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Purpose: To determine the utility of unilateral versus bilateral temporal artery biopsies in detecting the pathologic changes of giant cell arteritis., Methods: We performed a retrospective study to determine the utility of unilateral versus bilateral temporal artery biopsies in detecting the pathologic changes of giant cell arteritis. The pathologic reports of consecutive temporal artery biopsy specimens received at the Wilmer Ocular Pathology Laboratory over a 28-year period from 1968 to 1996 were reviewed., Results: Of 908 specimens examined from 758 patients, 300 specimens were simultaneous bilateral biopsies from 150 patients, 72 specimens were bilateral sequential biopsies from 36 patients, and the remaining 536 specimens were unilateral biopsies from 536 patients. Of the 186 patients who had bilateral simultaneous or nonsimultaneous biopsies, 176 had identical diagnoses on both sides. In four patients, no artery was obtained on one side. In each of the remaining six patients, five of whom had bilateral simultaneous biopsies and one of whom had bilateral sequential biopsies performed 8 days apart, the biopsy specimen from one side was interpreted as showing only arteriosclerotic changes with no evidence of active or healed arteritis, whereas the other specimen was interpreted as showing either probable healed arteritis (three specimens) or possible early arteritis (three cases). In none of the six patients with differing diagnoses between the two sides was one side interpreted as showing definite, active giant cell arteritis. Five of the six patients were subsequently determined to have giant cell arteritis, based on a combination of clinical findings, erythrocyte sedimentation rate, and response to treatment with systemic corticosteroids., Conclusions: The results of this study indicate that performing a bilateral simultaneous or sequential temporal artery biopsy improves the diagnostic yield in at least 3% of cases of giant cell arteritis, whereas in 97% of cases, the two specimens show the same findings. Thus, in patients in whom only one artery can be biopsied, there is a high probability of obtaining the correct diagnosis. Nevertheless, although the improvement in diagnostic yield of bilateral temporal artery biopsies is low, the consequences of both delayed diagnosis and treatment of giant cell arteritis as well as the use of systemic corticosteroids in patients who do not have giant cell arteritis are of such potential severity that consideration should always be given to performing bilateral temporal artery biopsies in patients suspected of having the disease.

Purpose: Acute retinal necrosis (ARN) syndrome is associated with members of the herpes virus family, but the mechanisms of infection remain unclear. The purpose of this study is to report a unique case of acute retinal necrosis syndrome associated with herpetic encephalitis in order to elucidate possible factors involved in herpetic central nervous system disease., Method: Case report., Results: A 64-year-old woman who developed acute herpes simplex virus encephalitis associated with bilateral acute retinal necrosis syndrome after craniotomy for resection of a suprasellar craniopharyngioma is presented. The results of lumbar puncture, magnetic resonance imaging, and ophthalmologic examination are consistent with herpetic infection. The origin of acute retinal necrosis syndrome and the association of acute retinal necrosis syndrome with encephalitis are reviewed., Conclusions: After craniotomy, we hypothesize reactivation of previously latent herpes simplex virus in the area of the inferior frontal lobe and optic chiasm. Reactivated virus may have migrated to the retina by axonal transport, through the optic nerves, to produce the acute retinal necrosis syndrome.

Purpose: To determine the frequency of remission in patients with essential blepharospasm and Meige syndrome., Method: Retrospective analysis of patients by means of questionnaire in an institutional setting., Results: Among 238 patients diagnosed in a single institution as having essential blepharospasm or Meige syndrome, 27 (11.3%) claimed to be symptom free without curative surgery. The average time between onset and resolution in the patients who claimed remission was 4.85 years (range, 3 months to 22 years). Among these patients, the duration of remission averaged 6.33 years (range, 1 year to 14 years)., Conclusion: Patients with essential blepharospasm or Meige syndrome have a small but definite potential for spontaneous remission of symptoms, particularly within the first 5 years after onset of symptoms.

Primary oculomotor nerve synkinesis is almost always caused by an intracavernous meningioma or aneurysm. We treated a patient who had signs of primary oculomotor nerve synkinesis from an unruptured extracavernous aneurysm located at the junction of the internal carotid artery and the posterior communicating artery. The aneurysm was successfully clipped, resulting in some improvement in ocular motility and alignment. Although most aneurysms affecting the subarachnoid portion of the oculomotor nerve cause an acute, painful oculomotor nerve paresis, such aneurysms may rarely produce painless primary oculomotor synkinesis and therefore should be considered in the differential diagnosis of this phenomenon.

