1/714. 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. ( info) |
2/714. 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. ( info) |
3/714. Pituitary macroadenoma manifesting as an isolated fourth nerve palsy. PURPOSE: To describe an unusual ophthalmic manifestation of a pituitary adenoma. methods: Case report. RESULTS: A 32-year-old man had left supraorbital and frontal headaches and new-onset vertical diplopia. Examination showed a left fourth nerve palsy and increased vertical fusional amplitudes. magnetic resonance imaging disclosed a sellar mass consistent with a pituitary macroadenoma. CONCLUSION: A pituitary adenoma may rarely manifest with an isolated fourth nerve palsy. ( info) |
| PURPOSE: To describe three children with acute fourth cranial nerve palsy secondary to pseudotumor cerebri. methods: We reviewed the medical records of children younger than 18 years who were diagnosed with pseudotumor cerebri between 1977 and 1997. pseudotumor cerebri was defined by normal neuro-imaging, elevated intracranial pressure measured by lumbar puncture, and normal cerebrospinal fluid composition. RESULTS: Three children with pseudotumor cerebri presented with vertical diplopia and clinical signs of fourth cranial nerve palsy including a hypertropia of the affected eye, which increased with adduction and ipsilateral head tilt. The fourth cranial nerve palsy resolved after reduction of the intracranial pressure in all three children. CONCLUSIONS: Fourth cranial nerve palsy may occur in children with pseudotumor cerebri and may be a nonspecific sign of elevated intracranial pressure. ( info) |
5/714. 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. ( info) |
6/714. 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. ( info) |
| 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. ( info) |
8/714. 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. ( info) |
9/714. hypoglossal nerve injury as a complication of anterior surgery to the upper cervical spine. Injury to the hypoglossal nerve is a recognised complication after soft tissue surgery in the upper part of the anterior aspect of the neck, e.g. branchial cyst or carotid body tumour excision. However, this complication has been rarely reported following surgery of the upper cervical spine. We report the case of a 35-year-old woman with tuberculosis of C2-3. She underwent corpectomy and fusion from C2 to C5 using iliac crest bone graft, through a left anterior oblique incision. She developed hypoglossal nerve palsy in the immediate postoperative period, with dysphagia and dysarthria. It was thought to be due to traction neurapraxia with possible spontaneous recovery. At 18 months' follow-up, she had a solid fusion and tuberculosis was controlled. The hypoglossal palsy persisted, although with minimal functional disability. The only other reported case of hypoglossal lesion after anterior cervical spine surgery in the literature also failed to recover. It is concluded that hypoglossal nerve palsy following anterior cervical spine surgery is unlikely to recover spontaneously and it should be carefully identified. ( info) |
| Transient sixth cranial nerves palsy may occur in rare cases after lumbar puncture, spinal anesthesia and myelography as well as in more rare cases of spontaneous intracranial hypotension. We report three cases of spontaneous intracranial hypotension with sixth cranial nerves palsy. One of these patients presented also third cranial nerve palsy, never reported in spontaneous intracranial hypotension. ( info) |