Doxycycline and intracranial hypertension.

The authors report seven patients from six neuro-ophthalmology referral centers who developed pseudo-tumor cerebri during treatment with doxycycline. All four female patients and one of three male patients were obese. Vision was minimally affected in most patients, but two had substantial visual acuity or visual field loss at presentation. Discontinuation of doxycycline, with or without additional intracranial pressure-lowering agents, yielded improvement, but permanent visual acuity or visual field loss occurred in five patients.

Use of corticosteroid sparing systemic immunosuppression for treatment of corticosteroid dependent optic neuritis not associated with demyelinating disease.

AIM: To describe the authors' experience and that in the published literature regarding the use of corticosteroid sparing systemic immunosuppression for patients with corticosteroid dependent optic neuritis not associated with demyelinating disease. METHODS: The records of 10 patients from the authors' clinical database, and 38 patients from the published literature with corticosteroid dependent optic neuritis, were retrospectively reviewed to determine patient demographics, diagnosis, clinical course, and outcomes. These patients had recrudescence of symptoms, such as decreased vision and pain, with attempted taper of corticosteroid. Many of these patients also suffered side effects from systemic corticosteroid use such as weight gain and uncontrolled hyperglycaemia. Antimetabolites (for example, methotrexate and azathioprine), cyclosporine and/or alkylating agents (for example, cyclophosphamide and chlorambucil) were given to enable taper of corticosteroid while effectively controlling optic neuritis. RESULTS: The study included 43 women and 5 men: 17 patients with systemic lupus erythematosus, 12 patients with sarcoidosis, 3 with other systemic autoimmune diseases, and 16 with no clinically identifiable systemic association. 79% of all patients benefited from the use of systemic immunosuppression in that they had successful corticosteroid taper, control of inflammation, improvement in symptoms, and/or tolerance of adverse effects. Mild toxicity was common and 19% of patients, most often those taking cyclophosphamide, discontinued medication because of adverse effects. 24 of 28 (86%) patients on alkylators benefited clinically, while 20 of 29 (69%) patients on antimetabolites had clinical benefit. CONCLUSION: Systemic immunosuppression may be a safer and more effective treatment alternative to chronic oral corticosteroid use in cases of corticosteroid dependent optic neuritis not associated with demyelinating disease.

MRI findings in Susac's syndrome.

BACKGROUND: Susac syndrome (SS) is a self-limited syndrome, presumably autoimmune, consisting of a clinical triad of encephalopathy, branch retinal artery occlusions, and hearing loss. All three elements of the triad may not be present or recognized, and MR imaging is often necessary to establish the diagnosis. OBJECTIVE: To determine the spectrum of abnormalities on MRI in SS. METHODS: The authors reviewed the MR images of 27 previously unreported patients with the clinical SS triad, and 51 patients from published articles in which the MR images were depicted or reported. RESULTS: All 27 patients had multifocal supratentorial white matter lesions including the corpus callosum. The deep gray nuclei (basal ganglia and thalamus) were involved in 19 (70%). Nineteen (70%) also had parenchymal enhancement and 9 (33%) had leptomeningeal enhancement. Of the 51 cases from the literature, at least 32 had callosal lesions. The authors could not determine the presence of callosal lesions in 18 of these patients, and only one was reported to have a normal MRI at the onset of encephalopathy. CONCLUSIONS: The MR scans in SS show a rather distinctive pattern of supratentorial white matter lesions that always involve the corpus callosum. There is often deep gray matter, posterior fossa involvement, and frequent parenchymal with occasional leptomeningeal enhancement. The central callosal lesions differ from those in demyelinating disease, and should support the diagnosis of SS in patients with at least two of the three features of the clinical triad.

Remarkable improvement in adult Leigh syndrome with partial cytochrome c oxidase deficiency.

Leigh syndrome (LS) is a heterogeneous disorder, usually due to a defect in oxidative metabolism. Typically, signs and symptoms commence in infancy or childhood, although rare cases of adult onset have been described. Progressive deterioration is the norm. The authors describe a 22-year-old woman with partial cytochrome c oxidase deficiency who developed fulminant LS following an acute febrile illness and who subsequently showed dramatic clinical and neuroradiologic improvement.

