Journal of Postgraduate Medicine
 Open access journal indexed with Index Medicus & EMBASE  
     Home | Subscribe | Feedback  

[Download PDF
Year : 2005  |  Volume : 51  |  Issue : 1  |  Page : 43-44  

Primary biliary cirrhosis complicated by transverse myelitis in a patient without Sjögren’s syndrome

V Papadopoulos1, A Micheli1, D Nikiforidis2, Konstantinos Mimidis1,  
1 First Department of Internal Medicine, Democritus University of Thrace, Greece
2 Department of Neurology, University General Hospital of Alexandroupolis, Greece

Correspondence Address:
Konstantinos Mimidis
First Department of Internal Medicine, Democritus University of Thrace


Transverse myelitis is an acute inflammatory process, affecting one or more segments of the spinal cord. Its association with primary biliary cirrhosis has been documented in only four cases – all along with Sjögren’s syndrome. Herein, we report for the first time, a patient who developed recurrent acute transverse myelitis in association with primary biliary cirrhosis without any clinical or histological indication of Sjögren’s syndrome. A 42-year-old woman with primary biliary cirrhosis developed acute onset quadriparesis and urinary retention. Diagnostic evaluation excluded the presence of Sjögren’s syndrome, other autoimmune syndromes, infections and multiple sclerosis. Magnetic resonance imaging of the spinal cord disclosed signal intensity abnormalities from C1 to T2 after gadolinium enhancement. As diagnosis of acute transverse myelitis was prominent, the patient was treated with intravenous methylprednisolone. The patient had a fair outcome despite an early recurrence of the symptoms after treatment withdrawal.

How to cite this article:
Papadopoulos V, Micheli A, Nikiforidis D, Mimidis K. Primary biliary cirrhosis complicated by transverse myelitis in a patient without Sjögren’s syndrome.J Postgrad Med 2005;51:43-44

How to cite this URL:
Papadopoulos V, Micheli A, Nikiforidis D, Mimidis K. Primary biliary cirrhosis complicated by transverse myelitis in a patient without Sjögren’s syndrome. J Postgrad Med [serial online] 2005 [cited 2023 Oct 4 ];51:43-44
Available from:

Full Text

Transverse myelitis (TM) is an acute or subacute focal inflammatory disorder of the spinal cord affecting motor, sensory and autonomic function.[1] Magnetic resonance imaging (MRI) and lumbar puncture usually show evidence of inflammatory process.[2] TM is an uncommon, well-described neurological manifestation of autoimmune diseases. Its association with primary biliary cirrhosis (PBC) has been documented in only four cases - all along with Sjögren's syndrome (SS).[3] Herein, we report the first case of PBC-related TM without any clinical or histological indications of SS.

 Case History

A 42-year-old Caucasian female was admitted to our hospital in December 2002 for severe myalgia, headache, neck stiffness and low grade fever over the last 5 days. Her medical history was significant for PBC since 2001, based upon the presence of anti-mitochondrial antibodies and a liver histology suggestive of PBC Stage II-III: mild chronic portal inflammation with bridging fibrosis, bile duct destruction, focal granulomas without necrosis and bile ductular proliferation. Her medication consisted of ursodeoxycholic acid, cholestyramine and vitamins D and K.

On admission, her blood pressure was 130/80 mmHg, pulse rate 96/min, and body temperature 37.1 oC. On the 7th day of her hospital stay the patient started complaining of low back pain and gradually aggravated weakness and numbness in both the lower extremities. Neurological evaluation revealed motor weakness of both upper and lower extremities with reduced tactile sensation being worse in the right arm and left leg, indicative of level C5 and below. A bilaterally positive Babinski sign was noted. No cognitive or cranial nerves disturbances were noted. During the following four days a gradually aggravated paralysis prevented her from moving the lower extremities. Simultaneously, she developed urinary retention necessitating urinary bladder catheterisation.

Laboratory work-up revealed normal hematocrit, white blood cell counts and platelets and an increased erythrocyte sedimentation rate (23 mm/hr). Subsequent ELISA-based serological tests showed the presence of anti-nuclear (titre 1/160), anti-mitochondrial (titre 1/320) and anti-Ro (SS-A) (titre 5.6 U/ml, normal et al.[4] The autoimmune nature of the TM process combined with the absence of a well-defined autoimmune process other than the underlying PBC, allows us to hypothesize that TM was really PBC-related in this patient.

The prognosis of TM is variable, with about one-third of the patients progressing to a syndrome indistinguishable from spinal muscular atrophy. Our patient's rapid primary response, in spite of the very acute course of the disease, may be well ascribed to the early use of methylprednisolone.[11]


1Linardaki G, Skopouli FN, Koufos C, Moutsopoulos HM. Subclinical multisystemic autoimmunity presenting as a progressive myelopathy. Lupus 1997;6:675-7.
2Transverse Myelitis Consortium Working Group. Proposed diagnostic criteria and nosology of acute transverse myelitis. Neurology 2002;59:499-505.
3Anantharaju A, Baluch M, Van Thiel DH. Transverse myelitis occurring in association with primary biliary cirrhosis and Sjogren's syndrome. Dig Dis Sci 2003;48:830-3.
4Vitali C, Bombardieri S, Moutsopoulos HM, Coll J, Gerli R, Hatron PY, et al. Assessment of the European classification criteria for Sjogren's syndrome in a series of clinically defined cases: results of a prospective multicentre study. The European Study Group on Diagnostic Criteria for Sjogren's Syndrome. Ann Rheum Dis 1996;55:116-21.
5Giobbia M, Carniato A, Scotton PG, Marchiori GC, Vaglia A. Cytomegalovirus-associated transverse myelitis in a nonimmunocompromized patient. Infection 1999;27:228-30.
6Goebels N, Helmchen C, Abele-Horn M, Gasser T, Pfister HW. Extensive myelitis associated with Mycoplasma pneumoniae infection: Magnetic resonance imaging and clinical long-term follow-up. J Neurol 2001;248:204-8.
7Muranjan MN, Deshmukh CT. Acute transverse myelitis due to spinal epidural hematoma - first manifestation of severe hemophilia. Indian Pediatr 1999;36:1151-3.
8Rutan G, Martnez AJ, Fieshko JT, Van Thiel DH. Primary biliary cirrhosis, Sjogren's syndrome, and transverse myelitis. Gastroenterology 1986;90:206-10.
9Jeffery DR, Mandler RN, Davis LE. Transverse myelitis: Retrospective analysis of 33 cases, with differentiation of cases associated multiple sclerosis and parainfectious events. Arch Neurol 1993;50:532-5.
10Hummers LK, Krishnan C, Casciola-Rosen L, Rosen A, Morris S, Mahoney JA, et al. Recurrent transverse myelitis associates with anti-Ro (SSA) autoantibodies. Neurology 2004;62:147-9.
11Zerbini CA, Fidelix TS, Rabello GD. Recovery from transverse myelitis of systemic lupus erythematosus with steroid therapy. J Neurol 1986;233:188-9.

Wednesday, October 4, 2023
 Site Map | Home | Contact Us | Feedback | Copyright  and disclaimer