11/48. Recombinant activated factor vii for cerebral injury-induced coagulopathy in pediatric patients. Report of three cases and review of the literature.brain injury remains one of the leading causes of death and disability in children. Appropriate therapy involves aggressive management of intracranial pressure (ICP) and cerebral perfusion pressure, which often requires placement of an intraparenchymal ICP monitor or intraventricular catheter. These potentially life-saving interventions require normal coagulation function; however, several factors may lead to coagulopathy in the head-injured patient. Standard therapies, which often include multiple doses of fresh frozen plasma (FFP), have a number of drawbacks when used in the pediatric population. The use of FFP requires time to type and crossmatch, thaw, and administer. It imposes a significant volume load on a child in whom cerebral edema remains a problem. Success in using recombinant activated factor vii (rFVIIa) in the hemophiliac population suggests an alternative therapy. Three patients suffered severe coagulopathy after cerebral injury. One patient received rFVIIa after repeated doses of FFP had failed to correct the coagulopathy; the other two patients received rFVIIa as the initial therapy. Treatment with rFVIIa consisted of a bolus of 90 microg/kg. Recombinant activated factor vii rapidly corrected the patients' coagulopathies, which allowed placement of intraparenchymal fiberoptic lines and intraventricular catheters to monitor ICP. The patients suffered no complication from the placement of ICP monitoring devices, as demonstrated on computerized tomography scans obtained within 24 hours after placement. brain injury-induced coagulopathy may lead to significant secondary injury and delays the invasive monitoring necessary for the aggressive management of intracranial hypertension. Fresh frozen plasma takes time to administer. may require repeated doses of significant volume for the pediatric patient, and may ultimately fail. Preliminary data indicated that rFVIIa provides a rapid and successful correction of coagulopathy in the head-injured patient.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
12/48. FKRP gene mutations cause congenital muscular dystrophy, mental retardation, and cerebellar cysts.BACKGROUND: Congenital muscular dystrophies (CMD) are autosomal recessive disorders that present within the first 6 months of life with hypotonia and a dystrophic muscle biopsy. CNS involvement is present in some forms. The fukutin-related protein gene (FKRP) is mutated in a severe form of CMD (MDC1C) and a milder limb girdle dystrophy (LGMD2I). Both forms have secondary deficiencies of laminin alpha2 and alpha-dystroglycan immunostaining. Structural brain involvement has not been observed in patients with FKRP gene mutations. methods: The authors studied two unrelated patients who had a pattern of muscle involvement identical to MDC1C, mental retardation, and cerebellar cysts on cranial MRI. The FKRP gene was analyzed along with the skeletal muscle expression of laminin alpha2 and alpha-dystroglycan. RESULTS: The muscle biopsy of both patients showed severe dystrophic findings, a reduction in laminin alpha2, and profound depletion of alpha-dystroglycan. Both patients had homozygous FKRP gene mutations not previously reported (C663A [Ser221Arg] and C981A [Pro315Thr]). CONCLUSIONS: Mutations within the FKRP gene can result in CMD associated with mental retardation and cerebellar cysts. This adds structural brain defects to the already wide spectrum of abnormalities caused by FKRP mutations. The severe depletion of alpha-dystroglycan expression suggests that FKRP is involved in the processing of alpha-dystroglycan.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
13/48. MR evaluation of crossed and uncrossed cerebral-cerebellar diaschisis.In three patients with infantile hemiplegia syndrome, MR imaging done later in life showed significant volume loss in the cerebellar hemisphere contralateral to the side of the affected cerebrum in two and ipsilateral in one. By comparison, the cerebellar volume loss seemed to correlate with the degree of volume loss in the contralateral cerebral hemispheres in two patients. These observations provide morphological evidence of the phenomenon of crossed and uncrossed/ipsilateral cerebral cerebellar diaschisis (CCD and ICD). functional neuroimaging studies in support of the concept of CCD and ICD have been critically reviewed in the light of the morphological changes demonstrated in the cases cited herein.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
14/48. Subdural empyema and cerebellar abscess due to chronic otitis media.The infratentorial variety of the subdural empyema, with or without coexisting cerebellar abscess, is a rare clinical entity that carries a high mortality rate. We briefly describe the case of a 49-year-old man presented with severe debility, fever and an obviously neglected chronic otitis media. The patient had refused surgical treatment several months ago. After admission, his level of consciousness began to deteriorate, and the radiological studies showed infratentorial subdural suppuration extending into the right cerebellar hemisphere, along with chronic pyogenic infection of the middle ear and the mastoid process. Radical mastoidectomy was performed first, followed by extensive right posterior fossa craniectomy. The two subdural collections and the cerebellar abscess were successfully evacuated. Subsequently, he received post-operative antibiotic treatment for 6 weeks. At follow-up, 10 months after surgery, his neurological recovery was complete except for a minor residual cerebellar dysfunction on the right. This unusual case highlights that in patients presented with severe intracranial complications of chronic otitis media, early diagnosis and radical surgical intervention may be life saving.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
15/48. Successful cardic surgery 24 hours after craniotomy in a patient with infective endocarditis and embolic cerebellar infarction: case report.Follow up management in a patient already treated with decompressive craniotomy for a space-occupying endocarditic stroke is difficult. While immediate valve replacement eliminates the focus and therefore the high risk of re-embolization, a neurosurgical intervention is considered a contraindication to early cardiosurgery. Herein, the first report is presented of a critically ill patient with bacterial endocarditis and a space-occupying cerebellar infarction with imminent herniation, who successfully underwent mitral valve replacement only 24 h after decompressive craniotomy. To prevent rebleeding, maximal hemostasis was ensured during the neurosurgical intervention. For cardiosurgery, the patient was cooled to 21 degrees C, mildly hyperventilated, and maintained at an adequate perfusion pressure during cardiopulmonary bypass. A bioprosthesis was used to reduce the time of anticoagulation. The patient did not develop new infarcts after either intervention, and there was only a very small hemorrhagic transformation without a relevant mass effect. At five months after surgery the patient had minimal neurological abnormalities and was able to conduct his daily life without help.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
