Users Online: 1374

Home Print this page Email this page Small font sizeDefault font sizeIncrease font size

Home | About us | Editorial board | Search | Ahead of print | Current issue | Archives | Submit article | Instructions | Subscribe | Contacts | Login 
     

   Table of Contents      
CASE REPORT
Year : 2013  |  Volume : 3  |  Issue : 4  |  Page : 392-395

Hepatic myelopathy in a patient with decompensated liver disease: An unusual complication


Department of Medicine, Government Medical College, Haldwani, Uttarakhand, India

Date of Submission24-Jun-2013
Date of Acceptance15-Jul-2013
Date of Web Publication15-Oct-2013

Correspondence Address:
Vivekanand Satyawali
Type 4 Block, k2, Government Medical College Campus, Haldwani - 263 139, Uttarakhand
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2231-0738.119858

Rights and Permissions
   Abstract 

Hepatic myelopathy (HM) is a rare neurological complication of the chronic liver disease usually seen in adults and presents as a disabling progressive pure motor spastic paraparesis, which is almost always associated with overt liver failure. We report a case of a 45-year-old male who presented with rapidly progressive spastic paraparesis due to HM and features of hepatic decompensation (postnecrotic cirrhosis), who presented with the second episode of hepatic encephalopathy. Patient had not undergone any shunt procedure.

Keywords: Hepatitis B, myelopathy, spastic paraparesis


How to cite this article:
Satyawali V, Singh Y, Khalil M, Kumar J. Hepatic myelopathy in a patient with decompensated liver disease: An unusual complication. Int J Nutr Pharmacol Neurol Dis 2013;3:392-5

How to cite this URL:
Satyawali V, Singh Y, Khalil M, Kumar J. Hepatic myelopathy in a patient with decompensated liver disease: An unusual complication. Int J Nutr Pharmacol Neurol Dis [serial online] 2013 [cited 2019 Nov 21];3:392-5. Available from: http://www.ijnpnd.com/text.asp?2013/3/4/392/119858


   Introduction Top


Hepatic myelopathy (HM) is an insidious onset pure motor spastic paraparesis without sensory or bladder or bowel involvement in patients with liver disease in which the neurological dysfunction cannot be attributed to another disorder. A progressive spastic paraparesis in patients with hepatic failure was first described by Leigh and Card, [1] followed by a detailed description of HM by other authors who observed this rare neurological complication of cirrhosis, especially in patients with portosystemic shunts. [2],[3],[4] In India, HM was reported for the first time by Pant et al., who described two cases of spastic paraparesis in patients with liver cirrhosis, one with a spontaneous portocaval shunt and the other with a surgical portocaval anastomosis. [5] The typical clinical presentation of this disorder is of a patient with underlying chronic liver disease, developing progressive pure motor spastic paraparesis with the minimal or no sensory deficit and without bowel and bladder involvement. Most patients report prior episodes of hepatic encephalopathy and in many cases, the development of myelopathy follows the creation of surgical shunts. [6],[7],[8] Early and accurate diagnosis of HM is important because patients with early stages of the disease can recover following liver transplantation. [9] Neuropathological studies show demyelination in the lateral corticospinal tracts, with varying degrees of axonal loss. [2] Motor-evoked potential studies may be suitable for the early diagnosis of HM, even in patients with preclinical stages of the disease. [10]


