與大師對話

黃國茂主任

臺北醫學大學附設醫院

影像醫學部

新光醫院放射診斷科

2017.06.20 臺北醫學大學附設醫院 影像醫學部 R2莊凱壹醫師/VS謝立群醫師

規則

依照臨床時序，請大師模擬一線放射科醫師；於未知診斷，或者有限度臨床線索之情形下，進行閱片及解讀。

鑑別診斷為主要，確定診斷為次要。

目的在於學習大師之影像判讀邏輯思考。

Focus on neuro images

大師評論本院影像品質建議及改進。

Protocols, techniques, etc.

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CASE 1

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Patient Profile

56 years old, female

Chief complaint

Rapidly progressive cognitive decline in recent 5 months

Poor memory and dysnomia, unsteady gait, visual blurring, and personality change

出門走不到目的地，不會搭公車，不會去廁所上廁所，存款密碼忘記，把乳液當牙膏，穿內衣出門

Past history

Cystic ovarian tumor

Left ductal carcinoma in situ

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Image

2017/04/05 MRI

2017/05/22 MRI

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Clinical Course

Underwent lumbar puncture

CSF: 14-3-3 protein positive

Diagnosis: Creutzfeldt-Jakob Disease

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Creutzfeldt-Jakob Disease

A spongiform encephalopathy that results in a rapidly progressive dementia and other non-specific neurological features and death usually within a year or less from onset

Clinical symptoms

Progressive dementia associated with myoclonic jerks and akinetic mutism

Variable constellation of pyramidal, extrapyramidal, and cerebellar signs

CSF protein biomarkers: 14-3-3 protein, total tau (t-tau) and neuron-specific enolase (NSE)

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Creutzfeldt-Jakob Disease

Four types

Sporadic (sCJD): accounts for 85-90% of

cases

Variant (vCJD): mad cow disease, Kuru

Familial (fCJD): 10% of cases (these

individuals carry a PRPc mutation)

Iatrogenic

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Creutzfeldt-Jakob Disease Best imaging clue: Progressive T2 hyperintensity

of basal ganglia (BG), thalamus, and cerebral cortex

Predominantly gray matter (GM): Caudate and putamen > globus pallidus (GP) Thalamus: Common in variant CJD (vCJD)

Cerebral cortex: Frontal, parietal, and temporal

2 signs seen in 90% of vCJD but can also occur in sporadic CJD (sCJD) "Pulvinar" sign: Symmetric T2 hyperintensity of

pulvinar of thalamus

"Hockey stick" sign: Symmetric pulvinar and dorsomedial thalamic nuclear hyperintensity

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Main Differential Diagnosis

Extrapotine osmotic demyelination

syndrome

Central pons T2 hyperintensity with sparing of

periphery

Location

Basal ganglia (BG)

Cerebral white matter (WM)

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CASE 2

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Patient Profile

42 years old, female

Past history

Sudden onset of reversible stroke on

2015/8/15

Chief complaint

Repeated numbness and transient numbness

and dysarthria for 2 weeks (2017.03)

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Image

2015.12.05 Angiography

2015.12.08 CT perfusion

2017.03.21 Angiography

2017.05.14 Brain MRI

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15 2015 2017

CB

F

CB

V

MT

T

Clinical Course

Clinical diagnosis: moyamoya disease ?

Underwent left fronto-temporal craniotomy

EC-IC bypass on 2017.05.15

2017/05/26 Brain MRI

Successful EC-IC bypass ?

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Moyamoya

Progressive narrowing of distal

ICA/proximal circle of Willis (COW)

vessels

With secondary collateralization → cloud-

like "puff of smoke" (moyamoya) at

angiography

Attenuated COW with multiple tiny basal

ganglia "flow voids" on MR

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Main Differential Diagnosis

PACNS

Circumferential, smooth, long-segment vessel

wall enhancement

"Beaded" arteries on DSA

Leptomeningeal arteries and veins are mostly

affected, but involves intracranial vessels of

any size

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Main Differential Diagnosis

RCVS

Reversible, multifocal cerebral artery

vasoconstrictions

Symptoms: severe headaches ± focal

neurological deficits

Involves large, medium-sized arteries

Diffuse, multifocal, segmental narrowing

Sometimes "string of beads" or "sausage

strings"

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20 Published February 22, 2013

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↓CBF ↑CBV

↑MTT ↑TTP

Before Surgery

CBF CBV

MTT TTP

After

Surgery

Zhang J, Wang J, Geng D, Li Y, Song D, Gu Y. Whole-Brain CT Perfusion and CT Angiography Assessment of

Moyamoya Disease before and after Surgical Revascularization: Preliminary Study with 256-Slice CT. Minnerup J,

ed. PLoS ONE. 2013;8(2):e57595. doi:10.1371/journal.pone.0057595.

Conclusion

The 256-slice whole-brain CTP and 3D-CTA have the potential for the non-invasive

assessment of the abnormalities of intracranial arteries, the graft patency and

cerebral perfusion changes in Moyamoya disease before and after surgery.

