Melkersson-Rosenthal syndrome: new clinicopathologic findings in 4 cases.

OBJECTIVE: To define the clinicopathologic features of eyelid involvement in Melkersson-Rosenthal syndrome (MRS). METHODS: Four patients with eyelid edema consistent with MRS were evaluated clinically, including diagnostic imaging in 2 patients. Eyelid tissue from these patients was examined by light microscopy and immunohistochemistry. Polymerase chain reaction for herpes simplex virus was performed in 1 case. RESULTS: The 3 men and 1 woman ranged in age from 33 to 74 years. All patients had insidious, painless, nonpitting eyelid edema. Three patients had unilateral edema; one had bilateral, asymmetric involvement. Ipsilateral lip edema was present in 1 case. Computed tomography demonstrated periorbital heterogeneous thickening that corresponded to the microscopic finding of scattered granulomas. All 4 patients demonstrated epithelioid granulomas inside and adjacent to dilated lymphatic vessels. Polymerase chain reaction testing was negative for herpes simplex virus. CONCLUSIONS: Isolated eyelid swelling that mimics thyroid-associated ophthalmopathy may occur in MRS. Computed tomography may be useful in the diagnosis. Biopsy should be performed in all cases of unexplained nonpitting eyelid edema. In the eyelid, MRS is characterized histopathologically by a granulomatous lymphangitis, a finding that seems to be unique to this condition.

The clinical spectrum of schwannomas presenting with visual dysfunction: a clinicopathologic study of three cases.

Schwannomas (neurilemomas) are benign tumors that arise from Schwann cells in the peripheral nervous system. The most commonly involved nerves that cause neuro-ophthalmic manifestations are cranial nerves V and VIII. In this series of three women, schwannomas presented as intraconal masses that mimicked a cavernous hemangioma, a superior orbital mass transgressing the superior orbital fissure, and an expansive frontal lobe mass with clinical symptoms and signs of increased intracranial pressure. Although all three complained of visual blurring, none of our patients presented with Vth or VIIIth cranial nerve dysfunction. Histopathologic studies demonstrated well-circumscribed, encapsulated spindle-cell lesions with classic Antoni A and B patterns. Histopathologic examination is essential to confirm the diagnosis of a schwannoma that may be otherwise clinically confusing. Direct optic nerve compression, globe indentation with induced hyperopia, or increased intracranial pressure with optic nerve compromise may be responsible for visual symptoms. A multidisciplinary approach is often required because of the size and location of schwannomas.

Infarction in the territory of the anterior cerebral artery.

Infarction in the anterior cerebral artery (ACA) territory is an uncommon cause of stroke. The clinical findings of ACA infarctions are not fully characterized but include contralateral hemiparesis, urinary incontinence, transcortical aphasia, agraphia, apraxia, and executive dysfunction. We report a patient with a large right ACA infarction, who in addition to previously reported findings also had a complete hemiplegia, profound sensory neglect, and micrographia.

MR of extraocular muscles in chronic progressive external ophthalmoplegia.

PURPOSE: Our goal was to determine whether the extraocular muscles in patients with chronic progressive external ophthalmoplegia (CPEO) could be distinguished from those of age-matched control subjects by MR imaging. METHODS: Nine patients with CPEO and eight age-matched healthy control subjects were studied. The extraocular muscles of eight of the patients (16 eyes) and all the control subjects (16 eyes) were measured digitally. Images consisted of 1.5-mm contiguous sections acquired using a volume (three-dimensional) gradient-echo acquisition. In all, measurements were performed on 11 interpolated 1.0-mm coronal sections, five on each side of the muscle center. Only the medial, inferior, and lateral rectus muscles were evaluated. The superior rectus was omitted to avoid averaging problems with the superior ophthalmic vein and levator palpebrae muscle. The 11 sections were summed to obtain a volume measurement of the central portion of each muscle. RESULTS: The digitally measured extraocular muscles in the patients with CPEO had statistically significantly smaller volumes than those of the control subjects. The average muscle volumes for the patients with CPEO were 215 mm3 for the medial rectus, 202 mm3 for the inferior rectus, and 269 mm3 for the lateral rectus. The average extraocular muscle volumes for the control subjects were 366 mm3 for the medial rectus, 365 mm3 for the inferior rectus, and 425 mm3 for the lateral rectus. CONCLUSION: MR imaging can show small extraocular muscles in patients with CPEO, which may help to distinguish this disorder from other entities. Since denervated extraocular muscles do not readily atrophy, this MR sign would support a myogenic pathologic substrate for CPEO. Variation in the degree of extraocular muscle atrophy may simply reflect the length of time the mitochondrial defect and ophthalmoplegia have been present.

Isolated abducens nerve palsy: MR imaging findings.

The abducens nerve, the sixth cranial nerve, innervates the lateral rectus muscle of the eye and is responsible for lateral horizontal ocular movement. A wide variety of abnormalities, both primary to the nerve itself and secondarily involving the nerve, can paralyze the abducens nerve. MR imaging offers the best opportunity to detect the underlying abnormality causing abducens nerve palsy. In this pictorial essay, we illustrate the MR imaging features of numerous conditions that cause isolated abducens nerve palsy.