Systemic Amyloidosis and Extraocular Muscle Deposition.

Isolated amyloid deposition in an extraocular muscle is a rare event but can be a presenting feature of systemic amyloidosis. A 67-year-old woman with an acquired exotropia and hypertropia was found to have unilateral diffuse extraocular muscle enlargement on magnetic resonance imaging. Owing to the progressive nature of her strabismus and the negative laboratory testing for thyroid disease, she underwent an extraocular muscle biopsy that revealed amyloid deposition. Further workup demonstrated a monoclonal gammopathy consistent with systemic amyloidosis. This case demonstrates the need to consider amyloidosis in the differential diagnosis of patients presenting with an atypical acquired strabismus. We review other reports of isolated amyloid deposition in extraocular muscles and its association with systemic amyloidosis, emphasizing the importance of the ophthalmologist in the early recognition of this disease to prevent irreversible, life-threatening end organ damage.

Optic nerve sheath fenestration for the treatment of papilledema secondary to cerebral venous thrombosis.

A 16-year-old adolescent girl with multiple risk factors for thrombosis presented with acute onset of headache, decreased vision, and papilledema. Evaluation demonstrated cerebral venous thrombosis (CVT) involving the left transverse and sigmoid sinuses and left internal jugular vein. Following bilateral optic nerve sheath fenestration (ONSF), she experienced improvement in vision and resolution of papilledema. In selected cases, ONSF is an effective surgical option for the treatment of papilledema due to CVT after medical treatment has failed.

The troubles I've seen.

This Hoyt lecture is composed of 2 topics. First, a series of patients with idiopathic intracranial hypertension (IIH) is presented, emphasizing the importance of magnetic resonance venography (MRV). Study of the cerebral venous sinuses in IIH may demonstrate focal stenosis or venous gaps and represent a manifestation of elevated intracranial pressure. Conversely, the clinical picture of IIH may occur following cerebral venous sinus thrombosis, and MRV may be essential in establishing this diagnosis. In the future, evaluation of flow in the cerebral venous sinuses may play an important role in determining the potential for visual failure. Second, I will review patients with visual cognitive changes, which often go unrecognized. These patients suffer from visuoperceptual disturbances, recognizing parts of visual scenes but not the entire picture and are unable to comprehend their visual environment. These findings are often part of the syndrome of posterior cortical atrophy characterized by parieto-occipital atrophy, enlargement of the atrial portion of the ventricular system, and diminished metabolic activity in the posterior portion of the brain demonstrated with positron emission tomography.

Delayed optic nerve complications after proton beam irradiation.

We present two cases of delayed optic nerve complications following proton beam radiotherapy. Patient 1 received radiotherapy after surgery for clival chordoma and developed sequential radiation optic neuropathy at 5 and 9 months. Patient 2 developed optic nerve hemorrhage post radiotherapy for optic nerve sheath meningioma. These two cases of blindness demonstrate the risks associated with proton beam radiotherapy.

Reversal of cilioretinal artery occlusion with intra-arterial tissue plasminogen activator.

PURPOSE: To present the reversal of a cilioretinal artery occlusion with the use of intra-arterial tissue plasminogen activator. OBSERVATIONS: A monocular 74 year old male presented with a cilioretinal artery occlusion. Treatment with intra-arterial tissue plasminogen activator 7 hours after the onset of symptoms led to a complete restoration of vision. CONCLUSIONS AND IMPORTANCE: Early intervention with tissue plasminogen activator reversed acute onset loss of vision from cilioretinal artery occlusion.

Antiphosphospholipid syndrome presenting with amaurosis fugax and cotton wool spots.

PURPOSE: To describe the importance of considering vaso-occlusive disease on the differential diagnosis of a patient presenting with amaurosis fugax (AF) and unilateral cotton wool spots (CWS). OBSERVATIONS: A 69-year-old female with history of obesity, hyperlipidemia and recent orthopedic surgery, presented with 3 days of worsening monocular AF and CWS in the right eye. She was diagnosed with antiphosphospholipid syndrome based on positive serologic testing for antiphosphatidylserine IgM, anticardiolipin IgM. The patient was treated with lipid lowering medication, long-term aspirin, and has followed a weightloss and physical therapy program under medical supervision. The CWS resolved and AF symptoms have not recurred. CONCLUSIONS AND IMPORTANCE: Antiphospholipid syndrome can be considered in the differential diagnosis of patients presenting with AF, assymetric CWS, and/or rapid progression of symptoms.

Transvenous n-butyl-cyanoacrylate infusion for complex dural carotid cavernous fistulas: technical considerations and clinical outcome.

BACKGROUND AND PURPOSE: Endovascular transvenous embolization has been advocated as the treatment technique for dural carotid cavernous fistulas (dCCFs). Most centers use platinum coils primarily. The purpose of this study was to evaluate the technical aspects, efficacy, and safety of transvenous n-butyl cyanoacrylate (n-BCA) infusion in dCCFs as a primary alternative or adjunct to coil embolization. METHODS: We retrospectively evaluated 14 patients with dCCFs who were treated at this institution from 1999 to 2004 by using n-BCA infusion alone or in combination with coils. The efficacy of treatment and safety aspects were studied in dCCFs of Barrow type B (4/14), C (2/14), and D (8/14). Six patients were treated with transvenous n-BCA infusion alone in the cavernous sinus, 7 with a combination of transvenous n-BCA and coil embolization, and one with transvenous n-BCA combined with transarterial polyvinyl alcohol (PVA)-particle embolization of the feeding arteries. RESULTS: An angiographic obliteration and clinical cure was achieved in all patients. Technical complications were nonsymptomatic and included spillage of an n-BCA droplet into a middle cerebral artery branch retrograde through the arteriovenous fistulas in one patient and perforation of the inferior petrosal sinus during microcatheter placement in another. A third patient developed temporary palsy of the sixth cranial nerve a few days after the treatment. CONCLUSION: In this small series, the use of n-BCA either alone or in conjunction with detachable coils was a safe and effective technique for the treatment of symptomatic patients presenting with complex dCCFs.

