Neurology India
menu-bar5 Open access journal indexed with Index Medicus
  Users online: 10333  
 Home | Login 
About Editorial board Articlesmenu-bullet NSI Publicationsmenu-bullet Search Instructions Online Submission Subscribe Videos Etcetera Contact
  Navigate Here 
  » Next article
  » Previous article 
  » Table of Contents
 Resource Links
  »  Similar in PUBMED
 »  Search Pubmed for
 »  Search in Google Scholar for
 »Related articles
  »  Article in PDF (125 KB)
  »  Citation Manager
  »  Access Statistics
  »  Reader Comments
  »  Email Alert *
  »  Add to My List *
* Registration required (free)  

  In this Article
 »  Abstract
 »  Introduction
 »  Case Report
 »  Discussion
 »  Conclusion
 »  References
 »  Article Figures

 Article Access Statistics
    PDF Downloaded304    
    Comments [Add]    
    Cited by others 8    

Recommend this journal


Year : 2006  |  Volume : 54  |  Issue : 3  |  Page : 298-300

Hepatic myelopathy: A rare complication following extrahepatic portal vein occlusion and lienorenal shunt

1 Department of Neurology, National Institute of Mental Health and Neurosciences, Bangalore, India
2 Department of Neuroimaging and Interventional Radiology, National Institute of Mental Health and Neurosciences, Bangalore, India

Date of Acceptance28-Feb-2006

Correspondence Address:
S Sinha
Department of Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), P.O. Box 2900, Dharmaram College P.O, Bangalore - 560 029
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.27159

Rights and Permissions

 » Abstract 

A 19-year-old gentleman presented with slowly progressive spastic paraparesis, 2 years after the therapeutic lienorenal shunt for portal hypertension secondary to cirrhosis and portal vein occlusion. After 2 years of initial evaluation, the motor functions had not worsened further. He did not have any obvious clinical or EEG features of hepatic encephalopathy. Other causes for myelopathy were ruled out. Contribution of portal vein occlusion to portosystemic shunting has not been reported previously in patients with 'hepatic myelopathy.' This uncommon complication needs to be considered in patients with shunt surgery for relieving portal hypertension.

Keywords: Hepatic myelopathy, lienorenal shunt, portal vein occlusion

How to cite this article:
Panicker J, Sinha S, Taly A B, Ravishankar S, Arunodaya G R. Hepatic myelopathy: A rare complication following extrahepatic portal vein occlusion and lienorenal shunt. Neurol India 2006;54:298-300

How to cite this URL:
Panicker J, Sinha S, Taly A B, Ravishankar S, Arunodaya G R. Hepatic myelopathy: A rare complication following extrahepatic portal vein occlusion and lienorenal shunt. Neurol India [serial online] 2006 [cited 2023 Jan 27];54:298-300. Available from: https://www.neurologyindia.com/text.asp?2006/54/3/298/27159

 » Introduction Top

Shunting of blood from the portal to systemic circulation in patients with chronic liver disease is associated with wide spectra of neurological manifestations. The syndrome of spastic paraparesis, called hepatic myelopathy (HM)[1] or portal-systemic myelopathy,[2] a well-recognized entity, is often subclinical in majority[3] and potentially reversible following timely liver transplantation.[4],[5]

We report slowly progressive spastic myelopathy observed in a young man, secondary to portal-vein occlusion and cirrhosis of unknown etiology.

 » Case Report Top

A 19-year-old farmer manifested symptoms of stiffness of legs, tripping over objects and tingling sensation of legs of 2 years duration. There was no involvement of upper limb, sphincter or erectile function, cognitive or behavioral changes, tremors or myoclonus. Four years back, he had recurrent hematemesis and pitting pedal edema. There was no history of jaundice or alcohol consumption. He had no family history of liver or neurological illness. He was earlier diagnosed to have extrahepatic portal-vein obstruction with portal-vein cavernoma causing cirrhosis of liver, splenomegaly and grade II esophageal varices. He underwent splenectomy with left lienorenal shunt, following which hematemesis and limb edema remitted.

