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1/12. Sudden unexpected death in young adults with chronic hydrocephalus.

    We present four cases of sudden unexpected death in young adults with chronic hydrocephalus. The patients were between 20 and 28 years of age and had suffered from aqueduct stenosis (two patients), spina bifida in combination with arnold-chiari malformation (type II) and fragile X-syndrome. The patients suddenly collapsed with cardiorespiratory failure and could not be resuscitated and none had a history of headache or seizures. The post-mortem examinations revealed no unusual findings and a definite cause of death could not be established. Neuropathological examination revealed chronically hydrocephalic brains without any signs of uncal or tonsillar herniation. We hypothesise that a sudden pressure-induced decompensation of cerebral neuronal pathways involving insular and limbic cortex, hypothalamus and brain stem nuclei, may have caused disturbances of the cardiopulmonary control centres in the reticular formation of the brain stem, which in turn may have led to instantaneous cardiorespiratory arrest resulting in sudden "neurogenic" cardiac death.
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2/12. Acute obstructive hydrocephalus associated with infratentorial subdural hygromas complicating Chiari malformation Type I decompression. Report of two cases and literature review.

    Obstructive hydrocephalus complicating foramen magnum decompression (FMD) for a Chiari malformation (CM) Type I is rare. Two female patients (17 and 55 years old) presented with strain-related headaches. In both cases magnetic resonance (MR) imaging studies confirmed a CM Type I, which was accompanied by syringomyelia in one case. Both patients underwent uncomplicated FMD with good initial recovery. Unfortunately, conditions in both patients deteriorated, with severe headaches occurring between Days 5 and 6 post-FMD. Decreased consciousness occurred in one case. In both patients, computerized tomography scanning demonstrated an acute obstructive hydrocephalus associated with bilateral infratentorial extraaxial fluid collections (EAFCs). In addition, left parafalcine and convexity EAFCs were present in one case. An emergency external ventricular drain was required in one patient, with delayed conversion to a ventriculo-peritoneal shunt. Spontaneous resolution occurred in the other patient without neurosurgical intervention. In both cases, MR imaging confirmed that each EAFC was subdural, resembled cerebrospinal fluid (CSF), and had distorted the superior cerebellum anteroinferiorly. Despite upper fourth ventricle/aqueduct compromise in one case, normal aqueduct flow artifacts were apparent on examination. All EAFCs resolved spontaneously. Obstructive hydrocephalus complicating FMD is rare but invariably associated with infratentorial EAFCs, which were confirmed to be subdural hygromas in this report. The authors assert that hydrocephalus results from upper fourth ventricle/aqueduct compromise as a result of CSF subdural dissection following a pinhole arachnoid tear on durotomy. Because such hygromas spontaneously resolve, permanent shunt insertion should be avoided.
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3/12. Resolution of trigeminal neuralgia following third ventriculostomy for hydrocephalus associated with Chiari I malformation: case report.

    OBJECTIVE AND IMPORTANCE: Cranial nerve dysfunction, including trigeminal neuralgia, has been associated with Chiari I malformations. In such cases, trigeminal neuralgia is thought to be related to tonsillar compression of the brainstem or to traction on the cranial nerves. hydrocephalus may be a contributing factor. CLINICAL PRESENTATION: A 38-year-old woman had right-sided lancinating facial pain typical of trigeminal neuralgia but was otherwise neurologically intact. magnetic resonance imaging showed no evidence of a compressing vessel. Moderate hydrocephalus and a Chiari I malformation were noted incidentally. The visibility of the aqueduct was poor. INTERVENTION: The patient underwent a third ventriculostomy and her symptoms resolved completely. CONCLUSION: This is the first case in which trigeminal neuralgia was treated with a third ventriculostomy and one of only four cases of isolated trigeminal neuralgia associated with a Chiari malformation. Acquired aqueductal stenosis may have caused the hydrocephalus which, in turn, caused the Chiari malformation configuration that caused the trigeminal neuralgia. The rationale for the treatment modality and possible causes of Chiari I-induced trigeminal neuralgia are discussed.
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4/12. Convergence nystagmus associated with arnold-chiari malformation.

