eMedicine Specialties > Neurology > Movement and Neurodegenerative Diseases

Striatonigral Degeneration: Differential Diagnoses & Workup

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

Differential Diagnoses

Cortical Basal Ganglionic Degeneration
Paraneoplastic Cerebellar Degeneration
Essential Tremor
Parkinson Disease
Idiopathic Orthostatic Hypotension and other Autonomic Failure Syndromes
Parkinson-Plus Syndromes
Olivopontocerebellar Atrophy
Progressive Supranuclear Palsy

Workup

Laboratory Studies

No laboratory studies are indicated for striatonigral degeneration (multiple system atrophy with predominantly parkinsonian features [MSA-P]).

Imaging Studies

  • Positron emission tomography (PET) scan - Decreased postsynaptic D2 receptor density, impaired uptake of fluoro-L-dopa6
  • Single-proton emission computed tomography (SPECT) scan – (A recent study revealed significantly decreased cerebellar and dorsolateral prefrontal perfusion in patients with multiple system atrophy relative to that of control subjects)5
  • MRI may show 1 or more of the following:7
    • Atrophy of the putamen, best seen on inversion-recovery coronal sequences, and/or putaminal hypointense signal on T2-weighted sequences may, in rare instances, see hyperintense bands lateral to the putamina.
    • 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.
  • CT scans may show cerebellar or brainstem atrophy late in the course of the disease.

Other Tests

  • Autonomic tests for orthostatic vital signs, urodynamic studies
  • Electromyography: Shows denervation of external sphincter (urethral or anal); however, normal findings do not exclude the disease.
  • Clonidine Growth Hormone Test8 : Studies show that after infusion of clonidine, serum growth hormone concentration does not subsequently rise in patients with multiple system atrophy while the normal response, an increase in secretion, is found in Parkinson disease, pure autonomic failure, and control subjects.9
  • Neuropsychiatric evaluation: Recent studies suggest that cognitive impairment is more common than previously thought. 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.5
  • 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 both survival and quality of life.10

Histologic Findings

Findings of 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.

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

  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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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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