Cases reported "ophthalmoplegia"

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1/1407. 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)

2/1407. 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)

3/1407. Fourth cranial nerve palsy in pediatric patients with pseudotumor cerebri.

    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)

4/1407. Absent pituitary gland and hypoplasia of the cerebellar vermis associated with partial ophthalmoplegia and postaxial polydactyly: a variant of orofaciodigital syndrome VI or a new syndrome?

    We report two sibs with features overlapping those of orofaciodigital syndrome type VI (Varadi syndrome). Both presented at birth with oculomotor abnormalities, dysmorphic facial features, and dysgenesis of the cerebellar vermis. There were minimal oral manifestations (high arched palate) in both of them and one had postaxial polydactyly of both hands and one foot. In addition, there was evidence of aplasia of the pituitary gland on MRI scan in both of them with evidence of hypopituitarism. Both responded well to hormone replacement therapy with improvement in their linear growth and mental ability. These cases may represent a new autosomal recessive midline defect syndrome with features overlapping OFDS VI. Alternatively the features in these children could represent variability within OFDS VI. ( info)

5/1407. Internuclear ophthalmoplegia following minor head injury: a case report.

    Internuclear ophthalmoplegia (INO) is a common sign of multiple sclerosis in young patients and of vascular diseases in older people. Traumatic bilateral internuclear ophthalmoplegia following severe head injuries may occur. We present the unusual case of a young patient suffered from bilateral INO as an isolated finding after a minor head injury, without other signs of brain stem or cortical injury. The ophthalmoplegia has persisted for 22 months. ( info)

6/1407. A case of the subacute brainstem encephalitis.

    A case of brainstem encephalitis of undetermined etiology is reported in 66-year-old woman who had a sudden onset of illness with left abducens palsy, nystagmus and ataxia. The symptoms progressed to complete paralysis of eye movements, dysphagia and left hemiparesis with generalized hyperreflexia. Examination of CSF, CT scan and MRI of the brain were normal. The patient died 4 months after onset of disease. Neuropathologic study disclosed in the brainstem numerous perivascular and nodular inflammatory cell infiltrations composed predominantly of lymphocytes T and B. Most intensive inflammation concerned midbrain and pontine tegmentum and to a lesser degree medulla oblongata, pontine nuclei and cerebellar nuclei. basal ganglia, cerebral and cerebellar cortex were unaffected. Neuropathological finding was reminiscent of brainstem encephalitides related to viral infection or to paraneoplastic syndrome. However, HSV-1, EBV, and CMV antigens were not detected by immunohistochemistry, as well as evidences of malignancy were not present in this case. ( info)

7/1407. Tonic ocular tilt reaction simulating a superior oblique palsy: diagnostic confusion with the 3-step test.

    BACKGROUND: The tonic ocular tilt reaction (OTR) consists of vertical divergence of the eyes (skew deviation), bilateral conjugate ocular torsion, and paradoxical head tilt. The head and superior pole of both eyes are rotated toward the hypotropic eye. OBJECTIVE: To describe ocular motility and torsion findings in 5 patients with OTRs that mimicked superior oblique palsies (SOPs). RESULTS: In 5 patients, results of the 3-step test suggested an SOP (bilateral in 1 patient); however, no patient had the expected excyclotorsion of the hypertropic eye. Two patients had conjugate ocular torsion (intorsion of the hypertropic eye and extorsion of the hypotropic eye), and 2 patients had only intorsion of the hypertropic eye. All had other neurologic features consistent with more widespread brainstem disease. CONCLUSIONS: Vertical ocular deviations that 3-step to an SOP are not always caused by fourth nerve weakness. When a patient with an apparent fourth nerve palsy has ocular torsion that is inconsistent with an SOP, OTR should be suspected, especially if vestibular system or posterior fossa dysfunction coexists. The rules for the 3-step test for an SOP may be fulfilled by damaging the otolithic projections corresponding to projections of the contralateral anterior semicircular canal. Because results of the Bielschowsky head tilt test may be positive in patients with the OTR, the feature distinguishing OTR from an SOP is the direction of torsion. We advocate use of a fourth step-evaluation of ocular torsion-in addition to the standard 3 steps. ( info)

8/1407. Congenital myasthenia gravis: clinical and HLA studies in two brothers.

    Two brothers with congenital myasthenia gravis are described. In both, ptosis and ophthalmoplegia responded poorly to oral anticholinesterase therapy and to thymectomy. The brothers had two different HLA haplotypes and neither had the HLA-A1-B8-DW3 haplotypes which are commonly associated with myathenia gravis in adult-onset cases. ( info)

9/1407. Congenital upper eyelid retraction.

    This report describes a six-year-old boy born with a unilateral upper eyelid retraction resulting in mild exposure keratopathy. The fissure on that side was 4 millimeters larger and lagophthalmos was present. At the time of levator recession surgery, the muscle was found to have a restriction at the medial and lateral horns preventing downward excursion. This condition has some of the features of congenital orbital fibrosis and is postulated to have been due to an intrauterine infection or inflammation. ( info)

10/1407. Cerebral B-cell lymphoma following treatment for tolosa-hunt syndrome.

    We herein report a unique case of cerebral lymphoma which occurred after lymphocytic neuritis of cranial nerves causing tolosa-hunt syndrome and demonstrate the histological difference between these two diseases. A 70-year-old woman developed a sensory disturbance in the first and third divisions of the left trigeminal nerve and a left ocular movement disturbance five years before death. Although she was clinically diagnosed to have a schwannoma in the left cavernous sinus, a histologic examination verified a diffuse infiltration of T lymphocytes in the left trigeminal ganglion. Corticosteroid therapy was effective. Thereafter she demonstrated a disturbance of consciousness and dysphasia four years after surgery. A T1-weighted magnetic resonance image (MRI) disclosed high intensity lesions in both the basal ganglia and corpus callosum. She also showed progressive spastic paralysis. At autopsy a diagnosis of primary intracranial B-cell lymphoma was made. Although there was no invasion of the lymphoma cells into the left trigeminal nerves, a mild inflammatory infiltration of T cells still remained. ( info)
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