Spinal seeding of a pilocytic astrocytoma in an adult, initially diagnosed 18 years previously.

Pilocytic astrocytoma (PA) is a slow-growing, well-circumscribed grade I glioma generally considered benign, with a low recurrence rate and an excellent prognosis following complete surgical resection. PA is the most common central nervous system glioma in the pediatric population and is rare in adults. We report a 26-year-old male with an intradural extramedullary PA at the thoracolumbar junction following subtotal cerebellar PA resection 18 years previously. Fifteen months after spinal PA resection, the patient is doing well, has regained the ability to stand independently, and has no evidence of any new or enlarging lesions. To our knowledge, this is the longest time reported from initial tumor resection of leptomeningeal dissemination to the distal spinal cord. PA patients with subtotal resection may benefit from continued follow-up for up to 20 years after the initial diagnosis and resection.

L5 vertebral osteomyelitis treated with L5 corpectomy and anterior instrumentation: case report.

STUDY DESIGN: A case report. OBJECTIVE: Pyogenic osteomyelitis is the most common form of vertebral infection and typically resolves following conservative treatment with antibiotics administered long term and immobilization. In cases of spinal instability, severe neurological deficit or disease refractory to medical management, neurosurgical intervention is warranted. Historically, these patients have undergone radical vertebral debridement and grafting with or without posterior instrumentation. We report the case of a 46-year-old female intravenous drug user presenting with L5 pyogenic osteomyelitis with L5 vertebral compression and cortex retropulsion following L2-L4 laminectomy for epidural abscess 8 weeks prior. METHODS: The patient underwent an anterior approach single-stage L5 corpectomy, L4/5 and L5/S1 discectomies, expandable titanium-cage insertion and anterior plating from L4 to the sacrum. RESULTS: The patient recovered without any complications. The infection was successfully eradicated and her fusion remains solid 18 months postoperatively. CONCLUSIONS: To our knowledge, this is the first case of L5 vertebral osteomyelitis treated with a single-stage corpectomy and anterior instrumentation.

Surgical treatment of multiple spine metastases from gastrinoma.

STUDY DESIGN: Case report. CLINICAL QUESTION: To report successful surgical therapy for spinal cord compression in a patient with spinal metastases from a pancreatic gastrinoma. METHODS: A 43-year-old man presented three times within 4 years with cervical and upper thoracic spinal cord compression because of metastatic gastrinoma. He had two previous spine metastases to the lower thoracic and lumbar spine, a T11 compressive lesion which required a T9L1 fusion, and an L4 lesion that was treated with chemotherapy and stereotactic radiation. The compression was relieved each time by surgery. RESULTS: The patient underwent three surgeries in 4 years: (1) debulking and removal of the rib head on the left at T3, and debulking of the tumor at T3 with hemilaminectomy and spinal cord decompression with internal fixation from T1-T5 using posterolateral instrumented fusion and allograft; (2) anterior C7 corpectomy with placement of a cage from C7-T1 with both anterior and posterior fusion of C2C7; and (3) T1-T3 laminectomy, T1-T3 exploration of wound, revision of hardware, T1-T3 removal of spinal tumor, and T3 bilateral transpedicular circumferential decompression. The patient is alive and regained the ability to walk 8 years after initial diagnosis, despite the appearance of spinal metastases 1 year after the diagnosis of liver metastases. CONCLUSION: Surgery for spinal cord compression in patients with metastatic neuroendocrine tumors can be effective in relieving radicular pain, weakness and numbness, and while not curative can greatly improve quality of life.

ATP7A (Menkes protein) functions in axonal targeting and synaptogenesis.

Menkes disease (MD) is a neurodegenerative disorder caused by mutations in the copper transporter, ATP7A, a P-type ATPase. We previously used the olfactory system to demonstrate that ATP7A expression is developmentally, not constitutive, regulated, peaking during synaptogenesis when it is highly expressed in extending axons in a copper-independent manner. Although not known to be associated with axonal functions, we explored the possibility that the inability of mutant ATP7A to support axon outgrowth contributes to the neurodegeneration seen in MD. In vivo analysis of the olfactory system in mottled brindled (Atp7aMobr) mice, a rodent model for MD, demonstrates that ATP7A deficiency affects olfactory sensory neuron (OSN) maturation. Disrupted OSN axonal projections and mitral/tufted cell dendritic growth lead to altered synapse integrity and glomerular disorganization in the olfactory bulbs of Atp7aMobr mice. Our data indicate that the neuronal abnormalities observed in MD are a result of specific age-dependent developmental defects. This study demonstrates a role for ATP7A and/or copper in axon outgrowth and synaptogenesis, and will further help identify the cause of the neuropathology that characterizes MD.