High-resolution magnetic resonance imaging of the intraorbital optic nerve and subarachnoid space in patients with papilledema and optic atrophy

OBJECTIVE: To evaluate the orbital portion of the optic nerve and the subarachnoid space using fast spin-echo magnetic resonance imaging in normal subjects and in patients with papilledema or optic atrophy. DESIGN: Measurements of the optic nerve complex on coronal images were made using high-resolution magnetic resonance imaging with fast spin-echo sequences. PATIENTS: Twenty-one patients, including 5 patients with papilledema due to congenital hydrocephalus, intracranial tumors, or meningitis, as well as 16 patients with optic atrophy, were studied. Sixteen healthy volunteers served as controls. MAIN OUTCOME MEASURES: The longitudinal diameter of the optic nerve, the longitudinal outer diameter of the subarachnoid space, the diameter ratio, and the area of the subarachnoid space were determined. RESULTS: In normal subjects, the ring-shaped area of high signal intensity that represented the subarachnoid space was widest behind the globe, then narrowed toward the orbital apex. In patients with papilledema, the area of the subarachnoid space was markedly dilated, the optic nerve was compressed, and the nerve sheath was widened, resulting in a small diameter ratio compared with that of controls. Patients with pallor of the temporal aspect of the optic disc appeared to exhibit dilation of the subarachnoid space; the size of the optic nerve was decreased more than that of the nerve sheath, resulting in a small diameter ratio compared with controls. Patients with complete pallor of the disc, however, exhibited hyperintense optic nerve complexes without a ring-shaped appearance toward the orbital apex. CONCLUSION: Fast spin-echo magnetic resonance imaging appears useful for objectively evaluating the optic nerve and surrounding subarachnoid space in patients with papilledema and optic atrophy.     

Quantitative evaluation of papilledema in pseudotumor cerebri

PURPOSE: To determine the feasibility of adapting confocal scanning laser (CSL) tomography of the optic disc for quantitative evaluation of papilledema in pseudotumor cerebri (PTC). METHODS: Confocal scanning laser tomography of the optic disc was performed in 11 patients with diagnosed PTC and 12 visually normal control subjects of similar age. In five patients with active papilledema, CSL tomography was performed serially over several months. To quantify optic disc characteristics, surface topography was measured in 0.1-mm steps along the horizontal and vertical meridians and four oblique meridians. Best fit polynomial functions, describing surface topography along each meridian, were derived using linear regression analysis. RESULTS: Third-order polynomials provided excellent fits (significantly better than the second-order functions) to the surface topography for all meridians in the control subjects and patients with PTC. In control subjects and PTC patients an asymmetry in the slope of the optic disc contours was evident along the horizontal but not the vertical meridian. In patients with active papilledema a significant elevation of the center of the disc was accompanied by a change in overall surface topography. Each of the PTC patients followed up serially had a pronounced posterior deformation of the disc (i.e., a reduction in papilledema) that was initially apparent in the temporal meridian and did not proceed uniformly across all meridians. CONCLUSIONS: Confocal scanning laser tomography can quantify the magnitude and monitor the resolution of papilledema in PTC. Studies of optic nerve head topography may provide further insight into optic nerve compliance with elevated intracranial pressure.     

Imaging of external instabilities of the patella. State of the art

Recent articles in the scientific literature have described major advances in our understanding of the anatomy and vascular relationships of the optic nerve (cranial nerve II) and of the diagnosis and treatment of a variety of disorders affecting this nerve, including congenital anomalies of the optic disc, dominant hereditary optic neuropathy, anterior and retrobulbar arteritic and nonarteritic ischemic optic neuropathy, optic neuritis, Cuban epidemic optic neuropathy, toxic and nutritional optic neuropathies, radiation-induced optic neuropathy, AIDS-related optic neuropathy, optic neuropathies caused by tumors, and papilledema.     

Bilateral optic nerve meningioma. Case report

A case of bilateral optic nerve meningioma is reported. The onset of the clinical symptoms, at age 27, resembled unilateral optic neuritis with papilledema, leading to bilateral amaurosis with optic atrophy 4 years later. Skull X-ray revealed a "blistering' type of bone reaction. In the carotid angiogram, the ophthalmic artery appeared quite enlarged and displaced. The CT scan showed a fusiform enlargement of both optic nerves. Pathological diagnosis was based upon an optic nerve biopsy. Including the present case, only 12 instances of bilateral optic nerve meningioma have been reported.     

