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This chapter first focuses on the ethics of animal models and then discusses the formal requirements general to any experimental model and the specific requirements for models of hydrocephalus. It also discusses the recent and current research areas in experimental hydrocephalus. Ethical standards for experimental studies involving animals are legally set by laws and regulations. Animal models resemble human disease by conditions which are genetically determined, naturally acquired, or induced by the investigator. Shunted animal models provide a unique potential for insight into questions concerning the destructive effects of hydrocephalus on the brain and its development, as it is rarely possible to obtain brain tissue from humans with hydrocephalus. Most of the hydrocephalus animal models are neonatal or juvenile animals and the majority of research is thus directed at congenital or pediatric hydrocephalus.
The recently updated Japanese guidelines draw attention to a specific MRI pattern of disproportionately enlarged subarachnoid space hydrocephalus (DESH), believed to be pathognomonic of idiopathic normal pressure hydrocephalus (iNPH). This chapter discusses why establishing the diagnosis of NPH remains a challenge fifty years after its classic description. The original diagnosis of NPH relied upon the presence of mild dementia, gait, and urinary difficulties (Hakim's triad) seen in association with ventriculomegaly on pneumo-encephalogram. More sensitive cognitive evaluation of iNPH patients requires specific tests for the assessment of subcortical frontal lobe deficits such as the Rey Auditory Verbal Learning Test, Stroop test, Grooved Pegboard, Trail Making A and B Test, and digit span test. This diagnostic test provides information about cerebrospinal fluid (CSF) dynamics and predicts outcome. It consists in either removal of CSF accompanied by pre and post functional evaluation, or an infusion (bolus or continuous) test.