SUMMARYA 62-year-old man presented with a right-sided hemichorea-hemiballismus secondary to underlying nonketotic hyperglycaemia. This condition is recognised to have a unique finding of unilateral basal ganglia lesion, which is hyperdense on CT and hyperintense on T1-weighted MRI. The clinical course of this condition is benign and has a good prognosis with early correction of the hyperglycaemia. BACKGROUND

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A 70-year-old male with a nine-year history of chronic hepatitis B infection and liver cirrhosis was admitted to the Kuala Lumpur General Hospital in Kuala Lumpur, Malaysia, in November 2012 with chills and jaundice. A physical examination revealed a distended abdomen without tenderness or masses. There were no other signs of chronic liver disease and the patient was haemodynamically stable. Liver function tests revealed elevated total bilirubin (172.0 μmol/L), alkaline phosphatase (163.0 U/L) and gamma-glutamyl transferase (134.0 U/L) levels. Alanine aminotransferase, white cell count and C-reactive protein levels were within normal limits. However, the patient's serum α-fetoprotein levels were elevated (919.3 ng/mL).An ultrasound examination of the abdomen showed a cirrhotic liver with fusiform dilatation of the right portal vein. Contrast-enhanced abdominal computed tomography (CT) confirmed the presence of a fusiform aneurysm of the distal right portal vein [ Figure 1]. The aneurysm measured 2.2 cm in diameter and the remainder of the right portal vein was also diffusely dilated. The main portal vein and its left branch, as well as the hepatic veins, were not dilated. These veins were patent with no filling defects to suggest thrombosis. The spleen was mildly enlarged (14.5 cm) and there were multiple varices in the perigastric region and splenic hilum. An illdefined mass in segment V of the liver was observed which demonstrated heterogeneous enhancement in the arterial phase and relative contrast washout in the portal venous phase. The greatest diameter of the enhancing component of the lesion in the arterial phase was 4.0 cm. There was extrinsic compression of the mass on the adjacent right intrahepatic duct resulting in dilatation of the proximal biliary system. A Figure 1A & B: Selected axial computed tomography images showing a concurrent portal venous aneurysm (asterisks) and a mass in segment V of the liver, most likely a hepatocellular carcinoma, in a 70-year-old male. The mass (white arrows) demonstrated (A) typical heterogeneous enhancement in the arterial phase and (B) washout in the portal venous phase. Additionally, it caused dilatation of the proximal intrahepatic biliary ducts (black arrows).interesting medical image

Background. Neuropsychiatric lupus (NPSLE) serves as a marker of severe disease in children with juvenile onset systemic lupus erythematosus (JSLE). This study aims to characterise the clinical and imaging features at diagnosis; and outcomes after 12 months in Malaysian children with NPSLE. Methods. A retrospective study of all NPSLE patients seen at the Pediatric Rheumatology Unit, Selayang Hospital from January 2004 to May 2017. Results. Twenty-eight (19.8%) of 141 JSLE patients had NPSLE with a median presenting age of 10 years (IQR 9 - 12), median follow-up of 7 years (IQR 4 - 11) and female: male ratio of 3.7:1. Twenty-three patients had single episodes of NPSLE and five patients had two distinct episodes each. The mean disease activity score (SLEDAI- 2K) was 24.9±11.8 at presentation with 81.8% having high disease activity (score > 12). Majority (60.6%) present with NPSLE within the first year of SLE diagnosis whilst the remainder occurred at a median of five years (IQR 3-7) post-SLE diagnosis. Majority (75.8%) had central nervous system (CNS) involvement commonly presenting with seizures, delirium and visual complaints whilst 24.2% had peripheral nervous system (PNS) involvement. Frequent accompanying features included hypocomplementemia, acute cutaneous lupus and lupus nephritis. Autoantibodies were common; ANA (100%), anti-dsDNA (78.8%) anti-RNP (39.4%) and anti-Sm (39.4%). Abnormalities were seen in 85.7% of the magnetic resonance imaging (MRI) studies performed, predominantly supratentorial white matter hyperintensities on T2 images whilst cerebrospinal fluid examination was normal in the majority. All patients with CNS involvement received corticosteroids with immunosuppressive therapy: Cyclophosphamide (20), Rituximab (2). Treatment for PNS involvement included corticosteroids with Azathioprine (6) or Mycophenolate mofetil (2). At 12 months post-NPSLE, majority (85.7%) recovered without any neurological sequelae. Conclusions. Juvenile-onset NPSLE presents with a myriad of clinical features. It is associated with high disease activity and non-specific MRI features. With early diagnosis and treatment, the majority had good prognosis.