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Grades of exophthalmos and thyrotropin-binding inhibitory immunoglobulin in patients with myasthenia gravis.

Exophthalmos and thyrotropin-inhibitory immunoglobulin (TBII) were examined in myasthenia gravis (MG) patients with normal thyroid function. Exophthalmos was found in 7 of 31 (22.6%) MG patients, and TBII was detected in 4 of 21 (19.0%) MG patients. The grades of exophthalmos did not decrease after extraocular muscle weakness was improved by treatment. Patients with MG whose diplopia was Tensilon unresponsive were only found among those with exophthalmos. TBII was more frequently detected in MG patients with exophthalmos than in those without. The present study revealed that exophthalmos is not rare in MG patients, even if they do not show any signs of Graves' disease other than exophthalmos and their thyroid functions are within the normal limit. The presence of TBII in MG patients may relate to the development of exophthalmos, and exophthalmos itself might enhance ocular symptoms of MG.

Adult↗

Fractures of the malar bone. Follow-up study based upon a questionnaire.

During the years 1969-1976 214 patients with malar bone fractures were treated. A retrospective study was carried out and a follow-up by the aid of a questionnaire. The clinical judgement at 1 week after surgery showed good results. The follow-up study revealed, however, a poorer picture. Among the surgically treated patients who answered the questionnaire, 89 of 143 reported different complaints. Anaesthesia in the check and diplopia were the most common complaints. It seems anyhow that the process of regeneration of the sensible nerve takes longer than predicted. The pre-operative evaluation of the eye motility is of utmost importance.

Adolescent↗

Ocular tilt reaction associated with a sudden idiopathic unilateral peripheral cochleovestibular loss.

We recently observed a female patient who was suffering from acute right peripheral cochleovestibular loss associated with a marked vertical diplopia. Otoneurological examination showed profound deafness, and absence of nystagmic response to caloric and pendular rotatory test in the right ear. Neuroophthalmological examination showed skew deviation with right hypotropia, excyclotorsion, and tilt of the static visual vertical directed to the right side. Immunological and serological examinations were normal. Neurological examination and extensive neuroradiological investigations failed to demonstrate any central nervous system involvement. In this patient, skew deviation and tilt of the static visual vertical were interpreted as signs of an acute unilateral otolithic dysfunction, due to a sudden idiopathic peripheral vestibular loss.

Diplopia↗

Fixation disparity in vertical vergence.

Generally, the vertical vergence response is smaller than the disparity that evokes it, even within the diplopia threshold. However, subjective and objective fixation disparity values obtained with Ogle's device agreed fairly well. The reference lines in themselves appeared to be a stimulus to align the eyes. This finding limits the value of the subjective fixation disparity method when studying the vertical fusional response. Vertical fixation disparity is dependent on time of exposure. Responses of increasing amplitude take longer to reach full amplitude from the position of rest than in the reverse direction.

Diplopia↗

Intraoperative suspension-recession technique for treatment of vertical strabismus in thyroid myopathy.

Ten patients underwent extraocular muscle surgery with an intraoperative suspension-recession technique for diplopia induced by vertical strabismus in thyroid myopathy. The average ratio of the reduction of vertical deviation to the amount of surgery was 1.8 degrees/mm (range, 1.0 degrees/mm-2.8 degrees/mm). Eight (80%) patients had single binocular vision restored in the primary position without prism or compensatory torticollis after surgery. One patient who showed evidence of abnormal thyroid function at surgery developed an overcorrection postoperatively.

Adult↗

Clival chordoma presenting as acute esotropia due to bilateral abducens palsy.

A case involving a 14-year-old girl having clival chordoma with symptoms of diplopia, acute esotropia, and bilateral abducens palsy is reported. Magnetic resonance imaging revealed a clival tumor invading the posterior edges of the bilateral cavernous sinuses. After removal of the tumor, no ocular manifestations were noted. The importance of acute esotropia as a sign indicating a possible brainstem mass is emphasized.

Abducens Nerve↗

A double-blind, randomized, placebo-controlled trial of oxcarbazepine in the treatment of bipolar disorder in children and adolescents.

