Hepatic Myelopathy with Painful Tonic Spasm: A Case Report


Hepatic myelopathy (HM) is a rare neurological complication of chronic liver disease, causing progressive spastic paraplegia or quadriplegia in the extremities with minimal sensory or sphincter impairment. In this article, the authors present one additional case with HM, with an exceptional clinical picture of painful tonic spasms within both thighs as the main complaint. A reduction in pain coinciding with a reduction in spasticity from treatments such as baclofen suggests a causal relationship between the two. We stress the importance of early recognizing this symptom before the spinal cord damage becomes irreversible.

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Liao, H. , Yan, Z. , Li, G. and Hong, H. (2015) Hepatic Myelopathy with Painful Tonic Spasm: A Case Report. Open Access Library Journal, 2, 1-6. doi: 10.4236/oalib.1102078.

Conflicts of Interest

The authors declare no conflicts of interest.


[1] Victor, M., Adams, R.D. and Cole, M. (1965) The Acquired (Non-Wilsonian) Type of Chronic Hepatocerebral Degeneration. Medicine (Baltimore), 44, 345-396.
[2] Leigh, A.D. and Card, W.I. (1949) Hepato-Lenticular Degeneration: A Case Associated with Postero-Lateral Column Degeneration. Journal of Neuropathology & Experimental Neurology, 8, 338-346.
[3] Ivanhoe, C.B. and Reistetter, T.A. (2004) Spasticity: The Misunderstood Part of the Upper Motor Neuron Syndrome. American Journal of Physical Medicine & Rehabilitation, 83, S3-S9.
[4] Spissu, A., Cannas, A., Ferrigno, P., Pelaghi, A.E. and Spissu, M. (1999) Anatomic Correlates of Painful Tonic Spasms in Multiple Sclerosis. Movement Disorders, 14, 331-335.
[5] Middleton, J.W., Siddall, P.J., Walker, S., Molloy, A.R. and Rutkowski, S.B. (1996) Intrathecal Clonidine and Baclofen in the Management of Spasticity and Neuropathic Pain Following Spinal Cord Injury: A Case Study. Archives of Physical Medicine and Rehabilitation, 77, 824-826.
[6] Kellett, M.W., Young, G.R. and Fletcher, N.A. (1997) Painful Tonic Spasms and Pure Motor Hemiparesis Due to Lacunar Pontine Infarct. Movement Disorders, 12, 1094-1096.
[7] Palace, J. (2001) Making the Diagnosis of Multiple Sclerosis. Journal of Neurology, Neurosurgery & Psychiatry, 71, i3-i8.
[8] Sage, J.I., Van Uitert, R.L. and Lepore, F.E. (1984) Alcoholic Myelopathy without Substantial Liver Disease. A Syndrome of Progressive Dorsal and Lateral Column Dysfunction. Archives of Neurology, 41, 999-1001.
[9] Tsuchiya, K., Watabiki, S., Shiojiri, T., Matsumoto, A. and Tsukagoshi, H. (1993) Alcoholic Cerebellar Degeneration with Pyramidal Sign—In Relation to Alcoholic Myelopathy. No to Shinkei, 45, 169-175.
[10] Anand, B.A., Agarwala, S. and Nundy, S. (1992) Encephalomyelopathy Following Portocaval Shunt in Noncirrhotic Portal Fibrosis: A Case Report. Tropical Gastroenterology, 13, 152-154.
[11] Gauthier, G. and Wildi, E. (1975) Porto-Systemic Encephalo-Myelopathy. Revue Neurologique Société de Neurologie de Paris, 131, 319-338.
[12] Lewis, M.B., Macquillan, G., Bamford, J.M. and Howdle, P.D. (2000) Delayed Myelopathic Presentation of the Acquired Hepatocerebral Degeneration Syndrome. Neurology, 54, 1011.
[13] Tazawa, K.I., Shimojima, Y., Okano, T., Yazaki, M., Takei, Y.I., Shimojo, H., et al. (2007) An Autopsy Case with Adult Onset Type II Citrullinemia Showing Myelopathy. Journal of the Neurological Sciences, 253, 77-80.