Deglutition in patients treated with reconstructive laryngectomy]

BACKGROUND: Chronic papilledema may lead to irreversible damage of optic nerve fibers. To preserve visual functions, a decompression of the optic nerve is recommended by means of a fenestration of the optic nerve sheath. In this study long-term results after optic nerve sheath fenestration in patients with idiopathic pseudotumor cerebri are reported. PATIENTS AND METHODS: 14 patients were re-examined 15 to 145 months (mean 62 months) after a fenestration of the retrobulbar optic nerve sheath on 23 eyes by a transconjunctival approach. Surgery was done to treat progressive visual loss or in severe obscurations. The patient's symptoms, visual acuity, visual fields, and ophthalmoscopic findings of the optic nerve head and the central fundus were compared to the preoperative status. RESULTS: Re-examination revealed improvement or stabilisation of objective and subjective findings in 17 eyes, one of them was operated on the more involved contralateral side only. Six eyes showed a recurrence of the papilledema without a functional change for the worse after an interval of 7 to 121 months. Three eyes of two patients ended up with optic atrophy and extensive visual loss. Preoperatively, these eyes had shown cotton wool spots in the optic nerve head and a rapid deterioration of vision. CONCLUSIONS: Fenestration of the retrobulbar optic nerve sheath can prevent further visual loss in most patients with pseudotumor cerebri, unless the eye has already become nearly blind. Postoperatively, ophthalmological controls are necessary at regular intervals because relapses after successful surgery can occur after months or years.     

Retinal nerve fiber layer changes and visual field loss in idiopathic intracranial hypertension

PURPOSE: The authors retrospectively analyzed changes in the retinal nerve fiber layer in patients with idiopathic intracranial hypertension and studied their relation to visual field loss to determine the clinical usefulness of retinal nerve fiber analysis in the clinical management of patients with papilledema. METHODS: Retinal nerve fiber layer photographs and visual fields from 36 eyes of 21 patients with papilledema due to idiopathic intracranial hypertension were analyzed for abnormalities in a masked fashion. RESULTS: Nerve fiber layer changes were found in 67% of eyes studied. Superior areas within the nerve fiber layer were affected 5.4 times more frequently than inferior regions. Visual field loss was more prevalent in eyes with diffuse nerve fiber layer loss (89%) than in eyes with slit defects (29%). The location of the nerve fiber layer changes correlated with corresponding areas of visual field loss. Nerve fiber layer changes were as common in mild to moderate as in atrophic papilledema; however, slit defects predominated in patients with mild to moderate papilledema, and diffuse loss predominated in atrophic papilledema. CONCLUSIONS: Changes in the retinal nerve fiber layer observed in patients with idiopathic intracranial hypertension provide objective information regarding the status of their optic nerve and may improve their clinical management.     

Evaluation of the retinal nerve fiber layer

In normal eyes, the retinal nerve fiber layer (RNFL) is usually best visible in the inferior temporal part of the fundus, followed by the superior temporal region, the nasal superior region and the nasal inferior region. This distribution correlates with the configuration of the neuroretinal rim, the diameter of the retinal arterioles, the location of the foveola, and the lamina cribrosa morphology. With increasing age, the RNFL visibility decreases diffusely without preferring special fundus regions and without the development of localized defects. With all optic nerve diseases, the visibility of the RNFL is decreased in addition to the age-related loss, in a diffuse and/or a localized manner. The localized defects are wedge-shaped and not spindle-like defects, running toward or touching the optic disk border. Typically occurring in about 20% of all glaucoma eyes, they can be found also in other ocular diseases, such as optic disk drusen, toxoplasmotic retinochoroidal scars, longstanding papilledema or optic neuritis due to multiple sclerosis. Since they are not present in normal eyes, they almost always signify an abnormality. RNFL evaluation is especially helpful for early glaucoma diagnosis and in glaucoma eyes with small optic disks. In advanced optic nerve atrophy, other examination techniques, such as perimetry, may be more helpful for following optic nerve damage. Considering its great importance in the assessment of optic nerve anomalies and diseases and taking into account the feasibility of its ophthalmoscopic evaluation using green light, the retinal nerve fiber layer should be examined during any routine ophthalmoscopy.     

