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Biomedical subjects

S L Galetta

Publications and source records attributed to S L Galetta.

At least 37 records · Page 2Linked to original sources

Treatment of Bickerstaff's brainstem encephalitis with immune globulin.

Bickerstaff's brainstem encephalitis (BBE) is an immune disorder of unknown etiology. Previous reports of successful treatment of BBE involved steroids or plasma exchange. We describe a patient with BBE who demonstrated rapid clinical recovery after treatment with intravenous immune globulin. Our findings suggest that IVIg is an alternative treatment in BBE, especially when plasma exchange is difficult or contraindicated. Our patient also demonstrated pseudoretraction and pseudoptosis during clinical recovery, an observation which supports a differential immune attack on components of the third nerve or supranuclear regions, and expands our understanding of the pathophysiology of BBE.

Adult↗

Chronic multiple paraneoplastic syndromes.

A patient presented with symptoms of limbic and brainstem encephalitis, motor and sensory neuronopathy, cerebellar dysfunction, and highly positive anti-Hu antibodies. He also harbored P/Q-type calcium channel antibodies and manifested the Lambert-Eaton myasthenic syndrome (LEMS). Small-cell lung cancer was found, and he received both antineoplastic therapy and intravenous immunoglobulin (IVIg). Remission of the malignancy was achieved. Although the anti-Hu-related manifestations improved after therapy, LEMS has persisted, leading to IVIg dependency.

Autoantibodies↗

Portable pupillography of the swinging flashlight test to detect afferent pupillary defects.

OBJECTIVE: To investigate the ability of a portable, personal computer-driven, pupillometer to record the pupillary response curve during the swinging flashlight test. Also, to determine whether these response curves can be used to identify and quantify relative asymmetry in the pupillary light reflex between eyes in healthy volunteers with simulated afferent pupil defects (APDs) and patients with optic neuropathies. DESIGN: Comparative, observational case series and instrument validation. PARTICIPANTS: Healthy volunteers with no known ocular disease and patients (n = 20) with various optic neuropathies noted to have relative APDs on examination. METHODS: Pupillary response curves of the right eye were recorded with a portable, electronic, infrared pupillometer from healthy volunteers (with and without simulated APDs) and patients with APDs while the light stimulus alternated between eyes, simulating the swinging flashlight test. Simulated APDs in healthy volunteers were created with increasingly dense neutral density filters in front of the left eye. MAIN OUTCOME MEASURES: Differences in constriction amplitude, latency, and constriction velocity of the pupillary response with right eye stimulation versus left eye stimulation in both groups of subjects. RESULTS: A significant correlation between neutral density filter strength and intereye differences was seen for all measurement parameters in volunteers with simulated APDs. Depending on the measurement parameter and stimulus intensity, simulated APDs of 0.6 log units or more could be distinguished from normal responses. Clinically graded true APDs had intereye differences similar to simulated APDs of the same density. Those with real and simulated APDs of 0.9 log units or more could be distinguished from healthy volunteers with 80% sensitivity and 92% specificity. Responses from those with real and simulated small APDs of 0.3 to 0.6 log units could not be distinguished reliably. CONCLUSIONS: Portable, personal-computer driven, electronic, infrared pupillography can record the swinging flashlight test accurately and identify large afferent pupillary defects. An affordable, portable, reliable device for identifying relative APDs would be useful in the identification and follow-up of patients with neurogenic vision loss.

Adult↗

Orbital drainage from cerebral arteriovenous malformations.

OBJECTIVE: To describe the neuro-ophthalmic findings in patients with orbital drainage from cerebral arteriovenous malformations (AVMs). METHODS: We reviewed the records of 100 consecutive adult patients with cerebral AVMs who presented to our institution during a 4-year period. All patients with orbital drainage were identified, and their neuro-ophthalmic evaluations were reviewed. RESULTS: Three patients (3%) were identified with orbital drainage from a cerebral AVM. The first patient presented with typical chiasmal syndrome (reduced visual acuity, bitemporal hemianopia, and optic atrophy). Magnetic resonance imaging demonstrated a large left temporal and parietal lobe AVM with compression of the chiasm between a large pituitary gland and a markedly enlarged carotid artery. The second patient presented with headaches and postural monocular transient visual obscurations. Examination revealed normal visual function with minimal orbital congestion and asymmetrical disc edema, which was worse in the left eye. Magnetic resonance imaging revealed a large right parietal and occipital lobe AVM without mass effect or hemorrhage and an enlarged left superior ophthalmic vein. The third patient had no visual symptoms and a normal neuro-ophthalmic examination; a right parietal lobe AVM was discovered during an examination for the cause of headaches. CONCLUSION: Orbital drainage from cerebral AVMs is rare. Manifestations may include anterior visual pathway compression, dilated conjunctival veins, orbital congestion, and asymmetrical disc swelling.

