Abele M, Burk K, Andres F, et al. (1997)
Autosomal dominant cerebellar ataxia
type I. Nerve conduction and evoked
potential studies in families with SCA1,
SCA2 and SCA3. Brain 120:2141–2148
Article
PubMed
Google Scholar
Bang OY, Huh K, Lee PH, Kim HJ.
(2003) Clinical and neuroradiological
features of patients with spinocerebellar
ataxias from Korean kindreds. Arch
Neurol 60:1566–1574
Article
PubMed
Google Scholar
Boesch SM, Schocke M, Burk K, et al.
(2001) Proton magnetic resonance
spectroscopic imaging reveals differences
in spinocerebellar types 2 and 6. J
Magnet Res Imag 13:553–559
Article
CAS
Google Scholar
Brenneis C, Bosch SM, Schocke M,
et al. (2003) Atrophy pattern in SCA2
determined by voxel-based morphometry.
Neuroreport 14:1799–1802
Article
PubMed
Google Scholar
Burk K, Globas C, Bosch S, et al. (1999)
Cognitive deficits in spinocerebellar
ataxia 2. Brain 122:769–777
Article
PubMed
Google Scholar
Chen JT, Lin YY, Lee YC, et al. (2004)
Prolonged central motor conduction
time of lower limb muscles in spinocerebellar
ataxia 6. J Clin Neurosci
11:381–383
Article
CAS
PubMed
Google Scholar
Durr A, Smadja D, Cancel G, Lezin A,
Stevanin G, Mikol J, Bellance R, Buisson
GG, Chneiweiss H, Dellanave J,
et al. (1995) Autosomal dominant cerebellar
ataxia type I in Martinique
(French West Indies). Clinical and
neuropathological analysis of 53 patients
from three unrelated SCA2 families.
Brain 118:1573–81
Article
PubMed
Google Scholar
Estrada R, Galarraga J, Orozco G,
Nodarse A, Auburger G. (1999) Spinocerebellar
ataxia 2 (SCA2): morphometric
analyses in 11 autopsies. Acta
Neuropathol 97:306–10
Article
CAS
PubMed
Google Scholar
Etchebehere EC, Cendes F, Lopes-Cendes
I, et al. (2001) Brain single-photon
emission computed tomography and
magnetic resonance imaging in Machado-
Joseph disease. Arch Neurol
58:1257–1263
Article
CAS
PubMed
Google Scholar
Gilman S, Markel DS, Koeppe RA, et al.
(1988) Cerebellar and brainstem hypometabolism
in olivopontocerebellar
atrophy detected with positron emission
tomography. Ann Neurol 23:223–230
Article
CAS
PubMed
Google Scholar
Gilman S, Koeppe RA, Junck L, et al.
(1994) Patterns of cerebral glucose
metabolism detected with positron
emission tomography differ in multiple
system atrophy and olivopontocerebellar
atrophy. Ann Neurol 36:166–175
Article
CAS
PubMed
Google Scholar
Giuffrida S, Saponara R, Restivo DA,
et al. (1999) Supratentorial atrophy in
spinocerebellar ataxia type 2: MRI
study of 20 patients. J Neurol 246:383–388
Article
CAS
PubMed
Google Scholar
Globas C, Bosch S, Zuhlke CH, Daum I,
Dichgans J, Burk K. (2003) The cerebellum
and cognition. Intellectual
function in spinocerebellar ataxia type
6. J Neurol 250:1482–1487
Article
CAS
PubMed
Google Scholar
Gomez CM, Thompson RM, Gammack
JT, Perlman SL, Dobyns WB, Truwit
CL, Zee DS, Clark HB, Anderson JH.
(1997) Spinocerebellar ataxia type 6:
gaze-evoked and vertical nystagmus,
Purkinje cell degeneration, and variable
age of onset. Ann Neurol 42:933–50
Article
CAS
PubMed
Google Scholar
Hayashi T, Nakajima T, Fukuhara N.
(2001) Analysis of regional cerebral
blood flow and distribution volume in
Machado-Joseph disease by iodine)123I
IMP single photon emission computed
tomography. Rinsho Shinkeigaku - Clin
Neurol 41:574–581
CAS
Google Scholar
Kim YK, Lee DS, Lee SK, et al. (2003)
Differential features of metabolic
abnormalities between medial and lateral
temporal lobe epilepsy: quantitative
analysis of (18)F-FDG PET using
SPM. J Nucl Med 44:1006–1012
PubMed
Google Scholar
Kuhl DE, Phelps ME, Markham CH,
et al. (1982) Cerebral metabolism and
atrophy in Huntington’s disease determined
by 18FDG and computed
tomographic scan. Ann Neurol 12:425–434
Article
CAS
PubMed
Google Scholar
Lee YC, Chen JT, Liao KK, Wu ZA,
Soong BW. (2003) Prolonged cortical
relay time and central motor conduction
in patients with spinocerebellar
ataxia type 6. Clin Neurophysiol
114:458–462
Article
PubMed
Google Scholar
Leenders KL, Palmer AJ, Quinn N, et al.
(1986) Brain dopamine metabolism in
patients with Parkinson’s disease measured
with positron emission tomography.
J Neurol Neurosurg Psychiatr
49:853–860
Article
CAS
PubMed
Google Scholar
Murata Y, Yamaguchi S, Kawakami H,
et al. (1998) Characteristic magnetic
resonance imaging findings in Machado-
Joseph disease. Arch Neurol 55:33–37
Article
CAS
PubMed
Google Scholar
Nestor PJ, Graham NL, Fryer TD, Williams
GB, Patterson K, Hodges JR.
