Interesting Spine Cases M. Castillo, MD, FACR University of North Carolina Chapel Hill.

Interesting Spine CasesInteresting 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 intrame-

dullary veins

2. It predominantly involves the arteries in

the subarachnoid space

3. It never results in cord contrast enhance-

ment

4. It affects heavy myelinated fibers

Case # 1. Dx: Amyloidosis involving Amyloidosis involving the piathe 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. Cysticercosis

• B. Exophytic syrinxes

• C. Post trauma arachnoid tears

• D. Post SAH arachnoid cysts

• E. All of the above

Case # 2. Which is the most likely diagnosis in this patient?

• A. Cysticercosis

• B. Exophytic syrinxes

• C. Post trauma arachnoid cysts

• D. Post SAH arachnoid cysts

• E. None of the above

Case # 2

Case courtesy of W. Kucharczyk, Toronto

Case # 2. Dx: Multiple spinal Multiple spinal ‘arachnoid’ cysts following aneurysmal ‘arachnoid’ cysts following aneurysmal

SAH.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. Metastasis

• B. Infection

• C. Degenerative disease

• D. Congenital

• E. 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 Acute enhancing Schmorl node.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

11-year-old boy with back pain of 2-months duration.

CASE # 4

CASE # 4

Case # 4. The most likely diagnosis is:

• A. Aneurysmal bone cyst

• B. Osteoid osteoma

• C. Osteoblastoma

• D. Giant cell tumor

• E. 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.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.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 Primary Angiitis of the CNS.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

45-year-old man with a chronic disorder now with a cauda equina syndrome.

CASE # 7

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 anti-inflammatory drugs or methotrexate, need blockers of TNF