PubMed Health⌕ Search

Biomedical subjects

S A Josephson

Publications and source records attributed to S A Josephson.

4 recordsLinked to original sources
PubMed2004

Evaluation of carotid stenosis using CT angiography in the initial evaluation of stroke and TIA.

BACKGROUND: Imaging of the carotid arteries is important for the evaluation of patients with ischemic stroke or TIA. CT angiography (CTA) of the head and neck is readily available and can be part of the routine imaging of stroke patients. To evaluate the accuracy of CTA, the authors compared the degree of stenosis found using CTA with digital subtraction angiography (DSA) in consecutive patients during a 3-year period. METHODS: The authors included all patients with interpretable CTA and DSA of the cervical carotid arteries from April 2000 to November 2002 at a single academic medical center. This yielded a total of 81 vessels. Stenosis on CTA of the internal carotid artery was measured in the axial plane at the point of maximum stenosis and referenced to the distal cervical internal carotid by two blinded readers. Two blinded readers measured stenosis from the DSA using the North American Symptomatic Carotid Endarterectomy Trial method. RESULTS: Using a 70% cutoff value for stenosis, CTA and DSA were in agreement in 78 of 81 (96%; 95% CI, 90 to 99%) vessels. CTA was 100% sensitive (n = 5) and 63% specific (95% CI, 25 to 88%), and the negative predictive value of a CTA demonstrating <70% stenosis was 100% (n = 73). CONCLUSIONS: In this consecutive series of patients with CT angiography of the neck and digital subtraction angiography, the authors found that CT angiography has a high sensitivity and high negative predictive value for carotid disease. CT angiography appears to be an excellent screening test for internal carotid artery stenosis, and the authors advocate its use for the initial imaging of patients with suspected stroke or TIA.

Angiography↗
PubMed2001

Autosomal dominant Kufs' disease: a cause of early onset dementia.

Kufs' disease is the rare, adult-onset form of the neuronal ceroid-lipofuscinoses (NCL). Two clinical Kufs' phenotypes have been described, one featuring generalized tonic-clonic seizures and the other characterized by dementia. Autosomal dominant inheritance of Kufs' disease has been reported for only two families. The genetic and molecular defects underlying Kufs' disease are unknown. We report a third family with apparent autosomal dominant Kufs' disease in a family of English ancestry. Ten individuals (five men, five women) have been affected over five generations. Age of onset typically is in the fourth decade of life and is heralded by seizures. Clinical and neuropsychological assessments in several affected individuals, however, confirm the presence of dementia and follow-up evaluations suggest that dementia is the primary disabling feature of the illness. Motor abnormalities also are frequent. Neuropathological examination (three cases) documents the presence of neuronal lipopigment accumulation consistent with NCL. The combination of dementia and seizures in this and two other reported families with autosomal dominant Kufs' disease suggest that this entity represents a distinctive clinicopathological entity. Dementia is prominent but is almost always associated with generalized seizures and motoric disturbances early in the disease course. Kufs' disease should be considered in the differential diagnosis of early onset, atypical dementia.

Adult↗
PubMed1997

Acid steatocrit: a simple, rapid gravimetric method to determine steatorrhea.

OBJECTIVES: The detection and evaluation of steatorrhea in a rapid, quantitative fashion are clinically needed in patients with suspected steatorrhea. Our aim was to evaluate the acid steatocrit method, on random spot stools in adults with and without steatorrhea, relative to the qualitative (microscopic) and quantitative assessments for fecal fat. METHODS: Stool samples were collected 72 h after a diet of 100 g of fat per day and randomly from 15 healthy controls, 14 patients with chronic pancreatitis, and seven patients with small bowel disease. All stools had quantitative, qualitative, and acid steatocrit analyses performed for fecal fat. RESULTS: The sensitivity and specificity for the detection of steatorrhea by the spot stool qualitative fecal fat were 78 and 70%, respectively. The spot stool acid steatocrit correlated linearly with the 72-h stool quantitative fecal fat (g/24 h), r = 0.761 and p < 0.001. The acid steatocrit on random spot stools, compared with the 72-h stool quantitative fecal fat, revealed a sensitivity of 100%, a specificity of 95%, and a positive predictive value of 90% for the detection of steatorrhea. It also estimated the quantitative fecal fat. CONCLUSIONS: The acid steatocrit can be performed accurately on random spot stools and can be used to detect the presence of steatorrhea and estimate the quantitative fecal fat. This assay can be done with readily available equipment for rapid evaluation. Use of a spot stool sample simplifies the acid steatocrit, further improving on the practicality of this test. This study also confirms the clinical usefulness of this simplified method to detect steatorrhea.

Adult↗