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2.
J Neuroophthalmol ; 21(2): 99-102, 2001 Jun.
Article in English | MEDLINE | ID: mdl-11450911

ABSTRACT

We report two patients who developed isolated visual symptoms and signs as initial manifestations of Creutzfeldt-Jakob disease (CJD). Both patients had normal conventional T1- and T2-weighted brain magnetic resonance (MR) images; in one patient, early cortical abnormalities were detected by diffusion-weighted and fluid attenuated inversion recovery (FLAIR) magnetic resonance imaging (MRI). Results from the cerebrospinal fluid assay for the 14-3-3 brain protein were also negative in one patient, despite pathologic confirmation of CJD at autopsy. The Heidenhain variant of CJD should be considered in all patients who present with isolated visual manifestations, including homonymous hemianopsia and normal conventional brain MRI. Diffusion-weighted and FLAIR MRI may demonstrate early cortical abnormalities in patients with CJD. The CSF assay for the 14-3-3 protein may be normal, even in pathologically confirmed cases.


Subject(s)
Brain/pathology , Creutzfeldt-Jakob Syndrome/diagnosis , Hemianopsia/diagnosis , 14-3-3 Proteins , Aged , Creutzfeldt-Jakob Syndrome/cerebrospinal fluid , Fatal Outcome , Female , Hemianopsia/cerebrospinal fluid , Humans , Magnetic Resonance Imaging , Male , Tomography, Emission-Computed , Tyrosine 3-Monooxygenase/cerebrospinal fluid
3.
Arch Ophthalmol ; 119(4): 516-29, 2001 Apr.
Article in English | MEDLINE | ID: mdl-11296017

ABSTRACT

OBJECTIVE: To demonstrate spontaneous regression of large, clinically symptomatic optic pathway gliomas in patients with and without neurofibromatosis type 1 (NF-1). METHODS: Patient cases were collected through surveys at 2 consecutive annual meetings of the North American Neuro-Ophthalmology Society (NANOS) and through requests on the NANOSNET Internet listserv. Serial documentation of tumor signal and size, using magnetic resonance imaging in 11 patients and computed tomography in 2 patients, was used to evaluate clinically symptomatic optic pathway gliomas. All tumors met radiologic criteria for the diagnosis of glioma and 4 patients had biopsy confirmation of their tumors. In 3 patients, some attempt at therapy had been made many years before regression occurred. In one of these, radiation treatment had been given 19 years before tumor regression, while in another, chemotherapy had been administered 5 years before signal changes in the tumor. In the third patient, minimal surgical debulking was performed 1 year before the tumor began to shrink. RESULTS: Spontaneous tumor shrinkage was noted in 12 patients. Eight patients did not have NF-1. In an additional patient without NF-1, a signal change within the tumor without associated shrinkage was detected. Tumor regression was associated with improvement in visual function in 10 of 13 patients, stability of function in 1, and deterioration in 2. CONCLUSIONS: Large, clinically symptomatic optic gliomas may undergo spontaneous regression. Regression was seen in patients with and without NF-1. Regression may manifest either as an overall shrinkage in tumor size, or as a signal change on magnetic resonance imaging. A variable degree of improvement in visual function may accompany regression. The possibility of spontaneous regression of an optic glioma should be considered in the planning of treatment of patients with these tumors.


Subject(s)
Brain Neoplasms/physiopathology , Neoplasm Regression, Spontaneous , Neurofibromatosis 1/physiopathology , Optic Nerve Glioma/physiopathology , Adolescent , Brain Neoplasms/diagnosis , Child , Child, Preschool , Female , Humans , Infant , Magnetic Resonance Imaging , Male , Neurofibromatosis 1/diagnosis , Optic Nerve Glioma/diagnosis , Tomography, X-Ray Computed
4.
Pediatr Neurosurg ; 31(6): 322-5, 1999 Dec.
Article in English | MEDLINE | ID: mdl-10702733

ABSTRACT

Intracranial aneurysms are rare in infancy. The commonest presentation is intracranial hemorrhage, but signs of mass effect are more frequent than in adults. We report 2 infants with cerebral aneurysms, one presenting with macrocephaly and another with strabismus. Both had visual loss and optic disc pallor; MRI revealed a suprasellar mass and anterior visual pathway compression. In both cases, the preoperative diagnosis was craniopharyngioma. It is essential to recognize that, although exceedingly uncommon, cerebral aneurysms do occur in infants and have features that differ from those in adults.