[14] Utku, U., Asil, T., Balci, K., Uzunca, I. and Celik, Y. (2005) Hepatic Myelopathy with Spastic Paraparesis. Clinical Neurology and Neurosurgery, 107, 514-516.
[15] Iwamura, K. and Inaba, R. (1983) Clinical Study on Latent Cirrhosis of the Liver. Tokai Journal of Experimental and Clinical Medicine, 8, 281-291.
[16] Sheean, D.G. (2009) Is Spasticity Painful? European Journal of Neurology, 16, 157-158.
[17] Zieve, L., Mendelson, D.F. and Goepfert, M. (1960) Shunt Encephalomyelopathy. II. Occurrence of Permanent Myelopathy. Annals of Internal Medicine, 53, 53-63.
[18] Liversed, L.A. and Rawson, M.D. (1966) Myelopathy in Hepatic Disease and Portosystemic Venous Anastomosis. The Lancet, 287, 277-279.
[19] Mendoza, G., Marti-Fabregas, J., Kulisevsky, J. and Escartin, A. (1994) Hepatic Myelopathy: A Rare Complication of Portacaval Shunt. European Neurology, 34, 209-212.
[20] Campellone, J.V., Lacomis, D., Giuliani, M.J. and Kroboth, F.J. (1996) Hepatic Myelopathy Case Report with Review of the Literature. Clinical Neurology and Neurosurgery, 98, 242-246.
[21] Lebovics, E., Dematteo, R.E., Schaffner, F. and Gendelman, S. (1985) Portal-Systemic Myelopathy after Portacaval Shunt Surgery. Archives of Internal Medicine, 145, 1921-1922.
[22] Counsell, C. and Warlow, C. (1996) Failure of Presumed Hepatic Myelopathy to Improve after Liver Transplantation. Journal of Neurology, Neurosurgery & Psychiatry, 60, 590.
[23] Panicker, J., Sinha, S., Taly, A.B., Ravishankar, S. and Arunodaya, G.R. (2006) Hepatic Myelopathy: A Rare Complication Following Extrahepatic Portal Vein Occlusion and Lienorenal Shunt. Neurology India, 54, 298-300.
[24] Weissenborn, K., Tietge, U.J.F., Bokemeyer, M., Mohammadi, B., Bode, U., Manns, M.P. and Caselitz, M. (2003) Liver Transplantation Improves Hepatic Myelopathy: Evidence by Three Cases. Gastroenterology, 124, 346-351.
[25] Troisi, R., Debruyne, J. and de Hemptinne, B. (1999) Improvement of Hepatic Myelopathy after Liver Transplantation. New England Journal of Medicine, 340, 151.
[26] Qu, B., Liu, C., Guo, L., Yang, Y., Li, J.H., Yu, L. and Lv, Y. (2009) The Role of Liver Transplantation in the Treatment of Hepatic Myelopathy: Case Report with Review of the Literature. Transplantation Proceedings, 41, 1987-1989.
[27] Chou, R., Peterson, K. and Helfand, M. (2004) Comparative Efficacy and Safety of Skeletal Muscle Relaxants for Spasticity and Musculoskeletal Conditions: A Systematic Review. Journal of Pain and Symptom Management, 28, 140-175.
[28] Mueller, M.E., Gruenthal, M., Olson, W.L. and Olson, W.H. (1997) Gabapentin for Relief of Upper Motor Neuron Symptoms in Multiple Sclerosis. Archives of Physical Medicine and Rehabilitation, 78, 521-524.
[29] Wissel, J., Haydn, T., Muller, J., Brenneis, C., Berger, T., Poewe, W. and Schelosky, L.D. (2006) Low Dose Treatment with the Synthetic Cannabinoid Nabilone Significantly Reduces Spasticity-Related Pain: A Double-Blind Placebo-Controlled Cross-Over Trial. Journal of Neurology, 253, 1337-1341.
[30] Wissel, J., Muller, J., Dressnandt, J., Heinen, F., Naumann, M., Topka, H. and Poewe, W. (2000) Management of Spasticity Associated Pain with Botulinum Toxin A. Journal of Pain and Symptom Management, 20, 44-49.

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