Neuro-ophthalmology, for the pediatrician

The pediatrician needs to develop some skills in evaluating afferent visual functions and ocular motor abnormalities. He must know some fundamental neuro-ophthalmologic facts to prevent his patients from undergoing unnecessary diagnostic and surgical procedures. In addition, he needs to understand the fundamentals of strabismus and amblyopia, which are briefly considered in this article but are explored thoroughly elsewhere in this issue of PEDIATRIC ANNALS. First, the most common type of nystagmus in children is congenital nystagmus. These children often have a head turn or tilt. Also, it should be remembered that numerous drugs may cause nystagmus. Second, any child with a head turn or tilt must be considered to have some ocular motor abnormality until a complete ophthalmologic evaluation has eliminated this possibility. In addition, before a child is considered to have an isolated sixth-nerve palsy, Duane's retraction syndrome should be looked for. Also, before an inferior oblique palsy is diagnosed, a Brown's tendon sheath syndrome should be considered. Thus, any ocular muscle abnormality deserves an ophthalmologic evaluation. Third, when bilateral swollen optic nerves are noted as an incidental finding, drusen of the optic nerve head should be suspected. A unilateral swollen disk with decreased visual acuity and a Marcus Gunn pupil should bring to mind a papillitis, which is a local inflammatory condition of the optic nerve head. Remember, papilledema is a bilateral condition secondary to increased intracranial pressure with normal vision. Children with papilledema usually have other signs of increased intracranial pressure. If the article has convinced you of only two facts -- that head tilts and turns are usually ocular in origin, and that bilaterally swollen optic nerves in an otherwise asymptomatic child may be optic nerve drusen -- it has accomplished its purpose.     

Musculocutaneous nerve palsy following traumatic spinal cord injury

Whipple's disease is a multisystem bacterial disease usually characterized by malabsorption, diarrhea and polyarthritis. Ocular manifestations include uveitis, vitreitis, retinis, myositis, papilledema and optic atrophy. We report a case of a chronic bilateral vitreitis in a 63 year-old man who had been treated for a Whipple's disease with gastrointestinal involvement 30 years before. The jejunal biopsy was negative but the polymerase-chain-reaction (PCR) revealed the presence of Trophyrema Whippelii in the vitreous prelevement of both eye. This new, original strategy of PCR is specific and more sensitive than histological diagnosis.     

Psychiatrist and nonphysician mental health provider staffing levels in health maintenance organizations

OBJECTIVE: To describe a patient with a paraneoplastic cerebellar syndrome and optic neuritis with circulating anti-CV2 antibodies and clinical improvement after excision of a small cell lung carcinoma. DESIGN: Report of a case. SETTING: A 62-year-old man simultaneously developed a severe cerebellar syndrome and a bilateral optic neuritis predominantly in the left eye (visual acuity, 20/25 in the right eye; < 20/400 in the left eye; and bilateral swelling of the optic discs). MAIN OUTCOME AND RESULTS: Anti-CV2 antibodies, recently described as associated with paraneoplastic neurological syndrome, were detected in the patient's serum sample. These antibodies were demonstrated to react with the cytoplasm of a subpopulation of oligodendrocytes in the white matter of rat brain in the cerebellum, brainstem, spinal cord, and optic chiasm. The patient was found to have a small cell lung carcinoma, which was removed. After excision of the tumor, the cerebellar syndrome improved dramatically and the papilledema disappeared despite aftereffects of the optic neuritis. CONCLUSIONS: These findings were consistent with the diagnosis of a paraneoplastic neurological syndrome, although both optic neuritis and remission of the cerebellar syndrome are uncommon patterns of paraneoplastic syndromes. CV2 antigen expression by the oligodendrocytes of the cerebellum, brainstem, spinal cord, and optic chiasm correlated with the clinical syndrome observed in our patient. However, the precise pathophysiological role of anti-CV2 antibodies is still unknown.     

The MRI appearance of the optic nerve sheath following fenestration for benign intracranial hypertension

Optic nerve fenestration is carried out in cases of severe benign intracranial hypertension. This study aimed to monitor the optic nerve sheath appearances and orbital changes that occur following this procedure. The eight patients were all female with an average age of 37.3 years and a range of 20-58 years. The duration of symptoms was 2-6 years. Symptoms included headaches, diplopia and visual obscurations. Examination revealed severe papilledema. All investigations, including MRI, biochemical and immunological tests, were negative. Patients had fenestration of a 2 mm x 3 mm segment of the medial aspect of the optic nerve sheath. Imaging was obtained with a 1 T MRI machine using a head coil. Coronal, axial and sagittal 3 mm contiguous sections using STIR sequences with TR 4900 ms, IT 150 ms and TE 60 ms were obtained. Five patients showed clinical improvement. The post-operative MRI findings in four of these included a decreased volume of cerebrospinal fluid (CSF) around the optic nerve sheaths and a localized collection of fluid within the orbit. There were no MRI changes in the three patients with no clinical improvement. Decreased CSF volume around the optic nerve and a fluid collection within the orbit may indicate a favorable outcome in optic nerve fenestration.     

