Table 1
Dystonia Classification
| Classification | |
|---|---|
| Body of distribution | Focal |
| Segmental | |
| Generalized | |
| Age of onset | Early onset (<26 years of age) |
| Late onset (∼30–50 years of age) | |
| Etiology | Primary torsion +/− tremor |
| Secondary | |
| Dystonia-plus syndrome | |
| Heredodegenerative | |
| Feature of another neurological condition |
Table 2
MRI with Voxel-Based Morphometry in Primary Dystonia
| Primary Dystonia | Study | No. Cases | Average Age | Gray Matter Volume (GMV) | GMV Correlation | ||
|---|---|---|---|---|---|---|---|
| Increased | Decreased | Severity | Duration | ||||
| BLS | Black et al., 1998 | 5 | --- | Putamen | -- | -- | |
| Etgen et al., 2006 | 16 | 67.4 | Putamen | L. Inf. Parietal lobe | -- | NR | |
| Obermann et al., 2007 | 11 | 52.6 | Caud., CBL | Putamen, thalamus | -- | -- | |
| Horovitz et al., 2012 | 14 | 59.9 | L. mid-temporal gyrus, R. Postcentral gyrus, B/L precuneus | R. OFC, L. M1, L. IFG, R. OC, R. ACC | NR | Inv.- R occ. | |
| Martino et al., 2011 | 25 | 64.9 | R. middle frontal gyrus | L. Postcentral gyrus, L. STG | Inv. – R. middle frontal gyrus | NR | |
| CD | Draganski et al., 2003 | 10 | 44 | M1, CBL, R. GP | R. SMA, R. PFC, R. OC | --- | --- |
| Obermann et al., 2007 | 9 | 57.6 | Caud., L. CBL, STL, thalamus | Putamen, L. STL | --- | --- | |
| Egger et al., 2007 | 11 | 49.6 | R. GP, medial frontal cortex, L. SMA OFC, cingulate cortex | --- | --- | ||
| Draganski et al., 2009 | 29 | Putamen, GP | Inv.-Putamen | --- | |||
| Pantano et al., 2011 | 19 | 55.5 | L. Caud., putamen, SMC, PMC | NR | NR | ||
| FHD | Black et al., 1998 | 8 | --- | Putamen | --- | --- | |
| Delmaire et al., 2007 | 30 (RH) | 49.7 | L. M1, L. S1, B/L CBL, pulvinar | --- | --- | ||
| Granert et al., 2011 | 14 WC (RH) | 50.6 | L. M1 | --- | --- | ||
| Garraux et al., 2004 | 36 (31 WC, 5 MD) | 53 | B/L M1/S1 (Hand region) | --- | --- | ||
| Egger et al., 2007 | 11 (9 WC, 2 MD) | 41.9 | R. GP | --- | --- | ||
| Granert et al., 2011 | 11 MD (RH) | 43 | R>L putamen | Pos. - Putamen | --- | ||
| SD | Simonyan and Ludlow 2012 | 40 (25 AD, 15 AB) | 56.9 | L. M1/S1, supramarginal gyrus, putamen, R. IFG, B/L CBL | Pos.- R. IFG, L. CBL | NR | |
| HD | Egger et al., 2007 | 9 (DYT1) | 39.8 | R GP | --- | --- | |
| Draganski et al., 2009 | 22 DYT1 (11 MN, 11 NMN) | B/L putamen (NMN>MN) | Inv., putamen |

Figure 1
Differences in Increases in GMV in Patients with SD compared to Healthy Controls are Dependent on Sample Size. The color bar represents F values and reflects the significance of changes in patients compared to controls.

Figure 2
Impact of the Choice of T1-weighted Sequence on Brain Tissue Segmentation for VBM analysis. The segmented gray matter images were acquired using MPRAGE (magnetization-prepared rapid acquisition with gradient echo, left column) and SPGR (spoiled gradient recalled echo, right column) sequences. Fully automated segmentation into gray matter, white matter, and CSF was performed using SPM8 software. The red arrows indicate the over-segmented primary motor cortex and basal ganglia.
Table 3
Diffusion Tensor Imaging in Primary Dystonia
| Primary Dystonia | Study | No. Cases | Average Age | DTI | DTI Correlations | ||
|---|---|---|---|---|---|---|---|
| Fractional Anisotropy (FA) | Mean diffusivity (MD) | Severity | Duration | ||||
| BLS | Fabbrini et al., 2008 | 16 | 66.5 | NS | NS | --- | --- |
| Horovitz et al., 2012 | 14 | 59.9 | Reduced connectivity | --- | --- | ||
| CD | Colosimo et al., 2005 | 15 | 51.9 | Inc. – Putamen | Dec. – L. GP, L. putamen, B/L Caud. | NR | NR |
| Dec. - genu CC | |||||||
| Bonilha et al., 2007 | 7 | 58 | Dec.-R. Thal., R. PFC | Inc.-B/L. GP, putamen, L. Caud. | --- | --- | |
| Fabbrini et al., 2008 | 18 | 55.5 | Inc. – Putamen | Inc.-B/L PFC, L.SMA | NR | NR | |
| Dec.- Body of CC | Dec.-R Caud | ||||||
| Blood et al., 2012 | 12 | 59.2 | Dec. GP-BS connectivity | --- | --- | ||
| FHD | Delmaire et al., 2009 | 26 | 42.8 | Inc.- IC, Thal., L. centrum semiovale | --- | --- | --- |
| SD | Simonyan et al., 2008 | 20 | 55 | Dec.- R. Genu IC | Inc.-CR, Genu & Posterior Limb IC, middle CBL peduncle, Thal., putamen | Pos. - MD in Thal. | --- |
| HD | Carbon et al., 2004 | 12 (DYT1) | 44.1 | Dec. SMC | --- | --- | |
| Carbon et al., 2008 | 7 (4 DYT1, 3 DYT6) | 29.3 | Dec. – Dorsal pontine BS, SMC, | --- | --- | --- | |
| Cheng et al., 2012 | 6 (DYT6) | Dec. - SMC | Inc. – SLF and CST | --- | --- | ||
| Van de Meer et al., 2012 | 16 (DYT11) | 43.5 | Inc. – R. STN, RN | Dec. –subgyral SMC; STN | --- | --- |
[i] Abbreviations: BS, brainstem; BLS, blepharospasm; Caud., caudate; CD, cervical dystonia; CC, corpus callosum; CR, corona radiata; Dec., decreased; FHD, focal hand dystonia; HD, hereditary dystonia; IC, internal capsule; Inc., increased; NR, no relationship; NS, no significance; Ped, peduncle; PFC, prefrontal cortex; RN, red nucleus; SD, spasmodic dysphonia; SMA–supplementary motor area; SMC, sensorimotor cortices; SLF, superior longitudinal fasciculus; STN, subthalamic nucleus; Thal., thalamus; (---), no data.
