Neuro-ophthalmological Complications of Chronic Inflammatory Demyelinating Polyradiculoneuropathy.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) can lead to prominent nerve hypertrophy, which can mimic other forms of neuropathy radiologically. Neuro-ophthalmological complications can also occur in CIDP, either at presentation or chronically in the disorder. This can also cause diagnostic difficulties. We report three cases of neuro-ophthalmological complications of CIDP: two cases of papilloedema and one case of proptosis. In all three cases cranial nerve hypertrophy was present. CIDP should be considered in neuro-ophthalmological presentations associated with cranial/spinal nerve root hypertrophy.

Isolated progressive visual loss after coiling of paraclinoid aneurysms.

BACKGROUND AND PURPOSE: The proximity of the paraclinoid segment of the internal carotid artery to the visual pathways may result in visual deficits when patients present with aneurysms in this segment. Although surgical clip ligation of these aneurysms has been the standard of care for decades, the advent of coil embolization has permitted endovascular therapy in those aneurysms with favorable dome-to-neck ratios. Although immediate nonprogressive visual loss after coil embolization of paraclinoid aneurysms has been well described, isolated progressive visual loss immediately or shortly following coil embolization, to our knowledge, has not. We have identified 8 patients who experienced progressive loss of vision, unassociated with any other neurologic deficits, developing immediately or shortly after apparently uncomplicated coil embolization of a paraclinoid aneurysm. MATERIALS AND METHODS: This study is a retrospective case series of 8 patients seen at 4 separate academic institutions. Inpatient and outpatient records were examined to determine patient demographics, previous ocular and medical history, and ophthalmic status before endovascular embolization. In addition, details of the primary endovascular therapy and subsequent surgical and nonsurgical interventions were recorded. Follow-up data, including most recent best-corrected visual acuity, postoperative course, and duration of follow-up were documented. RESULTS: Eight patients developed progressive visual loss in 1 or both eyes immediately or shortly after apparently uncomplicated coiling of a paraclinoid aneurysm. MR imaging findings suggested that the visual loss was most likely caused by perianeurysmal inflammation related to the coils used to embolize the aneurysm, enlargement or persistence of the aneurysm despite coiling, or a combination of these mechanisms. Most patients experienced improvement in vision, 2 apparently related to treatment with systemic corticosteroids. CONCLUSION: Patients in whom endovascular treatment of a paraclinoid aneurysm is contemplated should be warned about the potential for both isolated nonprogressive and progressive visual loss in 1 or both eyes. Patients in whom progressive visual loss occurs may benefit from treatment with systemic corticosteroids.

Effects of weight loss on the course of idiopathic intracranial hypertension in women.

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.

The clinical spectrum of schwannomas presenting with visual dysfunction: a clinicopathologic study of three cases.

Schwannomas (neurilemomas) are benign tumors that arise from Schwann cells in the peripheral nervous system. The most commonly involved nerves that cause neuro-ophthalmic manifestations are cranial nerves V and VIII. In this series of three women, schwannomas presented as intraconal masses that mimicked a cavernous hemangioma, a superior orbital mass transgressing the superior orbital fissure, and an expansive frontal lobe mass with clinical symptoms and signs of increased intracranial pressure. Although all three complained of visual blurring, none of our patients presented with Vth or VIIIth cranial nerve dysfunction. Histopathologic studies demonstrated well-circumscribed, encapsulated spindle-cell lesions with classic Antoni A and B patterns. Histopathologic examination is essential to confirm the diagnosis of a schwannoma that may be otherwise clinically confusing. Direct optic nerve compression, globe indentation with induced hyperopia, or increased intracranial pressure with optic nerve compromise may be responsible for visual symptoms. A multidisciplinary approach is often required because of the size and location of schwannomas.

Isolated sixth nerve palsy: an uncommon presenting sign of multiple sclerosis.

We describe three patients in whom an isolated sixth nerve palsy was the only clinical symptom or sign of multiple sclerosis (MS). Data were collected prospectively over 6 years on these three patients, who showed no other signs of brainstem dysfunction or prior symptoms; in addition. Retrospective analysis of all patients with MS and all patients with sixth nerve palsy referred to a neuro-ophthalmology service between 1982 and 1998 showed isolated sixth nerve palsy to be the presenting sign of MS in only 0.5% of these patients. MS was the cause of isolated sixth nerve palsy in 0.8% of all patients and in 1.6% of those aged 18-50 years. Although it has been previously suggested that sixth nerve palsy is a not uncommon presenting sign of MS, our results suggest it is rare.

Congenital and acquired lung hernias.

Lung hernia is defined as a protrusion of lung parenchyma beyond the confines of the musculoskeletal thorax. Lung hernias can be classified according to location (i.e., cervical, thoracic, or diaphragmatic) and etiology. Approximately 80% of reported cases of lung hernia are acquired, usually related to trauma or surgery. Symptoms are few, infrequent, and vague. A painless, local, and inconstant bulging is evident on physical examination. The diagnosis of a lung herniation is accomplished using chest radiographs in the most cases. Surgery is usually reserved for large hernias or incarcerated hernias.

Clinical characteristics, diagnostic criteria and therapeutic outcomes in autoimmune optic neuropathy.

