Intradural calcium pyrophosphate dihydrate deposition disease: case report

Abstract

Calcium pyrophosphate dihydrate (CPPD) crystal deposition disease, also known as pseudogout, is a crystalline arthropathy that usually affects large joints and periarticular tissue. Spinal involvement is rare and is usually limited to extradural articular and periarticular structures. Only one case of intradural disease has been previously reported. The authors report the second known case of intradural CPPD deposition disease. An 81-year-old man presented with an 8-week history of urinary and fecal incontinence on the background of long-standing back pain, lower-limb paresthesia, and a known L1 calcified intradural extramedullary mass. Slow growth of the L1 lesion had been documented over several decades on serial CT and MRI. A T12–L2 laminectomy and gross-total resection of the mass was performed. Histopathology demonstrated polarizing rhomboid-shaped crystals consistent with CPPD deposition disease. The patient had significant improvement in bowel and bladder function 6 months postoperatively and made a full recovery. The pathophysiology of intradural involvement remains uncertain. Further case series are required to clarify the true incidence and prognosis of the condition.

Pseudogout, an unconventional imitator of the lumbosacral spine with associated chronic epidural hematoma: illustrative cases

“… 9 To that point, recent literature suggests that CPPD disease of the spine is not limited to the extramedullary space, bone, and surrounding tissues. Gewolb et al 10 and Chakravorty et al 11 demonstrated an intramedullary lesion positive for CPPD disease, an extremely rare presentation, which further adds to the atypical presentations of this disease.…”

“…Many descriptions of the lesions involve either radiographic evidence of calcification or gross pathology description of yellow turbid fluid or chalky white appearance, but these findings are not specific for CPPD disease. [1][2][3][4][5][6][7][8][9][10][11] As evidenced by the various presentations throughout the literature, there is no single pathognomonic finding except for visualization of positively birefringent rhomboid crystals on polarized microscopy. Therefore, polarized light analysis should become more commonplace in spinal pathology.…”

Crystal Diseases of the Spine

“…Intradural extramedullary CPPD presenting as an intradural soft tissue mass compressing the cauda equina nerve roots has been exceptionally reported. 37 38 Although CT may demonstrate the calcified nature of the mass and MRI a T1 and T2 hypointensity with minimal peripheral contrast enhancement, the diagnosis is usually made by the histologic analysis of the lesion. CPPD of the filum terminale was also reported.…”