Faculty Bibliography

Revesz syndrome is a variant of dyskeratosis congenita characterized by aplastic anemia, retinopathy, and central nervous system abnormalities. We describe a 3-year-old boy in whom the spectrum of neuroimaging findings, including intracranial calcifications, cerebellar hypoplasia and unusual brain lesions were found by biopsy to be gliosis despite their enhancement and progression. In patients with dyskeratosis-related syndromes, non-neoplastic parenchymal brain lesions occur and gliosis should be considered in the differential diagnosis for progressive enhancing brain lesions. Should this finding be confirmed consistently in additional cases, brain biopsy could potentially be avoided.

OBJECTIVE AND IMPORTANCE/OBJECTIVE:Locked-in syndrome is a state of preserved consciousness in the setting of quadriplegia, anarthria, and usually also includes lateral gaze palsy. It is most commonly associated with upper brainstem infarction variably sparing the third cranial nerve nucleus. There are likely many etiologies that contribute to this clinical syndrome. These are incompletely understood, and the syndrome remains a rare but devastating complication that can occur after neurosurgical and neurovascular interventions. Advanced magnetic resonance imaging techniques such as perfusion and diffusion tensor imaging may help to elucidate the mechanism behind locked-in syndrome. To the authors' knowledge, there are no reports in the literature of perfusion and diffusion tensor findings in patients with this syndrome. A postprocedural case of locked-in syndrome is described with abnormalities on perfusion and diffusion tensor imaging in the absence of any changes in conventional magnetic resonance imaging. CLINICAL PRESENTATION/METHODS:A 57-year-old man who presented with acute onset headache, ataxia, and other nonspecific symptoms was found on imaging to have a giant fusiform basilar artery aneurysm. INTERVENTION/METHODS:A saphenous vein graft bypass between the proximal right external carotid artery and P2 segment of the right posterior cerebral artery followed immediately by endovascular embolization of the aneurysm sac and distal left vertebral artery was performed. CONCLUSION/CONCLUSIONS:Postprocedural angiography demonstrated patency of the bypass graft, and diffusion weighted imaging showed no evidence for acute brainstem infarction. Nevertheless, despite technically successful procedures and the absence of abnormalities on conventional magnetic resonance imaging, the patient developed quadriplegia and anarthria and remained in a locked-in state until he expired. Abnormalities were, however, seen on both perfusion and diffusion tensor imaging, where hypoperfusion, increased mean diffusivity, and decreased fractional anisotropy were observed in the ventral brainstem. The findings suggested a disruption of pontine white matter tracts. Advanced imaging techniques may allow us to image important microstructural changes that were previously not discernable and assist in the evaluation of patients with complex neurological sequelae such as locked-in syndrome.

BACKGROUND AND PURPOSE: Patients with nonlesional temporal lobe epilepsy have long been known to have abnormalities of memory. Recently, these patients have been shown to have increased diffusivity in the hippocampus. We hypothesized that in these patients, a negative correlation would exist between diffusivity measures of the mesial temporal lobe and performance on neuropsychological tests. METHODS: Twenty presurgical patients with temporal lobe epilepsy and 20 age- and sex-matched healthy controls underwent MR imaging of the brain. Apparent diffusion coefficient region of interest measures were taken in both hippocampi and parahippocampal gyri by 2 independent observers. Mean whole brain diffusivity was calculated. All patients completed neuropsychological testing. Electroencephalogram and pathology results were collected. Patients and controls were compared with respect to each apparent diffusion coefficient measure. In patients, apparent diffusion coefficients ipsilateral and contralateral to the seizure focus were compared. Associations were assessed between diffusivity measures and neuropsychological scores. RESULTS: Eleven patients had right-sided seizure foci and 9 had left-sided seizure foci. Patients demonstrated higher apparent diffusion coefficient values than controls over the whole brain, in the hippocampi, and in the parahippocampal gyri (P < .05). Patients demonstrated higher apparent diffusion coefficient within the ipsilateral hippocampus (1.19 +/- 0.22 x 10(-3) s/mm2) and parahippocampal gyrus (1.02 +/- 0.12 x 10(-3) s/mm2) compared with the contralateral side (1.02 +/- 0.16 x 10(-3) s/mm2 and 0.96 +/- 0.09 x 10(-3) s/mm2, respectively) (P < .05). Negative correlations were seen between hippocampal apparent diffusion coefficients and multiple memory tests (P < .05). CONCLUSION: Quantitative diffusion measurements in the hippocampus correlate with memory dysfunction in patients with temporal lobe epilepsy

