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Introduction
Published in David Robertson, Italo Biaggioni, Disorders of the Autonomic Nervous System, 2019
Shy and Drager (1960) described the pathology of two patients with autonomic failure accompanied by significant central involvement. Their description built upon the observations of Dr. Briggs and has led to improved understanding of the distinction between autonomic failure sui generis, and autonomic failure with evidence of other neurological involvement. This disorder, or group of disorders has been referred to as the Shy-Drager syndrome. About a decade after the observations of Shy and Drager, Oppenheimer used the term multiple system atrophy to describe these patients. A limitation of this term is its nonspecificity; but it remains widely used in the United Kingdom.
Case 7: An Elderly Patient Admitted with a Fall
Published in Layne Kerry, Janice Rymer, 100 Diagnostic Dilemmas in Clinical Medicine, 2017
Multisystem atrophy (Shy-Drager syndrome) can also present with features of Parkinsonism along with autonomic dysfunction. Signs may include a postural drop in blood pressure (20 mm Hg fall in systolic blood pressure and 10 mm Hg fall in diastolic blood pressure are considered significant) and urinary incontinence, both of which are present in this patient. She has a degree of postural hypotension, but she may also have lower blood pressures in the hours immediately following anti-hypertensive administration, which could prompt a fall.
Practice Paper 9: Answers
Published in Anthony B. Starr, Hiruni Jayasena, David Capewell, Saran Shantikumar, Get ahead! Medicine, 2016
Anthony B. Starr, Hiruni Jayasena, David Capewell
Shy–Drager syndrome is a degenerative disease of the autonomic nervous system. It presents with parkinsonian symptoms (tremor, bradykinesia and rigidity) and features of autosomal failure (e.g. incontinence, postural hypotension, gastroparesis and erectile dysfunction).
Chameleons, red herrings, and false localizing signs in neurocritical care
Published in British Journal of Neurosurgery, 2022
Boyi Li, Tolga Sursal, Christian Bowers, Chad Cole, Chirag Gandhi, Meic Schmidt, Stephan Mayer, Fawaz Al-Mufti
Cluster breathing is a respiratory syndrome that has been described with brainstem lesions, particularly low pontine or high medullary, and sometimes midbrain lesions.83 It is characterized by hyperventilation alternating with apnea of varying duration.80,84 Common causes include stroke, cerebellar hemorrhage with brainstem compression, Shy-Drager syndrome, and anoxic encephalophathy.80,83 As a FLS, cluster breathing has also been reported in one patient with bilateral hemispheric lesions and no brainstem lesions.84 The pathophysiology remains unknown, but the existence of this particular case of cluster breathing suggests cortical integration with brainstem control of respiration.84 If the patient is in critical condition due the brain injury, cluster breathing may become evident when a tracheostomy collar is placed and will occur while sleeping, excluding volitional hyperapena as the cause.84 The pattern of breathing itself is repetitive but may not be uniformly periodic.84 Diagnostic criteria also include respiratory alkalosis that is typically found in central neurogenic hyperventilation.84
Frequency analyses of posturography using logarithmic translation
Published in Acta Oto-Laryngologica, 2020
Hiroki Watanabe, Ayane Makabe, Chiaki Hirai, Takamori Takeda, Keiji Honda, Shinichi Demura, Takeshi Tsutsumi
The study cohort included a total of 219 participants: 172 healthy subjects with no hearing or vestibular disorders (81 males and 91 females; average age, 51.3 ± 22.9 years) and 47 patients with SCD (25 males and 22 females; average age, 60.0 ± 13.6 years, consisted of 3 cortical cerebellar atrophy, 10 multiple system atrophy cerebellar variant, 4 multiple system atrophy Parkinsonian variant, 1 Shy-Drager syndrome, 6 other multiple system atrophy, 1 episodic ataxia type 2, 4 spinocerebellar ataxia type 3, 4 spinocerebellar ataxia type 31, 5 spinocerebellar ataxia type 6, 6 other spinocerebellar ataxia, and 3 unknown SCD). The age distributions of the healthy subjects were as follows: 18 teenagers (eight males and 10 females; average age, 15.5 ± 0.9 years), 23 young adults aged 20–29 years (15 males and eight females, 24.0 ± 2.5 years), 22 adults aged 30–39 years (13 males and nine females; average age, 34.1 ± 2.7 years), 16 adults aged 40–49 years (five males and 11 females; average age, 45.2 ± 2.6 years), 16 adults aged 50–59 years (six males and 10 females; average age, 54.0 ± 2.2 years), 28 older adults aged 60–69 years (14 males and 14 females; average age, 65.3 ± 2.6 years), 28 elderly adults aged 70–79 years (13 males and 15 females; average age, 74.5 ± 2.2 years), and 21 elderly adults aged 80–89 years (seven males and 14 females; average age, 82.9 ± 2.6 years).
Application of bulbocavernosus reflex combined with anal sphincter electromyography in the diagnosis of MSA and PD
Published in International Journal of Neuroscience, 2022
Xiaoting Niu, Yifan Cheng, WangWang Hu, Zijian Fan, Wanli Zhang, Bei Shao, Binbin Deng
Multiple system atrophy (MSA) is a disease characterized by the pyramidal system, the extrapyramidal system, the cerebellum and the autonomic nervous system. Clinically, there is greater overlap between MSA and Parkinson's disease (PD), which can be easily confused during diagnosis. The only way to confirm the diagnosis is to rely on landmark changes confirmed by neuropathology at autopsy, i.e. the discovery of oligodendrocyte cytoplasmic inclusion bodies [1, 2]. Although identifying auxiliary indicators for the early diagnosis of MSA, including the detection of several biomarkers, is a current research hotspot, they are not operable and difficult to widely use in clinical practice. Fifty years after MSA was first defined in 1969, the diagnosis of MSA is still mainly based on clinical manifestations. Therefore, researchers hope to develop an auxiliary detection method with high sensitivity and strong specificity that is easy to operate for widespread use for the early diagnosis of MSA [1, 3]. In 1978, Sakuta et al. first reported the use of EAS-EMG to distinguish Shy-Drager syndrome from amyotrophic lateral sclerosis, which was the first step in achieving early diagnosis of MSA [4]. Since then, many studies have confirmed the value of EAS-EMG in the diagnosis of MSA. In 2015, Shao Bei reported the value of the bulbocavernosus reflex (BCR) in the diagnosis of PD and MSA [5]. However, in actual operation, although the two methods have simple operation techniques, they are characterized by varying degrees of sampling errors, resulting in false negative or false positive results. Therefore, we attempted to combine BCR and EAS-EMG to provide a basis for the early and differential diagnosis of MSA and early PD.