Abstract
We report the results of a consensus conference on the diagnosis of multiple system atrophy (MSA). We describe the clinical features of the disease, which include four domains: autonomic failure/urinary dysfunction, parkinsonism and cerebellar ataxia, and corticospinal dysfunction. We set criteria to define the relative importance of these features. The diagnosis of possible MSA requires one criterion plus two features from separate domains. The diagnosis of probable MSA requires the criterion for autonomic failure/urinary dysfunction plus poor levodopa responsive parkinsonism or cerebellar ataxia. The diagnosis of definite MSA requires pathological confirmation.
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Graham JG, Oppenheimer DR. Orthostatic hypotension and nicotine sensitivity in a case of multiple system atrophy.J Neurol Neurosurg Psychiat 1969; 32:28–34.
Quinn N. Multiple system atrophy. In: Marsden CD, Fahn S, eds.Movement disorders. London: Butterworths, 1996; 262–281.
Albanese A, Colosimo C, Bentivoglio AR, et al. Multiple system atrophy presenting as parkinsonism: clinical features and diagnostic criteria.J Neurol Neurosurg Psychiat 1995; 59:144–151.
Consensus Committee of the American Autonomic Society and the American Academy of Neurology. Consensus statement on the definition of orthostatic hypotension, pure autonomic failure, and multiple system atrophy.Neurology 1996; 46:1470.
Wenning GK, Ben-Shlomo Y, Magalaes M, Daniel SE, Quinn NP. Clinical features and natural history of multiple system atrophy: an analysis of 100 cases.Brain 1994; 117:835–845.
Klockgether T, Lüdtke R, Kramer B, et al. The natural history of degenerative ataxia: a retrospective study in 466 patients.Brain 1998; 121:589–600.
Gilman S. Multiple System Atrophy. In: Jankovic J, Tolosa E, eds.Parkinson's disease and movement disorders, 3rd edition. Baltimore: Williams and Wilkins, 1998; 245–262.
Sandroni P, Ahlskog JE, Fealey RD, Low PA. Autonomic involvement in extrapyramidal and cerebellar disorders.Clin Auton Res 1991; 1:147–155.
Mathias CJ, Williams AC. The Shy Drager Syndrome (and Multiple System Atrophy). In:Neurodegenerative diseases. Calne DB, ed. Philadelphia: WB Saunders, 1994; 743–768.
Beck RO, Betts CD, Fowler CJ. Genitourinary dysfunction in multiple system atrophy: clinical features and treatment in 62 cases.J Urology 1994; 151:1336–1341.
Daniel SE. The neuropathology and neurochemistry of multiple system atrophy. In: Bannister R, Mathias CJ, eds.Autonomic failure: a textbook of clinical disorders of the autonomic nervous system. Oxford: Oxford University Press; 1992:564–585.
Lantos PL. Multiple system atrophy.Brain Pathology 1997; 7:1293–1297.
Kluin KJ, Gilman S, Lohman M, Junck L. Characteristics of the dysarthria of multiple system atrophy.Arch Neurol 1996; 53:545–548.
Hughes AJ, Colosimo C, Kleedorfer B, Daniel SE, Lees AJ. The dopaminergic response in multiple system atrophy.J Neurol Neurosurg Psychiatry 1992; 55:1009–1013.
Parati EA, Fetoni V, Geminiani GC, et al. Response to L-DOPA in multiple system atrophy.Clin Neuropharmacol 1997; 16:139–144.
Fahn S, Elton R. Unified Parkinson's Disease Rating Scale Committee: Unified Parkinson's disease rating scale. In: Fahn S, Marsden CD, Calne D, eds.Recent developments in Parkinson's disease. New York: Macmillan; 1987:153–164.
Mathias CJ, Bannister R. Investigation of Autonomic Disorders. In: Autonomic failure.A textbook of clinical disorders of the autonomic nervous system. 3rd edition. Bannister R, Mathias CJ, eds. Oxford: Oxford University Press; 1992:255–290.
Low PA. The composite autonomic scoring scale for the laboratory quantitation of generalized autonomic failure.Mayo Clin Proc 1993; 68:748–752.
Palace J, Chandiramani VA, Fowler CJ. Value of sphincter electromyography in the diagnosis of multiple system atrophy.Muscle Nerve 1997; 20:1396–1403.
Schrag A, Kingsley D, Phatouros C, et al. Clinical usefulness of magnetic resonance imaging in multiple system atrophy.J Neurol Neurosurg Psychiatry 1998; 65:65–71.
Lang AE, Curran T, Provias J, Bergeron C. Striatonigral degeneration: iron deposition in putamen correlates with the slit-like void signal of magnetic resonance imaging.Can J Neurol Sci 1994; 21:311–318.
Testa D, Savoiardo M, Fetoni V, et al. Multiple system atrophy. Clinical and MR observations on 42 cases.Ital J Neuro Sci 1993; 14:211–216.
Mathias CJ. Autonomic dysfunction.Brit J Hosp Med 1987; 38:238–243.
Schulz JB, Klockgether T, Petersen D, et al. Multiple system atrophy: natural history, MRI morphology, and dopamine receptor imaging with 123IBZM-SPECT.J Neurol Neurosurg Psychiatry 1994; 57:1047–1056.
Litvan I, Goetz CG, Jankovic J, et al. What is the accuracy of the clinical diagnosis of multiple system atrophy? A clinicopathologic study.Arch Neurol 1997; 54:937–944.
Litvan I, Agid Y, Calne D, et al. NINDS-SPSP clinical criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-(Olszewski syndrome).Neurology 1996; 47:1–9.
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Gilman, S., Low, P., Quinn, N. et al. Consensus statement on the diagnosis of multiple system atrophy. Clinical Autonomic Research 8, 359–362 (1998). https://doi.org/10.1007/BF02309628
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DOI: https://doi.org/10.1007/BF02309628