Fatal deterioration of neurological disease after orthotopic liver transplantation for valproic acid-induced liver damage.
We describe a 12-year-old girl with an early onset neurologic disease of slow progressiveness and electro-encephalography showing epileptic activity. The girl developed fulminant liver failure 5 months after the start of valproic acid treatment. Repeated mitochondrial assays failed to prove a mitochondrial disorder, but muscle biopsies were slightly pathological. Liver histology indicated acute-on-chronic liver disease. Six weeks after a successful orthotopic liver transplantation her neurological condition deteriorated rapidly, soon leading to generalized cortical disease and death. Post-mortem brain examination showed advanced central nervous destruction. We suggest that this is a late-onset Huttenlocher variant of Alpers' syndrome, where fulminant liver failure can be triggered by valproic acid, and orthotopic liver transplantation can subsequently trigger a fatal neurologic deterioration. Our case illustrates that when a referral center receives a previously unknown patient with hepatocellular insufficiency, it might be impossible to differentiate between fulminant vs. acute-on-chronic liver failure, and the decision whether to perform a liver transplantation or not would become difficult.
Department of Pediatrics, Karolinska Institutet, Huddinge University Hospital, Sweden.
N
N
Kayihan
I
I
Nennesmo
B G
BG
Ericzon
A
A
Németh
D000293:Adolescent; D000927:Anticonvulsants / Q000009:adverse effects*; D002549:Diffuse Cerebral Sclerosis of Schilder / Q000150:complications* / Q000473:pathology*; D004569:Electroencephalography; D004827:Epilepsy / Q000150:complications / Q000188:drug therapy; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D017114:Liver Failure, Acute / Q000139:chemically induced* / Q000601:surgery*; D016031:Liver Transplantation; D012307:Risk Factors; D014635:Valproic Acid / Q000009:adverse effects*
D000927:Anticonvulsants; D014635:Valproic Acid