    A case of convergence nystagmus associated with an Arnold-Chiari type I malformation is presented. The nystagmus appeared in the absence of fixation, was provoked during Valsalva's maneuver and neck flexion and extension, and attenuated on deep inspiration. Sagittal magnetic resonance images showed that the diameter of the cerebral aqueduct increased with the neck in full flexion and in full extension. Surgical foramen magnum decompression considerably reduced the nystagmus and abolished the postural variation of aqueduct diameter. It is postulated that this nystagmus was due to a combination of mechanical distortion and abnormal transmission of cerebrospinal fluid pressure to the aqueductal region.
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5/12. arnold-chiari malformation. Report of four cases with contamination of the central nervous system by amniotic contents.

    Four patients with the arnold-chiari malformation had massive prenatal contamination of the spinal cord and brainstem (and also of the cerebral ventricles in one case) by amniotic squamous cells and lanugo hair, which had entered through open meningomyeloceles. Amniotic contents were present in the subarachnoid space, central canal, fourth ventricle, and aqueduct and were also embedded in neural tissue, causing severe gliosis, fibrosis, and blockage. The mechanism of the complication and its implications in the prognosis of patients with the arnold-chiari malformation are discussed.
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6/12. Pyocephalus in a case of encephalocele and Arnold-Chiari anomaly: interactions between malformations and inflammation.

    This report deals with a 7-wk-old infant with an unusual tamponade empyema of the ventricular system associated with an occipital encephalocele and Arnold-Chiari anomaly. The infection extended from the ruptured encephalocele to the ventricular system. arnold-chiari malformation locked the infection within the ventricular system through herniation and occlusion of the ventricular foramina and prevented from spreading into subarachnoid space. The long survival of the infant allowed for the formation of pyocephalus. There was also an extensive coarctation of the ventricular walls and aqueductal occlusion which probably resulted from the infectious process in the early postnatal life.
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7/12. Cerebellocele and associated central nervous system anomalies in the Meckel syndrome.

    Three cases with the Meckel syndrome were autopsied and found to have: arhinencephaly , polymicrogyria , aqueductal stenosis, heterotopia of glial tissue, hypoplasia or agenesis of the cerebellar vermis, cranium bifidum associated with large occipital ventriculocele and others. The anomalies at the level of posterior fossa in this condition are classified as those belonging to the Chiari type III group of anomalies. This unusual set of anomalies which forms pathogenetic link between the Dandy-Walker and Chiari-Arnold group of anomalies in the posterior fossa seems to be very frequent in the Meckel syndrome. The therapeutic emphasis is on genetic counseling in view of the recessive inheritance of the syndrome.
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8/12. Dilated fourth ventricle in arnold-chiari malformation type II: isolated fourth ventricle as sequelae of shunt?--Case report.

    A female infant with arnold-chiari malformation type II developed cystic dilatation of the fourth ventricle at age 15 months. She received shunt emplacement into the dilated fourth ventricle to restore communication to the subarachnoid space and achieved improved symptoms and decreased ventricular size. Such dilatation is a typical feature of this malformation, and the isolation of the fourth ventricle is supposed to result from the cerebrospinal fluid shunting procedure. In this patient, the cause appeared to be external compression of the aqueduct by the beaking deformity of the mesencephalic spur, a characteristic of arnold-chiari malformation type II.
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9/12. 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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10/12. Pulsatile tinnitus associated with congenital central nervous system malformations.

    Pulsatile tinnitus as a manifestation of congenital central nervous system malformations has not been previously described. We present two patients with type I arnold-chiari malformation and one patient with congenital stenosis of the sylvian aqueduct with pulsatile tinnitus. Pulsatile tinnitus in these patients is believed to be secondary to increased intracranial pressure. tinnitus subsided in one patient with arnold-chiari malformation after suboccipital decompression. The other two patients declined surgery. Audiologic and radiologic findings are presented, and our diagnostic algorithm for pulsatile tinnitus is described. The pathophysiology of pulsatile tinnitus can be a manifesting symptom in some patients with congenital central nervous system malformations.
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