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  • Print publication year: 2005
  • Online publication date: August 2010

43 - Multiple system atrophy

from Part VII - Parkinson's and related movement disorders

Summary

Historical review

The term multiple system atrophy (MSA) was introduced by Graham and Oppenheimer in 1969 to denote a neurodegenerative disease characterized clinically by various combinations of autonomic, parkinsonian, cerebellar or pyramidal symptoms and signs and pathologically by cell loss and gliosis in some or all of the following structures: putamen, caudate nucleus, globus pallidus, substantia nigra, locus ceruleus, inferior olives, pontine nuclei, cerebellar Purkinje cells, intermediolateral cell columns and Onuf's nucleus of the spinal cord.

Previously, cases of MSA were reported under the rubrics of olivopontocerebellar atrophy (OPCA), idiopathic orthostatic hypotension (IOH) or progressive autonomic failure (PAF), Shy–Drager syndrome (SDS) and striatonigral degeneration (SND). Although Dejerine and Thomas were the first to introduce the term OPCA in 1900 reporting two sporadic cases of late-onset ataxia, the case of Stauffenberg in 1918, diagnosed in life as OPCA, was the first to associate cerebellar, parkinsonian and autonomic features with identified pathological lesions not only of olives, pons and cerebellum, but also of basal ganglia pigmentation and atrophy of putamen, with additional cell loss in caudate and globus pallidus (Quinn, 1994).

The term SND was introduced in 1960 by van der Eecken, Adams and van Bogaert (van der Eecken et al., 1960; Adams et al., 1961, 1964) who noted pronounced shrinkage and brownish discoloration of the putamen and pallidum as well as depigmentation of the substantia nigra in three patients with progressive and severe parkinsonism associated with cerebellar, pyramidal and autonomic features.

REFERENCES
Abbruzzese, G., Marchese, R. & Trompetto, C. (1997). Sensory and motor evoked potentials in multiple system atrophy: comparative study with Parkinson's disease. Mov. Disord., 12(3), 315–21
Abele, M., Schulz, J. B., Burk, K., Topka, H., Dichgans, J. & Klockgether, T. (2000a). Evoked potentials in multiple system atrophy (MSA). Acta Neurol. Scand., 101(2), 111–15
Abele, M., Schulz, J. B., Burk, K., Topka, H., Dichgans, J. & Klockgether, T. (2000b). Nerve conduction studies in multiple system atrophy. Eur. Neurol., 43(4), 221–3
Abele, M., Burk, K., Schols, L.et al. (2002). The aetiology of sporadic adult-onset ataxia. Brain, 125(5), 961–8
Abeliovich, A., Schmitz, Y., Farinas, I.et al. (2000). Mice lacking alpha-synuclein display functional deficits in the nigrostriatal dopamine system. Neuron, 25(1), 239–52
Adams, R. D., Bogaert, L. & Eecken, H. (1961). Dégénérescences nigro-striées et cérébello-nigro-striees. Psychiat. Neurol. 142, 219–59
Adams, R. D., Bogaert, L. & Eecken, H. (1964). Striato-nigral degeneration. J. Neuropathol. Expl. Neurol., 23, 584–608
Alam, M., Smith, G., Bleasdal-Barr, K., Pavitt, D. V. & Mathias, C. J. (1995). Effects of the peptide release inhibitor, octreotide, on daytime hypotension and on nocturnal hypertension in primary autonomic failure. J. Hypertens., 13(12/2), 1664–9
Albanese, A., Colosimo, C., Bentivoglio, A. R.et al. (1995). Multiple system atrophy presenting as parkinsonism: clinical features and diagnostic criteria. J. Neurol. Neurosurg. Psychiatr., 59(2), 144–51
Allan, S. M. & Rothwell, N. J. (2001). Cytokines and acute neurodegeneration. Nat. Rev. Neurosci., 2(10), 734–44
Aloe, L. & Fiore, M. (1997). TNF-alpha expressed in the brain of transgenic mice lowers central tyroxine hydroxylase immunoreactivity and alters grooming behaviour. Neurosci. Lett., 238(1–2), 65–8
Ansorge, O., Lees, A. J., Brooks, D. J. & Daniel, S. E. (1997a). Multiple system atrophy presenting as progressive supranuclear palsy and vice-versa: two unusual cases. Mov. Disord., 12(suppl.1), 96
Ansorge, O., Lees, A. J. & Daniel, S. E. (1997b). Pathological overlap of Alzheimer's disease, Parkinson's disease and multiple system atrophy. Neuropathol. Appl. Neurobiol., 23, 179
Antonini, A., Kazumata, K., Feigin, A.et al. (1998). Differential diagnosis of parkinsonism with [18F]fluorodeoxyglucose and PET. Mov. Disord., 13(2), 268–74
Arima, K., Nakamura, M., Sunohara, N.et al. (1997). Ultrastructural characterization of the tau-immunoreactive tubules in the oligodendroglial perikarya and their inner loop processes in progressive supranuclear palsy. Acta Neuropathol. (Berl.), 93(6), 558–66
Arima, K., Ueda, K., Sunohara, N.et al. (1998). NACP/alpha-synuclein immunoreactivity in fibrillary components of neuronal and oligodendroglial cytoplasmic inclusions in the pontine nuclei in multiple system atrophy. Acta Neuropathol. (Berl.), 96(5), 439–44
Baker, M., Litvan, I., Houlden, H.et al. (1999). Association of an extended halotype in the tau gene with progressive supranuclear palsy. Hum. Mol. Genet., 8(4), 711–15
Bandmann, O., Wenning, G. K., Quinn, N. P. & Harding, A. E. (1995). Arg296 to Cys296 polymorphism in exon 6 of cytochrome P-450–2D6 (CYP2D6) is not associated with multiple system atrophy. J. Neurol. Neurosurg. Psychiatr., 59(5), 557
Bandmann, O., Sweeney, M. G., Daniel, S. E.et al. (1997). Multiple-system atrophy is genetically distinct from identified inherited causes of spinocerebellar degeneration. Neurology, 49(6), 1598–604
Bannister, R. & Oppenheimer, D. R. (1972). Degenerative diseases of the nervous system associated with autonomic failure. Brain, 95(3), 457–74
Bannister, R. & Mathias, C. J. (1999a). Clinical features and evaluation of the primary chronic autonomic failure syndromes. In Autonomic Failure. A Textbook of Clinical Disorders of the Autonomic Nervous System., ed. C. J. Mathias & R. Bannister. Oxford: Oxford University Press, pp. 307–16
Bannister, R. & Mathias, C. J. (1999b). Investigation of autonomic disorders. In Autonomic Failure. A Textbook of Clinical Disorders of the Autonomic Nervous System, ed. C. J. Mathias & R. Bannister. Oxford: Oxford University Press, pp. 169–95
Barres, B. A., Hart, I. K., Coles, H. S.et al. (1992). Cell death and control of cell survival in the oligodendrocyte lineage. Cell, 70(1), 31–46
Baser, S. M., Meer, J., Polinsky, R. J. & Hallett, M. (1991). Sudomotor function in autonomic failure. Neurology, 41(10), 1564–6
Beck, R. O., Betts, C. D. & Fowler, C. J. (1994). Genitourinary dysfunction in multiple system atrophy: clinical features and treatment in 62 cases. J. Urol., 151(5), 1336–41
Benarroch, E. E., Smithson, I. L., Low, P. A. & Parisi, J. E. (1998). Depletion of catecholaminergic neurons of the rostral ventrolateral medulla in multiple systems atrophy with autonomic failure. Ann. Neurol., 43(2), 156–63
Ben-Shlomo, Y., Wenning, G. K., Tison, F. & Quinn, N. P. (1997). Survival of patients with pathologically proven multiple system atrophy: a meta-analysis. Neurology, 48, 384–93
Berciano, J., Combarros, O., Polo, J. M., Pascual, J. & Oterino, A. (1998). Who first described striatonigral degeneration?Neurology, 50(suppl 4), 120
Berciano, J., Valldeoriola, F., Ferrer, I.et al. (2002). Presynaptic parkinsonism in multiple system atrophy mimicking Parkinson's disease: a clinicopathological case study. Mov. Disord., 17(4), 812–16
Bermingham-McDonogh, O., McCabe, K. L. & Reh, T. A. (1996). Effects of GGF/neuregulins on neuronal survival and neurite outgrowth correlate with erbB2/neu expression in developing rat retina. Development, 122(5), 1427–38
Bösch, S. M., Schocke, M., Seppi, K., Hollosi, P., Poewe, W. & Wenning, G. K. (2002a). Abnormal striatal dopaminergic function in SCA2 revealed by beta-CIT and IBZM SPECT. Mov. Disord., 17(Suppl 5), 311
Bösch, S. M., Wenning, G. K., Ransmayr, G. & Poewe, W. (2002b). Dystonia in multiple system atrophy. J. Neurol. Neurosurg. Psychiatr., 72(3), 300–3
Boka, G., Anglade, P., Wallach, D., Javoy-Agid, F., Agid, Y. & Hirsch, E. C. (1994). Immunocytochemical analysis of tumor necrosis factor and its receptors in Parkinson's disease. Neurosci. Lett., 172(1–2), 151–4
Botez, M. I. & Young, S. N. (2001). Biogenic amine metabolites and thiamine in cerebrospinal fluid in heredodegenerative ataxias. Can. J. Neurol. Sci., 28(2), 134–40
Bower, J. H., Maraganore, D. M., McDonnell, S. K. & Rocca, W. A. (1997). Incidence of progressive supranuclear palsy and multiple system atrophy in Olmsted County, Minnesota, 1976 to 1990. Neurology, 49, 1284–8
Braak, H. & Braak, E. (1989). Cortical and subcortical argyrophilic grains characterize a disease associated with adult onset dementia. Neuropathol. Appl. Neurobiol., 15(1), 13–26
Braune, S., Auer, A., Schulte-Mönting, J., Schwerbrock, S. & Lücking, C. H. (1996a). Cardiovascular parameters: sensitivity to detect autonomic dysfunction and influence of age and sex in normal subjects. Clin. Auton. Res., 6, 3–15
Braune, S., Schulte-Monting, J., Schwerbrock, S. & Lucking, C. H. (1996b). Retest variation of cardiovascular parameters in autonomic testing. J. Auton. Nerv. Syst., 60(3), 103–7
Braune, S., Elam, M., Baron, R. & Low, P. A. (1999a). Assessment of blood pressure regulation. The International Federation of Clinical Neurophysiology. Electroencephalogr. Clin. Neurophysiol., Supp., 52, 287–91
Braune, S., Reinhardt, M., Schnitzer, R., Riedel, A. & Lücking, C. H. (1999b). Cardiac uptake of (123I)MIBG separates Parkinson's disease from multiple system atrophy. Neurology, 53(5), 1020–5
Brenneis, C., Seppi, K., Schocke, M. F.et al. (2003). Voxel-based morphometry detects cortical atrophy in the parkinson variant of multiple system atrophy. Mov. Disord., 18(10), 1132–8
