Interesting Spine Cases M. Castillo, MD, FACR University of North Carolina Chapel Hill Case # 1 • 43-year-old male with a chronic history of dysesthesias and hypesthesias in all extremities. He has a chronic disease of which the most important findings are liver failure and decreased vision. Several members of his family had a similar history. Case # 1 Case # 1 Case # 1. Which of the following is/are cause(s) of pial enhancement? • • • • • 1. Metastases 2. Sarcoidosis 3. Amyloidosis 4. Lymphoma 5. All of the above Case # 1. Regarding amyloidosis involving the spinal cord, which is true: 1. It predominantly involves the intramedullary veins 2. It predominantly involves the arteries in the subarachnoid space 3. It never results in cord contrast enhancement 4. It affects heavy myelinated fibers Case # 1. Dx: Amyloidosis involving the pia. Familial amyloid polyneuropathy: – Deposition of amyloid in pial/subarachnoid arteries & arterioles • Results in destruction of the blood-cord-barrier – Sensory nerves & unmyelinated fibers most affected – Precursors of FAP are metabolized by liver, thus liver transplantation is useful » AJNR 2004; 25: 1599 Spine involvement, amyloidosis. CNS Amyloidosis. CNS Amyloidosis. Infiltrative lesion Case # 2 Young patient presenting with a myelopathy 2 years after a ‘stroke’. Case # 2 Case # 2. The following may result in spinal ‘cysts’: • • • • • A. B. C. D. E. Cysticercosis Exophytic syrinxes Post trauma arachnoid tears Post SAH arachnoid cysts All of the above Case # 2. Which is the most likely diagnosis in this patient? • • • • • A. B. C. D. E. Cysticercosis Exophytic syrinxes Post trauma arachnoid cysts Post SAH arachnoid cysts None of the above Case # 2 Case courtesy of W. Kucharczyk, Toronto Case # 2. Dx: Multiple spinal ‘arachnoid’ cysts following aneurysmal SAH. • Cysts may develop after hemorrhage, trauma & inflammation • Pre-existing or de novo? may have hemosiderin • Composed of single layer of meningothelial cells • May produce back pain/myelopathy that may be intermittent (syrinx) Other extramedullary ‘cysts’ ‘Idiopathic’ subarachnoid cyst Other extramedullary ‘cysts’ Extradural cysts Case # 3 Young male with a history of melanoma presents with lower back pain. Case # 3 Case # 3. The most likely diagnosis is related to which category of disease: • • • • • A. B. C. D. E. Metastasis Infection Degenerative disease Congenital None of the above Case # 3. Which is false regarding the abnormality shown here: • A. Trauma is a predisposing factor • B. Disc herniation is a part of it • C. Weakening of the end-plate may be a secondary factor • D. Contrast enhancement may occur • E. Infection plays a role Case # 3. Dx: Acute enhancing Schmorl node. • Pre-requisites: soft end-plate/bone trabeculae – Congenital: nutrient blood vessels – Metabolic diseases, tumors – Scheuermann disease • May appear ‘cystic’ due to: – Intra-nodal hemorrhage, mucous degeneration • Contrast enhancement: granulation tissues • Cause pain before MRI findings, pain disappears by 3 years & node ‘stabilizes” Cystic Schmorl Nodes Giant cystic Schmorl;s nodes. AJR 2001; 176: 969 CASE # 4 11-year-old boy with back pain of 2-months duration. CASE # 4 Case # 4. The most likely diagnosis is: • • • • • A. B. C. D. E. Aneurysmal bone cyst Osteoid osteoma Osteoblastoma Giant cell tumor Fibrous dysplasia Case # 5. Which is false regarding spinal osteoblastoma: • A. If predominantly affects the posterior elements • B. It may occasionally cross intervertebral space • C. It is a benign process • D. It is a lesion found in middle age and older individuals Case # 4. Dx: Osteoblastoma. • Rare tumor (0.5-2%) comprised of osteoid, primitive woven bone amidst fibrovascular connective tissues • Chronic pain, salicylates not helpful • Sclerotic or lucent lesion, 25% have aggressive features • Choice of Tx: en bloc resection, curettage with bone packing, XRT for malignant ones Case # 5 A 10-year-old child with a longstanding right hemiparesis now with progressive left lower extremity weakness. Case courtesy H. Alvarez, Paris Case # 5. The findings shown are due to: • • • • 1. Spinal AVM 2. Hematomyelia 3. Spinal AVF 4. Spinal cavernous malformation Case # 5. Imaging of the brain in this patient may show: • • • • • 1. old infarctions 2. one or more AVM’s 3. Wallerian degeneration 4. hemiatrophy 5. all of the above Case # 6. The diagnosis in this patient is: • 1. moyamoya disease • 2. spinal arterial malformation syndrome (SAMS) • 3. Rendu-Osler-Weber disease • 4. Von Hippel Lindau disease Case # 5. Dx: ROW. • Abnormalities in chromosomes 9 & 12 • Defect in synthesis of ‘endoglin’ which is needed for growth/remodelling of capillaries • > common in males • Multiple skin/mucosa telangiectasias Case # 6. A 30-year-old male presents with a subacute onset of a myelopathy. Case # 6. The differential diagnosis in this case includes: • • • • • 1. Multiple sclerosis 2. Acute disseminated encephalomyelitis 3. Vasculitis 4. Sarcoidosis 5. All of the above Case # 6. Primary Angiitis of the CNS. • Spinal cord vasculitis: idiopathic, associated with Hodgkin, thyroiditis, drug allergy, Sjogren, viral-induced, hepatitis • Perivascular (artery & vein) infiltration by lymphocytes, cavitation, pial inflammation • Prognosis is very poor, some temporary symptom relief with steroids, necrosis of spinal cord CASE # 7 45-year-old man with a chronic disorder now with a cauda equina syndrome. Courtesy M, Thurnher, Vienna Case # 7. The most likely diagnosis is: • A. Neurofibromatosis I with dural ectasia • B. Marfan syndrome with dural ectasia • C. Ankylosing spondylitis with erosive dural ectasia • D. Epidermoid with bone scalloping Case # 7.All but one of the following are complications of ankylosing spondylitis: • A. “Banana” type fractures • B. Erosive dural ectasia w/cauda equina syndrome • C. Epidural hematomas • D. Infectious diskitis/osteomyelitis • E. Non-infectious diskitis/osteomyelitis (amyloidosis?) Case # 7.Ankylosing Spondylitis, Newer Concepts • B27 gene: – 95% of Europeans, only 25% in Middle East – Antiviral properties high in American Indians who survived European viruses during conquest • Bowel infection is a predisposing factor – Antibodies with cross reaction to joints • Spine disease is not improved with antiinflammatory drugs or methotrexate, need blockers of TNF