Communicating chronic hydrocephalus: A review

Abstract

Formerly called normal pressure hydrocephalus, communicating chronic hydrocephalus (CCH) is a condition affecting 0.1 to 0.5% of patients over 60years of age. The pathophysiology of this disease is poorly understood, but a defect in cerebrospinal fluid (CSF) resorption appears to be commonly defined as the cause of the neurological disorders. The last important discovery is the description of the glymphatic system and its implication in CCH and CSF resorption. Comorbidities (Alzheimer's disease, microangiopathy, parkinsonism) are very frequent, and involve a diagnostic challenge. The clinical presentation is based on the Hakim and Adams triad, comprising gait disorders, mainly impairing walking, cognitive disorders, affecting executive functions, episodic memory, visuospatial cognition, and sphincter disorders as urinary incontinence (detrusor hyperactivity). The diagnosis is suspected through a set of arguments, combining the clinical presentation, the radiological data of the magnetic resonance imaging (MRI) showing a ventriculomegaly associated with signs of transependymomous resorption of the CSF and disappearance of the cortical sulci, and the clinical response to the depletion of CSF. In the presence of all these elements, or a strong clinical suspicion, the standard treatment will be of a permanent CSF shunt, using a ventriculoatrial or ventriculoperitoneal shunt. The effectiveness of this treatment defines the diagnosis. The clinical improvement is better when treatment occurs early after the onset of the disorders, reaching 75 to 90% of motor improvement.

Keywords: CSF tap test; Communicating chronic hydrocephalus; Hydrocéphalie chronique communicante; Hydrocéphalie à pression normale; Lumbar puncture; Normal pressure hydrocephalus; Ponction lombaire; Tests hydrodynamiques.