1/416. Acute sensorimotor polyneuropathy with tonic pupils and an abduction deficit: an unusual presentation of polyarteritis nodosa. A tonic pupil may occur in isolation or as part of a systemic disorder. We report a patient who developed tonic pupils and an abduction deficit in the setting of polyarteritis nodosa. The combination of a tonic pupil and an abduction deficit should suggest the possibility of a vasculopathic disorder, because the ciliary ganglion and lateral rectus muscle are both supplied by the lateral muscular artery. Widespread small artery and arteriolar narrowing and occlusion are the hallmarks of polyarteritis nodosa. Unusual ischemic syndromes may occur, such as this rare combination of neuro-ophthalmic signs, by involvement of both the nutrient artery and its collaterals. We are unaware of other reports of neuropathic tonic pupils in association with polyarteritis nodosa. ( info) |
| PURPOSE: To report the occurrence of acquired Brown syndrome and associated magnetic resonance imaging findings in a patient with psoriasis. methods: A 42-year-old woman with a history of psoriasis developed pain, double vision, and limited elevation of her left eye in adduction. An orbital magnetic resonance image with gadolinium enhancement was obtained. RESULTS: Orbital magnetic resonance image disclosed abnormal enhancement of the left trochlea/tendon complex. The patient's symptoms resolved with corticosteroid therapy. CONCLUSIONS: Acquired Brown syndrome may be associated with psoriasis. The inflammation of the trochlea/tendon complex that can cause acquired Brown syndrome can be demonstrated on magnetic resonance image. ( info) |
3/416. Monocular involuntary eyelid closure. OBJECTIVES: Monocular blepharospasm (MBLS) is precipitated by ipsilateral ocular pathology and becomes bilateral over time. Two patients are reported with monocular visual disturbance exhibiting monocular involuntary eyelid closure (MIEC), resembling MBLS. MATERIAL AND methods: A 31-year-old female with hyperthyroidism developed monocular polyopia with left eye vision on binocular fixation followed by ipsilateral visual extinction. Monocular vision was otherwise normal. A 57-year-old male with renal failure developed monocular blindness secondary to retinal hemorrhage. RESULTS: The first patient had anisocoria and proptosis. brain magnetic resonance (MR) was normal. The second patient had involuntary movements of the left eye. Head computerized tomography (CT) was normal. Both patients exhibited MIEC of the eye with visual impairment that could be overcome voluntarily, but reappeared upon distraction. Neither of the two developed BLS in long-term follow-up. CONCLUSION: MIEC represents a complication of ipsilateral ocular pathology in the absence of other involuntary movements, apraxia of eyelid opening, or blepharospasm (BLS). MIEC needs to be distinguished from MBLS, since not all MBLS becomes bilateral. ( info) |
4/416. Growing skull fracture of the orbital roof. Case report. Growing skull fractures are rare complications of head trauma and very rarely arise in the skull base. The clinical and radiological finding and treatment of a growing fracture of the orbital roof in a 5-year-old boy are reported, and the relevant literature is reviewed. The clinical picture was eyelid swelling. Computed tomography (CT) scan was excellent for demonstrating the bony defect in the orbital roof. Frontobasal brain injury seems to play an important role in the pathogenesis of the fracture growth. Growing skull fracture of the orbital roof should be considered in the differential diagnosis in cases of persistent ocular symptoms. craniotomy with excision of gliotic brain and granulation tissue, dural repair and cranioplasty is the treatment of choice. ( info) |
| The association of an acute reversible encephalopathy with transient occipital lobe abnormalities on imaging studies is well known. This condition has been called reversible posterior leukoencephalopathy syndrome. The clinical presentation usually includes seizures, headache, altered mental status, and blindness, often associated with hypertension and immunosuppressants. The authors discuss a two-year-old male with down syndrome who presented 2 months after allogeneic bone marrow transplantation with severe oculogyric crisis, without other complaints. The patient was being treated for hypertension and was receiving cyclosporine for prophylaxis of graft-vs-host disease. A computed tomography scan of the head revealed marked bilateral lucencies mainly involving the white matter of the occipital lobes, with a few foci of punctate hemorrhage. The condition improved when cyclosporine was discontinued, but an area of leukomalacia was identified on follow-up magnetic resonance imaging. To the authors' knowledge, oculogyric crisis as a presentation of reversible posterior leukoencephalopathy has not been previously described. Recognizing this association is important, because patients receiving cyclosporine are often receiving other medications that can potentially cause dystonic eye movements, possibly leading to a delay in diagnosis and treatment, which can result in an irreversible neurologic deficit. ( info) |
