International Journal of Nutrition, Pharmacology, Neurological Diseases

: 2013  |  Volume : 3  |  Issue : 4  |  Page : 392--395

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

Vivekanand Satyawali, Yatendra Singh, Mohammad Khalil, Jainendra Kumar 
 Department of Medicine, Government Medical College, Haldwani, Uttarakhand, India

Correspondence Address:
Vivekanand Satyawali
Type 4 Block, k2, Government Medical College Campus, Haldwani - 263 139, Uttarakhand


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.

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;3:392-395

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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 20 ];3:392-395
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Full Text


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

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}

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}{Figure 2}{Figure 3}{Figure 4}

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.


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.


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