OBJECTIVE: This multicenter trial examined the efficacy and safety of oxcarbazepine in the treatment of bipolar disorder in children and adolescents. METHOD: A total of 116 outpatients 7 to 18 years of age with bipolar I disorder, manic or mixed, were recruited at 20 centers in the United States and randomly assigned to receive 7 weeks of double-blinded, flexibly dosed treatment with oxcarbazepine (maximum dose 900-2400 mg/day) or placebo. The primary efficacy measure was the mean change from baseline to endpoint in the Young Mania Rating Scale (YMRS), using the last-observation-carried-forward method. RESULTS: Oxcarbazepine (mean dose=1515 mg/day) did not significantly improve YMRS scores at endpoint compared with placebo [adjusted mean change: oxcarbazepine, -10.90 (N=55); placebo, -9.79 (N=55)]. Dizziness, nausea, somnolence, diplopia, fatigue, and rash were each reported in at least 5% of the patients in the oxcarbazepine group with an incidence at least twice that of the placebo group. The majority of adverse events were mild to moderate and occurred during the titration period. Eleven patients (19%) in the oxcarbazepine group discontinued the study because of adverse events, compared with two (4%) in the placebo group. CONCLUSIONS: Oxcarbazepine is not significantly superior to placebo in the treatment of bipolar disorder in youths. While the overall adverse event profile was similar to that reported for patients with epilepsy, the incidence of psychiatric adverse events for both the oxcarbazepine and placebo groups was higher than that reported for the epilepsy population.

Adolescent↗

Minimally invasive orbital decompression for Graves' ophthalmopathy.

We reviewed a 7-year experience at a tertiary-care, academic medical center with balanced, minimally invasive decompression for Graves' ophthalmopathy, in an effort to define the goals, risks, and outcomes of surgical intervention. Endoscopic medial decompression was performed in 26 patients; 23 underwent lateral decompression as well, and 13 also had inferior decompression. Septoplasty, turbinate reduction, and orbital rim augmentation were performed as needed. The indications for surgery were threat to vision (n = 10) and proptosis with a desire to return to the predisease state (rehabilitative, n = 16). The exophthalmos improved by a mean of 4.4 mm (p < .001). All patients who had surgery for threatened vision had improved vision after the operation. There were 3 patients with new-onset postoperative diplopia, 2 of whom underwent strabismus surgery. There was 1 case of postoperative sinusitis, which resolved with oral antibiotics and nasal decongestion, and 1 case of transient ulnar neuropathy. There were no other intraoperative or postoperative complications. Modern methods of orbital decompression provide a minimally invasive, effective, and relatively safe approach to the treatment of Graves' ophthalmopathy.

Adult↗

Childhood-onset chronic inflammatory demyelinating polyradiculoneuropathy with cranial nerve involvement.

A 17-year-old male presented with chronic diplopia and generalized motor weakness. He was previously diagnosed with chronic inflammatory demyelinating polyradiculoneuropathy and acute disseminated encephalomyelitis in childhood. Cranial magnetic resonance imaging (MRI) revealed a rarely reported finding of thickening and enhancement of multiple cranial nerves. Nerve conduction studies and electromyography showed peripheral nerve demyelination with axonal involvement. There was improvement in the clinical examination, MRI, and electrophysiologic studies after combined corticosteroid and plasma exchange therapy. We review the clinical presentation, course, and response to therapy among children with chronic inflammatory demyelinating polyradiculoneuropathy, with specific emphasis on the frequency and pattern of cranial nerve involvement.

Adolescent↗

Liposarcoma metastatic to the orbit.

PURPOSE: To describe a patient with liposarcoma metastatic to the left orbit. METHODS: A 72-year-old man was admitted with diplopia and proptosis of the left eye. Previously, a retroperitoneal liposarcoma had been surgically removed followed by postoperative radiation. Visual acuity was normal. There was proptosis of the left eye, increased retrobulbar resistance and reduced mobility. Trans-septal orbital biopsies showed liposarcoma. The patient was initially treated with prednisolone and later received radio- and chemotherapy. Despite treatment he lost vision of the left eye and died a few months later. RESULTS: Histological examination of the orbital tumor revealed a spindle-cell liposarcoma similar to the primary tumor of the retroperitoneum. CONCLUSIONS: Liposarcoma metastatic to the orbit is rare, but should be suspected in a patient with proptosis caused by a space-occupying lesion and a history of liposarcoma.

Aged↗

Transient third nerve palsy in a young patient with intracranial arteriovenous malformation.

PURPOSE: To describe a patient with transient third nerve palsy as the possible presenting sign of intracranial arteriovenous malformation. METHOD: Case report. RESULT: A 24-year-old female presented to ophthalmic casualty with sudden onset binocular diplopia and was diagnosed to have right sided partial third nerve palsy. Within 30 hours the third nerve palsy had recovered completely. A MRI scan and subsequent carotid angiogram revealed a large, high flow, trans-cortical Spetzler-Martin grade 4 arteriovenous malformation. The feeder vessel of the AVM originated from the right middle cerebral artery. Superficial venous drainage was via the superficial middle cerebral vein to the right transverse sinus. The deep venous drainage was via thalamostriate veins into markedly dilated internal cerebral vein and vein of Galen (Great cerebral vein). Venous reflux was noted around the midbrain from the vein of Galen. CONCLUSIONS: Transient third nerve palsy may rarely occur secondary to intracranial arteriovenous malformation. Ophthalmologists should consider neuroimaging in the investigations for transient cases of III nerve palsy in young patients.

