1/216. Acquired and isolated asymmetrical palatal palsy.Benign acquired and isolated asymmetrical palatal palsy is a rare condition in childhood. We report on three cases. Typical features include: sudden onset, abnormality of the palatal components of speech (rhinolalia), nasal escape of fluids from the ipsilateral nostril. It is supposed to be caused by viral infection, but attempts at viral isolation were unsuccessful. Complete spontaneous recovery is usual, taking a few weeks. Our paper seems to be the first report of magnetic resonance imaging of the brain in this condition. It did not disclose any abnormalities in the 2 cases in which it was performed.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
2/216. Schwannoma in patients with isolated unilateral trochlear nerve palsy.PURPOSE: To describe the clinical features of patients with isolated unilateral trochlear nerve palsy secondary to imaging-defined schwannoma of the trochlear nerve. methods: A chart review of all patients seen at the Neuro-ophthalmology Unit at Emory University since 1989. Of 221 patients with trochlear nerve palsy, six had a lesion consistent with a trochlear nerve schwannoma. RESULTS: The six patients had isolated unilateral trochlear nerve palsy. Duration of diplopia before diagnosis averaged 6 months. magnetic resonance imaging demonstrated circumscribed, enhancing lesions along the cisternal course of the trochlear nerve, all measuring less than 5 mm in greatest dimension. Five of the patients were seen in follow-up, over periods ranging from 11 to 26 months from initial presentation (mean, 15.6 months; standard deviation, 6.0 months). All of these patients remained stable except one, who was slightly worse at 15 months by clinical measurements and magnetic resonance imaging. None of these patients have developed additional symptoms or signs of cranial nerve or central nervous system involvement. CONCLUSIONS: The differential diagnosis of an isolated unilateral fourth cranial nerve palsy should include an intrinsic neoplasm of the trochlear nerve. magnetic resonance imaging is useful, both for diagnosis and follow-up. These patients can remain stable and may not require neurosurgical intervention.- - - - - - - - - - ranking = 2keywords = ms (Clic here for more details about this article) |
3/216. Surgically created fourth-third cranial nerve communication: temporary success in a child with bilateral third nerve hamartomas. Case report.Shortly after birth, an otherwise healthy infant developed eye deviation and ptosis due to a hamartomatous lesion of the interpeduncular segment of the right oculomotor nerve. The left nerve became similarly involved when the child was 1.5 years of age. Direct nerve repair was not possible. Instead, the trochlear nerve was divided and its proximal end was attached to the distal end of the third nerve. Elevation of the upper eyelid and partial adduction of the eye developed gradually over the ensuing 3 to 5 months. Both functions were lost after an additional 2 months, presumably as a result of tumor recurrence or neuroma formation. This case report shows that surgically created fourth-third cranial nerve communication is feasible and may merit consideration under similar circumstances.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
4/216. Chronic steadily progressive central and peripheral predominantly motor demyelination, involving the cranial nerves, responsive to immunoglobulins.The association of central and peripheral demyelination was reported previously. Most of the cases refer to central chronic relapsing demyelination with clinical criteria for multiple sclerosis associated with later signs of peripheral nerve involvement. Other authors, described central lesions in patients with chronic inflammatory demyelinating polyneuropathy and in guillain-barre syndrome, as a seldom occurrence. We report a patient in which a chronic steadily progressive central and peripheral predominantly motor nervous system demyelination, involving the cranial nerves, was identified. The patient improved after intravenous immunoglobulin suggesting an immune-mediated mechanism. To our knowledge this presentation was not described before.- - - - - - - - - - ranking = 1929.1895797178keywords = multiple sclerosis, sclerosis (Clic here for more details about this article) |
5/216. Cranial polyneuropathies in multiple sclerosis: case report and literature review.Although cranial neuropathies are common disorders in multiple sclerosis patients, multiple cranial nerve involvement is an unusual occurrence. Correlation of clinical symptoms with magnetic resonance imaging evidence of demyelinating central nervous system lesions can confirm the diagnosis. The authors report on the case of a 43-year-old woman who initially was thought to have suffered a brainstem infarct but, in fact, had developed multiple cranial nerve functional deficits. Treatment of multiple sclerosis remains primarily supportive in nature, with corticosteroids used for acute exacerbations and chronic progression.- - - - - - - - - - ranking = 11576.137478307keywords = multiple sclerosis, sclerosis, ms (Clic here for more details about this article) |
6/216. Neuro-ophthalmologic manifestations of Maffucci's syndrome and Ollier's disease.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.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
