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Striatonigral Degeneration Medication

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

Medication Summary

The drugs in the tables below are specific to the treatment of parkinsonism and postural hypotension associated with multiple system atrophy with predominantly parkinsonian features (MSA-P). As previously mentioned, in patients with multiple system atrophy, the response to antiparkinsonian medications is suboptimal at best. Because better options are not available, however, these agents remain the treatment of choice for the disease.

Adjunct medications

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. The use of a fiber supplement or another bowel regimen may be necessary for constipation.

A selective serotonin reuptake inhibitor (SSRI) or similar drug may be required for the treatment of depression often associated with all subtypes of multiple system atrophy. For patients who suffer from REM sleep behavioral disorder, clonazepam may be beneficial. Botulinum toxin (BOTOX®) injection to the vocal cords has been used for the treatment of stridor.

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Antiparkinson Agents, Dopamine Agonists

Class Summary

Dopaminergic drugs can exacerbate orthostatic hypotension. They must be initiated at low doses and cautiously titrated up.

Levodopa-carbidopa (Sinemet, Parcopa)

 

Levodopa is a dopamine precursor used to increase central nervous system (CNS) dopamine concentration, as it is not possible for dopamine to cross the blood-brain barrier. Carbidopa is a peripheral dopa decarboxylase inhibitor that prevents premature conversion of levodopa to dopamine in the tissues prior to entering the CNS. It increases the efficiency of levodopa therapy, allows for lower dosages, and also decreases the side effects associated with peripheral conversion.

Standard release forms of levodopa-carbidopa are available in 25/100-, 10/100-, and 25/250-mg tablets. Controlled-release preparations are available in 50/200 mg and 25/100 mg.

Pramipexole (Mirapex)

 

Pramipexole is a nonergot dopamine agonist that is used with or without concomitant levodopa therapy. It binds D2 and D3 dopamine receptors. Due to pramipexole's high specificity for D3 receptors (relative to other dopamine agonists), it may cause less orthostatic hypotension. It has no significant effect on other adrenergic or serotonergic receptors. The drug's absolute bioavailability is greater than 90%. Its peak serum concentration is reached in approximately 2 hours and its half-life is approximately 8 hours.

There are no known metabolites; roughly 90% of this drug is renally excreted in its unchanged form. Tablets are available in 0.125-, 0.25-, 0.5-, 0.75-, 1-, and 1.5-mg forms.

Ropinirole (Requip)

 

Ropinirole is a nonergot dopamine agonist that is used with or without concomitant levodopa therapy. It binds to D2 and D3 receptors but has a greater affinity for D3. Ropinirole's bioavailability is 55%, its peak plasma concentration is reached in 1-2 hours, and its half-life is approximately 6 hours. Ropinirole is extensively metabolized by the liver via P450 CYP1A2. Less than 10% of the drug is renally excreted; no dosage change is required in mild to moderate renal insufficiency. If ropinirole is used as adjunct therapy, it may be possible to titrate levodopa dosage slowly downward.

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Corticosteroids

Class Summary

These are used to treat orthostatic hypotension that is refractory to nonpharmacologic recommendations.

Fludrocortisone

 

Fludrocortisone is a synthetic steroid with predominantly mineralocorticoid activity. It acts on renal distal tubules to enhance the reabsorption of sodium and increase the urinary excretion of potassium. The net effect is an increase in plasma volume and an elevation of blood pressure. The drug's metabolism is primarily hepatic.

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Alpha1 Agonists

Class Summary

These drugs are used to treat orthostatic hypotension that is refractory to nonpharmacologic recommendations.

Midodrine

 

Midodrine is a selective alpha1-adrenergic agonist used for the treatment of hypotension.

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Benzodiazepines

Class Summary

These drugs are used for REM sleep behavior disorder (RBD).

Clonazepam (Klonopin)

 

Clonazepam is a benzodiazepine which binds to gamma aminobutyric acid (GABA)-A receptors in the CNS. It may help regulate sleep disorders.

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Contributor Information and Disclosures
Author

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.

Coauthor(s)

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 College of Medicine

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

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; Eisai; Glaxo Smith Kline; Lundbeck; Sunovion; UCB<br/>Received research grant from: Cyberonics; Lundbeck; Sepracor; Sunovion; UCB; Upsher-Smith.

Acknowledgements

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

References
  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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