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Striatonigral Degeneration Treatment & Management

  • Author: Ahmad El Kouzi, MD; Chief Editor: Selim R Benbadis, MD  more...
Updated: May 26, 2015

Approach Considerations

In patients with multiple system atrophy, response to antiparkinsonian medications is suboptimal at best. However, these drugs remain the treatment of choice in the absence of better options.[29] Other medical therapies used in multiple system atrophy target associated symptoms (eg, orthostatic hypotension).[30] Surgical treatment

Currently, no surgical treatment is appropriate for multiple system atrophy. Because it can be difficult to clinically distinguish multiple system atrophy from Parkinson disease, there are cases of multiple system atrophy patients undergoing placement of deep brain stimulators. The outcomes have generally been poor, even in patients who responded well to levodopa therapy.[31, 32]

Inpatient care

Multiple system atrophy with predominantly parkinsonian features (MSA-P) is unlikely to be the primary cause of a patient’s hospitalization. Thus, the focus of care would be treatment of the diagnoses that required admission.

Outpatient care

Outpatient care includes the following:

  • Management of antiparkinsonian drug regimen (if used)
  • Patient education regarding orthostatic hypotension
  • Assessment for history or physical signs of falls at each office visit
  • Assistive devices as needed - Cane, walker, wheelchair, and household implements
  • Patient education regarding self-catheterization if urinary retention develops
  • Referrals as needed


Consultations in multiple system atrophy can include professionals in the following specialties:

  • Neurology
  • Sleep medicine
  • Otolaryngology
  • Physical and occupational therapy
  • Speech-language pathology
  • Psychiatry or counseling


Unless there are contraindications, patients with symptomatic postural hypotension may benefit from increased salt intake.


Patients with symptomatic postural hypotension should be advised to avoid activities or environments that produce excessive vagal stimulation or vasodilation (eg, extreme heat, overeating, straining at stool) and to rise slowly and carefully from seated or recumbent positions.

Contributor Information and Disclosures

Ahmad El Kouzi, MD Resident Physician, Department of Neurology, Southern Illinois University School of Medicine

Ahmad El Kouzi, MD is a member of the following medical societies: American Academy of Neurology, International Parkinson and Movement Disorder Society

Disclosure: Nothing to disclose.


Stephen A Berman, MD, PhD, MBA Professor of Neurology, University of Central Florida College of Medicine

Stephen A Berman, MD, PhD, MBA is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, Phi Beta Kappa

Disclosure: Nothing to disclose.

Paula K Rauschkolb, DO Assistant Professor of Neurology and Medicine, Geisel School of Medicine at Dartmouth; Consulting Staff Physician, Department of Neurology, Department of Medicine, Section of Hematology/Oncology, Dartmouth-Hitchcock Medical Center

Paula K Rauschkolb, DO is a member of the following medical societies: American Academy of Neurology, American Medical Association, American Society of Clinical Oncology, Society for Neuro-Oncology

Disclosure: Nothing to disclose.

Chief Editor

Selim R Benbadis, MD Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, Tampa General Hospital, University of South Florida Morsani College of Medicine

Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, American Medical Association

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Cyberonics; Eisai; Lundbeck; Sunovion; UCB; Upsher-Smith<br/>Serve(d) as a speaker or a member of a speakers bureau for: Cyberonics (Livanova); Eisai; Lundbeck; Sunovion; UCB<br/>Received research grant from: Cyberonics (Livanova); GW, Lundbeck; Sunovion; UCB; Upsher-Smith.


Maritza Arroyo-Muñiz, MD Associate Program Director, Professor of Neurology, Department of Neurology, University of Puerto Rico

Maritza Arroyo-Muñiz, MD is a member of the following medical societies: American Academy of Neurology, National Stroke Association

Disclosure: Nothing to disclose.

Syed T Arshad, MD Staff Physician, Department of Neurology, Dartmouth Hitchcock Medical Center

Syed T Arshad, MD is a member of the following medical societies: American Academy of Family Physicians, American Medical Association

Disclosure: Nothing to disclose.

