| papilledema and raised intracranial pressure have been reported in association with guillain-barre syndrome. papilledema is usually asympotomatic or associated with mild visual field defects, without any visual loss. The cerebrospinal fluid protein is usually reported to be high. A case of a 35 year old lady is reported, who presented with headache, diplopia and progressive visual loss in both eyes and limb weakness with hyporeflexia. Optic fundus examination showed bilateral papilledema. She had features of pseudotumor cerebri. Nerve conduction studies were suggestive of polyradiculopathy. The unusual things in this case, were the profound visual loss normal cerebrospinal fluid profiles and the presentation of papilledema before the limb weakness. ( info) |
| PURPOSE: To examine the microvascular changes in the retrobulbar optic nerve in idiopathic intracranial hypertension (PTC). methods: Both optic nerves from a 29-year-old man with a two year history of PTC were examined histologically and morphometrically. A semi-automated image analysis system and paraphenylenediamine (PPD) stain were employed to resolve sufficiently the microvascular images for counts and measurement. RESULTS: There were 150 vessels distributed in the optic nerves which revealed the following: The average lumen of the vessels in outer sectors were larger than those of the inner sector vessels (168.17 microns 2 vs. 46.99 microns 2; p = 0.0338; OD; and 251.96 microns 2 vs. 130.02 microns 2; p = 0.029; OS) while in the normal control optic nerve the outer and inner area lumens were reversed in size-differential, but this did not show a statistical difference. The thickness of the PTC optic nerve vessel walls in the outer sectors was also greater than that of the walls in the inner sectors (4.95 microns vs. 2.67 microns; p = 0.013; OD and 5.25 microns vs. 3.34 microns; p = 0.019; OS); the same measurements in the normal optic nerve showed a reversed ratio, which was opposite that of the experimental group and but not statistically different. CONCLUSION: This pattern of microvascular changes is consistent with the selective axonal loss in the peripheral area of each optic nerve as much more severe than that in the inner sectors. ( info) |
63/475. Dural arteriovenous fistulae causing pseudotumour cerebri syndrome in an elderly man. The clinical presentation of dural arteriovenous fistulae (DAVF) is dependent on their location and the nature of their venous drainage. The latter plays a critical part in determining whether or not the fistula gives rise to intracranial hypertension, which is present in only a minority of cases. We present a case of the pseudotumour cerebri syndrome in an elderly man with bilateral intracranial DAVF supplied by the occipital arteries. cerebral angiography was required for definitive diagnosis, and to characterise the abnormal venous drainage. The pathophysiology of intracranial hypertension in DAVF is discussed. ( info) |
| Idiopathic intracranial hypertension, also referred to as pseudotumor cerebri (PTC), is characterized by high intracranial pressure, normal cerebral ventricles,and cerebrospinal fluid. Although papilledema is described in various anemias, only one case of PTC with idiopathic aplastic anemia has been reported in the English literature. We report a case of papilledema in an adolescent with aplastic anemia and propose its mechanism. ( info) |
| PURPOSE: To report a case of papilledema and pseudotumor cerebri developed in association with sjogren's syndrome. methods: Case-report of a 38-year-old woman with history, imaging and histology confirming the diagnosis of both pseudotumor cerebri and sjogren's syndrome who presented with bilateral decrease of vision. RESULTS: papilledema associated with pseudotumor cerebri was observed in both eyes. The patient's visual acuity improved transiently with the administration of intravenous steroids and cyclophosphamide; subsequently she needed a ventriculoperitoneal shunt. CONCLUSION: sjogren's syndrome should be considered in the different etiologies of pseudotumor cerebri. The major improvement with corticosteroids and ventriculoperitoneal shunt makes prompt diagnosis essential. ( info) |
