CASEWORK

Casework Block Placement: Clinical Conditions with Adults and Older Adults

Casework Block Placement: Clinical Conditions with Adults and Older Adults

Reflections on Practice

Classical Conditions is a general name of the list of deceases which are termed as classical conditions. This condition may arise as a result of a congenital lymphatic dysplasia (primary lymphedema) or may result from diseases (secondary lymphedema) as breast cancer surgically treated with mastectomy or as a result of repeated infections of the lymphatic vessels. Both situations result in a reduction of the lymphatic transport and the consequent local accumulation of fluid in the tissues is a highly disabling condition.

Encephalopathy

Hepatic encephalopathy is characterized by a variety of neurological symptoms associated with acute or chronic liver diseases and/or portosystemic shunting. Movement disorders may be observed as part of the clinical picture in a particular form of chronic encephalopathy that has been called chronic acquired hepatocerebral degeneration (Victor et al., 1965). These neurologic symptoms develop after repeated bouts of hepatic encephalopathy, but in some patients these may become manifest in the absence of discrete episodes of hepatic coma. The pathophysiology of movement dysfunction is not fully understood and treatment of neurological abnormalities might be disappointing in some patients left with permanent neurologic squeal.

For explanation, I would like to discuss a case of an older adult 30-year-old female was first evaluated in our institution for primary biliary cirrhosis (PBC) in 1990. After 4 years of uneventful evolution, she bled from varices in 1994 and varices were successfully obliterated by repeated endoscopic variceal band ligation. Ascites was observed for the first time in early 1999 and became resistant to medical treatment in November of the same year. She was then considered to be a candidate for liver transplantation, despite portal and mesenteric vein thrombosis found incidentally when the pretransplant evaluation was performed. At that time, neurological status was normal.

She was readmitted in February 2000 for asterixis and buco-linguo-facial dyskinesia. Neurological examination also disclosed diffuse hyperreflexia. EEG showed a diffuse slowing of electrical activity without any focal abnormality. MRI scan demonstrated bilateral T1-weighted pallidal hyperintensity.

Because of the severity of liver failure (Pugh score 10/15), the patientwas listed for liver transplantation. During the waiting time, abnormal movements were only slightly improved by a combination of lactulose, metronidazole, and sodium benzoate.

This patient was transplanted on April 30. Because of the portal venous thrombosis, an anastomosis between the portal vein of the donor and the vena cava of the recipient was performed. Postoperative evolution was characterized by a progressive normalization of neurological status. Movement disorders were no longer detectable 2 months after liver transplantation.

This case report is a good example of severe movement disorder associated with chronic liver failure and extensive portosystemic shunting that are occasionally observed; the original description of this syndrome was reported by Victor et al. (1965) and it was called acquired hepatocerebral degeneration or pseudo-Wilson syndrome. Complete reversal of the neurological picture after transplantation suggests a metabolic pathogenesis.

Clinical Presentation

Abnormal movements may be part of the clinical ...