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1/10. Late onset X-linked hydrocephalus with normal cerebrospinal fluid pressure.

    A family with X-linked hydrocephalus with normal cerebrospinal fluid (CSF) pressure and in which three brothers and a grandson of case 1, a proband, were affected is reported. The symptoms at onset were epileptic attacks that started in adulthood in the three brothers and at the age of 6 years in the grandson. In the three brothers, from 10 to 27 years after the onset of epileptic episodes, disorganization of intelligence and psychiatric deterioration were gradually noticed by their families. At the same time, they showed occasional urinary incontinence. Brain computed tomography (CT) scans revealed dilatation of the ventricular systems. Based on the results of the measurement of CSF pressure and radioactive-iodinated human serum albumin (RISA)-cysternography, two of the brothers were diagnosed as having normal pressure hydrocephalus (NPH), and they were treated neurosurgically. However, no obvious improvement in clinical symptoms was observed. Although the grandson had shown normal psychomotor development during his early childhood, temporal epilepsy and temper tantrums started at the age of 6 years. Computed tomography-scanning revealed dilatation of the ventricular system similar to the other three cases at the age of 8 years. With the diagnosis of NPH, the patient underwent a shunt operation, which resulted in no obvious effects. As it is reasonable to surmise that the pathological gene would have been transferred via the daughter of the proband to the grandson, it is suggested that the inheritance manner might be X-linked recessive. The cases presented here are different from the cases of hydrocephalus due to stenosis of the aqueduct Sylvius (HSAS) and other types of X-linked hydrocephalus reported previously in terms of the age of onset, course, symptoms, and CT findings. Thus, it is suggested that the present cases might be a new type of X-linked hydrocephalus.
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2/10. Unusual arachnoid cyst of the quadrigeminal cistern in an adult presenting with apneic spells and normal pressure hydrocephalus--case report.

    A 67-year-old woman was admitted to our clinic with symptoms of normal pressure hydrocephalus, lower cranial nerve pareses, and pyramidal and cerebellar signs associated with respiratory disturbances. Computed tomography (CT) and magnetic resonance imaging revealed a 4.7 x 5.4 cm quadrigeminal arachnoid cyst causing severe compression of the tectum and entire brain stem, aqueduct, and cerebellum, associated with moderate dilation of the third and lateral ventricles. Emergency surgery was undertaken due to sudden loss of consciousness and impaired breathing. The cyst was totally removed by midline suboccipital craniotomy in the prone position. Postoperatively, her symptoms improved except for the ataxia and impaired breathing. She was monitored cautiously for over 15 days. CT at discharge on the 18th postoperative day revealed decreased cyst size to 3.9 x 4.1 cm. Histological examination confirmed the diagnosis of the arachnoid cyst of the quadrigeminal cistern. The patient died of respiratory problems on the 5th day after discharge. Quadrigeminal arachnoid cysts may compress the brain stem and cause severe respiratory disturbances, which can be fatal due to apneic spells. patients should be monitored continuously in the preoperative and postoperative period until the restoration of autonomous ventilation is achieved.
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3/10. Failure of endoscopic third ventriculostomy in the treatment of idiopathic normal pressure hydrocephalus.

    Even though the main indication for neuroendoscopic management of normal pressure hydrocephalus (NPH) is the presence of an aqueductal block, recent reports suggest the possible efficacy of endoscopic third ventriculostomy (ETVS) in idiopathic NPH. We present 14 cases with apparently idiopathic NPH treated by ETVS, and report on the low rate of success (21 %). A closer analysis of the successful cases reveals possible elements which may explain the good outcome, and should be taken into consideration when defining the best strategy to address NPH.
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4/10. Fiberscopic fenestration and prophylactic stenting of periaqueductal cysts: technical report on three cases.

    The authors report on three consecutive cases of periaqueductal cysts, causing non-communicating hydrocephalus, successfully treated with endoscopic fenestration and aqueductal stenting. Navigation and cyst fenestration were accomplished using a slim (1.1 mm outside diameter) optic fiberscope inserted via a pre-coronal-paramedian burr hole. Third ventriculostomy was also performed after cyst fenestration in two cases. Because of the lack of data regarding such lesions and the possible recurrence with simple fenestration, an aqueductal stent connected to a subcutaneous reservoir was placed, in all 3 cases, under direct visualization. Average duration of the procedure was less than sixty minutes and there were no intra- or perioperative complications. All patients recovered clinically and their postoperative neuroimaging assessment confirmed a decrease in size of both the ventricular system and cyst (mean follow-up: 6.8 months). The authors conclude than this minimally invasive procedure is a promising, safe and effective method to treat cerebral symptomatic periaqueductal cysts and associated non-communicating hydrocephalus.
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5/10. Shoplifting associated with normal-pressure hydrocephalus: report of a case.

