Most shunt-dependent hydrocephalic patients present with predictable symptoms of headache and mental status changes when their cerebrospinal fluid shunts malfunction. Their intracranial pressure (ICP) is usually high, and they usually respond to routine shunt revision. This report describes 12 shunted patients who were admitted with the full-blown hydrocephalic syndrome but with low to low-normal ICP. All 12 patients had been maintained previously on medium-pressure shunts. Their symptoms included headache, lethargy, obtundation, and cranial neuropathies. At peak symptoms, their ventricular sizes were large (ventricular/biparietal ratio of 0.35 to 0.45) in six and massive (ventricular/biparietal ratio > 0.45) in six and their ICPs ranged from 2.2 to 6.6 mm Hg, with a mean of 4.4 +/- 1.3 mm Hg (+/- standard deviation), i.e., below or well within the pressure range of their shunts. The pressure volume index of three patients at peak symptoms ranged from 39.2 to 48.5 ml, with a mean of 43.9 +/- 4.6 ml, which represents a 190% increase from the predicted normal value. Seven patients failed to improve with multiple shunt revisions, including the use of low-pressure valves. In 11 patients, symptoms and ventriculomegaly were not reversed except with prolonged external ventricular drainage at subzero pressures (mean external ventricular drainage nadir pressure of -5.7 +/- 3.6 mm Hg, for a mean period of 22.2 days). During external ventricular drainage treatment, symptoms correlated only with ventricular size and not with ICP. All 11 were subsequently treated successfully with a new medium- or low-pressure shunt. One patient was treated successfully with prolonged shunt pumping. We postulate that: 1) the development of this low-pressure hydrocephalic state is related to alteration of the viscoelastic modulus of the brain, secondary to expulsion of extracellular water from the brain parenchyma, and to structural changes in brain tissues due to prolonged overstretching; 2) certain patients are susceptible to developing low-pressure hydrocephalic state because of an innate low brain elasticity due to bioatrophic changes; 3) low-pressure hydrocephalic state symptoms are due not to pressure changes but to brain tissue distortion and cortical ischemia secondary to severe ventricular distortion and elevated radial compressive stresses within the brain; and 4) treatment must be directed toward allowing the entry of water into the brain parenchyma and the restoration of baseline brain viscoelasticity.
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