Idiopathic Normal Pressure Hydrocephalus (NPH) is a debilitating condition of the elderly. The patient is typically "wet, wobbly and wonky", to different degrees of the triad. The diagnosis is supported by the radiologic finding of dilated ventricles, determined by an elevated Evan's Index (EI) without a demonstrable cause. Patients with newly diagnosed NPH typically respond to ventriculo-peritoneal shunting (VPS). NPH-related dementia is possibly the only surgically reversible dementia. An elevated cerebrospinal fluid (CSF) fl ow rate (FR) is associated with a positive response to shunting. However, post-shunting EI and FRs are unpredictable. Of late, intracranial apparent diffusion coefficient (ADC) quantification via Diffusion Weighted Imaging (DWI) has been emerging as a possible marker in NPH diagnosis. A local study, conducted on a national level, to study the relationship of EI, FR and ADC to pre- and post-shunt clinical measurements has just ended. This review seeks to reconcile the current thinking of NPH, magnetic resonance imaging (MRI) quantification and clinical evaluation, and in the process shed some light on major pathophysiological determinants of the disease.
Biomarkers ; cerebrospinal fluid ; Cerebrospinal Fluid ; physiology ; secretion ; Diffusion Magnetic Resonance Imaging ; Humans ; Hydrocephalus, Normal Pressure ; diagnosis ; physiopathology

INTRODUCTION: In Europe and North America, the majority of children with high-risk neuroblastoma survive the disease. Elsewhere, the treatment outcomes are poor. METHODS: A retrospective review of children treated for high-risk neuroblastoma in a single institution in Singapore from 2007 to 2019 was carried out. Treatment consisted of intensive chemotherapy, surgery aimed at gross total resection of residual disease after chemotherapy, consolidation with high-dose therapy followed by autologous stem cell rescue, and radiotherapy to the primary and metastatic sites followed by maintenance treatment with either cis-retinoic acid or anti-disialoganglioside monoclonal antibody therapy. Survival data were examined on certain clinical and laboratory factors. RESULTS: There were 57 children (32 male) treated for high-risk neuroblastoma. Their mean age was 3.9 (range 0.7-14.9) years. The median follow-up time was 5.5 (range 1.8-13.0) years for the surviving patients. There were 31 survivors, with 27 patients surviving in first remission, and the five-year overall survival and event-free survival rates were 52.5% and 47.4%, respectively. On log-rank testing, only the group of 17 patients who were exclusively treated at our centre had a survival advantage. Their five-year overall survival rate compared to patients whose initial chemotherapy was done elsewhere was 81.6% versus 41.1% (P = 0.011), and that of event-free survival was 69.7% versus 36.1% (P = 0.032). Published treatment results were obtained from four countries in Southeast Asia with five-year overall survival rates from 13.5% to 28.2%. CONCLUSION Intensified medical and surgical treatment for high-risk neuroblastoma proved to be effective, with superior survival rates compared to previous data from Southeast Asia.
Child ; Humans ; Male ; Infant ; Child, Preschool ; Adolescent ; Disease-Free Survival ; Neuroblastoma/pathology* ; Hematopoietic Stem Cell Transplantation/methods* ; Treatment Outcome ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use* ; Asia, Southeastern/epidemiology* ; Combined Modality Therapy