    A 52-year-old woman who had been apprehended for shoplifting was found to have normal-pressure hydrocephalus due to aqueduct stenosis. We ascribe the shoplifting to impairment of social judgment, which was one of the observed features of a dementia. Cerebrospinal fluid diversion by means of a ventriculo-peritoneal shunt resulted in resolution of her symptoms.
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6/10. Normal pressure hydrocephalus due to membranous obstruction of the sylvian aqueduct.

    In a patient presenting with the clinical features of normal pressure hydrocephalus, a membranous obstruction of the aqueduct of Sylvius was demonstrated by combined air encephalography and contrast ventriculography. Ventriculoatrial shunting resulted in clinical improvement without significant change in ventricular size.
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7/10. dementia, gait disturbance, incontinence and hydrocephalus.

    Normal pressure hydrocephalus is frequently emphasised as a cause of reversible dementia, but is seldom encountered. Over a 2 year period, 5 patients presented with cognitive decline, and gait disturbance, with or without incontinence. CT head examination revealed hydrocephalus. Although none had symptoms of raised intracranial pressure on initial presentation, there were clinical or CT signs of raised intracranial pressure in 4 of the 5. Underlying pathologies were meningeal lymphocytic lymphoma, idiopathic meningeal fibrosis, periaqueductal glioma, basilar aneurysm and basilar invagination. All patients responded to the insertion of a shunt. Over the same period, only 1 patient was shunted for idiopathic normal pressure hydrocephalus, without improvement. We challenge the concept of idiopathic normal pressure hydrocephalus as a cause of cognitive deterioration.
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8/10. Non-tumoural aqueduct stenosis and normal pressure hydrocephalus in the elderly.

    From 1981 to 1985 a prospective study on normal pressure hydrocephalus was performed. One of the aims of this study was to determine the site of CSF obstruction. Among 17 consecutive patients with a tentative diagnosis of normal pressure hydrocephalus, nine appeared to have non-communicating hydrocephalus most probably due to primary non-tumoural aqueduct stenosis. This unexpected finding provides evidence that non-tumoural aqueduct stenosis is a frequent cause of normal pressure hydrocephalus in older patients. Some clinical, aetiological and therapeutic aspects in this particular subgroup are discussed.
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ranking = 6
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9/10. Parkinsonian syndromes associated with hydrocephalus: case reports, a review of the literature, and pathophysiological hypotheses.

    We present nine cases of obstructive hydrocephalus (OH) associated with marked parkinsonism. Four patients had noncommunicating OH (NCOH) [three nontumoral aqueductal stenosis (AS), one tumoral AS]. The presentation was that of acute or subacute parkinsonism, usually at the time of acute recurrent ventricular obstruction. Three had a marked response to levodopa and required short-term treatment after shunting. However, one has remained levodopa dependent after 2 1/2 years. Three of the five patients with communicating OH (COH) presented with shunt-responsive normal pressure hydrocephalus (NPH), only later to develop progressive parkinsonism. One of these was found to have progressive supranuclear palsy (PSP) at autopsy and PSP was clinically suspected in one other patient. A third had an atypical course suggestive of PSP; however, autopsy demonstrated the combination of Lewy body parkinsonism and the sequelae of hydrocephalus. The remaining two COH patients presented with levodopa-responsive parkinsonism. Subsequent clinical features and imaging studies suggested the presence of NPH. The pathophysiology of hydrocephalic parkinsonism probably involves variable sites of dysfunction in the nigrostriatal pathway and/or the cortico-striato-pallido-thalamo-cortical circuit. At certain locations these pathways lie in close proximity to the ventricular system and may be subjected to mass effects and ischemic changes secondary to ventriculomegaly. The additional importance of possible associations between subcortical cerebral ischemia, NPH, and "degenerative" disorders such as PSP and Parkinson's disease is discussed.
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10/10. On the evaluation of cranio-cervical decompression: normalisation of the cerebral spinal fluid circulation in MRI?

    1) By means of "cine-MRI" a semi-quantitative analysis of CSF pulsations in the aqueduct of Sylvius can be carried out. 2) This investigation enables a qualitative evaluation of the cranio-spinal CSF flow phenomena. On the one hand this information is a valuable aid for neurosurgeons for diagnosis and on the other, cine-MRI progress controls allow an evaluation of the success of an operation in the sense of a quality control.
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