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[Guest guest]

Hi ,

I’ve been looking for information on pediatric

encephalopathy in the hopes of helping & Noah. I’ve looked everywhere, tried

every key word search I could think of, all to no avail. Found information on encephalopathy

for a few other pediatric diseases (HIV, Infections, lead poisoning, etc), but nothing

for liver disease. This has me

totally stumped and I’m usually pretty good at finding something. I hate puzzles with missing pieces…..could

you please (when you have time) take a look and see if you find anything?

Thank you,

Barb

[Guest guest]

Hi Barb and ;

From what I've gathered from reading 's posts [ please

correct me if I am wrong], Noah's doctors have not found any evidence

for clinical symptoms of hepatic encephalopathy, yet has seen

(and is very concerned by) behavioral changes in Noah. So the

question becomes ... is there any evidence for brain/behavioral

changes in liver disease in children prior to hepatic encephalopathy?

And are there non-invasive techniques that can be used to assess

brain patterns in children with liver disease prior to appearance of

clinical symptoms of hepatic encephalopathy? According to this study,

MRI might be one such technique:

AJNR Am J Neuroradiol. 2000 May;21(5):845-51.

MR imaging of CNS involvement in children affected by chronic liver

disease.

Genovese E, Maghnie M, Maggiore G, Tinelli C, Lizzoli F, De Giacomo

C, Pozza S, Campani R

Institute of Radiology, University of Pavia, Istituto Policlinico S.

Donato, Italy.

BACKGROUND AND PURPOSE: MR imaging sheds new light on CNS involvement

in the course of acquired chronic liver disease; however, the exact

pathogenetic mechanisms of hepatic encephalopathy and associated MR

abnormalities remain unclear. Our purpose was to relate MR signal

intensity abnormalities of the CNS to clinical, biochemical, and

pathologic features of childhood-onset chronic liver disease.

METHODS: Twenty-one patients (12 male and nine female patients) were

included in the study; two had Crigler-Najjar disease type 2, 17 had

chronic liver disease of different causes, and two had idiopathic

copper toxicosis. Twelve patients had histologically proved liver

cirrhosis, with a median disease duration of 175 months at the time

of MR study. None had clinical symptoms of hepatic encephalopathy. MR

imaging was performed using spin-echo T1- and T2-weighted sequences.

RESULTS: Eleven patients had abnormal MR imaging findings of the

brain revealed by T1-weighted MR sequences; two of the 11 had

idiopathic copper toxicosis. The affected sites were the hypothalamus

and globus pallidus, presenting symmetrical and bilateral high signal

intensities, or the pituitary gland, which appeared homogeneously

hyperintense, or both findings. Eight of the 12 patients with

cirrhosis had abnormal MR signals of the brain. In these, the median

cirrhosis duration was shorter (169 months) than in the remaining

four patients with normal MR signals (177 months). A significant

correlation was found between abnormal MR signals of the brain and

cirrhosis (P = .008) and factor V activity (P = .008). CONCLUSION: MR

imaging confirms the presence of abnormal brain signals in the globus

pallidus, hypothalamus, and pituitary gland in patients with

childhood-onset liver disease in the absence of clinical symptoms of

encephalopathy. Signal intensity abnormalities are likely caused by

an as yet unidentified metabolic process partially correlated with

the severity of liver disease. PMID: 10815659.

The full text of the above article is available at:

http://www.ajnr.org/cgi/content/full/21/5/845

Others have noted brain MRI changes (apparently unrelated to hepatic

encephalopathy) especially in cholestatic liver diseases:

Eur Radiol. 1997;7(6):905-9.

Brain MRI changes in chronic liver disease.

Skehan S, Norris S, Hegarty J, Owens A, MacErlaine D

Department of Diagnostic Imaging, St. 's Hospital, Elm Park,

Dublin 4, Ireland.

Cirrhotic patients are known to have abnormally high signal

principally in the globus pallidus on non-contrast T1-weighted MRI.

The purpose of this study was to relate MR changes to clinical and

pathological features of chronic liver disease. We confirmed

abnormally high signal in the globus pallidus on T1-weighted images

in 25 of 28 patients with chronic liver disease, showing that it also

occurs in patients who have not yet progressed to cirrhosis. Changes

were seen in patients both with and without clinical portosystemic

shunting. This abnormality is not responsible for hepatic

encephalopathy. Cholestatic disease was more likely to produce marked

changes than non-cholestatic disease. No statistically significant

correlation was demonstrated between the severity of liver disease

and the degree of MR abnormality. However, marked improvement in MR

appearances was seen after successful liver transplantation. PMID:

9228107.

Therefore, it seems quite plausible that brain and/or behavioral

changes can take place prior to cirrhosis and HE. Perhaps Noah might

be experiencing something of this kind?

I hope that this is of some help.

Best regards,

Dave

(father of (21), PSC 07/03; UC 08/03)

[Guest guest]

Thank you for posting this- my son Braden has been having symptoms of

HE for a while despite being on xifaxan. I am hopeful that we may be

able to find some answers soon for him as well as for Noah.

Lori

lucky mom to 10 year old wonderfully wild triplets

including Braden w/short bowel syndrome, PSC, portal hypertension & FAP

[Guest guest]

,

Yes, I would agree with that assessment. I almost think we keep

missing the acute phase of WHATEVER is going on with him. By the time

the lab orders get to us his behavior has improved temporarily, but we

draw the labs...but then the labs come back normal. One time we got a

high ammonia and that was written off as a possible bad draw. Now I

know we have also heard of ammonia not being the be all end all in HE

diagnosis. So I guess I don't know why all the reliance on this ONE test.

So the MRI is interesting to me. I do have one thing in my favor

here. He is scheduled for an MRI for his migraine diagnosis recently.

I would assume that they could see WHATEVER on that same scan too?!

Is that a safe assumption? I would think the radiologist would just

be reading for any and all abnormalities.

Would he need to present in the acute phase for something like this?

He is becoming a danger to himself. I almost took him to the ER

today. He does not get it either that is the thing.

Thank you all for talking to me about this. I know Lori and I have

been sort of laughing at how similar our boys are...but it is not so

funny anymore. I guess it is good to not be alone though. I welcome

any and all suggestions. This poor child is so out of it. He has

changed so much since Thanksgiving. We thought it was jetlag. HA!

Blessings,

www.caringbridge.org/visit/noahwmartens