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pages 105 to 106) In nonprogressive meningeal and ependymal diseases, hydrocephalus may develop and reach a stable stage It is said to be compensated, in the sense that formation of CSF equilibrates with absorption The formation of CSF diminishes slightly, perhaps because of compression of the choroid plexuses; absorption increases in proportion to CSF pressure Once equilibrium is attained, the ICP gradually falls, though it still maintains a slightly higher gradient from ventricle to basal cistern to cerebral subarachnoid space A stage is reached where the CSF pressure reaches a high normal level of 150 to 200 mmH2O while the patient still manifests the cerebral effects of the hydrocephalic state The name given to this condition by Hakim, Adams and Fisher was normal-pressure hydrocephalus (NPH) A triad of clinical ndings is characteristic of NPH a slowly progressive gait disorder is usually the earliest feature, followed by impairment of mental function, and sphincteric incontinence Grasp re exes in the feet and falling attacks may also occur but there are no Babinski signs Headaches are infrequently a complaint, and there is no papilledema The gait disturbance may be of several different types, as discussed in Chap 7; some of them are dif cult to classify, but certain features predominate Most often it takes the form of unsteadiness and impairment of balance, with the greatest dif culty being encountered on stairs and curbs (Fisher) Weakness and tiredness of the legs are also frequent complaints, although examination discloses no paresis or ataxia The gait in NPH may convey an impression of Parkinson disease, with short steps and stooped, forward-leaning posture, but there is no rigidity, slowness of alternating movement, or tremor Other patients present with unexplained falls, often helplessly backward, but on casual inspection the gait may betray no abnormality at all When the condition remains untreated, the steps become shorter, with frequent shuf ing and falls; eventually standing and sitting and even turning over in bed become impossible Fisher refers to this advanced state as hydrocephalic astasia-abasia The mental changes in the cases we have encountered have been, broadly speaking, frontal in character and embody mainly apathy, dullness in thinking and actions, and slight inattention Memory trouble is usually a component of the overall problem and has been predominant in some cases, for which reason the diagnosis of Alzheimer disease has been made There is usually a degree of affective indifference but the patient reports little in the way of emotionality The extensive study of 63 patients by De Mol largely corroborated these impressions but also found dif culties in verbal, graphical, and calculation skills with which we have not been impressed It is notable that his patients with verbal dif culties did not improve with shunting, suggesting to us that they had a degenerative dementia Unfortunately, beyond the above-noted defects that are elicitable by routine testing, we have not found neuropsychologic tests of great value in the diagnosis of NPH Urinary symptoms appear relatively late in the illness Initially, they consist of urgency and frequency Later, the urgency is associated with incontinence, and ultimately there is frontal lobe incontinence, in which the patient is indifferent to his lapses of continence This syndrome of NPH may follow subarachnoid hemorrhage from ruptured aneurysm or head trauma, a resolved acute meningitis or a chronic meningitis (tubercular, syphilitic, or other), Paget.

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disease of the base of the skull, mucopolysaccharidosis of the meninges, and achondroplasia However, in most cases, the cause cannot be established; presumably it is due to an asymptomatic brosing meningitis That the mechanical effect of ventricular enlargement on the adjacent brain is responsible for the syndrome is supported by Fisher s observations that a reduction in ventricular size caused even by extrinsic compression from subdural collections is associated with clinical improvement Veri cation of the diagnosis of NPH and the selection of patients for ventriculoatrial or ventriculoperitoneal shunt has presented dif culties A lumbar puncture should be performed for diagnostic purposes and the pressure measured carefully As mentioned, the large ventricles, even at a normal pressure, continue to exert a force against the tracts in the cerebral white matter In most cases of NPH, the CSF pressure is above 155 mmH2O, but the disorder has occurred infrequently with lower pressures, in a few instances as low as 130 mmH2O or even less The CT scan, as shown in Fig 30-3 (enlarged ventricles without convolutional atrophy), radionuclide cisternography (re ux into ventricles and delayed pericerebral diffusion), and particularly the clinical response to the removal of CSF have been the most helpful ancillary examinations in our experience (see below) However, the ndings are not always clear-cut, and several small series suggest that a negative test does not preclude bene t from shunting (see, for example Walchenbach et al) Moreover, in these same series, improvement after removal of CSF has had a high predictive value for success with shunting The span of the frontal horns of the lateral ventricles by CT scanning has also been used as a rough guide to the likelihood of success from ventricular shunting (see below) More relevant is a disproportionate enlargement of the ven-

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