CASE REPORT Hepatocerebral degeneration a case report BJSher FCRad(D)SA I C Duncan FFRad(D)SA Sunninghi/l Hospital Sandton Abstract Acquired hepatocerebral degenera- tion is a subtype of chronic recurrent hepatic encephalopathy and is charac- terised by movement disorders and increased signal intensity in the basal ganglia on Tl-weighted MR! images. Case report A 49-year-old female patient was admitted to a local hospital casualty department after having had a grand- mal seizure. Clinically she was found to have hepatomegaly, anaemia and stigmata of chronic alcohol abuse. Biochemical analysis showed deranged liver enzymes and haemato- logical analysis showed anaemia but a normal clotting profile. Radiological investigations includ- ed unenhanced MR! and CT scans of the brain, and an abdominal ultra- sound. On the MR! scan the Tl-weighted scans showed high signal intensity in the lenticular nuclei bilaterally and Fig. 1. Axial unenhanced T1 MR scan showing the increased signal intensity within the lenticular nuclei in a symmetrical distribution. Fig. 2. Sagittal unenhanced T1 MR scan showing increased signal intensity within the mesen- cephalon with sparing of the red nuclei. symmetrically, primarily within the globus pallidus (Fig. I), as well as in the midbrain with sparing of the red nuclei (Fig. 2). T2-weighted scans showed subtle hypointensity in the same areas (Fig. 3). An unenhanced 34 SA JOURNAL OF RADIOLOGY • September 2002 Rg. 3. Axial T2 MR scan at the same level as Fig 1. showing the subtle signal hypointensity with- In the same areas of the lentiform nuclei. Fig. 4. Axial unenhanced CT scan shows no cal- cification or other visible changes within the basal ganglia. CT scan of the brain revealed no basal ganglia calcification (Fig.4). Ultrasound examination of the abdomen showed a diffuse increase in echogenicity of the liver parenchyma indicating fatty infiltration and mild splenomegaly. There was no evidence of portal hypertension. The patient had no seizures while CASE REPORT Table I. Causes of increased signal intensity in the basal ganglia on Tl-weighted images Calcification Neurofibromatosis Parenteral nutrition Hepatocerebral degeneration in hospital and was discharged a week later. Discussion The causes for increased TI- weighted signal intensity in the basal ganglia on MRI scans of the brain are shown in Table I. In our patient, calcification as a cause was ruled out on the unen- hanced CT scan of the brain and there was no history of neurofibromatosis or previous parenteral nutrition. Given the history of alcohol abuse with the related biochemical and imaging findings the diagnosis in this case was probably hepatocerebral degeneration. Acquired hepatocerebral degenera- tion is an irreversible neuro-degenera- tive syndrome occurring in associa- tion with chronic liver disease such as alcoholic cirrhosis and chronic hepatitis as well as with portosystemic shunts. Positive imaging findings cor- relate strongly with plasma ammonia levels or brain magnesium levels." MR spectroscopic studies have shown elevated glutamine levels and reduced choline metabolite and myo-inositol levels in patients with chronic hepatic encephalopathy.' Due to financial constraints, these tests were not per- formed in our patient. In addition to the areas in the brain described above, the anterior pituitary, caudate nucle- us, quadrigeminal plate and subthala- mic regions may also show signal intensity alterations. Hepatocerebral degeneration is a clinical syndrome and a subtype of chronic recurrent hepatic encephalo- pathy. In some cases the development of the cerebral dysfunction is rapid, developing over a matter of days resulting in altered consciousness from confusion through stupor, coma and death. In other cases the clinical picture is largely progressive and is characterised by movement disorders, dysarthria, tremor and ataxia. Pathologically, the brain shows enlargement and hyperplasia of astro- cytes as well as neuronal abnormali- ties in the cerebral and cerebellar cor- tices, basal ganglia and diencephalic nuclei. Necrosis with cavitation, glio- sis and myelin breakdown occur at these sites, Medical treatment is often disap- pointing, but in selected cases liver transplantation may be curative. The basal ganglia hyperintensity may resolve following liver transplantation. References 1. Osborn AC. Acquired metabolic, white matter, and degenerative diseases of the brain. In: Osborn AC, ed. Diagnostic Neuroradiology. St Lollis: Mosby, 1994: 748-781. 2. Layrargues GP. Movement dysfunction and hepatic encephalopathy. Metab Bmir' Dis 2001; 16: 27-35. 3. Kreis R, Ross BO, Parrow NA, et al. Metabolic disorders of the brain in chronic hepatic encephalopathy detected with H-l MR spec- troscopy. Radiology 1992; 182: 19-27. Primary Pediatric Radiology ------+----f Susan Williamson This text encompasses the most essential information in the field of pediatric radiology in a concise, easy-to-use source. Highly focused, succinct chapters, organized by symptom, help readers to select the most appropriate imaging approaches and arrive at correct diagnoses based on patients' presenting signs. Most significantly, the book separates coverage of neonates from material on older infants and children, since radiographic presentation differs so dramatically between these two groups. It includes valuable chapters on the neonate with respiratory distress, the neonate with birth trauma, the child and adolescent with abdominal pain and recognising cases of child abuse. It describes all relevant imaging modalities used in pediatric radiology, including conventional radiology, computed tomography, ultrasonography, magnetic resonance imaging and nuclear medicine. Each section begins with images and descriptions of normal anatomy as a foundation for better understanding of abnormal radiologic images. It discusses specific technical considerations and common mistakes, and provides memory hints and charts for quick reference, and features over 525 outstanding, never-before-published illustrations that cover the entire gamut of pediatric imaging findings. Oct 2001 , paperback, 246 pp, 527 illustrations, WBS, R799 Orders: SA Medical Association, Private Bag X1, Pinelands 7430. Tel (021) 530-6527. Fax (021) 531-4126. E-mail: fpalm@samedlcal.org Prices subject to foreign exchange fluctuations. Allow 2-3 weeks for delivery. 35 SA JOU RNAL OF RADIOLOGY • September 2002 mailto:fpalm@samedlcal.org