TABLE 2.
Main Brain and Spine Magnetic Resonance Imaging Features Analyzed in a Cohort of 44 Adult Polyglucosan Body Disease Patients
| Characteristic | Value |
|---|---|
| Age, yr (range) | 58 ±7 (39–73) |
| WM lesions | |
| Predominant affected regions | |
| Periventricular | 68% |
| Deep | 9% |
| Subcortical | 7% |
| None | 16% |
| Predominant affected lobes | |
| Occipital | 54% |
| Parietal | 7% |
| None | 39% |
| Capsules | |
| Internal posterior limb | 77% |
| Internal anterior limb | 7% |
| External | 67% |
| Cerebellum | |
| Hilus dentatus | 30% |
| Inferior peduncle | 21% |
| Mid peduncle | 23% |
| Superior peduncle | 14% |
| Midbrain | |
| Medial lemniscus | 89% |
| Pyramidal tract | 95% |
| Pons | |
| Medial lemniscus | 93% |
| Pyramidal tract | 91% |
| Medulla | |
| Decussatio, medial lemniscus | 95% |
| Pyramidal tract | 98% |
| Aspect of WM lesions | |
| Confluent | 84% |
| Multifocal | 70% |
| Symmetrical | 86% |
| Atrophy | |
| Ventricles, enlarged | 32%, 11% mild |
| Cerebral spaces, increased | 18%, 7% mild |
| Corpus callosum | 51%, 23% mild |
| Cerebellum hemispheres | 14% |
| Vermis | 70%, 20% mild |
| Medulla oblongata | 100% |
| Spine | 100% |
WM = white matter.