Bacillary angiomatosis associated with myositis in a patient infected with human immunodeficiency virus

PURPOSE: To report a patient with optic nerve avulsion caused by forceful rotation of the globe that occurred when his thumb penetrated the orbit while he was diving. METHODS: A 17-year-old boy was initially examined for sudden loss of vision after jumping feet first from a bridge 50 feet above a river. Upon hitting the water, he felt his right thumb push into his right globe. The patient underwent ophthalmologic and imaging examination. RESULT: Examination disclosed a tear of the optic nerve head from the sclera temporally in the right eye. CONCLUSION: Optic nerve avulsion occurs secondary to forceful rotation of the eye with tearing of the optic nerve as it exits the globe.     

The effect of immunotoxins directed against CD80 and CD86 on primary T-cell alloresponses

A 13-year-old boy presented with acute loss of vision in his right eye of 2 weeks' duration. He had a high fever and was ill for several days, then improved but suffered recurrent episodes of sweating and a high fever. Ophthalmoscopy of the right eye showed optic disk edema, mild vitreous cells, and minimal exudates in the macula. Bartonella henselae titers were positive. A diagnosis of optic disk edema with a macular star secondary to cat-scratch disease was made. The patient was treated with doxycycline and made a dramatic improvement to visual acuity of 20/30 with a minimal residual relative central scotoma. The optic disk edema and macular star resolved, and the patient was left with mild optic atrophy in the right eye.     

Human bites of the face. A review of 22 cases

A 41-year-old woman presented with a unilateral optic neuropathy that progressed to no light perception 7 weeks later. The patient was hospitalized for progressive dyspnea; respiratory failure ensued and the patient died 10 days after admission. Antemortem pulmonary biopsies and the results of necropsy revealed lymphomatoid granulomatosis with extensive involvement of the lungs and left optic nerve. This is the first case report of lymphomatoid granulomatosis presenting as a unilateral optic neuropathy.     

The cupped disc. Who needs neuroimaging?

OBJECTIVE: To determine the incidence of positive neuroradiologic studies in consecutive patients with glaucoma associated with normal intraocular pressure and to compare the psychophysical and clinical characteristics of these eyes with eyes with disc cupping associated with intracranial masses. DESIGN: Retrospective case-controlled study. PARTICIPANTS: Fifty-two eyes of 29 patients with glaucoma associated with normal intraocular pressure and 44 eyes of 28 control patients with compressive lesions were reviewed. INTERVENTION: The medical records of consecutive glaucoma patients with normal intraocular pressure who underwent brain magnetic resonance imaging or computed tomography scanning as part of a diagnostic evaluation between January 1, 1985, and July 1, 1995, were reviewed. A masked reading of optic nerve photographs and visual fields was performed by one observer. A similar analysis was performed on a control group of consecutive patients with nonglaucomatous optic nerve cupping with known intracranial mass lesions. MAIN OUTCOME MEASURES: The neuroradiologic findings, clinical characteristics, optic nerve head appearance, and patterns of visual field loss were compared between groups. RESULTS: None of the patients diagnosed with glaucoma had neuroradiologic evidence of a mass lesion involving the anterior visual pathway. Compared to control subjects, patients with glaucoma were older (P = 0.0001), had better visual acuity (P = 0.002), greater vertical loss of neuroretinal rim tissue (P = 0.0001), more frequent optic disc hemorrhages (P = 0.01), less neuroretinal rim pallor (P = 0.0001), and more nerve fiber bundle visual field defects aligned at the horizontal midline (P = 0.0001). Visual acuity less than 20/40, vertically aligned visual field defects, optic nerve pallor in excess of cupping, and age younger than 50 years were 77%, 81%, 90%, and 93% specific for nonglaucomatous cupping associated with compressive lesions, respectively. CONCLUSIONS: Anterior visual pathway compression is an uncommon finding in the neuroradiologic evaluation of patients with a presumptive diagnosis of normal-tension glaucoma. Younger age, lower levels of visual acuity, vertically aligned visual field defects, and neuroretinal rim pallor may increase the likelihood of identifying an intracranial mass lesion.     

