1/12. Clinical and molecular analysis of disseminated hemangioblastomatosis of the central nervous system in patients without von hippel-lindau disease. Report of four cases.Hemangioblastomas of the central nervous system (CNS) may occur sporadically or in association with von Hippel-Lindau (VHL) syndrome. The authors present four patients with no family history or clinical evidence of VHL syndrome in whom extensive, progressive, en plaque coating of the brainstem and spinal cord with hemangioblastomas developed 1 to 8 years after complete resection of a solitary cerebellar hemangioblastoma. Analysis included detailed physical, biochemical, radiological, and pathological examinations in all four patients, combined with family pedigree analysis. In addition, a detailed investigation of the VHL gene was undertaken. Allelic loss, comparative genomic hybridization (CGH), single-stranded conformational polymorphism screening, CpG island methylation status, and x chromosome inactivation clonality analyses were performed. Although there was no evidence of germline alterations in the VHL gene on clinical and radiological examination or in the family history (all four patients) or analysis of peripheral blood (three patients), somatic deletion of one copy of the VHL gene occurred in these tumors. These findings indicate that the multiple, separate deposits of tumors were likely derived from a single clone. Results of CGH indicate that one or several additional genes are probably involved in the malignant behavior of the hemangioblastomas in these patients. Furthermore, the malignant biological and clinical behavior of these tumors, in which multiple sites of subarachnoid dissemination developed 1 to 8 years after initial complete resection, followed by progressive tumor growth and death of the patients, occurred despite a histological appearance typical of benign hemangioblastomas. Malignant hemangioblastomatosis developed 1 to 8 years after resection of an isolated cerebellar hemangioblastoma. Alterations of the VHL gene may be permissive in this setting, but other genes are likely to be the source of the novel biological and clinical presentation of the disseminated hemangioblastomas in these patients. This appears to represent a novel condition in which the product of one or more mutations in several genes permits malignant tumor behavior despite retention of a benign histological picture, a circumstance previously not recognized in CNS tumors.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
2/12. Investigating the causes of vertigo in breast cancer survivors.The vertigo symptom in breast cancer survivors has rarely been mentioned. The aim of this study was to investigate the causes of vertigo in breast cancer survivors with vertigo. From May 1997 to April 2003, 36 consecutive female breast cancer survivors with vertigo underwent a battery of tests including physical examination, neurological examination, serum lipid profile, plain chest radiograph, whole body bone scan, liver sonography, audiometry, electronystagmography (ENG) and MRI scan. Based on these tests, the causes of vertigo were attributed to peripheral labyrinthine origin in 14 patients (39%) and central origin in 22 patients (61%) consisting of 11 cases of vascular insufficiency, eight of hyperlipidemia and three of posterior fossa metastases (8%). The latter included one case of cerebellopontine angle and two of cerebellum, accompanied by extracranial systemic metastases, e.g., of the lung, bone or liver. In conclusion, vertigo in breast cancer survivors warrants concern, especially in those with extra-cranial systemic metastasis accompanied by headache. In addition to 8% occurrence of posterior fossa metastasis, other possible causes for vertigo in breast cancer survivors consist of vascular insufficiency, hyperlipidemia and labyrinthine lesion.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
3/12. A rare post-transplant malignancy, cerebellar hemangioblastoma: a case report.INTRODUCTION: Post-transplant malignancies are among the most important complications in organ transplantation. hemangioblastoma (HB) is especially prevalent in the cerebellum. CASE REPORT: A 20-year-old male who first started dialysis therapy, and then underwent kidney transplantation from a living-relative donor. Five years after transplantation, the patient suffered from vertigo and imbalance when walking. On cranial magnetic resonance imaging (MRI), a mass lesion in the right cerebellar hemisphere was observed, 3 x 3 x 3 cm in size, which was pushing against the fourth ventricle, and the right cerebellar peduncle. The patient had significant hydrocephaly. The mass lesion was removed by craniectomy. The pathological diagnosis was cerebellar hemangioblastoma. The symptoms and clinical findings improved. The patient was diagnosed with sporadic hemangioblastoma. Rapamycin therapy was started instead of cyclosporine, and the patient is being followed up without further problems. DISCUSSION: HB causes 2% of all intracranial tumors in the general population. It is generally sporadic in nature and approximately 20% can be associated with von Hippel-Lindau (VHL) syndrome. As in this case, MRI is preferred for the diagnosis. There was no pathology related to VHL disease in this patient's physical examination, family history, routine biochemical tests and abdominal MRI. The treatment is surgical excision of the tumor, as in this case. CONCLUSION: When cerebellar symptoms occur or a cerebellar mass lesion is detected in an organ recipient, HB should be considered in the differential diagnosis. The examination of patients with HB for a possible association with VHL disease is also required.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
