Article Figures & Tables
Tables
- Table 1
Structural imaging studies that have correlated brain morphology with cognitive performance in Huntington’s disease
Study Subjects Imaging technique/procedure Cognitive tasks Evidence of correlation?* Comments and additional findings Striatum Frontal lobe Temporal lobe Bamford et al (27) 60 H CT WAIS, TMT, Stroop, Porteus Mazes, PVT, RSPM Yes N/A N/A Atrophy in striatum corr. with attention, working memory and executive functions Harris et al (37) 15 H
19 CMRI MMSE Yes N/A N/A Atrophy in caudate corr. with MMSE Harris et al (29) 10 H
9 CMRI
SPECTMMSE, WCST, TMT, Stroop, Digit Span, Luria, BVRT Yes N/A N/A Caudate volume and thalamus rCBF corr. with MMSE Global atrophy corr. with TMT Aylward et al (30) 20 H
20 CMRI (1.5 T)
Manual tracingMMSE, WAIS, TMT, Stroop, COWA, DTVMI, BTA, HVLT, BVRT No Yes N/A Smaller total brain, frontal lobe and frontal white matter. Frontal lobe volume corr. with MMSE and verbal and spatial ability Kassubek et al (33) 44 H
22 CMRI (1.5 T)
VBMCOWA, Stroop, WAIS Yes N/A N/A Decreases of grey matter of the thalamus corr. with COWA and Stroop BTA = Brief Test of Attention; BVRT = Benton Visual Retention Test; C = controls; corr. = correlated; COWA = Control Oral Word Association; CT = computed tomography; Digit Span = Digit-Span Memory Test; DTVMI = Developmental Test of Visual Motor Integration; H = subjects with Huntington’s disease; HVLT = Hopkins Verbal Learning Test; Luria = Luria Sequential Hand Positions Tests; MMSE = Mini-Mental State Examination; MRI = magnetic resonance imaging; PPVT = Peabody Picture Vocabulary Test; rCBF = regional cerebral blood flow; RSPM = Raven’s Standard Progressive Matrices; SPECT = single-photon emission computed tomography; Stroop = Stroop Test; TMT = Trail-Making Test; VBM = voxel-based morphometric analysis; WAIS = Wechsler Adult Intelligence Scale; WCST = Wisconsin Card Sorting Test.
↵* N/A = region not assessed; Yes = correlation; No = no correlation.
- Table 2
Functional imaging studies that have correlated cerebral resting-state activity with cognitive performance in Huntington’s disease
Study Subjects Imaging technique/procedure Cognitive tasks Evidence of correlation?* Comments and additional findings Striatum Frontal lobe Temporal lobe Tanahashi et al (40) 16 H
6 C133Xe
SPECTMMSE N/A ↓ ↓ Reduced frontotemporal rCBF assoc. with deficits in MMSE Berent et al (39) 15 H
15 C18FDG
PETWAIS, WMS ↓ N/A N/A Caudate hypometabolism assoc. with deficits in memory and verbal learning performance Kuwert et al (48) 23 H
21 C18FDG
PETWAIS, BVRT, d2 test, Raven’s ↓ ↓ ↓ Lentiform hypometabolism assoc. with severity of chorea
Frontoparietal hypometabolism assoc. with deficits in episodic memory, reasoning, visuomotor abilities and attentionBrandt et al (54) 21 H
8 C†[11C]3-Nmethylspiperone
PETTMT, SDMT ↓ N/A N/A Binding in the caudate assoc. with deficits in tests of rapid coding and set alternation
Binding in the putamen assoc. with duration of illnessHasselbalch et al (41) 18 H
19 C99mTc-HMPAO SPECT WCST, BDT, FWRT ↓ ↓ N/A Reduced caudate rCBF assoc. with deficits in WCST
Reduced frontal rCBF assoc. with deficits in BDT and FWRTBäckman et al (34) 5 H
5 C[11C]SCH
[11C]raclopride
PETWCST, TMT, Tower of Hanoi test, verbal fluency test, Rey–Osterreith Complex Figure test ↓ ↓ No Reduced striatum D1 dopamine, D2, DAT binding assoc. with executive function, visuospatial skills, episodic memory, verbal fluency, perceptual speed and reasoning deficits Lawrence et al (55) 23 H‡
20 C[11C]raclopride
PETCANTAB (spatial recognition memory, spatial span, spatial working memory) ↓ N/A N/A Caudate and putamen D1 and D2 receptor binding assoc. with deficits in verbal fluency, pattern recognition, spatial information span, complex problem solving and sequence generation Sanchez-Pernaute et al (57) 10 H
10 CMRS TMT, Stroop, SDMT, verbal fluency test No N/A N/A Atrophy in the striatum and reduced NAA levels, but not corr. with cognitive tests Kremer et al (50) 26 H 18FDG
PETMMSE, WAIS, verbal fluency test, immediate recall test, paired associates test ↓ N/A N/A Caudate and putamen hypometabolism assoc. with functional capacity, but not assoc. with cognitive measures. Bachoud-Levi et al (51) 5 H 18FDG
PETMMSE, TMT, Stroop, RAVLT, Raven’s, Token Test, JLOT ↑ N/A N/A Caudate and putamen hypermetabolism assoc. with deficits in attention and executive functioning Pavese et al (56) 12 H [11C]raclopride
PETMMSE, TMT, Stroop, WMS, SDMT, Digit Span, BDT, CANTAB, Tower of London test ↓ ↓ ↓ Reduced [11C]raclopride over time in striatum, frontal and temporal lobes.