An extensive cranial base meningioma extending bilaterally into Meckel's cave: case report

OBJECTIVE AND IMPORTANCE: A patient with an extensive cranial base meningioma that included bilateral invasion of Meckel's cave underwent surgical resection and had an unexpected rare complication, malocclusion from bilateral trigeminal dysfunction. CLINICAL PRESENTATION: A 19-year-old male patient was admitted to our hospital with alternating painful ophthalmoplegia. He had been blind since the age of 10 years. At the time of admission, neurological findings included bilateral visual loss and optic atrophy. Magnetic resonance images showed an extensive tumor located at the planum sphenoidale, tuberculum sellae, and bilaterally at Meckel's cave and the medial tentorial incisura. INTERVENTION: The patient underwent a two-stage operation. During the first procedure, the masses in the planum sphenoidale, tuberculum sellae, and the left side of Meckel's cave were excised intradurally. At the second operation, the mass in the right side of Meckel's cave was excised extradurally and the tentorial mass was removed intradurally. The patient's postoperative course was complicated by bilateral trigeminal nerve dysfunction, which caused malocclusion. CONCLUSION: Bilateral dysfunction of the trigeminal nerve may cause a number of problems. Thus, extreme caution must be taken to preserve the function of this nerve.     

Orbital compression syndrome in sickle cell disease

BACKGROUND: Orbital complications are an uncommonly reported finding in sickle cell disease. METHODS: The authors review the reported orbital manifestations of sickle cell disease and discuss a patient with hemoglobin sickle beta(0) thalassemia in whom rapidly progressive bilateral orbital compression developed. RESULTS: Computed tomography of the orbits in a patient with fever, headache, orbital swelling, and optic nerve dysfunction displayed bilateral superior subperiosteal cystic masses. Surgical exploration showed bilateral liquefied hematomas, which were evacuated. Recovery was complete 13 days after surgery. A mild recurrence 14 months later resolved with conservative treatment. The literature contains 11 reports of 16 young patients with sickle cell disease (15 sickle cell disease [Hb SS] and 1 hemoglobin sickle cell disease [Hb SC]) with rapidly developing findings ranging from frontal headache, fever, and eyelid edema to bilateral complete orbital compression syndrome. Including our patient, 60% had orbital hemorrhage on computed tomography. Ten of 12 patients tested were found to have orbital bone marrow infarctions. Sixteen of 17 patients had complete recovery; 13 were treated conservatively and 4 surgically. Only 2 of 17 had recurrence. CONCLUSIONS: Orbital complications in sickle cell disease are unusual manifestations in which a vaso-occlusive process in the marrow space around the orbit results in frontal headache, fever, eyelid edema, and often orbital compression syndrome. Subperiosteal hematomas are common and appear to result from bone marrow infarctions. Appropriate management requires a thorough evaluation to exclude other hemorrhagic, infectious or neoplastic processes, as well as vigilant ophthalmic monitoring. Supportive care is effective, unless optic nerve dysfunction or large hematomas are present, which would indicate that surgical evacuation is warranted to prevent loss of vision and to speed recovery.     

Chromogranin A in cerebrospinal fluid: a biochemical marker for synaptic degeneration in Alzheimer''s disease?

OBJECTIVE: To describe the clinical, radiologic and histopathological features of two cases of carcinoma metastatic to the optic nerve with different pathways of spread. DESIGN: Chart review. SETTING: Oncology clinic in Montreal. PATIENTS: One patient previously treated for breast carcinoma and one patient previously treated for small-cell carcinoma of the lung. RESULTS: The patient with breast carcinoma was found to have an elevated mass involving her right optic nerve head. This finding was confirmed by B-scan ultrasonography. The other patient initially presented with left retinal metastasis and subsequently manifested massive secondary involvement of the ipsilateral optic nerve, documented by computed tomography. Both patients were initially treated with radiotherapy to the eye and orbit, but postmortem histopathological study showed that this treatment had little effect on the tumour. CONCLUSIONS: The histopathological observations suggest that if radiotherapy is to be used in the treatment of metastasis to the optic nerve, the dosage of radiation should be higher than that recommended for the treatment of choroidal metastasis.     

