Table 2 PET and SPECT in chorea of non-HD etiology

ChAc

1989 Dubinsky et al. [21]

PET

[¹⁸F]FDG

Regional glucose metabolism

2 ChAc (brothers)

Decreased in C/Pu

1991 Brooks et al. [22]

PET

[¹⁸F]FDG, [¹¹C]raclopride, C¹⁵O₂

Regional glucose metabolism, D2 binding, regional cerebral blood flow

[¹⁸F]FDG - 6 ChAc; [¹¹C]raclopride with steady-state inhalation of C¹⁵O₂ -30 HC, 16 levodopa responsive PD, 3 ChAc

Normal [¹⁸F]FDG uptake in C and anterior Pu, reduced [¹⁸F]FDG uptake in posterior Pu(similar to PD), reduced [¹¹C]raclopride uptake in C > Pu, reduced regional blood flow to C/Pu

1998 Tanaka et al. [26]

PET

¹⁵O labeled O₂, ¹⁵O labeled CO₂

Regional cerebral metabolic oxygen rate, regional cerebral blood flow

3 ChAc, 7 HC

Reduced regional cerebral blood flow and oxygen metabolism in C/Pu, bilateral frontal and left temporal regions; reduced cerebral blood flow in left parietal and bilateralthalamic regions

2006 Muller-Vahl et al. [25]

PET, SPECT

[¹⁸F]FDG, ¹²³-I-FP-CIT

Regional glucose metabolism, presynaptic dopamine transporters

2 ChAc (monozygotic twins)

Bilateral hypometabolism in C/Pu, reduced ¹²³-I-FP-CIT binding in right hemisphere of one twin (corresponds to more severe left chorea), normal binding in other twin

2010 Selcuk et al. [20]

PET

[¹⁸F]FDG

Regional glucose metabolism

1 ChAc

No FDG uptake in C/Pu

2015 Cui et al. [19]

PET

[¹⁸F]FDG

Regional glucose metabolism

1 ChAc

Decreased in bilateral C/Pu

McLeod syndrome

2001 Jung et al. [27]

PET

[¹⁸F]FDG

Regional glucose metabolism

5 affected males, 2 female mutation carriers, 2 healthy males (all members of a single family)

Reduced regional glucose metabolism in C/Pu correlated with disease duration

2001 Oechsner et al. [28]

PET

[¹⁸F]FDG

Regional glucose metabolism

2 affected males (unrelated), 7 HC

Reduced in C in both patients and in Pu only in one patient with chorea

2012 Miranda et al. [53]

SPECT

⁹⁹Tc-TRODAT-1

Dopamine transporter binding

1 McLeod syndrome

Reduced in Pu

SCA17

2005 Minnerop et al. [30]

PET, SPECT

[¹⁸F]FDG, ¹²³-I-FP-CIT

Regional glucose metabolism, presynaptic dopamine transporters

2 SCA17

Reduced glucose metabolism in Pu in both, and in C, cerebellum, inferior and superior parietal cortex in one; reduced presynaptic dopamine transporters in C/Pu in both

2012 Brockmann et al. [29]

PET

[¹⁸F]FDG, [¹¹C]raclopride, [11C]-D-threo-methylphenidate

Regional glucose metabolism, D2 binding, dopamine transporters

9 SCA17 (5 symptomatic, 4 asymptomatic) of 3 unrelated families

Reduced glucose metabolism in C/Pu, cuneus, cingulum, and parietal lobe; reduced D2 levels and dopamine transporter levels in C/Pu

BHC

1986 Suchowersky et al. [31]

PET

[¹⁸F]FDG

Regional glucose metabolism

3 BHC, 10 HD, 7 HC

Reduced glucose metabolism in C in both BHC and HD

2013 Konishi et al. [32]

PET

[¹¹C]CFT, [¹¹C]raclopride

Dopamine presynaptic transporter binding, D2 binding

2 related BHC patients with a mutation in NKX2.1 gene

Reduced [¹¹C]raclopride binding and normal [¹¹C]CFT binding in C/Pu

Sydenham chorea

Goldman et al. 1993 [36]

PET

[¹⁸F]FDG

Regional glucose metabolism

1 SC

Increased in contralateral C/Pu during chorea, return to normal levels after resolution

1993 Heye et al. [58]

SPECT

^99mTc-HMPAO

Regional cerebral blood flow

1 SC

Hypoperfusion of left BG 5 days after onset of chorea

1993 Weindl et al. [35]

PET

[¹⁸F]FDG

Regional glucose metabolism

2 SC

Increased in C and lentiform nucleus, return to normal levels after resolution of chorea in 1 patient

1999 Lee et al. [59]

SPECT

^99mTc-ECD

Regional cerebral blood flow

1 SC

Increased perfusion of BG and thalamus acutely; normal after symptom resolution

2002 Barsottini et al. [57]

SPECT

^99mTc-HMPAO

Regional cerebral blood flow

10 SC

Increased in BG in 6/10 (scanned closer to symptom onset) compared to 4/10 who exhibited normal perfusion

