1/85. Optic nerve cysticercosis in the optic canal.The authors present a first case of cysticercosis in the optic canal in a fifteen-year-old female patient. cysticercosis of the optic nerve is rare. A cyst in the optic canal, beneath the sheath of the optic nerve, has never been reported. The patient presented with rapidly diminishing vision in the left eye, headache and papillitis. A magnetic resonance imaging revealed a cystic lesion at the entrance of the optic canal. Surgery performed was a transcranial orbitotomy which included deroofing of the optic canal and removal of the cyst from under the sheath of the optic nerve. The cyst proved to be cysticercus histopathologically. The outcome was a remarkable visual recovery.- - - - - - - - - - ranking = 1keywords = canal (Clic here for more details about this article) |
2/85. Acquired mitochondrial impairment as a cause of optic nerve disease.BACKGROUND: blindness from an optic neuropathy recently occurred as an epidemic affecting 50,000 patients in cuba (CEON) and had clinical features reminiscent of both tobacco-alcohol amblyopia (TAA) and Leber's hereditary optic neuropathy (Leber's; LHON). Selective damage to the papillomacular bundle was characteristic, and many patients also developed a peripheral neuropathy. Identified risk factors included vitamin deficiencies as well as exposure to methanol and cyanide. In all 3 syndromes, there is evidence that singular or combined insults to mitochondrial oxidative phosphorylation are associated with a clinically characteristic optic neuropathy. PURPOSE: First, to test the hypothesis that a common pathophysiologic mechanism involving impairment of mitochondria function and, consequently, axonal transport underlies both genetic optic nerve diseases such as Leber's and acquired toxic and nutritional deficiency optic neuropathies. According to this hypothesis, ATP depletion below a certain threshold leads to a blockage of orthograde axonal transport of mitochondria, which, in turn, leads to total ATP depletion and subsequent cell death. Second, to address several related questions, including (1) How does impaired energy production lead to optic neuropathy, particularly since it seems to relatively spare other metabolically active tissues, such as liver and heart? (2) Within the nervous system, why is the optic nerve, and most particularly the papillomacular bundle, so highly sensitive? Although there have been previous publications on the clinical features of the Cuban epidemic of blindness, the present hypothesis and the subsequent questions have not been previously addressed. methods: patients in cuba with epidemic optic neuropathy were personally evaluated through a comprehensive neuro-ophthalmologic examination. In addition, serum, lymphocytes for dna analysis, cerebrospinal fluid (CSF), sural nerves, and eyes with attached optic nerves were obtained from Cuban patients, as well as from Leber's patients, for study. Finally, we developed an animal model to match the low serum folic acid and high serum formate levels found in the CEON patients, by administering to rats low doses of methanol after several months of a folic acid-deficient diet. Optic nerves and other tissues obtained from these rats were analyzed and compared with those from the Cuban patients. RESULTS: patients from the Cuban epidemic of optic neuropathy with clinical evidence of a selective loss of the papillomacular bundle did much better once their nutritional status was corrected and exposure to toxins ceased. patients with CEON often demonstrated low levels of folic acid and high levels of formate in their blood. Histopathologic studies demonstrated losses of the longest fibers (in the sural nerve) and those of smallest caliber (papillomacular bundle) in the optic nerve, with intra-axonal accumulations just anterior to the lamina cribrosa. Our animal model duplicated the serologic changes (low folic acid, high formate) as well as these histopathologic changes. Furthermore, ultrastructural examination of rat tissues demonstrated mitochondrial changes that further matched those seen on ultrastructural examination of tissues from patients with Leber's. CONCLUSION: mitochondria can be impaired either genetically (as in Leber's) or through acquired insults (such as nutritional or toxic factors). Either may challenge energy production in all cells of the body. While this challenge may be met through certain compensatory mechanisms (such as in the size, shape, or number of the mitochondria), there exists in neurons a threshold which, once passed, leads to catastrophic changes. This threshold may be that point at which mitochondrial derangement leads to such ATP depletion that axonal transport is compromised, and decreased mitochondrial transport results in even further ATP depletion. neurons are singularly dependent on the axonal transport of mitochondria. (- - - - - - - - - - ranking = 0.0073963402379721keywords = spinal (Clic here for more details about this article) |
