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Multiple system atrophy — Clinical Guidelines

Overview

Multiple system atrophy (MSA) is a rare, progressive neurodegenerative disorder characterized by autonomic failure, parkinsonism, and cerebellar dysfunction. It significantly impacts quality of life due to motor and non-motor symptoms including speech difficulties and pain 1 4.

Diagnosis

Clinical criteria based on Gilman et al. (2008) diagnostic criteria 2

Neurological examination focusing on parkinsonism, cerebellar ataxia, and autonomic dysfunction 17

Exclusion of other similar disorders through imaging (e.g., MRI showing characteristic changes) and laboratory tests 17

Management

First-line treatments:

- Levodopa and dopamine agonists for motor symptoms (specific doses not detailed) 17 - Symptomatic management for autonomic dysfunction (e.g., fludrocortisone for orthostatic hypotension) 17

Adjunctive therapies:

- Speech therapy approaches like ClearSpeechTogether for speech difficulties 1 - Scrambler Therapy for pain management in MSA-parkinsonian subtype (case report evidence) 3

Special Populations

Comorbidities: Patients often have complex medication profiles requiring careful management to avoid adverse interactions 2

No specific data on pregnancy, pediatrics, or elderly adaptations provided in the abstracts 1 2 3 4 17

Key Recommendations

Implement symptomatic speech therapy interventions like ClearSpeechTogether to address speech difficulties in MSA-C patients (Evidence: Moderate) 1

Consider innovative pain management strategies such as Scrambler Therapy for MSA patients experiencing chronic pain (Evidence: Weak) 3

Closely monitor and manage comorbidities and medication regimens to ensure patient safety and efficacy (Evidence: Moderate) 2

References

1 Lowit A, Xing K, Shanmugarajah DP, Foster E, Duty S, Young D et al.. Speech Treatment for People with Cerebellar Multiple System Atrophy (MSA-C): A Pilot Randomised Controlled Trial of Two Approaches. Cerebellum (London, England) 2025. link 2 Ye L, Greten S, Wegner F, Doll-Lee J, Krey L, Heine J et al.. The comorbidity profiles and medication issues of patients with multiple system atrophy: a systematic cross-sectional analysis. Journal of neurology 2024. link 3 Wang EJ, Berninger LE, Pantelyat AY, Hunsberger JB, Smith TJ. Scrambler Therapy for the Treatment of Multiple System Atrophy-Parkinsonian Subtype Pain: A Case Report. A&A practice 2022. link 4 Carlisle D. Rare but deadly. Nursing standard (Royal College of Nursing (Great Britain) : 1987) 2013. link 5 . Fever, jaundice, and multiple organ system failure associated with 17D-derived yellow fever vaccination, 1996-2001. MMWR. Morbidity and mortality weekly report 2001. link 6 Splendiani G, Mazzarella V, Cipriani S, Zazzaro D, Casciani CU. Continuous renal replacement therapy: our experience in intensive care unit. Renal failure 2001. link 7 Somogyi-Zalud E, Zhong Z, Lynn J, Dawson NV, Hamel MB, Desbiens NA. Dying with acute respiratory failure or multiple organ system failure with sepsis. Journal of the American Geriatrics Society 2000. link 8 Hasegawa N, Kandra TG, Husari AW, Veiss S, Hart WT, Hedgpeth J et al.. The effects of recombinant human thrombomodulin on endotoxin-induced multiple-system organ failure in rats. American journal of respiratory and critical care medicine 1996. link 9 Yao YM, Sheng ZY, Tian HM, Wang YP, Yu Y, Fu XB et al.. Gut-derived endotoxemia and multiple system organ failure following gunshot wounds combined with hemorrhagic shock: an experimental study in the dog. The Journal of trauma 1995. link 10 Tighe D, Moss R, Haywood G, Webb A, al-Saady N, Heath F et al.. Dopexamine hydrochloride maintains portal blood flow and attenuates hepatic ultrastructural changes in a porcine peritonitis model of multiple system organ failure. Circulatory shock 1993. link 11 Grootendorst AF, van Bommel EF. The role of hemofiltration in the critically-ill intensive care unit patient: present and future. Blood purification 1993. link 12 Dong YL, Sheng CY, Herndon DN, Waymack JP. Metabolic abnormalities of mitochondrial redox potential in postburn multiple system organ failure. Burns : journal of the International Society for Burn Injuries 1992. link90148-n) 13 Alóe FS, Marchiori PE, Reimão RN, Scaff M. Shy-Drager syndrome: a case report with polysomnography. Arquivos de neuro-psiquiatria 1990. link 14 Zobel G, Trop M, Ring E, Grubbauer HM. Arteriovenous hemofiltration in children with multiple organ system failure. The International journal of artificial organs 1987. link 15 Wilkinson JD, Pollack MM, Glass NL, Kanter RK, Katz RW, Steinhart CM. Mortality associated with multiple organ system failure and sepsis in pediatric intensive care unit. The Journal of pediatrics 1987. link80448-1) 16 Keller GA, West MA, Cerra FB, Simmons RL. Macrophage-mediated modulation of hepatic function in multiple-system failure. The Journal of surgical research 1985. link90124-6) 17 Bannister R. Clinical studies of autonomic function and dysfunction. Journal of the autonomic nervous system 1983. link90076-0)

Multiple system atrophy