Reduced striatial [11C]raclopride corr. with Tower of London testAssoc. = associated; BDT= Block Design test; BVRT= Benton Visual Retention Test; CANTAB = Cambridge Neuropsychological Test Automated Battery; C = controls; corr. = correlated; d2 test = visuomotor test; DAT = dopamine transporter; FWRT= Face and Word Recognition Test; H = patients with Huntington’s disease; JLOT = Judgment of Line Orientation Task; MMSE = Mini-Mental State Examination; MRS = magnetic resonance spectroscopy; NAA = N-acetyl aspartate; PET= positron emission tomography; Raven’s = Raven’s Standard Progressive Matrices; RAVLT = Ray Auditory Verbal Learning Test; rCBF = regional cerebral blood flow; SDMT =Symbol Digit Modality Test; SPECT= single-photon emission computed tomography; TMT= Trail-Making Test; WAIS = Wechsler Adult Intelligence Scale; WMS = Wechsler Memory Scale; WSCT = Wisconsin Card Sorting Test.
↵* ↑ = increased activity; ↓ = decreased activity; N/A = region was not assessed; No = no correlation.
↵† Subjects at risk for Huntington’s disease (HD).
↵‡ 5 symptomatic patients with HD and 17 asymptomatic mutation carriers.
- Table 3
Functional imaging studies that have assessed cerebral activity during cognitive performance in Huntington’s disease
Study Subjects Imaging technique/procedure Cognitive tasks Differences in activation?* Comments and additional findings Striatum Frontal lobe Temporal lobe Goldberg et al (58) 11 H
11 Sc133Xe
SPECTWAIS, WCST No ↑ No Increased frontal activation assoc. with WCST performance Weinberger et al (59) 14 H
14 C133Xe
SPECTWAIS, WCST No No N/A No association between frontal rCBF and WCST performance Deckel et al (60) 7 H
9 C99Tc
SPECTStroop Colour Test, Digital Symbol Modalities Test, Word Test, Maze Test ↓ No No Reduced caudate rCBF during Maze Test Clark et al (61) 3 H fMRI Maze Test ↓ ↑ No Reduced caudate and parietal fMRI signal and increased frontal fMRI signal during Maze testing Voermans et al (62) 12 H
18 CfMRI WAIS, WMS, CVLT, route recognition test ↓ ↑ ↑ Reduced caudate activity and increased temporal activity during route recognition Kim et al (63) 8 H fMRI MMSE, SRTT No ↓ No Reduced activation in frontal and occipital regions during SRTT C = controls; CVLT = California Verbal Learning Test; fMRI = functional magnetic resonance imaging; H = subjects with Huntington’s disease; MMSE = Mini-Mental State Examination; rCBF=regional cerebral blood flow; Sc = subjects with schizophrenia; SPECT = single-photon emission computed tomography; SRTT = serial reaction time task paradigm; WAIS = Wechsler Adult Intelligence Scale; WCST = Wisconsin Card Sorting Test; WMS = Wechsler Memory Scale.
↵* ↑ = greater activation in H; ↓ = less activation in H; No = no differences; N/A = region was not assessed.