He had sparse axillary and pubic hair. There was no skin hyperpigmentation, KF ring, pedal edema, spider-nevi, gynecomastia, testicular atrophy and caput medusae. Liver span was reduced to 8 cm. He scored 30/30 on MMSE (Folstein). There was bilateral Achilles' tendon contracture and spasticity with pyramidal weakness in both lower limbs. Muscle stretch reflexes in the lower limbs were exaggerated and plantar response was extensor bilaterally. Gait was spastic. There was no other deficit.

There was reduced hemoglobin (11.6 gm/dL) and normocytic hypochromic anemia. Liver function tests revealed normal serum bilirubin (0.6 mg/dL) and alanine aminotransferase (39 U/L), minimally raised aspartate aminotransferase (68 U/L), reduced total protein (5 gm/dL) and albumin (2.6 gm/dL) and abnormal A:G ratio (1.1:1). Plasma ammonia was elevated to 99 mmol/L (N=11-32 mmol/L). Renal function tests and serum electrolytes were normal. Craniospinal magnetic resonance imaging (MRI) was normal [Figure - 1]a, b, c. CSF was acellular with normal protein (18 mg/dl). Immunological tests (CSF) for syphilis, tuberculosis and HTLV-1 were negative. CSF immunoelectrophoresis showed oligoclonal bands. Visual, brain stem auditory and somatosensory evoked potentials and motor (median, ulnar, common peroneal) and sensory (median, ulnar, sural) nerve conduction studies were within normal limits. Thyroid functions and serum B 12 estimation were normal. Serum HIV (ELISA) was negative. Serum Hexoseaminidase level was normal. Ultrasound (abdomen) showed features of cirrhosis of liver with multiple portal vein collaterals and nonvisualization of spleen. Doppler study showed functioning lienorenal shunt. Contrast-enhanced CT (abdomen) confirmed these observations [Figure - 2]. Upper gastrointestinal endoscopy disclosed esophageal varices. Stool for occult blood was negative. Liver biopsy was attempted but sample could not be obtained. Investigations for Wilson's disease and markers for chronic viral hepatitis were negative.

A diagnosis of spastic paraparesis due to HM secondary to portacaval shunting was considered. In view of significant esophageal varices, lienorenal shunt was not reversed. He was treated conservatively with protein restriction, physiotherapy and baclofen. At follow-up after 2 years, he was neurologically stable. The myelopathic features, including MRC grade and activities of daily living had not worsened. Repeat liver function tests were normal except for reduced total protein (4.8 gm/dl) and albumin (2.3 gm/dl) and raised plasma ammonia of 89 mmol/L. EEG showed normal background activity without any triphasic waves or paroxysmal activity [Figure - 1]d. His MMSE (Folstein) score remained 30/30; however, detailed neuropsychological assessment revealed slight impairment in visual learning and memory, abstract thinking and working memory, suggesting minimal involvement of bifrontal (right>left) and right temporal lobe functions.

 » Discussion Top

Leigh and Card were the first to describe the occurrence of pure motor spastic paraparesis following liver failure and many such reports have been documented thereafter.[6] Gospe et al[7] were the first to report a progressive HM due to hepatic failure in a 14-year-adolescent. Interestingly, ours is the second such report of HM, with onset at 17 years. Interval between shunt surgery and onset of neurological illness is varied and ranges from 6 to 36 months. The delay is longer in patients with lienorenal shunts.[1] The characteristic profile consists of insidious progression of lower limb spasticity and weakness. Typically, patients have bouts of hepatic encephalopathy. Our patient did not have any obvious clinical or electrophysiological (EEG) features of hepatic encephalopathy.

