"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.

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.

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.

Progressive outer retinal necrosis in immunocompetent patients treated initially for optic neuropathy with systemic corticosteroids.

PURPOSE: To report two cases of progressive outer retinal necrosis occurring in immunocompetent individuals after treatment with corticosteroids for presumed optic neuropathy. DESIGN: Observational case report. SETTING: University-based tertiary eye hospital. METHODS: Retrospective review of existing clinical records. RESULTS: Two patients were treated empirically with systemic corticosteroids for suspected inflammatory papillopathy. Subsequently, both were diagnosed with necrotizing herpetic retinitis with features of progressive outer retinal necrosis. Anterior chamber paracentesis confirmed varicella-zoster infection. Both patients were human immunodeficiency virus negative; one patient with rheumatoid arthritis was taking etanercept. Both became completely blind in one eye despite intensive treatment with antiviral medication intravenously and intravitreally. CONCLUSIONS: Progressive outer retinal necrosis is not confined to patients with underlying severe immunodeficiency, such as acquired immune deficiency syndrome. Initial treatment of acute, unexplained vision loss with systemic corticosteroids may lead to catastrophic visual loss in patients with evolving necrotizing herpetic retinopathy.

Optic disk anomalies [abstract]

There are a number of development defects of the nerve that result in disk anomalies that present as diagnostic and management problems. These include pseudo-papilloedema, disk hypoplasia; crescent anomalies; and partial colobomas. These will be discussed in clinical context, especially "misdiagnosis". (AU)

The Pupil

Pupil examination is a lost art and too often ignored. The spectrum of pupillary test and abnormalities will be reviewed, including: pupillary light testing; afferent defects; differential diagnosis of simple anisocoria; the clinical dilemma of the "dilated pupil"; tonic pupil syndromes; Horner's syndrome and other causes of relative miosis; pharmacological testing procedures. A brief review of pertinent anatomy will be included. (AU)

Common optic nerve disorders [abstract]

A clinically oriented overview of common optic nerve disease: the differential diagnosis, clinical findings, diagnostic work-up, and rational for therapy. Included will be: congenital anomalies mistaken for acquired neurologic disease; differential diagnosis of the "swollen disk"; ischaemic optic neuropathies, common and uncommon; optic neuritis with and without multiple sclerosis; heredofamilial optic atrophies. This summary is designed to provide an up-to-date review for the general practitioner in opthalmology, neurology or internal medicine. Necessary and unnecessary diagnostic procedures will be stressed. (AU)