1/54. Pseudo Chiari type I malformation secondary to cerebrospinal fluid leakage.Cerebrospinal fluid (CSF) leakage may occur spontaneously, iatrogenically or from spinal trauma. Postural headache is the cardinal symptom; dizziness, diminished hearing, nausea and vomiting are additional symptoms. In neurological examinations cranial nerve palsies may be found. Due to low CSF pressure neuroimaging studies may reveal dural enhancement and vertical displacement of the brain. We describe a patient with the history of an uncomplicated lumbar discectomy at the level L4-5 and the typical clinical symptoms of intracranial hypotension. MRI of the craniocervical junction showed typical features of a Chiari type-I malformation. After neurosurgical ligation of a CSF leak at L4-5 caused by lumbar disc surgery, the patient was free of orthostatic headache. A repeated MRI showed a striking reduction of the previous downward displacement of the cerebellar tonsils and pons.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
2/54. Brainstem dysfunction in chiari malformation presenting as profound hypoglycemia: presentation of four cases, review of the literature, and conjecture as to mechanism.OBJECTIVE: We report four patients whose cases resulted in our observation that profound hypoglycemia resulting from intermittent hyperinsulinism plays a significant role in patients with brainstem dysfunction from Chiari I or II malformations who have intermittent autonomic dysfunction ("blue spells"). methods: The records of four children with severe brainstem dysfunction associated with hindbrain herniation (Chiari I or II malformation) were reviewed retrospectively. Each patient had severe lower cranial nerve dysfunction that required tracheotomy and feeding tube placement. After we found that profound hypoglycemia had occurred during a spell of autonomic dysfunction in one patient, the charts of the other three patients were reviewed for evidence of hypoglycemia. Now, whenever one of them has evidence of autonomic dysfunction, prospective studies of glucose and insulin levels are performed. Three of the patients had Chiari II malformation in association with myelomeningocele, and one patient had a Chiari I malformation resulting from Pfeiffer's syndrome. RESULTS: hypoglycemia occurred in these patients episodically, and usually when their shunts were functioning. The hypoglycemia was associated with hyperinsulinemia in each patient. The brainstem structures of these children (presumably the dorsal motor nuclei of the vagus) were extremely sensitive to changes in local or regional intracranial pressure. These changes were triggered by intermittent shunt failure, agitation from pain, abdominal distention from constipation, and retention of CO2. In patients with Chiari malformations, even mild increases in intracranial pressure lead to brainstem dysfunction. One possible explanation is that pressure on the deformed Xth cranial nerve nuclei may lead to insulin release and life-threatening hypoglycemia. Continuous-drip feeds are necessary to prevent this complication. CONCLUSION: patients with severe intermittent brainstem dysfunction after decompression of Chiari I or Chiari II malformations should have laboratory studies of glucose levels performed at the time of the episodes to rule out hypoglycemia.- - - - - - - - - - ranking = 3keywords = pressure (Clic here for more details about this article) |
3/54. Antley-Bixler syndrome, description of two new cases and review of the literature.OBJECTIVE: Antley-Bixler syndrome (ABS) is a rare disorder characterized by multiple malformations of cartilage and bone including multisynostotic osteodysgenesis, midface hypoplasia, choanal atresia or stenosis, femoral bowing, neonatal fractures and multiple joint contractures and, occasionally, urogenital, gastrointestinal or cardiac defects. Since the first report in 1975, at least 34 cases (including this report) have been described. We present 2 cases of congenital hydrocephalus, suggesting the cause of craniosynostosis and midface hypoplasia is associated with ABS. CLINICAL PRESENTATION: The first case was a 1-day-old female with arnold-chiari malformation, multiple cranial synostosis, obstructive hydrocephalus and radioulnahumeral synostosis. Ventriculoperitoneal (V-P) shunting was performed when she was 7 days old. She died 42 days later due to cardiopulmonary failure. The second case was a 2-month-old female with bilateral coronal synostosis, obstructive hydrocephalus and brachycephaly. V-P shunting was done soon after her admission, and bilateral lateral canthal advancement with the floating forehead procedure was performed 1 month later. She is still alive with good development. DISCUSSION: The main anomalies of the ABS can be divided into 4 categories: craniofacial, skeletal, extremity and urogenital anomalies. They may be the result of mutation in the fibroblast growth factor receptor 2 (Ser351Cys) gene, which was confirmed in our case 2. Craniosynostosis combined with hydrocephalus created congenital increased intracranial pressure (IICP). Early V-P shunt implantation and surgical release of the closed suture and lateral canthal advancement should be done as soon as possible, ideally when the patient is younger than 3 months. CONCLUSION: Early correction of craniosynostosis was feasible and safe for one of our patients. We offer our experience in the treatment of hydrocephalus and correction of craniosynostosis to relieve IICP.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
4/54. Orofacial pain as the sole manifestation of syringobulbia-syringomyelia associated with arnold-chiari malformation.This is a case report of a male patient who presented with orofacial pain for a year as the only manifestation of syringobulbia-syringomyelia associated with arnold-chiari malformation. This article places emphasis on the clinical presentation and possible differential diagnoses. The pain was continuous and affected the left side of the face. It was exacerbated by coughing and physical effort, possibly as a consequence of an increase in intracranial pressure. Paroxysmal pain crises developed over this background of continuous pain, compatible with neurogenic trigeminal pain of the left second branch, together with pain episodes similar to cluster headache on the same side. The symptoms were resolved following neurosurgical management with amplification of the foramen magnum.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