Our patient developed symptoms within 2 years of shunt surgery with a profile consistent with HM. Interestingly; there was no worsening of neurological features for the last 2 years. We speculate that early onset of myelopathy may have been due to significant shunting as a result of several factors: cirrhosis and spontaneous shunting, portal-vein occlusion and cavernoma leading to anastomosis and shunting and lastly, therapeutic lienorenal shunting. Portacaval shunting plays a substantial role in the pathogenesis of HM. Spontaneous shunting occurs within the liver as a compensatory mechanism. In cirrhosis with portal hypertension, surgical shunting is performed to reduce the portal pressure, as in portacaval shunts and lienorenal shunt. In the latter, portal vein is anastomosed to the splenic vein.[1],[8] HM was initially noted following surgical placement of a portacaval shunt, but it may also occur after transjugular intrahepatic portosystemic shunt (TIPS).[9] Our patient had cirrhosis at the time of presentation and also had additional spontaneous portacaval shunting. Doppler and CT scan confirmed the functioning of lienorenal shunt. In addition, there was hyperammonemia and absence of some secondary sexual features. Symptomatic myelopathy following lienorenal shunt is uncommon. Craniospinal MRI, CSF studies, serum B 12 and multimodal evoked potentials were normal. There was no clinical or MRI evidence to suggest adrenoleucodystrophy. Hexoseaminidase levels were normal. Infections like tuberculosis, syphilis, HTLV, HIV were ruled out. The significance of positive oligoclonal bands in CSF is unclear. Whether it will provide insight regarding pathophysiological mechanism needs to be explored.

Autopsy studies in patients with HM have shown selective demyelination in the corticospinal tracts, Betz cell loss and some authors have even regarded HM as a restricted form of encephalopathy.[10] Nitrogenous toxins, e.g., ammonia that inappropriately enters the systemic circulation as a result of shunting, are implicated in its pathogenesis.[10] HM responds poorly to treatment. Liver transplantation may reverse some of the neurological changes.[4],[5]

 » Conclusion Top

We report an unusual instance of chronic HM following lienorenal shunting at a young age without any obvious features of hepatic encephalopathy. This rare complication needs to be considered in patients offered shunt surgery for relieving portal hypertension. Portal vein occlusion as a contributory cause to portosystemic shunting has not been previously reported in patients with hepatic myelopathy.

 » References Top

1.Campellone JV, Lacomis D, Giuliani MJ, Kroboth FJ. Hepatic myelopathy: Case report with review of the literature. Clin Neurol Neurosurg 1996;98:242-6.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]
2.Gauthier G, Wildi E. Porto-systemic encephalo-myelopathy. Rev Neurol (Paris) 1975;131:319-38.   Back to cited text no. 2  [PUBMED]  [FULLTEXT]
3.Nardone R, Buratti T, Oliviero A, Lochmann A, Tezzon F. Corticospinal involvement in patients with portosystemic shunt due to liver cirrhosis; A MEP study. J Neurol 2006;253:81-5.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]
4.Nolano M, Guardascione MA, Amitrano L, Perretti A, Fiorillo F, Ascione A, et al . Cortico-spinal pathways and inhibitory mechanisms in hepatic encephalopathy. Electroenceph Clin Neurophysiol 1997;105:72-8.  Back to cited text no. 4    
5.Weissenborn K, Tietge UJ, Bokemeyer M, Mohammadi B, Bode U, Manns MP, et al . Liver transplantation improves hepatic myelopathy: Evidence by three cases. Gastroenterology 2003;124:346-51.   Back to cited text no. 5    
6.Mendoza G, Marti-Fabregas J, Kulisevsky J, Escartin A. Hepatic myelopathy: A rare complication of portacaval shunt. Eur Neurol 1994;34:209-12.  Back to cited text no. 6  [PUBMED]  [FULLTEXT]
7.Gospe SM Jr, Caruso RD, Clegg MS, Keen CL, Pimstone NR, Ducore JM, et al . Paraparesis, hypermanganesaemia and polycythaemia: A novel presentation of cirrhosis. Arch Dis Childhood 2000;83:439-42.  Back to cited text no. 7    
8.Robinson CE, Anzarut A. Myelopathy in cirrhosis. Paraplegia 1978;16:3-4.  Back to cited text no. 8  [PUBMED]  [FULLTEXT]
9.Wang MQ, Dake MD, Cui ZP, Wang ZQ, Gao YA. Portal-systemic myelopathy after transjugular intrahepatic portosystemic shunt creation: Report of four cases. J Vasc Interv Radiol 2001;12:879-81.  Back to cited text no. 9  [PUBMED]  [FULLTEXT]
10.Pant SS, Bhargava AN, Singh MM, Dhanda PC. Myelopathy in hepatic cirrhosis. Br Med J 1963;1:1064-5.  Back to cited text no. 10    