(2003) Progressive non-fluent aphasia
is associated with hypometabolism
centred on the left anterior insula.
Brain 126:2406–2418
Article
PubMed
Google Scholar
Otsuka M, Ichiya Y, Kuwabara Y, et al.
(1989) Cerebral blood flow, oxygen and
glucose metabolism with PET in progressive
supranuclear palsy. Ann Nucl
Med 3:111–118
Article
CAS
PubMed
Google Scholar
Poline JB, Worsley KJ, Holmes AP,
et al. (1995) Estimating smoothness in
statistical parametric maps: variability
of p values. J Comput Assist Tomogr
19:788–796
Article
CAS
PubMed
Google Scholar
Salmon E, Collette F, Degueldre C,
Lemaire C, Franck G (2000) Voxelbased
analysis of confounding effects
of age and dementia severity on cerebral
metabolism in Alzheimer’s disease.
Hum Brain Mapp 10:39–48
Article
CAS
PubMed
Google Scholar
Schols L, Peters S, Szymanski S, et al.
(2000) Extrapyramidal motor signs in
degenerative ataxias. Arch Neurol
57:1495–1500
Article
CAS
PubMed
Google Scholar
Schwenkreis P, Tegenthoff M, Witscher
K, et al. (2002) Motor cortex activation
by transcranial magnetic stimulation in
ataxia patients depends on the genetic
defect. Brain 125:301–309
Article
PubMed
Google Scholar
Shinotoh H, Thiessen B, Snow BJ, et al.
(1997) Fluorodopa and raclopride PET
analysis of patients with Machado-Joseph
disease. Neurology 49:1133–1136
CAS
PubMed
Google Scholar
Soong B, Cheng C, Liu R, Shan D
(1997) Machado-Joseph disease: clinical,
molecular, and metabolic characterization
in Chinese kindreds. Ann
Neurol 41:446–452
Article
CAS
PubMed
Google Scholar
Soong BW, Liu RS (1998) Positron
emission tomography in asymptomatic
gene carriers and patients with Machado-
Joseph disease. J Neuro Neurosurg
Psychiatr 64:499–504
Article
CAS
Google Scholar
Soong BW, Lu YC, Choo KB, Lee HY
(2001) Frequency analysis of autosomal
dominant cerebellar ataxias in Taiwanese
patients and clinical and
molecular characterization of spinocerebellar
ataxia type 6. Arch Neurol
58:1105–1109
Article
CAS
Google Scholar
Soong B, Liu R, Wu L, et al. (2001)
Metabolic characterization of spinocerebellar
ataxia type 6. Arch Neurol
58:300–304
Article
CAS
PubMed
Google Scholar
Storey E, Forrest SM, Shaw JH, et al.
(1999) Spinocerebellar ataxia type 2:
clinical features of a pedigree displaying
prominent frontal-executive dysfunction.
Arch Neurol 56:43–50
Article
CAS
PubMed
Google Scholar
Takahashi H, Ikeuchi T, Honma Y,
Hayashi S, Tsuji S (1998) Autosomal
dominant cerebellar ataxia (SCA6):
clinical, genetic and neuropathological
study in a family. Acta Neuropathol
95:333–7
Article
CAS
PubMed
Google Scholar
Takiyama Y, Oyanagi S, Kawashima S,
Sakamoto H, Saito K, Yoshida M, Tsuji
S, Mizuno Y, Nishizawa M (1994) A
clinical and pathologic study of a large
Japanese family with Machado-Joseph
disease tightly linked to the DNA
markers on chromosome 14q. Neurology
44:1302–8
CAS
PubMed
Google Scholar
Talairach J, Tournoux P (1988) Coplanar
stereotaxic atlas of the human
brain 3-dimensional proportional system:
an approach to cerebral imaging.
New York: Thiemen Medical
Google Scholar
Tang B, Liu C, Shen L, et al. (1997)
Frequency of SCA1, SCA2, SCA3/MJD,
SCA6, SCA7, and DRPLA CAG trinucleotide
repeat expansion in patients
with hereditary spinocerebellar ataxia
from Chinese kindreds. Arch Neurol
57:540–544
Article
Google Scholar
Taniwaki T, Sakai T, Kobayashi T, et al.
(1997) Positron emission tomography
(PET) in Machado-Joseph disease. J
Neurol Sci 145:63–67
Article
CAS
PubMed
Google Scholar
Trouillas P, Takayanagi T, Hallett M,
et al. (1997) International Cooperative
Ataxia Rating Scale for pharmacological
assessment of the cerebellar syndrome.
The Ataxia
Neuropharmacology Committee of the
World Federation of Neurology. J
Neurol Sci 145:205–211
Article
CAS
PubMed
Google Scholar
Varrone A, Salvatore E, De Michele G,
et al. (2004) Reduced striatal [123 I]FPCIT
binding in SCA2 patients without
parkinsonism. Ann Neurol 55:426–430
Article
PubMed
CAS
Google Scholar
Wullner U, Reimold M, Abele M, Burk
K, Minnerop M, Dohmen BM, Machulla
HJ, Bares R, Klockgether T (2005)
Dopamine transporter positron emission
tomography in spinocerebellar
ataxias type 1, 2, 3, and 6. Arch Neurol
62:1280–5
Article
PubMed
Google Scholar
Zawacki TM, Grace J, Friedman JH,
Sudarsky L (2002) Executive and emotional
dysfunction in Machado-Joseph
disease. Mov Disord 17:1004–1010
Article
PubMed
Google Scholar