Brooks, D. J., Luthert, P., Gadian, D. & Marsden, C. D. (1989). Does signal-attenuation on high-field T2-weighted MRI of the brain reflect regional cerebral iron deposition? Observations on the relationship between regional cerebral water proton T2-values and iron levels. J. Neurol. Neurosurg. Psychiatr., 52, 108–11
Brooks, D. J., Ibanez, V., Sawle, G. V.et al. (1990a). Differing patterns of striatal 18-F-Dopa uptake in Parkinson's disease, multiple system atrophy and progressive supranuclear palsy. Ann. Neurol., 28, 547–55
Brooks, D. J., Salmon, E. P., Mathias, C. J.et al. (1990b). The relationship between locomotor disability, autonomic dysfunction, and the integrity of the striatal dopaminergic system in patients with multiple system atrophy, pure autonomic failure, and Parkinson's disease, studied with PET. Brain, 113, 1539–52
Brooks, D. J., Ibanez, V., Sawle, G. V.et al. (1992). Striatal D2 receptor status in patients with Parkinson's disease, striatonigral degeneration, and progressive supranuclear palsy, measured with 11C-raclopride and positron emission tomography. Ann. Neurol., 31(2), 184–92
Brown, R. G., Pillon, B., Uttner, I., Payan, C. & Lacomblez, L. (2002). Members of the Neuropsychology Working Group and NNIPPS Consortium. Cognitive function in patients with Progressive Supranuclear Palsy (PSP) and Multiple System Atrophy (MSA). Mov. Disord., 17(Suppl 5), 221
Brücke, T., Wenger, S. & Asenbaum, S. (1993). Dopamine D2 receptor imaging and measurement with SPECT. Adv. Neurol., 60, 494–500
Brücke, T., Asenbaum, S., Pirker, W.et al. (1997). Measurement of the dopaminergic degeneration in Parkinson's disease with [123I] beta-CIT and SPECT. Correlation with clinical findings and comparison with multiple system atrophy and progressive supranuclear palsy. J. Neural Transm. Suppl., 50, 9–24
Brücke, T., Djamshidian, S., Bencsits, G., Pirker, W., Asenbaum, S. & Podreka, I. (2000). SPECT and PET imaging of the dopaminergic system in Parkinson's disease. J. Neurol., 247(4), IV/2–7
Burden, S. & Yarden, Y. (1997). Neuregulins and their receptors: a versatile signaling module in organogenesis and oncogenesis. Neuron, 18(6), 847–55
Burn, D. J. & Jaros, E. (2001). Multiple system atrophy: cellular and molecular pathology. Mol. Pathol., 54(6), 419–26
Burn, D. J., Sawle, G. V. & Brooks, D. J. (1994). Differential diagnosis of Parkinson's disease, multiple system atrophy, and Steele–Richardson–Olszewski syndrome: discriminant analysis of striatal 18F-dopa PET data. J. Neurol. Neurosurg. Psychiatr., 57(3), 278–84
Burn, D. J., Rinne, J. O., Quinn, N. P., Lees, A. J., Marsden, C. D. & Brooks, D. J. (1995). Striatal opioid receptor binding in Parkinson's disease, striatonigral degeneration and Steele–Richardson–Olszewski syndrome. A [11C]diprenorphine PET study. Brain, 118(4), 951–8
Buttery, P. C. & ffrench-Constant, C. (1999). Laminin-2/integrin interactions enhance myelin membrane formation by oligodendrocytes. Mol. Cell. Neurosci., 14(3), 199–212
Cairns, N. J., Atkinson, P. F., Hanger, D. P., Anderton, B. H., Daniel, S. E. & Lantos, P. L. (1997a). Tau protein in the glial cytoplasmatic inclusions of multiple system atrophy can be distinguished from abnormal tau in Alzheimer's disease. Neurosci. Lett., 230(1), 49–52
Cairns, N. J., Atkinson, P. F., Kovacs, T., Lees, A. J., Daniel, S. E. & Lantos, P. L.Apolipoprotein E ε4 allele frequency in patients with multiple system atrophy. Neurosci. Lett., 221(2–3), 161–4
Canoll, P. D., Musacchio, J. M., Hardy, R., Reynolds, R., Marchionni, M. A. & Salzer, J. L. (1996). GGF/neuregulin is a neuronal signal that promotes the proliferation and survival and inhibits the differentiation of oligodendrocyte progenitors. Neuron, 17(2), 229–43
Canoll, P. D., Kraemer, R., Teng, K. K., Marchionni, M. A. & Salzer, J. L. (1999). GGF/neuregulin induces a phenotypic reversion of oligodendrocytes. Mol. Cell. Neurosci., 13(2), 79–94
Caplan, L. R. (1984). Clinical features of sporadic (Dejerine-Thomas) olivopontocerebellar atrophy. Adv. Neurol., 41, 217–24
Chandiramani, V. A., Palace, J. & Fowler, C. J. (1997). How to recognize patients with parkinsonism who should not have urological surgery. Br. J. Urol., 80(1), 100–4
Chin, S. S. & Goldman, J. E. (1996). Glial inclusions in CNS degenerative diseases. J. Neuropathol. Exp. Neurol., 55(5), 499–508
Chio, A., Magnani, C. & Schiffer, D. (1998). Prevalence of Parkinson's disease in northwestern Italy: comparison of tracer methodology and clinical ascertainment of cases. Mov. Disord., 13(3), 400–5
Churchyard, A., Donnan, G. A., Hughes, A.et al. (1993). Dopa resistance in multiple-system atrophy: loss of postsynaptic D2 receptors. Ann. Neurol., 34(2), 219–26
Clarke, C. E. & Lowry, M. (2000). Basal ganglia metabolite concentrations in idiopathic Parkinson's disease and multiple system atrophy measured by proton magnetic resonance spectroscopy. Eur. J. Neurol., 7(6), 661–5
Clarke, C. E., Ray, P. S. & Speller, J. M. (1999). Failure of the clonidine growth hormone stimulation test to differentiate multiple system atrophy from early or advanced idiopathic Parkinson's disease. The Lancet, 353, 1329–30
Clayton, D. F. & George, J. M. (1999). Synucleins in synaptic plasticity and neurodegenerative disorders. J. Neurosci. Res., 58(1), 120–9
Cohen, J., Low, P., Fealey, R., Sheps, S. & Jiang, N. S. (1987). Somatic and autonomic function in progressive autonomic failure and multiple system atrophy. Ann. Neurol., 22, 692–9
Colosimo, C. (1998). Pisa syndrome in a patient with multiple system atrophy. Mov. Disord., 13(3), 607–9
Colosimo, C. & Pezzella, F. R. (2002). The symptomatic treatment of multiple system atrophy. Eur. J. Neurol., 9(3), 195–9
Colosimo, C., Merello, M. & Pontieri, F. E. (1996). Amantadine in parkinsonian patients unresponsive to levodopa: a pilot study. J. Neurol., 243, 422–5
Colosimo, C., Albanese, A., Hughes, A. J., Bruin, V. M. & Lees, A. J. (1995). Some specific clinical features differentiate multiple system atrophy (striatonigral variety) from Parkinson's disease. Arch. Neurol., 52(3), 294–8
Colosimo, C., Inghilleri, M. & Chaudhuri, K. R. (2000). Parkinson's disease misdiagnosed as multiple system atrophy by sphincter electromyography. J. Neurol., 247, 559–61
Colosimo, C., Geser, F. and Wenning, G. K. (2004). Clinical spectrum and pathological features of multiple system atrophy. In: Animal Models of Movement Disorders, ed. M. LeDoux, pp. 541–70. Amsterdam: Elsevier
Consensus statement on the definition of orthostatic hypotension, pure autonomic failure, and multiple system atrophy. (1996). J. Neurol. Sci., 144(1–2), 218–19
Cortes, R., Camps, M., Gueye, B., Probst, A. & Palacios, J. M. (1989). Dopamine receptors in human brain: autoradiographic distribution of D1 and D2 sites in Parkinson syndrome of different etiology. Brain Res., 483(1), 30–8
Daniel, S. E. (1999). The neuropathology and neurochemistry of multiple system atrophy. In Autonomic Failure. A Textbook of Clinical Disorders of the Autonomic Nervous System, ed. C. J. Mathias & R. Bannister. Oxford: Oxford University Press, pp. 321–8
Davie, C. A., Wenning, G. K., Barker, G. J.et al. (1995). Differentiation of multiple system atrophy from idiopathic Parkinson's disease using proton magnetic resonance spectroscopy. Ann. Neurol., 37, 204–10
Dejerine, J. & Thomas, A. A. (1900). L'atrophie olivo-ponto-cérébelleuse. Nouv. Iconogr. Salpêtrièr., 13, 330–70
Delalande, I., Hache, J. C., Forzy, G., Bughin, M., Benjadjali, J. & Destee, A. (1998). Do visual-evoked potentials and spatiotemporal contrast sensitivity help to distinguish idiopathic Parkinson's disease and multiple system atrophy?Mov. Disord., 13(3), 446–52
Volder, A. G., Francart, J., Laterre, C.et al. (1989). Decreased glucose utilization in the striatum and frontal lobe in probable striatonigral degeneration. Ann. Neurol., 26, 239–47
Dexter, D. T., Carayon, A., Javoy-Agid, F.et al. (1991). Alterations in the levels of iron, ferritin and other trace metals in Parkinson's disease and other neurodegenerative diseases affecting the basal ganglia. Brain, 114(4), 1953–75
Dickson, D. W., Liu, W., Hardy, J.et al. (1999a). Widespread alterations of alpha-synuclein in multiple system atrophy. Am. J. Pathol., 155(4), 1241–51
Dickson, D. W., Lin, W., Liu, W. K. & Yen, S. H. (1999b). Multiple system atrophy: a sporadic synucleinopathy. Brain Pathol., 9(4), 721–32
Drayer, B. P., Olanow, W., Burger, P., Johnson, G. A., Herfkens, R. & Ricderer, S. (1986). Parkinson Plus Syndrome: diagnosis using high field MR imaging of brain iron. Radiology, 159, 493–8
Duda, J. E., Shah, U., Arnold, S. E., Lee, V. M. & Trojanowski, J. Q. (1999). The expression of alpha-, beta-, and gamma-synucleins in olfactory mucosa from patients with and without neurodegenerative diseases. Exp. Neurol., 160(2), 515–22
Eardley, I., Quinn, N. P., Fowler, C. J.et al. (1989). The value of urethral sphincter electromyography in the differential diagnosis of parkinsonism. Br. J. Urol., 64, 360–2
Eichhorn, T. E. & Oertel, W. H. (2001). Macrogol 3350/electrolyte improves constipation in Parkinson's disease and multiple system atrophy. Mov. Disord., 16(6), 1176–7
Eidelberg, D., Takikawa, S., Moeller, J. R.et al. (1993). Striatal hypometabolism distinguishes striatonigral degeneration from Parkinson's disease. Ann. Neurol., 33, 518–27
El-Agnaf, O. M., Curran, M. D., Wallace, A.et al. (1998). Mutation screening in exons 3 and 4 of alpha-synuclein in sporadic Parkinson's and sporadic and familial dementia with Lewy bodies cases. Neuroreport, 9(17), 3925–7
Ellis, C., Lemmens, G., Williams, S. C. R., Simmons, A., Leigh, P. N. & Chaudhuri, K. R. (1996). Striatal changes in striatonigral degeneration and Parkinson's disease: a proton magnetic resonance spectroscopy study. Mov. Disord., 11, 104
