摘要:

Microangiopathy of brain, retina, and inner ear is a rare syndrome manifesting as arteriolar occlusions of the brain, retina, and inner ear, with resultant encephalopathy, visual, and hearing loss. Despite exhaustive laboratory examinations in these patients, no evidence of a systemic disease can be found. We treated and followed an adolescent with this disorder who initially presented with a branch retinal artery occlusion. A unique finding in this case was retinal vessel wall hyperfluorescence noted five days prior to retinal infarction. The patient developed recurrent branch artery occlusions, sensorineural hearing loss, and central nervous system infarctions despite anticoagulation and immunosuppressive treatment.

ISSN:1070-8022    

出版商:OVID    

年代:1995

数据来源: OVID

摘要:

PurposeThe diagnoses of both anterior ischemic optic neuropathy (AION) and optic neuritis are clinical ones with significant overlap of symptoms and signs. This study investigates the presence and character of pain at the onset of AION, in order to evaluate this symptom as a differentiating diagnostic feature between optic neuritis and AION.MethodsForty-one consecutive patients over 45 years of age with a clinical syndrome consistent with AION were questioned about the presence and character of associated pain.MethodsPain was reported by 12% (5 of 41) of the patients with AION. This is contrasted with data compiled on 448 patients enrolled in the optic neuritis treatment trial, of whom 92.2% complained of pain.MethodsWhile there is overlap in the incidence and character of pain in AION and optic neuritis, its presence/absence remains a useful differentiating feature.

ISSN:1070-8022    

出版商:OVID    

年代:1995

数据来源: OVID

摘要:

A 54-year-old man developed an acquired monocular elevation paresis. Forced lid closure or oculocephalic reflexes failed to elevate the affected eye in contrast to the contralateral eye, which turned fully upward using either maneuver. A mass lesion compressing the lateral aspect of the right oculomotor nerve at the exit of the brainstem was found. The monocular elevation paresis in this patient reflects damage of the oculomotor nerve fascicles that supply the inferior oblique and superior rectus muscles. This case supports the accepted topographical fascicular arrangement of the oculomotor nerve, with the inferior oblique and superior rectus being the most lateral and caudal, and the pupilloconstrictor fibers and the inferior rectus being most medial and rostral.

ISSN:1070-8022    

出版商:OVID    

年代:1995

数据来源: OVID

摘要:

Investigation of a maternal family of three generations of Leber's hereditary optic neuropathy (LHON) showed four affected and three unaffected individuals. Two of the four patients had recovered near-normal vision, one spontaneously, and one following treatment with idebenone, a quinol compound. One patient whose visual impairment persisted was a heavy consumer of alcohol and tobacco. Molecular genetic analysis of 12 known primary or secondary mutations in mitochondrial DNA (mtDNA) associated with LHON revealed only the 11778 mutation in a homoplasmic fashion with no secondary mutations. The variations in clinical outcome thus could not be explained by synergistically interacting secondary mutations in mtDNA. Environmental factors may play an etiologic role in the development of optic atrophy.

ISSN:1070-8022    

出版商:OVID    

年代:1995

数据来源: OVID

摘要:

A patient with multiple neurological deficits and biopsyproven mitochondrial myopathy with mutation of tRNALEUat nucleotide 3243 was referred for eye movement evaluation. He had restricted range of voluntary motions in all directions and full range of eye movements on passive rotation of head while fixating a visual target. Eye movement recordings revealed decreased horizontal and vertical saccadic velocities and markedly decreased smooth pursuit gain in both directions. The vestibulo-ocular reflex showed gain abnormalities with many saccadic intrusions on the smooth reflex response. His brother, with similar mutation, was clinically asymptomatic. However, his eye movement recordings revealed slow horizontal saccadic velocities leftward and normal saccadic velocities rightward in both eyes as well as in upward and downward direction. Smooth pursuit and vestibulo-ocular reflexes were within normal limits. Although eye movement abnormalities are seen commonly in mitochondrial myopathies, the exact mechanism is not known. Our cases suggest supranuclear dysfunction as one of the mechanisms for ophthalmoparesis.

