Presentation on theme: "Neuroradiology–Neuropathology Conference University of North Carolina Hospitals Michael Solle MD, Neuroradiology Thomas W. Bouldin MD, Neuropathology."— Presentation transcript:
1 Neuroradiology–Neuropathology Conference University of North Carolina Hospitals Michael Solle MD, Neuroradiology Thomas W. Bouldin MD, Neuropathology February, 2011
2 Case 1History48-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 SurgeryT2 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 keyIntramedullaryExtramedullary, intraduralExtraduralIdentifying the CSF containing space, the dura and the epidural fat help to localize the abnormality.
8 Case 2History30-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 historyIn 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 SurgeryImage-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 encephalopathyvisual loss secondary to branch retinal artery occlusionsensorineural hearing loss Answer: Susac’s SyndromeCaused by microangiopathy involving the brain, cochlea, and retina
18 Susac’s SyndromeHeadache, often severe and sometimes migrainous, is an almost constant complaintmay be the major presenting feature of the encephalopathycognitive changes, confusion, and memory and psychiatric disturbancesMR often interpreted as "typical" for MS or ADEMIn unexplained encephalopathy, a neuro- ophthalmologist or retinal specialist should perform a dilated funduscopic examination…Susac’s SyndromeJohn O. Susac, Guest Editorialist, Winter Haven, FL
19 Susac’s syndrome (continued) Clinical course is usually self-limited, variable.2–4 yearsas short as 6 months or as long as 5 years Some patients recover with little residual disease; others are profoundly impaired.cognitive deficitsgait disturbancehearing lossVision is not usually seriously impaired.Susac’s SyndromeJohn 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 steroidsin conjunction with cyclophosphamide or immunoglobulinSome respond to monotherapy with steroids, cyclophosphamide, or immunoglobulin. Anticoagulation has no role.Susac’s SyndromeJohn O. Susac, Guest Editorialist, Winter Haven, FL
21 Case 3History20-year-old woman with 2–3 year history of complex partial seizures.
30 SurgerySuboccipital/posterior fossa craniectomy for excision of left cerebellar infarct and duraplasty.Placement of a right frontal external ventricular drain.
31 PathologyPurkinje cells (arrow) with eosinophilic cytoplasm and pyknotic nuclei (red neurons)Widespread nuclear pyknosis in neurons of the internal granular layerOnly a few inflammatory cellsThe histologic changes are consistent with an acute cerebellar infarct.
32 Cerebellar Infarction PICA > superior cerebellar > AICAEtiology often:dissection of the vertebral arteries (particularly in younger patients)basilar artery disease (in older patients).Cerebellar infarcts may lead rapidly to deathcaused by compression of the brain stemacute obstructive hydrocephalus from compression of 4th ventricleHerniationsUpward 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 5History20-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 SurgeryLarge 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 PathologySpindle 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” schwannomaBenign tumor arising from Schwann cells of the vestibulocochlear nerveLarge tumorsAvidly enhancing mass, with expansion of the porus acusticus, and an "ice cream on cone“ appearance when the mass is both within the CPA and IACSmall tumorsHigh-resolution T2 MR: small low signal “filling defect" in high signal CSFMost common presentation is an adult with unilateral sensorineural hearing lossIf bilateral, consider NF-2 as underlying diagnosisImportant to comment on involvement of cochlear aperture
38 DDx of cerebellopontine angle mass Vestibular schwannomaMost common CPA massEpidermoid cystmimic rare cystic schwannoma, no enhancementFLAIR/CISS/DWI to differentiate from arachnoid cystArachnoid cystfollows CSF on all MR sequences; no enhancementMeningiomadural tail; eccentric to porus acusticus without widening itFacial nerve schwannomaLook for labyrinthine segment "tail" to differentiateMetastasisLymphomaAneurysm