Early stridor onset and stridor treatment predict survival in 136 patients with MSA

Giannini, Giulia; Calandra-Buonaura, Giovanna; Mastrolilli, Francesca; Righini, Matteo; Reggiani, Maria Letizia Bacchi; Cecere, Annagrazia; Barletta, Giorgio; Guaraldi, Pietro; Provini, Federica

doi:10.1212/wnl.0000000000003156

Cited by 65 publications

(52 citation statements)

References 35 publications

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“…The prevalence of stridor in patients with MSA varies from 15% to 40% (Ghorayeb et al, 2002) and can, in some cases, be the presenting feature of the disease (Kaufmann et al, 2017a). Vocal cord paralysis and early stridor in patients with MSA are risk factors for shorter survival (Giannini et al, 2016; Lalich et al, 2014). Indirect laryngoscopy is useful in the diagnosis of stridor, and serial examinations may be required.…”

Section: Sleep Disordersmentioning

confidence: 99%

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Diagnosis of multiple system atrophy

Palma

Norcliffe‐Kaufmann

Kaufmann

2018

Autonomic Neuroscience

123

114

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Multiple system atrophy (MSA) may be difficult to distinguish clinically from other disorders, particularly in the early stages of the disease. An autonomic-only presentation can be indistinguishable from pure autonomic failure. Patients presenting with parkinsonism may be misdiagnosed as having Parkinson disease. Patients presenting with the cerebellar phenotype of MSA can mimic other adult-onset ataxias due to alcohol, chemotherapeutic agents, lead, lithium, and toluene, or vitamin E deficiency, as well as paraneoplastic, autoimmune, or genetic ataxias. A careful medical history and meticulous neurological examination remain the cornerstone for the accurate diagnosis of MSA. Ancillary investigations are helpful to support the diagnosis, rule out potential mimics, and define therapeutic strategies. This review summarizes diagnostic investigations useful in the differential diagnosis of patients with suspected MSA. Currently used techniques include structural and functional brain imaging, cardiac sympathetic imaging, cardiovascular autonomic testing, olfactory testing, sleep study, urological evaluation, and dysphagia and cognitive assessments. Despite advances in the diagnostic tools for MSA in recent years and the availability of consensus criteria for clinical diagnosis, the diagnostic accuracy of MSA remains sub-optimal. As other diagnostic tools emerge, including skin biopsy, retinal biomarkers, blood and cerebrospinal fluid biomarkers, and advanced genetic testing, a more accurate and earlier recognition of MSA should be possible, even in the prodromal stages. This has important implications as misdiagnosis can result in inappropriate treatment, patient and family distress, and erroneous eligibility for clinical trials of disease-modifying drugs.

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Section: Sleep Disordersmentioning

confidence: 99%

“…Stridor represents a life-threatening condition as it may lead to respiratory failure (Yamaguchi et al, 2003). It is unclear whether tracheostomy placement prolongs survival (Giannini et al, 2016). …”

Section: Sleep Disordersmentioning

confidence: 99%

Diagnosis of multiple system atrophy

Palma

Norcliffe‐Kaufmann

Kaufmann

2018

Autonomic Neuroscience

123

114

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“…Similarly, in patients with Parkinson's disease, the presence of OSAS was shown to be associated with worse cognitive functioning [19]. In patients with multiple system atrophy, the presence of stridor occurring early in the disease course was demonstrated as an independent predictor of a shorter survival [20], and SBD were suggested as a potential link to worse prognosis.…”

Section: Discussionmentioning

confidence: 98%

Effects of Sleep-Related Disorders on the Prognosis of Amyotrophic Lateral Sclerosis

Reyhani¹,

Şenel

Karadeniz

2019

Neurodegener Dis

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Background/Objective: In this study, we demonstrated the effects of obstructive sleep apnea syndrome (OSAS) on the prognosis of amyotrophic lateral sclerosis (ALS). However, the effects of sleep-related disorders other than breathing problems are still waiting to be delineated. Methods: In this longitudinal retrospective and prospective study, we investigated 73 patients with ALS compared to 20 healthy subjects, to determine sleep-related disorders and their impact on disease prognosis. Results: In patients with ALS, the mean respiratory disturbance index (RDI) was 28.0/h, which was significantly higher than that in the controls (p < 0.001). OSAS was present in 67% of the patients, sleep-related hypoxemia was observed in 13.7% of the patients, and 5.4% had central sleep apnea syndrome. In patients with bulbaronset disease, higher RDI was almost significantly associated with lower survival (p = 0.056). The mean index of periodic leg movements in sleep was significantly higher in patients with ALS (34.0 ± 19.9/h) than that in the controls (12.5 ± 15.5/h; p < 0.001). Periodic leg movements disorder (PLMD) was diagnosed in 23 patients with ALS (31.5%); fragmentary myoclonus was present in 13.7% of patients, and REM-sleep behavior disorder was diagnosed in 4 patients (5.4%). The presence of PLMD in addition to OSAS was significantly associated with worse prognosis and poorer survival (p = 0.040). Conclusions: These findings emphasize that sleeprelated disorders other than OSAS deserve attention in ALS. ALS is a catastrophic and frustrating disease for both patients and physicians; thus, the diagnosis and treatment of comorbid sleep disorders could improve the survival of patients with ALS.

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“…The discrepancy might have occurred because stridor is not assessed by clinicians but by caregivers without an objective tool such as video-polysomnography, in this study. A previous study demonstrated that 16.1% of patients presented stridor as an early (< 3 years) manifestation and early stridor onset is an independent risk factor for shorter survival in MSA patients [32]. Furthermore, it has been reported that stridor was an initial manifestation in 4-5.2% of patients with MSA [31].…”

Section: Discussionmentioning

confidence: 99%

Various Motor and Non-Motor Symptoms in Early Multiple System Atrophy

et al. 2019

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Background: Multiple system atrophy (MSA) patients present a variety of symptoms other than autonomic dysfunctions, parkinsonism, and cerebellar ataxia. The aim of this study was to evaluate the frequency of various motor and non-motor symptoms including so-called "red flags" in patients with early MSA and to determine whether the frequency of these symptoms was different between the parkinsonian (MSA-P) and cerebellar (MSA-C) subtypes. Methods: Sixty-one probable or possible MSA patients with disease duration of 3 years or less were included. Patients were classified into MSA-P, MSA-C, and MSA-PC. The frequency of 13 features including various motor and non-motor symptoms that commonly occur in MSA was assessed. Results: Dysarthria was the most prevalent feature (98.4%) followed by sexual dysfunction (95.1%). Probable REM sleep behavior disorder was present in 90.2%. The frequency of constipation (82.0%), dysphagia (68.9%), and snoring (70.5%) was also high. Stridor was present in 42.6% and more common in MSA-C than in MSA-P. Conclusions: Increasing awareness of various motor and non-motor symptoms may assist clinicians to make an early, accurate diagnosis and to improve management of patients with MSA. We suggest that the diagnostic accuracy can be improved if these features are appropriately reflected in the new diagnostic criteria for MSA.

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Early stridor onset and stridor treatment predict survival in 136 patients with MSA

Abstract: Our results demonstrated that early stridor onset is an independent predictor for shorter survival and that tracheostomy could control stridor, influencing disease duration.

Cited by 65 publications

References 35 publications

Diagnosis of multiple system atrophy

Diagnosis of multiple system atrophy

Effects of Sleep-Related Disorders on the Prognosis of Amyotrophic Lateral Sclerosis

Various Motor and Non-Motor Symptoms in Early Multiple System Atrophy

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