in 50% of cases. Nine percent of healthy sleepers meet the criteria for EFM, raising the question if current, arbitrarily defined, cutoffs are valid. Hypnagogic foot tremor, rhythmic feet movements, alternating leg muscle activation, and high-frequency leg movements are somewhat overlapping minor motor activities during sleep which may exist on their own or represent stereotyped movements to relieve RLS-like symptoms. Neck myoclonus is probably a physiological phenomenon related to REM twitching. RBD is formally a parasomnia but a relevant differential diagnosis when evaluating sleep-related movement disorders. In particular, prodromal RBD is characterized by electromyographic and behavioral findings on video-polysomnography which needs to be differentiated by minor sleep-related movement disorders.SummaryMinor SRMD beyond the well-known main motor disorders of sleep should be correctly diagnosed, distinguished from differential diagnosis, and understood in their potential clinical relevance, in order also to start an appropriate treatment if needed.

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Propriospinal myoclonus associated with gluten sensitivity in a young woman

Cited by 10 publications

References 10 publications

Propriospinal myoclonus: The spectrum of clinical and neurophysiological phenotypes

Propriospinal myoclonus: The spectrum of clinical and neurophysiological phenotypes

Propriospinal myoclonus

Diagnostic Criteria, Differential Diagnosis, and Treatment of Minor Motor Activity and Less Well-Known Movement Disorders of Sleep

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