1. Neuroradiology–Neuropathology Conference University of North Carolina Hospitals Michael Solle MD, Neuroradiology Thomas W. Bouldin MD, Neuropathology February, 2011

2. Case 1
History
48-year-old woman with history of end-stage renal disease secondary to lupus, on hemodialysis, who presented with new acute onset of left lower extremity weakness and difficulty walking after dialysis.

3. Imaging MRI of the thoracic spine
Hyperintense signal within the spinal cord extending from T3–T6. This could possibly relate to vasculitis from patient’s lupus.
T2 hypointense/T1 iso- to hyper-intense mass within the spinal canal of the upper thoracic spine with associated cord displacement. This is concerning for an epidural hematoma.
Bilateral pleural effusions.

6. Surgery
T2 through T5 laminectomy for the evacuation of an intradural hematoma.
Preoperative Diagnosis: T3–T5 extramedullary hematoma.
Postoperative Diagnosis: T3–T5 subdural hematoma.
Pathology: acute blood clot.

7. Review of spinal lesions
Location is key
Intramedullary
Extramedullary, intradural
Extradural
Identifying the CSF containing space, the dura and the epidural fat help to localize the abnormality.

8. Case 2
History
30-year-old previously healthy man with three weeks of episodic right-sided numbness and tingling along with a complete right homonymous hemianopsia and difficulty naming objects.

9. Additional history
In 2009 intermittent tinnitus had become a loud humming that would sometimes become so loud that he could not understand what was said to him.
In early 2010, was seen by an ophthalmologist, who noted retinal vasculitis.
In the interval between visits he underwent a cochlear implant without a magnet in order to facilitate continued MRI screening. The implant improved his hearing significantly in his right ear; he remains essentially deaf in his left ear.

14. Surgery
Image-guided left occipital craniotomy for open biopsy of enhancing left occipital lobe lesion.

15. 
Biopsy of occipital lesion revealed nonspecific brain necrosis () and reactive gliosis in surrounding brain. The histologic changes are compatible with an infarct.

16. Biopsy also revealed atypical lymphoid infiltrates around blood vessels in area of brain necrosis. The significance of these lymphoid infiltrates is not known.

17. Name that Eponym? Consisting of classic clinical triad of
subacute encephalopathy
visual loss secondary to branch retinal artery occlusion
sensorineural hearing loss 
Answer: Susac’s Syndrome
Caused by microangiopathy involving the brain, cochlea, and retina

18. Susac’s Syndrome
Headache, often severe and sometimes migrainous, is an almost constant complaint
may be the major presenting feature of the encephalopathy
cognitive changes, confusion, and memory and psychiatric disturbances
MR often interpreted as "typical" for MS or ADEM
In unexplained encephalopathy, a neuro- ophthalmologist or retinal specialist should perform a dilated funduscopic examination…
Susac’s Syndrome
John O. Susac, Guest Editorialist, Winter Haven, FL

19. Susac’s syndrome (continued)
Clinical course is usually self-limited, variable.
2–4 years
as short as 6 months or as long as 5 years 
Some patients recover with little residual disease; others are profoundly impaired.
cognitive deficits
gait disturbance
hearing loss
Vision is not usually seriously impaired.
Susac’s Syndrome
John O. Susac, Guest Editorialist, Winter Haven, FL

20. Susac’s syndrome (continued)
Pathogenesis is unknown.
Difficult to evaluate the results of treatment:
IV methylprednisolone followed by oral steroids
in conjunction with cyclophosphamide or immunoglobulin
Some respond to monotherapy with steroids, cyclophosphamide, or immunoglobulin. 
Anticoagulation has no role.
Susac’s Syndrome
John O. Susac, Guest Editorialist, Winter Haven, FL

21. Case 3
History
20-year-old woman with 2–3 year history of complex partial seizures.

25. Biopsy of cortical lesion revealed a proliferation of oligodendrocyte-like cells in a myxoid background. A neuron (arrow) appears to “float” within the myxoid matrix.

26. DNET (DNT) Dysembryoplastic neuroepithelial tumor, WHO grade I
Cortical location; temporal, frontal lobe
Commonly present with complex partial seizures
Slow growing—may remodel/scallop inner table of skull
Variable enhancement
May have microcystic appearance

27. Case 4
History
40-year-old woman with hypertension was transferred from an outside hospital ED because of cerebellar infarcts.

30. Surgery
Suboccipital/posterior fossa craniectomy for excision of left cerebellar infarct and duraplasty.
Placement of a right frontal external ventricular drain.

31. Pathology
Purkinje cells (arrow) with eosinophilic cytoplasm and pyknotic nuclei (red neurons)
Widespread nuclear pyknosis in neurons of the internal granular layer
Only a few inflammatory cells
The histologic changes are consistent with an acute cerebellar infarct.

32. Cerebellar Infarction
PICA > superior cerebellar > AICA
Etiology often:
dissection of the vertebral arteries (particularly in younger patients)
basilar artery disease (in older patients).
Cerebellar infarcts may lead rapidly to death
caused by compression of the brain stem
acute obstructive hydrocephalus from compression of 4th ventricle
Herniations
Upward cerebellar herniation (seen as effacement of quadrigeminal plate cistern)
Downward herniation of cerebellar tonsils may also occur.
Emergency resection of involved cerebellum and/or a ventriculostomy may be required as a life-saving measure.

33. Case 5
History
20-year-old man who began to notice significant dysequilibrium on movement, which interfered with his playing of baseball. He noticed that he was not able to track a baseball while moving nor to swing a bat and hit the ball. He had previously been very good at this.

35. Surgery
Large vestibular schwannoma with multiple large vessels contained within it was densely adherent along the brainstem to blood vessels.
Facial nerve was dissected in its entirety and stimulated at 0.1 milliamps at the completion of the case.
There is a small residual pancake-like tumor along brainstem, densely adherent to several large vessels.

36. Pathology
Spindle cell tumor with some nuclear palisading (Verocay bodies).
Compact Antoni type A tissue (upper left of photo) and loose Antoni type B tissue (lower right of photo) are present.
The histologic features are consistent with a schwannoma.

37. Vestibular schwannoma
AKA, “acoustic” schwannoma
Benign tumor arising from Schwann cells of the vestibulocochlear nerve
Large tumors
Avidly enhancing mass, with expansion of the porus acusticus, and an "ice cream on cone“ appearance when the mass is both within the CPA and IAC
Small tumors
High-resolution T2 MR: small low signal “filling defect" in high signal CSF
Most common presentation is an adult with unilateral sensorineural hearing loss
If bilateral, consider NF-2 as underlying diagnosis
Important to comment on involvement of cochlear aperture

38. DDx of cerebellopontine angle mass
Vestibular schwannoma
Most common CPA mass
Epidermoid cyst
mimic rare cystic schwannoma, no enhancement
FLAIR/CISS/DWI to differentiate from arachnoid cyst
Arachnoid cyst
follows CSF on all MR sequences; no enhancement
Meningioma
dural tail; eccentric to porus acusticus without widening it
Facial nerve schwannoma
Look for labyrinthine segment "tail" to differentiate
Metastasis
Lymphoma
Aneurysm