Ellis, C. E., Schwartzberg, P. L., Grider, T. L., Fink, D. W. & Nussbaum, R. L. (2001). Alpha-synuclein is phosphorylated by members of the Src family of protein-tyrosine kinases. J. Biol. Chem., 276(6), 3879–84
Engelender, S., Kaminsky, Z., Guo, X.et al. (1999). Synphilin-I associates with alpha-synuclein and promotes the formation of cytosolic inclusions. Nat. Genet., 22(1), 110–14
Fearnley, J. M. & Lees, A. J. (1990). Striatonigral degeneration. A clinico-pathological study. Brain, 113(6), 1823–42
Fearnley, J. M. & Lees, A. J. (1991). Ageing and Parkinson's disease: substantia nigra regional selectivity. Brain, 114(5), 2283–301
Federico, F., Simone, I. L., Lucivero, V.et al. (1999). Usefulness of proton magnetic resonance spectroscopy in differentiating parkinsonian syndromes. Ital. J. Neurol. Sci., 20(4), 223–9
Fernagut, P., Diguet, E., Stefanova, N.et al. (2002). Subacute systemic 3-nitropropionic acid intoxication induces a distinct motor disorder in adult C57Bl/6 mice: behavioural and histopathological characterisation. Neuroscience, 114(4), 1005–17
Fleischhacker, H. (1924). Afamiliäre chronisch progressive Erkrankung des mittleren Lebensalters vom Pseudosklerosetyp. Z. Gesamte Neurol. Psychiatri., 91, 1–22
Flugel, K. A., Mosler, T. A. & Wild, R. H. (1984). Bulbar paralytic symptoms including acute vocal cord paralysis in Shy-Drager syndrome. Nervenarzt, 55(6), 293–8
Frost, E. E., Buttery, P. C., Milner, R. & ffrench-Constant, C. (1999). Integrins mediate a neuronal survival signal for oligodendrocytes. Curr. Biol., 9(21), 1251–4
Fujita, T., Doi, M., Ogata, T., Kanazawa, I. & Mizusawa, H. (1993). Cerebral cortical pathology of sporadic olivopontocerebellar atrophy. J. Neurol. Sci., 116(1), 41–6
Futamura, N., Matsumura, R., Fujimoto, Y., Horikawa, H., Suzumura, A. & Takayanagi, T. (1998). CAG repeat expansions in patients with sporadic cerebellar ataxia. Acta Neurol. Scand., 98(1), 55–9
Gahring, L. C., Rogers, S. W. & Twyman, R. E. (1997). Autoantibodies to glutamate receptor subunit GluR2 in nonfamilial olivopontocerebellar degeneration. Neurology, 48(2), 494–500
Gai, W. P., Power, J. H., Blumbergs, P. C. & Blessing, W. W. (1998). Multiple-system atrophy: a new alpha-synuclein disease?Lancet, 352(9127), 547–8
Gai, W. P., Pountney, D. L., Power, J. H.et al. (2003). α-Synuclein fibrils constitute the central core of oligodendroglial inclusion filaments in multiple system atrophy. Exp. Neurol., 181(1), 68–78
Garratt, H., Wenning, G. K., Barnes, C., Howard, R. & Quinn, N. P. (1994). Speech dysfunction and levodopa response in multiple system atrophy. N. Trends Clin. Neuropharmacol., 206–7
Gash, D. M., Zhang, Z. & Gerhardt, G. (1998). Neuroprotective and neurorestorative properties of GDNF. Ann. Neurol., 44(3 Suppl 1), 121–5
Gerhard, A., Banati, R., Cagnin, A.et al. (2000). In vivo imaging of activated microglia with [11C]PK11195 positron emission tomography in patients with multiple system atrophy. Mov. Disord., 15(Suppl. 3), 215–16
Ghaemi, M., Hilker, R., Rudolf, J., Sobesky, J. & Heiss, W. D. (2002). Differentiating multiple system atrophy from Parkinson's disease: contribution of striatal and midbrain MRI volumetry and multi-tracer PET imaging. J. Neurol. Neurosurg. Psychiatr., 73(5), 517–23
Ghorayeb, I., Fernagut, P. O., Aubert, I.et al. (2000). Toward a primate model of L-dopa-unresponsive parkinsonism mimicking striatonigral degeneration. Mov. Disord., 15(3), 531–6
Ghorayeb, I., Fernagut, P. O., Hervier, L., Labattu, B., Bioulac, B. & Tison, F. (2002a). A ‘single toxin-double lesion’ rat model of striatonigral degeneration by intrastriatal 1-methyl-4-phenylpyridinium ion injection: a motor behavioural analysis. Neuroscience, 115(2), 533–46
Ghorayeb, I., Fernagut, P. O., Stefanova, N., Wenning, G. K., Bioulac, B. & Tison, F. (2002b). Dystonia is predictive of subsequent altered dopaminergic responsiveness in a chronic 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine+3-nitropropionic acid model of striatonigral degeneration in monkeys. Neurosci. Lett., 335(1), 34–8
Ghorayeb, I., Yekhlef, F., Chrysostome, V., Balestre, E., Bioulac, B. & Tison, F. (2002c). Sleep disorders and their determinants in multiple system atrophy. J. Neurol. Neurosurg. Psychiatr., 72(6), 798–800
Giladi, N., Simon, E. S., Korczyn, A. D.et al. (2000). Anal sphincter EMG does not distinguish between multiple system atrophy and Parkinson's disease. Muscle Nerve, 23(5), 731–4
Gilman, S., Koeppe, R. A., Junck, L., Kluin, K. J., Lohman, M. & St. Laurent, R. T. (1994). Patterns of cerebral glucose metabolism detected with positron emission tomography differ in multiple system atrophy and olivopontocerebellar atrophy. Ann. Neurol., 36, 166–75
Gilman, S., Koeppe, R. A., Junck, L., Kluin, K. J., Lohman, M. & St. Laurent, R. T. (1995). Benzodiazepine receptor binding in cerebellar degenerations studied with positron emission tomography. Ann. Neurol., 38, 176–85
Gilman, S., Sima, A. A., Junck, L., Kluin, K. J., Koeppe, R. A. & Lohman, M. E. & Little, R. (1996). Spinocerebellar ataxia type 1 with multiple system degeneration and glial cytoplasmic inclusions. Ann. Neurol., 39(2), 241–55
Gilman, S., Low, P., Quinn, N.et al. (1998). Consensus statement on the diagnosis of multiple system atrophy. American Autonomic Society and American Academy of Neurology. Clin. Auton. Res., 8(6), 359–62
Gilman, S., Low, P., Quinn, N.et al. (1999). Consensus statement on the diagnosis of multiple system atrophy. J. Neurol. Sci., 163(1), 94–8
Gilman, S., Little, R., Johanns, J.et al. (2000). Evolution of sporadic olivopontocerebellar atrophy into multiple system atrophy. Neurology, 55(4), 527–32
Goedert, M. & Spillantini, M. G. (1998). Lewy body diseases and multiple system atrophy as alpha-synucleinopathies. Mol. Psychiatr., 3(6), 462–5
Gomez-Tortosa, E., Gonzalo, I., Newell, K., Yebenes, Garcia J., Vonsattel, P. & Hyman, B. T. (2002). Patterns of protein nitration in dementia with Lewy bodies and striatonigral degeneration. Acta Neuropathol. (Berl.), 103(5), 495–500
Gonzalez-Quevedo, A., Garcia, J. C., Fernandez, R. & Cartaya, Fernandez L. (1993). Monoamine metabolites in normal human cerebrospinal fluid and in degenerative diseases of the central nervous system. Bol. Estud. Med. Biol., 41(1–4), 13–19
Gosset, A., Pellissier, J. F., Delpuech, F. & Khalil, R. (1983). Degenerescence striato-nigrique associée a une atrophie olivo-ponto-cerebelleuse. Rev. Neurol. (Paris., 139(2), 125–39
Goto, S., Hirano, A. & Matsumoto, S. (1989a). Subdivisional involvement of nigrostriatal loop in idiopathic Parkinson's disease and striatonigral degeneration. Ann. Neurol., 26(6), 766–70
Goto, S., Hirano, A. & Rojas-Corona, R. R. (1989b). Calcineurin immunoreactivity in striatonigral degeneration. Acta Neuropathol. (Berl.), 78(1), 65–71
Goto, S., Hirano, A. & Rojas-Corona, R. R. (1989c). Immunohistochemical visualization of afferent nerve terminals in human globus pallidus and its alteration in neostriatal neurodegenerative disorders. Acta. Neuropathol. (Berl.), 78(5), 543–50
Goto, S., Hirano, A. & Matsumoto, S. (1990). Met-enkephalin immunoreactivity in the basal ganglia in Parkinson's disease and striatonigral degeneration. Neurology, 40(7), 1051–6
Goto, S., Matsumoto, S., Ushio, Y. & Hirano, A. (1996). Subregional loss of putaminal efferents to the basal ganglia output nuclei may cause parkinsonism in striatonigral degeneration. Neurology, 47(4), 1032–6
Gouider-Khouja, N., Vidailhet, M., Bonnet, A. M., Pichon, J. & Agid, Y. (1995). “Pure” striatonigral degeneration and Parkinson's disease: a comparative clinical study. Mov. Disord., 10(3), 288–94
Graham, J. G. & Oppenheimer, D. R. (1969). Orthostatic hypotension and nicotine sensitivity in a case of multiple system atrophy. J. Neurol. Neurosurg. Psychiatr., 32(1), 28–34
Gu, M., Gash, M. T., Cooper, J. M.et al. (1997). Mitochondrial respiratory chain function in multiple system atrophy. Mov. Disord., 12(3), 418–22
Hashida, H., Goto, J., Zhao, N.et al. (1998). Cloning and mapping of ZNF231, a novel brain-specific gene encoding neuronal double zinc finger protein whose expression is enhanced in a neurodegenerative disorder, multiple system atrophy (MSA). Genomics, 54, 50–8
Hashimoto, M., Rockenstein, E., Mante, M., Mallory, M. & Masliah, E. (2001). Beta-synuclein inhibits alpha-synuclein aggregation: a possible role as an antiparkinsonian factor. Neuron, 32(2), 213–23
Hayashi, M., Isozaki, E., Oda, M., Tanabe, H. & Kimura, J. (1997). Loss of large myelinated nerve fibres of the recurrent laryngeal nerve in patients with multiple system atrophy and vocal cord palsy. J. Neurol. Neurosurg. Psychiatr., 62(3), 234–8
Hirsch, E. C., Hunot, S., Damier, P. & Faucheux, B. (1998). Glial cells and inflammation in Parkinson's disease: a role in neurodegeneration?Ann. Neurol., 44(3 Suppl 1), 115–20
Holmberg, B., Rosengren, L., Karlsson, J. E. & Johnels, B. (1998). Increased cerebrospinal fluid levels of neurofilament protein in progressive supranuclear palsy and multiple-system atrophy compared with Parkinson's disease. Mov. Disord., 13(1), 70–7
Holmberg, B., Johnels, B., Ingvarsson, P., Eriksson, B. & Rosengren, L. (2001). CSF-neurofilament and levodopa tests combined with discriminant analysis may contribute to the differential diagnosis of Parkinsonian syndromes. Parkinsonism Relat. Disord., 8(1), 23–31
Horoupian, D. S. & Dickson, D. W. (1991). Striatonigral degeneration, olivopontocerebellar atrophy and “atypical” Pick disease. Acta Neuropathol. (Berl.), 81(3), 287–95
Hu, M. T. M., Simmons, A., Glover, A.et al. (1998). Proton magnetic resonance spectroscopy of the putamen in Parkinson's disease and multiple system atrophy. Mov. Disord., 13, 182
