eMedicine Specialties > Neurology > Movement and Neurodegenerative Diseases

Striatonigral Degeneration: Follow-up

Author: Paula K Rauschkolb, DO, Staff Physician, Department of Neurology, Dartmouth-Hitchcock Medical Center
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
Contributor Information and Disclosures

Updated: Mar 9, 2009

Follow-up

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.   

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)

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.

Complications

  • Vocal fold paresis and glottic airway compromise requiring continuous positive airway pressure support or tracheostomy12
  • Aspiration pneumonia secondary to dysphagia and vocal fold paresis
  • Sudden death, often occurring at night and associated with sleep-disordered breathing

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

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.

Miscellaneous

Medicolegal Pitfalls

  • Differentiating multiple system atrophy with predominantly parkinsonian features from Parkinson disease can be challenging but is critical as patients with the former condition should not be considered candidates for placement of deep brain stimulators.
  • Advance directives should be established, particularly with regard to endotracheal intubation, tracheostomy, and placement of gastrostomy (feeding) tubes.
 
Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous authors Maritza Arroyo-Muñiz, MD and Syed T Arshad, MD to the development and writing of this article.



More on Striatonigral Degeneration

Overview: Striatonigral Degeneration
Differential Diagnoses & Workup: Striatonigral Degeneration
Treatment & Medication: Striatonigral Degeneration
Follow-up: Striatonigral Degeneration
References
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References

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Further Reading

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Keywords

striatonigral degeneration, SND, neurodegenerative disease, multiple system atrophy, MSA, MSA-P, Shy-Drager syndrome, sporadic olivopontocerebellar degeneration, sporadic OPCA, sOPCA, parkinsonism, MSA with predominantly parkinsonian features, MSA with predominantly cerebellar features, MSA-C, parkinsonian MSA, cerebellar MSA, parkinsonian multiple system atrophy, cerebellar multiple system atrophy

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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.

Medical Editor

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.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

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.

CME 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.

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.

 
 
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