Biomedical subjects
L B Kline
Publications and source records attributed to L B Kline.
Dominant optic atrophy. The clinical profile.
We examined 24 individuals in four family pedigrees with dominantly inherited optic atrophy (DOA); 12 patients met the criteria for diagnosis of DOA and two were suspect. Our data indicate that (1) insidious onset usually occurred in childhood, but subjective visual symptoms may evolve in adulthood; (2) visual function was minimally (20/25) to moderately (20/400) abnormal, could be strikingly asymmetric in an individual (eg, 20/30 in the right eye and 20/200 in the left eye), and showed considerable intrafamilial and interfamilial variation; (3) visual field defects consisted of central and centrocecal scotomas, but no peripheral isopter abnormalities were found; (4) color-vision screening with Hardy-Rand-Rittler plates revealed dyschromotopsias, but only Farnsworth-Munsell 100-hue examination disclosed the typical tritan defects; (5) pattern-reversal visual-evoked responses were characterized by diminished amplitudes and prolonged latencies, consistent with neural conduction defects; (6) disc pallor was limited to the temporal segment in all cases, and 16 of 24 eyes showed focal temporal excavation, which is probably pathognomonic of DOA.
Oculomotor abnormalities in Friedreich's ataxia.
A clinical neuro-ophthalmological and electro-oculographic study was made on fourteen patients with Friedreich's ataxia. None had evidence of optic nerve dysfunction. No patient complained of oscillopsia although all had ocular motor deficits of varying degrees, which appeared to be related to the severity of the general manifestations of the disease. The defects comprised square wave jerks, jerky pursuit with inability to maintain eccentric gaze resulting in gaze paretic nystagmus and rebound nystagmus. There was failure to suppress by fixation the vestibulo-ocular reflex. The slow phase velocity of caloric nystagmus was always of reduced velocity. There was inability to augment the slow phase velocity of optokinetic nystagmus with increasing stimulus velocity. Abnormalities of the saccadic system were manifest particularly as hypermetria. These signs in combination are suggestive of disease involving the cerebellar flocculus and vermis or their brain stem connections. No abnormalities were found in 17 parents or siblings.
Traumatic central retinal vein occlusion.
A patient with the clinical picture of thrombosis of the central retinal vein following relatively minor head trauma is discussed. Only 2 reports in the literature have documented a similar event. It is suggested that the condition may have arisen as a result of an abnormality of the central retinal venous outflow system since such an abnormal pattern was discovered on orbital venography.
Herpes zoster conjunctival ulceration.
A 70 year old man acquired a herps zoster infection of the ophthalmic division of his right trigeminal nerve. During the course of the illness he developed the rare complication of a cojunctival ulcer. It is suggested that the ulceration may be the result of an ischemic ischemic vasculitis.
Uveal melanoma presenting as post-traumatic choroidal hemorrhage and panophthalmitis.
A 63-year-old Caucasian man presented with loss of vision in one eye. The loss of vision was considered to be due to a post-traumatic intraocular hemorrhage. He developed panophthalmitis 3 months later and necrosis in a malignant melanoma was diagnosed. The diagnosis of melanoma in patients with blind eyes and opaque ocular media is discussed.
Monocular elevator paresis, Argyll Robertson pupils and sarcoidosis.
A patient with sarcoidosis involving the central nervous system is described who developed monocular paralysis of elevation and Argyll Robertson pupils. A schema for the control system for vertical eye movements is presented as well as an explanation for monocular elevator palsy. The postulated lesion for monocular elevator palsy could well produce at the same time Argyll Robertson pupils and the cause of this papillary abnormality is discussed.
Visual disturbances of migraine.
Migraine, a clinical syndrome of unknown etiology, is a common cause of a variety of visual disturbances. This review describes the visual alterations associated with migraine syndromes of particular interest to the ophthalmologist; acephalgic, ocular, and ophthalmoplegic. Several current theories of migraine pathophysiology are discussed. Migrainous episodes are common and must be differentiated from neurologic dysfunction due to ischemia, inflammation, seizure, and compression. The differentiating characteristics of these conditions as well as a diagnostic algorithm are presented.
The Tolosa-Hunt syndrome.
Painful ophthalmoplegia due to idiopathic granulomatous inflammation of the cavernous sinus/superior orbital fissure has been termed Tolosa-Hunt syndrome (THS). This report is based upon 146 patients with this syndrome published in the English literature. The clinical profile, natural history, diagnostic evaluation, pathologic findings, differential diagnosis, and therapy of THS are presented. Careful initial patient examination and scrupulous follow-up evaluation are essential, as THS is categorically a diagnosis of exclusion. While the etiology of THS is unknown, current pathogenetic theories are reviewed.
Computed tomography in the evaluation of the optic chiasm.