7/216. Surgical findings in idiopathic trigeminal neuropathy mimicking a trigeminal neurinoma.Idiopathic trigeminal neuropathy is a benign disorder where the main clinical feature is facial numbness limited to the territory of one or more divisions of the trigeminal nerve, persisting for a few weeks to several years and in which no underlying disease can be identified. The case of a 37-year-old man with a brief history of sensory and motor trigeminal symptoms who showed magnetic resonance imaging (MRI) findings consistent with a small trigeminal neurinoma is reported. The patient was operated on but no tumour could be found during surgery and a biopsy was taken from the portio major. Pathological diagnosis was chronic non granulomatous inflammatory reaction with areas of coagulation necrosis. Control MRI showed complete resolution of the trigeminal abnormalities. It is concluded that in patients with MRI findings suggestive of a small trigeminal schwannoma, benign idiopathic trigeminal neuropathy should also be considered in the differential diagnosis. A conservative approach with sequential MRI studies may avoid an unnecessary surgical exploration.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
8/216. Bilateral recurrent nerve paralysis associated with multinodular substernal goiter: a case report.Substernal goiter is an infrequent occurrence and is found in two to five per cent of all patients undergoing thyroid surgery. These lesions are well known to cause respiratory symptoms and alterations in phonation due to direct compression of airway structures. Infrequently, unilateral recurrent nerve palsy has been reported in patients with substernal goiter. We report a case of bilateral recurrent nerve palsy associated with multinodular substernal goiter in an 89-year-old female who presented in respiratory distress.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
9/216. Ocular amyloidosis and secondary glaucoma.OBJECTIVE: To report the clinical and histopathologic findings in two cases of secondary glaucoma associated with amyloidosis. DESIGN: Two case reports. methods: Retrospective review of clinical findings, course, and treatment of the two patients. The histopathologic findings from available biopsy material were also reviewed. MAIN OUTCOME MEASURES: intraocular pressure (IOP), visual field changes, and surgical outcome. RESULTS: The first case describes a 76-year-old woman with orbital amyloidosis who developed gradual unilateral elevation of IOP that was poorly responsive to medical therapy and underwent filtration surgery. Episcleral venous pressure was elevated on the affected side, and histopathologic analysis of the conjunctival tissue confirmed perivascular amyloid deposits, further suggesting raised episcleral venous pressure to be a possible mechanism of glaucoma. The second case describes a 47-year-old white woman with familial amyloid neuropathy with a transthyretin cys-114 mutation. The association of glaucoma with this mutation has not been described previously. Persisting elevation of IOP in one eye was initially responsive to topical antiglaucoma medications but eventually required filtration surgery. Amyloid particles were found in the aqueous and on the lens surface. Histopathologic analysis of the aqueous and sclerectomy specimens demonstrated amyloid, suggesting outflow obstruction as a possible mechanism of glaucoma. Conjunctival buttonholing complicated filtration surgery in both cases, and the leaks eventually resolved with good control of IOP. CONCLUSIONS: Amyloid associated with glaucoma may involve different pathophysiologic mechanisms. The elevated IOP may not respond well to medical therapy. Cautious surgical manipulation of the conjunctiva is warranted in these cases.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
10/216. Unusual cranial nerve palsy caused by cavernous sinus aneurysms. Clinical and anatomical considerations reviewed.BACKGROUND: Two cases of intracavernous internal carotid artery aneurysm with unusual clinical findings are reported. The pathomechanism and clinical significance are discussed. CASE DESCRIPTION: The first patient was a 49-year-old woman who presented with 6th nerve palsy and Horner's syndrome caused by a posteriorly located intracavernous aneurysm. The symptoms improved gradually in proportion to the size of the aneurysm. The second patient was a 69-year-old woman with isolated oculomotor superior division palsy caused by an anteriorly located large aneurysm. CONCLUSION: In the first case, a local aneurysmal compression at both the 6th nerve and the sympathetic fibers sent from the plexus on the intracavernous internal carotid artery is the most probable explanation. In the second case, the aneurysm might have selectively compressed the superior division of the oculomotor nerve at the anterior cavernous sinus. Clinical recognition of these syndromes results in a better diagnostic orientation. The authors discuss the pertinent anatomy and pathophysiology of the lesions because these findings are rarely seen clinically or in the literature.- - - - - - - - - - ranking = 5keywords = ms (Clic here for more details about this article) |
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