Faculty Bibliography

Idiopathic sclerosing inflammation of the orbit is a distinct form of orbital inflammatory disease characterized by slow and relentless involvement of orbital structures. It is this insidious and relentless course that makes distinction from neoplastic lesions clinically difficult. We report the case of a patient with a several-week history of headache and decreased vision that was originally thought to represent an optic nerve sheath meningioma, based on clinical and radiographic evaluation. Subsequent histopathology from an optic nerve biopsy, however, was more consistent with optic nerve glioma. Eventually, pathologic examination of whole sections through the optic nerve was required to establish and confirm the actual diagnosis of sclerosing orbital inflammation.

We report a case of a patient who developed a left posterior cerebral artery aneurysm 5 years after balloon occlusion of the right internal carotid artery for a giant cavernous aneurysm. The location of the new aneurysm was outside of the primary collateral pathways to the contralateral, proximally occluded, anterior circulation, illustrating the complexity of hemodynamic factors contributing to the development of intracranial saccular aneurysms. The appearance of an aneurysm in this setting supports the hypothesis that degenerative factors and hemodynamic stresses are important in the etiology of intracranial aneurysms

BACKGROUND: Despite the plenitude of reports concerning partial or complete third nerve palsies, especially as presenting symptoms with posterior communicating artery (PCoA) aneurysms, we present a patient with an unusual variation. CASE DESCRIPTION: A 66-year-old woman presented with progressive right-sided headaches and diplopia and was found to have a partial, pupil-sparing third nerve palsy. A small right-sided PCoA aneurysm, nearly indistinguishable from an infundibulum, was identified on magnetic resonance angiography and subsequent digital subtraction angiography. The third nerve palsy improved before surgical repair of the aneurysm. RESULTS: Microsurgical exploration revealed a small PCoA aneurysm, which was tethered to the third nerve by arachnoid adhesions. Adhesions were lysed and the aneurysm was repaired sparing the PCoA and its branches. The patient's third nerve function recovered completely postoperatively. CONCLUSIONS: Even a very small PCoA aneurysm may present with an improving, pupil-sparing partial third nerve palsy. Selection of patients for imaging studies should take this unusual variant into consideration. We describe the anatomy and potential mechanisms of this pupil-sparing third nerve palsy

Treatment decisions for ocular myasthenia gravis (OMG) should be based on symptomatology. Local, nonpharmacologic treatment of ptosis or diplopia is successful in relatively few patients, and the majority of patients require drug therapy for satisfactory resolution of their symptoms. Response to anticholinesterase agents is variable, but should always be used as the first-line agent or adjunctive therapy in the treatment of OMG. It is unknown whether early treatment of OMG with corticosteroids or other immunosuppressive agents prevents or delays the development of generalized myasthenia, although some observations support this speculation. Corticosteroids are usually necessary for adequate improvement of ophthalmoplegia or ptosis. Surgical correction of ptosis or ocular motility deficits is not recommended for most patients with OMG, because of the fluctuating nature of the deficits and the high rate of recurrent deficits following surgery. All patients with OMG should be screened for the presence of thymus tumors, and thymectomy is recommended for all patients with a thymoma. Some patients with OMG who do not harbor a thymus tumor may also benefit from thymectomy.

The Controlled High Risk Avonex Multiple Sclerosis Study (CHAMPS) tested whether interferon beta la (Avonex) treatment would benefit patients who had experienced a first acute demyelinating event involving the optic nerve, brain stem/cerebellum, or spinal cord, and who displayed MRI brain signal abnormalities that have previously predicted a high likelihood of future MS-like events. The study randomized 383 patients into an Avonex-treated and a placebo-treated group; both groups received intravenous methylprednisolone 1 gm/d followed by prednisone 1 mg/kg for 11 days. The Avonex-treated group demonstrated a 44% reduction in the 3-year cumulative probability of developing clinically definite multiple sclerosis (rate ratio 0.56, 95% confidence interval 0.38 to 0.8; P = 0.002). At 18 months, treatment with Avonex was associated with a significant reduction of new T2 lesions, gadolinium enhanced lesions and T2 lesion volume. Among placebo-treated patients, 82% had developed a new subclinical MRI signal abnormality by the eighteenth month after study entry. Treatment benefit was observed irrespective of the qualifying event. The findings of this study support the efficacy of Avonex therapy in significantly reducing the 3-year likelihood of future neurologic events and worsening of the brain MRI in patients with a first acute CNS demyelinating event.

A 33-year-old man developed a complete third nerve palsy in the setting of acute bacterial endocarditis. MRI revealed an ischemic stroke in the cerebral peduncle involving the third nerve fascicle. Subsequently, he was observed to have paradoxic elevation of the eyelid on adduction and downgaze. To the authors' knowledge, this is the first demonstration of oculomotor synkinesis after an acquired, ischemic CNS lesion.

The clinical characteristics, differential diagnosis, and treatment options are presented for five different categories of neuro-ophthalmic disease. Nystagmus, optic neuritis, diplopia, pseudotumor cerebri, and temporal arteritis, are frequently encountered in neuro-ophthalmic practice. This article focuses on current therapies for these neuro-ophthalmic disorders. Potential differences in approach to pediatric versus adult patients are emphasized.

The authors report three patients with acute, chronic, and recurrent neuropathy associated with varicella zoster virus (VZV) infection but without zoster rash. CSF of all three patients contained VZV immunoglobulin G antibody, but not herpes simplex virus. In two patients, serum/CSF ratios of VZV immunoglobulin G were reduced compared to normal ratios for immunoglobulin G and albumin, and one patient also had VZV immunoglobulin M in CSF. All three patients received antiviral therapy and improved. The diagnosis of nervous system infection by VZV may be confirmed by the presence of antibody to VZV in CSF even without detectable VZV DNA.