2004 Demiroren et al. [60]

SPECT

^99mTc-HMPAO

Regional cerebral blood flow

17 SC (SPECT performed in all patients in acute phase and 6 had repeat scan after resolution of chorea)

Hyperperfusion of BG and thalamus seen in 16/17 patients in the acute phase, perfusion in the recovery phase similar to controls

2005 Aron [38]

PET

[¹⁸F]FDG

Regional glucose metabolism

2 SC

Increased C/Pu acutely; normal after symptom resolution on repeat imaging in 1 patient

2011 Paghera et al. [37]

PET

[¹⁸F]FDG

Regional glucose metabolism

1 SC

Increased glucose metabolism in C/Pu during acute phase with return to baseline after resolution

2014 Beato et al. [61]

SPECT

^99mTc-ethyl cysteinate dimer

Regional cerebral blood flow

12 women with SC in remission, 18 HC

Hyperperfusion in left Pu in patients with SC in remission

Polycythemia vera

2008 Kim et al. [56]

SPECT

^99mTc-HMPAO

Regional cerebral blood flow

1 polycythemia vera with acute onset chorea

No change acutely or after symptom resolution

2011 Huang et al. [39]

PET, SPECT

[¹⁸F]FDG, ⁹⁹Tc-TRODAT

Regional glucose metabolism, dopamine transporter levels

1 polycythemia vera with left > right chorea

Increased [¹⁸F]FDG uptake in right dorsolateral prefrontal cortex, left insular cortex and increased ⁹⁹Tc-TRODAT in the right C during acute chorea; return to normal after symptom resolution

Primary antiphospholipid syndrome

1998 Sunden-Cullberg et al. [40]

PET

[¹⁸F]FDG

Regional glucose metabolism

1 primary APL with right hemichorea

Increased in contralateral C and lentiform acutely; normal values after symptom resolution

2009 Nordal et al. [55]

SPECT

Does not specify

Specific tracer and imaging technique not specified

1 primary APL with hemichorea

Decreased circulation in bilateral BG and medial temporal lobes acutely; normal levels after resolution of chorea

2010 Demonty et al. [41]

PET

[¹⁸F]FDG

Regional glucose metabolism

1 patient with chorea associated with APL

Increased in C/Pu acutely; normal after symptom resolution

Thyroid dysfunction

2009 Yu et al. [62]

SPECT

^99mTc-ECD

Regional cerebral blood flow

1 patient with acute chorea as initial presentation of Graves disease

Decreased in bilateral BG, thalamus and right anterior temporal cortex

2013 Chung et al. [42]

PET

[¹⁸F]FDG

Regional glucose metabolism

1 patient with chorea associated with hyperthyroidism

Increased in BG

Hyperglycemia

2007 Nguyen [43]

PET

[¹⁸F]FDG

Regional glucose metabolism

1 hemiballism-hemichorea associated with hyperglycemia (patient developed right chorea several weeks after nonketotic hyperosmolar coma)

Decreased in contralateral C and lentiform nucleus, increased in contralateral motor cortex

2012 Hashimoto et al. [44]

PET

[¹⁸F]FDG

Regional glucose metabolism

2 diabetic patients with sudden onset hemichorea due to non-ketotic hyperglycemia

No change in C/Pu acutely; reduced in C/Pu, GP after resolution of chorea

2014 Tan et al. [45]

PET

[¹⁸F]FDG

Regional glucose metabolism

2 diabetic patients with sudden onset hemichorea in setting of hyperglycemia with positive urine ketones

Increased in contralateral BG in one patient, decreased in contralateral BG in other patient at 55 days from symptom onset

2011 Belcastro et al. [54]

SPECT

¹²³-I-FP-CIT

Presynaptic dopamine transporters

1 patient with hemichorea-hemiballismus due to hyperglycemia

Reduced in contralateral Pu

DRPLA, HD, ChAc, progressive chorea and dementia of unknown etiology (HD negative), vascular hemichorea

1987 Hosokawa et al. [34]

PET

[¹⁸F]FDG

Regional glucose metabolism

5 patients with chorea of differing etiologies (DRPLA, HD, ChAc, progressive chorea and dementia of unknown etiology (HD negative), vascular hemichorea)

Reduced in C/Pu in all patients regardless of etiology (hypometabolism seen only on contralateral side in patient with hemichorea)

Acute stroke, non-ketotic hyperglycemia, SLE

2002 Kim et al. [63]

SPECT

^99mTc-HMPAO

Regional cerebral blood flow

6 patients with acute onset hemichorea (4 acute stroke, 1 non-ketotic hyperglycemia, 1 SLE

Decreased in contralateral BG; increased in thalamus