Faculty Bibliography

Neurosurgical practice requires a solid foundation in the principles and interpretation of the neuro-ophthalmologic examination. In this article, the techniques used in the neuro-ophthalmologic examination to assess visual acuity, ocular motility, visual fields, the pupils, the eyelids, and the fundus are reviewed. An emphasis is placed on those techniques most relevant to neurosurgical practice.

The authors describe five patients with trochlear nerve palsy and MS to characterize this rare association. In two patients, trochlear nerve palsy was the initial clinical manifestation of MS. In the other three patients, this sign occurred after previous neurologic events. MRI did not identify a lesion of the fourth nerve nucleus or fascicle. Ophthalmoplegia resolved within 2 months in four of the five patients. A reason this association is rare is that the fascicular course of the trochlear nerve is exposed to little myelin.

OBJECT: The authors sought to treat potentially catastrophic intracranial dural and deep cerebral venous thrombosis by using a multimodality endovascular approach. METHODS: Six patients aged 14 to 75 years presented with progressive symptoms of thrombotic intracranial venous occlusion. Five presented with neurological deficits, and one patient had a progressive and intractable headache. All six had known risk factors for venous thrombosis: inflammatory bowel disease (two patients), nephrotic syndrome (one), cancer (one), use of oral contraceptive pills (one), and puerperium (one). Four had combined dural and deep venous thrombosis, whereas clot formation was limited to the dural venous sinuses in two patients. All patients underwent diagnostic cerebral arteriograms followed by transvenous catheterization and selective sinus and deep venous microcatheterization. Urokinase was delivered at the proximal aspect of the thrombus in dosages of 200,000 to 1,000,000 IU. In two patients with thrombus refractory to pharmacological thrombolytic treatment, mechanical wire microsnare maceration of the thrombus resulted in sinus patency. Radiological studies obtained 24 hours after thrombolysis reconfirmed sinus/vein patency in all patients. All patients' symptoms and neurological deficits improved, and no procedural complications ensued. Follow-up periods ranged from 12 to 35 months, and all six patients remain free of any symptomatic venous reocclusion. Factors including patients' age, preexisting medical conditions, and duration of symptoms had no statistical bearing on the outcome. CONCLUSIONS: Patients with both dural and deep cerebral venous thrombosis often have a variable clinical course and an unpredictable neurological outcome. With recent improvements in interventional techniques, endovascular therapy is warranted in symptomatic patients early in the disease course, prior to morbid and potentially fatal neurological deterioration.

We report the MR findings in two patients with clinically and histologically proved Dejerine-Sottas disease. One patient had spinal involvement with multiple thickened and clumped nerve roots of the cauda equina; the second had multiple enlarged and enhancing cranial nerves. Although these findings are not specific for Dejerine-Sottas disease, they are suggestive of the diagnosis, which is further corroborated with history and confirmed with sural nerve biopsy and laboratory studies.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronic, multifocal disorder usually defined as limited to the peripheral nervous system. Multifocal motor neuropathy, an acquired demyelinating neuropathy with conduction block affecting motor neurons only, may be a pathogenically distinct syndrome or a predominantly motor variant of chronic inflammatory demyelinating polyneuropathy. Central nervous system demyelination including optic neuropathy has been reported uncommonly previously in these entities. We report two cases and review the literature on the possible association of optic neuropathy and chronic acquired polyneuropathy.

Patients with Ollier's disease (multiple skeletal enchondromas) and Maffucci's syndrome (multiple enchondromas associated with subcutaneous hemangiomas) may develop skull base chondrosarcomas or low-grade astrocytomas as a delayed consequence of these disorders. We report three patients with Ollier's disease and Maffucci's syndrome who had diplopia as the initial manifestation of intracranial tumors. Since patients with Maffucci's syndrome and Ollier's disease are at risk for the delayed development of brain and systemic neoplasms, neuroophthalmologists must be aware of the need for long-term surveillance in patients affected by these conditions.

Isolated inferior division third nerve palsies are rare. The authors report a patient with an isolated, pupil-involving inferior division third cranial nerve palsy from an intraorbital dural arteriovenous malformation. Despite spontaneous thrombosis of the malformation, the third nerve palsy persisted. To the authors' knowledge, this is the first case report of an inferior division third nerve palsy caused by an orbital dural arteriovenous malformation.

The relation between obesity and age in children with idiopathic intracranial hypertension (pseudotumor cerebri) has remained uncertain. The authors reviewed the records of 45 consecutive children with newly diagnosed idiopathic intracranial hypertension seen at two medical centers. Forty-three percent of patients aged 3 to 11 years were obese, whereas 81% of those in the 12- to 14-year age group and 91% of those in the 15- to 17-year age group met criteria for obesity (p = 0.01). Younger children with idiopathic intracranial hypertension are less likely to be obese than are older children or adults.