Hughes, A. J., Colosimo, C., Kleedorfer, B., Daniel, S. E. & Lees, A. J. (1992). The dopaminergic response in multiple system atrophy. J. Neurol. Neurosurg. Psychiatr., 55(11), 1009–13
Hughes, A. J., Daniel, S. E., Ben-Shlomo, Y. & Lees, A. J. (2002). The accuracy of diagnosis of parkinsonian syndromes in a specialist movement disorder service. Brain, 125(4), 861–70
Hughes, R. G., Gibbin, K. P. & Lowe, J. (1998). Vocal fold abductor paralysis as a solitary and fatal manifestation of multiple system atrophy. J. Laryng. Otol., 112(2), 177–8
Hunot, S., Dugas, N., Faucheux, B.et al. (1999). Fc-epsilon RII/CD23 is expressed in Parkinson's disease and induces, in vitro, production of nitric oxide and tumor necrosis factor-alpha in glial cells. J. Neurosci., 19(9), 3440–7
Hussain, I. F., Brady, C. M., Swinn, M. J., Mathias, C. J. & Fowler, C. J. (2001). Treatment of erectile dysfunction with sildenafil citrate (Viagra) in parkinsonism due to Parkinson's disease or multiple system atrophy with observations on orthostatic hypotension. J. Neurol. Neurosurg. Psychiatr., 71, 371–4
Ichikawa, N. (1986). Study on monoamine metabolite contents of cerebrospinal fluid in patients with neurodegenerative diseases. Tohoku J. Exp. Med., 150(4), 435–46
Iranzo, A., Santamaria, J. & Tolosa, E. (2000). Continuous positive air pressure eliminates nocturnal stridor in multiple system atrophy. Barcelona Multiple System Atrophy Study Group. The Lancet, 356, 1329–30
Ito, H., Kusaka, H., Matsumoto, S. & Imai, T. (1996). Striatal efferent involvement and its correlation to levodopa efficacy in patients with multiple system atrophy. Neurology, 47(5), 1291–9
Iwahashi, K., Miyatake, R., Tsuneoka, Y.et al. (1995). A novel cytochrome P-450IID6 (CYPIID6) mutant gene associated with multiple system atrophy. J. Neurol. Neurosurg. Psychiatr., 58(2), 263–4
Jankovic, J., Gilden, J. L., Hiner, B. C.et al. (1993a). Neurogenic orthostatic hypotension: a double-blind, placebo-controlled study with midodrine. Am. J. Med., 95, 38–48
Jankovic, J., Rajput, A. H., Golbe, L. & Goodman, J. C. (1993b). What is it? Case 1. Parkinsonism, dysautonomia, and ophthalmoparesis. Mov. Disord., 8, 525–32
Jaros, E. & Burn, D. J. (2000). The pathogenesis of multiple system atrophy: past, present and future. Mov. Disord., 15(5), 784–8
Johnsen, J. A. & Miller, V. T. (1986). Tobacco intolerance in multiple system atrophy. Neurology, 36, 986–8
Kahle, P. J., Neumann, M., Ozmen, L.et al. (2002). Hyperphosphorylation and insolubility of alpha-synuclein in transgenic mouse oligodendrocytes. EMBO Rep., 3(6), 583–8
Kan, A. E. (1978). Striatonigral degeneration. Pathology, 10(1), 45–52
Kato, S., Oda, M., Hayashi, H.et al. (1995). Decrease of medullary catecholaminergic neurons in multiple system atrophy and Parkinson's disease and their preservation in amyotrophic lateral sclerosis. J. Neurol. Sci., 132(2), 216–21
Kaufmann, H., Oribe, E., Miller, M., Knott, P., Wiltshire-Clement, M. & Yahr, M. D. (1992). Hypotension-induced vasopressin release distinguishes between pure autonomic failure and multiple system atrophy with autonomic failure. Neurology, 42(3), 590–3
Kawamoto, Y., Nakamura, S., Akiguchi, I. & Kimura, J. (1999). Increased brain-derived neurotrophic factor-containing axons in the basal ganglia of patients with multiple system atrophy. J. Neuropathol. Exp. Neurol., 58(7), 765–72
Kew, J., Gross, M. & Chapman, P. (1990). Shy–Drager syndrome presenting as isolated paralysis of vocal cord abductors. Br. Med. J., 300, 1441
Kihara, M., Suganoya, J. & Takahashi, A. (1991). The assessment of sudomotor dysfunction in multiple system atrophy. Clin. Auton. Res., 1, 297–302
Kikuchi, A., Takeda, A., Onodera, H.et al. (2002). Systemic increase of oxidative nucleic acid damage in Parkinson's disease and multiple system atrophy. Neurobiol. Dis., 9(2), 244–8
Kim, Y. J., Ichise, M., Ballinger, J. R.et al. (2002). Combination of dopamine transporter and D2 receptor SPECT in the diagnostic evaluation of PD, MSA, and PSP. Mov. Disord., 17(2), 303–12
Kimber, J. R., Watson, L. & Mathias, C. J. (1997). Distinction of idiopathic Parkinson's disease from multiple system atrophy by stimulation of growth-hormone release with clonidine. The Lancet, 349, 1877–81
Kimber, J., Watson, L. & Mathias, C. J. (1999a). Abnormal suppression of arginine-vasopressin by clonidine in multiple system atrophy. Clin. Auton. Res., 9(5), 271–4
Kimber, J., Watson, L. & Mathias, C. J. (1999b). Neuroendocrine responses to levodopa in multiple system atrophy (MSA). Mov. Disord., 14(6), 981–7
Kimber, J., Mathias, C. J., Lees, A. J.et al. (2000). Physiological, pharmacological and neurohormonal assessment of autonomic function in progressive supranuclear palsy. Brain, 123(7), 1422–30
Kirby, R., Fowler, C. J., Gosling, J. & Bannister, R. (1986). Urethro-vesical dysfunction in progressive autonomic failure with multiple system atrophy. J. Neurol. Neurosurg. Psychiatr., 49, 554–62
Kiyosawa, K., Mokuno, K., Murakami, N.et al. (1993). Cerebrospinal fluid 28-kDa calbindin-D as a possible marker for Purkinje cell damage. J. Neurol. Sci., 118(1), 29–33
Klein, C., Wenning, G. K. & Quinn, N. P. (1995). Pseudotransitory ischemic attacks as the initial symptom of multiple system atrophy. Nervenarzt, 66(2), 133–5
Klein, C., Brown, R., Wenning, G. & Quinn, N. (1997). The “cold hand sign” in multiple system atrophy. Mov. Disord., 12(4), 514–18
Klockgether, T., Schroth, G., Diener, H. C. & Dichgans, J. (1990). Idiopathic cerebellar ataxia of late onset: natural history and MRI morphology. J. Neurol. Neurosurg. Psychiatr., 53, 297–305
Kluin, K. J., Gilman, S., Lohmann, M. & Junck, L. (1996). Characteristics of the dysarthria of multiple system atrophy. Arch. Neurol., 53, 545–8
Kofler, M., Müller, J., Wenning, G. K.et al. (2001). The auditory startle reaction in parkinsonian disorders. Mov. Disord., 16(1), 62–71
Kofler, M., Muller, J., Seppi, K. & Wenning, G. K. (2003). Exaggerated auditory startle responses in multiple system atrophy: a comparative study of parkinson and cerebellar subtypes. Clin. Neurophysiol., 114(3), 541–7
Konagaya, M., Konagaya, Y., Honda, H. & Iida, M. (1993). A clinico-MRI study of extrapyramidal symptoms in multiple system atrophy – linear hyperintensity in the outer margin of the putamen. No To Shinkei, 45(6), 509–13
Konagaya, M., Konagaya, Y. & Iida, M. (1994). Clinical and magnetic resonance imaging study of extrapyramidal symptoms in multiple system atrophy. J. Neurol. Neurosurg. Psychiatr., 57, 1528–31
Konagaya, M., Sakai, M., Matsuoka, Y., Goto, Y., Yoshida, M. & Hashizume, Y. (1998). Patho-MR imaging study in the putaminal margin in multiple system atrophy. No To Shinkei, 50(4), 383–5
Konagaya, M., Sakai, M., Matsuoka, Y., Konagaya, Y. & Hashizume, Y. (1999). Multiple system atrophy with remarkable frontal lobe atrophy. Acta Neuropathol. (Berl.), 97(4), 423–8
Konagaya, M., Konagaya, Y., Sakai, M., Matsuoka, Y. & Hashizume, Y. (2002). Progressive cerebral atrophy in multiple system atrophy. J. Neurol. Sci., 195(2), 123–7
Konings, C. H., Kuiper, M. A., Teerlink, T., Mulder, C., Scheltens, P. & Wolters, E. C. (1999). Normal cerebrospinal fluid glutathione concentrations in Parkinson's disease, Alzheimer's disease and multiple system atrophy. J. Neurol. Sci., 168(2), 112–15
Konno, H., Yamamoto, T., Iwasaki, Y. & Iizuka, H. (1986). Shy–Drager syndrome and amyotrophic lateral sclerosis. Cytoarchitectonic and morphometric studies of sacral autonomic neurons. J. Neurol. Sci., 73(2), 193–204
Kraft, E., Schwarz, J., Trenkwalder, C., Vogl, T., Pfluger, T. & Oertel, W. H. (1999). The combination of hypointense and hyperintense signal changes on T2-weighted magnetic resonance imaging sequences: a specific marker of multiple system atrophy?Arch. Neurol., 56(2), 225–8
Kraft, E., Trenkwalder, C. & Auer, D. P. (2002). T2*-weighted MRI differentiates multiple system atrophy from Parkinson's disease. Neurology, 59(8), 1265–7
Krüger, R., Kuhn, W., Muller, T.et al. (1998). Ala30Pro mutation in the gene encoding alpha-synuclein in Parkinson's disease. Nat. Genet., 18(2), 106–8
Krüger, R., Vieira-Saecker, A. M., Kuhn, W.et al. (1999). Increased susceptibility to sporadic Parkinson's disease by a certain combined alpha-synuclein/apolipoprotein E genotype. Ann. Neurol., 45(5), 611–17
Kuiper, M. A., Visser, J. J., Bergmans, P. L., Scheltens, P. & Wolters, E. C. (1994). Decreased cerebrospinal fluid nitrate levels in Parkinson's disease, Alzheimer's disease and multiple system atrophy patients. J. Neurol. Sci., 121(2), 46–9
Kuiper, M. A., Teerlink, T., Visser, J. J., Bergmans, P. L., Scheltens, P. & Wolters, E. C. (2000). L-glutamate, L-arginine and L-citrulline levels in cerebrospinal fluid of Parkinson's disease, multiple system atrophy, and Alzheimer's disease patients. J. Neural Transm., 107(2), 183–9
Kume, A., Takahashi, A. & Hashizume, Y. (1993). Neuronal cell loss of the striatonigral system in multiple system atrophy. J. Neurol. Sci., 117(1–2), 33–40
Lambie, C. G., Latham, O. & Mac Donald, G. L. (1947). Olivo-ponto-cerebellar atrophy (Marie's ataxia). Med. J. Aust., 2, 626–32
Lang, A. E., Curran, T., Provias, J. & Bergeron, C. (1994). Striatonigral degeneration: iron deposition in putamen correlated with the slit-like void signal of magnetic resonance imaging. Can. J. Neurol. Sci., 21, 311–18
Lang, A. E., Lozano, A., Duff, J.et al. (1997). Medial pallidotomy in late-stage Parkinson's disease and striatonigral degeneration. Adv. Neurol., 74, 199–211
Langston, W. (1936). Orthostatic hypotension: report of a case. Ann. Int. Med., 10, 688–95
Lantos, P. L. (1998). The definition of multiple system atrophy: a review of recent developments. J. Neuropathol. Exp. Neurol., 57, 1099–111
Lees, A. J. (1999). The treatment of the motor disorders of multiple system atrophy. In Autonomic Failure, ed. C. J. Mathias & R. Bannister. Oxford: Oxford University Press, pp. 357–63