BACKGROUND/AIMS: Autoimmune optic neuropathy (AON) is characterised by chronically progressive or recurrent visual loss associated with serological or cutaneous evidence of autoimmunity, without a defined systemic autoimmune illness. It may improve with large doses of corticosteroids alone, or in combination with immunosuppressive agents. The aim was to determine the relative effectiveness of various therapeutic regimens in AON. METHODS: All patients in this study fulfilled these criteria: (1) multiple attacks in one eye or attacks in both eyes (at least three total attacks); (2) a minimum of 12 months of neuro-ophthalmic follow-up; (3) serological abnormalities or skin biopsy changes consistent with AON; (4) no diagnosis of a defined collagen vascular disease or neurological autoimmune disease throughout follow-up, with the exception of one patient, later shown to be shown to be neuromyelitis optic antibody positive. RESULTS: Nine cases were found (female = 7, male = 2, ages 8-74). One case received corticosteroids alone, the others received corticosteroids in combination with methotrexate/gammaglobulin (n = 1), methotrexate (n = 1), gammaglobulin (n = 1), chlorambucil (n = 2), cyclophosphamide (n = 1) and azathioprine/chlorambucil (n = 1), and one received multiple combinations of agents. Criteria for diagnosis are proposed. CONCLUSION: As AON is quite rare, no formal recommendation can be made regarding its best therapy, although there is a suggestion that chlorambucil, although potentially toxic, may yield long-term remission.

Arachnoid cyst of the cavernous sinus resulting in third nerve palsy.

A 67-year-old man exhibited long-standing left third nerve palsy. Magnetic resonance imaging revealed a cystic lesion in the left cavernous sinus with signal characteristics typical of arachnoid cyst. Intradural cavernous sinus arachnoid cyst has not reported previously. Pathogenetic mechanisms are discussed.

Monocular band optic atrophy.

Band or "bow tie" optic atrophy is characterized by well-described ophthalmoscopic findings in the optic nerve and nerve fiber layer and homonymous hemianopia. It is typically associated with compressive lesions of the pregeniculate postchiasmal visual pathway or, less commonly, congenital malformations affecting the postgeniculate radiations or cortex. A unique case with band optic atrophy is described because of the unilateral visual defect. The optic atrophy is strictly unilateral and without an obvious structural lesion that could explain the optic disc damage. However, incidental cerebral gray matter cortical heterotopia may mark a congenital insult that contributed to both of these abnormal findings.

Radiologic differentiation of intraocular glass: evaluation of imaging techniques, glass types, size, and effect of intraocular hemorrhage.

OBJECTIVE: The accurate detection of intraocular foreign bodies is critically important in treating ocular trauma. The purpose of this study was to evaluate the efficacy of CT, MR imaging, and sonography in detecting seven types of glass varying in size and placed in three locations in the globe, and to examine the effect of intraocular hemorrhage. MATERIALS AND METHODS: Glass pieces were cut into 1.5-, 1.0-, and 0.5-mm pieces and implanted on the corneal surface and the anterior and posterior chambers of 42 fresh porcine eyes. Twenty-one eyes were scanned comparing axial CT, helical CT, and MR imaging. The remaining 21 eyes were scanned using helical CT and sonography after implantation in a simulated human skull before and after placement of blood in the anterior chamber (hyphema). RESULTS: Detection rates were 57.1% for helical CT, 41.3% for axial CT, and 11.1% for T1-weighted MR imaging (n = 63 fragments). Results were significant (p < 0.0001). Sonography detected 43% of glass fragments in the posterior chamber and 24% in the anterior chamber. Detectability was greatest for green beer bottle glass (90.3%) and least for spectacle glass (43.1%) (p < 0.0001). Detection rates for size ranged from 96.2% at 1.5 mm to 48.3% at 0.5 mm, which was also significant (p < 0.0001). On helical CT, anterior chamber glass was easiest to detect (91.7%) and corneal surface glass the most difficult (64.9%). Hyphema made no statistical difference (p < 0.0001). CONCLUSION: Helical CT was the most sensitive imaging modality for the detection of intraocular glass. The sensitivity of detection was unaffected by hyphema but was determined by the type of glass, size, and location.

Evaluation of image quality using 1 : 1 pitch and 1.5 : 1 pitch helical CT in children: a comparative study.

OBJECTIVE: The objective of this study was to compare the quality of 1 : 1 and 1.5 : 1 pitch helical contrast-enhanced thoracic and abdominal CT images in children who cannot cooperate for breath holding. MATERIALS AND METHODS: This is a retrospective study of 33 contrast-enhanced CT examinations in 11 children of 0-4 years of age. All children had an initial CT study using 1 : 1 pitch helical scanning followed over the next 6-36 months by one to four CT examinations with 1.5 : 1 pitch. Radiation dose with the two techniques was measured with a pencil ionization chamber. RESULTS: The two techniques provided comparable overall image quality. There was 33 % less radiation dose with 1.5 : 1 pitch helical scanning. CONCLUSION: The 1.5 : 1 pitch helical CT provides comparable quality images and a smaller radiation dose than 1 : 1 pitch in examining children aged 0-4 years.