Adult↗

Visual performance after congenital nystagmus surgery using extended hang back recession of the four horizontal rectus muscles.

PURPOSE: To evaluate the results of surgical correction of congenital nystagmus based on the use of extensive recessions of the four horizontal rectus muscles. The outcome of this procedure in terms of visual performance of the patients has been analyzed in follow-ups of at least 1 year. METHODS: Surgery was performed on 42 patients. An extended retroequatorial recession of the four horizontal rectus muscles was performed between 13 and 15 mm from the muscle insertion with hang back sutures in every case. Preoperatively, all cases showed either a horizontal pendular (4 cases) or jerk nystagmus (38 cases). RESULTS: After surgery, all cases showed full preservation of ductions in all gaze positions. Torticollis decreased in all cases. Binocular best-corrected vision improved in 19 (45.2%) patients and in the remaining 23 (54.8%) it remained unchanged. Consecutive exotropia was observed postoperatively in 7 patients. A second surgical procedure was performed in 9 cases (21.4%) and a botulinum injection in 3 cases (7%). All cases of diplopia resolved. Patient satisfaction was high in 38 cases (96%). CONCLUSIONS: Extended recession of horizontal rectus muscles using hang back sutures offers a good option for the surgical correction of sensorial and neuromuscular nystagmus, providing improvement in binocular best-corrected vision in 45% of the patients (21.4% more than 0.2 logMar units), a high satisfaction rate, and few associated complications. In patients with low vision this can be considered a success that may improve quality of life.

Adolescent↗

Medial rectus muscle incarceration in pediatric medial orbital wall trapdoor fractures.

PURPOSE: We describe two cases of orbital trapdoor fractures with medial rectus muscle incarceration. METHODS: Small interventional case series. RESULTS: This is a retrospective university based report of two healthy males (11 and 14 years old) who developed diplopia following blunt orbital trauma. Both patients had decreased horizontal ocular motility of the involved eye with minimal additional evidence of trauma. Computed tomography (CT) demonstrated no significant bony displacement; however, the left medial rectus muscle was located within the ethmoid sinus in the first and had an abnormal size and shape in the second case. In both cases, during urgent surgical repair, the incarcerated medial rectus muscle was gently released from linear non-displaced medial wall fractures and ocular motility normalized postoperatively. CONCLUSIONS: In pediatric patients sustaining blunt orbital trauma, medial rectus incarceration should be considered and managed accordingly.

Adolescent↗

Lessons to be learned: a case study approach. Primary hyperparathyroidism simulating an acute severe polyneuritis.

The case is presented of a 65 year old lady with recent onset of neuromuscular manifestations, comprising paraparesis, areflexia and unsteady gait, along with episodes of slurring of speech and diplopia, later confirmed to be due to severe hypercalcaemia--which itself was caused by primary hyperparathyroidism. Restoration of normocalcaemia, by means of rehydration and bisphosphonate therapy, resulted in clinical improvement--whilst subsequent parathyroidectomy was followed by complete resolution of all symptoms. In order to make prompt differentiation between the neurological sequelae of hyperparathyroidism and a primary neurological disorder, a high index of suspicion is required. An urgent serum calcium assay, as part of a bone profile, is mandatory in patients who present with neurological symptoms--especially the elderly, amongst whom hyperparathyroidism is especially common.

Acute Disease↗

Locoregional proton radiotherapy of a primary cavernous sinus non-Hodgkin's lymphoma: case report.

Primary B cell lymphomas of the skull base are uncommon and lack well-defined treatment guidelines. We report a case of diffuse, large B-cell lymphoma of the cavernous sinus with sphenoid sinus and clivial extension, treated with partial resection, chemotherapy, and proton beam irradiation. To our knowledge, this is the first report of a skull-base lymphoma treated with protons. A 53-year-old female presented with a two-month history of diplopia, persistent headaches, and paresthesia over the left side of her mouth. A skull MRI revealed an enhancing mass in the right cavernous sinus and right sphenoid sinus. Transsphenoidal subtotal resection of the mass confirmed the presence of a diffuse, large B-cell lymphoma. Treatment consisted of CHOP-R chemotherapy and locoregional radiation with protons. Locoregional radiation of the lesion required moderate doses, below the radiation tolerance of adjacent normal structures. Conformal protons were utilized to minimize the volume of normal brain receiving radiation. Conformal proton beam radiotherapy to a moderate dose proved valuable in this case because it minimized the volume of normal brain receiving low to moderate doses of radiation.

