Hereditary spastic paraplegia (HSP) linked to the spastic gait gene 4 (SPG4) is controversial, as the “pure”form traditionally has been considered confined to the long axons of the spinal cord. However, recent immunolabeling experiments have demonstrated extensive Spastin expression in the cortex and striatum. This could indicate a more widespread neuropathology from mutations in the SPG4 gene than previously assumed. The aim of this study was therefore to ascertain the extent of cerebral involvement in SPG4 linked HSP by neuropsychological examination and measurement of the regional cerebral blood flow (rCBF) as an indirect marker of regional neuronal activity. Eighteen SPG4 patients and 18 matched control subjects were studied. Resting state rCBF was measured using Positron Emission Tomography (PET) and the 15O-labelled water bolus technique and relative group differences were explored using Statistical Parametric Mapping (SPM 99). Neuropsychological assessment was performed using established and nationally validated tests (RH Basic Battery). Compared to healthy controls, the patient group had significantly decreased rCBF in the left fronto-temporal cortex (P < 0.05), and more extensive changes were observed in a separate analysis of the most disabled individuals. The neuropsychological assessment revealed only significantly impaired recognition memory for faces. In summary, the findings support cerebral pathology in SPG4-linked HSP, although the decreased rCBF in fronto-temporal cortex was not associated with severe cognitive impairment.
新生儿为了生存需要从周期性的Ⅱ乎吸形式变成连续性的呼吸形式，早产儿呼吸中枢发育不成热，易发生呼吸暂停。早产儿Ⅱ乎吸暂停（Apnea of prematurity，AOP）的发生率随着胎龄的增加而降低。早产儿本身神经系统发育不成熟，而Ⅱ乎吸暂停时常伴有脑血流量减少导致脑损伤，如果严重的反复发作的Ⅱ乎吸暂停处理不当，可以导致脑室周围白质软化、脑性瘫痪、耳聋等不良后果.故减少AOP的发生对早产儿远期预后有重要影响。本文就AOP的诊治进展进行综述。