We studied the clinical, histopathologic, neuroradiologic, biochemical, and genetic profile of a patient with the myo-neuro-gastrointestinal encephalopathy syndrome, a recently described multisystem mitochondriopathy characterized by blepharoptosis and ophthalmoparesis. The patient had severe intestinal pseudo-obstruction and a mixed demyelinating and axonal neuropathy. Abnormal collections of mitochondria in nerve and muscle as well as diffuse white matter disease were present. Cytochrome oxidase activity in muscle mitochondria was reduced. No mitochondrial DNA deletions were detected.

We studied three patients who developed oculomotor nerve paresis from different causes. Each patient improved somewhat over several months, after which there was no further improvement for at least six months. Although the pareses were thought to be stable after the period of no improvement, each patient subsequently had further improvement in both motility and alignment with resolution of diplopia in primary position and in more than one of the cardinal positions of gaze. Patients with oculomotor nerve paresis may improve further after an initial period of improvement followed by several months of stability.

A 25-year-old man had signs of optic nerve dysfunction. A diagnosis of optic neuritis was made and was supported by rapid resolution of signs and symptoms with use of corticosteroids. After initial negative radiologic investigations, his corticosteroid dosage was tapered, and the recurrence of his symptoms and signs prompted repeat computed tomographic scans. A suprasellar mass and a posterior-fossa mass were observed, and frontal craniotomy disclosed typical medulloblastoma, confirmed histologically. Although visual signs and symptoms in medulloblastoma have been reported secondary to optochiasmatic arachnoiditis and to papilledema, to the best of our knowledge this is the first reported case in which direct compression of the optic nerve has occurred as a result of extension of a posterior-fossa medulloblastoma. The favorable response of this compressive tumor to corticosteroids, stimulating an inflammatory optic neuritis, emphasizes the importance of reassessing any optic neuritis that follows an atypical course.

Of 15 biopsy specimens identified as showing sarcoid involving the extraocular orbital tissues, three were from patients known to have systemic sarcoidosis before biopsy. Of the other 12 patients who were not known to have systemic disease at the time of biopsy, 11 were found to have evidence of systemic sarcoidosis after the biopsy. One patient, however, had no evidence of sarcoidosis at the time of the biopsy nor did she develop sarcoidosis during a seven-year follow-up period. Our results suggest that most patients with sarcoid involving the orbit will be found to have evidence of systemic sarcoidosis, but that rare patients exist in whom the orbital lesion appears to represent an isolated process. There was no histologic difference between orbital specimens from patients with systemic sarcoidosis and the specimens from the patient with isolated sarcoid of the orbit.

Postmortem quantification of the remaining optic nerve fibers in three eyes with nonarteritic anterior ischemic optic neuropathy showed that the loss of fibers was complete in the superior half of each nerve and included loss of peripheral fibers in the other half. The fibers with the greatest resistance to atrophy were found in the middle of the nerve cross section. The pattern was similar to that found in a previous example of anterior ischemic optic neuropathy and unlike that found in glaucoma or other optic neuropathies studied in similar fashion.

A 2 and a half year-old boy with neurofibromatosis developed unilateral proptosis, decreased visual acuity, and optic disk edema. After the discovery and removal of an optic nerve glioma, the patient had ten years of excellent health until he began having headaches, nausea, and vomiting. He had papilledema in his remaining eye. At exploration, a cerebellar astrocytoma and a neuroglial hamartoma were removed. The occurrence of a glioma of right anterior visual pathway associated with other primary intracranial lesions in patients with neurofibromatosis was not previously reported.

Neovascularization of the optic disk occurred in a patient with a carotid-cavernous fistula. Treatment with scatter-type argon laser photocoagulation successfully promoted regression of the new vessels.

In most cases of isolated oculomotor nerve palsy in 30 patients under age 20 years the palsy was congenital, and aberrant regeneration was present. The most common causes of acquired oculomotor nerve palsy were blunt trauma and infectious processes, both local and systemic. Neoplasms, aneurysms, and ophthalmoplegic migraine caused the other cases. The causes of isolated oculomotor nerve palsy in childhood differ from those in adults both in nature and in frequency of occurrence.