16/48. Progressive cerebellar tonsillar herniation with recurrent divergence insufficiency esotropia.The Chiari malformations are characterized by herniation of posterior fossa contents through the foramen magnum. Chiari I malformation is currently defined as ectopia of the cerebellar tonsils more than 5 mm below the foramen magnum. Extension of the cerebellar tonsils up to 3 mm may be found in the normal population. Although Chiari malformations are congenital, symptoms often do not manifest until the third and fourth decades of life, or even later. patients usually present with headache, lower cranial nerve palsies, downbeat nystagmus, ataxia, or dissociated anesthesia of the trunk and extremities. Definitive diagnosis is made by magnetic resonance imaging (MRI), which shows the compressed tonsils extending through the foramen magnum into the cervical subarachnoid space. One of the rare presenting signs of Chiari I malformations is acquired esotropia with a divergence insufficiency pattern. We report such a case in which the initial neuroimaging showed tonsillar herniation, but of insufficient magnitude to meet diagnostic criteria for Chiari I malformation. When the strabismus recurred after initially successful eye muscle surgery, follow-up scan showed progressive tonsillar herniation.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
17/48. Distal aneurysms of the superior cerebellar artery and posterior inferior cerebellar artery feeding an associated arteriovenous malformation: case report.The authors report a case of two distal aneurysms of the cerebellar arteries, one arising from the vermian branch of the posterior inferior cerebellar artery, the other arising from the hemispheric branch of the superior cerebellar artery, and both feeding an associated arteriovenous malformation (AVM). The aneurysm of the distal posterior inferior cerebellar artery was considered the source of a cerebellar hemorrhage because of the location of a hematoma in the cerebellar vermis. The life-threatening hematoma was evacuated in an emergency operation 6 hours after the acute onset of symptoms. The cerebellar aneurysms and the AVM were clipped or extirpated successfully after the patient's condition improved. The association of two rare types of aneurysms with an AVM strongly supports the theory that increased hemodynamic stress derived from the AVM plays an important role in aneurysm formation. The authors think that one should operate on the symptomatic lesion first or both the aneurysm and the AVM in the same operative procedure.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
18/48. Acute cerebellitis complicated by hydrocephalus and impending cerebral herniation.We present a 7-year-old boy with acute cerebellitis who required an emergency ventriculoperitoneal shunt for hydrocephalus caused by cerebellar swelling. This represents a very unusual, potentially life-threatening complication of a usually self-limiting condition. early diagnosis of this complication is essential in view of the propensity to sudden and fatal deterioration. magnetic resonance imaging (MRI) is useful in differentiating this unusual course of acute cerebellar ataxia from that of a posterior fossa tumor. In developing countries, however, computed tomography (CT) is often the only existing diagnostic modality, and access to MRI, when available, is limited. Our case demonstrates that the shape of the fourth ventricle on CT can be helpful in differentiating between a tumor and edema of the cerebellum and thus can assist in management.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
19/48. Cerebellar infarction in the young.BACKGROUND AND PURPOSE: Ischemic cerebrovascular disease in children and young adults usually affects the anterior circulation. SUMMARY OF REPORT: We describe two cases of cerebellar infarction in the territory of vertebral artery supply, associated with physical exertion, in a young adult and in a child. review of 31 previous cases of cerebellar infarction occurring in the first 2 decades of life demonstrated a mostly obscure causation; where a likely cause was found, trauma was most frequent. In 12 of the 31 patients, a vertebral artery (usually the left) was occluded. patients were sometimes predisposed to such occlusions by subluxation between the first and second cervical vertebrae, allowing abnormal neck movements that can cause arterial injury and thromboembolism. Some of these cerebellar infarcts, like those of our patients, have followed physical exertion. CONCLUSIONS: Cerebellar infarction can be life-threatening, but half of the patients, including ours, have had complete or near-complete recovery.- - - - - - - - - - ranking = 2keywords = life (Clic here for more details about this article) |
20/48. Management of acute cerebellar stroke.Acute cerebellar infarction or hemorrhage may initially manifest in a clinically indolent manner only to later deteriorate into a life-threatening neurologic catastrophe. At the other end of the spectrum, some patients with cerebellar stroke may present in a moribund comatose state. In both patient groups, it is often unclear at what point surgical intervention should be considered either to prevent further neurologic deterioration or to try to salvage a meaningful neurologic recovery. In this review, we present clinical cases that illustrate decision points in the management of patients with acute cerebellar stroke, with emphasis on clinical and imaging characteristics. We conclude with an analysis of clinical decision making in the management of patients with space-occupying cerebellar stroke. The management of acute cerebellar infarction or hemorrhage often requires difficult and prompt decisions by treating neurologists, and certain easily identifiable clinical and imaging findings may assist in appropriate patient triage and timely neurosurgical intervention.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
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