Lees, A. J. & Bannister, R. (1981). The use of lisuride in the treatment of multiple system atrophy with autonomic failure (Shy Drager syndrome). J. Neurol. Neurosurg. Psychiatr., 44, 347–51
Leggo, J., Dalton, A., Morrison, P. J.et al. (1997). Analysis of spinocerebellar ataxia types 1,2,3, and 6, dentatorubral-pallidoluysian atrophy, and Friedreich's ataxia genes in spinocerebellar ataxia patients in the UK. J. Med. Genet., 34(12), 982–5
Libelius, R. & Johannson, F. (2000). Quantitative electromyography of the external anal sphincter in Parkinson's disease and multiple system atrophy. Muscle Nerve, 23(8), 1250–6
Lindsay, R. M., Wiegand, S. J., Altar, C. A. & DiStefano, P. S. (1994). Neurotrophic factors: from molecule to man. Trends Neurosci., 17(5), 182–90
Litvan, I., Agid, Y., Calne, D.et al. (1996). Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele–Richardson–Olszewski syndrome): report of the NINDS-SPSP international workshop. Neurology, 47(1), 1–9
Litvan, I., Goetz, C. G., Jankovic, J.et al. (1997). What is the accuracy of the clinical diagnosis of multiple system atrophy? A clinicopathologic study. Arch. Neurol., 54(8), 937–44
Litvan, I., Booth, V., Wenning, G. K.et al. (1998). Retrospective application of a set of clinical diagnostic criteria for the diagnosis of multiple system atrophy. J. Neural Transm., 105(2–3), 217–27
Low, P. A., Gilden, J. L., Freeman, R., Sheng, K. N. & McElligott, M. A. (1997). Efficacy of midodrine vs placebo in neurogenic orthostatic hypotension. A randomized, double-blind multicenter study. Midodrine Study Group. J. Ann. Med. Assoc., 277, 1046–51
Magalhães, M., Wenning, G. K., Daniel, S. E. & Quinn, N. P. (1995). Autonomic dysfunction in pathologically confirmed multiple system atrophy and idiopathic Parkinson's disease – a retrospective comparison. Acta Neurol. Scand., 91, 98–102
Mallipeddi, R. & Mathias, C. J. (1988). Raynaud's phenomenon after sympathetic denervation in patients with primary autonomic failure: questionnaire survey. Br. Med. J., 316(7129), 438–9
Martignoni, E., Blandini, F., Petraglia, F., Pacchetti, C., Bono, G. & Nappi, G. (1992). Cerebrospinal fluid norepinephrine, 3-methoxy-4-hydroxyphenylglycol and neuropeptide Y levels in Parkinson's disease, multiple system atrophy and dementia of the Alzheimer type. J. Neural Transm. Park Dis., Dement. Sect., 4(3), 191–205
Martinelli, P. & Coccagna, G. (1978). Etude electromyographique du sphincter strie de l'anus dans trois cas de syndrome de Shy-Drager. In Electromyographie, ed. L. Arbus & J. Cadilhac. Toulouse: Premières Journées Languedociènnes d'Electromyographie, 321–6
Mathias, C. J. & Kimber, J. R. (1999). Postural hypotension: causes, clinical features, investigation, and management. Annu. Rev. Med., 50, 317–36
Mathias, C. J., Fosbraey, P., da Costa, D. F., Thornley, A. & Bannister, R. (1986). The effect of desmopressin on nocturnal polyuria, overnight weight loss, and morning postural hypotension in patients with autonomic failure. Br. Med. J. (Clin. Res. Ed.), 293, 353–4
Mathias, C. J., Senard, J. M., Braune, S.et al. (2001). L-threo-dihydroxyphenylserine (L-threo-DOPS; droxidopa) in the management of neurogenic orthostatic hypotension: a multi-national, multi-center, dose-ranging study in multiple system atrophy and pure autonomic failure. Clin. Auton. Res., 11(4), 235–42
McGuire, S. O., Ling, Z. D., Lipton, J. W., Sortwell, C. E., Collier, T. J. & Carvey, P. M. (2001). Tumor necrosis factor alpha is toxic to embryonic mesencephalic dopamine neurons. Exp. Neurol., 169(2), 219–30
Meco, G., Gasparini, M. & Doricchi, F. (1996). Attentional functions in multiple system atrophy and Parkinson's disease. J. Neurol. Neurosurg. Psychiatr., 60, 393–8
Merlo, I. M., Occhini, A., Pacchetti, C. & Alfonsi, E. (2002). Not paralysis, but dystonia causes stridor in multiple system atrophy. Neurology, 58(4), 649–52
Mizutani, T., Satoh, J. & Morimatsu, Y. (1988). Neuropathological background of oculomotor disturbances in olivopontocerebellar atrophy with special reference to slow saccade. Clin. Neuropathol., 7(2), 53–61
Mogi, M., Harada, M., Riederer, P., Narabayashi, H., Fujita, K. & Nagatsu, T. (1994). Tumor necrosis factor alpha (TNF-alpha) increases both in the brain and in the cerebrospinal fluid from parkinsonian patients. Neurosci. Lett., 165(1–2), 208–10
Montagna, P., Martinelli, P., Rizzuto, N., Salviati, A., Rasi, F. & Lugaresi, E. (1983). Amyotrophy in Shy–Drager syndrome. Acta Neurol. Belg., 83, 142–57
Morris, H. R., Vaughan, J. R., Datta, S. R.et al. (2000). Multiple system atrophy/progressive supranuclear palsy: alpha-synuclein, synphilin, tau, and APOE. Neurology, 55(12), 1918–20
Moseley, M. L., Benzow, K. A., Schut, L. J.et al. (1998). Incidence of dominant spinocerebellar and Friedreich triplet repeats among 361 ataxia families. Neurology, 51(6), 1666–71
Müller, J., Wenning, G. K., Jellinger, K., McKee, A., Poewe, W. & Litvan, I. (2000). Progression of Hoehn and Yahr stages in parkinsonian disorders: a clinicopathologic study. Neurology, 55(6), 888–91
Müller, J., Wenning, G. K., Verny, M.et al. (2001). Progression of dysarthria and dysphagia in postmortem-confirmed parkinsonian disorders. Arch. Neurol., 58(2), 259–64
Murer, M. G., Yan, Q. & Raisman-Vozari, R. (2000). Brain-derived neurotrophic factor in the control human brain, and in Alzheimer's disease and Parkinson's disease. Prog. Neurobiol., 63(1), 71–124
Nakamura, S., Ohnishi, K., Nishimura, M.et al. (1996). Large neurons in the tuberomammillary nucleus in patients with Parkinson's disease and multiple system atrophy. Neurology, 46(6), 1693–6
Nakamura, T., Yamashita, H., Takahashi, T. & Nakamura, S. (2001). Activated Fyn phosphorylates alpha-synuclein at tyrosine residue 125. Biochem. Biophys. Res. Commun., 280(4), 1085–92
Nee, L. E., Gomez, M. R., Dambrosia, J., Bale, S., Eldridge, R. & Polinsky, R. J. (1991). Environmental – occupational risk factors and familial associations in multiple system atrophy: a preliminary investigation. Clin. Auton. Res., 1(1), 9–13
Negro, A., Brunati, A. M., Donella-Deana, A., Massimino, M. L. & Pinna, L. A. (2002). Multiple phosphorylation of alpha-synuclein by protein tyrosine kinase Syk prevents cosin-induced aggregation. FASEB J., 16(2), 210–12
Neumann, M. A. (1977). Pontocerebellar atrophy combined with vestibular-reticular degeneration. J. Neuropathol. Exp. Neurol., 36(2), 321–37
Nicholl, D. J., Bennett, P., Hiller, L.et al. (1999). A study of five candidate genes in Parkinson's disease and related neurodegenerative disorders. European Study Group on Atypical Parkinsonism. Neurology, 53(7), 1415–21
Nutt, J. G., Mrox, E. A., Leeman, S. E., Williams, A. C., Engel, W. K. & Chase, T. N. (1980). Substance P in human cerebrospinal fluid: reductions in peripheral neuropathy and autonomic dysfunction. Neurology, 30(12), 1280–5
O'Brien, C., Sung, J. H., McGeachie, R. E. & Lee, M. C. (1990). Striatonigral degeneration: clinical, MRI, and pathologic correlation. Neurology, 40(4), 710–11
Oertel, W. H., Wachter, T., Quinn, N. P., Ulm, G. & Brandstadter, D. (2003). Reduced genital sensitivity in female patients with multiple system atrophy of parkinsonian type. Mov. Disord., 18(4), 430–2
Olanow, C. W. (1992). Magnetic resonance imaging in parkinsonism. Neurol. Clin., 10, 405–420
Olanow, C. W. & Tatton, W. G. (1999). Etiology and pathogenesis of Parkinson's disease. Annu. Rev. Neurosci., 22, 123–44
Oppenheimer, D. R. (1980). Lateral horn cells in progressive autonomic failure. J. Neurol. Sci., 46(3), 393–404
Orimo, S., Ozawa, E., Nakade, S., Sugimoto, T. & Mizusawa, H. (1999). (123)I-metaiodobenzylguanidine myocardial scintigraphy in Parkinson's disease. J. Neurol. Neurosurg. Psychiatr., 67(2), 189–94
Orozco, G., Estrada, R., Perry, T. L.et al. (1989). Dominantly inherited olivopontocerebellar atrophy from eastern Cuba. Clinical, neuropathological, and biochemical findings. J. Neurol. Sci., 93(1), 37–50
Osaki, Y., Wenning, G. K., Daniel, S. E.et al. (2002). Do published criteria improve clinical diagnostic accuracy in multiple system atrophy?Neurology, 59(10), 1486–91
Ostrerova, N., Petrucelli, L., Farrer, M.et al. (1999). alpha-synuclein shares physical and functional homology with 14-3-3 proteins. J. Neurosci., 19(14), 5782–91
Ozaki, M., Sasner, M., Yano, R., Lu, H. S. & Buonanno, A. (1997). Neuregulin-beta induces expression of an NMDA-receptor subunit. Nature, 390(6661), 691–4
Ozawa, T., Takano, H., Onodera, O.et al. (1999). No mutation in the entire coding region of the alpha-synuclein gene in pathologically confirmed cases of multiple system atrophy. Neurosci. Lett., 270(2), 110–12
Ozawa, T., Okuizumi, K., Ikeuchi, T., Wakabayashi, K., Takahashi, H. & Tsuji, S. (2001). Analysis of the expression level of alpha-synuclein mRNA using post-mortem brain samples from pathologically confirmed cases of multiple system atrophy. Acta Neuropathol.(Berl.), 102(2), 188–90
Palace, J., Chandiramani, V. A. & Fowler, C. J. (1997). Value of sphincter electromyography in the diagnosis of multiple system atrophy. Muscle & Nerve, 20(11), 1396–403
Papp, M. I. & Lantos, P. L. (1992). Accumulation of tubular structures in oligodendroglial and neuronal cells as the basic alteration in multiple system atrophy. J. Neurol. Sci., 107(2), 172–82
Papp, M. I. & Lantos, P. L. (1994). The distribution of oligodendroglial inclusions in multiple system atrophy and its relevance to clinical symptomatology. Brain, 117(2), 235–43
Papp, M. I., Kahn, J. E. & Lantos, P. L. (1989). Glial cytoplasmic inclusions in the CNS of patients with multiple system atrophy (striatonigral degeneration, olivopontocerebellar atrophy and Shy–Drager syndrome). J. Neurol. Sci., 94(1–3), 79–100
