Cases reported "Cerebellar Diseases"

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1/34. Hyperbaric chamber-related decompression illness in a patient with asymptomatic pulmonary sarcoidosis.

    An asymptomatic 46-yr-old male sustained an acute neurologic insult, appearing during the decompression phase of a 50-m dry hyperbaric chamber dive. The right hemisyndrome was most probably related to diving, since symptoms responded rapidly to the early commenced recompression therapy. Further diagnostics revealed a previously unknown pulmonary sarcoidosis with bilateral pulmonary opacities and pleural adhesions that might have predisposed to arterial gas embolism secondary to pulmonary barotrauma. This case may illustrate a potential risk of decompression illness even during dry chamber dives in patients suffering from asymptomatic pleuro-parenchymal pulmonary disease. The value of chest X-ray in the medical assessment of fitness to dive is therefore emphasized.
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2/34. Postoperative brainstem high intensity is correlated with poor outcomes for patients with spontaneous cerebellar hemorrhage.

    OBJECTIVE: The outcomes for patients with cerebellar hemorrhage are thought to be influenced by anatomic damage to the brainstem. In this study, we investigated the magnetic resonance imaging findings in the brainstem, to examine the relationship between the degree of brainstem damage and the outcomes for patients with spontaneous cerebellar hemorrhage who are in poor-grade condition. methods: The results for 31 patients with spontaneous cerebellar hemorrhage, with Glasgow coma Scale scores of 8 or less at admission, who underwent magnetic resonance imaging examinations were reviewed. All patients underwent surgical intervention. The patients were divided into two groups according to their glasgow outcome scale scores at the time of discharge, i.e., patients who experienced good recoveries or exhibited moderate disabilities (Group I, n = 8) and patients who exhibited severe disabilities, were in a persistent vegetative state, or had died (Group II, n = 23). We investigated obliteration of the fourth ventricle and the perimesencephalic cistern and the presence of hydrocephalus in initial computed tomographic scans and the presence of areas of high signal intensity in the brainstem in T2-weighted images. RESULTS: Eight patients experienced good outcomes, and 23 patients experienced poor outcomes. The overall mortality rate was 32.3%. There were no significant differences between groups with respect to computed tomographic findings such as hematoma size, but the incidence of high signal intensities in the pons and midbrain in T2-weighted images for Group II was significantly higher than that for Group I (P < 0.01). CONCLUSION: magnetic resonance imaging clearly demonstrated brainstem damage, and high signal intensity in the brainstem was a significant prognostic factor for determining outcomes for patients with spontaneous cerebellar hemorrhage who were in poor-grade condition.
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3/34. A case of sarcoidosis presenting as multiple pulmonary nodules, nasopharyngeal and cerebellopontine tumors.

    A 64-year-old woman presented with multiple pulmonary nodules, and after spontaneous regression of the pulmonary lesions in six months, nasopharyngeal and right cerebellopontine tumors developed. Noncaseous epithelioid cell granulomas were demonstrated histologically in both resected pulmonary and nasopharyngeal tumors. She complained of hearing loss and tinnitus probably due to the cerebellopontine tumors. Corticosteroid therapy resulted in the improvement of these symptoms and the gradual decrease of tumor size. Although histological probe of the cerebellopontine tumors was not diagnostic, this patient was finally diagnosed as having sarcoidosis, based on the clinicopathological features, including systemic granulomatous lesions, MRI findings, and good response to corticosteroid therapy. The diagnosis of sarcoidosis is sometimes difficult when its clinical manifestations are uncommon.
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4/34. MR diagnosis of cerebellar infarction due to vertebral artery dissection in children.

    Posterior circulation infarction is uncommon in children. We describe the clinical presentation and radiological findings in two children with cerebellar infarction resulting from dissection of the vertebral artery. We emphasize that vertebral artery injury should be considered in a child with acute symptoms and signs of ischaemia in the posterior circulation. MRI and MRA may be helpful in the diagnosis of cerebellar infarction and vertebral artery abnormality.
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5/34. A craniocervical injury-induced syringomyelia caused by central canal dilation secondary to acquired tonsillar herniation. Case report.

    The authors report on a 19-year-old man with an acquired tonsillar herniation caused by a craniocervical junction injury in which serial magnetic resonance (MR) images demonstrated patent and isolated segments of the central canal participating in the dilation and then formation of a cervical syrinx. The patient was involved in a motor vehicle accident; he developed tonsillar herniation as a complication of subarachnoid and epidural hemorrhage, predominantly observed around the cisterna magna and upper cervical canal. Repeated MR images obtained over an 11-month period indicated the for mation and acute enlargement of the syrinx. Ten months after the accident, the patient presented with sensory disturbance in both upper extremities and spasticity due to syringomyelia. He underwent craniocervical decompressive surgery and doraplasty, which reduced the size of syringomyelia. The authors postulate that the patent central canal may play a role in determining the location of a syrinx remote from a focus of cerebrospinal fluid obstruction.
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6/34. Supratentorial hydatid cyst with cerebellar signs: a rare case of diaschisis.

