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Movement disorders
Published in Henry J. Woodford, Essential Geriatrics, 2022
No specific treatments have been developed for MSA. Around 30% of people with MSA-P will have a beneficial response to levodopa therapy but it is often only short-lived and most people are poorly responsive.118 The management of OH and urinary incontinence are discussed in Chapters 15 and 11, respectively. Erectile dysfunction may be managed with sildenafil but this treatment can precipitate or aggravate OH.79
Degenerative Diseases of the Nervous System
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
James A. Mastrianni, Elizabeth A. Harris
Multiple system atrophy (MSA) is a sporadic, adult-onset, progressive, neurodegenerative disease of unknown etiology, characterized by autonomic dysfunction combined with parkinsonism and/or ataxia. While clinically protean, different manifestations of the disease are unified by common cellular pathology featuring glial cytoplasmic inclusions.
Adrenergic Antagonists
Published in Sahab Uddin, Rashid Mamunur, Advances in Neuropharmacology, 2020
It is an antagonist of the adrenergic β receptor and is cardioselective. It does not show MSA. It produces weak bronchodilating and vasodilating action due to β2 agonism and produces a relaxation action on smooth muscle (Milne et al., 1991; Florey, 2008). It also blocks peripheral α2 receptors, promotes NO production, and inhibits oxidative stress. At β2 receptor it produces intrinsic sympathomimetic action. It lowers the BP and the rate of the heart. The adverse events include headache, fatigue, dizziness, and swollen ankles. The drug absorption is fast from gastrointestinal tract after an oral dose and there is no first pass metabolism. It is employed in treating angina and hypertension (Brunton et al., 2011; Florey, 2008). The drug has a bioavailability of 30–70% and reaches a maximum plasma concentration at 2–4 h. About 25% of given dose of the drug is protein bound. The drug is not affected by first-pass effect and excretion is majorly via feces with about 11% through the urine. For parenteral administered drug 50% excretion happens via urine and 31% through feces.
Severe rhabdomyolysis due to idiopathic inflammatory myopathies, a wary manifestation of a heterogenous pathology
Published in Acta Clinica Belgica, 2023
Niels Schepens, Pauline H. Herroelen, An-Sofie Decavele, An Vanacker
This case showed a single high positive result for anti-NXP2 on LIA. This result could not be verified by another Belgian laboratory as they all use LIA or DB and no immunoprecipitation (IP) was available. As false-positive results are frequently detected, one should consider the intensity of the reaction, which was high (100) on LIA Euroimmun, indicating a higher probability of a true positive result. When interpreting MSA results, correlation with the result obtained by Hep-2 IIFA (ANA) is necessary. For example, a speckled cytoplasmic pattern is highly associated with anti-SRP and anti-ARS antibodies. However, for most MSA, no strong association exists. In case of anti-NXP2, multiple nuclear dots (AC-6) are described, but with low sensitivity, hence a negative ANA does not exclude anti-NXP2. Therefore, when clinical suspicion for IIM is high, a negative ANA result does not exclude IIM and the first test of choice remains a multi-specific immunoassay for the whole spectrum of MSA [13].
Alpha-synuclein levels in patients with multiple system atrophy: a meta-analysis
Published in International Journal of Neuroscience, 2018
Fei Yang, Wan-Jun Li, Xu-Sheng Huang
Alpha-synuclein, a 140-amino acid protein normally present in presynaptic neuronal terminals, is a precursor of the non-amyloid component of plaques [22]. Alpha-synuclein is thought to play a role in vesicular transport, membrane interactions and neuronal plasticity [23,24]. The pathogenesis of MSA involves degeneration of oligodendroglial cells causing the loss of trophic support to neurons and leading to neurodegeneration. Alpha-synuclein is produced by neurons from where it reaches extracellular matrix and then enters glial cells in a prion-like fashion [25]. Recently, it has been reported that oligodendrocytes also express α-synuclein mRNA, suggesting that it is produced by both the neurons and the glia [26]. The pathogenesis may also be substantiated by the astrocytes-induced neuroinflammation due to the accumulation of α-synuclein [27].
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.