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Biomedical subjects

R E Appen

Publications and source records attributed to R E Appen.

At least 19 recordsLinked to original sources
PubMed1991

Delayed visual loss due to trauma of the internal carotid artery.

The group of six patients in this study experienced delayed visual loss following head trauma. Visual loss occurred from 1 day to 13 years after the initial injury. All patients suffered indirect trauma to the internal carotid artery resulting in formation of either an aneurysm or pseudoaneurysm or a carotid-cavernous fistula. Review of the radiologic and clinical findings was performed in six patients. The diagnosis was established by computed tomography, magnetic resonance imaging, and angiography. All patients had follow-up clinical evaluation and imaging studies. Treatment by neurosurgical or interventional neuroradiologic procedures resulted in significant visual improvement in five patients. Different pathophysiologic mechanisms could be correlated with the delayed visual loss produced by the two types of lesions. The pathologic changes associated with the aneurysms/pseudoaneurysms included direct compression of optic nerves and/or chiasm and intracranial hematoma. A carotid-cavernous fistula caused delayed visual loss by either hematoma at the orbital apex or compression of the chiasm and/or optic nerves by saccular dilatation of the cavernous sinus. The delayed onset of decreased vision following head trauma should alert the physician to the possibility of a traumatic aneurysm/pseudoaneurysm or a carotid-cavernous fistula. Different neuro-ophthalmologic symptoms can usually be correlated with the pathologic changes demonstrated by neuroimaging procedures.

Adolescent↗
PubMed1989

Spinocerebellar ataxia, hypogonadotropic hypogonadism, and choroidal dystrophy (Boucher-Neuhäuser syndrome)

We describe two families (including one previously reported) in which cerebellar or spinocerebellar ataxia, hypogonadotropic hypogonadism, and choroidal dystrophy result from abnormal function of an autosomal recessive gene. Review of the literature adds one other family with this disorder. These three examples confirm the existence of this traid as a specific, pleiotropic, single-gene syndrome. Careful ophthalmologic evaluation of patients with ataxia and hypogonadotropic hypogonadism may identify additional cases.

Adult↗
PubMed1987

Recurrent scintillating scotoma and homonymous hemianopia due to metastatic melanoma.

Acute left homonymous hemianopia and recurrent scintillating scotoma occurred in a 43-year-old woman due to metastases from a cutaneous malignant melanoma that had been resected 5 years previously. Computed tomography initially demonstrated small, probably embolic, areas of occipital lobe infarction. Five months after the onset of her visual symptoms, massive cerebral hemorrhage occurred from a large right occipital lobe tumor that had not been present initially. Autopsy demonstrated malignant melanoma. Visual symptoms related to tumor embolization and/or cerebral hemorrhage may be the presenting features of metastatic cutaneous malignant melanoma.

Adult↗
PubMed1984

Optic atrophy and the Wyburn-Mason syndrome.

A 14-year-old girl developed impaired vision and optic atrophy of the left eye as a manifestation of an orbital and parachiasmal arteriovenous malformation. Despite the lack of a retinal arteriovenous malformation, this condition appears to be a variant of the Wyburn -Mason syndrome, representing a vascular dysgenesis of the orbit and midbrain resulting in focal arteriovenous communications without interposed capillaries.

Adolescent↗
PubMed1982

Diplopia in autoimmune thyroid disease.

Diplopia without obvious exophthalmos, caused by infiltrative endocrine ophthalmopathy, developed in 12 patients with clinical and laboratory confirmation of autoimmune thyroid disease. In eight patients, the diplopia alone prompted medical attention, which led to the diagnosis of autoimmune thyroid disease. A hypotropia secondary to restrictive tightening of the inferior rectus muscle, producing vertical diplopia, was the most common manifestation of the disorder. The ocular muscle imbalance in autoimmune thyroid disease is caused by mechanical orbital restriction of the extraocular muscle, not by an innervational defect. Evidence of orbital restriction of the eye muscles can establish the diagnosis, regardless of the patient's thyroid function.

Adult↗
PubMed1981

Ischemic oculopathy. A manifestation of carotid artery disease.

Six patients experienced ischemic oculopathy, a condition in which there is ischemia in both the anterior and posterior segments of the eye caused by occlusive carotid artery disease. The abnormalities in the anterior segment include episcleral vascular congestion, anterior chamber flare and cells, a mid-dilated, sluggish, or unreactive pupil, rubeosis iridis, and abnormal intraocular pressure. The posterior segment abnormalities include ischemic insults to the retina or optic nerve, venous-stasis retinopathy, and low ophthalmodynamometry values. Ophthalmodynamometry is particularly helpful in recognizing the pathogenesis of this disorder. Superficial temporal artery-middle cerebral artery anastomosis surgery may have particular merit for patients with ischemic oculopathy.

Aged↗
PubMed1980

Diabetic papillopathy.

A 13-year-old girl and a 20-year-old man with juvenile-onset diabetes mellitus developed transient bilateral optic disk edema with minimal impairment of optic nerve function and with minimal diabetic retinopathy. This conditions, termed diabetic papillopathy appears to be a local optic disk vasculopathy, and generally resolves without treatment. Diabetic papillopathy should not be mistaken for papilledema or for the optic disk neovascularization of diabetic retinopathy.

