PubMed HealthSearch

Biomedical subjects

N J Volpe

Publications and source records attributed to N J Volpe.

18 recordsLinked to original sources

Severe sudden visual loss caused by pseudotumor cerebri and lumboperitoneal shunt failure.

PURPOSE: Severe vistral acuity loss associated with pseudotumor cerebri is usually caused by chronic optic disk edema or a retinal abnormality. METHODS: We treated a women, with known pseudotumor cerebri treated with a lumboperitoneal shunt, who developed acute pallied optic disk swelling and visual acuity of R.E.: no light perception and L.E.: 20/70 in association with lumboperitoneal shunt failure. There were no contributory retinal lesions. RESULTS: The patient underwent optic nerve sheath fenestration and lumboperitoneal shunt revision. Visual acuity improved to 20/20 in both eyes. The papilledema resolved. CONCLUSION: The severe sudden visual loss was attributed to axoplasmic stasis and optic nerve ischemia associated with a sudden rise in intracranial pressure.

Adult

Prominent proptosis in childhood thyroid eye disease.

BACKGROUND: Orbital signs and symptoms occur in approximately one half of children with Graves disease, but the symptoms are usually minor and limited to the eyelids. Prominent proptosis is uncommon in children with this disorder. METHODS: Review of eight children with prominent proptosis associated with thyroid eye disease. Four patients were treated at the Children's Hospital of Philadelphia, the other four at the Columbia Presbyterian Medical Center. RESULTS: At initial presentation, children ranged in age from 3 to 16 years. There were five girls and three boys. Seven of eight children had hyperthyroidism at ophthalmic presentation. Four patients had restrictive myopathy, and all of the seven patients who underwent neuroimaging had extraocular muscle enlargement. Five patients were treated with lubrication. Two underwent orbital fat decompression. One patient had thyroid eye disease and myasthenia gravis. CONCLUSIONS: Proptosis in childhood thyroid eye disease usually is associated with a hyperthyroid state. The proptosis may be dramatic, but corneal exposure and restrictive myopathy are seen in only some of the patients. Neuroimaging shows enlarged extraocular muscles. Most children with this complication can be treated conservatively with topical lubrication, but orbital fat decompression may be considered in patients with more advanced conditions.

Adolescent

Diagnostic tests in neuro-ophthalmology.

This article reviews the diagnostic testing used in the evaluation of several common neuro-ophthalmologic entities including optic nerve disease, pseudotumor cerebri, anisocoria, ptosis, and ocular motor palsies. Emphasis is placed on these bedside tests that help to establish the diagnosis of these common clinical problems. The utility of the cocaine and Tensilon (edrophonium chloride) tests as well as the role of neuroimaging in these conditions are reviewed.

Brain Diseases

Gangliogliomas involving the optic chiasm.

We report three patients with gangliogliomas involving the optic chiasm via distinct mechanisms. The ganglioglioma in one patient likely originated in the temporal lobe and spread medially to involve the chiasm, and diffuse spinal cord dissemination also occurred. Chiasmal involvement in this manner and dissemination at presentation are unusual for gangliogliomas. The tumor in a second patient was intrinsic to the hypothalmus and chiasm, while in the third patient, it involved both optic tracts, and a cyst compressed the chiasm laterally. Two patients developed severe bilateral visual loss, while the other had a stable bitemporal hemianopsia. Two patients received radiotherapy, but one continued to lose vision. Although gangliogliomas rarely involve chiasm, the mechanisms by which they produce chiasmal visual loss may be diverse, and the long-term visual prognosis is variable.

Adolescent

The crossed paralyses. The original brain-stem syndromes of Millard-Gubler, Foville, Weber, and Raymond-Cestan.

In this article, the crossed syndromes of Millard-Gubler (facial palsy and contralateral hemiparesis), Foville (facial palsy, conjugate gaze paralysis, and contralateral hemiparesis), Weber (oculomotor palsy and contralateral hemiparesis), and Raymond-Cestan (internuclear ophthalmoplegia and contralateral hemiparesis) are detailed from the original reports. These and other related syndromes were instrumental in establishing important principles in brain-stem localization: the occurrence of cranial nerve palsies contralateral to hemibody motor or sensory disturbances, the concepts of the medial longitudinal fasciculus and conjugate gaze, and the corticobulbar innervation of the facial nerve nucleus.

Brain Stem

Tuberculous meningitis with blindness and perichiasmal involvement on MRI.

An 11-year-old boy with tuberculous meningitis developed blindness, a rare complication of this disease in the United States. Magnetic resonance imaging demonstrated perichiasmal enhancement, suggesting that arachnoiditis caused the visual loss. Serial neuroimaging over 7 months revealed a persistent inflammatory process in the chiasmatic cistern, hydrocephalus, and progressive cerebral infarctions.

Arachnoiditis

Persistent positive visual phenomena in migraine.

Ten patients with migraine developed persistent positive visual phenomena lasting months to years. The complaints were similar in their simplicity and involvement of the entire visual field and usually consisted of diffuse small particles such as TV static, snow, lines of ants, dots, and rain. Neurologic and ophthalmologic examinations were normal, and EEGs were normal in eight of eight patients tested. MRI was normal in all patients except one who had nonspecific biparietal white matter lesions and another with a small venous angioma. Treatment of this unusual complication of migraine was unsuccessful.

Adult

Cyclodeviation in skew deviation.

