Monday, July 14, 2008

Patient with Lyme and Guillan Barre

Guianne Barre syndrome acquired notoriety in 1976 when it was associated with the Swine flu vaccine that year. It is a rare neurological syndrome. It is a scary disorder. Patients have a progressive paralysis of the lower extremities and it "ascends" up the trunk. At times it can involve the respiratory muscles requiring the use of a ventilator. Usually it is a post infectious disease and improves spontaneously over time. It is due to an acute demylinating peripheral neuropathy which is acute and sever. It is probably autoimmune in nature. Circulating antibodies or antibody/antigen complexes attack the nervous system causing a rapid loss of the protective myelin sheath of peripheral nerves. Patients have a flaccid paralysis with a loss of deep tendon reflexes. This distinguishes the disease from a motor neuron disease such as ALS or MS. A large number of viral infections as well as bacterial infections have been associated with the disease. Normal treatment is intravenous Ig (immunoglobulin g) derived from a large pool of patient sera, or plasmapherisis, a filtering of the blood which removes immune complexes triggering the disease. The disease is self limited and improves over time. In this case a middle aged gentleman in good health presented with an acute flu like illness associated with fever. Over a period of a week he developed progressive weakness and was diagnosed with Guillan Barre. The spinal fluid showed inflammation and was positive by PCR for Lyme. Note here that it is unusual to get a positive Lyme PCR of any fluid. Lyme antibodies, either C6 peptide or Western Blots are more likely to be positive. Non Lyme literate doctors will not order these tests. This patient was brought to my office after hospital discharge because the family found that the other treating physicians were mystified by the case. Lyme is a factor here but I wonder if there was not another inciting viral infection as well. Currently the patient is being treated. Unfortunately a culture of the spinal fluid revealed an unusual fungus. I am sure this is a red herring, a false positive contaminant. His physicians have placed him on a powerfull and toxic antifungal drug which worries me. I have spoken with a neurologist who is bright and familiar with the causes of this disorder. The patient had an allergic reaction to IV Ig (herx?) and will be treated with plasmapherisis as well as with antibiotics. This case is interesting because it shows one of the myriad and unpredictable Lyme associated neurological syndromes. I all neurological syndromes of the peripheral nerves, brain or motor nerves, Lyme needs always be considered as a possible cause.

1 comment:

dogdoc said...

What was the unusual fungus? Don't know it you'll get this on such an old post.