Parati, E. A., Fetoni, V., Geminiani, G. C.et al. (1993). Response to L-Dopa in multiple system atrophy. Clin. Neuropharmacol., 16(2), 139–44
Pastakia, B., Polinsky, R., Di Chiro, G.et al. (1989). Multiple system atrophy (Shy–Drager syndrome): MR imaging. Radiology, 159, 499–502
Pellecchia, M. T., Salvatore, E., Pivonello, R.et al. (2001). Stimulation of growth hormone release in multiple system atrophy, Parkinson's disease and idiopathic cerebellar ataxia. Neurol. Sci., 22(1), 79–80
Perani, D., Bressi, S., Testa, D.et al. (1995). Clinical/metabolic correlations in multiple system atrophy. A fludeoxyglucose F18 Positron Emission Tomography Study. Arch. Neurol., 52, 179–85
Perera, R., Isola, L. & Kaufmann, H. (1995). Effect of recombinant erythropoietin on anaemia and orthostatic hypotension in primary autonomic failure. Clin. Auton. Res., 5(4), 211–13
Perez, D. (2001). Overstimulation of the alpha1B-adrenergic receptor causes a “seizure plus” syndrome. Nat. Med., 7(2), 132–3
Pillon, B., Gouider-Khouja, N., Deweer, B.et al. (1995). Neuropsychological pattern of striatonigral degeneration: comparison with Parkinson's disease and progressive supranuclear palsy. J. Neurol. Neurosurg. Psychiatr., 58, 174–9
Pirker, W., Asenbaum, S., Bencsits, G.et al. (2000). (123I)beta-CIT SPECT in multiple system atrophy, progressive supranuclear palsy, and corticobasal degeneration. Mov. Disord., 15(6), 1158–67
Pirker, W., Djamshidian, S., Asenbaum, S.et al. (2002). Progression of dopaminergic degeneration in Parkinson's disease and atypical parkinsonism: a longitudinal beta-CIT SPECT study. Mov. Disord., 17(1), 45–53
Plaitakis, A., Flessas, P., Natsiou, A. B. & Shashidharan, P. (1993). Glutamate dehydrogenase deficiency in cerebellar degenerations: clinical, biochemical and molecular genetic aspects. Can. J. Neurol. Sci., 20(Suppl 3), S109–16
Plazzi, G., Corsini, R. & Provini, F. (1997). REM sleep behavior disorders in multiple system atrophy. Neurology, 48, 1094–7
Polinsky, R. J., Brown, R. T., Lee, G. K.et al. (1987). Beta-endorphin, ACTH, and catecholamine responses in chronic autonomic failure. Ann. Neurol., 21(6), 573–7
Polinsky, R. J., Kopin, I. J., Ebert, M. H. & Weise, V. (1981). Pharmacologic distinction of different orthostatic hypotension syndromes. Neurology, 31(1), 1–7
Polinsky, R. J., Taylor, I. L., Chew, P., Weise, V. & Kopin, L. J. (1982). Pancreatic polypeptide responses to hypoglycaemia in chronic autonomic failure. J. Clin. Endocrinol. Metab., 54(1), 48–52
Polinsky, R. J., McRae, A., Baser, S. M. & Dahlstrom, A. (1991). Antibody in the CSF of patients with multiple system atrophy reacts specifically with rat locus ceruleus. Neurol. Sci., 106(1), 96–104
Polymeropoulos, M. H., Lavedan, C., Leroy, E.et al. (1997). Mutation in the alpha-synuclein gene identified in families with Parkinson's disease. Science, 276(5321), 2045–7
Powell, S. K., Williams, C. C., Nomizu, M., Yamada, Y. & Kleinman, H. K. (1998). Laminin-like proteins are differentially regulated during cerebellar development and stimulate granule cell neurite outgrowth in vitro. J. Neurosci. Res., 54(2), 233–47
Pramstaller, P., Wenning, G. K., Smith, S. J. M., Beck, R. O., Quinn, N. P. & Fowler, C. J. (1995). Nerve conduction studies, skeletal muscle EMG, and sphincter EMG in multiple system atrophy. J. Neurol. Neurosurg. Psychiatr., 58, 618–21
Price, R. H., Albin, R. L., Sakurai, S. Y., Polinsky, R. J., Penney, J. B. & Young, A. B. (1993). Cerebellar excitatory and inhibitory amino acid receptors in multiple system atrophy. Neurology, 43(7), 1323–8
Quik, M., Spokes, E. G., Mackay, A. V. & Bannister, R. (1979). Alterations in [3H]spiperone binding in human caudate nucleus, substantia nigra and frontal cortex in the Shy–Drager syndrome and Parkinson's disease. J. Neurol. Sci., 43(3), 429–37
Quinn, N. (1989a). Disproportionate antecollis in multiple system atrophy. Lancet, 1(8642), 844
Quinn, N. (1989b). Multiple system atrophy – the nature of the beast. J. Neurol. Neurosurg. Psychiatr., 52(Suppl.), 78–89
Quinn, N. (1994). Multiple system atrophy. In Movement Disorders 3, ed. C. D. Marsden & S. Fahn. London: Butterworth-Heinemann, pp. 262–81
Quinn, N. P., Wenning, G. & Marsden, C. D. (1995). The Shy–Drager syndrome. What did Shy and Drager really describe?Arch. Neurol., 52(7), 656–7
Raabe, T. D., Suy, S., Welcher, A. & DeVries, G. H. (1997). Effect of neu differentiation factor isoforms on neonatal oligodendrocyte function. J. Neurosci. Res., 50(5), 755–68
Raimbach, S. J., Cortelli, P., Kooner, J. S., Bannister, R., Bloom, S. R. & Mathias, C. J. (1989). Prevention of glucose-induced hypotension by the somatostatin analogue octreotide (SMS 201–995) in chronic autonomic failure: haemodynamic and hormonal changes. Clin. Sci. (Lond.), 77, 623–8
Rajput, A. H., Rozdilsky, B., Rajput, A. & Ang, L. (1990). Levodopa efficacy and pathological basis of Parkinson syndrome. Clin. Neuropharmacol., 13(6), 553–8
Ranum, L. P., Lundgren, J. K., Schut, L. J.et al. (1995). Spinocerebellar ataxia type 1 and Machado–Joseph-disease: incidence of CAG expansions among adult-onset ataxia patients from 311 families with dominant, recessive, or sporadic ataxia. Am. J. Hum. Genet., 57(3), 603–8
Rascol, O., Sabatini, U., Simonetta-Moreau, M., Montastruc, J. L., Rascol, A. & Clanet, M. (1991). Square wave jerks in Parkinsonian syndromes. J. Neurol. Neurosurg. Psychiatr., 54, 599–602
Rascol, O., Sabatini, U., Fabre, N.et al. (1995). Abnormal vestibuloocular reflex cancellation in multiple system atrophy and progressive supranuclear palsy but not in Parkinson's disease. Mov. Disord., 10(2), 163–70
Riley, D. E. & Chelimsky, T. C. (2003). Autonomic nervous system testing may not distinguish multiple system atrophy from Parkinson's disease. J. Neurol. Neurosurg. Psychiatr., 74(1), 56–60
Rinne, J. O., Burn, D. J., Mathias, C. J., Quinn, N. P., Marsden, C. D. & Brooks, D. J. (1995). Positron emission tomography studies on the dopaminergic system and striatal opioid binding in the olivopontocerebellar atrophy variant of multiple system atrophy. Ann. Neurol., 37, 568–73
Rivest, J., Quinn, N. & Marsden, C. D. (1990). Dystonia in Parkinson's disease, multiple system atrophy, and progressive supranuclear palsy. Neurology, 40, 1571–8
Robbins, T. W., James, M., Lange, K. W., Owen, A. M., Quinn, N. P. & Marsden, C. D. (1992). Cognitive performance in multiple system atrophy. Brain, 115(1), 271–91
Sakakibara, R., Hattori, T., Uchiyama, T.et al. (2000a). Urinary dysfunction and orthostatic hypotension in multiple system atrophy: which is the more common and earlier manifestation?J. Neurol. Neurosurg. Psychiatr., 68(1), 65–9
Sakakibara, R., Hattori, T., Uchiyama, T.et al. (2000b). Are alpha-blockers involved in lower urinary tract dysfunction in multiple system atrophy? A comparison of prazosin and moxisylyte. J. Auton. Nerv. Syst., 79, 191–5
Salazar, G., Valls-Sole, J., Marti, M. J., Chang, H. & Tolosa, E. S. (2000). Postural and action myoclonus in patients with parkinsonian type of multiple system atrophy. Mov. Disord., 15(1), 77–83
Sandroni, P., Ahlskog, J. E., Fealy, R. D. & Low, P. A. (1991). Autonomic involvement in extrapyramidal and cerebellar disorders. Clin. Auton. Res., 1, 147–55
Santens, P., Crevits, L. & Linden, C. (1996). Raynaud's phenomenon in a case of multiple system atrophy. Mov. Disord., 11, 586–8
Satoh, J. I. & Kuroda, Y. (2001). Alpha-synuclein expression is up-regulated in NTera2 cells during neuronal differentiation but unaffected by exposure to cytokines and neurotrophic features. Parkinsonism Relat. Disord., 8(1), 7–17
Savoiardo, M., Strada, L. & Girotti, F. (1990). Olivopontocerebellar atrophy: MR diagnosis and relationship to multisystem atrophy. Radiology, 174, 693–6
Schelhaas, H. J., Hageman, G. & Post, J. G. (1997). Cerebellar ataxia, dementia, pyramidal signs, cortical cataract of the posterior pole and a raised IgG index in a patient with a sporadic form of olivopontocerebellar atrophy. Clin. Neurol. Neurosurg., 99(2), 99–101
Schelosky, L., Hierholzer, J., Wissel, J., Cordes, M. & Poewe, W. (1993). Correlation of clinical response in apomorphine test with D2-receptor status as demonstrated by 123I IBZM-SPECT. Mov. Disord., 8(4), 453–8
Scherer, H. J. (1933a). Beiträge zur pathologischen Anatomie des Kleinhirns. III. Mitteilung: Genuine Kleinhirnatrophien. Z. Gesamte. Neurol. Psychiatri., 145, 335–405
Scherer, H. J. (1933b). Extrapyramidale Störungen bei der olivopontocerebellären Atrophie. Z. Gesamte. Neurol. Psychiatri., 145, 406–19
Scherfler, C., Puschban, Z., Ghorayeb, I.et al. (2000). Complex motor disturbances in a sequential double lesion rat model of striatonigral degeneration (multiple system atrophy). Neuroscience, 99(1), 43–54
Schocke, M., Seppi, K., Esterhammer, R.et al. (2002). Diffusion-weighted MRI differentiates the Parkinson variant of multiple system atrophy from PD. Neurology, 58(4), 575–80
Schöls, L., Szymanski, S., Peters, S.et al. (2000). Genetic background of apparently idiopathic sporadic cerebellar ataxia. Hum. Genet., 107(2), 132–7
Schrag, A., Kingsley, D., Phatouros, C.et al. (1998). Clinical usefulness of magnetic resonance imaging in multiple system atrophy. J. Neurol. Neurosurg. Psychiatr., 65(1), 65–71
Schrag, A., Ben-Shlomo, Y. & Quinn, N. P. (1999). Prevalence of progressive supranuclear palsy and multiple system atrophy: a cross-sectional study. Lancet, 354, 1771–5
Schrag, A., Good, C. D., Miszkiel, K.et al. (2000). Differentiation of atypical parkinsonian syndromes with routine MRI. Neurology, 54(3), 697–702
Schulz, J. B., Klockgether, T., Petersen, D.et al. (1994). Multiple system atrophy: natural history, MRI morphology, and dopamine receptor imaging with 123IBZM-SPECT. J. Neurol. Neurosurg. Psychiatr., 57(9), 1047–56