    BACKGROUND: Intracranial hydatid disease has a distinct predominance in the pediatric age group and still causes serious problems in endemic areas. CASE REPORT: A 7-year-old girl admitted with a 3-month history of illness involving the main symptoms of ataxic gait, apraxia, headache, and tremor and with positive cerebellar signs and papilledema is presented. RESULTS: Cranial computerized tomography and magnetic resonance imaging revealed a right temporoparietal spherical lesion measuring 50 x 60 x 80 mm, which had a significant mass effect. A preoperative diagnosis of intracranial hydatid cyst was confirmed during the surgical procedure, which allowed removal of the cyst intact. The postoperative course was uneventful. CONCLUSIONS: The aim of this presentation is to emphasize the necessity for considering diaschisis--inhibition of function produced by a focal disturbance in a portion of the brain at some distance from the original site of injury, but anatomically connected with it through fiber tracts. We believe that this case, with a mass lesion in a temporoparietal location and definite clinical manifestations with plentiful cerebellar signs, is a good example of this rare phenomenon.
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7/34. Neurenteric cyst in the cerebellopontine angle with xanthogranulomatous changes: serial MR findings with pathologic correlation.

    We report serial MR findings in a middle-aged woman with a neurenteric cyst involving the cerebellopontine angle cistern with xanthogranulomatous changes. On the initial gadolinium-enhanced T1-weighted MR images, the solid portion of the lesion had homogeneously strong enhancement. Follow-up MR images obtained 6 months later showed that the mass had increased in size; however, the solid portion decreased in size. The enhancing solid portion corresponded to the xanthogranulomatous changes on pathologic correlation.
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8/34. Distal anterior inferior cerebellar artery syndrome after acoustic neuroma surgery.

    OBJECTIVE: To define a clinicopathologic syndrome associated with persistent cerebellar dysfunction after acoustic neuroma (AN) excision. STUDY DESIGN: Case series derived from radiographic and clinical chart review. SETTING: Tertiary referral center. patients: In 12 patients with AN, persistent cerebellar dysfunction developed after AN removal. Each case demonstrated abnormality in the ipsilateral cerebellar peduncle on postoperative magnetic resonance imaging. MAIN OUTCOME MEASURES: Cerebellar function and ambulatory status over the first postoperative year. RESULTS: On magnetic resonance imaging scans, the extent of cerebellar peduncle infarcts was variable. It ranged from focal brain injury (<1 cm) involving only one third of the peduncle to diffuse defects (>2 cm) spanning the full thickness of the peduncle. Peduncular infarcts were associated with large tumor size (average 3.8 cm, range 2.0-5.5 cm diameter). The long-term functional outcomes (>1 yr) varied. Dysmetria was unchanged or improved in over half of the patients (6 of 11 patients). gait recovered to normal or to preoperative levels in 5 patients. In the 6 patients with persistent impaired mobility, 2 had mild gait disturbance, 3 required regular use of a cane, and 1 has been dependent on a walker. One patient had sustained mild motor weakness. Three of 11 patients remained dependent on others for activities of daily living. CONCLUSIONS: Peduncle injury most likely stems from interruption of distal branches of the anterior inferior cerebellar artery (AICA). These small vessels are intimately related to the capsule of the tumor and may supply both the neoplasm and the brain parenchyma. It has long been recognized that interruption of the proximal segment of the AICA results in severe injury to the pons, with devastating neurologic sequelae. A limited AICA syndrome caused by loss of its distal ramifications seems a more plausible explanation for peduncular infarction than either venous insufficiency or direct surgical trauma.
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9/34. Idiopathic scoliosis as a presenting sign of familial neurologic abnormalities.

    STUDY DESIGN: Familial cases of "idiopathic" scoliosis associated with neurologic abnormalities are reported with a review of the literature. OBJECTIVE: To investigate the prevalence of neurologic abnormalities such as syringomyelia, Chiari 1 malformation, and tonsillar ectopia in patients with genetically determined "idiopathic" scoliosis. SUMMARY OF BACKGROUND DATA: Idiopathic scoliosis is widely considered to be a genetic disorder of unknown etiology. magnetic resonance imaging (MRI) studies have shown that several cases of "idiopathic" scoliosis show neurologic abnormalities including syringomyelia and Chiari 1 malformation. Recently, several familial cases of either syringomyelia or Chiari malformation were reported, and it is suspected that genetic factors may influence the development of the craniovertebral malformation. It was hypothesized that some cases of "idiopathic" scoliosis include a craniovertebral malformation that is genetically determined. methods: This study, using clinical examinations and MRI, investigated 71 patients with scoliosis and a family history of "idiopathic" scoliosis in third-degree relatives for the presence of neurologic abnormalities. If neurologic abnormalities were confirmed with MRI, the relatives affected with scoliosis were also examined. RESULTS: Nine (13%) patients showed neurologic abnormalities on MRI. magnetic resonance imaging showed syringomyelia with Chiari 1 malformation in four patients, Chiari 1 malformation in three patients, and tonsillar ectopia in two patients. Among the relatives of these patients, 4 of 15 individuals affected with scoliosis also showed neurologic abnormalities on MRI. CONCLUSIONS: It is suggested that familial neurologic abnormalities may have a wide range of expression, and that some patients with "idiopathic" scoliosis present with genetically determined craniovertebral malformations such as syringomyelia, Chiari 1 malformation, and tonsillar ectopia.
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10/34. A case of giant cholesteatoma extending to the posterior fossa.

    We present a 43-year-old male patient who developed a giant cholesteatoma with lateral sinus obliteration and involvement of the posterior cranial fossa. The only complaints were left-sided aural discharges and total sensorineural hearing loss. Examination revealed a mass lesion consistent with cholesteatoma within the left middle ear. Computed tomography and magnetic resonance imaging showed an extensive acquired cholesteatoma in the left middle ear, invading the posterior cranial fossa and leading to lateral sinus obliteration. The patient underwent left radical mastoidectomy. He had an uneventful postoperative follow-up for 13 months. This case emphasizes the value of preoperative radiologic assessment of giant cholesteatomas that may present with relatively few or limited symptoms.
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