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MAJOR CATEGORIES OF NEUROLOGIC DISEASE
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Characteristically in these cases, there is both a generalized dilatation of the ventricular system and basal cisterns (possibly due to increased CSF volume) and an asymmetrical enlargement of the lateral ventricles due to obstruction of one foramen of Monro Clinical Picture of Chronic Hydrocephalus This varies with the age of the patient and chronicity of the condition Four main clinical syndromes are recognized one that occurs very early in life and causes enlargement of the head (overt tension hydrocephalus) and another in which the hydrocephalus becomes symptomatic after the cranial sutures have fused and the head remains normal in size (occult hydrocephalus) A special a form of the latter is arrested or compensated hydrocephalus of late adult life (normalpressure hydrocephalus) The fourth type is acute hydrocephalus Overt Congenital or Infantile Hydrocephalus The cranial bones fuse by the end of the third year; for the head to enlarge, the tension hydrocephalus must develop before this time It may begin in utero but usually happens in the rst few months of life Even up to 5 years of age (and very rarely beyond this time), a marked increase of ICP, particularly if it evolves rapidly, may separate the newly formed sutures (diastasis) Tension hydrocephalus, even of mild degree, also molds the shape of the skull in early life, and in radiographs the inner table is unevenly thinned, an appearance referred to as beaten silver or as convolutional or digital markings The frontal regions are unusually prominent (bossed) and the skull tends to be brachiocephalic except in the Dandy-Walker syndrome, where, because of bossing of the occiput from enlargement of the posterior fossa, the head is dolichocephalic With marked enlargement of the skull, the face looks relatively small and pinched and the skin over the cranial bones is tight and thin, revealing prominent distended veins The usual causes of this disorder are (1) intraventricular matrix hemorrhages in premature infants, (2) fetal and neonatal infections, (3) type II Chiari malformation, (4) aqueductal atresia and stenosis, and (5) the Dandy-Walker syndrome In this type of hydrocephalus, the head usually enlarges rapidly and soon surpasses the 97th percentile The anterior and posterior fontanels are tense even when the patient is in the upright position The infant is fretful, feeds poorly, and may vomit frequently With continued enlargement of the brain, torpor sets in and the infant appears languid, uninterested in his surroundings, and unable to sustain activity Later it is noticed that the upper eyelids are retracted and the eyes tend to turn down; there is paralysis of upward gaze, and the sclerae above the irises are visible This is the setting-sun sign and has been incorrectly attributed to downward pressure of the frontal lobes on the roofs of the orbits The fact that it disappears on shunting the lateral and third ventricles indicates that it is due to hydrocephalic pressure on the mesencephalic tegmentum Gradually the infant adopts a posture of exed arms and exed or extended legs Signs of corticospinal tract damage are usually elicitable Movements are feeble and sometimes the arms are tremulous There is no papilledema, but later the optic discs become pale and vision is reduced If the hydrocephalus becomes arrested, the infant or child is retarded but often surprisingly verbal The head may be so large that the child cannot hold it up and must remain in bed If the head is only moderately enlarged, the child may be able to sit but not stand or stand but not walk If ambulatory, the child is clumsy Acute exacerbations of hydrocephalus or an intercurrent febrile illness may cause vomiting, stupor, or coma
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The special features of congenital hydrocephalus associated with the Chiari malformation, aqueductal atresia and stenosis, and the Dandy-Walker syndrome are discussed in Chap 38 Occult Tension Hydrocephalus Here the hydrocephalus becomes evident only after the cranial sutures have closed (Fig 302) The causes of obstruction to the ow of CSF are diverse, and although some are clearly congenital, symptoms may be delayed as late as adolescence or early adult life or even later In some instances the condition gives rise to a normal-pressure hydrocephalus, as discussed below and in Chap 7 The clinical features of occult hydrocephalus and the course of the illness are quite variable Some instances of arrested hydrocephalus are truly occult in that the disease is unrecognized during life or is discovered only by chance imaging of the brain or on postmortem examination In other cases, the symptoms are intermittent and relatively mild; in still others, they are slowly or rapidly progressive, in the latter instance giving the clinical impression of a subacute disease The patient may complain of bifrontal or bioccipital headaches, but often these are not present Other symptoms and signs are predominantly those of a frontal lobe disorder of mentation or of gait Slowness of mental response (abulia), inattentiveness, distractibility, perseveration, and inability to plan activity or to sustain any type of complex cognitive function are characteristic The immediate responses to verbal and other stimuli are normal, though memory may be slightly impaired Conspicuous by their absence are apraxia, agnosia, or aphasia Gait deteriorates early in the course of hydrocephalus; such deterioration may be present for years before other symptoms become manifest The features of gait deterioration are hard to characterize, but the main aspects are discussed below, under Normal-Pressure Hydrocephalus We have seen a few cases in adults in which the gait disturbance appeared abruptly
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Figure 30-2 MRI of adult tension hydrocephalus from a congenital stenosis of the cerebral aqueduct There is transependymal movement of water that appears as a T2 signal rimming the lateral ventricles The third ventricle, but not the fourth, was enlarged
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