Subject(s)
Carotid Artery, Internal , Hydrocephalus/complications , Intracranial Aneurysm/complications , Optic Atrophy/etiology , Optic Nerve Diseases/etiology , Calcinosis/pathology , Calcinosis/surgery , Carotid Artery, Internal/pathology , Carotid Artery, Internal/surgery , Child , Child, Preschool , Diagnosis, Differential , Female , Humans , Hydrocephalus/diagnosis , Hydrocephalus/surgery , Infant , Intracranial Aneurysm/diagnosis , Intracranial Aneurysm/surgery , Magnetic Resonance Imaging , Optic Atrophy/pathology , Optic Atrophy/surgery , Optic Chiasm/blood supply , Optic Chiasm/pathology , Optic Chiasm/surgery , Optic Nerve Diseases/pathology , Optic Nerve Diseases/surgery , Tomography, X-Ray Computed
6.
J Neuroophthalmol ; 16(2): 115-9, 1996 Jun.
Article in English | MEDLINE | ID: mdl-8797168

ABSTRACT

Systemic mastocytosis is characterized by an increased number of mast cells in multiple organs particularly skin. A 55-year-old man with mastocytosis presented with nyctalopia caused by malabsorption of vitamin A. Diagnosis was made by documenting a low vitamin A level and an ERG that showed rod-cone deficiency with rods affected more than cones. Vitamin A therapy led to return of good visual function. To our knowledge, this is the first reported case of mastocytosis induced nyctalopia. Vitamin A deficiency should be considered as a potential cause of visual loss in patients with sudden onset of night blindness.


Subject(s)
Mastocytosis/complications , Night Blindness/etiology , Electroretinography , Humans , Male , Mastocytosis/pathology , Middle Aged , Night Blindness/metabolism , Night Blindness/physiopathology , Visual Acuity , Visual Fields , Vitamin A Deficiency/complications , Vitamin A Deficiency/metabolism
7.
Am J Med ; 98(4A): 60S-62S, 1995 Apr 24.
Article in English | MEDLINE | ID: mdl-7726193

ABSTRACT

Although ocular manifestations of Lyme disease have long been noted, they remain a rare feature of the disease. The spirochete invades the eye early and remains dormant, accounting for both early and late ocular manifestations. A nonspecific follicular conjunctivitis occurs in approximately 10% of patients with early Lyme disease. Keratitis occurs often within a few months of onset of disease and is characterized by nummular nonstaining opacities. Inflammatory syndromes, such as vitritis and uveitis, have been reported; in some cases, a vitreous tap is required for diagnosis. Neuro-ophthalmic manifestations include neuroretinitis, involvement of multiple cranial nerves, optic atrophy, and disc edema. Seventh nerve paresis can lead to neurotrophic keratitis. In endemic areas, Lyme disease may be responsible for approximately 25% of new-onset Bell's palsy. Criteria for establishing that eye findings can be attributed to Lyme disease include the lack of evidence of other disease, other clinical findings consistent with Lyme disease, occurrence in patients living in an endemic area, positive serology, and, in most cases, response to treatment. Management of ocular manifestations often requires intravenous therapy.


Subject(s)
Borrelia burgdorferi Group , Eye Diseases/microbiology , Lyme Disease/complications , Borrelia burgdorferi Group/isolation & purification , Humans
8.
Surv Ophthalmol ; 39(3): 169-210, 1994.
Article in English | MEDLINE | ID: mdl-7878520

ABSTRACT

Ocular myasthenia is a localized form of myasthenia clinically involving only the extraocular, levator palpebrae superioris, and/or orbicularis oculi muscles. Ocular manifestations can masquerade as a variety of ocular motility disorders, including cranial nerve and gaze palsies. A history of variable and fatiguable muscle weakness suggests this diagnosis, which may be confirmed by the edrophonium (Tensilon) test and acetylcholine receptor antibody titer. Anticholinesterases, corticosteroids and other immunosuppressive agents, and other therapeutic modalities, including thymectomy and plasmapheresis, are used in treatment. As the pathophysiology of myasthenia has been elucidated in recent years, newer treatment strategies have evolved, resulting in a much more favorable prognosis than several decades ago. This review provides historical background, pathophysiology, immuno-genetics, diagnostic testing, and treatment options for ocular myasthenia, as well as a discussion of drug-induced myasthenic syndromes.