Infraorbital nerve palsy: a complication of laser in situ keratomileusis.

PURPOSE: To report infraorbital nerve dysfunction after laser in situ keratomileusis. DESIGN: Observational case report. METHODS: Neuro-ophthalmologic examination with brain and orbital magnetic resonance imaging (MRI) and orbital computed tomography (CT). RESULTS: During laser in situ keratomileusis, two healthy women, aged 42 and 46 years, experienced acute onset of sharp ipsilateral cheek pain. Both cases occurred during manipulation of the eyelid speculum. Postoperatively, ipsilateral numbness and tingling or pain of the upper cheek was reported, and examination showed decreased sensation in the distribution of the infraorbital nerve. In both cases, brain and orbit MRI and orbit CT were normal. Both patients were managed medically. In one patient, mild symptoms persisted 1 year postoperatively, and in the second patient, moderate discomfort persisted 8 months postoperatively. CONCLUSION: Infraorbital nerve palsy is a potential complication of laser in situ keratomileusis. Symptoms improve but may persist.

"Idiopathic" intracranial hypertension caused by venous sinus thrombosis associated with contraceptive usage.

BACKGROUND: Cerebral venous sinus thrombosis (CVST) is a life-threatening condition that may present with symptoms and signs identical to idiopathic intracranial hypertension (IIH). Both conditions consist of increased intracranial pressure (ICP); however, IIH shows no evidence of contributory intracranial pathology. Oral hormonally based contraceptive usage has been associated with both conditions as well. Because disc edema often occurs in CVST, and IIH and is evident in other sight- and life-threatening conditions such as intracranial mass lesions, neuroimaging and other ancillary testing must be conducted for definitive diagnosis. CASE: An 18-year-old white woman with IIH previously diagnosed presented with headaches and severe visual loss in the left eye. Bilateral disc edema with macular edema in the left eye (O.S.) was present. Threshold perimetry found bilaterally enlarged blind spots as well as a central scotoma O.S. Her medical history was significant for a right transverse, sigmoid and jugular siphon thrombosis secondary to a hypercoagulable state and associated with her use of oral birth control medication. Subsequent magnetic resonance venography (MRV) found the presence of CVST. Despite anticoagulation therapy and acetazolamide, she had severe, nonresolving papilledema. Treatment with oral prednisone was initiated. She recovered full visual fields and excellent visual acuity. CONCLUSION: Because of the similarity in clinical presentation of CVST and IIH, it is important to differentiate distinguishing characteristics of these diseases for correct diagnosis and prompt treatment.

Retinopathy in patients with diabetic ophthalmoplegia.

PURPOSE: To review the clinical characteristics, prevalence, and severity of retinopathy in diabetics with cranial nerve (CN) 3, 4, and/or 6 palsies, and to determine the relationship between type and duration of diabetes mellitus (DM), presence of retinopathy, and occurrence of CN palsy. DESIGN: Retrospective, comparative cohort study. PARTICIPANTS: Chart reviews of 2229 patients with CN 3, 4, and/or 6 palsies were performed at the Bascom Palmer Eye Institute (BPEI) from January 1991 through December 1997 and at the Dean A. McGee Eye Institute (DMEI) from January 1994 through July 2001. A total of 306 patients qualified for the study group. The Wisconsin Epidemiologic Study of Diabetic Retinopathy (WESDR) was used as a control. METHODS: Demographic and clinical data were extracted to determine characteristics of patients with diabetic ophthalmoplegia. The subsets of data regarding type of DM and level of diabetic retinopathy in the study population were compared with the WESDR control data for statistical analysis. MAIN OUTCOME MEASURES: The prevalence of diabetic retinopathy in patients with diabetic ophthalmoplegia. RESULTS: Of 2229 patients at both institutions with ocular motor CN palsy, 306 (13.7%) were associated with DM. The frequency of CN involvement was 6 (50.0%), 3 (43.3%), and 4 (6.7%). There was a total of 12 patients (3.9%) with consecutive palsies and 8 patients (2.6%) with simultaneous palsies (5 unilateral and 3 bilateral). At both institutions, the prevalence of retinopathy controlling for duration of DM was lower in both insulin-dependent DM (IDDM) and non-IDDM (NIDDM) type II diabetics as compared with controls (BPEI, P = 0.009 and P = 0.005; DMEI, P = 0.004 and P = 0.29). When data from both locations were combined, the difference was even more significant (IDDM, P = 0.001 and NIDDM, P = 0.006). There were no significant differences between the two institutions in gender, type or duration of DM, age at presentation, or frequency of CN involvement. CONCLUSIONS: Diabetic ophthalmoplegia most commonly involves CN 3 and 6, with relative sparing of CN 4. Multiple cranial nerves are affected simultaneously in 2.6% of cases, and consecutive palsies occurred in 3.9% of cases. Type II diabetics with ocular motor CN palsy have significantly less diabetic retinopathy than do controls. This may imply a different pathophysiologic mechanism for these two microvascular complications of DM.