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• Acquired hepatocerebral degeneration syndrome - Authors' response
  Andrea Stracciari
  Published on: 13 April 2016
• Acquired hepatocerebral degeneration syndrome
  Mark Lewis
  Published on: 13 April 2016

• Published on: 13 April 2016

  Acquired hepatocerebral degeneration syndrome - Authors' response

  • Andrea Stracciari
  • Other Contributors:
    • Maria Guarino

  Dear Editor,

  We thank Dr Mark Lewis for his interest in our paper,[1] but his comments deserve some replies. He writes that we suggest that the pathogenesis of the acquired hepatocerebral degeneration syndrome (AHD) is likely to be related to repeated episodes of hepatic encephalopathy. This is incorrect. We did not suggest any pathogenesis for AHD, remarking that its pathogenesis is still unclear. We only refer...

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  Dear Editor,

  We thank Dr Mark Lewis for his interest in our paper,[1] but his comments deserve some replies. He writes that we suggest that the pathogenesis of the acquired hepatocerebral degeneration syndrome (AHD) is likely to be related to repeated episodes of hepatic encephalopathy. This is incorrect. We did not suggest any pathogenesis for AHD, remarking that its pathogenesis is still unclear. We only referred to some possible pathogenetic mechanisms reported in the literature, simply recalling that such syndrome often appears after repeated episodes of hepatic encephalopathy: this obviously does not imply that the relationship of AHD with the acute form of hepatic encephalopathy is clearly established. Portal-systemic shunting, as mentioned by Dr. Lewis, may certainly play an important pathogenetic role in determining AHD, but clarifying the pathogenesis of AHD was not the aim of our paper.

  Dr Lewis suggests being cautious in advocating liver transplantation (LT) for these patients. Why? Criteria for selection of LT candidates are well defined and include patients with end stage liver disease for whom, we know, LT is the only chance of survival. Our patient, as well described in our paper,[1] underwent LT for liver failure and not for neurological complications, according to international criteria. Instead, it is unclear whether the indication for LT in the patient with hepatic myelopathy[2] cited by Dr Lewis was the hepatic or neurological worsening (it seems to have been the latter). The poor outcome of his patient was referred to fecal peritonitis, his previous neurological status not conditioning the post-operative period. We think, as general rule, that liver failure should be the sole indication for LT. It is trivial to remark that the patient has to be advised about the risk of surgery, because this is the rule in any case of operation. Finally, we emphasize the importance of our observation: the full recovery of the cerebral dysfunction related to AHD after LT. This should be stressed because surgeons are often reluctant to operate patients fulfilling indications for LT but with evident stable signs of encephalopathy, thinking that they have dementia and will not have benefit from LT. Our experience indicates that the presence of a hepatic dementia, like that due to AHD, must not be considered a controindication to surgery. On the contrary, besides being life-saving, LT may also be able to restore normal neurological and neuropsychological status.

  References
  (1) Stracciari A, Guarino M, Pazzaglia P, et al. Acquired hepatocerebral degeneration: full recovery after liver transplantation. J Neurol Neurosurg Psychiatry 2001;70:136-7.

  (2) Lewis MB, MacQuillan G, Bamford JM, et al. Delayed myelopathic presentation of the acquired hepatocerebral degeneration syndrome. Neurology 2000;54:1011.

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  Conflict of Interest:
  None declared.

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• Published on: 13 April 2016

  Acquired hepatocerebral degeneration syndrome

  • Mark Lewis, Clinical Research Fellow in Neurology and Liver Transplantation

  Dear Editor,

  I read with interest the recent article by Stracciari et al.[1] They suggest that the pathogenesis of the acquired hepatocerebral degeneration syndrome (AHCD) is likely to be related to repeated episodes of hepatic encephalopathy; however, by their own admission there are cases where no such episodes have occurred.[2] Assuming therefore that these “never encephalopathic” cases are manifestations of the sa...

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  Dear Editor,

  I read with interest the recent article by Stracciari et al.[1] They suggest that the pathogenesis of the acquired hepatocerebral degeneration syndrome (AHCD) is likely to be related to repeated episodes of hepatic encephalopathy; however, by their own admission there are cases where no such episodes have occurred.[2] Assuming therefore that these “never encephalopathic” cases are manifestations of the same entity, another process must be responsible.

  A common feature to all these cases is the presence of portal-systemic shunting, either spontaneous or iatrogenic, and it would seem more likely that this is the pathological mechanism rather than simply, repeated episodes of hepatic encephalopathy. The authors also need to be cautious in advocating a high risk treatment, such as liver transplantation, when the evidence cited consists solely of two case reports plus their own.[1][3][4] Liver transplantation has actually been performed in other patients with AHCD[2] and also in patients with the related condition of hepatic myelopathy, and not all have done well.[5][6] Clearly there is likely to be reporting bias here as well.

  In addition, when advising patients, we must not forget the risks of the procedure itself. In the case we reported, the patient died of complications related to the transplant only two weeks after the procedure.[2]

  References
  (1) Stracciari A, Guarino M, Pazzaglia P, Marchesini G, Pisi P. Acquired hepatocerebral degeneration: full recovery after liver transplantation. J Neurol Neurosurg Psychiatry 2001;70:136-7.

  (2) Lewis MB, MacQuillan G, Bamford JM, Howdle PD. Delayed myelopathic presentation of the acquired hepatocerebral degeneration syndrome [Clinical/scientific note]. Neurology 2000;54:1011.

  (3) Powell EE, Pender MP, Chalk JB, Parkin PJ, Strong R, Lynch S, Kerlin P, Cooksley WG, Cheng W, Powell LW. Improvement in chronic hepatocerebral degeneration following liver transplantation. Gastroenterology 1990;98:1079-82.

  (4) Parkes JD, Murray-Lyon IM, Williams R. Neuropsychiatric and electro-encephalographic changes after transplantation of the liver. Q J Med 1970;39:515-27.

  (5) Counsell C, Warlow C. Failure of presumed hepatic myelopathy to improve after liver transplantation [letter]. J Neurol Neurosurg Psychiatry 1996;60:590.

  (6) Troisi R, Debruyne J, de Hemptinne B. Improvement of hepatic myelopathy after liver transplantation [letter]. N Engl J Med 1999;340:151.

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  Conflict of Interest:
  None declared.

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