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MAJOR CATEGORIES OF NEUROLOGIC DISEASE
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Whether to shunt all hydrocephalic infants soon after birth is a controversial issue In several large series of cases that have been treated in this way, the number surviving with normal mental function has been small (see review of Leech and Brumback) The series of Dennis and associates is representative They examined 78 shunted hydrocephalic children and found that 56 (72 percent) had full-scale IQs between 70 and 100; in 22 patients, the IQ was between 100 and 115; in 3 patients, it was below 70, and in 3 others, it was above 115 Mental functions improved unevenly and performance scores lagged behind verbal ones at all levels The use of the carbonic anhydrase inhibitor acetazolamide or other diuretics to inhibit CSF formation has not been successful in the hands of our colleagues, but several authors believe that by giving 250 to 500 mg of acetazolamide orally daily, shunting can be avoided in both in adult normal-pressure and infantile hydrocephalus (Aimard et al; Shinnar et al)
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Table 30-1 Causes and pathogenetic associations of pseudotumor cerebri I Idiopathic ( benign ) intracranial hypertension II Cerebral venous hypertension (diagnosis by imaging of cerebral vasculature) A Occlusion of superior sagittal or lateral venous sinus: 1 Hypercoagulable states (cancer, birth control pills, dehydration, antiphospholipid antibody, etc) 2 Traumatic 3 Postsurgical 4 Infectious (mainly of transverse venous sinus due to mastoiditis) B Increased blood volume due to high- ow arteriovenous malformation, dural stulas, and other vascular anomalies III Meningeal diseases (diagnosis by examination of CSF) A Carcinomatous and lymphomatous meningitis B Chronic infectious and granulomatous meningitis (fungal, tuberculous, spirochetal, sarcoidosis, etc) IV Gliomatosis cerebri V Toxic A Hypervitaminosis A (especially isotretinoin, used for the treatment of acne) B Lead C Tetracycline D As an infrequent idiosyncratic effect of various drugs (amiodarone, quinolone antibiotics, estrogen, phenothiazines, etc) VI Metabolic disturbances A Administration or withdrawal of corticosteroids B Hyper- and hypoadrenalism C Myxedema D Hypoparathyroidism VII Associated with greatly elevated protein concentration in the CSF A Guillain-Barre syndrome B Spinal oligodendroglioma C Systemic lupus erythematosus
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Occlusion of the major dural venous sinuses (superior longitudinal and lateral) results in increased ICP This is not surprising in view of the direct effect of venous obstruction on CSF pressure One such form, due to lateral sinus thrombosis, was referred to by Symonds as otitic hydrocephalus a name that he later conceded was inappropriate insofar as the ventricles are not enlarged in this circumstance As indicated earlier, venous congestion that complicates heart failure and superior mediastinal obstruction also raise the CSF pressure, again without enlargement of the ventricles This may happen as well with large, high- ow arteriovenous malformations of the brain The effects of cerebral venous occlusion are considered further in the discussion of pseudotumor cerebri (below) and in Chap 34
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Pseudotumor Cerebri
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The term pseudotumor cerebri was coined by Nonne in 1914 and has remained a useful means of designating a common and highly characteristic syndrome of headache, papilledema (unilateral or bilateral), minimal or absent focal neurologic signs, and normal CSF composition, all occurring in the absence of enlarged ventricles or an intracranial mass on CT scanning or MRI Being a syndrome and not a disease, pseudotumor cerebri has a number of causes or pathogenetic associations (Table 30-1) Actually, the most common form of the syndrome has no rmly established cause ie, it is idiopathic and is now generally referred to as benign or idiopathic intracranial hypertension Idiopathic Intracranial Hypertension This syndrome was rst described in 1897 by Quincke, who called it serous meningitis It is particularly frequent in overweight adolescent girls and young women, attaining an incidence of 19 to 21 per 100,000 in this group, as compared with 1 to 2 per 100,000 in the general population (Radhakrishnan et al) Increased ICP develops over a period of weeks or months Relatively unremitting but uctuating headache, described as dull or a feeling of pressure, is the cardinal symptom; it can be mainly occipital, generalized, or somewhat asymmetrical Other less frequent complaints are blurred vision, a vague dizziness, minimal horizontal diplopia, transient visual obscurations that often coincide with the peak intensity of the headache, or a tri ing numbness of the face on one side Rarely, the
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presenting feature may be a nasal CSF leak, as pointed out by Clarke and colleagues Self-audible bruits have been reported by some of our patients; this has been attributed to turbulence created by differences in pressure between the cranial and jugular veins The patient is then discovered to have agrant papilledema (Figs 13-8 and 13-9), immediately raising the specter of a brain tumor (rarely, papilledema is only minimally developed or absent) The risk of visual loss and the severity of headache in many instances make the term benign intracranial hypertension less acceptable The CSF pressure is found to be elevated, usually in the range of 250 to 450 mmH2O, but it is not clear whether the brain itself is swollen or, as is more likely, the increased pressure is due entirely to a change in the pressure within the CSF and venous compartments When the CSF pressure is monitored for many hours, there are uctuations taking the form of irregularly occurring plateau waves of increased pressure lasting 20 to 30 min and then falling abruptly nearly to normal (Johnston and Paterson) Aside from papilledema, there is remarkably little to be found on neurologic examination perhaps a slight unilateral or bilateral abducens palsy, ne uninterpretable nystagmus on far lateral gaze,
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