Adolescent↗
PubMed1980

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.

Adult↗
PubMed1980

Meningiomatous changes in the optic canan: a polytomographic study.

Eleven patients who had unilateral insidious compression of the optic nerve but exhibited no detectable abnormality on plain skull radiographs or non-tomographic views of the optic canal were studied. Paracanalicular meningioma was proved surgically in 10. Complex-motion tomography demonstrated characteristic alterations in the bone forming the optic canal in all cases. These changes consisted of canal enlargement, changes in contour, and an atypical radiographic appearance of the cortical bone, seen as (a) diffuse, spongy, non-homogeneous thickening of the canal margins; (b) multiple tiny saw-tooth-like exostoses projecting into the canal lumen; or (c) short, well-circumscribed areas of smooth bone thickening. Other techniques such as angiography and pneumoencephalography were not as helpful in the diagnosis as complex-motion tomography.

Adult↗
PubMed1979

Ophthalmodynamometry: a reappraisal.

The accuracy of ophthalmodynamometry (ODM) was retrospectively analyzed in 36 patients who had ophthalmic examinations prior to carotid arteriography. In 75% of those patients, ODM correctly predicted the presence of absence of significant stenosis (ie, 50% or greater) or occlusion of the internal carotid artery. A 30% false-negative rate and 19% false-positive rate was found in this study. The accuracy was not improved when the examiner had more experience with the test. The accuracy of ODM with staff members who regularly perform the examination was 68% as compared to the ophthalmology residents' accuracy of 76%. Causes of false-negative and positive results include subjectivity of the examination, stenosis of arteries other than the internal carotids, and development of collateral circulation as was demonstrated arteriographically in 2 of our patients.

Aged↗
PubMed1978

Meningeal carcinomatosis with blindness.

A 53-year-old man had bilateral blindness secondary to meningeal carcinomatosis from pulmonary adenocarcinoma. Histopathologic examination of the visual system showed extensive infiltration of the arachnoid of the proximal optic nerves and chiasm, with minimal invasion of the optic nerves themselves. The visual pathways within the cerebral cortex were not affected.

Adenocarcinoma↗
PubMed1978

Amaurosis and blood loss.

A 57-year-old alcoholic man sustained permanent bilateral blindness and optic atrophy as a complication of hemorrhage from peptic ulcer disease. Post-hemorrhagic visual loss occurs in middle-aged, debilitated persons a short time after repeated episodes of hemorrhage from any site.

Alcoholism↗
PubMed1978

Bilateral homonymous hemianopia.

Bilateral homonymous hemianopia occurred in 15 patients seen during a four-year period. Persons with this visual deficit had similarly shaped visual field defects on corresponding sides of the vertical midline for each eye, equal visual acuity which is generally normal, and normal pupil and fundus examinations. The most common causes of the bilateral posterior cerebral artery insufficency were arteriosclerosis (40%), uncalherniation (20%), and migraine (13%).

Adult↗
PubMed1978

Raeder's paratrigeminal syndrome.

Three middle-aged patients, 2 of whom are females, with unilateral trigeminal pain and associated isolated oculosympathetic paralysis are presented as characteristic of the benign form of Raeder's paratrigeminal syndrome. Their nonprogressive course is representative of the fact that this syndrome is not likely to be caused by aneurysms or mass lesions, and suggests that neuroradiologic contrast studies are generally not initially required in the investigation of such patients. If atypical features are present, or the pain is protracted, further investigation may be warranted.

Adult↗
PubMed1977

Anatomic considerations for computed tomography of the optic chiasm.

When the clinical distinction between lesions of the optic nerves or the chiasm is not apparent, computed tomographic scans should be performed at both 0 degrees and 25 degrees or 35 degrees to the orbital-meatal line (Reid's baseline). The former plane is preferable for the demonstration of the optic nerves or orbital structures, while the latter allows superior visualization of perichiasmatic structures.

Female↗
PubMed1977

Progressive multifocal leukoencephalopathy. A cause of visual loss.

A patient had been treated for chronic lymphocytic leukemia for five years before developing visual blurring as the manifestation of occipital lobe lesions of progressive multifocal leukoencephalopathy (PML). The disease is caused by an infection of the CNS oligodendrocytes by a papovavirus, whose replication is facilitated by an impairment of the host's cell-mediated immunologic system. The multiplying virus destroys the oligodendrocytes and causes extensive demyelination of the white matter of the brain. Antiviral agents, such as cytarabine, may be beneficial in treating patients with PML.

Cytarabine↗
PubMed1976

Ocular and cerebral involvement in familial lymphohistiocytosis.

A 5-month-old infant girl died of familial lymphohistiocytosis. The central nervous system showed widespread perivascular infiltration of the cerebral pia and arachnoid, the cerebral white matter and deep gray matter, the cerebellum, and brain stem by lymphocytes, benign appearing histiocytes, and macrophages with erythro-and lymphophagocytosis. The eyes had mild infiltration of the anterior uveal tract, moderate involvement of the inner retina, and marked infiltration of the optic nerves by identical cells.

Brain↗