5/54. When does low mean high? Isolated cerebral ventricular increased intracranial pressure in a patient with a Chiari I malformation.OBJECTIVE: To present an unusual case of pseudotumor cerebri with increased intracranial pressure isolated to the cerebral ventricles resulting from a Chiari I malformation. MATERIALS AND methods: The patient received a complete ophthalmologic examination on initial presentation and subsequent visits, including visual acuity, pupillary examination, intraocular pressures, dilated fundus examination with assessment of degree of papilledema, and visual field testing. intracranial pressure was measured by lumbar puncture and subsequently by intracranial pressure bolt monitoring. magnetic resonance imaging (MRI) was used to diagnose the Chiari I malformation. RESULTS: The patient initially presented with bitemporal headaches, elevated opening pressure on lumbar puncture, and mild papilledema with a normal MRI. After lumboperitoneal shunt placement and several revisions, the patient presented with decreased vision OD secondary to Terson syndrome and worsening papilledema. Subsequent evaluation revealed normal lumbar opening pressures and a Chiari I malformation. She underwent ventriculoperitoneal shunt placement with resolution of her symptoms. CONCLUSIONS: Tonsillar herniation is a well-documented complication of lumboperitoneal shunt revision. Obstruction of cerebrospinal flow through the foramina of Magendie and Luschka can result in increased intracranial pressure isolated to the cerebral ventricles. In a patient with signs and symptoms of increased intracranial pressure but normal lumbar opening pressure, a Chiari I malformation should be suspected, particularly with a history of multiple lumboperitoneal shunt revisions.- - - - - - - - - - ranking = 13keywords = pressure (Clic here for more details about this article) |
6/54. 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.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
7/54. Pseudotumour cerebri occurring in association with the Chiari malformation.Summary The case is described of a 12-year-old girl presenting with raised intracranial pressure without ventricular dilatation and a type 1 Chiari malformation. This was taken to be a coincidental association of pseudotumour cerebri and the Chiari malformation. Treatment of the pseudotumour with Diamox gave rapid and sustained relief of the intracranial hypertension without change in the Chiari malformation. In addition, a series of 156 cases of pseudotumour cerebri was reviewed for evidence of the Chiari malf ormation. An overall incidence of 1.3%, rising to 2.7% in patients with MR scanning [excluding the case described] was found.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
8/54. Resolution of papilledema after neurosurgical decompression for primary Chiari I malformation.PURPOSE: To report a causal relationship between Chiari I malformation and its rare, but recognized manifestation of bilateral papilledema. DESIGN: Interventional case series. methods: Four adult female patients (mean age, 48, age range 25-59 years) with bilateral papilledema, signs and symptoms of increased intracranial pressure, and cranial magnetic resonance imaging (MRI) evidence of a Chiari I malformation ranging from 7 to 22 mm of tonsillar herniation underwent suboccipital decompression. RESULTS: In all four patients, suboccipital decompression was followed by resolution of bilateral papilledema and signs and symptoms of increased intracranial pressure. CONCLUSION: patients with bilateral papilledema and presumed pseudotumor cerebri require a cranial MRI to determine if they have a Chiari I malformation, because patients with increased intracranial pressure and papilledema from a Chiari I malformation may benefit from suboccipital decompression.- - - - - - - - - - ranking = 3keywords = pressure (Clic here for more details about this article) |
9/54. Regression of symptomatic syringomyelia after resection of posterior fossa tumour.syringomyelia associated with posterior fossa tumours is a very infrequent combination of pathological entities. The few cases which have been reported generally were asymptomatic in respect of the spinal cavitations. The authors report on a 36-year-old woman with a large extradural posterior fossa epidermoid tumour with a concomitant holocord symptomatic syringomyelia. Some of her symptoms were clearly attributed to the intraspinal cavitation. The lesions were both diagnosed by magnetic resonance imaging (MR). The patient did well after surgery of the brain lesion, with an objective improvement in her neurological status and a complete resolution of the syrinx documented by the MR 7 months after tumour removal.syringomyelia in this case could be explained by blockage of the cerebrospinal fluid (CSF) circulation at the foramen magnum which in turn resulted in cranio-spinal pressure dissociation. This led to an accumulation of extracellular fluid (ECF) in the central canal, starting cavitation. Consequently, the syrinx was slowly expanded by the long-standing "slosh" effect of the systolic pressure waves. However, also via a distortion mechanism within the posterior fossa a pathologically plugged obex could have contributed to syrinx formation by means of preventing drainage of fluid from the ventricular CSF system.- - - - - - - - - - ranking = 2keywords = pressure (Clic here for more details about this article) |
10/54. Resolution of Chiari malformation after repair of a congenital thoracic meningocele: case report and literature review.OBJECTIVE AND IMPORTANCE: Many theories have been proposed regarding potential causative factors for Chiari malformations. An unusual case is described in which regression of a congenital Chiari malformation was observed after repair of a thoracic meningocele without direct surgical intervention to decompress the craniocervical junction. This supports the importance of an in utero craniospinal pressure gradient as a potential cause for congenital, but reversible, cerebellar herniation. CLINICAL PRESENTATION: A newborn baby was observed to have a thoracic meningocele. magnetic resonance imaging scan revealed a concomitant Chiari malformation. No neurological deficits were present at initial examination. INTERVENTION: The patient underwent surgical closure of the thoracic meningocele and untethering of the spinal cord at the site of the dural defect. A postoperative magnetic resonance imaging scan obtained 3 months after the operation revealed complete resolution of the cerebellar herniation. CONCLUSION: The resolution of the Chiari malformation in this child may have resulted from restoration of normal cerebrospinal fluid flow and elimination of the meningocele-related cerebrospinal fluid pressure gradient between the intracranial and intraspinal compartments.- - - - - - - - - - ranking = 2keywords = pressure (Clic here for more details about this article) |
| | Next -> |