Computed tomography (CT) is the procedure of choice for initial evaluation of patients suspected of having chiasmal or perichiasmal lesions. Over 1000 patients were examined with high resolution CT, using axial and coronal scans, contrast enhancement, multiplanar reformations, and, when indicated, metrizamide CT cisternography. The normal anatomic appearance of the chiasm and neighboring structures are: (1) symmetric or asymmetric enlargement of the chiasm; (2) areas of abnormal density, without or with contrast enhancement, in the chiasm or perichiasmal region; (3) alteration of the suprasellar cistern; and (4) distortion of the anterior-inferior third ventricle.
Magnetic resonance imaging of the optic chiasm.
Magnetic resonance imaging (MRI) has become an important diagnostic modality for the evaluation of patients with chiasmal or perichiasmal disease. In this review, normal chiasmal anatomy and various pathologic conditions will be illustrated to demonstrate the advantages of MRI over computed tomography (CT). MRI is the neuroimaging method of choice in chiasmal evaluation, but sometimes it may be complemented by CT scanning.
Visual loss and a suprasellar mass complicated by pregnancy.
A 43-year-old woman who was 12 weeks pregnant presented with a one-month history of visual loss in the right eye. Neuroimaging studies revealed multiple intracranial aneurysms, one of which compressed the right optic nerve. Her pregnancy raised many issues concerning her management. Evidence of aneurysm enlargement and the increased risk of aneurysm rupture as gestation progressed led to early, successful surgical intervention.
Big muscles and big nerves.
For 11 years, a 50-year-old woman with euthyroid Graves' disease experienced intermittent exacerbations of her orbitopathy associated with a decline in visual acuity. On each occasion, treatment with systemic corticosteroids led to prompt recovery of vision. Upon referral for consideration for orbital decompressive surgery, computed tomography and magnetic resonance imaging scanning detected bilateral optic-nerve sheath meningiomas, as well as typical findings of Graves' disease. Orbital radiation therapy led to stabilization of visual function and orbital findings, eliminating the need for systemic steroids. To our knowledge, this is the first reported case of Graves' disease associated with bilateral optic nerve sheath meningiomas.
Metastatic disease to the cavernous sinus: clinical syndrome and CT diagnosis.
We analyzed the clinical constellation of signs and symptoms and the radiographic studies of 17 patients with histologic verification of cavernous sinus metastases. Although most patients presented with acute, unilateral, painful ophthalmoplegia, and with a rapidly progressive course, the clinical diagnosis of metastatic disease was often delayed. This was probably due to the fact that, in the majority of patients, cavernous sinus symptoms were either the first expression of an unknown malignancy or the first manifestation of metastatic disease in those with a known primary. Computed tomography was found to be an indispensable diagnostic aid. In 16 of the 17 patients CT established the presence of an enhancing mass in the cavernous sinus sometimes associated with bone erosion. Thin section, contrast enhanced high resolution CT in axial and coronal projections represents the imaging procedure of choice for metastatic disease to the cavernous sinus.
Ophthalmoplegia and ptosis in congenital fiber type disproportion.
Bilateral ophthalmoplegia and ptosis is reported for the first time in a patient with a rare neuromuscular disorder, congenital fiber type disproportion (CFTD). The importance of limb muscle biopsy in the diagnostic evaluation is emphasized. A summary is presented of other congenital neuromuscular diseases which may have associated ophthalmoplegia.
Pitfalls in computed tomographic evaluation of the cavernous sinus.
Computed tomography (CT) is the diagnostic procedure of choice in cavernous sinus evaluation. Two patients are reported with painful ophthalmoplegia due to metastatic carcinoma to the cavernous sinus in which initial CT scanning was considered to be normal. These cases illustrate the necessity of thin tomographic sections (5 mm or less), multiple projections (axial, coronal) and large doses of intravenous contrast medium (bolus and drip infusion). Careful technique is essential in order to thoroughly evaluate the parasellar region and avoid a false-negative CT examination.
Department of Ophthalmology, University of Alabama, School of Medicine, Birmingham.
Evaluation of over 255 patients with sellar and parasellar lesions allowed study of the computed tomographic (CT) appearance of the cavernous sinus. Optimal visualization of the cavernous sinus was obtained with continuous contrast enhancement of both axial and coronal tomograms. The coronal projection provided imaging of cranial nerves with the cavernous sinus. The intracavernous carotid artery was seen only if it were calcified or encased by tumor extending into or invading the sinus. CT criteria suggesting an abnormal cavernous sinus are: (1) asymmetry of size, (2) asymmetry of shape, particularly the lateral wall, and (3) focal areas of abnormal density within the sinus. The neurovascular anatomy of the cavernous sinus is reviewed and correlated with CT findings. Discrepancies between anatomic and CT appearance are discussed.