Schulz, J. B., Skalej, M., Wedekind, D.et al. (1999). Magnetic resonance imaging-based volumetry differentiates idiopathic Parkinson's syndrome from multiple system atrophy and progressive supranuclear palsy. Ann. Neurol., 45, 65–74
Schwarz, J., Tatsch, K., Arnold, G.et al. (1992). 123I-iodobenzamide-SPECT predicts dopaminergic responsiveness in patients with de novo parkinsonism. Neurology, 42, 556–61
Schwarz, J., Tatsch, K., Arnold, G.et al. (1993). 123I-iodobenzamide-SPECT in 83 patients with de novo parkinsonism. Neurology, 43, 17–20
Schwarz, J., Weis, S. & Kraft, E. (1996). Signal changes on MRI and increases in reactive microgliosis, astrogliosis, and iron in the putamen of two patients with multiple system atrophy. J. Neurol. Neurosurg. Psychiatr., 60, 98–101
Schwarz, J., Tatsch, K., Gasser, T.et al. (1998). 123I-IBZM binding compared with long-term clinical follow up in patients with de novo parkinsonism. Mov. Disord., 13(1), 16–19
Seppi, K., Puschban, Z., Stefanova, N. et al. (2001). Overstimulation of the alpha1B-adrenergic receptor causes a “seizure plus” syndrome. Nat. Med., 7(2), 132
Seppi, K., Schocke, M. F., Esterhammer, R.et al. (2003). Diffusion-weighted imaging discriminates progressive supranuclear palsy from PD, but not from the parkinson variant of multiple system atrophy. Neurology, 60(6), 922–7
Shinotoh, H., Aotsuka, A., Inoue, O.et al. (1991). Imaging of dopamine D1 and D2 receptors by a high resolution positron emission tomography. Adv. Exp. Med. Biol., 287, 294–53
Shinotoh, H., Inoue, O., Hirayama, K.et al. (1993). Dopamine D1 receptors in Parkinson's disease and striatonigral degeneration: a positron emission tomography study. J. Neurol. Neurosurg. Psychiatr., 56(5), 467–72
Shy, G. M. & Drager, G. A. (1960). A neurological syndrome associated with orthostatic hypotension. A clinicopathological study. Arch. Neurol., 2, 511–27
Silber, M. H. & Levine, S. (2000). Stridor and death in multiple system atrophy. Mov. Disord., 15(4), 699–704
Silveira, I., Lopes-Cendes, I., Kish, S.et al. (1996). Frequency of spinocerebellar ataxia type 1, dentatorubropallidoluysian atrophy, and Machado–Joseph disease mutations in a large group of spinocerebellar ataxia patients. Neurology, 46(1), 214–18
Sima, A. A. F., Caplan, M., D'Amato, C. J., Pevzner, M. & Furlong, J. W. (1993). Fulminant multiple system atrophy in a young adult presenting as motor neuron disease. Neurology, 43, 2031–5
Spillantini, M. G., Schmidt, M. L., Lee, V. M., Trojanowski, J. Q., Jakes, R. & Goedert, M. (1979). Alpha-synuclein in Lewy bodies. Nature, 388(6645), 839–40
Spillantini, M. G., Crowther, R. A., Jakes, R., Cairns, N. J., Lantos, P. L. & Goedert, M. (1998). Filamentous alpha-synuclein inclusions link multiple system atrophy with Parkinson's disease and dementia with Lewy bodies. Neurosci. Lett., 251(3), 205–8
Spokes, E. G., Bannister, R. & Oppenheimer, D. R. (1979). Multiple system atrophy with autonomic failure: clinical, histological and neurochemical observations on four cases. J. Neurol. Sci., 43(1), 59–82
Stauffenberg, (1918). Zur Kenntnis des extrapyramidalen motorischen Systems und Mitteilung eines Falles von sog. “Atrophie olivo-pontocérébelleuse”. Z. Gesamte. Neurol. Psychiatri., 39, 1–55
Stefanis, L., Kholodilov, N., Ridcout, H. J., Burke, R. E. & Greene, L. A. (2001). Synuclein-1 is selectively up-regulated in response to nerve growth factor treatment in PC12 cells. J. Neurochem., 76(4), 1165–76
Stell, R. & Bronstein, A. M. (1994). Eye abnormalities in extrapyramidal diseases. In Movement Disorders 3, ed. C. D. Marsden & S. Fahn. London: Butterworth-Heinemann, pp. 88–113
Stern, M. B., Braffman, B. H., Skolnick, B. E., Hurtig, H. I. & Grossman, R. I. (1989). Magnetic resonance imaging in Parkinson's disease and parkinsonian syndromes. Neurology, 39, 1524–6
Stocchi, F., Badiali, D., Vacca, L.et al. (2000). Anorectal function in multiple system atrophy and Parkinson's disease. Mov. Disord., 15(1), 71–6
Strijks, E., van't Hof, M., Sweep, F., Lenders, J. W., Oyen, W. J. & Horstink, M. W. (2002). Stimulation of growth-hormone release with clonidine does not distinguish individual cases of idiopathic Parkinson's disease from those with striatonigral degeneration. J. Neurol., 249(9), 1206–10
Suemaru, S., Suemaru, K., Kawai, K.et al. (1995). Cerebrospinal fluid corticotrophin-releasing hormone in neurodegenerative diseases: reduction in spinocerebellar degeneration. Life Sci., 57(24), 2231–5
Sung, J. H., Mastri, A. R. & Segal, E. (1979). Pathology of Shy–Drager syndrome. J. Neuropathol. Exp. Neurol., 38(4), 353–68
Takahashi, M., Ohta, S., Matsuoka, S., Mori, H. & Mizuno, Y. (2002). Mixed multiple system atrophy and progressive supranuclear palsy: a clinical and pathological report of one case. Acta Neuropathol. (Berl.), 103(1), 82–7
Takatsu, H., Nagashima, K., Murase, M., Fujiwara, H., Nishida, H. & Matsuo, H. (2000). Differentiating Parkinson disease from multiple-system atrophy by measuring cardiac iodine-123 metaiodobenzymguanidine accumulation. J. Am. Med. Assoc., 284(1), 44–5
Taki, J., Nakajima, K., Hwang, E. H., Matsunari, I., Komai, K. & Yoshita, M. (2000). Peripheral sympathetic dysfunction in patients with Parkinson's disease without autonomic failure is heart selective and disease specific. Eur. J. Nucl. Med., 27, 566–73
Testa, D., Filippini, G., Farinotti, M., Palazzini, E. & Caraceni, T. (1996). Survival in multiple system atrophy: a study of prognostic factors in 59 cases. J. Neurol., 243, 401–4
Thobois, S., Broussolle, E., Toureille, L. & Vial, C. (2001). Severe dysphagia after botulinum toxin injection for cervical dystonia in multiple system atrophy. Mov. Disord., 16, 764–5
Tison, F., Wenning, G. K., Daniel, S. E. & Quinn, N. (1995a). The pathophysiology of parkinsonism in multiple system atrophy. Eur. J. Neurol., 2, 435–44
Tison, F., Wenning, G. K., Quinn, N. P. & Smith, S. J. (1995b). REM sleep behaviour disorder as the presenting symptom of multiple system atrophy. J. Neurol. Neurosurg. Psychiatr., 58(3), 379–80
Tison, F., Arne, P., Sourgen, C., Chrysostome, V. & Yeklef, F. (2000a). The value of external anal sphincter electromyography for the diagnosis of multiple system atrophy. Mov. Disord., 15(6), 1148–57
Tison, F., Yekhlef, F., Chrysostome, V. & Sourgen, C. (2000b). Prevalence of multiple system atrophy. Lancet, 355(9202), 495–6
Togo, T., Iseki, E., Marui, W., Akiyama, H., Ueda, K. & Kosaka, K. (2001). Glial involvement in the degeneration process of Lewy body-bearing neurons and the degradation process of Lewy bodies in brains of dementia with Lewy bodies. J. Neurol. Sci., 184(1), 71–5
Tranchant, C., Guiraud-Chaumeil, C., Echaniz-Laguna, A. & Warter, J. M. (2000). Is clonidine growth hormone stimulation a good test to differentiate multiple system atrophy from idiopathic Parkinson's disease?J. Neurol., 247(11), 853–6
Trenkwalder, C., Schwarz, J., Gebhard, J.et al. (1995). Starnberg trial on epidemiology of parkinsonism and hypertension in the elderly. Prevalence of Parkinson's disease and related disorders assessed by a door-to-door survey of inhabitants older than 65 years. Arch. Neurol., 52, 1017–22
Trojanowski, J. Q. (2002). Tauists, baptists, syners, apostates, and new data. Ann. Neurol., 52(3), 263–5
Tsuchiya, K., Ozawa, E., Haga, C.et al. (2000). Constant involvement of the Betz cells and pyramidal tract in multiple system atrophy: a clinicopathological study of seven autopsy cases. Acta Neuropathol. (Berl.), 99(6), 628–36
Tsuda, T., Onodera, H., Okabe, S., Kikuchi, Y. & Itoyama, Y. (2002). Impaired chemosensitivity to hypoxia is a marker of multiple system atrophy. Ann. Neurol., 52(3), 367–71
Tu, P. H., Galvin, J. E., Baba, M.et al. (1998). Glial cytoplasmatic inclusions in white matter oligodendrocytes of multiple system atrophy brains contain insoluble alpha-synuclein. Ann. Neurol., 44(3), 415–22
Urenjak, J., Williams, S. R., Gadian, D. G. & Noble, M. (1993). Proton nuclear magnetic resonance spectroscopy unambiguously identifies different neuronal cell types. J. Neurosci., 13, 981–9
Uversky, V. N. & Fink, A. L. (2002). Amino acid determinants of alpha-synuclein aggregation: putting together pieces of the puzzle. FEBS Lett., 522(1–3), 9–13
Uversky, V. N., Li, J., Souillac, P.et al. (2002). Biophysical properties of the synucleins and their propensities to fibrillate: inhibition of alpha-synuclein assembly by beta- and gamma-synucleins. J. Biol. Chem., 277(14), 11970–8
Valldeoriola, F., Valls-Sole, J., Tolosa, E. S. & Marti, M. J. (1995). Striated anal sphincter denervation in patients with progressive supranuclear palsy. Mov. Disord., 10(5), 550–5
Vanacore, N., Bonifati, V., Fabbrini, G.et al. (2000). Smoking habits in multiple system atrophy and progressive supranuclear palsy. Neurology, 54, 114–19
Vanacore, N., Bonifati, V., Fabbrini, G. et al. (ESGAP) (2004). Case-control study of multiple system atrophy. Mov. Disord. (in press)
Eecken, H., Adams, R. D. & Bogaert, L. (1960). Striopallidal-nigral degeneration. A hitherto undescribed lesion in paralysis agitans. J. Neuropathol. Exp. Neurol., 19, 159–61
Royen, E., Verhoeff, N. F., Speelman, J. D., Wolters, E. C., Kuiper, M. A. & Janssen, A. G. (1993). Multiple system atrophy and progressive supranuclear palsy. Diminished striatal D2 dopamine receptor activity demonstrated by 123I-IBZM single photon emission computed tomography. Arch. Neurol., 50(5), 513–16
Varrone, A., Marek, K. L., Jennings, D., Innis, R. B. & Seibyl, J. P. (2001). (123)I-beta-CIT SPECT imaging demonstrates reduced density of striatal dopamine transporters in Parkinson's disease and multiple system atrophy. Mov. Disord., 16, 1023–32
