Disclosures: Michael Lew, DO: No financial relationships or conflicts of interest

Case Diagnosis: Foix-Alajouanine Syndrome

Case Description: A 52-year-old woman presented in September 2021 with burning pain and decreased temperature sensation in her left hand. In October 2021, she developed numbness in her left leg. By January 2022, she noticed herself leaning left when ambulating. Over the next several months, she developed blurry vision, word-finding difficulty, and diminished dexterity and strength in her left hand. In March 2022, an MRI of her cervical/thoracic spine revealed an expansile non-enhancing central intramedullary spinal cord lesion from T1 to T7 with associated hemorrhage and necrosis. She underwent T3-5 laminectomy, with biopsy and excision of the lesion, and T3-T5 laminoplasty. Histologic findings were characteristic for Foix Alajouanine Syndrome (FAS) or Subacute Ascending Necrotizing Myelitis. Her rehabilitation was complicated by worsening neuropathic pain. Gabapentin was titrated up to 800mg three times a day but not until pregabalin was added, at a dose of 50mg/25mg/25mg was she able to tolerate three hours of therapy a day.

Setting: Acute Inpatient Rehabilitation

Assessment/Results: While treatment consists of vascular embolization or excision, the rehabilitation course for FAS is not commonly discussed. We found a key component to the rehabilitation course involved addressing neuropathic pain. Once her pain was controlled, the patient responded well to therapy and regained enough strength and function to be discharged home.

Discussion: FAS is characterized by spinal cord necrosis and large, tortuous, thrombosed veins. While the necrotic myelitis is often attributed to thrombosis, there usually is no angiographic evidence of venous thrombosis or vascular malformation. The necrosis mostly involves the corticospinal tract, sparing anterior horn cells, and is most prominent in the thoracolumbar region.

Conclusion: FAS is a progressive myelopathy that is often misdiagnosed or missed, but once diagnosed, management of neuropathic pain seems to be a key component to successful recovery.

Level of Evidence: Level V

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PM&R Meeting Abstracts - https://pmrjabstracts.org/abstract/pain-management-during-rehabilitation-in-foix-alajouanine-syndrome/