The authors are to be congratulated on a good article. Do you they have any experience with this method in the treatment of epilepsy? Thank you and please continue the good work.
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...
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.
Eftychiadis and Chen describe the fascinating history of St Vitus. Sydenham's description of a movement disorder which "attacks boys
and girls from the tenth year till they have done growing..." certainly would make one think of tic disorders or Tourette syndrome.
In my
clinical practice, it is very common for people with tic disorders to give
a family history of "St Vitus Dance". This, tied in with...
Eftychiadis and Chen describe the fascinating history of St Vitus. Sydenham's description of a movement disorder which "attacks boys
and girls from the tenth year till they have done growing..." certainly would make one think of tic disorders or Tourette syndrome.
In my
clinical practice, it is very common for people with tic disorders to give
a family history of "St Vitus Dance". This, tied in with the increased
prevalence of obssesive compulsive symptoms in people with Sydenham's
chorea[1] suggests a nosological connection between
these disease entities.
Reference
(1) Swedo SE, et al. High prevalence of obsessive-compulsive
symptoms in patients with Sydenham's chorea. Am J Psychiatry 1989;146:246-9.
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...
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.
(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.
Editor,
The authors are to be congratulated on a good article. Do you they have any experience with this method in the treatment of epilepsy? Thank you and please continue the good work.
Dear Editor,
This is an excellent review. I would like to ask the authors did you find aspiration (food particles or saliva) as one of the causes of SUDEP?
Dear Editor
Were these patients all on same dosage and on the same medication. Can some dementia be medication related? Just a thought.
Continue good work.
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...
Eftychiadis and Chen describe the fascinating history of St Vitus. Sydenham's description of a movement disorder which "attacks boys and girls from the tenth year till they have done growing..." certainly would make one think of tic disorders or Tourette syndrome.
In my clinical practice, it is very common for people with tic disorders to give a family history of "St Vitus Dance". This, tied in with...
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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