Acute esotropia may be a presenting sign of intracranial neoplasm

Here we report eight consecutive patients with intracranial neoplasm at different ages clinically presenting with acute esotropia. Among these patients, two had divergent paresis, four had bilateral abducens paresis and the other two had unilateral abducens nerve paresis. All of these patients have intracranial neoplasm demonstrated by neuroimaging studies. Besides of acute esotropia, four had papilledema, one had optic atrophy, and three had associated other neurological signs. Of the eight patients, three patients are children, and the other five are adults. In the adult group, four patients are less than 45 years old. In conclusion, acute esotropia may be an early presenting sign of brain tumor and careful evaluation of associated clinical symptoms/signs is mandatory, especially in the young age group.     

Bilateral disc edema in retinitis pigmentosa

Retinitis pigmentosa (RP), one of the most common forms of hereditary retinal degeneration, is characterized by night blindness and constricted visual fields. In addition to bone spicule pigmentation, other ocular findings may include posterior subcapsular cataracts, cystoid macular edema, and hyaline bodies or drusen of the optic nerve. Rarely, optic nerve head (ONH) edema has been reported to be associated with RP. A 44-year-old white male with RP and neurosensory hearing loss (Usher's syndrome type II) presented to our clinic for routine examination. A dilated fundus examination revealed bone spicule pigmentation, vessel attenuation, several flame hemorrhages on or adjacent to the nerves, and ONH edema in the right eye. B-scan ultrasonography revealed drusen of the right ONH but not of the left. Late stage fluorescein angiography showed hyperfluorescence and dye leakage from both optic discs which was more pronounced in the right eye than the left. Computed tomography (CT) of the head and orbits and cerebrospinal fluid (CSF) examination by lumbar puncture were normal. The differential diagnosis of bilateral ONH edema in this case included ONH drusen or papilledema secondary to increased intracranial pressure. This patient was found to have RP with asymmetric, bilateral ONH edema of unknown cause. One theory regarding the cause of the ONH edema is disc vessel leakage secondary to an inflammatory reaction caused by rapid photoreceptor and retinal pigment epithelium (RPE) degeneration.     

Coexistence of migraine and idiopathic intracranial hypertension without papilledema

Eighty-five patients with refractory transformed migraine type of chronic daily headache (CDH) had spinal tap as a part of diagnostic work-up. Twelve had increased intracranial pressure without papilledema, transient visual obscurations, or visual field defects. The headache profile of these 12 patients was not different from that of transformed migraine type of CDH. Acute headache exacerbations responded to specific antimigraine agents such as ergotamine, dihydroergotamine (DHE), and sumatriptan, whereas prophylactic antimigraine medications were only partially helpful. Addition of agents such as acetazolamide and furosemide, after the diagnosis of increased intracranial pressure, resulted in better control of symptoms. These observations suggest a link between migraine and idiopathic intracranial hypertension that needs further research. In refractory CDH with migrainous features, a spinal tap to exclude coexistent idiopathic intracranial hypertension without papilledema may be indicated.     

Quantification of optic nerve blood flow changes using magnetic resonance imaging

PURPOSE: To evaluate the possibilities of magnetic resonance imaging (MRI) for quantification of pharmacologically induced changes in optic nerve microcirculation. METHOD: T2-weighted MRI sequences were used to image the eye, optic nerve, and frontal cortex in rats. Two sets of control images before and one set during Gd (DTPA) infusion were recorded. Blood flow values for two regions of the optic nerve (an anterior part, including the optic nerve head, and a more posterior part) and the frontal cortex were calculated by image analysis from the change in signal intensity, as already reported for cerebral blood flow. For each rat, a control experiment before drug administration and a second experiment 30 minutes after subcutaneous injection of either placebo (n = 7), timolol (n = 7), or SDZ GLC-756, a dopamine D-1 antagonist and D-2 agonist (n = 7), were carried out in a double-blind fashion. RESULTS: Mean basal blood flow values were found between 29.4 and 45.6 ml/100 g per minute in the anterior part of the optic nerve, 38.3 and 42.9 ml/100 g per minute in the posterior part of the optic nerve, and 68.0 and 75.0 ml/100 g per minute in the frontal cortex. Placebo and timolol did not cause significant changes. SDZ GLC-756 significantly increased blood flow by 238% +/- 65% in the anterior part and by 87% +/- 40% in the posterior part of the optic nerve. CONCLUSIONS: These results suggest that MRI provides quantification of optic nerve blood flow and that dopaminergic substances increase optic nerve blood flow.     

