Fig Table 1 SCA3 Machado Joseph disease MJD SCA6 SCA31 Dentate rubro pallido luysian atrophy DRPLA SCA2 SCA1 Ataxin 3 Calcium channel

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1 Review Opinion for Spine Surgeons Key words spinocerebellar degeneration multiple system atrophy progressive supranuclear palsy Parkinson disease spinal cord disease Spinal Surgery Parkinson 1 spinocerebellar degeneration SCD SCD Fig. 1 2 Machado Joseph MJD SCA3 6 spinocerebellar ataxia type 6 SCA6 31 SCA31dentato rubro pallido luysian atrophy DRPLA Table 1 Friedreich 3 2 early onset ataxia with oculomotor apraxia and hypoalbuminemia EAOH AOA1Table 2 cortical cerebellar atrophy CCA multiple system atrophy MSA 3 2 MSA Fig. 1 SCD Address reprint requests to Haruo Shimazaki, M. D., Division of Neurology, Department of Internal Medicine, Jichi Medical University School of Medicine, Yakushiji , Shimotsuke shi, Tochigi , Japan VOL. 26 NO

2 Fig Table 1 SCA3 Machado Joseph disease MJD SCA6 SCA31 Dentate rubro pallido luysian atrophy DRPLA SCA2 SCA1 Ataxin 3 Calcium channel subunit CACNA1A BEAN TK2 Atrophin 1 Ataxin 2 Ataxin 1 W W Table 2 Early onset ataxia with oculomotor apraxia and hypoalbuminemia EAOH AOA1 Autosomal recessive spastic ataxia of Charevoix Saguenay ARSACS Ataxia with oculomotor apraxia type 2 AOA2 SCAR2 Ataxia with isolated vitamin E deficiency AVED Aprataxin Sacsin Senataxin Tocopherol transport protein W W W AFP CK W hereditary spastic paraplegia HSP HSP X W spastic paraplegia 4 SPG4 3 SPG11 Table 3 SCD MRI CCA Fig. 2 a b MJD SCA3 Fig. 2 c d TRH 4 HSP SCD VOL. 26 NO

3 Table 3 SPG4 SPG31 SPG3A SPG11 Spastin Receptor expression enhancing protein 1 REEP1 Atlastin 1 Spatacsin W a b c d Fig. 2 CCA MJD SCA3 MRI CCA MRI T1 a T2 b a b Machado Joseph MJD SCA3 MRI T1 c T2 d c d 2 multiple system atrophy MSA olivopontocerebellar atrophy OPCAShy Drager syndrome SDSstriatonigral degeneration SND OPCA MSA with predominant cerebellar ataxia MSA C SND MSA with predominant parkinsonism MSA P 5 N glial cytoplasmic inclusion GCI neuronal cytoplasmic inclusion NCI 6 Parkinson synucleinopathy SCD SCD MSA C MSA P 7 MSA C MSA C Parkinson MSA P Parkinson L Parkinson Parkinson MSA MRI T2 FLAIR Fig. 3 a Fig. 3 VOL. 26 NO

4 a b c Fig. 3 MSA MRI MSA MRI FLAIR a T2 bmsa MRI T1 c T2 d a c b d d b d Fig. 3 c MSA Parkinson Parkinson 8 MSA 24 SCD progressive supranuclear palsy PSP1964 Steele Richardson Olszewski PSP 10 MRI humming bird sign Fig. 4 aflair Fig. 4 b PSP PSP L 11 PSP parkinsonism PSP P LParkinson 12 PSP C 13 Parkinson ADL VOL. 26 NO

5 a b c Fig. 4 PSP MRI PSP MRI T1 aflair b T1 c PSP humming bird sign a b FLAIR b 4 Parkinson 1 SCD HSP W MRI SCD 2 Parkinson Parkinson MSA P MSA P L Parkinson MSA P MRI Parkinson 123 I MIBG Parkinson MSA Parkinson MSA P 14 PET 11 C BF 227 MSA 15 Parkinson PSP P LParkinson L MRI SCA2 16 SPG11 17 L Parkinsonism 5 Parkinson 1 MRI Tsuji S, Onodera O, Goto J, et al Sporadic ataxias in Japan a population based epidemiological study. Cerebellum , Annual Review Miyai I, Ito M, Hattori N, et al Cerebellar ataxia rehabilitation trial in degenerative cerebellar diseases. Neurorehabil Neural Repair , Gilman S, Low PA, Quinn N, et al Consensus statement on the diagnosis of multiple system atrophy. J Neurol Sci , Wakabayashi K, Yoshimoto M, Tsuji S, et al Alpha synuclein immunoreactivity in glial cytoplasmic inclusions in multiple system atrophy. Neurosci Lett , Ozawa T, Tada M, Kakita A, et al The phenotype spectrum of Japanese multiple system atrophy. J Neurol Neu- VOL. 26 NO

6 rosurg Psychiatry , Ubhi K, Rockenstein E, Mante M, et al Rifampicin reduces alpha synuclein in a transgenic mouse model of multiple system atrophy. Neuroreport , Steele JC, Richardson JC, Olszewski J Progressive supranuclear palsy. A heterogeneous degeneration involving the brain stem, basal ganglia and cerebellum with vertical gaze and pseudobulbar palsy, nuchal dystonia and dementia. Arch Neurol , Litvan I, Agid Y, Calne D, et al Clinical research criteria for the diagnosis of progressive supranuclear palsy Steele Richardson Olszewski syndromereport of the NINDS SPSP international workshop. Neurology , º Williams DR, de Silva R, Paviour DC, et al Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy Richardson s syndrome and PSP parkinsonism. Brain , Kanazawa M, Shimohata T, Toyoshima Y, et al Cerebellar involvement in progressive supranuclear palsy A clinicopathological study. Mov Disord , Kikuchi A, Baba T, Hasegawa T, et al Differentiating Parkinson s disease from multiple system atrophy by 123 I meta iodobenzylguanidine myocardial scintigraphy and olfactory test. Parkinsonism Relat Disord , Kikuchi A, Takeda A, Okamura N, et al In vivo visualization of alpha synuclein deposition by carbon 11 labelled 222 dimethylaminothiazol 5 yl ethenyl 62fluoro ethoxy benzoxazole positron emission tomography in multiple system atrophy. Brain , Payami H, Nutt J, Gancher S, et al SCA2 may present as levodopa responsive parkinsonism. Mov Disord , Guidubaldi A, Piano C, Santorelli FM, et al Novel mutations in SPG11 cause hereditary spastic paraplegia associated with early onset levodopa responsive Parkinsonism. Mov Disord , 2011 VOL. 26 NO

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