Pancreatitis induced by codeine: a case report with positive rechallenge

PURPOSE: To postulate a causal relation between optic nerve hypoplasia and a suprasellar teratoma. METHOD: Case report. RESULTS: A 6-month-old infant with suprasellar teratoma was visually inattentive and had searching nystagmus. He had moderately severe, bilateral optic nerve hypoplasia with the left eye being somewhat worse than the right eye. CONCLUSIONS: Optic nerve hypoplasia is a major cause of impaired vision in children and rarely has been attributed to an intracranial tumor. Our case, involving a patient with a suprasellar teratoma and optic nerve hypoplasia, supports a causal relation between the two.     

Primary carcinoma of the female urethra

A 26-year-old man went blind as part of a multifocal central nervous system disease. Bilateral optic nerve head pallor developed four weeks later. There had been no papilledema. In this setting, the appearance of optic atrophy without preceding papilledema in part led to the clinical diagnosis of severe disseminated encephalomyelitis. At autopsy multiple brain abscesses were found, including an area of inflammation within the chiasm.     

Unilateral neonatal cystic disease of the kidney as first manifestation of tuberous sclerosis]

Traumatic optic neuropathy (TON) causes blindness of varied severity and occurs infrequently as a complication of closed head injury. A case is presented of TON that occurred in a patient who suffered complete T4 paraplegia from a motorcycle accident but in whom no severe head injury took place. In this case, high-dose intravenous methylprednisolone was begun for the spinal cord injury and repeated 24 hours later for the TON. Vision improved from near total blindness to 20/400 in the left eye (OS) and 20/130 in the right eye (OD). Two weeks later, however, the patient's vision suddenly worsened. Magnetic resonance imaging (MRI) using fat suppression confirmed a lesion along the optic nerve consistent with TON. A third course of methylprednisolone again led to improved vision. The steroids were then tapered orally over 2 weeks and the patient had no further relapses. Moderate to severely impaired vision of 20/ 400 OS and 20/130 OD continues to interfere with the patient's function and spinal cord rehabilitation program. It was concluded that a steroid taper was important in maintaining initial visual gains in this case. Awareness of TON and careful attention to the patient's clinical course can minimize deficit and maximize functional outcomes.     

Profile of some risk factors for coronary heart disease in a developing country: Nigeria

We examined visual evoked potentials and pattern electroretinograms in a patient with Tolosa-Hunt syndrome associated with optic nerve involvement. The 82-year-old woman developed unilateral painful ophthalmoplegia and visual loss in the right eye. Magnetic resonance imaging showed an abnormal soft-tissue area in the right cavernous sinus and the right orbital apex. Symptoms responded rapidly to treatment with corticosteroid. Visual evoked potentials to flash and pattern stimuli were both remarkably reduced and delayed in the right eye in the acute stage; however they improved to almost normal after steroid therapy. The pattern electroretinogram recorded in the acute stage was normal bilaterally. These results indicate that optic nerve involvement in Tolosa-Hunt syndrome can be mild and reversible.     

Role of hydrogen peroxide in experimental optic neuritis. A serial quantitative ultrastructural study

In order to determine the role of H2O2 in demyelination of the optic nerve, serial quantitative analysis of H2O2-derived cerium perhydroxide reaction product particles was obtained by computerized digitization of electron micrographs of the myelinated retrobulbar optic nerve, unmyelinated optic nerve head, and the optic nerve sheath of guinea pigs sensitized for experimental allergic encephalomyelitis (EAE) and euthanized 3-14 days later. We found that cerium perhydroxide reaction product particles were greatest in the myelinated optic nerve 3 days after antigenic sensitization, but at this focus decreased 7-14 days after antigenic sensitization. Reaction product accumulated in the unmyelinated optic nerve head and optic nerve sheath 3-14 days after sensitization. These results in the myelinated optic nerve suggest H2O2 consumption results in peroxidation of myelin lipid as demyelination proceeds 7-14 days after antigenic sensitization. Hydrogen peroxide accumulation in the optic nerve head and the optic nerve sheath appears to provide a reservoir for diffusion of H2O2 into the retrobulbar optic nerve and adjacent perineural nerve, contributing to the frequent predilection for optic nerve involvement in EAE and perhaps in multiple sclerosis.     

