Subject(s)
Abducens Nerve Diseases/etiology , Cavernous Sinus/diagnostic imaging , Horner Syndrome/etiology , Melanoma/complications , Neoplasm Recurrence, Local/diagnostic imaging , Parkinsonian Disorders/etiology , Abducens Nerve Diseases/diagnostic imaging , Horner Syndrome/diagnostic imaging , Humans , Male , Melanoma/diagnostic imaging , Middle Aged , Parkinsonian Disorders/diagnostic imagingABSTRACT
PURPOSE: We report the first case of congenital ocular neuromyotonia (ONM) and the results of strabismus surgery for this patient's co-existing cranial nerve (CN) III palsy. PATIENTS AND METHOD: The patient presented at 18 months with strabismus that had reportedly been present since the time of birth. On exam, she had persistent exotropia (RXT) and hypertropia (RHT) with episodes of esotropia in the right eye that could be evoked by sustained left gaze. A diagnosis of ONM with partial CN III palsy was made. T1-weighted, T2-weighted, and fluid-attenuated inversion recovery magnetic resonance imaging failed to reveal intracranial pathology. RESULTS: Gaze induced intermittent esotropia resolved with carbamazepine. Surgery was performed to improve the patient's RXT and RHT. Post-operatively, the patient's RXT had improved from 12 to 15 prism diopters (∆) at near and 20∆ at a distance to 10∆ RXT at near with no horizontal deviation at distance. Her deviation has remained stable for 13 years, as has her neurological exam and good state of health. CONCLUSION: This case demonstrates that ONM may present congenitally and adds to the body of knowledge regarding surgical outcomes on concurrent CN palsies in these patients.
Subject(s)
Isaacs Syndrome/congenital , Oculomotor Nerve Diseases/congenital , Strabismus/congenital , Carbamazepine/therapeutic use , Eye Movements , Female , Humans , Infant , Isaacs Syndrome/diagnosis , Isaacs Syndrome/therapy , Oculomotor Muscles/innervation , Oculomotor Nerve Diseases/diagnosis , Oculomotor Nerve Diseases/surgery , Ophthalmologic Surgical Procedures , Sodium Channel Blockers/therapeutic use , Strabismus/diagnosis , Strabismus/therapyABSTRACT
We report a case of a 42-year-old woman who presented with a one-year history of a painless right orbital mass and right upper lid ptosis. Magnetic resonance imaging (MRI) revealed a superotemporal right orbital mass involving the lacrimal gland, and subsequent tissue biopsy and histopathologic evaluation was consistent with amyloidosis. An otherwise negative workup by hematology/oncology confirmed a diagnosis of primary isolated lacrimal gland amyloidosis. To the best of our knowledge, this is the first documented case of isolated lacrimal gland amyloidosis without immunoglobulin (Ig) light chain restriction on in situ hybridization testing with a report of MRI findings. In addition, this is the second reported case of the disease without Ig light chain restriction on immunohistochemistry staining, and it is the third case with reported MRI results.
