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1/5. A case of myoedema and normal pressure hydrocephalus.

    A cachectic 55-year-old man presented with ataxia and metabolic instability. On physical examination, he had prominent myoedema in all muscle groups. In addition, a CT scan and lumbar puncture showed extensive nonobstructive hydrocephalus. Further clinical evaluation revealed elevated creatine phosphokinase and liver enzyme levels, although the patient was euthyroid. The patient improved neurologically and metabolically with supportive therapy but the myoedema persisted. Previous cases have emphasized that myoedema is a localized, electrically silent, benign myopathic disorder of unknown cause. As with a previous case with ventricular enlargement, myoedema may be part of systemic pathology. Finally, as in most other reports, myoedema is a rare condition; only 3 cases (of 44) with palpable (but not visible) myoedema were uncovered in this study.
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2/5. Symptomatic congenital hydrocephalus in the elderly simulating normal pressure hydrocephalus.

    In a series of 30 older patients shunted for symptomatic hydrocephalus, we found 3 with a head circumference at or greater than the 98th percentile. In 2, we demonstrated deterioration over 6 and 12 months by serial videotaping of gait and neuropsychological testing. In the 3rd, serial lumbar punctures over a 6-month period gave temporary improvement. In each, CTs showed ventriculomegaly without transependymal flow. One patient had an Arnold-Chiari type I abnormality identified by MRI. All had systemic hypertension. CSF pressure monitoring showed CSF pressure greater than 15 mm Hg 39% of the time in 1 patient, and 100% in another. All improved with ventriculoperitoneal shunting. patients with probable compensated congenital hydrocephalus who functioned well throughout most of their lives may become symptomatic as they age but improve with shunt surgery. The head circumference should be measured in all older hydrocephalic patients.
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3/5. Normal pressure hydrocephalus. Recognition and relationship to neurological abnormalities in Cockayne's syndrome.

    Normal pressure hydrocephalus (NPH) in adults is a well-known cause of dementia. We describe NPH in children having the recessively inherited Cockayne's syndrome (CS). Cockayne's syndrome is characterized by cachectic dwarfism, neurological dysfunction, and cutaneous sunlight sensitivity. We noted that the NPH-associated triad of dementia, gait disturbance, and incontinence developed in CS patients. Computerized tomography of the brain in our four CS patients showed hydrocephalic enlargement of the brain ventricles greatest in the older patients. There was no evidence of cortical atrophy except in the one patient who had CS with xeroderma pigmentosum. Lumbar puncture and radionuclide cisternography in the two patients tested showed normal CSF pressure, with complete blockade to flow of radionuclide above the tentorium cerebelli, ventricular reflux, and delayed absorption. Studies of NPH in CS may elucidate the pathophysiology of NPH and methods to alter its sequelae.
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4/5. Case report: Wegener's granulomatosis accompanied by communicating hydrocephalus.

    A case of Wegener's granulomatosis (WG) accompanied by communicating hydrocephalus is described. An elderly woman with rapidly progressive renal failure was referred to the authors' hospital. Renal histologic study showed necrotizing granulomatous glomerulonephritis with some multinucleated giant cells, which suggested a diagnosis of WG. After admission, a gait disturbance, incontinence, and dementia developed in the patient. Diagnostic procedures including lumbar puncture, computed tomography (CT), and scintigraphy showed findings compatible with communicating hydrocephalus with a normal cerebrospinal fluid (CSF) pressure. Removal of 20 mL of CSF led to a marked improvement in symptoms. Because the presence of subarachnoid hemorrhage, meningitis, and brain tumor was excluded, the final diagnosis was communicating hydrocephalus secondary to WG.
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5/5. Abducens palsy following shunting for hydrocephalus.

    Over a period of 12 years, 80 patients underwent ventricular shunting for normal pressure hydrocephalus. Three developed sixth cranial nerve palsy in the first two weeks after surgery. This uncommon complication is usually transitory following the same pattern of abducens palsy after lumbar puncture or spinal anesthesia. traction on the nerve with local ischemia has been involved as the responsible mechanism in both instances.
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