Vartanian, T., Corfas, G., Li, Y., Fischbach, G. D. & Stefansson, K. (1994). A role for the acetylcholine receptor-inducing protein ARIA in oligodendrocyte development. Proc. Natl Acad. Sci., USA, 91(24), 11626–30
Vartanian, T., Goodearl, A., Vichover, A. & Fischbach, G. (1997). Axonal neuregulin signals cells of the oligodendrocyte lineage through activation of HER4 and Schwann cells through HER2 and HER3. J. Cell. Biol., 137(1), 211–20
Vidailhet, M., Rivaud, S., Gouider-Khouja, N.et al. (1994). Eye movements in parkinsonian syndromes. Ann. Neurol., 35, 420–6
Vila, M., Jackson-Lewis, V., Guegan, C.et al. (2001). The role of glial cells in Parkinson's disease. Curr. Opin. Neurol., 14(4), 483–9
Visser-Vandewalle, V., Temel, Y., Colle, H. & Linden, C. (2003). Bilateral high-frequency stimulation of the subthalamic nucleus in patients with multiple system atrophy – parkinsonism. Report of four cases. J. Neurosurg., 98(4), 882–7
Vodusek, B. (2001). Sphincter EMG and differential diagnosis of multiple system atrophy. Mov. Disord., 16(4), 600–7
Vogels, O. J., Veltman, J., Oyen, W. J. & Horstink, M. W. (2000). Decreased striatal dopamine D2 receptor binding in amyotrophic lateral sclerosis (ALS) and multiple system atrophy (MSA): D2 receptor down-regulation versus striatal cell degeneration. J. Neurol. Sci., 180(1–2), 62–5
Wakabayashi, K., Hayashi, S., Kakita, A.et al. (1998a). Accumulation of alpha-synuclein/NACP is a cytopathological feature common to Lewy body disease and multiple system atrophy. Acta Neuropathol. (Berl.), 96(5), 445–52
Wakabayashi, K., Ikeuchi, T., Ishikawa, A. & Takahashi, H. (1998b). Multiple system atrophy with severe involvement of the motor cortical areas and cerebral white matter. J. Neurol. Sci., 156(1), 114–17
Wakabayashi, K., Yoshimoto, M., Tsuji, S. & Takahashi, H. (1998c). Alpha-synuclein immunoreactivity in glial cytoplasmic inclusions in multiple system atrophy. Neurosci. Lett., 249(2–3), 180–2
Wakabayashi, K., Engelender, S., Yoshimoto, M., Tsuji, S., Ross, C. A. & Takahashi, H. (2000). Synphilin-1 is present in Lewy bodies in Parkinson's disease. Ann. Neurol., 47(4), 521–3
Wakabayashi, K., Engelender, S., Tanaka, Y.et al. (2002). Immunocytochemical localization of synphilin-1, an alpha-synuclein-associated protein, in neurodegenerative disorders. Acta Neuropathol. (Berl.), 103(3), 209–14
Wakai, M., Kume, A., Takahashi, A., Ando, T. & Hashizume, Y. (1994). A study of parkinsonism in multiple system atrophy: clinical and MRI correlation. Acta Neurol. Scand., 90, 225–31
Waldner, R., Puschban, Z., Scherfler, C., Seppi, K., Jellinger, K. & Poewe, W. (2001). No functional effects of embryonic neuronal grafts on motor deficits in a 3-nitropropionic acid rat model of advanced striatonigral degeneration (multiple system atrophy). Neuroscience, 102(3), 581–92
Watanabe, H., Saito, Y., Terao, S.et al. (2002). Progression and prognosis in multiple system atrophy: an analysis of 230 Japanese patients. Brain, 125, 1070–83
Wenning, G. K., Wagner, S., Daniel, S. E. & Quinn, N. P. (1993). Multiple system atrophy: sporadic or familial?Lancet, 342(8872), 681
Wenning, G. K., Ben Shlomo, Y., Magalhaes, M., Daniel, S. E. & Quinn, N. P. (1994a). Clinical features and natural history of multiple system atrophy. An analysis of 100 cases. Brain, 117(4), 835–45
Wenning, G. K., Quinn, N., Magalhaes, M., Mathias, C. & Daniel, S. E. (1994b). “Minimal change” multiple system atrophy. Mov. Disord., 9(2), 161–6
Wenning, G. K. & Geser, F. (2003). Atrophie multisystematisee. Rev. Neurol. (Paris., 159(5 Pt 2), 31–8
Wenning, G. & Quinn, N. (1994). Are Lewy bodies non-specific epiphenomena of nigral damage?Mov. Disord., 9(3), 378–9
Wenning, G. K. & Quinn, N. P. (1997a). Multiple system atrophy. In Bailliere's Clin. Neurol., ed. N. P. Quinn, 6, 187–204
Wenning, G. K. & Smith, S. J. M. (1997b). Magnetic brain stimulation in multiple system atrophy. Mov. Disord., 12(3), 452–3
Wenning, G. K., Ben-Shlomo, Y., Magalhaes, M., Daniel, S. E. & Quinn, N. P. (1995). Clinicopathological study of 35 cases of multiple system atrophy. J. Neurol. Neurosurg. Psychiatr., 58, 160–6
Wenning, G. K., Granata, R., Laboyrie, P. M., Quinn, N. P., Jenner, P. & Marsden, C. D. (1996a). Reversal of behavioural abnormalities by fetal allografts in a novel rat model of striatonigral degeneration. Mov. Disord., 11(5), 522–32
Wenning, G. K., Quinn, N. P., Daniel, S. E., Garratt, H. & Marsden, C. D. (1996b). Facial dystonia in pathologically proven multiple system atrophy: a video report. Mov. Disord., 11(1), 107–9
Wenning, G. K., Tison, F., Elliott, L., Quinn, N. P. & Daniel, S. E. (1996c). Olivopontocerebellar pathology in multiple system atrophy. Mov. Disord., 11(2), 157–62
Wenning, G. K., Kraft, E., Beck, R.et al. (1997a). Cerebellar presentation of multiple system atrophy. Mov. Disord., 12(1), 115–17
Wenning, G. K., Tison, F., Ben Shlomo, Y., Daniel, S. E. & Quinn, N. P. (1997b). Multiple system atrophy: a review of 203 pathologically proven cases. Mov. Disord., 12(2), 133–47
Wenning, G. K., Ebersbach, G., Verny, M.et al. (1999a). Progression of falls in postmortem-confirmed parkinsonian disorders. Mov. Disord., 14, 947–50
Wenning, G. K., Granata, R., Puschban, Z., Scherfler, C. & Poewe, W. (1999b). Neural transplantation in animal models of multiple system atrophy: a review. J. Neural Transm., 55, 103–13
Wenning, G. K., Scherfler, C., Granata, R., Bösch, S., Verny, M. & Chaudhuri, K. R. (1999c). Time course of symptomatic orthostatic hypotension and urinary incontinence in patients with postmortem confirmed parkinsonian syndromes: a clinicopathological study. J. Neurol. Neurosurg. Psychiatr., 67, 620–3
Wenning, G. K., Ben-Shlomo, Y., Hughes, A., Daniel, S. E., Lees, A. J. & Quinn, N. P. (2000a). What clinical features are most useful to distinguish definite multiple system atrophy from Parkinson's disease?J. Neurol. Neurosurg. Psychiatr., 68(4), 434–40
Wenning, G. K., Jellinger, K. J., Quinn, N. P. & Poewe, W. H. (2000b). An early report of striatonigral degeneration. Mov. Disord., 15(1), 159–62
Wenning, G. K., Tison, F., Scherfler, C.et al. (2000c). Towards neurotransplantation in multiple system atrophy: clinical rationale, pathophysiological basis, and preliminary experimental evidence. Cell Transpl., 9, 279–88
Wenning, G. K., Seppi, K., Sampaio, C., Quinn, N. P., Poewe, W. & Tison, F. (2002). European Multiple System Atrophy Study Group (EMSA-SG): Validation of the Unified MSA Rating Scale (UMSARS). Mov. Disord., 17(Suppl 5), 252
Wenning, G. K., Geser, F. & Poewe, W. (2003a). The ‘risus sardonicus’ of multiple system atrophy. Mov. Disord., 18(10), 1211
Wenning, G. K., Geser, F., Stampfer-Kountchev, M. & Tison, F. (2003b). Multiple system atrophy: an update. Mov. Disord., 18 (suppl. 6), S34–42
Wermuth, L., Joensen, P., Bunger, N. & Jeune, B. (1997). High prevalence of Parkinson's disease in the Faroe Islands. Neurology, 49, 426–32
Wiedemann, P., Bonisch, H., Oerters, F. & Bruss, M. (2002). Structure of the human histamine H3 receptor gene (HRH3) and identification of naturally occurring variations. J. Neural Transm., 109(4), 443–53
Williams, A., Hanson, D. & Calne, D. B. (1979). Vocal cord paralysis in the Shy–Drager syndrome. J. Neurol. Neurosurg. Psychiatr., 42, 151–3
Winkler, A. S., Marsden, J., Parton, M., Watkins, P. J. & Chaudhuri, K. R. (2001). Erythropoietin deficiency and anaemia in multiple system atrophy. Mov. Disord., 16, 233–9
Wright, R. A., Kaufmann, H. C., Perera, R.et al. (1998). A double-blind, dose response study of midodrine in neurogenic orthostatic hypotension. Neurology, 51(1), 120–4
Wüllner, U., Klockgether, T., Petersen, D., Naegele, T. & Dichgans, J. (1993). Magnetic resonance imaging in hereditary and idiopathic ataxia. Neurology, 43, 318–25
Yamada, T. & McGeer, P. L. (1990). Oligodendroglial microtubular masses: an abnormality observed in some human neurodegenerative diseases. Neurosci. Lett., 120(2), 163–6
Yang, X., Kuo, Y., Devay, P., Yu, C. & Role, L. (1998). A cysteine-rich isoform of neuregulin controls the level of expression of neuronal nicotinic receptor channels during synaptogenesis. Neuron, 20(2), 255–70
Yekhlef, F., Ballan, G., Macia, F., Delmer, O., Sourgen, C. & Tison, F. (2003). Routine MRI for the differential diagnosis of Parkinson's disease, MSA, PSP, and CBD. J. Neural Transm., 110(2), 151–69
Yoshita, M. (1998). Differentiation of idiopathic Parkinson's disease from striatonigral degeneration and progressive supranuclear palsy using iodine-123 meta-iodobenzylguanidine myocardial scintigraphy. J. Neurol. Sci., 155, 60–7
Yuan, J. & Yankner, B. A. (2000). Apoptosis in the nervous system. Nature, 407(6805), 802–9
Zesiewicz, T. A., Helal, M. & Hauser, R. A. (2002). Sildenafil citrate (Viagra) for the treatment of erectile dysfunction in men with Parkinson's disease. Mov. Disord., 15, 305–8
Ziegler, M. G., Lake, C. R. & Kopin, I. J. (1977). The sympathetic-nervous-system defect in primary orthostatic hypotension. N. Engl. J. Med., 296(6), 293–7
Zoukos, Y., Thomaides, T., Pavitt, D. V., Cuzner, M. L. & Mathias, C. J. (1993). Beta-adrenoceptor expression on circulating mononuclear cells of idiopathic Parkinson's disease and autonomic failure patients before and after reduction of central sympathetic outflow by clonidine. Neurology, 43(6), 1181–7
Zuscik, M. J., Sands, S., Ross, S. A.et al. (2000). Overexpression of the alpha1B-adrenergic receptor causes apoptotic neurodegeneration: multiple system atrophy. Nat. Med., 6(12), 1388–94
Zuscik, M. J., Chalothorn, D., Hellard, D.et al. (2001). Hypotension, autonomic failure, and cardiac hypertrophy in transgenic mice overexpressing the alpha 1B-adrenergic receptor. J. Biol. Chem., 276(17), 13738–43