Figure 6.

Mucopolysaccharide (MPS) Type VI. 12-year-old male suffering from repeated convulsions and on tegretol and epilim. Early in his life, the boy was noticed to have a big head, coarse facial features, a flat nasal bridge, protruding eyes, short stature, lumbar lordosis, hearing impairment and progressively increasing bilateral corneal haziness leading to blindness. He also has bilateral inguinal hernia and multiple joint stiffness including wrists, elbows, hips, knees and ankles. He is currently on enzyme replacement therapy. (a) T₂ weighted axial image of brain showing moderate hydrocephalus (black arrow), bi-frontal white matter T₂ hyperintensities suggestive of bifrontal demyelination (arrowhead). (b) MR spine T₂ weighted sagittal image showing flattened vertebral bodies and disc protrusions at multiple levels (black arrowhead). Spinal canal narrowing and cord indentation is noted at the cervicomedullary junction (white arrowhead). Short segment syringomyelia is present at C4–6 levels (white arrow).