OBJECTIVE: Our purpose was to determine the clinical significance of polyps missed on CT colonography using histologic analysis and the natural history of colorectal polyps and to propose guidelines for follow-up colon surveillance based on CT colonographic findings. SUBJECTS AND METHODS. One hundred eighty-six men (age range, 40-87 years; mean, 62.3 years) underwent CT colonography immediately before conventional colonoscopy. All polyps detected on CT colonography were measured and imaged, and their segmental location was documented. All polyps detected on colonoscopy were measured, photographed, biopsied, and histologically analyzed. Results of CT colonography and conventional colonoscopy were compared with the final pathology reports. Conventional colonoscopy was used as the gold standard unless CT colonography showed a lesion measuring 10 mm or more that was not detected on conventional colonoscopy and had characteristics of a polyp. In these cases, follow-up conventional colonoscopy was offered. RESULTS: One hundred ninety-one polyps were detected on conventional colonoscopy. CT colonography prospectively detected 53 polyps. Histologic analysis of the polyps not detected on CT colonography showed that of those 5 mm or smaller, 58.1% were not adenomas, and of those measuring 6-9 mm, 42.8% were not adenomas. Both missed polyps at CT colonography of 10 mm or more were adenomas. Of the 22 polyps measuring 10 mm or more, three were not detected on conventional colonoscopy. Of these three, CT colonography showed a lesion having characteristics of a polyp, follow-up endoscopy confirmed the presence of the lesion, and histologic analysis showed a villous adenoma, a tubulovillous adenoma, and a tubular adenoma. CONCLUSION: If CT colonography shows no abnormality, follow-up screening in 5 years is recommended. If CT colonography detects a lesion smaller than 5 mm, follow-up imaging in 3-5 years is recommended. If CT colonography detects a lesion measuring 6 mm or more, endoscopy and polypectomy should be offered unless contraindicated

PURPOSE: To compare thin-section multi-detector row computed tomographic (CT) colonography with conventional colonoscopy in the evaluation of colorectal polyps and cancer in asymptomatic average-risk patients. MATERIALS AND METHODS: Sixty-eight asymptomatic men (age > 50 years) scheduled to undergo screening colonoscopy were enrolled in this study. CT colonography was followed by conventional colonoscopy, performed on the same day. Supine and prone CT colonography were performed after colonic insufflation with room air. A gastroenterologist measured all polyps, which were categorized as 1-5, 6-9, or over 10 mm. Biopsy and histologic evaluation were performed of all polyps. CT colonography and colonoscopy results were compared for location, size, and morphology of detected lesions. Point estimates and 95% CIs were provided for specificity and sensitivity of CT by using results at conventional colonoscopy as the reference standard. RESULTS: At colonoscopy, 98 polyps were identified in 39 patients; 21 (21.4%) of 98 were detected at CT colonography. Sensitivity was 11.5% (nine of 78) for polyps 1-5 mm, 52.9% (nine of 17) for polyps 6-9 mm, and 100% (three of three) for polyps over 10 mm. Results at colonoscopy were normal in 29 (42.6%) of 68 patients; at CT colonography, results were correctly identified as normal in 26 of these 29 patients. In one of these patients, a lesion larger than 10 mm was detected at CT colonography. The per-patient specificity of CT was 89.7% (26 of 29; 95% CI: 72.7%, 97.8%). The mean time for CT image interpretation was 9 minutes. CONCLUSION: In patients at average risk for colorectal cancer, CT colonography is a sensitive and specific screening test for detecting polyps 10 mm or larger; the sensitivity for detecting smaller polyps is decreased. Examination findings can be interpreted in a clinically feasible amount of time

PURPOSE: To evaluate if differences exist in the interpretation of thin- and thick-section reconstructions at computed tomographic (CT) colonography. MATERIALS AND METHODS: Twenty-five patients underwent multi-detector row CT colonography prior to colonoscopy. CT images were reconstructed with two methods: 1.25-mm sections reconstructed every 1 mm (thin) and 5-mm sections reconstructed every 2 mm (thick). Two independent readers interpreted thin sections, then waited a minimum of 15 days before interpreting thick sections. With colonoscopy as the reference standard, comparisons were made between interpretation of thin and thick sections, including sensitivity, specificity, and number of false-positive observations. Interpretation times were recorded, and comparisons were made by using repeated measures analysis of variance. For all tests, P <.05 indicated a statistically significant difference. RESULTS: At colonoscopy, 10 patients had 12 polyps (</=5 mm, n = 7; 6-9 mm, n = 2; >/=10 mm, n = 3). Sensitivity for polyp detection was statistically indistinguishable for thin and thick sections. Reader 1 had three false-positive findings with thin sections and six with thick sections. Reader 2 had six false-positive findings with thin sections and 11 with thick sections. For both readers, the number of false-positive findings was significantly lower for thin sections than for thick sections (P =.035). Specificity was 93.3% with thin sections and 80.0% with thick sections for reader 1 and 80.0% with thin sections and 73.3% with thick sections for reader 2. Mean interpretation time for reader 1 was significantly longer with thin sections (P <.001). Mean interpretation time for reader 2 was 13.0 minutes for both thin and thick sections. CONCLUSION: Specificity improved for both readers with thin sections, with no difference in sensitivity.

Numerous filling defects may be detected in the colon during interpretation of data sets obtained with computed tomographic (CT) colonography. A series of 230 patients were evaluated with thin-section multidetector row CT colonography immediately before conventional colonoscopy. In all cases, the interpreting radiologist and gastroenterologist reviewed the imaging findings as well as the results of histologic analysis of biopsy specimens to determine the causes of filling defects. In many cases, the cause of a filling defect can be confidently determined at CT colonography by using combinations of two- and three-dimensional images. However, lesions will occasionally be indeterminate because of overlapping features and will require further evaluation with endoscopy. With knowledge of the morphologic and attenuation characteristics of the various filling defects in the colon, one should be able to differentiate those filling defects detected at CT colonography that require no further evaluation from those that require endoscopic interrogation