Monocular elevator paresis in neurofibromatosis type 2.

A retrospective review of 29 consecutive unselected patients referred for neuro-ophthalmic evaluation after the diagnosis of neurofibromatosis type 2 (NF2) showed that four of them had a monocular elevator paresis. In two of the four MRI demonstrated lesions, presumed to be schwannomas, of the third nerve. These findings indicate that monocular elevator paresis is a common neuro-ophthalmic finding in NF2, which the authors suspect is probably a sign of third nerve infiltration or compression by a schwannoma.

Monocular blindness as a complication of trigeminal radiofrequency rhizotomy.

PURPOSE: To raise the awareness of the rare complication of monocular blindness resulting from radiofrequency rhizotomy for trigeminal neuralgia. METHODS: Case series from tertiary referral centers. Patients were referred after complaint of loss of vision. RESULTS: We report three patients who had trigeminal neuralgia and underwent treatment by radiofrequency rhizotomy. Each developed complete loss of vision in one eye immediately after the procedure. One patient died of an unrelated cause, and pathologic analysis revealed changes compatible with acute trauma to the right optic nerve. CONCLUSION: Our third case confirms the hypothesis that traumatic optic neuropathy after trigeminal radiofrequency rhizotomy results from malpositioning of the rhizotomy needle through the inferior orbital fissure into the orbital apex rather than the foramen ovale.

Update on ischemic stroke.

Stroke is the second most common cause of death worldwide, exceeded only by heart disease. Epidemiologic studies have greatly enhanced our understanding of the factors that increase stroke risk. There have also been many recent developments in the understanding of the various etiologies of stroke as well as specific new treatments. The characteristic sudden onset and rapid tissue damage make stroke particularly challenging to treat. The most promising therapy for acute ischemic stroke is the use of a thrombolytic agent. This has been the focus of recent large trials and remains a challenging treatment for cerebral ischemic stroke as well as for retinal artery occlusion. Because neuro-ophthalmic symptoms and signs such as vision loss and diplopia are common in patients with stroke, patients are often seen by ophthalmologists prior to their primary care physicians or neurologists. The ophthalmologist should be aware of some of the new diagnostic and therapeutic issues in the management of patients with acute ischemic stroke. This review emphasizes some of the controversial topics published during the past few years.

Visual field deficits in conventional anterior temporal lobectomy versus amygdalohippocampectomy.

OBJECTIVE: To evaluate and identify the incidence of visual field defects (VFD) after anterior temporal lobectomy (ATL) versus amygdalohippocampectomy (AH). VFD occur frequently after ATL and are usually superotemporal quadrantanopias. Little is known about the incidence of VFD after AH and this surgical method offers the possibility of a seizure-free survival without visual loss. METHODS: Patients with similar characteristics were examined. All patients had intractable seizures and mesial temporal sclerosis, small tumors localized to only the uncus, amygdala, or hippocampus, or no known pathology. Postoperative kinetic field testing using the I4e isopter on a Goldmann perimeter was performed 30 days or more after surgery. RESULTS: Of 29 patients examined, 14 underwent AH and 15 had ATL. Four of 14 AH patients (28%) had a VFD at 10 degrees from center and 11/14 (78%) had VFD at 40 degrees. One of 15 ATL patients (7%) had a VFD at 10 degrees from center and 11/15 (73%) had VFD at 40 degrees. There was no significant difference between surgery types. CONCLUSIONS: AH in this study was associated with a significant number of VFD. No significant difference was found between the frequency of VFD produced from AH and ATL. The mechanism of injury is due to direct trauma to the optic radiations while accessing the mesial temporal structures. Because all patients in the study were asymptomatic for VFD, it remains to be determined whether these VFD are clinically significant.

Fibronectin immunoreactivity in neural trauma.

The presence of extracellular matrix protein fibronectin (FN) at the site of neural trauma was examined using immunohistochemical methods. At 2, 4 and 7 days following a penetrating wound through the rat cerebral cortex, FN staining was detected in the neuropil and in non-neuronal cells adjacent to the wound. FN-stained cells were GFAP-negative and were identified as brain macrophages based on cell surface staining for CR3 complement receptor. Our findings suggest a role for FN in the repair of neural trauma.