Antibodies, Monoclonal↗

Bilateral ischemic optic neuropathy after transurethral prostatic resection: a case report.

BACKGROUND: Nonarteritic ischemic optic neuropathy affects the anterior portion of the optic nerve and is characterized by sudden, painless visual loss. The affected eye has a relative afferent pupillary defect. The typical funduscopic appearance includes optic disc edema, with associated nerve fiber layer hemorrhage. Risk factors include advanced age, systemic hypertension, nocturnal hypotension, diabetes mellitus, and a small cup-to-disc ratio. Bilateral presentation is rare. Postoperative optic neuropathy has been associated with nonocular surgery; risk factors include a combination of prolonged surgical times, acute systemic hypotension, anemia due to blood loss, or prone positioning. We report for the first time a patient with bilateral, simultaneous anterior ischemic optic neuropathy after elective transurethral prostatic resection. CASE PRESENTATION: A 66-year old man underwent surgery for benign prostatic hyperplasia. The preoperative blood pressure was 140/85 mmHg, hemoglobin 15.9 g/dL, and hematocrit 48.6%. Two hours postoperatively, the blood pressure, hemoglobin, and hematocrit dropped dramatically. One day later, transient horizontal diplopia developed. Funduscopy showed a congenitally small cup-to-disc ratio without papillary edema. Other ocular findings were unremarkable. By 4 days postoperatively, sudden and painless amaurosis bilaterally developed when the patient awoke with nausea and vomiting. Visual acuity was no light perception bilaterally. The optic discs were swollen with small hemorrhages. Scans of the head and orbits and electrolyte levels were normal. There were no responses on visual evoked potentials bilaterally. The blood pressure was 90/50 mm Hg, the hemoglobin 7.0 g/dL, and the hematocrit 22.9%, necessitating infusion of three units of packed red blood cells. The blood pressure, hematocrit, and hemoglobin increased to normal levels. Three months later the visual acuity remained no light perception. The pupils were unreactive and there was marked optic disc atrophy bilaterally. CONCLUSION: Bilateral and simultaneous acute ischemic optic neuropathy may be a rare but devastating surgical complication. The combination of anemia and hypotension may increase the risk of anterior ischemic optic neuropathy postoperatively after transurethral prostatic resection.

Aged↗

Are MuSK antibodies the primary cause of myasthenic symptoms?

OBJECTIVE: To investigate the morphologic, electrophysiologic, and molecular correlates of muscle-specific tyrosine kinase-seropositive [MuSK(+)] myasthenia gravis (MG). BACKGROUND: Anti-MuSK antibodies are detected in some of acetylcholine receptor-seronegative [AChR(-)] patients with MG with prominent facial, bulbar, and respiratory muscle involvement. The morphologic and electrophysiologic correlates of MuSK(+) MG have not been investigated to date. METHODS: Immunohistochemistry, electron microscopy, and in vitro electrophysiology studies were performed on an intercostal muscle specimen of a patient with MuSK(+) MG and in control subjects. MUSK was directly sequenced, and the nucleotide changes were traced with allele-specific PCR in control subjects. RESULTS: A man aged 34 years has had facial weakness since childhood and progressive bulbar and respiratory muscle weakness and intermittent diplopia since age 21 years. He has thin temporalis and masseter muscles, a high-arched palate, and an atrophic tongue. EMG shows a 36% decrement in facial muscles. His mother has similar facial features. His endplates (EPs) show no AChR or MuSK deficiency, but the amplitudes of the miniature EP potentials and currents are reduced to 35% and 55% of normal, respectively. EP ultrastructure is well preserved, but some junctional folds immunostain faintly for immunoglobulin G. Mutation analysis of MUSK reveals one rare and two common DNA polymorphisms. CONCLUSIONS: 1) The circulating anti-muscle-specific tyrosine kinase antibodies caused neither muscle-specific tyrosine kinase nor acetylcholine receptor deficiency at the endplates; 2) the reduced intercostal miniature endplate potential and current amplitudes were not accounted for by acetylcholine receptor deficiency; 3) the faint immunoglobulin G deposits at the endplates may or may not represent anti-muscle-specific tyrosine kinase antibodies; and 4) the anti-muscle-specific tyrosine kinase antibodies may not be the primary cause of myasthenic symptoms in this patient.

Adult↗

Epileptic skew deviation.

We describe a 43-year-old neurologically intact patient who reported episodes of diplopia and oscillopsia associated with a right-beating nystagmus and a skew deviation. These symptoms and signs were related to a left posterior epileptic EEG discharge. We suggest that these ocular motor signs derived from an ictal activation of the vestibular cortex, which in turn activated descending projections to the vestibular nuclei, leading to both a dynamic (right-beating nystagmus) and a static (skew deviation) vestibular imbalance.

Adult↗