Subject(s)
Myasthenia Gravis/physiopathology , Ocular Motility Disorders/physiopathology , Oculomotor Muscles/physiopathology , Circadian Rhythm/drug effects , Humans , Myasthenia Gravis/diagnosis , Myasthenia Gravis/immunology , Myasthenia Gravis/therapy , Ocular Motility Disorders/diagnosis , Ocular Motility Disorders/immunology , Ocular Motility Disorders/therapy
9.
Dermatol Clin ; 10(4): 763-75, 1992 Oct.
Article in English | MEDLINE | ID: mdl-1395157

ABSTRACT

Lyme disease is a multisystem disorder caused by the spirochete Borrelia burgdorferi. It is transmitted to human and animal hosts primarily by ticks of the Ixodes ricinis complex. Recognition of its characteristic skin and eye manifestations facilitates diagnosis and treatment.


Subject(s)
Borrelia burgdorferi Group , Erythema Chronicum Migrans/microbiology , Eye Infections, Bacterial/microbiology , Lyme Disease/complications , Acrodermatitis/diagnosis , Anti-Bacterial Agents/therapeutic use , Arthritis/diagnosis , Arthritis/etiology , Borrelia burgdorferi Group/isolation & purification , Conjunctivitis, Bacterial/diagnosis , Conjunctivitis, Bacterial/etiology , Erythema Chronicum Migrans/diagnosis , Eye Infections, Bacterial/diagnosis , Humans , Lyme Disease/diagnosis , Lyme Disease/drug therapy , Serologic Tests
10.
J Clin Neuroophthalmol ; 12(3): 173-7, 1992 Sep.
Article in English | MEDLINE | ID: mdl-1401163

ABSTRACT

We report a patient with bilateral "acute idiopathic blind spot enlargement" (AIBSE) in whom visual symptoms and enlarged blind spots persisted for over 6 years and preceded the development of peripapillary hyperfluorescence on fluorescein angiography. These findings confirm the prolonged course that AIBSE can sometimes take and the suggestion that this rare disorder is due to peripapillary retinal dysfunction.


Subject(s)
Optic Disk/pathology , Optic Nerve Diseases/pathology , Acute Disease , Adult , Female , Fluorescein Angiography , Humans , Hypertrophy , Retinal Diseases/pathology , Visual Acuity , Visual Fields
11.
Ophthalmology ; 97(6): 699-706, 1990 Jun.
Article in English | MEDLINE | ID: mdl-2374674

ABSTRACT

Lyme disease is a tick-borne spirochetal infection characterized by skin rash, neurologic, cardiac, and arthritic findings. The authors report six patients with Lyme disease who had neuro-ophthalmologic manifestations. One patient had meningitis with papilledema, two had optic neuritis, and one had neuroretinitis. Three patients had sixth nerve paresis, two of whom cleared quickly, whereas multiple cranial nerve palsies and subsequent optic neuropathy developed in another. Early recognition of neuro-ophthalmologic findings can help in the diagnosis and treatment of Lyme disease.


Subject(s)
Eye Diseases/etiology , Lyme Disease/complications , Optic Nerve Diseases/etiology , Abducens Nerve/physiopathology , Adolescent , Adult , Child , Female , Fundus Oculi , Humans , Magnetic Resonance Imaging , Male , Meningitis/etiology , Middle Aged , Papilledema/etiology , Retinitis/etiology , Visual Acuity , Visual Fields
12.
Am J Ophthalmol ; 109(5): 535-43, 1990 May 15.
Article in English | MEDLINE | ID: mdl-2333917

ABSTRACT

We studied eight patients who had visual field defects secondary to prolactin-secreting macroadenomas and who had improved visual function with bromocriptine treatment without surgery or radiation. We recommend bromocriptine as a primary treatment for prolactin-secreting macroadenomas. If therapy is effective, continued regular neuro-ophthalmologic, endocrine, and imaging studies are necessary, because treatment with bromocriptine must be continued indefinitely.


