1137 - A Case of Froin Syndrome in a Spinal Cord Injury Patient Treated with Intrathecal Baclofen

Froin Syndrome


Case Description: A 38-year-old male with a T10 ASIA A spinal cord injury (SCI) due to gunshot wound, complicated by spastic paraplegia, developed worsening back pain and spasticity despite several increases in his intrathecal baclofen (ITB) pump rate. A catheter access procedure was performed to assess intrathecal catheter patency, during which 3 ml of cerebrospinal fluid (CSF) was aspirated from the catheter port. The CSF was notably xanthrochromic and analysis revealed a markedly elevated protein level of 2,334 mg/dL, with no growth on culture. CT imaging confirmed bone and bullet fragments in his spinal canal at T9-10 through mid T11 level and moderate to severe canal stenosis at T10 superior endplate level due to bone deformities. Froin syndrome (FS) was suspected and subsequently confirmed by neuro-immunology. The patient was not deemed a candidate for surgical treatment.  He experienced improvement in spasticity following a further increase in his ITB pump rate.

Discussions:

FS is characterized by CSF xanthochromia, elevated protein and hypercoagulability. It is believed to result from obstruction of normal CSF flow due to processes such as neoplasia, non-malignant mechanical compression, or meningeal infection, leading to stagnant CSF. Clinical manifestations vary depending on the underlying cause, but may include paralysis or paresis, back pain, altered mental status, and headache. Although FS is a rare entity, it is crucial for clinicians to recognize it and address the underlying pathology, as it can potentially have fatal consequences.


Conclusions: In summary, this unique case highlights a rare complication in a patient with a remote SCI who presented with worsening spasticity despite intrathecal baclofen therapy and xanthochromic CSF. FS should be considered in patients on intrathecal baclofen therapy who exhibit a poor or inadequate clinical response, although the effect of FS on ITB therapy effectiveness needs to be further studied.