We examined four patients with dorsolateral pontomedullary lesions and skew deviation, with ocular torsion of varying symmetry. In three patients, the hypotropic eye was excyclodeviated relative to the fellow eye. Observations of these patients, combined with recent evidence, suggest that cyclodeviation is a frequent component of skew deviation and may result from variable involvement of utricular, semicircular canal, and cerebelloocular pathways. Although cyclodeviation is a feature of trochlear-nerve palsies, its presence does not exclude skew deviation and underlying brainstem lesions.

Adult

Isolated fascicular abducens nerve palsy and Lyme disease.

A patient with Lyme disease developed an isolated sixth nerve palsy at the end of a 1 month course of oral antibiotics. Magnetic resonance imaging disclosed high-signal abnormality at the left pontomedullary junction, implicating involvement of the distal sixth nerve fascicle. Although facial numbness ensued during a subsequent course of intravenous antibiotics, corticosteroid therapy was associated with prompt improvement of neurologic signs, suggesting an immunologic mechanism for the central nervous system dysfunction.

Abducens Nerve

Remitting sixth nerve palsy in skull base tumors.

OBJECTIVE: Spontaneous recovery of a sixth nerve palsy is thought to rule out a neoplastic origin. We reviewed cases of sixth nerve palsy that improved without treatment but that ultimately proved to be caused by a tumor at the base of the skull. DESIGN: Case series. SETTING: Hospital-based, neuro-ophthalmology referral practice. PATIENTS: Seven patients with an age range from 7 to 61 years had sixth nerve palsy secondary to a slow-growing neoplasm at the skull base. MAIN OUTCOME MEASURES: Return of lateral rectus function and resolution of diplopia without intervention. RESULTS: Seven patients with sixth nerve palsy caused by skull base tumors experienced spontaneous improvement of their deficit. Recovery time ranged from 1 week to 18 months. No patient was diabetic or had evidence of vascular disease. In one patient, the palsy improved once prior to becoming a fixed deficit, and spontaneous improvement occurred on two to five occasions in the other patients. CONCLUSION: Spontaneous recovery of a sixth nerve palsy can occur in the presence of an extramedullary compression by a tumor at the base of the brain. Possible mechanisms for recovery include remyelination, axonal regeneration, relief of transient compression (eg, resorption of hemorrhage), restoration of impaired blood flow, slippage of a nerve previously stretched over the tumor, or immune responses to the tumor.

Abducens Nerve

Neuro-ophthalmologic findings in chordoma and chondrosarcoma of the skull base.

Review of the clinical features of 48 patients with chordoma and 49 patients with low-grade chondrosarcoma of the skull base disclosed overlapping clinical profiles but distinctive features. Both tumors occurred at all ages but chondrosarcoma tended to occur in the third and fourth decades. Twenty-five (52%) of the patients with chordoma and 24 (49%) of the patients with chondrosarcoma had ocular symptoms (diplopia or visual impairment) as the initial manifestation of the disease. Of the 59 patients (both groups) with diplopia, the diplopia was initially intermittent in 25 (42%). Headache and diplopia from an insidious abducens nerve palsy was most common in both groups. Abducens nerve palsy occurred in 22 (46%) of the patients with chordoma and 23 (47%) of the patients with chondrosarcoma. Normal examination results were more common in patients with chordoma, whereas visual loss, facial numbness, and multiple cranial neuropathies were more common in patients with chondrosarcoma. The similarities in the clinical features of these tumors reflect their common origin at the central skull base and the vulnerability of the abducens nerves at that site. The differences reflect the tendency of chordomas to originate from the clivus and chondrosarcomas to originate from the temporal bone.

Adolescent

Simultaneous corneal laceration repair, cataract removal, and posterior chamber intraocular lens implantation.

Frequently, patients with lens laceration or traumatic cataract coincident with corneal laceration, or both, must undergo two separate procedures-primary repair of the corneal laceration and secondary lens removal with or without intraocular lens implantation. We performed simultaneous corneal laceration repair, extracapsular cataract extraction, and posterior chamber lens implantation in seven patients with lacerating ocular injuries who met inclusion criteria for this procedure. With average follow-up of 10 1/2 months, all seven patients achieved visual acuity of 20/40 or better with spectacle correction. YAG posterior capsulotomy was the only additional procedure. One patient had macular pigmentation consistent with either traumatic or photic maculopathy. There were no other complications attributable to the surgical procedures. We believe that certain lacerating injuries of the anterior segment are particularly amenable to cataract extraction and lens implantation at the time of primary laceration repair. This approach obviates additional operative and anesthetic risks, while affording more timely visual rehabilitation.

Adult

Secondary hemorrhage in traumatic hyphema.

We analyzed the records of 132 patients hospitalized between July 1986 and February 1989 for management of traumatic hyphema. The incidence of secondary hemorrhage was compared between patients treated with or without systemic administration of aminocaproic acid in addition to an otherwise identical protocol. Results among patients who were examined within one day of injury disclosed a 4.8% secondary hemorrhage rate in aminocaproic acid-treated patients (three of 63 patients) compared with a 5.4% rate in the patients not treated with aminocaproic acid (three of 56 patients, P = .31). All six patients sustaining secondary hemorrhage recovered visual acuities of 20/40 or better, with five of six patients achieving 20/20 visual acuities. A separate group of 13 patients who were examined more than one day after injury were found to have a secondary hemorrhage rate of 38.5% (five of 13 patients). Macular injury, not secondary hemorrhage, was most often responsible among those patients suffering permanent visual loss. In this study of a predominantly white population, patients had a relatively low incidence of secondary hemorrhage and did not demonstrate detectable benefit from aminocaproic acid administration. Because of the recognized side effects and cost of treatment, further analysis to determine which patients will benefit from treatment with aminocaproic acid is indicated.

Adolescent