Adult↗

The representation of the horizontal meridian in the primary visual cortex.

The authors report the findings of two patients that confirm the location of the horizontal meridian in the human visual cortex. The first patient had an inferior quadrant defect with a band of horizontal meridian sparing. Magnetic resonance imaging showed a lesion concentrated along the medial striate cortex. The second patient had a homonymous horizontal defect that resulted from removal of an arteriovenous malformation located in the lateral striate cortex. The findings of these two patients demonstrate that the horizontal meridian is represented at the calcarine fissure base in the primary visual cortex.

Adult↗

4.0 Tesla magnetic resonance imaging of brainstem lesions with ocular motility deficits.

The authors studied six patients with brainstem ocular motility deficits with 4.0 Tesla (T) magnetic resonance imaging to investigate whether a higher field strength would produce superior images compared with 1.5T. In four patients whose lesions were evident on 1.5T, the increased signal-to-noise achieved with 4.0T allowed for better resolution at 1-mm slice thickness than was achieved at the standard 5-mm slice thickness with 1.5T. In the two patients with unremarkable 1.5T scan results, 4.0T also failed to demonstrate a lesion. Therefore, 4.0T imaging has superior resolution to 1.5T imaging and can provide more detailed images of lesions identified by 1.5T.

Adolescent↗

Use of a portable head mounted perimetry system to assess bedside visual fields.

AIM: This study was designed to test the ability of a portable computer driven, head mounted visual field testing system to perform automated perimetry on patients at their bedside and to compare these results with the "gold standard" for bedside examinations, confrontation visual fields. METHODS: The Kasha visual field system is a portable automated perimeter which utilises a virtual reality headset. 37 neurosurgery patients were examined at their bedside with a central 24 degree suprathreshold testing strategy after confrontation visual field testing. The patterns of visual field defects were categorised and compared with the results of confrontation testing. RESULTS: A total of 42 field examinations were completed on 37 patients, and the average testing time for both eyes was 4.8 minutes with the perimetry system. Each of the 11 fields (100%) classified with defects on confrontation testing was similarly categorised on head mounted perimetry. 26 out of 31 (84%) visual fields were normal on both confrontation and perimetry testing, while five out of the 31 fields (16%) which were full on confrontation had visual field defects identified by head mounted perimetry. CONCLUSION: The head mounted, automated perimetry system proved easily portable and convenient for examining neurosurgical patients at their bedside in the perioperative period. The device demonstrated equal sensitivity to confrontation visual field testing methods in detecting field defects and offers the advantage of standardised, quantifiable testing with graphic results for follow up examinations.

Adult↗

New low-contrast vision charts: reliability and test characteristics in patients with multiple sclerosis.

The quantitative assessment of visual function in multiple sclerosis (MS) clinical trials has been limited to Snellen visual acuity. The purpose of this study was to examine the inter-rater reliability and test characteristics of a new visual outcome measure, the Low-Contrast Sloan Letter Charts, in patients with MS and visually-asymptomatic volunteers. Contrast letter acuity scores (letter scores) were measured at each of four contrast levels (100, 5, 1.25 and 0.6%) by two independent raters. Inter-rater agreement was described with the intraclass correlation coefficient (ICC) and comparison of mean scores. Excellent inter-rater agreement (ICC=0.86 - 0.95) was demonstrated at each contrast level among MS patients (n=100) and visually-asymptomatic volunteers (n=33). Average letter scores at the lowest contrast level (0.6%) were highly variable in the MS group, even among patients with visual acuities of 20/20 or better, and among those who required no assistance for ambulation. Low-Contrast Sloan Letter Chart testing is a highly reliable method of visual assessment, and provides information on an aspect of neurologic impairment in MS which is not captured by Snellen visual acuity or ambulation status. This new method demonstrates excellent potential as a visual function outcome measure for future MS clinical trials.