Optic disk vasculitis

A 44-year-old woman developed progressive loss of vision associated initially with a swollen optic disk, and later with optic atrophy and a diffuse retinal vasculopathy, which caused extensive retinal hemorrhagagic. Histopathologic examination showed hemorrhagagic infarction of the retina, as well as infarction of the anterior optic nerve. In the optic nerve, the central retinal vessels showed extensive phlebitis and occlusion of many small arterioles.     

Regulation of human mast cell and basophil function by cAMP

BACKGROUND: Ischemic optic neuropathy (ION) is an infarction of the anterior or, less frequently, posterior part of the optic nerve, usually due to a disease of small arteries supplying the optic nerve. Carotid stenosis or occlusions are rare causes, and among them, carotid dissections have been so far reported in only 5 cases. METHODS: We describe 4 patients with ION (2 anterior and 2 posterior) due to internal carotid artery dissection of a consecutive series of 110 patients with internal carotid artery dissection (3.6%). RESULTS: None of the patients had signs of central retinal artery occlusion or ischemic ocular syndrome. Ischemic optic neuropathy occurred after a mean of 5.3 days (range, 3-8 days) following the first symptom, which was headache in 1 patient, transient monocular blindness in 2, and hemispheric transient ischemic attack in 1. One patient had associated Homer syndrome, and 2 had severe ipsilateral headache and orbital pain. None of the patients developed a cerebral infarction. These features differ from those observed in "classic" nonarteritic anterior ION and might therefore point to carotid dissection. CONCLUSION: Ischemic optic neuropathy may occur as an early sign of carotid dissection: young age, previous transient monocular blindness, an association with pain, Horner syndrome, or hemispheric transient ischemic attacks are suggestive of this cause and should prompt confirmatory investigations.     

Fenestrated oculomotor nerve caused by internal carotid-posterior communicating artery aneurysm: case report

OBJECTIVE AND IMPORTANCE: The fenestrated oculomotor nerve associated with the internal carotid-posterior communicating artery aneurysm is very rare. CLINICAL PRESENTATION: A 48-year-old woman had a history of subarachnoid hemorrhage caused by a ruptured right middle cerebral artery aneurysm, which was wrapped with good postoperative course. Twenty years later, the patient suffered frontal headache with a mild oculomotor nerve paresis in the right side. Follow-up neuroimaging studies demonstrated a de novo right internal carotid-posterior communicating artery aneurysm. INTERVENTION: The aneurysm was exposed and clipped via a right pterional route. The fenestrated oculomotor nerve associated with the aneurysm was confirmed at surgery. CONCLUSION: We speculated that the fenestration was most likely caused, by the growth of the aneurysm.     

Optic nerve decompression in fibrous dysplasia: indications, efficacy, and safety

Optic nerve decompression is controversial in posttraumatic visual loss and as a prophylactic therapy in fibrous dysplasia involving the optic canal. It is less controversial for the treatment of optic nerve compression in fibrous dysplasia. Thirteen patients with craniomaxillofacial fibrous dysplasia who underwent 16 optic nerve decompressions at the Chang Gung Craniofacial Centre for both therapeutic (10) and prophylactic (6) indications are reported. One patient sustained a clinically useful improvement in vision in response to two therapeutic decompressions, and another demonstrated marked improvement after therapeutic decompression, with visual acuity improving from 0.4 to 1.0 in the affected eye. No patient underwent a permanent deterioration of vision as a result of either therapeutic or prophylactic decompression. One patient suffered extraocular muscle dysfunction that was corrected with strabismus surgery. A review of the literature clarifies the controversies and allows establishment of indications for optic nerve decompression.     

Juvenile pilocytic astrocytoma masquerading as amblyopia

PURPOSE: A healthy 13-year-old girl, previously diagnosed with amblyopia in her right eye, was seen in consultation after her vision continued to decrease. METHODS: A complete ophthalmologic examination including visual field testing and optic nerve photography was performed in the neuro-ophthalmologic clinic. Magnetic resonance imaging study was also obtained. RESULTS: Visual sensory deficits and pale optic nerves were noted on clinical examination. Visual field testing showed a chiasmatic junctional defect. Magnetic resonance imaging verified a large chiasmatic mass, histologically proven to be a juvenile pilocytic astrocytoma. CONCLUSION: Early recognition of signs and symptoms of chiasmatic lesions is essential for preventing visual loss.