Intradural calcium pyrophosphate dihydrate deposition disease: case report.

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.

Multifocal intradural extramedullary anaplastic ependymoma of the spine.

Ependymomas are neuroectodermal tumours arising from the ependymal lining of the ventricles and central canal of the spinal cord. Intradural extramedullary (IDEM) ependymomas which are multifocal, and/or anaplastic (WHO grade III) at presentation are exceedingly rare. We present the second case of multifocal anaplastic IDEM ependymoma in the literature. A 47-year old female presented with left gluteal and thigh pain radiating to the groin associated with paraesthesiae. She had a normal neurological examination. Magnetic resonance imaging of the lumbar spine and subsequent magnetic resonance imaging (MRI) of the remaining neuroaxis demonstrated >10 lesions throughout cervical, thoracic and lumbosacral levels. There were no intracranial lesions. The patient initially underwent surgery for removal of three symptomatic lesions at S2. She recovered well. One year later she had further surgery for three progressing lesions at T5. Four of six lesions were WHO grade III. Two smaller nodules at T5 were WHO grade II. The patient had mild sensory disturbance over the right side of the trunk which resolved postoperatively. There were no long-term sequelae. The patient subsequently underwent full craniospinal irradiation using proton beam therapy. Due to their rarity, there are no guidelines for the management of multifocal IDEM ependymoma. The only previously published case of multifocal anaplastic IDEM ependymoma by Schuurmans et al. involved surgical resection and 20 cycles of whole-spine radiotherapy. Schuurmans patient unfortunately died two years post-diagnosis with progressive cranial metastases and post-radiation myelopathy. In our case, all remaining lesions are stable and she is neurologically intact at 48-month follow up.

Accuracy of percentage of signal intensity recovery and relative cerebral blood volume derived from dynamic susceptibility-weighted, contrast-enhanced MRI in the preoperative diagnosis of cerebral tumours.

Conventional magnetic resonance imaging (MRI) is the technique of choice for diagnosis of cerebral tumours, and has become an increasingly powerful tool for their evaluation; however, the diagnosis of common contrast-enhancing lesions can be challenging, as it is sometimes impossible to differentiate them using conventional imaging. Histopathological analysis of biopsy specimens is the gold standard for diagnosis; however, there are significant risks associated with the invasive procedure and definitive diagnosis is not always achieved. Early accurate diagnosis is important, as management differs accordingly. Advanced MRI techniques have increasing utility for aiding diagnosis in a variety of clinical scenarios. Dynamic susceptibility-weighted contrast-enhanced (DSC) MRI is a perfusion imaging technique and a potentially important tool for the characterisation of cerebral tumours. The percentage of signal intensity recovery (PSR) and relative cerebral blood volume (rCBV) derived from DSC MRI provide information about tumour capillary permeability and neoangiogenesis, which can be used to characterise tumour type and grade, and distinguish tumour recurrence from treatment-related effects. Therefore, PSR and rCBV potentially represent a non-invasive means of diagnosis; however, the clinical utility of these parameters has yet to be established. We present a review of the literature to date.