Adolescent↗

Self-reported visual dysfunction in multiple sclerosis: results from the 25-Item National Eye Institute Visual Function Questionnaire (VFQ-25).

Visual impairment is one of the most common clinical manifestations of Multiple Sclerosis (MS), and is strongly related to overall health-related quality of life (HRQOL) in MS and other disorders. However, the assessment of vision-specific HRQOL in patients with MS has been limited. The purpose of this study was to examine self-reported visual dysfunction in a clinically heterogeneous MS cohort using the 25-Item National Eye Institute Visual Function Questionnaire (VFQ-25). The VFQ-25 was administered by telephone interview to a subset of participants in a follow-up study to a phase III trial of interferon beta-1a for relapsing-remitting MS. Mean VFQ-25 composite scores and selected sub-scale scores were significantly lower (worse) among patients in our MS cohort (n=35) compared with a published reference group of patients with no history of chronic eye disease (n= 118). These differences were observed despite a relatively younger age and tighter distribution of binocular visual acuities in the MS cohort Patients with MS in this study thus demonstrated a greater degree of self-reported visual dysfunction, as measured by the VFQ-25, compared with an eye disease-free reference group. The VFQ-25 is a potentially useful measure of vision-specific HRQOL in patients with MS.

Adult↗

Sixth nerve palsy as a presenting sign of intracranial plasmacytoma and multiple myeloma.

Multiple myeloma and plasmacytoma are rare causes of mass lesions at the skull base and cavernous sinus. Sixth nerve palsy, in isolation or in combination with other cranial neuropathies, may occur rarely as the initial presenting feature of multiple myeloma. We report the neuro-ophthalmologic, radiologic, and pathologic findings for two patients who developed sixth nerve palsies as an initial manifestation of intracranial plasmacytoma and multiple myeloma. One patient presented with an isolated sixth nerve palsy in the setting of multiple vasculopathic risk factors. Treatable skull base lesions, including plasmacytoma and multiple myeloma, must be considered in patients with sixth nerve palsies, especially among those who demonstrate a progressive course or multiple cranial neuropathies.

Abducens Nerve Diseases↗

Evidence for preganglionic pupillary involvement in superficial siderosis.

A 36-year-old man presented with spinal myoclonus, ataxia, hearing loss, and unilateral pupillary dilation. MRI demonstrated hemosiderin deposition along the superficial surfaces of the brain, brainstem, cerebellum, and spine. The pupillary changes were localized to the preganglionic oculomotor nerve. In contrast to vasculopathic oculomotor nerve palsies, superficial siderosis may cause selective involvement of the superficially located pupillary fibers.

Adult↗

Isolated trochlear nerve palsy in patients with multiple sclerosis.

The authors describe five patients with trochlear nerve palsy and MS to characterize this rare association. In two patients, trochlear nerve palsy was the initial clinical manifestation of MS. In the other three patients, this sign occurred after previous neurologic events. MRI did not identify a lesion of the fourth nerve nucleus or fascicle. Ophthalmoplegia resolved within 2 months in four of the five patients. A reason this association is rare is that the fascicular course of the trochlear nerve is exposed to little myelin.

Adult↗

Neuro-ophthalmologic manifestations of a paraneoplastic syndrome and testicular carcinoma.

The authors report two patients with testicular cancer who exhibited supranuclear gaze disorders as a manifestation of a paraneoplastic brainstem encephalomyelitis. In the first patient, neuro-ophthalmic dysfunction was accompanied by a prominent limbic encephalitis whereas in the second patient, an unusual, mixed pendular and jerk nystagmus was manifested. Neuroimaging revealed an enhancing hypothalamic mass in the first patient and was negative in the second. Blood from both patients contained an antibody previously reported in a patient with limbic encephalitis and testicular cancer.

Adult↗

Idiopathic intracranial hypertension: relation of age and obesity in children.

The relation between obesity and age in children with idiopathic intracranial hypertension (pseudotumor cerebri) has remained uncertain. The authors reviewed the records of 45 consecutive children with newly diagnosed idiopathic intracranial hypertension seen at two medical centers. Forty-three percent of patients aged 3 to 11 years were obese, whereas 81% of those in the 12- to 14-year age group and 91% of those in the 15- to 17-year age group met criteria for obesity (p = 0.01). Younger children with idiopathic intracranial hypertension are less likely to be obese than are older children or adults.

Adolescent↗