6/416. Paraneoplastic opsoclonus-myoclonus secondary to malignant melanoma. Opsoclonus-myoclonus is a well-described paraneoplastic syndrome that most often occurs in association with small cell carcinoma of the lung and breast carcinoma. To the best of our knowledge, we describe the first reported case of paraneoplastic opsoclonus-myoclonus occurring is association with malignant melanoma. Antineuronal antibodies were not identified despite repeated testing. No therapeutic benefit was observed following corticosteroid administration. ( info) |
7/416. Acquired Pseudo-Brown's syndrome immediately following Ahmed valve glaucoma implant. A 76-year-old woman noted vertical and horizontal diplopia one day following placement of an Ahmed valve in the superonasal quadrant of her left eye. She was unable to elevate her left eye, especially in adduction. She refused implant removal and strabismus surgery alone failed to satisfactorily resolve her problem. On forced duction testing, the implant became wedged between the globe and orbit superonasally. Subsequent repositioning of the valve resulted in resolution of her motility problem. Implant-orbital disproportion can produce a pseudo-Brown's syndrome. Surgeons are encouraged to perform forced duction testing at the time of glaucoma implant placement to detect and prevent this complication. ( info) |
8/416. The somatic component of schizophrenia: a dissociation of the goals of visual attention and bifoveal fixation? The presence of disorders of eye movements is today regarded as 'the strongest candidate for a genetically transmitted biological trait marker of schizophrenic disorders' (1). The present study is based on the experience, rather than the behaviour, of one patient in a search for a method of objectifying his visual problems. This method was found to be a simple test, which demonstrates a disturbance of fixation: while one eye accommodated on the figure without vergence, the other, vergent, eye fused with the image of the related background. The disorder had been misdiagnosed as 'exophoria' in conventional ophthalmological examinations, because prevailing ophthalmological theory accepts only one mode of vision; according to the most recent researches, however, it is necessary to distinguish two complementary modes of vision--one for panorama and one for detail--which differ in their coordination of vergence and accommodation. This new bimodal theory of vision--presented here for the first time--made it possible to understand the cause of the disorder as a substitution of sighting for fixation, due either to a disinhibition of panorama vision during fixation vision, or to an interchange of ipsilateral temporal and contralateral nasal projections from the retina, both associated with a fixation disparity. After correction of the patient's fixation disparity according to an unusual method, the dissociation of the visual goals was remedied and the mental disturbances of the patient vanished. ( info) |
9/416. Palatal myoclonus in postinfectious opsoclonus myoclonus syndrome : a case report. An adult male presenting with acute onset opsoclonus, myoclonus and cerebellar ataxia is being reported. Patient had myoclonus involving limbs and palate. There are only a few reported cases associated with palatal myoclonus. Patient showed gradual spontaneous recovery. Possibility of underlying malignancy was excluded by detailed investigations. ( info) |
10/416. A case of paroxysmal tonic upgaze of childhood with ataxia. Paroxysmal tonic upgaze of childhood is a rare, distinctive, childhood syndrome that may be associated with ataxia and sometimes strabismus or amblyopia. Neurological examination as well as metabolic studies, electroencephalogram and neuroradiological investigations are normal in these patients. Although it has been considered as an age-related, dopa-sensitive dystonia, the exact pathogenetic mechanism is still unknown. Aggravation of attacks by fatigue, intercurrent infection or vaccination, and possible corticomesencephalic dysmaturation may underlie this abnormality. We report on a sporadic case of paroxysmal tonic upgaze with ataxia in which there was prompt aggravation of symptoms with sleep without response to levodopa treatment. This case suggests a different underlying pathogenetic mechanism from dopaminergic pathways for this syndrome. ( info) |