Abstract Details

Title Acute transverse myelitis revealing primary biliary cirrhosis

Topic Autoimmune Neurology

Presentation(s) P1 - Poster Session 1 (5:30 PM-6:30 PM)

Poster/Presentation
Number 15-052

Objective

Not applicable

Background Primary biliary cirrhosis is a chronic autoimmune liver disease. More common in women than in men, it is characterized by biological cholestasis and positive anti-mitochondrial antibodies. It can be associated with extrahepatic manifestations. However, neurologic complications remain rare, affecting mainly the central nervous system. Patients can develop impaired concentration and memory, disturbed sleep pattern, and autonomic dysfunction. There is no correlation between the severity of the disease and neurological manifestations. We report a rare case of an acute transverse myelitis, not associated with Sjogren’s syndrome, revealing a primary biliary cirrhosis.

Design/Methods

Not applicable

Results A 68-year-old woman with no medical comorbidities presented with a subacute weakness of the lower limbs. The initial examination showed an anicteric patient, with a left-side predominant paraparesis, brisk reflexes and a left T4 sensory level. Cerebrospinal fluid (CSF) analysis showed mild elevated proteins (0,49 g/l). Magnetic resonance imaging (MRI) of the spine showed an extensive transverse myelitis from T3 to T11. Gadolinium contrast-enhanced MRI demonstrated mild enhancing lesions. Liver enzyme tests revealed an isolated biological cholestasis. Abdominal ultrasound was normal. Antimitochondrial antibodies were highly positive. Anti-SSA and anti SSB auto-antibodies were negative. The patient had no history of mouth or eyes dryness. She received systemic corticosteroids then we maintained her on oral prednisone with an improvement of the symptomatology.

Conclusions Neurologic complications of PBC have been rarely reported in literature. Exceptional cases of chronic aseptic meningitis and progressive multifocal leukoencephalopathy were reported. Studies have shown association between primary biliary cirrhosis and Sjogren’s syndrome. Our patient had no sicca symptoms and immunologic tests for Sjogren’s syndrome were negative. The diagnosis of PBC should be considered in patient with myelitis and biological cholestasis. This is, as far as we know, the first case reported of a transverse myelitis associated with PBC.

Authors/Disclosures
PRESENTER	No disclosure on file
	No disclosure on file
	No disclosure on file
	No disclosure on file
Samir Belal, MD	No disclosure on file
Jihene B. Sassi (Residence La Jarkin)	Ms. Sassi has nothing to disclose.
Faycal Hentati, MD	No disclosure on file

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