   Case Report Top


A 45-year-old male farmer, hailing from Uttar Pradesh with a history of hepatitis B virus infection for 4 years (incidentally detected during the blood transfusion) presented to us with the complaints of difficulty in walking due to stiffness of lower limbs for 1 year, associated with symmetrical weakness. There was no history of fasciculations or wasting. 2 weeks ago, he noted fever, which rapidly caused prostration. His family reported altered behavior, forgetfulness, lack of attention with disturbed sleep wake cycle. There was no history of bowel and bladder involvement, ocular or vision abnormalities, seizures, diabetes mellitus, hypertension, tuberculosis, trauma, exposure to industrial toxins or radiation, blood or blood component therapy, bleeding disorders, promiscuity, or similar complaints in the family or neighborhood. His history was significant for one other factor, occasional intake of Lathyrus sativus (khesari dal), but not in a quantity or frequency enough to cause lathyrism. He had no significant history of alcohol intake. He did not smoke or consume tobacco. General examination was normal and there was mild splenomegaly. The patient presented with hepatic encephalopathy, with impaired attention and flapping tremors. Spastic paraparesis (Grade 3 by Medical Research Council Scale) was present along with hyperreflexia and bilateral extensor plantar response. He had ankle and patellar clonus. Rest central nervous system (CNS) examination was normal. All routine investigations were normal, only bilirubin was raised mildly [Table 1].
Table 1: Hematological and biochemical parameter of patient

Click here to view


Cerebrospinal fluid (CSF) was normal. Upper gastrointestinal (GI) endoscopy was having Grade 12 varices. Electroencephalography revealed background slowing without any spikes suggestive of metabolic encephalopathy. Patient's serum was reactive to hepatitis B, viral marker. Patient's serum was nonreactive to hepatitis A, C, and E viral markers as well as to human immunodeficiency virus I and II. Magnetic resonance imaging of the spine and brain showed non-specific ischemic changes [Figure 1], [Figure 2] and [Figure 3]. Ultrasound of the abdomen showed mildly nodular liver with coarsened echotexture with span 13 cm, splenomegaly, a dilated portal vein, and mild ascites [Figure 4]. Nerve conduction velocity was normal.
Figure 1: Magnetic resonance imaging brain showing mild cerebral and cerebellar atrophy

Click here to view
Figure 2: Magnetic resonance imaging brain showing mild chronic ischemic changes

Click here to view
Figure 3: Normal magnetic resonance imaging spine

Click here to view
Figure 4: Ultrasound sonography abdomen showing coarse echotexture of liver

Click here to view


He developed massive lower GI bleed after 1 month and a colonoscopy could not be done. The patient was transfused with fresh frozen plasma and other suppourtive measures were done, but life could not be saved. In view of no significant history of alcohol and positive serology for hepatitis B virus, the patient's diagnosis was postnecrotic cirrhosis with portal hypertension with Grade 12 varices with massive GI bleed.


   Discussion Top


The diagnosis of HM entails the presence of an insidious onset rapidly progressive pure motor paraparesis without bowel bladder or sensory involvement in patients of documented hepatic insufficiency or those having undergone shunt surgery or liver transplantation. [1],[2],[3],[4],[5],[6],[7],[8] Imaging has been non-contributory in these cases.

There is marked paucity of data regarding the pathogenesis of HM. There have been reports of chronic hepatic insufficiency (idiopathic and post-infective) and post-liver transplantation or shunt surgery (transjugular intrahepatic portal systemic shunting) patients developing HM along with rare reports of infantile portal vein thrombosis, [8] congenital hepatic fibrosis [11] and acute hepatitis E exposure [12] leading to the same.

In most of the reported cases, episodes of overt hepatic encephalopathy have preceded the development of the myelopathy; it has therefore been postulated that nitrogenous products such as ammonia, fatty acids, indoles and mercaptans, bypassing the liver through the portocaval shunt, play an important role. These nitrogenous products cause injury to the axon cylinder, neuronal cell bodies and myelin. Another explanation is the deficiency of essential nutrients to the CNS secondary to the alteration of the hepatic metabolism. [13],[14],[15]

Differential diagnoses of HM are amyotrophic lateral sclerosis, multiple sclerosis, paraneoplastic effect, radiation myelopathy, human T-lymphotropic virus-I associated myelopathy and vascular spinal cord disease.

