Striatonigral Degeneration Treatment & Management

  • Author: Paula K Rauschkolb, DO; Chief Editor: Selim R Benbadis, MD   more...
 
Updated: Mar 9, 2009
 

Medical Care

Response to anti-parkinsonian medications is sub-optimal at best for treatment of multiple system atrophy; however, it remains the treatment of choice in the absence of better options. Other medical therapies used in multiple system atrophy target associated symptoms (eg, orthostatic hypotension). See Medication and In/Out Patient Meds below.

Next

Surgical Care

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.[11]

Previous
Next

Consultations

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

Diet

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

Previous
Next

Activity

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.

Previous
Proceed to Medication
 
 
Contributor Information and Disclosures
Author

Paula K Rauschkolb, DO  Staff Physician, Department of Neurology, Dartmouth-Hitchcock Medical Center

Paula K Rauschkolb, DO is a member of the following medical societies: American Academy of Neurology and American Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Stephen A Berman, MD, PhD  Professor, Department of Internal Medicine, Section of Neurology, Dartmouth Medical School; Chief, Neurology Service, White River Junction Veterans Medical Center

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

Disclosure: Nothing to disclose.

Specialty Editor Board

Joseph Quinn, MD  Assistant Professor, Department of Neurology, Portland VA Medical Center, Oregon Health Sciences University

Joseph Quinn, MD is a member of the following medical societies: American Academy of Neurology, Society for Neuroscience, and Society for Pediatric Radiology

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

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.

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

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, and American Medical Association

Disclosure: Nothing to disclose.

Chief Editor

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

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, and American Medical Association

Disclosure: Nothing to disclose.

References

  1. Gilman S, Low PA, Quinn N, et al. Consensus statement on the diagnosis of multiple system atrophy. J Neurol Sci. Feb 1 1999;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. Aug 26 2008;71(9):670-6. [Medline].

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

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

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

  6. 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. Nov 2002;73(5):517-23. [Medline].

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

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

  9. 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. Jun 28 1997;349(9069):1877-81. [Medline].

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

  11. 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. Mar 2004;100(3):553-6. [Medline].

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

  13. 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. Jul-Aug 2004;23(4):201-8. [Medline].

  14. Gilman S. Parkinsonian syndromes. Clin Geriatr Med. Nov 2006;22(4):827-42, vi. [Medline].

  15. Jaros E, Burn DJ. The pathogenesis of multiple system atrophy: past, present, and future. Mov Disord. Sep 2000;15(5):784-8. [Medline].

  16. Kofler M, Wenning GK, Poewe W. Cortical and brain stem hyperexcitability in striatonigral degeneration. Mov Disord. May 1998;13(3):602-7. [Medline].

  17. Kurisaki H. [Prognosis of multiple system atrophy--survival time with or without tracheostomy]. Rinsho Shinkeigaku. May 1999;39(5):503-7. [Medline].

  18. Miwa H, Kondo T, Mizuno Y. [Striatonigral degeneration and sporadic olivopontocerebellar atrophy: a consideration of the clinical entity of multiple system atrophy]. No To Shinkei. Apr 1999;51(4):305-12. [Medline].

  19. Palace J, Chandiramani VA, Fowler CJ. Value of sphincter electromyography in the diagnosis of multiple system atrophy. Muscle Nerve. Nov 1997;20(11):1396-403. [Medline].

  20. Saper CB. "All fall down": the mechanism of orthostatic hypotension in multiple systems atrophy and Parkinson's disease. Ann Neurol. Feb 1998;43(2):149-51. [Medline].

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

  22. 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). Mar 2006;162(3):363-70. [Medline].

  23. Tarsy D, Apetauerova D, Ryan P, Norregaard T. Adverse effects of subthalamic nucleus DBS in a patient with multiple system atrophy. Neurology. Jul 22 2003;61(2):247-9. [Medline].

  24. Terada S. An autopsy case of short course striatonigral degeneration with numerous argyrophilic thread-like structures in the pons ADDED. Neuropathology. 2006;26(pt 2):A33.

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

  26. Wenning GK, Seppi K, Tison F, Jellinger K. A novel grading scale for striatonigral degeneration (multiple system atrophy). J Neural Transm. Mar 2002;109(3):307-20. [Medline].

 
Previous
Next
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.