ISSN:1070-8022    

出版商:OVID    

年代:1995

数据来源: OVID

摘要:

We report a patient who developed conjugate horizontal gaze deviation and ipsipulsion of saccades from a lateral medullary infarction. Recent evidence suggests that the gaze deviation may result from increased inhibition of the ipsilateral vestibular nucleus and ipsipulsion of saccades from decreased excitation of the contralateral ocular premotor areas of the brainstem reticular formation. Interruption of the olivocerebellar pathways may account for both of these ocular motor abnormalities.

ISSN:1070-8022    

出版商:OVID    

年代:1995

数据来源: OVID

摘要:

Direct carotid-cavernous sinus fistulas that present with signs and symptoms contralateral to the arterial supply of the fistulas are not uncommon. We present a thoroughly documented case of adural-cavernous sinus fistula with symptoms exclusively contralateral to the arterial source, a rarer entity. The patient presented with a red, proptotic right eye and a history of transient horizontal diplopia and a “feeling of fullness” in that eye. Magnetic resonance imaging (MRI) of the brain and orbits performed at another hospital had shown no abnormalities. Carotid angiography performed on the right side was normal; carotid angiography performed on the left side showed a dural-cavernous sinus fistula, with shunting from branches of the left external carotid artery directly to the right cavernous sinus. Orbital duplex color-flow sonography showed reverse flow in a dilated right superior ophthalmic vein. This unusual manifestation of a dural-cavernous sinus fistula offers insight into the pathophysiology of arteriovenous fistulas involving the cavernous sinus, and is a reminder that bilateral injections are required when performing carotid angiography to characterize these disorders.

ISSN:1070-8022    

出版商:OVID    

年代:1995

数据来源: OVID

摘要:

Temporal arteritis is a systemic necrotizing vasculitis for which the only effective treatment is systemic corticoste-roids. A review of the literature suggests that there is a significant mortality rate in untreated patients but that those patients who receive adequate corticosteroid treatment rarely experience a reduced life expectancy. I had the opportunity to evaluate a patient with temporal arteritis who eventually died from disseminated aspergillosis 6 weeks after beginning corticosteroid treatment. A high index of suspicion for infections with opportunistic organisms should be maintained in patients with temporal arteritis receiving corticosteroids.

ISSN:1070-8022    

出版商:OVID    

年代:1995

数据来源: OVID

摘要:

We report a case of transient ictal cortical blindness in a 63-year-old woman. We reviewed the literature of all seizure-induced bilateral blindness. After careful evaluation of 44 cases, it was evident that the cases could be categorized into three groups with different etiologies, duration of blindness, and probable visual prognosis. Transient cortical blindness may occur with unilateral focal seizure or with bilateral seizure activity of the primary generalized type. Seizure-induced blindness may be ictal or postictal (Todd's) phenomenon or a permanent consequence following status epilepticus. Its duration varies between less than 1 minute to 4 months, or can be permanent. Our case of cortical blindness is related to new onset occipital epilepsy from a left occipital focus, and adds to the evidence that cortical blindness occasionally occurs as an uncommon manifestation of seizure.

ISSN:1070-8022    

出版商:OVID    

年代:1995

数据来源: OVID

摘要:

A 46-year-old woman observed transient unilateral my-driasis during a classic migraine attack. One week later she experienced a similar episode after which anisocoria was persistent. Subsequent examination showed the clinical and pharmacologic features of a postganglionic parasympathetic paresis (Adie's tonic pupil). This case confirms the hypothesis that transient mydriasis accompanying migraine is due to interruption of parasympathetic innervation rather than sympathetic overactivity.

ISSN:1070-8022    

出版商:OVID    

年代:1995

数据来源: OVID