Pseudotumor cerebri: What We Have Learned from the Idiopathic Intracranial Hypertension Treatment Trial.

Idiopathic intracranial hypertension, also known as pseudotumor cerebri, is an unexplained increase in intracranial pressure associated with permanent severe visual loss in 25% of cases and debilitating headaches. The condition is often associated with obesity. The Idiopathic Intracranial Hypertension Treatment Trial, a large, randomized, collaborative clinical trial, evaluated the efficacy of acetazolamide with weight loss versus placebo with weight loss in participants. Herein, we describe the major components of the clinical trial and discuss its shortcomings. [Full article available at http://rimed.org/rimedicaljournal-2016-05.asp, free with no login].

Contralateral botulinum injections improve drinking ability and facial symmetry in patients with facial paralysis.

PURPOSE: To report two patients who experienced improved drinking ability as well as facial appearance with contralateral injection of botulinum toxin. DESIGN: Retrospective case reports. METHODS: Two patients were treated with botulinum toxin contralateral to the VIIth nerve palsy to improve drinking ability as well as facial asymmetry. RESULTS: Botulinum toxin injections improved facial asymmetry as well as drinking ability in two patients with facial nerve palsies. CONCLUSIONS: Contralateral botulinum toxin injections improved drinking ability in two patients with facial nerve palsy.

The clinical spectrum of amiodarone-associated optic neuropathy.

PURPOSE: To describe the clinical spectrum of amiodarone-associated optic neuropathy. METHODS: Observational cases series and review. RESULTS: Of 55 cases, the median interval for onset of optic neuropathy was four months after initiating amiodarone; 88% occurred within 12 months. Seven (13%) patients were asymptomatic. Twenty-two (40%) patients presented with sudden visual loss, while 26 (47%) had insidious loss of vision. Visual acuity ranged from 20/15 to light perception; 10 (18%) patients had legal blindness with visual acuity of 20/200 or worse. Visual field loss was present in 91% of cases. Color vision loss was present in eight (40%) of 20 cases. Optic disc edema was present in 85% of cases, while eight (15%) patients had retrobulbar optic neuropathy, without evidence of disc edema. Optic disc edema resolved over a median time of three months. Five patients had raised intracranial pressure on lumbar puncture. CONCLUSION: We were able to classify amiodarone-associated optic neuropathy into five clinical categories with respect to temporal characteristics and optic nerve appearance: insidious-onset (43%), acute-onset (28%), retrobulbar (13%), increased intracranial pressure (8%), and delayed-progressive onset (8%). Most cases of optic neuropathy commenced within 12 months of initiating amiodarone, with the median onset being four months. Over 10% of patients will have no visual symptoms at the onset. Ophthalmologic examinations within the first 12 months--and particularly within four months of initiating amiodarone--should improve early detection of amiodarone-associated optic neuropathy.

Spermatic cord leiomyosarcoma metastatic to the orbit.

PURPOSE: To report a case of spermatic cord leiomyosarcoma metastatic to the orbit. DESIGN: Case report. METHODS: A 78-year-old man presented with progressive redness, proptosis, and decreasing vision of the left eye of 3 weeks' duration. Computed tomography revealed an intraconal mass (1.5 x 2 cm) inferior to and displacing the left optic nerve. RESULTS: A left lateral orbitotomy was performed, and a well-demarcated mass was removed from the surrounding tissue. A follow-up computed tomography of the chest and abdomen was consistent with metastatic disease to the lungs and liver. CONCLUSIONS: Spermatic cord leiomyosarcoma is a rare cause of metastatic lesions to the orbit.

Cause and prognosis of nontraumatic sixth nerve palsies in young adults.

OBJECTIVE: To review the causes and prognosis of sixth nerve palsies in patients who are 20 to 50 years of age. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: All patients aged 20 to 50 years with a nontraumatic sixth nerve palsy seen in a neuro-ophthalmic practice from 1994 to 2000. INTERVENTION: Diagnostic testing to determine the cause of the palsy and surgical intervention to correct persistent stable strabismus. MAIN OUTCOME MEASURES: Cause of the palsy. The patients' clinical courses were reviewed. RESULTS: The most common cause for a sixth nerve palsy in this age group was a central nervous system (CNS) mass lesion, although the most common cause for an isolated sixth nerve palsy in this age group was multiple sclerosis. Patients with a CNS mass lesion responsible for their palsy had the highest rate of nonresolution requiring strabismus surgery. CONCLUSIONS: Sixth nerve palsies are unusual in young adults, but in the practice of author (GBK) most are secondary to CNS mass lesions and, when isolated, multiple sclerosis. Deferring neuroimaging or other appropriate investigations presuming a microvascular cause for the palsy in this age group is not recommended.

Gorham disease of the orbit.

PURPOSE: To report a case of Gorham disease, also known as vanishing bone disease, of the orbit. DESIGN: Interventional case report. METHODS: A 43-year-old Caucasian woman developed a depression in her left temple, pulsation in her left upper eyelid and temple, and bulging of the left upper eyelid and headache. Computerized tomography revealed a lytic lesion in the roof of the left orbit. RESULTS: Intraoperative examination of the lesion revealed a bony defect in the left orbital roof. Surgical intervention resulted in improvement of her symptoms, and pathology failed to reveal evidence of malignancy or other benign neoplastic process. CONCLUSION: Gorham disease should be considered in the differential diagnosis of lytic bony lesions of the orbit.