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Idiopathic intracranial hypertension is a disease with a predilection for young obese women. The most common symptoms are headache, transient visual obscuration and pulsatile tinnitus. The only focal neurologic finding is false-localizing 6th cranial nerve palsy. Papilledema is usually present and this can lead to optic atrophy with progressive permanent visual loss. The earliest visual loss is constriction of peripheral visual field, usually starting with the inferior nasal quadrant. Numerous theories have been entertained as to the pathogenesis but this still remains an open controversy. The most prevalent current theories involve increased resistance to cerebrospinal fluid reabsorption at the arachnoid granulations, either from intrinsic disease in the granulations or secondary to elevated pressure in the dural venous sinuses into which the cerebrospinal fluid is absorbed across the granulations. The syndrome of idiopathic intracranial hypertension was long ago recognized as a complication of recurrent otitis media with resultant thrombosis of the transverse and sigmoid dural venous sinuses. Cases secondary to dural venous sinus thrombosis are seldom encountered today because the incidence of chronic otitis is much less than in the past. The prevalent concept has been that the idiopathic cases in obese young women were not associated with pathology in the dural venous sinuses. A recent study using ATECO MR venography, which the authors claim to be more reliable than even conventional catheter venography, has demonstrated stenosis of the transverse and sigmoid dural venous sinuses distinguishes cases of idiopathic intracranial hypertension from controls with a high degree of sensitivity and specificity. The authors believe the stenosis is secondary to intracranial hypertension but that it may further aggravate the hypertension when it occurs.
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PMID:Recent developments in idiopathic intracranial hypertension (IIH). 1551 4

Chondroid chordoma commonly presents as clival osseous and extradural mass. A 15-year-old boy presented with progressive visual deficit, headaches and diplopia since three years. Computed tomography (CT scan) showed a skull base tumour, but was wrongly reported at the time as chronic sphenoidal sinusitis and nasal polyps. In the past three months, he developed dysphagia, urinary retention and constipation. Terminally, he had weakness of all limbs. Fundoscopy showed optic atrophy. Temporal and spatial variation in symptoms led to a clinical diagnosis of multiple sclerosis with optic neuritis. Partial brain autopsy revealed small gelatinous tumour nodules in the subarachnoid space of middle cranial fossa encasing base of brain like arachnoiditis. Tumour deposits extended down into the spinal cord along the subarachnoid space as far as vision allowed. Histopathology and immunohistochemistry confirmed a diagnosis of chondroid chordoma. Awareness of this rare mode of dissemination will avoid misdiagnosis and delay in treatment.
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PMID:Craniospinal dissemination of clival chondroid chordoma. 1633 98

Pseudotumor cerebri is a clinical syndrome characterized by raised intracranial pressure with normal ventricular size, anatomy and position. Headache, vomiting and diplopia are the most common symptoms. Signs include those of raised intracranial pressure including papilledema and absence of focal neurological signs. A secondary cause is identifiable in 50% of children; the most common predisposing conditions are otitis media, viral infection and medications. Management is mainly directed towards identifying and treating the cause and measures to reduce the raised intracranial pressure. Though it is mostly a self limited condition, optic atrophy and blindness can occur. Oculomotor nerve palsy is very rarely associated with pseudotumor cerebri. We report a unique case of pseudotumor cerebri who had left Oculomotor palsy with sparing of the pupillary fibres, which resolved following treatment with oral acetazolamide.
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PMID:Pseudotumor cerebri with transient oculomotor palsy. 1638 55

Idiopathic hypertrophic cranial pachymeningitis is a rare chronic inflammatory and fibrosing process of unknown etiology. This entity is characterized by fibrosis and thickening of the dura mater and resulting in neurological syndrome. The authors report a 72 year-old woman who presented with progressive bifrontal headache, bilateral visual loss and transient episode of confusion. Neurological examination revealed bilateral optic atrophy, apathy and no focal neurological deficit. Investigations showed anemia of chronic disease, elevated erythrocyte sedimentation rate and polyclonal hypergammaglobulinemia. No specific inflammatory diseases or malignancy such as systemic lupus erythematosus, syphilis, hematologic malignancy were found MRI of the brain revealed thickened and enhanced dura mater and leptomeninges at the inferior aspect of bilateral frontal lobes as well as vasogenic edema of the frontal lobes. Cerebrospinal fluid showed mild pleocytosis, high protein level and normal glucose level. Meningeal biopsy revealed nonspecific inflammatory process of the dura and leptomeninges. There was no granuloma formation or evidence of vasculitis. Special stain for tuberculous bacilli, fungus and malignancy were all negative. The diagnosis of "idiopathic hypertrophic pachymeningitis" was made. The patient was treated with oral prednisolone 45 mg/day. Her headache was improved, but the profound vision loss in both eyes remained unchanged after 2 years of follow-up. Prednisolone was tapered within 18 months. Idiopathic hypertrophic cranial pachymeningitis usually involves dura at tentorium cerebelli, cavernous sinus and base of the skull. The extensive involvement at the anterior cranial fossa is extremely rare.
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PMID:Extensive anterior cranial fossa idiopathic hypertrophic pachymeningitis: a case report and review of the literature. 1651 96

We report a case of multiple gangliogliomas of the optic pathway in an 18-year-old boy. He presented with visual disturbance mainly in his left eye, non-specific headaches, and episodes of sensory disturbance in his left arm. Visual acuity was 6/9 and 6/24 in his right and left eye respectively. He did not have any Lisch nodules. Optic atrophy was noted in his left eye. MRI scan revealed multiple enhancing lesions involving the optic chiasm, left optic tract, right lateral geniculate body, and right optic radiation in the temporal lobe. Stereotactic biopsy of the temporal tumour confirmed ganglioglioma. Multiple gangliogliomas of the optic pathway has not been hitherto described.
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PMID:Multiple gangliogliomas of the optic pathway. 1743 99

