Fowler G W, Sukoff M, Hamilton A, Williams J P
Childs Brain. 1975;1(4):251-4. doi: 10.1159/000119574.
The authors have seen eight cases of communicating hydrocephalus in children with genetic metabolic disorders, namely, one mucopolysaccharidosis I (MPS I or Hurler syndrome), one MPS II (Hunter's disease), four MPS III (Sanfilippo syndrome) two of which were siblings, and two achondroplasias. The authors recommend surgical treatment of the latter but are doubtful about the former in which case hydrocephalus is only a contributing cause to severe dementia. In MPS hydrocephalus is due to accumulation of storage material in the piaarachnoid, as indicated by RISA cisternography carried out in six cases.
作者见过8例患有遗传性代谢疾病的儿童交通性脑积水病例，即1例黏多糖贮积症I型（MPS I或Hurler综合征）、1例MPS II型（Hunter病）、4例MPS III型（Sanfilippo综合征，其中2例为 siblings）以及2例软骨发育不全。作者建议对后者进行手术治疗，但对前者存疑，因为在前者中脑积水只是导致严重痴呆的一个促成因素。在黏多糖贮积症中，脑积水是由于软脑膜蛛网膜中蓄积了储存物质，6例病例的放射性核素脑池造影显示了这一点。
