Spinal Cord Gray and White Matter Damage in Different Hereditary Spastic Paraplegia Subtypes

American Journal of Neuroradiology

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FIG 1.
Axial T2* slices of the cervical spinal cord in a healthy control (upper row) and patients with HSP-SPG3A, HSP-SPG4, HSP-SPG7, and HSP-SPG11 (lower rows). In each case, the original image is shown in the left column, whereas the segmented white (dark gray) as well as the gray matter (light gray) cross-sectional areas are shown in the right column.
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FIG 2.
Box-and-whisker plots showing the distribution of the SC (upper row), GM (middle row), and WM (lower row) areas of patients with HSP-SPG4 versus healthy controls (left side) and patients with HSP-SPG11 versus healthy controls (right side) along cervical levels C2, C3, and C4.

Demographic and clinical data from genotype-specific patients with HSP included in this study^{^a}

Parameter	SPG3A	SPG4	SPG7	SPG11
Sample (No.)	7	12	10	8
Sex (M/F)	2:5	6:6	5:5	4:4
Age (yr)	37.0 (12.0)	57.0 (12.5)	63.0 (10.0)	29.0 (6.0)
Age at onset (yr)	0 (3.5)	37.5 (8.5)	35.0 (10.0)	17.5 (3.5)
Disease duration (yr)	33.0 (10.2)	21.0 (13.0)	27.0 (24.0)	10.0 (6.0)
SPRS-BR	21.0 (18.0)	18.5 (9.0)	22.5 (23.0)	24.0 (9.5)
Leg spasticity	1 (0–2)	1 (0–2)	1 (0–2)	1 (0–2)
Leg muscle strength	4 (3–5)	4 (4–5)	5 (4–5)	4 (4–5)

↵a Parameters are expressed using median (interquartile range). Muscle spasticity and strength were assessed in the following lower limb muscles: thigh adductors and abductors, quadriceps, hamstrings, ankle plantar flexors and dorsal flexors using the Ashworth scale and the Medical Research Council Scale for Muscle Strength, respectively. Results shown in the table represent an average value from all muscles tested in each patient.