The age of presentation, rapidly developing disability, sparing of upper limbs, no sensory signs, bladder involvement with normal CSF findings and imaging, in a patient of the chronic liver disease with features of hepatic decompensation favored the diagnosis of HM. This case merits attention in being one among the only few case reports of patients hepatitis B related cirrhosis with such long duration of symptoms and episode of hepatic encephalopathy after development of symptoms of myelopathy. Second, he had confounding exposure to grass pea, known locally as khesari dal (L. sativus). However, our patient reported infrequent intake of the legume. The reported toxic dose is about 300 g of the legume per day for a period of 3 months. [16] Third, he did not have any prior episodes of GI bleeding and the terminal episode of lower GI bleed.

 
   References Top

1.Leigh AD, Card WI. Hepato-lenticular degeneration; a case associated with postero-lateral column degeneration. J Neuropathol Exp Neurol 1949;8:338-46.  Back to cited text no. 1
    
2.Zieve L, Mendelson DF, Goepfert M. Shunt encephalomyelopathy. II. Occurrence of permanent myelopathy. Ann Intern Med 1960;53:53-6.  Back to cited text no. 2
    
3.Brown IA. Liver-Brain Relationships. Springfield: Thomas Y Crowell; 1957.  Back to cited text no. 3
    
4.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 Child 2000;83:439-42.  Back to cited text no. 4
    
5.Pant SS, Bhargava AN, Singh MM, Dhanda PC. Myelopathy in hepatic cirrhosis. Br Med J 1963;1:1064-5.  Back to cited text no. 5
    
6.Mendoza G, Marti-Fàbregas J, Kulisevsky J, Escartín A. Hepatic myelopathy: A rare complication of portacaval shunt. Eur Neurol 1994;34:209-12.  Back to cited text no. 6
    
7.Panicker J, Sinha S, Taly AB, Ravishankar S, Arunodaya GR. Hepatic myelopathy: A rare complication following extrahepatic portal vein occlusion and lienorenal shunt. Neurol India 2006;54:298-300.  Back to cited text no. 7
[PUBMED]  Medknow Journal  
8.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. 8
    
9.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. 9
    
10.Nardone R, Buratti T, Oliviero A, Lochmann A, Tezzon F. Corticospinal involvement in patients with a portosystemic shunt due to liver cirrhosis: A MEP study. J Neurol 2006;253:81-5.  Back to cited text no. 10
    
11.Demirci M, Tan E, Elibol B, Gedikoðlu G, Saribaþ O. Spastic paraparesis associated with portal-systemic venous shunting due to congenital hepatic fibrosis. Neurology 1992;42:983-5.  Back to cited text no. 11
    
12.Mandal K, Chopra N. Acute transverse myelitis following hepatitis E virus infection. Indian Pediatr 2006;43:365-6.  Back to cited text no. 12
    
13.Kincaid JC. Myelitis and myelopathy. In: Joynt RJ, editor. Clinical Neurology, Vol. 3. Philadelphia: JB Lippincott Company; 1992. p. 1-36.  Back to cited text no. 13
    
14.Lefer LG, Vogel FS. Encephalomyelopathy with hepatic cirrhosis following portosystemic venous shunts. Arch Pathol 1972;93:91-7.  Back to cited text no. 14
    
15.Sriram K, Shankar SK, Boyd MR, Ravindranath V. Thiol oxidation and loss of mitochondrial complex I precede excitatory amino acid-mediated neurodegeneration. J Neurosci 1998;18:10287-96.  Back to cited text no. 15
    
16.Ravindranath V. Neurolathyrism: Mitochondrial dysfunction in excitotoxicity mediated by L-beta-oxalyl aminoalanine. Neurochem Int 2002;40:505-9.  Back to cited text no. 16
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
    Tables

  [Table 1]


This article has been cited by
1 Partial splenic artery embolization for severe hepatic myelopathy in cirrhosis
Cyriac Abby Philips,Lijesh Kumar,Philip Augustine
Hepatology. 2018;
[Pubmed] | [DOI]



 

Top
 
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this article
    Abstract
   Introduction
   Case Report
   Discussion
    References
    Article Figures
    Article Tables

 Article Access Statistics
    Viewed2764    
    Printed63    
    Emailed0    
    PDF Downloaded110    
    Comments [Add]    
    Cited by others 1    

Recommend this journal