Striatonigral Degeneration Workup

  • Author: Arif I Dalvi, MD; Chief Editor: Selim R Benbadis, MD   more...
 
Updated: Apr 13, 2012
 

Approach Considerations

No laboratory studies are diagnostic for multiple system atrophy with predominantly parkinsonian features (MSA-P). If a family history is noted, then testing for the SCA-3 mutation can identify Machado-Joseph disease.

Electromyography

Electromyography shows denervation of the external sphincter (urethral or anal); however, normal findings do not exclude the disease.

Clonidine growth hormone test

Studies show that after infusion of clonidine, serum growth hormone concentration does not subsequently rise in patients with multiple system atrophy. In contrast, the normal response, an increase in secretion, is found in Parkinson disease, pure autonomic failure, and control subjects.[14, 15]

Neuropsychiatric evaluation

Studies suggest that cognitive impairment is more common than previously thought in multiple system atrophy. Due to the nature of the deficits associated with this disease, the standard mental status examination has been found to be a poor tool for assessment. Neuropsychiatric testing is more sensitive and may be more a more helpful resource in multiple system atrophy.[12]

Sleep studies

Sleep disorders, particularly nocturnal stridor and REM sleep behavior disorder, are common in multiple system atrophy. Formal sleep studies should be considered, as research suggests that treatment can improve survival and quality of life.[16]

Histologic findings

Findings in MSA-P include widespread glial cytoplasmic inclusions (primarily in oligodendrocytes) and, to a lesser degree, neuronal cytoplasmic inclusions and neuronal nuclear inclusions. Immunostaining of inclusion bodies reveals the presence of alpha-synuclein fibrils.[17]

Other tests

Autonomic tests for orthostatic vital signs such as the tilt-table test and urodynamic studies can be conducted. Scintigraphy with iodine-123-metaiodobenzylguanidine (MIBG) has recently shown utility in the differential diagnosis between Parkinson disease and multiple system atrophy; this method shows high sensitivity and adequate specificity in this field.[18]

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Imaging Studies

Computed tomography (CT) scans may show cerebellar or brainstem atrophy late in the course of the disease.

A study using single-photon emission CT (SPECT) scanning revealed significantly decreased cerebellar and dorsolateral prefrontal perfusion in patients with multiple system atrophy, relative to that of control subjects.[12]

Magnetic resonance imaging (MRI) may show 1 or more of the following[19] :

  • Atrophy of the putamen is best seen on inversion-recovery coronal sequences and/or putaminal hypointense signal on T2-weighted sequences. In rare instances, hyperintense bands lateral to the putamen may be seen.
  • There may be narrowing and hypointensity of the pars compacta of the substantia nigra, which can give the appearance of fusion between the pars reticularis and the red nucleus

Positron emission tomography (PET) scans demonstrate decreased postsynaptic D2-receptor density and impaired uptake of fluoro-L-dopa.[20] The recently approved DaTscan single-photon emission computed tomography (SPECT) imaging can help confirm the diagnosis of parkinsonism but does not distinguish idiopathic Parkinson disease from multiple system atrophy.[21]

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Contributor Information and Disclosures
Author

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.

Coauthor(s)

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.

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, and Phi Beta Kappa

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 Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association

Disclosure: UCB Pharma Honoraria Speaking, consulting; Lundbeck Honoraria Speaking, consulting; Cyberonics Honoraria Speaking, consulting; Glaxo Smith Kline Honoraria Speaking, consulting; Pfizer Honoraria Speaking, consulting; Sleepmed/DigiTrace Honoraria Speaking, consulting

Additional Contributors

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.

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. 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. 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. Feb 2012;38(1):4-24. [Medline].

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

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

  6. 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].

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

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

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

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

  11. 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. Aug 2011;55(4):476-83. [Medline].

  12. 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].

  13. 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].

  14. 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].

  15. 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].

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

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

  18. 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. Dec 2011;113(10):823-9. [Medline].

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

  20. 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].

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

  22. 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].

  23. 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].

 
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