66/475. pseudotumor cerebri syndrome: venous sinus obstruction and its treatment with stent placement. OBJECT: pseudotumor cerebri, or benign intracranial hypertension, is a condition of raised intracranial pressure in the absence of a mass lesion or cerebral edema. It is characterized by headache and visual deterioration that may culminate in blindness. pseudotumor cerebri is caused by venous sinus obstruction in an unknown percentage of cases. The purpose of this study was to investigate the role of cerebral venous sinus disease in pseudotumor cerebri and the potential of endoluminal venous sinus stent placement as a new treatment. methods: Nine consecutive patients in whom diagnoses of pseudotumor cerebri had been made underwent examination with direct retrograde cerebral venography (DRCV) and manometry to characterize the morphological features and venous pressures in their cerebral venous sinuses. The cerebrospinal fluid (CSF) pressure was measured simultaneously in two patients. If patients had an amenable lesion they were treated using an endoluminal venous sinus stent. Five patients demonstrated morphological obstruction of the venous transverse sinuses (TSs). All lesions were associated with a distinct pressure gradient and raised proximal venous sinus pressures. Four patients underwent stent insertion in the venous sinuses and reported that their headaches improved immediately after the procedure and remained so at 6 months. Vision was improved in three patients, whereas it remained poor in one despite normalized CSF pressures. CONCLUSIONS: patients with pseudotumor cerebri should be evaluated with DRCV and manometry because venous TS obstruction is probably more common than is currently appreciated. In patients with a lesion of the venous sinuses, treatment with an endoluminal venous sinus stent is a viable alternative for amenable lesions. ( info) |
67/475. Pseudotumour cerebri, CSF rhinorrhoea and the role of venous sinus stenting in treatment. We present a case of pseudotumour cerebri (PTC), which is important as it illustrates the effects of chronically raised CSF pressure, the relationship between PTC and venous sinus obstruction and the successful treatment of PTC using a venous sinus stent. A 38-year-old woman, previously diagnosed with PTC and unsuccessfully treated 10 years previously re-presented with spontaneous CSF rhinorrhoea. Imaging revealed dramatic changes of chronically raised CSF pressure and a defect in the anterior cranial fossa. The CSF leak was corrected surgically and a lumbo-peritoneal shunt inserted to correct a large postoperative subgaleal CSF collection. Direct retrograde cerebral venography (DRCV) demonstrated venous sinus obstruction due to a filling defect. This was associated with a pressure gradient and a high superior sagittal sinus pressure. The venous sinus obstruction was successfully treated with a venous sinus stent and the lumbo-peritoneal shunt was removed. Chronically raised CSF pressure in untreated cases of PTC may cause widespread changes in the skull, which in this case, culminated in a spontaneous CSF leak despite relatively mild headache and visual symptoms. Furthermore, cases of PTC secondary to venous sinus obstruction may be successfully treated using venous sinus stenting. The index of suspicion for venous sinus stenosis or obstruction should be raised in the investigation of patients with PTC. ( info) |
| A case of idiopathic intracranial hypertension (IIH) with visual field loss was decompressed in the intracanalicular segment of the optic nerve transnasally under endoscopic control. Visual functions improved after surgery. ( info) |
69/475. Mesalazine-associated benign intracranial hypertension in a patient with ulcerative colitis. PURPOSE: To report a case of benign intracranial hypertension related to mesalazine use. DESIGN: Observational case report. methods: A 23-year-old-woman with sudden visual loss, headache, and a history of ulcerative colitis treated with mesalazine underwent ophthalmologic examination, echography, magnetic resonance imaging, and lumbar puncture. RESULTS: The examinations disclosed benign intracranial hypertension that resolved when mesalazine was discontinued and recurred when the drug was restarted. CONCLUSIONS: We recommend periodic ocular fundus examination for patients undergoing long-term therapy with mesalazine, especially if decreased vision, headaches, or neck stiffness are present, to avoid potentially severe complications of intracranial hypertension ( info) |
| To our knowledge, pseudotumor cerebri as a complication of lyme disease has been described only in children. We report the first case of an adult with pseudotumor cerebri due to lyme disease and the use of serial lumbar punctures as the sole therapy. ( info) |