We encountered a rare case of neurohypophyseal germinoma with a prominent granulomatous reaction, which invaded the right cavernous sinus. The neuroimaging and histopathology features in this case were unique, distinguishing it from other types of suprasellar lesions. A 13-year-old boy presented with loss of appetite and polyuria; both symptoms were present for 1 year, and headache, general fatigue and blurred vision present for the prior 2 months. On admission, neurological examination indicated bitemporal hemianopsia and optic atrophy. Endocrinological exam showed panhypopituitarism. Tumor markers such as alpha-fetoprotein, human growth hormone, carcinoembryonic antigen, and placental alkaline phosphatase were negative. Brain CT revealed a suprasellar tumor with calcification. MR T(1)-weighted and T(2)-weighted images showed the tumor to be isointense to normal brain parenchyma and to be enhanced densely. The tumor also involved the right cavernous sinus, so that a biopsy was performed by the transsphenoidal approach. On pathologic examination of the specimen, typical large tumor cells with lymphocytic cell infiltration and prominent granulomatous reaction were observed. Neurohypophyseal granulomatous germinoma was diagnosed. Radiotherapy was performed with a total dose of 51 Gy and the tumor shrank remarkably. The patient returned to school under hormone replacement therapy.
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PMID:Neurohypophyseal granulomatous germinoma invading the right cavernous sinus: case report and review of the literature. 1762 46

Charles-Bonnet syndrome (CBS) occurs after the loss of vision or a decrease in visual ability and is characterized by visual hallucinations with insight and preserved cognitive status. In this paper, 2 cases in which vision was lost (in one patient due to hypophyseal macroadenoma and in the other due to diabetic retinopathy) are presented. The first case is a 35-year-old male referred to the psychiatry department for depressive complaints. He lost his vision due to optic atrophy following multiple surgeries for hypophyseal macroadenoma and visual hallucinations developed afterwards. Ziprasidone 80 mg/day was started and the hallucinations disappeared on the fifth day, but the medication had to be withdrawn because of severe vascular type headaches. Olanzapine 5 mg/day was subsequently used without benefit. He refused further medication and the visions gradually changed into sparkling lights and simple figures at the 7-month follow-up. The second case was a 54-year-old woman with a 20-year history of type-II diabetes mellitus. Visual hallucinations developed after bilateral visual loss due to diabetic retinopathy 4 years earlier. She responded well to treatment with quetiapine 100 mg/day and paroxetine 20 mg/day. Neither of the cases had reported their visual symptoms to their physicians; the second case was diagnosed after 4 years by active screening. Both of the cases had neuroanatomic lesions. CBS symptoms should be screened actively in patients with visual loss and physicians should be educated about diagnosing CBS. Novel antipsychotics, such as ziprasidone and quetiapine, seem to be effective alternatives for the treatment of CBS.
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PMID:[Charles-Bonnet Syndrome: a report of two cases]. 1785 83

Pseudotumor cerebri is a clinical condition marked by papilledema, normal cerebrospinal fluid composition, normal or small ventricles on radiography, and absence of an intracranial mass. In this condition, headache, tinnitus, dizziness, blurred vision, and diplopia are frequently observed. The cause is often unknown but can occur with certain drug ingestions or systemic inflammatory and metabolic diseases. The treatment is primarily focused on the correction of the underlying cause with measures to reduce the raised intracranial pressure. The most important complication is optic atrophy, which results in visual loss. The sixth cranial nerve is most commonly affected. The involvement of the third cranial nerve is distinctly unusual. The present report describes a unique case of bilateral oculomotor palsy with sparing of the papillary fibers. It resolved promptly on administration of acetazolamide.
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PMID:Transient bilateral oculomotor palsy in pseudotumor cerebri. 1844 74

Tobacco consumption is the leading preventable cause of disease, disability, and premature death but little is known about its deleterious effect on the ocular health of workers handling tobacco. The goal of this study was to identify probable effects of occupational tobacco exposure among south Indian bidi-industry workers. This study included 310 females (mean age, 34.8 +/- 10.9 years) actively involved in bidi-rolling presenting with eye symptoms to a tertiary eye care hospital. Results suggested that a wide spectrum of ocular complications exist among these workers. Common ocular symptoms were defective vision, dull-aching headache and eye irritation. The main ocular findings were papillary conjunctival hyperplasia, hyperpigmentation of ocular surface, punctate epithelial erosion or superficial punctate keratitis, cataract or pseudophakia and segmental optic atrophy. Abstaining from work, supplementation of Vitamin B complex rich in B 12 and appropriate surgical or medical management reversed visual loss due to corneal disease or cataract but was not effective in optic neuropathy.
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PMID:Ocular manifestations in bidi industry workers: possible consequences of occupational exposure to tobacco dust. 1857 93

An intracranial pilocytic astrocytoma was diagnosed in a 13-year-old boy after he presented with headaches and visual disturbances. The initial ophthalmoscopic examination revealed papilledema bilaterally and myelinated retinal nerve fibers (MRNFs) in the left eye. Serial ophthalmoscopic examinations demonstrated gradual fading of the MRNFs beginning 2 months after tumor resection and their complete disappearance by 2 years after surgery. The disappearance of MRNFs has been described in the presence of ischemia, compression, glaucoma, and demyelinating disease of the optic nerve. This patient demonstrates that loss of myelinated nerves fibers may also occur with the optic atrophy that may follow chronic papilledema.
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PMID:Loss of myelinated retinal nerve fibers from chronic papilledema. 1876 89


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