We studied the eyes of a 10-year-old girl with retinal degeneration, acanthocytosis, and normal betalipoprotein levels. The ophthalmoscopic pattern was characterized initially by a flecked retina and later by bone-spicule formation and "bull's-eye" annular maculopathy. On ultrastructural study, the retinal pigment epithelium varied in size and contained large, round single-membrane-bound aggregates composed of complex melanolipofuscin granules. Cells that had migrated into the outer retinal layers contained similar melanolipofuscin aggregates; these cells were identified as macrophages and correlated with the flecks and macular annulus seen on ophthalmoscopy. The cells around the retinal blood vessels contained normal melanin pigment, were identified as retinal pigment epithelial cells, and correlated with the bone spicule pigmentation found on ophthalmoscopic examination.

A 53-year-old woman had a six-month history of proptosis, pain, and decreased vision in the right eye. Skull X-ray films and polytomography demonstrated a giant, mushroom-shaped osteoma originating from the roof of the maxillary sinus and invading the orbit. The tumor was removed by a lateral orbital approach with prompt resolution of proptosis and pain, and recovery of normal vision and ocular motility. This patient is one of 34 patients with 38 paranasal sinus osteomas examined here in the last 25 years. Only six patients have had orbital symptoms or signs.

We examined three patients with intracranial mass lesions that damaged the anterior visual pathway and studied this damage histologically after the patients died. Estimation of the number of surviving axons in the six optic nerves generally showed the greatest atrophy to be in the temporal sector in cross sections immediately behind the globe. More posterior to the globe, atrophy appeared greatest in the center of several nerves. This pattern of damage may result from particular vulnerability of macular axons to damage along the anterior visual pathway. Optic disk pallor was present in two eyes that had less than 40% of the normal number of axons remaining, while one optic disk appeared to be normal with 70% of the axons intact. Afferent pupillary defects were observed in two patients who had two to three times as many remaining axons in the contralateral optic nerve as in the nerve on the side of the defect.

A 45-year-old man with extensive myelinated retinal nerve fibers had an attack of anterior ischemic optic neuropathy. Serial fundus photographs documented the loss of myelin. Visual acuity in the affected eye decreased to 6/90 (20/300) and color perception was markedly reduced. The visual fields in the affected eye showed dense superior and inferior arcuate scotomas and central depression. These findings were unchanged nine months later, but the areas of myelinated nerve fibers had become smaller.

Two young women with idiopathic pseudotumor cerebri were treated successfully with lumboperitoneal shunts. Their shunts were removed electively several years later, following which there was rapid increase of cerebrospinal fluid pressure. In one patient, rapid increase of intracranial pressure resulted in bilateral visual loss. These cases provide further evidence that idiopathic pseudotumor cerebri may not be a self-limited condition.

Three patients developed anisocoria as a result of third nerve palsies caused by intracranial lesions: two in the cavernous sinus and one in the mesencephalon. Pharmacologic testing using low concentration pilocarpine showed apparent preganglionic parasympathetic supersensitivity of the abnormal pupil. These findings are compatible with the existence of a proposed direct pathway from the midbrain to the eye, without synapse at the ciliary ganglion. Cholinergic hypersensitivity can occur, not only with postciliary ganglionic but also with preciliary ganglionic parasympathetic lesions.

We reviewed the records of 218 children in whom a diagnosis of optic atrophy had been made between 1978 and 1987. A cause for the atrophy was determined for 195 patients (89%). Tumor, the most frequent cause, was found in 63 patients (29%). The most common tumor was a glioma of the anterior visual pathway; it was found in 27 patients (43% of tumors; 12% overall). The second most frequently encountered tumor, a craniopharyngioma, was found in 14 patients. Inflammation, the second most common cause of optic atrophy, occurred in 38 children (17%). Trauma caused optic atrophy in 24 patients (11%). No cause could be found for 23 patients (11%). Thirteen patients were less than 1 year of age at the time of diagnosis. Three of these patients had tumors. One was a cerebral glioblastoma, and the other two were optic gliomas. The diagnosis of optic atrophy in infancy does not imply a benign cause.

We treated two patients with trigeminal schwannomas, each of whom had an isolated, ipsilateral, abducens nerve palsy. Both patients initially had normal computed tomographic scan results and no neurologic signs of trigeminal nerve dysfunction. Trigeminal dysfunction did not develop until several years after the initial sixth nerve palsy, at which time a repeat examination disclosed the schwannoma. Accurate detection of a trigeminal schwannoma requires a careful clinical examination and appropriate neuroimaging studies. When such studies give normal results, they should be repeated if symptoms worsen or if new symptoms develop.