4/12. Pyriformis syndrome in a 10-year-old boy as a complication of operation with the patient in the sitting position.We present a case of sciatic neuropathy due to the pyriformis syndrome after operation in the sitting position. Neither sciatic nerve injury nor the pyriformis syndrome has been reported after operation in the sitting position, although a low incidence of common peroneal nerve injury has been reported as a complication of operation on patients who are in the sitting position. The clinical findings of sciatic neuropathy, external rotation of the ipsilateral foot in the position of comfort, and a therapeutic response to local anesthetic injection into the pyriformis muscle are diagnostic of the syndrome. Nerve conduction studies should be performed to aid in the differentiation between a common peroneal and sciatic neuropathy. The syndrome may occur because of extreme flexion of the hips and prolonged pressure while in the sitting position, leading to pyriformis muscle trauma, resultant spasm, and sciatic compression. The prognosis is for complete recovery after symptomatic treatment with nonsteroidal antiinflammatory medication and physical therapy.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
5/12. Treatment of medulloblastoma using a computer-controlled tracking cobalt unit.radiation therapy of the length of the spinal column presents various clinical and physical problems. The completed plan may be complicated to set up, be time-consuming and require daily variation to achieve reasonable dose homogeneity. A case of medulloblastoma is used to illustrate the steps in producing a plan for dynamic treatment using a computer-controlled tracking cobalt unit. After definition by computed tomography, the target is considered in segments in order to develop a plan which keeps the spinal cord constantly positioned at the beam isocentre. The main computer is used to develop the patient treatment file and information is transferred to a second computer which controls and monitors the safe functioning of the cobalt unit. The cranial fields are treated separately in a conventional way. Good and consistent control of the dose distribution is achieved along the entire target volume. This technique is a marked improvement over all existing methods of treating the spinal axis.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
6/12. Positional nystagmus.Positional nystagmus may be persistent or transitory. Of the persistent forms, type I refers to nystagmus changing direction in different head positions and type II to nystagmus beating in a single direction. Both types may denote either peripheral or central abnormality. Transitory, or paroxysmal, positional nystagmus is classified as type III, and the history and physical and electronystagmographic features of its accompanying vertigo and nystagmus permit subdivision into typical and atypical forms. Guidelines are given for the differentiation of typical and atypical forms. In the great majority of cases type III positional nystagmus denotes a harmless inner ear disturbance, but the occasional serious intracranial lesion is also causative. Most instances of the latter, but not all, will fit in the atypical group. A case is reported of vermis metastasis with presenting features of postural vertigo and paroxysmal downbeat positional nystagmus.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
7/12. Long-term effects of radiation for medulloblastoma on intellectual and physical development. A case report of monozygotic twins.BACKGROUND. Recent advances in treatment have improved the prognosis for medulloblastoma. Although postoperative radiation of the central nervous system (CNS) is widely practiced, late radiation sequelae, especially in long-term survivors, pose a constant risk. methods. To assess the long-term effects of CNS radiation, a monozygotic twin girl with medulloblastoma was compared with her normal twin. Treatment included total removal of the mass, postoperative chemotherapy, and CNS radiation with 47.8 Gy directed to the posterior fossa, 30.2 Gy to the cerebral hemispheres, and 20 Gy to the whole spine. RESULTS. Not only the radiated patient's spine but also the unradiated upper and lower limbs were shorter than the control subject's. The patient's thyroid hormone level was within normal range; however, thyroid-stimulating hormone was higher than normal. The patient's intellectual ability deteriorated, and cognitive dysfunction has not improved as of 6 years after treatment. CONCLUSIONS. Treatment systems that do not cause late sequelae affecting physical and intellectual development should be pursued.- - - - - - - - - - ranking = 5keywords = physical (Clic here for more details about this article) |
