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Normal Pressure Hydrocephalus

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Abstract

Although it has been nearly 50 years since its first description of clinical and radiological, there is considerable uncertainty about the diagnosis of normal pressure hydrocephalus because it shares the semiotics with the group of dementias. Hakim’s triad (impaired gait, initially type clumsiness of the lower limbs followed over time by inability to ambulate or maintain an erect posture; cognitive impairment, initially limited to worsening deficits in memory fixation and execution of complex actions; the urinary disorder, initially type “urgent urination” and then complete urinary incontinence) characterizes the progressive course of the adult chronic hydrocephalus. The clinical onset is typically nonspecific, subtle, and most often monosymptomatic. The first diagnostic procedure is a head CT scan and/or brain MRI, which shows an abnormal dilatation of the lateral ventricles and the third ventricle, associated to variable brain atrophy. Not all subjects will develop a set of symptoms, since the altered cerebrospinal fluid dynamics can remain stable for many years or get progressively worse until the appearance of the clinical triad of normal pressure hydrocephalus. The test of intrathecal infusion (Katzman test) carried out at constant speed with the introduction of saline solution into the lumbar subarachnoid space and the concomitant detection of cerebrospinal fluid pressure establishes that patients with outflow resistance ranging from 12 to 19 mmHg/ml/min can improve clinically after surgery. This method requires extensive and prolonged experience of the center of application and the use of computer systems. The withdrawal of lumbar cerebrospinal fluid provides for the evacuation of 30–50 cc of cerebrospinal fluid by lumbar puncture under local anesthesia, preceded and followed by gait assessment and neuropsychological tests. It also uses the continuous withdrawal of CSF with an intrathecal catheter placed for 3 days, in order to drain approximately 135 ml/24 h and the aim of reducing false negatives. After surgery, the patient is usually able to regain a good quality of life, with independence in daily living activities. The duration of such postsurgical improvement is variable, but patients may improve again readjusting the opening pressure of the programmable valve, although a high comorbidity index is strictly related to a poor outcome.

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Missori, P., Daniele, A., Colosimo, C. (2014). Normal Pressure Hydrocephalus. In: Merello, M., Starkstein, S. (eds) Movement Disorders in Dementias. Springer, London. https://doi.org/10.1007/978-1-4471-6365-7_14

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