[Figure - 1], [Figure - 2]

This article has been cited by
1 Liver Transplantation Reverses Hepatic Myelopathy in 2 Children With Hepatitis A Infection
Roshan Koul, Bikrant Bihari Lal, Viniyendra Pamecha, Shiv Sarin, Seema Alam
Child Neurology Open. 2021; 8: 2329048X20
[Pubmed] | [DOI]
2 Neurological disorders in liver transplant candidates: Pathophysiology and clinical assessment
Paolo Feltracco,Annachiara Cagnin,Cristiana Carollo,Stefania Barbieri,Carlo Ori
Transplantation Reviews. 2017; 31(3): 193
[Pubmed] | [DOI]
3 Evaluation of mid- and long-term efficacy of shunt limiting for hepatic myelopathy after transjugular intrahepatic portosystemic shunt
Hongwei Zhao,Fuquan Liu,Zhendong Yue,Lei Wang,Zhenhua Fan
Clinics and Research in Hepatology and Gastroenterology. 2015;
[Pubmed] | [DOI]
4 Treatment of Portosystemic Shunt Myelopathy with a Stent Graft Deployed through a Transjugular Intrahepatic Route
Deepak Jain,Ankur Arora,Pranjal Deka,Amar Mukund,Shorav Bhatnagar,Deepti Jindal,Niteen Kumar,Viniyendra Pamecha
CardioVascular and Interventional Radiology. 2013; 36(4): 1161
[Pubmed] | [DOI]
5 Hepatic Myelopathy in a Patient with Decompensated Alcoholic Cirrhosis and Portal Colopathy
Madhumita Premkumar,Avishek Bagchi,Neha Kapoor,Ankit Gupta,Gaurav Maurya,Shubham Vatsya,Siddharth Kapahtia,Premashish Kar
Case Reports in Hepatology. 2012; 2012: 1
[Pubmed] | [DOI]
6 Hepatic myelopathy: Morphology of the thoracic and lumbar cord in liver cirrhosis and non-cirrhosis corpses and comparison of neuron functional protein
Lei, Y. and Liu, Z. and Huang, H. and Zhang, S. and Zhou, Y. and Wu, S. and Hou, X. and Gong, J. and Wu, A.
Neural Regeneration Research. 2011; 6(29): 2273-2278
7 Use of model for end-stage liver disease exception points for early liver transplantation and successful reversal of hepatic myelopathy with a review of the literature
Cary Caldwell, Norman Werdiger, Sofia Jakab, Michael Schilsky, Antonios Arvelakis, Sanjay Kulkarni, Sukru Emre
Liver Transplantation. 2010; 16(7): 818
[VIEW] | [DOI]
8 Clinical features of hepatic myelopathy in patients with chronic liver disease
Yin, Y.H., Ma, Z.J., Guan, Y.H., Ren, Y.D., Zhang, Z.L.
Postgraduate Medical Journal. 2009; 85(1000): 64-68


Print this article  Email this article
Previous article Next article
Online since 20th March '04
Published by Wolters Kluwer - Medknow