Subject(s)
Bromocriptine/therapeutic use , Pituitary Neoplasms/drug therapy , Prolactinoma/drug therapy , Vision, Ocular/drug effects , Adult , Aged , Female , Humans , Male , Middle Aged , Prolactin/blood , Tomography, X-Ray Computed , Visual Acuity/drug effects , Visual Fields/drug effects
13.
Br J Ophthalmol ; 73(12): 1015-8, 1989 Dec.
Article in English | MEDLINE | ID: mdl-2692700

ABSTRACT

A 68-year-old black woman who was put on D-penicillamine therapy (250-500 mg per day, total dose 15 g) for rheumatoid arthritis developed ocular myasthenia gravis. Two weeks after she discontinued D-penicillamine her signs and symptoms cleared with no other treatment. Review of previous cases and possible immunological mechanisms are discussed.


Subject(s)
Blepharoptosis/chemically induced , Diplopia/chemically induced , Myasthenia Gravis/chemically induced , Penicillamine/adverse effects , Aged , Female , Humans
14.
Ophthalmology ; 96(8): 1194-7, 1989 Aug.
Article in English | MEDLINE | ID: mdl-2477779

ABSTRACT

Lyme disease, caused by the spirochete Borrelia burgdorferi, has ophthalmic manifestations. The authors describe two cases of Lyme keratitis characterized by multiple focal, nebular opacities at varying levels of the stroma which may progress to edema, neovascularization, and scarring. Close observation, in addition to systemic antibiotic therapy, may be sufficient if the visual axis is not involved, and the patient is asymptomatic.


Subject(s)
Keratitis/etiology , Lyme Disease/complications , Adult , Child , Cornea/blood supply , Corneal Opacity/complications , Edema/complications , Female , Humans , Male , Neovascularization, Pathologic/diagnosis , Visual Acuity
15.
J Clin Neuroophthalmol ; 9(2): 98-104, 1989 Jun.
Article in English | MEDLINE | ID: mdl-2526164

ABSTRACT

A case of steroid-responsive malignant lymphoma of the optic chiasm is reported in a 58-year-old woman presenting with subacute vision loss, headaches, and decreased hearing. The exquisite sensitivity of magnetic resonance imaging (MRI) of the parasellar area and the usefulness of MR-directed stereotactic biopsy in identifying a chiasmatic lesion are shown. Primary CNS lymphomas are briefly reviewed. Ophthalmologists will be asked to evaluate patients with malignant lymphoma of the optic chiasm with increased frequency in the future, as CNS lymphomas are expected to be the most common neurological neoplasm by 1991.


Subject(s)
Cranial Nerve Neoplasms/diagnosis , Lymphoma/diagnosis , Optic Chiasm , Biopsy , Cranial Nerve Neoplasms/pathology , Cranial Nerve Neoplasms/radiotherapy , Female , Humans , Lymphoma/pathology , Magnetic Resonance Imaging , Middle Aged , Optic Chiasm/pathology , Tomography, X-Ray Computed , Visual Acuity , Visual Fields
16.
Br J Ophthalmol ; 67(6): 349-51, 1983 Jun.
Article in English | MEDLINE | ID: mdl-6849854

ABSTRACT

Basal encephaloceles are often associated with other midline anomalies such as hypertelorism, broad nasal root, cleft lip, and cleft palate. Optic disc anomalies such as pallor, dysplasia, optic pit, coLoboma, and megalopapilla have been reported to occur in patients with basal encephalocele We report a case of a child with a sphenoethmoidal encephalocele and morning glory syndrome of the optic nerve. The presence of such optic nerve anomalies with facial midline anomalies should alert the clinician to the possible presence of a basal encephalocele.


