Adhesive Arachnoiditis in Mixed Connective Tissue Disease: A Rare Neurological Manifestation
Maria Usman Khan1,3, Joseph Devlin1, Alexander Fraser1,3
1. Department of Rheumatology, University Hospital Limerick, Limerick, Ireland 2. Graduate Entry Medical School, University of Limerick, Limerick, Ireland
Mixed Connective tissue disease (MCTD) has a wide spectrum of clinical manifestations. Adhesive arachnoiditis (AA) is a rare disorder, characterized by inflammation of the arachnoid layer of the meninges that leads to fibrosis, resulting in nerve roots adhesion to the Dural sac or to each other (clumping). The usual symptoms of arachnoiditis are severe back pain, paraesthesia, lower limb weakness and dissociative sensory loss. It is diagnosed on clinical grounds and supportive MRI findings.
We present to our knowledge the first case of AA in a MCTD patient that resulted in myelo-radiculopathic symptoms leading to significant neurological comprise.
A 33-year-old female with 7-years history of MCTD presented with progressive worsening of low back pain radiating to right leg associated with pins and needles, 2-3 episodes of fecal incontinence, poor balance, perineal and perianal numbness and altered sensations in both legs. She had no major neurological symptoms in last few years and her MCTD was treated with immunosuppressants (Hydroxy chloroquine / Azathioprine / mycophenolate mofetil), oral steroids and Aspirin over the course of her disease. Clinically she had restricted right SLR test, absent right knee jerk, diminished bilateral ankle jerks, reduced pin-prick sensation in both legs extending up to T10 level with no sacral sparing. Her Romberg’s test was positive. Laboratory tests showed raised ESR (46mm/h, N=5-12), persistently low C4, positive ANA (titre1/1600) and anti U1RNP. This time her serology was positive for anti dsDNA antibody (EIA= 268, N=0-100), anti ribosomal P-protein and negative for anti cardiolipin IgG and anti-Beta-2 glycoprotein IgG antibody. At this stage she clearly had MTCD with Lupus overlap. On her urgent MRI lumbar-spine, cauda equina syndrome was ruled out. Her immunosuppressants (azathioprine and prednisolone) were up-titrated and she received two fluoroscopic guided caudal epidural injections with partial improvement in backache. Her CSF analysis was normal & her lower limb neurophysiologic studies did not report evidence of radiculopathy or sensory motor polyneuropathy.
Her non-contrast CT scan of thoraco-lumbar spine showed thoracic spinal cord arachnoid calcification and raised concern for chronic adhesive arachnoiditis. It was further evaluated with MRI thoraco-lumbar spine which reported abnormally distributed lower lumbar spine and thecal sac nerve roots demonstrating clumping and augmented the existing concern for chronic arachnoiditis. On discussion with neuroradiology, it was decided that she likely had adhesive arachnoiditis related to her connective tissue disorder which possibly started at the time when she initially presented with backache and had progressed since then possibly due to scarring.
While she was on azathioprine, rituximab was added aimed at her neurological complaints which successfully stopped progression of her symptoms.
18-month follow-up showed progressive improvement in her neurological symptoms with no recurrence. Her backache responded to analgesics.
Adhesive arachnoidis could be considered in MCTD patient presenting with myelo-radiculopathic symptoms.