Lhermitte-Duclos disease (LDD) is a rare cerebellar lesion characterized by a hamartomatous lesion of the cerebellum. Mainly diagnosed by MRI, the clinical presentation is usually made of neurological symptoms. Modern neuroimaging techniques such as MRI have led to accurate diagnosis of this disease in both its preand post-operative periods. We present the case of a 68-year-old male with a past medical history of cardiac stenting and coronary artery disease who originally presented to the emergency department as a transfer for evaluation of possible obstructing hydrocephalus and left posterior inferior cerebellar artery (PICA) infarct. Based on the clinical presentation and imaging, the favored diagnosis of his left cerebellar abnormality was LDD rather than an unusual acute/subacute infarct or a metastatic lesion. The rapid progression of symptoms with rapidly progressive cytotoxic edema on serial CTs helped exclude LDD, which is nearly always more of a chronic process. The classic neuroimaging findings and clinical presentation of LDD are also discussed.
Introduction:
We previously demonstrated that the quantitative volumetric assessment of iodinated contrast extravasation (ICE) present on post-intervention imaging was closely associated with the likelihood of an acute ischemic stroke patient having PH-1 or PH-2, and thus ICE may serve as a useful CT biomarker to assess risk of reperfusion injury (hemorrhagic conversion (HC) and blood brain barrier (BBB) disruption). Here we used receiver operator curve (ROC) analysis to compare the efficacy of ICE, infarct volume, and 24hr NIHSS change as a predictor of discharge mRS and HC post-reperfusion therapy.
Method:
Data on ischemic stroke patients treated with reperfusion therapy were obtained from our Institutional Review Board approved database from January 2017 to November 2019 that had evaluable images within 24 hours of admission. Ischemic volume (IV) was measured on diffusion-weighted imaging. ICE was measured on CT head. A freehand 3D region of interest tool on the Visage Imaging PACS System was used to measure volumes. Susceptibility weighted MRI sequences were used to grade HC. Data analysis was conducted with regression modeling and ROC analysis.
Results:
Of the 82 patients, median age was 73 (interquartile range (IQR) 61- 77, 49% were women, admission NIHSS was 12 (IQR 7 - 21), 24hr NIHSS change was 4 (IQR 0 -13), IV was 50.6 +/- 7.1 mL, 48% were treated with thrombectomy, 7% had PH-1 or PH-2 identified on MRI, median systolic blood pressure was 154 (IQR 137-175), 56% were MCA territory strokes, and 37% had a discharge mRS of 0-2. ICE volume was 2.6 +/- 1.0 mL. ICE increased the likelihood of PH-1 or PH-2 HC (odds ratio (OR) 14.34, 95% confidence interval (CI) 5.74 - 22.94) and decreased the likelihood of discharge with mRS of 0-2, OR of 0.09 (CI 0.008-0.972). IV was a better predictor of 0-2 mRS (ROC area under the curve (AUC) 0.832) than ICE (AUC 0.640) and 24hr NIHSS change (AUC 0.557), but ICE was a better predictor of PH-1 or PH-2 (AUC 0.942) than IV (AUC 0.667) and 24hr NIHSS change (AUC 0.447).
Conclusion:
ICE may predict reperfusion injury and functional outcome, but it is a better predictor of hemorrhagic conversion in patients treated with reperfusion therapy.
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