3/85. Idiopathic intracranial hypertension: a case report with optic nerve histopathology.We present the clinical and pathologic findings in an atypical case of idiopathic intracranial hypertension. A 51-year-old man had headaches, visual deterioration, papilloedema, and deafness. neuroimaging was normal, and cerebrospinal fluid pressure monitoring confirmed increased intracranial pressure. The patient was treated with a ventriculo-peritoneal shunt. Histopathology revealed grossly atrophic optic nerves with almost complete axonal loss. The prelaminar portion of the optic nerves was thickened by gliosis and hyalinized capillaries, which have not been described previously.- - - - - - - - - - ranking = 0.0073963402379721keywords = spinal (Clic here for more details about this article) |
4/85. Results of transmedial-canthal ethmoidal decompression for severe dysthyroid optic neuropathy.PURPOSE: To study the effects of ethmoidal wall (one-wall) decompression using a transmedial-canthal approach (transmedial-canthal ethmoidectomy) for the treatment of dysthyroid optic neuropathy. methods: The ethmoidal wall and air cells were completely removed using a transmedial-canthal approach in 6 eyes of 4 patients (mean age = 55 years; age range, 46-69 years) with dysthyroid optic neuropathy. Similar surgery was performed on 2 contralateral eyes in 2 of the patients for cosmetic reasons. The preoperative corrected visual acuity in the 6 eyes ranged from hand motion to 20/100. Centrocecal scotomas were detected using automatic static threshold perimetry in the 6 eyes. RESULTS: After transmedial-canthal ethmoidectomy, the corrected visual acuity improved to better than 20/20 in the 6 eyes, and the centrocecal scotomas had almost completely resolved. There were no major complications, such as cerebrospinal fluid leakage or diplopia associated with the surgery. There were no relapses during an average follow-up period of 29 months. CONCLUSIONS: These findings suggest that transmedial-canthal ethmoidectomy is an effective and safe therapy for dysthyroid optic neuropathy.- - - - - - - - - - ranking = 0.0073963402379721keywords = spinal (Clic here for more details about this article) |
5/85. Surgical management of symptomatic intrasellar arachnoid cysts--two case reports.Two patients with symptomatic intrasellar arachnoid cyst were successfully treated. A 67-year-old female with a cyst 20 mm in diameter developed headache and visual disturbance. She was treated by transsphenoidal surgery. A 59-year-old male with a cyst measuring 35 x 30 x 50 mm causing headache, visual disturbance, and deterioration of consciousness was managed by wide resection of the cyst wall via craniotomy. Postoperative courses in both patients were uneventful. Transsphenoidal surgery may be suitable for small to medium-sized cysts, although tight packing of the sella is mandatory to prevent leakage of cerebrospinal fluid. However, craniotomy is recommended for large intra- and suprasellar arachnoid cysts to avoid this complication, and to achieve sufficient communication between the cyst and the subarachnoid cistern.- - - - - - - - - - ranking = 0.0073963402379721keywords = spinal (Clic here for more details about this article) |
6/85. Visual loss associated with fibrous dysplasia of the anterior skull base. Case report and review of the literature.The authors present a case of visual loss associated with fibrous dysplasia of the anterior skull base and the surgical management of this case. Preoperative computerized tomography scanning in this patient demonstrated a patent optic foramen and a rapidly growing cystic mass within the orbit, which was responsible for the patient's visual loss. A literature review revealed that this case is typical, in that cystic mass lesions of various types are frequently responsible for visual loss associated with fibrous dysplasia. The authors did not find significant evidence in the literature to support the notion that visual loss associated with fibrous dysplasia is the result of progressive optic canal stenosis, thus raising questions about the value of prophylactic optic canal decompression. Instead, as demonstrated by this case and those uncovered in the literature review, most instances of visual loss result from the rapid growth of mass lesions of cystic fibrous dysplasia, mucoceles, or hemorrhage. Findings of the literature review and the present case of fibrous dysplasia of the anterior skull base support a role for extensive surgical resection in these cases and indicate a need for additional prospective analysis of a larger number of patients with this disease.- - - - - - - - - - ranking = 0.25keywords = canal (Clic here for more details about this article) |