Arif I Dalvi, MD Director, Movement Disorders Center, NorthShore University HealthSystem, Clinical Associate Professor of Neurology, University of Chicago Pritzker Medical School

Arif I Dalvi, MD is a member of the following medical societies: European Neurological Society and Movement Disorders Society

Disclosure: Nothing to disclose.

Nestor Galvez-Jimenez, MD, MSc, MHA Chairman, Department of Neurology, Program Director, Movement Disorders, Department of Neurology, Division of Medicine, Cleveland Clinic Florida

Nestor Galvez-Jimenez, MD, MSc, MHA is a member of the following medical societies: American Academy of Neurology, American College of Physicians, and Movement Disorders Society

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Reference Salary Employment

  1. Gilman S, Low PA, Quinn N, et al. Consensus statement on the diagnosis of multiple system atrophy. J Neurol Sci. 1999 Feb 1. 163(1):94-8. [Medline].

  2. Gilman S, Wenning GK, Low PA, et al. Second consensus statement on the diagnosis of multiple system atrophy. Neurology. 2008 Aug 26. 71(9):670-6. [Medline]. [Full Text].

  3. Yoshida M, Sone M. [Mechanism of neuronal degeneration of multiple system atrophy]. Brain Nerve. 2009 Sep. 61(9):1051-60. [Medline].

  4. Ahmed Z, Asi YT, Sailer A, Lees AJ, Houlden H, Revesz T, et al. The neuropathology, pathophysiology and genetics of multiple system atrophy. Neuropathol Appl Neurobiol. 2012 Feb. 38(1):4-24. [Medline].

  5. Jellinger KA. Neuropathology of multiple system atrophy: New thoughts about pathogenesis. Mov Disord. 2014 Oct 9. [Medline].

  6. Jellinger KA. Neuropathological spectrum of synucleinopathies. Mov Disord. 2003 Sep. 18 Suppl 6:S2-12. [Medline].

  7. Nagaishi M, Yokoo H, Nakazato Y. Tau-positive glial cytoplasmic granules in multiple system atrophy. Neuropathology. 2011 Jun. 31(3):299-305. [Medline].

  8. Jellinger KA. Neuropathology of multiple system atrophy: new thoughts about pathogenesis. Mov Disord. 2014 Dec. 29(14):1720-41. [Medline].

  9. Chrysostome V, Tison F, Yekhlef F, Sourgen C, Baldi I, Dartigues JF. Epidemiology of multiple system atrophy: a prevalence and pilot risk factor study in Aquitaine, France. Neuroepidemiology. 2004 Jul-Aug. 23(4):201-8. [Medline].

  10. Vanacore N. Epidemiological evidence on multiple system atrophy. J Neural Transm. 2005 Dec. 112(12):1605-12. [Medline].

  11. Yoshida M. [Multiple system atrophy - synuclein and neuronal degeneration]. Rinsho Shinkeigaku. 2011 Nov. 51(11):838-42. [Medline].

  12. Schrag A, Wenning GK, Quinn N, Ben-Shlomo Y. Survival in multiple system atrophy. Mov Disord. 2008 Jan 30. 23(2):294-6. [Medline].

  13. Figueroa JJ, Singer W, Parsaik A, Benarroch EE, Ahlskog JE, Fealey RD, et al. Multiple system atrophy: prognostic indicators of survival. Mov Disord. 2014 Aug. 29(9):1151-7. [Medline]. [Full Text].

  14. Blumin JH, Berke GS. Bilateral vocal fold paresis and multiple system atrophy. Arch Otolaryngol Head Neck Surg. 2002 Dec. 128(12):1404-7. [Medline].

  15. Camacho V, Marquié M, Lleó A, et al. Cardiac sympathetic impairment parallels nigrostriatal degeneration in Probable Dementia with Lewy Bodies. Q J Nucl Med Mol Imaging. 2011 Aug. 55(4):476-83. [Medline].

  16. Kawai Y, Suenaga M, Takeda A, et al. Cognitive impairments in multiple system atrophy: MSA-C vs MSA-P. Neurology. 2008 Apr 15. 70(16 Pt 2):1390-6. [Medline].

  17. Klein C, Brown R, Wenning G, Quinn N. The "cold hands sign" in multiple system atrophy. Mov Disord. 1997 Jul. 12(4):514-8. [Medline].

