Cerebral and occipito-atlanto-axial involvement in mucopolysaccharidosis patients: clinical, radiological, and neurosurgical features

Table 1 The occurrence rate of hydrocephalus/ventriculomegaly, brain atrophy, and spinal stenosis in patients with mucopolysaccharidosis (MPS)

	Hydrocephalus/ventriculomegaly	Atrophy	Spinal stenosis
MPS I
Hurler	+++	++	++
Hurler/Scheie	++	+	++
Scheie	+++	+	++
MPS II (Hunter)	+++	+++	+
MPS III (Sanfilippo)
III A	++	+	+
III B	++	+++	–
III C	–	–	–
III D	+	+	–
MPS IV (Morquio)
IV A	+/−	–	+++
IV B	+/−	–	+++
MPS VI (Maroteaux-Lamy)	+	+	+++
MPS VII	++	–	+
MPS IX	–	–	–

Cerebral pathology is more common in MPS I, II, and III, while spinal involvement principally occurs in MPS IV and VI
Modified from Zareiriou et al. [3]

ISSN: 1824-7288