Striatonigral Degeneration Follow-up

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

Further Inpatient Care

Striatonigral degeneration (multiple system atrophy with predominantly parkinsonian features [MSA-P]) is unlikely to be the primary cause for hospitalization. Thus, the focus of care would be treatment of the diagnoses that required admission.

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Further Outpatient Care

  • Management of anti-parkinsonian drug regimen (if used)
  • Patient education regarding orthostatic hypotension (see Patient Education)
  • Assessment for history or physical signs of falls at each office visit
  • Assistive devices as needed (cane, walker, wheelchair and household implements)
  • Teach patient to self-catheterize if urinary retention develops
  • Referrals as needed (see Consultations)
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Inpatient & Outpatient Medications

Also see Medication section.

  • Anticholinergic medications, such as oxybutynin, are sometimes used for incontinence but often lead to subsequent retention.
  • Although sildenafil has been used for treatment of erectile dysfunction, it is generally not recommended due to its high potential to provoke or exacerbate hypotension.
  • Fiber supplement or other bowel regimen may be necessary for constipation.
  • An SSRI or similar drug may be required for treatment of depression often associated with all subtypes of multiple system atrophy.
  • For those who suffer from REM sleep behavioral disorder, clonazepam may be beneficial.
  • Botox injection to the vocal cords has been used for treatment of stridor.
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Complications

  • Vocal fold paresis and glottic airway compromise requiring continuous positive airway pressure support or tracheostomy[12]
  • Aspiration pneumonia secondary to dysphagia and vocal fold paresis
  • Sudden death, often occurring at night and associated with sleep-disordered breathing
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Prognosis

Multiple system atrophy is a progressive neurodegenerative disorder without remission. Survival time is less than a decade from symptom onset. In a study by Blumin et al, median survival time was 8.6 years for men and 7.3 years for women.[12]

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Patient Education

Those with symptomatic postural hypotension should be educated on the following:

  • Activities or environments that produce excessive vagal stimulation or vasodilation (eg, extreme heat, overeating, alcohol, straining at stool) should be avoided.
  • Always rise slowly and carefully from seated or recumbent positions.
  • Sit or lie down as soon as symptoms appear.
  • Consider pressure stockings, elevating the head of the bed, and increasing sodium intake.
  • Be aware that there is a risk of fall with associated trauma; always seek medical attention for any but the most minor of falls.
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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].

 
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