No latent chromosome damage in oxygen-exposed premature neonates

BACKGROUND: Benign intracranial hypertension is due to an increased intracranial pressure of unknown cause. The initial symptoms, complications and associations with medical conditions are discussed. CASE REPORT: A 6-year-old girl developed symptoms of benign intracranial hypertension following reduction of oral corticosteroid therapy. Laboratory studies and head-computed tomographic scan were normal. Examination of the optic discs showed bilateral papilledema and the cerebrospinal fluid pressure was increased. The patient was given prednisone therapy 1 mg/kg daily initially, associated with acetazolamide, and removal of 25 mL of cerebrospinal fluid. All the symptoms resolved and the treatment was gradually decreased. The child developed no further visual failure. CONCLUSION: Benign intracranial hypertension with the risk of permanent visual loss is a complication underrecognized in children. All patients receiving large doses of the corticosteroids who complain of headache or blurring vision, particularly following a reduction of corticosteroid dosage, should have an ophtalmoscopic examination to exclude this complication.     

Association of xerophthalmia, flecked retina, and pseudotumor cerebri caused by hypovitaminosis A

PURPOSE: To report an unusual case of hypovitaminosis A with bilateral papilledema and flecked retina that regressed after restoration of vitamin A deficiency. METHOD: Case report. A 27-year-old woman had undergone a biliopancreatic bypass for obesity in 1990. In 1995, she presented with bilateral xerophthalmia, bilateral papilledema, and bilateral flecked retina. RESULTS: Laboratory tests demonstrated low serum levels of vitamin A (0.8 micromol/l) and vitamin E (8.54 micromol/l). Xerophthalmia, papilledema, and flecked retina disappeared after restoration of normal vitamin A blood levels. CONCLUSION: Hypovitaminosis A after biliopancreatic bypass for obesity may be associated with xerophthalmia, pseudotumor cerebri, and flecked retina.     

Paraganglioma of the filum terminale: case report and literature review

A rare case of a paraganglioma of the filum terminale is reported. The patient presented with headache and papilledema. Magnetic resonance imaging demonstrated enlarged ventricles of the brain and a well-demarcated intradural spine tumor. After resection of the tumor, the papilledema and headaches resolved, and the ventricles returned to normal size. Pathologic examination revealed a paraganglioma of the filum terminale. We present this rare case and review the literature of spinal paragangliomas as well as the association of spinal tumors with hydrocephalus.     

Primary spinal leptomeningeal gliomatosis presenting visual disturbance as the initial symptom: case report

We report a case of diffuse leptomeningeal gliomatosis which spread from the cervical to the sacral spine. A 60-year-old man was admitted with visual disturbance due to papilledema. Magnetic resonance imaging revealed holocord leptomeningeal gliomatosis without a definite intraparenchymal lesion, and the patient's neurological examination was unremarkable except for papilledema. Intracranial hypertension secondary to spinal tumor is well known but unusual, and the mechanism is still unclear. In our case, an elevated protein concentration of cerebrospinal fluid is suggested as the cause of intracranial hypertension.     

How to use Chlamydia antibody testing in subfertility patients

OBJECTIVE: To determine the role of weight loss in the treatment of idiopathic intracranial hypertension (IIH) in obese women. METHODS: Chart review of 250 patients with suspected IIH revealed 58 women who met our criteria, did not undergo early surgical intervention, and had adequate documentation of visual status, papilledema, and weight at the baseline evaluation and at 6 months or longer. Patients were divided into two groups based on whether weight loss > or = 2.5 kg occurred during any 3-month interval. Papilledema grade, visual acuity, and visual field grade at 6 months or longer and the time to improve each were recorded. RESULTS: Mean time in months to improve one grade for papilledema and visual field in one eye was 4.0 versus 6.7 (p = 0.013) and 4.6 versus 12.2 (p = 0.032), respectively, for the 38 patients with weight loss compared with the 20 patients with no weight loss. Papilledema resolved in 28/38 with weight loss (mean, 7.6 months) and 8/20 without weight loss (mean, 10.2 months; p = 0.352). There were no differences in final visual acuity or visual field between the two groups, but the papilledema grade was slightly better in the worst eye in each patient at baseline in the weight loss group (p = 0.03). CONCLUSIONS: Weight reduction is associated with more rapid recovery of both papilledema and visual field dysfunction in patients with IIH compared with those who do not lose weight.