Biomedical subjects

Fiona Costello

Publications and source records attributed to Fiona Costello.

7 recordsLinked to original sources

PubMed2006

Treatment of Susac syndrome with gamma globulin and corticosteroids.

Susac syndrome is a rare vasculopathy characterized by visual, hearing, and cognitive dysfunction. Optimal treatment is unknown, but many patients require chemotherapy to control disease activity. We describe two patients with Susac syndrome and their response to intravenous immune globulin (IVIg) and corticosteroids. Both patients improved following acute treatment with IVIg and intravenous methylprednisolone (IVMP), and no further relapses were observed. One patient showed significant improvement in hearing and MRI lesions shortly following acute treatment. Treatment with IVIg and corticosteroids provides a therapeutic option that avoids the toxicities of chemotherapy and suggests the possible importance of pathologic antibodies in the pathogenesis of Susac syndrome.

Adrenal Cortex Hormones↗

PubMed2006

Quantifying axonal loss after optic neuritis with optical coherence tomography.

OBJECTIVE: To determine to what degree changes in retinal nerve fiber layer (RNFL) thickness after optic neuritis (ON) correlate with either visual recovery or impairment. METHODS: ON can cause visible defects within the RNFL, which can be quantified using optical coherence tomography (OCT). It may be possible to predict visual recovery by measuring RNFL loss after ON. Fifty-four patients underwent repeated evaluations with optical coherence tomography and standardized ophthalmic testing after ON. Regression analyses were used to determine the relationship between RNFL thickness and visual function. RESULTS: Thinning of the RNFL was seen in the majority of patients (74%), and it tended to occur within 3 to 6 months of ON. The average RNFL value was thinner (p<0.0001) in the affected (78 microm) compared with the unaffected eye (100 microm). Patients with incomplete visual recovery demonstrated greater RNFL loss after ON. Regression analyses demonstrated a threshold of RNFL thickness (75 microm), below which RNFL measurements predicted persistent visual dysfunction. INTERPRETATION: Determination of RNFL thickness may predict visual recovery after ON, and lower RNFL values correlate with impaired visual function. Optical coherence tomography may have a potential role as a surrogate marker for axonal integrity within the optic nerve among patients with ON.

Adolescent↗

PubMed2006

Optical coherence tomography in multiple sclerosis.

We do not have currently satisfactory clinical and anatomical correlates to gauge disability in multiple sclerosis. Structural biomarkers (such as MRI) are hindered because they cannot precisely segregate demyelination from axonal elements of tissue injury within the CNS. Axonal degeneration in multiple sclerosis is related to irreversible disability, which suggests that the confirmation of neuroprotective strategies needs highly quantifiable measures of axon loss that can be correlated with reliable measures of physiological function. The coupling of quantifiable measures of visual function with ocular imaging techniques, such as optical coherence tomography, enables us to begin to understand how structural changes in the visual system influence function in patients with multiple sclerosis. In this review, we consider the usefulness of optical imaging of the retina as a biomarker for neurodegeneration in multiple-sclerosis.

Biomarkers↗

PubMed2005

Bilateral simultaneous central retinal artery occlusions in wegener granulomatosis.

A 46-year old woman developed simultaneous central retinal artery occlusions (CRAOs) in Wegener granulomatosis (WG). She had presented six years earlier with xerostomia, skin rash, and arthralgias and received a diagnosis of Sjogren syndrome. Anti-neutrophilic cytoplasmic antibody (ANCA) was negative. Months prior to the CRAOs, she had developed hearing loss, proptosis, and scleritis that were not responsive to prednisone 50 mg/d. The CRAOs occurred while she was being treated at this dose level. ANCA was now positive. This is the 12th case of CRAO in WG and the 6th case of bilateral CRAOs reported in the English literature. It emphasizes that serious irreversible visual complications may occur even when the patient is being treated with substantial corticosteroid doses.

Antibodies, Antineutrophil Cytoplasmic↗

PubMed2003

Distinguishing ischemic stroke from the stroke-like lesions of MELAS using apparent diffusion coefficient mapping.

We report two patients with migraine, acute visual field defects and other neurological symptoms who were found to have high T(2) signal and FLAIR abnormalities on brain MRI in temporal and parieto-occipital regions. In these patients, the apparent diffusion coefficient (ADC) of their lesions was increased, distinguishing these lesions from those of ischemic stroke. Both were ultimately diagnosed with mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS). We conclude that conventional MRI when used with diffusion-weighted MR imaging may be invaluable in detecting mitochondrial-related CNS dysfunction.

Adult↗

PubMed2002

Papilledema as the presenting manifestation of spinal schwannoma.

A 63-year-old woman with headache, blurred vision, bilateral optic disc edema, and normal cranial magnetic resonance imaging scan underwent lumbar puncture that revealed an elevated opening pressure (290 mm water), a protein level of 114 mg/dl, and mild pleocytosis. Spinal magnetic resonance imaging later demonstrated a sacral tumor, which proved to be a schwannoma with sarcoid-like features. After surgical removal of the tumor, the patient's manifestations resolved. This case emphasizes that low spinal cord tumors can cause elevated intracranial pressure without causing markedly elevated cerebrospinal fluid protein or cells, or any myelopathic manifestations, perhaps by obstructing sacral cerebrospinal drainage. Comprehensive spine imaging should be a part of the evaluation of a patient with papilledema who has normal brain imaging but abnormal spinal fluid constituents.

Female↗

PubMed2002

Childhood-onset chronic inflammatory demyelinating polyradiculoneuropathy with cranial nerve involvement.

A 17-year-old male presented with chronic diplopia and generalized motor weakness. He was previously diagnosed with chronic inflammatory demyelinating polyradiculoneuropathy and acute disseminated encephalomyelitis in childhood. Cranial magnetic resonance imaging (MRI) revealed a rarely reported finding of thickening and enhancement of multiple cranial nerves. Nerve conduction studies and electromyography showed peripheral nerve demyelination with axonal involvement. There was improvement in the clinical examination, MRI, and electrophysiologic studies after combined corticosteroid and plasma exchange therapy. We review the clinical presentation, course, and response to therapy among children with chronic inflammatory demyelinating polyradiculoneuropathy, with specific emphasis on the frequency and pattern of cranial nerve involvement.

Adolescent↗