Anal muscle electromygrams differ in amyotrophic lateral sclerosis and Shy‐Drager syndrome

Sakuta, Manabu; Nakanishi, Takao; Toyokura, Y

doi:10.1212/wnl.28.12.1289

Cited by 66 publications

(35 citation statements)

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“…Although reports from these laboratories have shown electromyographic changes of chronic reinnervation in the motor units of the striated muscle of the sphincter in 75-100% of MSA patients compared to only 0-17% of IPD patients, [14][15][16][17][18] other studies have disputed this finding, and the test is not universally accepted. 19 However, SpEMG has been found to be clinically useful in identifying abnormalities found in patients subsequently diagnosed as having MSA, differentiating them from patients with IPD and coincidental urogenital symptoms.…”

Section: Introductionmentioning

confidence: 99%

Erectile and urinary dysfunction may be the presenting features in patients with multiple system atrophy: a retrospective study

et al. 2003

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Multiple system atrophy (MSA) is a progressive neurodegenerative disease characterized by parkinsonism and cerebellar, autonomic, urinary, and/or pyramidal dysfunction. Urinary and erectile dysfunction (ED) symptoms are prominent early features in men with MSA. Autonomic failure, considered until recently to be the cause of ED in these men, is commonly expressed through symptoms of orthostatic hypotension (OH). The aim of this retrospective study is to examine the chronological relationship between the development of urogenital symptoms and those of OH in patients diagnosed with MSA and discuss its significance in the aetiology of ED in these patients. A total of 71 male patients, referred to a Uro-Neurology department with a diagnosis of 'probable MSA', were reviewed in terms of 'autonomic' symptoms onlyFOH and lower urinary tract symptoms, accompanied by EDFpresent at the time of their referral. Laboratory investigations including anal sphincter EMG and/or autonomic function tests (AFTs) were performed in 75 and 90% of the patients, respectively. At presentation, urinary complaints were recorded in 96% of patients and ED in all patients that this was inquired about. The onset of ED had preceded the onset of bladder symptoms in 58% and the onset of OH symptoms in 91% of these men. Bladder symptoms also preceded symptoms of OH in 76% of patients. Sphincter EMG was abnormal in 91% and AFTs in 77% of the patients tested. Almost all patients with abnormal EMG had troublesome urinary symptoms. AFTs showed similar sensitivity relating to symptoms. At presentation, urogenital symptoms are common in patients with probable MSA and are often not accompanied by symptoms of OH. The earlier occurrence of ED in men with MSA suggests a lack of a causal relationship to hypotension. The notion that MSA possibly affects the dopaminergic mechanism of erectile function is discussed.

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Section: Introductionmentioning

confidence: 99%

Erectile and urinary dysfunction may be the presenting features in patients with multiple system atrophy: a retrospective study

et al. 2003

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“…These cells are preserved in some neurodegenerative conditions affecting the spinal cord 5 but are affected in MSA and PSP. 6 Sphincter electromyography (spEMG) of the striated urethral or anal sphincter can detect denervation 7 and has been proposed as a method of differentiating between MSA and PD. 8,9 One shortcoming of using spEMG in this context is that results can be abnormal in long-standing PD and Machado-Joseph disease (SCA 3).…”

mentioning

confidence: 99%

Is sphincter electromyography a helpful investigation in the diagnosis of multiple system atrophy? A retrospective study with pathological diagnosis

Paviour¹,

Williams²,

Fowler

et al. 2005

Movement Disorders

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Sphincter electromyography (spEMG) is often used as an ancillary test when multiple system atrophy (MSA) is suspected. Our aim was to determine the clinical features associated with spEMG being performed, the influence of the result on the final clinical diagnosis, and its utility as a clinical investigation. A retrospective audit of all cases in the Queen Square Brain Bank between 1989 and 2002 was performed. The clinical features and diagnostic accuracy were compared between patients in whom spEMG was performed and those in whom it was not. From 845 sets of complete clinical records, we identified 37 (4.4%) cases that had been investigated with spEMG. Thirty of these cases had a pathological diagnosis of MSA. Of these 30, 24 had abnormal spEMGs, 5 had a borderline result, and only 1 had a normal spEMG. Sixty-six cases had pathologically proven MSA but no spEMG. Those investigated with spEMG were younger at disease onset (P Ͻ 0.001), more frequently male (P ϭ 0.03), and more likely to have had other investigations performed. They had a greater incidence of pyramidal tract signs at final clinical diagnosis, and the final clinical diagnostic accuracy was higher (P ϭ 0.04). Due to the retrospective nature of the study, balanced populations for calculation of sensitivity and specificity were not available. In this selected series of pathologically confirmed cases, investigation with spEMG was one of several factors associated with improved clinical diagnostic accuracy. A normal spEMG is unlikely in pathologically proven MSA, at least in cases with a mean symptom duration of more than 5 years when the test is performed.

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“…62 None of them exhibited neurogenic changes in the MUPs, which contrasted with common neurogenic changes in the limb muscles in this disorder. These EMG findings correspond to the postmortem selective sparing of sacral Onuf's nucleus, which contrasts with severe loss of anterior horn cells innervating the limbs, tongue, and bulbar muscles 2 .…”

Section: Other Diseasesmentioning

confidence: 84%

“…33,37 The first reports on neurogenic changes of EAS-EMG in MSA are attributed to Sakuta et al (1978). 62 Since then, EAS-EMG results for over 500 MSA patients have been reported, with abnormality rates of more than 70% in many studies 5,30,36,38,47,53,62,64,71 . EAS-EMG is better tolerated and yields identical results to those from EUS investigation 5 .…”

Section: Methods and Interpretations Of Sphincter Emgmentioning

confidence: 99%

Sphincter EMG for Diagnosing Multiple System Atrophy and Related Disorders

Sakakibara¹,

Uchiyama²,

Yamamoto³

et al. 2012

Computational Intelligence in Electromyography Analysis - A Perspective on Current Applications and Future Challenges

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Anal muscle electromygrams differ in amyotrophic lateral sclerosis and Shy‐Drager syndrome

Cited by 66 publications

References 0 publications

Erectile and urinary dysfunction may be the presenting features in patients with multiple system atrophy: a retrospective study

Erectile and urinary dysfunction may be the presenting features in patients with multiple system atrophy: a retrospective study

Is sphincter electromyography a helpful investigation in the diagnosis of multiple system atrophy? A retrospective study with pathological diagnosis

Sphincter EMG for Diagnosing Multiple System Atrophy and Related Disorders

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