ABSTRACT
Amyotrophic Lateral Sclerosis (ALS) is a rare, devastating motor neuron disease characterized by the degeneration of upper and lower motor neurons causing muscular weakness, paralysis, and eventual death. MRI plays a supportive role in the diagnosis; its primary role is to exclude other clinical mimics. Some of the imaging features associated with ALS include hypointense signal along the motor cortices on susceptibility or T2*-weighted imaging and hyperintensity along the corticospinal tracts (CST) within the cerebral hemispheres, brainstem, and spinal cord on the T2 weighted imaging. In this report, we discuss the value of T1 hyperintensity along the CST, especially in the spinal cord.
ABSTRACT
Budd-Chiari syndrome (BCS) occurs as a result of hepatic venous outflow obstruction. In the pediatric population, the etiologies vary as compared with the adult population. Decompensation can occur faster in this set of patients. Ultrasound and Doppler represent important imaging modalities for diagnosing BCS in children. The imaging features differ depending upon the level of obstruction, acuteness of the condition, and secondary decompensation. Caudate lobe hypertrophy is a salient feature. Obstruction at the level of hepatic veins may be manifested by ostial narrowing, echogenic thrombus, and altered flow patterns in the form of turbulent flow, nonvisualization of the veins, or reversal of flow. Obstruction in the inferior vena cava may present as an echogenic web, membrane, or thrombus with turbulent flow to absent flow within depending upon the degree of luminal compromise. Collateral formation is an important distinctive feature of subacute and chronic BCS. Collaterals that develop may be of intrahepatic or extrahepatic type. Secondary signs of liver failure would be present in late stages. Understanding the clinical presentation and imaging features can help in achieving the correct diagnosis because an early diagnosis of the disease will impact patient management.