Subject(s)
Encephalocele/complications , Meningocele/complications , Optic Nerve/abnormalities , Ethmoid Bone , Humans , Infant , Macula Lutea/abnormalities , Male , Sphenoid Bone , Syndrome
17.
J Clin Neuroophthalmol ; 1(2): 153-60, 1981 Jun.
Article in English | MEDLINE | ID: mdl-6213655

ABSTRACT

Because there have been reports of ocular and/or cerebral ischemic events associated with mitral valve prolapse, we reviewed a series of patients with mitral valve prolapse to determine the frequency of amaurosis fugax. Among 59 patients, 13 (22%) had symptoms while among 75 controls, only one had amaurosis fugax. We conclude that patients with mitral valve prolapse have an increased risk of ocular and/or cerebral ischemia.


Subject(s)
Blindness/etiology , Mitral Valve Prolapse/complications , Adult , Brain Ischemia/etiology , Eye/blood supply , Female , Humans , Ischemia/etiology , Male , Middle Aged , Mitral Valve Prolapse/diagnosis , Physical Exertion
18.
Arch Ophthalmol ; 98(11): 1993-5, 1980 Nov.
Article in English | MEDLINE | ID: mdl-7436832

ABSTRACT

Four days after a 32-year-old woman was hit in the right eye with a racquetball, a paralysis of the right third nerve developed. Computerized axial tomography with metrizamide demonstrated an arachnoid cyst in the interpeduncular fossa. After the cyst was surgically decompressed, the third-nerve paralysis cleared.


Subject(s)
Arachnoid/surgery , Athletic Injuries/complications , Cysts/complications , Eye Injuries/complications , Ophthalmoplegia/etiology , Adult , Arachnoid/diagnostic imaging , Cysts/diagnostic imaging , Cysts/surgery , Female , Humans , Metrizamide , Sports , Tomography, X-Ray Computed
19.
Br J Ophthalmol ; 63(10): 699-703, 1979 Oct.
Article in English | MEDLINE | ID: mdl-508683

ABSTRACT

A 52-year-old man with mild diabetes and acute stem cell leukaemia developed an orbitofacial mucormycosis. Cultures showed the fungus to be Rhizopus oryzae. Vigorous treatment with amphotericin B and other bactericidal and bacteriostatic antibiotics for a concurrent sepsis failed to suppress the infections, and the patient died. On post-mortem examination characteristic haematoxylin-staining, broad, aseptate fungal hyphae were found in the right eye, orbit, and lung. A striking and unusual feature of this case is the presence of brightly birefringent crystals within the severely degenerated eye. These were found by histochemical staining and x-ray diffraction studies to be calcium salts of fatty acids, apparently liberated from necrotic adipose tissue of the orbit.


Subject(s)
Eye Diseases/pathology , Mucormycosis/pathology , Calcinosis/pathology , Dermatomycoses/complications , Diabetes Complications , Eye/pathology , Eye Diseases/complications , Facial Dermatoses/complications , Humans , Leukemia/complications , Male , Middle Aged , Mucormycosis/complications , Rhizopus
20.
Am J Ophthalmol ; 87(5): 682-7, 1979 May.
Article in English | MEDLINE | ID: mdl-443340

ABSTRACT

A 17-year old girl received prednisone and azathioprine for the treatment of systemic lupus erythematosus. She developed a fever and hallucinations 18 months later; cryptococcal meningitis was diagnosed. An internal ophthalmoplegia with loss of accommodation and dilation of the pupils developed together with bilateral lateral rectus palsy. Treatment with intravenous amphotericin resulted in disappearance of papilledema, muscle palsy, and internal ophthalmoplegia. We believe that the internal ophthalmoplegia was secondary to involvement of the accommodative and pupillary fibers of both third nerves at the base of the brain.


Subject(s)
Cryptococcosis/complications , Lupus Erythematosus, Systemic/complications , Ophthalmoplegia/etiology , Papilledema/etiology , Adolescent , Adult , Aged , Amphotericin B/therapeutic use , Azathioprine/therapeutic use , Child , Child, Preschool , Cryptococcosis/cerebrospinal fluid , Cryptococcosis/drug therapy , Female , Humans , Lupus Erythematosus, Systemic/drug therapy , Male , Meningitis/cerebrospinal fluid , Meningitis/complications , Meningitis/drug therapy , Middle Aged , Prednisone/therapeutic use
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