Table 2

Functional imaging studies that have correlated cerebral resting-state activity with cognitive performance in Huntington’s disease

StudySubjectsImaging technique/procedureCognitive tasksEvidence of correlation?*Comments and additional findings
StriatumFrontal lobeTemporal lobe
Tanahashi et al (40)16 H
6 C
MMSEN/AReduced frontotemporal rCBF assoc. with deficits in MMSE
Berent et al (39)15 H
15 C
WAIS, WMSN/AN/ACaudate hypometabolism assoc. with deficits in memory and verbal learning performance
Kuwert et al (48)23 H
21 C
WAIS, BVRT, d2 test, Raven’sLentiform hypometabolism assoc. with severity of chorea
Frontoparietal hypometabolism assoc. with deficits in episodic memory, reasoning, visuomotor abilities and attention
Brandt et al (54)21 H
8 C
TMT, SDMTN/AN/ABinding in the caudate assoc. with deficits in tests of rapid coding and set alternation
Binding in the putamen assoc. with duration of illness
Hasselbalch et al (41)18 H
19 C
99mTc-HMPAO SPECTWCST, BDT, FWRTN/AReduced caudate rCBF assoc. with deficits in WCST
Reduced frontal rCBF assoc. with deficits in BDT and FWRT
Bäckman et al (34)5 H
5 C
WCST, TMT, Tower of Hanoi test, verbal fluency test, Rey–Osterreith Complex Figure testNoReduced 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
CANTAB (spatial recognition memory, spatial span, spatial working memory)N/AN/ACaudate 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 C
MRSTMT, Stroop, SDMT, verbal fluency testNoN/AN/AAtrophy in the striatum and reduced NAA levels, but not corr. with cognitive tests
Kremer et al (50)26 H18FDG
MMSE, WAIS, verbal fluency test, immediate recall test, paired associates testN/AN/ACaudate and putamen hypometabolism assoc. with functional capacity, but not assoc. with cognitive measures.
Bachoud-Levi et al (51)5 H18FDG
MMSE, TMT, Stroop, RAVLT, Raven’s, Token Test, JLOTN/AN/ACaudate and putamen hypermetabolism assoc. with deficits in attention and executive functioning
Pavese et al (56)12 H[11C]raclopride
MMSE, TMT, Stroop, WMS, SDMT, Digit Span, BDT, CANTAB, Tower of London testReduced [11C]raclopride over time in striatum, frontal and temporal lobes.
Reduced striatial [11C]raclopride corr. with Tower of London test
  • Assoc. = 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.