  18. Pellecchia MT, Pivonello R, Colao A, Barone P. Growth hormone stimulation tests in the differential diagnosis of Parkinson's disease. Clin Med Res. 2006 Dec. 4(4):322-5. [Medline].

  19. Kimber JR, Watson L, Mathias CJ. Distinction of idiopathic Parkinson's disease from multiple-system atrophy by stimulation of growth-hormone release with clonidine. Lancet. 1997 Jun 28. 349(9069):1877-81. [Medline].

  20. Santamaria J, Iranzo A. Multiple System Atrophy and Sleep. Sleep Med Clin. 2008. 3:337-345.

  21. Abbott SM, Videnovic A. Sleep Disorders in Atypical Parkinsonism. Mov Disord Clin Pract (Hoboken). 2014 Jun 1. 1(2):89-96. [Medline]. [Full Text].

  22. Yoshida M. Multiple system atrophy -synuclein and neuronal degeneration. Rinsho Shinkeigaku. 2011 Nov. 51(11):838-42. [Medline].

  23. Treglia G, Stefanelli A, Cason E, Cocciolillo F, Di Giuda D, Giordano A. Diagnostic performance of iodine-123-metaiodobenzylguanidine scintigraphy in differential diagnosis between Parkinson's disease and multiple-system atrophy: a systematic review and a meta-analysis. Clin Neurol Neurosurg. 2011 Dec. 113(10):823-9. [Medline].

  24. Haga R, Sugimoto K, Nishijima H, Miki Y, Suzuki C, Wakabayashi K, et al. Clinical Utility of Skin Biopsy in Differentiating between Parkinson's Disease and Multiple System Atrophy. Parkinsons Dis. 2015. 2015:167038. [Medline]. [Full Text].

  25. Massimo G, Limbucci N, Catalucci A, Massimo C. Neurodegenerative Diseases. Radiol Clin N Am. 2008. 46:799-817.

  26. Deguchi K, Ikeda K, Kume K, Takata T, Kokudo Y, Kamada M, et al. Significance of the hot-cross bun sign on T2*-weighted MRI for the diagnosis of multiple system atrophy. J Neurol. 2015 Apr 7. [Medline].

  27. Ghaemi M, Hilker R, Rudolf J, Sobesky J, Heiss WD. Differentiating multiple system atrophy from Parkinson's disease: contribution of striatal and midbrain MRI volumetry and multi-tracer PET imaging. J Neurol Neurosurg Psychiatry. 2002 Nov. 73(5):517-23. [Medline].

  28. Hauser RA, Grosset DG. [(123) I]FP-CIT (DaTscan) SPECT Brain Imaging in Patients with Suspected Parkinsonian Syndromes. J Neuroimaging. 2011 Mar 16. [Medline].

  29. Nissen T, Malek N, Grosset KA, Newman EJ, Patterson J, Hadley D, et al. Baseline [(123) I]FP-CIT SPECT (DaTSCAN) severity correlates with medication use at 3 years in Parkinson's disease. Acta Neurol Scand. 2014 Mar. 129(3):204-8. [Medline].

  30. Kuzdas-Wood D, Stefanova N, Jellinger KA, Seppi K, Schlossmacher MG, Poewe W, et al. Towards translational therapies for multiple system atrophy. Prog Neurobiol. 2014 Jul. 118:19-35. [Medline]. [Full Text].

  31. Chou KL, Forman MS, Trojanowski JQ, Hurtig HI, Baltuch GH. Subthalamic nucleus deep brain stimulation in a patient with levodopa-responsive multiple system atrophy. Case report. J Neurosurg. 2004 Mar. 100(3):553-6. [Medline].

  32. Talmant V, Esposito P, Stilhart B, Mohr M, Tranchant C. [Subthalamic stimulation in a patient with multiple system atrophy: a clinicopathological report]. Rev Neurol (Paris). 2006 Mar. 162(3):363-70. [Medline].

  33. O'Sullivan SS, Massey LA, Williams DR, et al. Clinical outcomes of progressive supranuclear palsy and multiple system atrophy. Brain. 2008 May. 131:1362-72. [Medline].

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