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Zhang J, Wang J, Geng D, Li Y, Song D, Gu Y. Whole-Brain CT Perfusion and CT Angiography Assessment of

Moyamoya Disease before and after Surgical Revascularization: Preliminary Study with 256-Slice CT. Minnerup J,

ed. PLoS ONE. 2013;8(2):e57595. doi:10.1371/journal.pone.0057595.

CASE 3

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Patient Profile

39 years old, male

Chief complaint

Persistent neck and low back pain, the worst

symptoms in the morning, the symptoms

worsened by sitting

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Image

2015/10/12 L-spine MRI

2017/05/17 T-spine MRI

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Clinical Course

Image diagnosis: myxopapillary

ependymomas

Underwent laminectomy at T5~T6, and

hemilaminectomy at T4 with removal of

tumor

Pathology: schwannoma

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Myxopapillary Ependymoma

Usually spans 2-4 vertebral segments

May fill entire lumbosacral thecal sac

Ovoid, lobular, sausage-shaped

CT/radiographs

± osseous canal expansion, thinned pedicles, vertebral

scalloping

May enlarge, extend through neural foramina

T1WI: Isointense→ hyperintense to cord

T2WI: Almost always hyperintense to cord

Hypointensity at tumor margin = hemosiderin

T1WI C+: Intense enhancement

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Spinal Schwannoma 70-75% intradural extramedullary

Most common intradural extramedullary mass

15% completely extradural

15% transforaminal, "dumbbell" masses

Size varies from small intradural mass to large intraspinal or paravertebral mass ("giant schwannoma") extending ≥ 2 vertebral segments

Bone remodeling due to large intraspinal or intraforaminal tumor common

Cystic change common

Calcifications, hemorrhage are rare

Uniform, heterogeneous, or peripheral enhancement patterns

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It is very important to differentiate SCHs and MPEs before surgery, because there

are reported cases of dissemination of MPEs through cerebrospinal fluid

throughout the neuraxis.

MPEs must be removed en block to prevent dissemination

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Moriwaki T, Iwatsuki K, Ohnishi Y, Ninomiya K, Yoshimine T.

Extramedullary Conus Ependymoma Involving a Lumbar Nerve Root

with Filum Terminale Attachment. Clinical Medicine Insights Case

Reports. 2015;8:101-104. doi:10.4137/CCRep.S24719.

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Schwannoma Myxopapillary Ependymoma

Moriwaki T, Iwatsuki K, Ohnishi Y, Ninomiya K, Yoshimine T.

Extramedullary Conus Ependymoma Involving a Lumbar Nerve Root

with Filum Terminale Attachment. Clinical Medicine Insights Case

Reports. 2015;8:101-104. doi:10.4137/CCRep.S24719.

CASE 4

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Patient Profile

34 years old, female

Chief complaint

Seizure attack twice since April 2016

(2016.11)

Past history

Nil

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Image

2016/09/20 Brain MRI

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Clinical Course

2016/11/04 underwent left temporal

craniotomy

Pathology

Epithelioid malignant peripheral nerve sheath

tumor (MPNST) and malignant melanoma

However, primary or secondary (metastatic)

origin of the tumor cannot be determined

based on histology

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Image

2016/12/20 Brain MRI (Post operation)

2017/03/20 Brain MRI

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2016.09.20

2016.12.20 2017.03.20

Clinical Course

2017/03/21 underwent left temporal

craniotomy

Pathologic consultation in the USA

Epithelioid GBM

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2016 WHO Classification

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Epithelioid Glioblastomas Predilection for children and younger adults

Present as superficial cerebral or diencephalic masses

Sometimes co-exist with pleomorphic xanthoastrocytomas

Pathology Large epithelioid cells with abundant eosinophilic cytoplasm,

vesicular chromatin, and prominent nucleoli, and variably present rhabdoid cells

Gene Often harbor a BRAF V600E mutation

IDH-wildtype epithelioid glioblastomas often lack other molecular features of conventional adult IDH wildtype glioblastomas, such as EGFR amplification and chromosome 10 losses

Frequent hemizygous deletions of ODZ3

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CT T2 Flair GRE

T1 +C DWI AD

C

Liebelt BD, Boghani Z, Takei H, Fung SH, Britz GW. Epithelioid

glioblastoma presenting as massive intracerebral hemorrhage: Case

report and review of the literature. Surgical Neurology International.

2015;6(Suppl 2):S97-S100. doi:10.4103/2152-7806.153643.

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Diffuse sheets of markedly atypical epithelioid cells

with pleomorphic nuclei are noted in a hemorrhagic

background. Prominent microvascular proliferation

is seen (arrows)

Glial fibrillary acidic protein (GFAP)

immunohistochemical stain reveals cytoplasmic

positivity in tumor cells

Liebelt BD, Boghani Z, Takei H, Fung SH, Britz GW. Epithelioid

glioblastoma presenting as massive intracerebral hemorrhage: Case

report and review of the literature. Surgical Neurology International.

2015;6(Suppl 2):S97-S100. doi:10.4103/2152-7806.153643.

Differential Diagnosis

Ganglioglioma

Pleomorphic xanthoastrocytoma

DIA/DIG, Desmoplastic Infantile

Astrocytoma and Ganglioglioma

Oligodendroglioma

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