8/12. Appropriateness of magnetic resonance imaging in sudden sensorineural hearing loss.Idiopathic sudden sensorineural hearing loss is an enigmatic condition of unknown cause. Although the treatment for sudden sensorineural hearing loss is controversial, the evaluation for a cause should not be. All patients are evaluated with a complete history, physical examination, audiologic examination, and blood draw to evaluate complete blood count, general chemistry screen, thyroid function test results, erythrocyte sedimentation rate, and fluorescent treponemal antibody absorbance. magnetic resonance imaging with gadolinium contrast is essential in the evaluation of idiopathic sudden sensorineural hearing loss, even if there is a complete response to either treatment or no treatment. During the last year we treated 16 patients for idiopathic sudden sensorineural hearing loss with our protocol of intravenous dextran/Hypaque or oral high-dose steroids. Fifteen patients were evaluated immediately before treatment with a magnetic resonance imaging scan. An additional patient had been treated successfully with high-dose steroids at an outside institution and came in for an evaluation. Of these 16 patients, 3 (18.75%) were found to have significant pathologic conditions on magnetic resonance imaging scan. The patient who had been treated successfully on the outside was noted to have a 5-mm intracanalicular acoustic neuroma, the second patient was found to have a multiple sclerosis lesion at the level of the superior olive, and the third patient, who had had a normal magnetic resonance imaging scan 18 months previously, was now found to have a large 4- to 5-cm meningioma in the cerebellopontine angle. We believe it is essential that all patients with idiopathic sudden sensorineural hearing loss be evaluated at some point during their treatment with a magnetic resonance imaging scan with gadolinium contrast.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
9/12. rehabilitation of balance in two patients with cerebellar dysfunction.The treatment of two patients with cerebellar dysfunction is described. One patient was a 36-year-old woman with a 7-month history of dizziness and unsteadiness following surgical resection of a recurrent pilocystic astrocytoma located in the cerebellar vermis. The other patient was a 48-year-old man with cerebrotendinous xanthomatosis (CTX) and diffuse cerebellar atrophy, and a 10-year history of progressive gait and balance difficulties. Each patient was treated with a 6-week course of physical therapy that emphasized the practice of activities that challenged stability. The patient with the cerebellar tumor resection also performed eye-head coordination exercises. Each patient had weekly therapy and performed selected balance retraining exercises on a daily basis at home. Measurements taken before and after treatment for each patient included self-perception of symptoms, clinical balance tests, and stability during selected standing and gait activities; for the patient with the cerebellar tumor resection, vestibular function tests and posturography were also performed. Both patients reported improvements in symptoms and demonstrated similar improvements on several kinematic indicators of stability during gait. The patient with the cerebellar tumor resection improved on posturography following treatment, whereas the patient with CTX improved on clinical balance tests. This case report describes two individualized treatment programs and documents functional improvements in two patients with different etiologies, durations, and clinical presentations of cerebellar dysfunction. The outcomes suggest that patients with cerebellar lesions, acute or chronic, may be able to learn to improve their postural stability.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
10/12. cerebellopontine angle meningiomas with primary otologic symptoms.The majority of cerebellopontine angle (CPA) tumors are acoustic neuromas (AN). However, an intracranial meningioma may occur at this site and will produce symptoms similar to an AN. The most common presenting symptoms of CPA meningiomas are hearing loss, tinnitus, dizziness and dysequilibrium. It cannot be easily distinquished from an AN only on the history and physical examination. Even with an audiogram, evoked response audiometry (ERA) and vestibular function tests, it still cannot be distinquished. CT scan and MRI are helpful in differentiating these two tumors radiographically. In this article, we report two cases of CPA meningiomas which presented with otologic symptoms. The diagnosis and treatment of CPA meningioma is discussed.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
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