Faculty Bibliography

Hodgkin's disease has not been reported to produce an isolated cavernous sinus syndrome, although this phenomenon is well-described in non-Hodgkin's lymphoma. We review the 16 cases of cavernous sinus syndrome caused by non-Hodgkin's lymphoma and report two patients with Hodgkin's disease in clinical remission who developed recurrent disease in the cavernous sinus. MRI revealed a mass lesion in the left cavernous sinus in each patient. Corticosteroids and radiation therapy were effective palliative measures. In both patients, recurrence in the cavernous sinus preceded other systemic evidence of recurrent Hodgkin's disease.

PURPOSE: To present the magnetic resonance imaging findings for a patient with traumatic oculomotor nerve injury. METHODS: We examined a patient with a right pupil-involving oculomotor nerve palsy after severe closed head trauma. RESULTS: Magnetic resonance imaging of the brain demonstrated marked signal hypointensity on gradient-echo T2*-weighted images consistent with hemorrhage at the midbrain exit site of the right oculomotor nerve. CONCLUSIONS: Distal fascicular damage or partial rootlet avulsion is a mechanism of injury in some traumatic oculomotor nerve palsies. Gradient-echo T2*-weighted magnetic resonance imaging is the most sensitive method to detect hemorrhagic changes associated with shearing injury.

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.

We report a patient with pretectal eyelid lag without retraction. MRI showed high signal abnormality dorsal to the right red nucleus in a region thought responsible for premotor eyelid control. The dissociation of eyelid lag from eyelid retraction in this patient would suggest that these lid signs may have separate neural mechanisms or pathways. We propose that the lesion interrupts inhibitory connections between the vertical gaze center and the central caudal nucleus.

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.

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.

A 57-year-old awake man developed central neurogenic hyperventilation associated with a pontine mass. Serum pH reached as high as 7.72 with serum carbon dioxide of 6 torr. Examination of CSF during overbreathing showed that CSF pH was markedly alkaline. Pathologic study showed a well-differentiated pontine astrocytoma. The combination of alkaline CSF and an infiltrating pontine lesion supports a structural, rather than chemical, mechanism for central hyperventilation.

We report three patients who exhibited ophthalmoparesis as an early manifestation of progressive paraneoplastic brainstem encephalitis. In two patients, anti-Hu antibodies were detected, whereas in a third, found at postmortem to have thyroid cancer, no antibodies were identified. Postmortem examination of two patients disclosed extensive gliosis, perivascular inflammation, and cell loss in the midbrain and pontine tegmentum. In one of these patients, there was selective neuronal loss within the third, fourth, and sixth nerve nuclei. We conclude that supranuclear or nuclear ophthalmoparesis may be the initial manifestation of paraneoplastic brainstem encephalitis. Our pathologic data suggest that the ophthalmoparesis may result from selective neuronal death within the brainstem tegmentum and ocular motor nuclei.