7/85. Craniofacial fibrous dysplasia complicated by acute, reversible visual loss: report of two cases.We report two cases of craniofacial fibrous dysplasia which presented with acute visual loss. The first patient had a sphenoid sinus mucocele compressing the optic chiasm. In the second patient the optic canal was narrowed by dysplastic bone. In both cases optic nerve decompression restored vision to normal.- - - - - - - - - - ranking = 0.125keywords = canal (Clic here for more details about this article) |
8/85. Bilateral blindness and lumbosacral myelopathy associated with high-dose carmustine and cisplatin therapy.PURPOSE: To report the early ocular pathologic findings associated with high-dose carmustine and cisplatin therapy. methods: A patient with metastatic breast carcinoma developed an acute onset of branch retinal artery occlusion, bilateral blindness, and a myelopathy involving the lower extremities after high-dose chemotherapy and bone marrow transplant. RESULTS: Histopathologic examination of the eye and optic nerves at autopsy disclosed nerve fiber layer infarction secondary to right inferior temporal retinal artery thrombosis. Patchy necrosis of both optic nerves, medulla oblongata, and spinal cord was associated with focal small-vessel thrombosis. CONCLUSIONS: The syndrome of retinal vascular occlusion, optic neuropathy, and myelopathy is associated with the high-dose chemotherapeutic agents carmustine and cisplatin. The distribution of necrosis suggests an ischemic event rather than direct neurotoxic effects.- - - - - - - - - - ranking = 0.0073963402379721keywords = spinal (Clic here for more details about this article) |
9/85. Superficial siderosis of the central nervous system: a 70-year-old man with ataxia, depression and visual deficits.BACKGROUND: Superficial siderosis of the central nervous system (SSNS) is caused by cerebral, cerebellar and spinal cord tissue deposition of hemosiderin, often related to repeated episodes of subarachnoid hemorrhage. Typical symptoms include ataxia, sensorineural deafness and dementia. methods AND RESULTS: An elderly patient with SSNS presenting with ataxia, depression and severe visual impairment was admitted to the Unit of geriatrics of the University Hospital of Perugia, italy. Late diagnosis and the association of symptoms with SSNS prevented the possible surgical treatment of the disease. CONCLUSIONS: Recognition of uncommon clinical variants may facilitate early diagnosis of SSNS and improve therapeutic results.- - - - - - - - - - ranking = 0.0073963402379721keywords = spinal (Clic here for more details about this article) |
10/85. Treatment of myelopathy in Sjogren syndrome with a combination of prednisone and cyclophosphamide.BACKGROUND: Peripheral neuropathy is a common complication of primary Sjogren syndrome, but central nervous system involvement also occurs and may be the only extraglandular manifestation. Sicca symptoms may also be minimal. Combinations of lesions along with relapses and remissions can suggest multiple sclerosis in the proper clinical setting, making the correct diagnosis elusive. OBJECTIVES: To report a case of progressive transverse myelopathy with previous optic neuropathy in primary central nervous system Sjogren syndrome (CNS-SS), and to review 17 previously reported cases and the patient's responses to various therapies. DESIGN: Case report and literature review. SETTING: University hospital. PATIENT: A 63-year-old Hispanic woman with a 10-month history of progressive spastic paraparesis associated with optic neuropathy and a T10 sensory level. magnetic resonance imaging demonstrated multifocal, contrast-enhancing lesions in the spinal cord. The patient was diagnosed as having CNS-SS because of the presence of sicca symptoms, abnormal serological test results, and salivary gland biopsy results, which fulfilled San Diego criteria for "definite" Sjogren syndrome. She responded to treatment with a combination of prednisone and cyclophosphamide. CONCLUSIONS: Diagnosis of primary CNS-SS requires a high index of suspicion and specialized clinical testing. Treatment with pulse doses of corticosteroids alone may be suboptimal, but results of treatment with a combination of corticosteroids and either cyclophosphamide or chlorambucil have been encouraging.- - - - - - - - - - ranking = 0.0073963402379721keywords = spinal (Clic here for more details about this article) |
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