Neurospot
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This document contains 20 quiz cases related to neuroimaging. Each case provides the radiological sign, associated condition, and brief description. The cases cover a wide range of neurologic conditions seen on CT and MRI, including infections, demyelinating diseases, vascular abnormalities, tumors and developmental anomalies. Key information about the featured conditions is also summarized.
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Neurospot
- 1. Neurospot 17.06.2016 THORSANG CHAYOVAN R3/AJ. NUTTHA SANGHAN
- 2. Quiz 1: Mount Fuji • Bilateral subdural hypertensive pneumocephalus • Compression of the frontal lobes • Neurosurgical emergency • Differentiation between hypertensive VS non-hypertensive • Hypertensive pneumocephalus afters • Subdural hematomas drainage • Skull base, paranasal sinuses and posterior fossa surgeries • Head trauma
- 3. Quiz 2: Dense artery • Early sign of acute middle cerebral artery infarction • An increase in density of MCA proximal segments secondary to thrombosis • DDx • Parietal calcification • Focal subarachnoid hemorrhage
- 4. Quiz 3: Caput medusae • Developmental venous anomaly • A network of dilated, abnormal medullary veins with radial distribution, converging into a dominant, transparenchymal vein • May drain into a cortical vein, dural sinuses or into the deep venous system • Most frequent intracranial vascular abnormalities • Associated with cavernomas • May lead to intracranial hemorrhage, thrombosis and venous infarction
- 5. Quiz 4: Popcorn ball • Cavernoma (Cavernous Malformation) • Benign vascular hamartoma • Composed of packed immature blood vessels with microhemorrhage • Hyperdense on noncontrast CT; 50% Calcification • Lobulated appearance; no mass effect • Low signal T2w (HS) • High signal T1w (Met-Hb) • No to faint enhancement on post contrast • DDx: AVM--incomplete hemosiderin rim, flow voids on T2w
- 6. Quiz 5: Eye-of-the-tiger • T2w-Low-signal globus palladi surrounds a central, small hyperintense area • Hallervorden-Spatz (HS) syndrome • Neurodegeneration with brain iron accumulation (NBIA) • Pantothenate kinase II (PANC2)- associated neurodegeneration • Excessive iron accumulation • Central high signal: gliosis, increased water content, and neuronal loss with disintegration, vacuolization, and cavitation of the neurophil
- 7. Hallervorden-Spatz (HS) syndrome • Neurodegenerative disorder associated with extrapyramidal dysfunction and dementia • Triad of iron deposition, axonal spheroids, and gliosis in the globus pallidi • MRI: differentiating HS syndrome from infantile axonal dystrophy—no iron deposition • DDx other extrapyramidal Parkinsonian disorders • Cortical-basal ganglionic degeneration • Steele-Richardson-Olszewski syndrome • Early-onset levodopa-responsive Parkinsonism
- 8. Quiz 6: Dawson's fingers • Multiple sclerosis • White matter inflammatory changes around the perimedullary veins • Ovoid lesions; longest axis perpendicular to corpus callosum • Demyelinating plaques • Juxtacortical white matter • Periventricular white matter • Corpus callosum • Callosal-septal interface • High signal intensity on sequences with long repetition time • Hyposignal intensity on T1w
- 9. Multiple sclerosis • Perivascular lymphocyte infiltration • McDonald criteria 2016 Dissemination in space Dissemination in time CorticL/Juxtacortical (involves U-fiber) New T2w/Gd-enhancing lesion Periventricular (Dawson’s finger) Multistage Infratentorial Spinal cord Optic nerve
- 10. Wernicke encephalitis • Congestion and petechial hemorrhage • Subependymal regions • Mammillary bodies
- 11. CADASIL: Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy • Recurrent lacunar and subcortical white matter strokes in multiple vascular territories • Young and middle age patients without known vascular risk factors • Diffuse; anterior temporal lobe (86%) and external capsule (93%) • Microhemorrhages • Cerebral atrophy
- 12. Marchiafava-Bignami disease • Chronic alcoholism • Corpus callosum necrosis and demyelination • Atrophy and necrosis • T1w hypointense, T2w hyperintense linear/punctate regions
- 13. Quiz 7: Salt and pepper • Paraganglioma • Salt = tumor matrix • T2w hyperintense (slow intratumor flow and hemorrhage) • Vivid enhancement (rapid wash-in and wash-out) • Pepper = small vessels within these masses • Flow-voids on T1w and T2w
- 14. Paraganglioma • Near nerves and vessels • Neuroectodermal origin • Head and neck; 4 most common sites • Glomus valgale tumor • Carotid body tumor • Glomus jugulare tumor • Glomus jugulotympanicum tumor • Hypervascular tumor • Homogeneously enhancing when small • Heterogeneously enhancing when large • Flow voids • Erode skull base, hourglass shape
- 15. Quiz 8: Eccentric target • CNS toxoplasmosis • A ring enhancing abscess associated with an enhancing mural nodule • Highly specific but low sensitivity (found in 30% of cases) • Believed to represent internal folds and invaginations of the abscess walls
- 16. CNS Toxoplastomosis • Propensity for the basal ganglia, corticomedullary junction, white matter, and periventricular regions • Calcification in congenital • Uncommon calcification in acquired • Mass effect • Thin, smooth, or poorly defined rim of enhancement • Solid eccentric nodular enhancement/no enhancement • T1w: hypointense/peripheral hyperintensity >> not lymphoma • T2w: high/mixed signal intensity • DWI: peripheral hyperintensity (hemorrhage in walls)
- 17. Quiz 9: Molar tooth • Altered mesencephalon on axial sections • Thickened and more horizontally oriented superior cerebellar peduncles extending perpendicularly from brainstem • Joubert syndrome
- 18. Quiz 10: Hot cross bun • Multiple systems atrophy type C • Cruciform pontine hyperintensity • Selective loss of neurons of transverse pontocerebellar fibers • Neurodegenerative disorder with basal ganglia and olivopontocerebellar complex involvement
- 19. Quiz 11: Figure eight • Lissencephaly • Defective neuronal migration between 8th-14th gestational weeks • Figure eight configuration • Colpocephaly, flat gyri, thickened cortex and cortico/subcortical atrophy • Miller-Dieker syndrome
- 20. Quiz 12: Bilateral CP angle masses • AMEN • A: acoustic schwannoma • M: meningioma • E: ependymoma • N: neuroepithelial cyst (arachnoid/epidemoid)
- 21. Quiz 13: Martini glass • Persistent hyperplastic primary vitreous (PHPV) • Congenital embryonic remnants of hyaline vessels in Cloquet’s canal • Fibrovascular tissue • Attached lens to optic nerve head • Connecting laterally to abnormally elongated ciliary process • Retinal detachment 30%
- 22. Quiz 14: Tram-track • A central linear hypodensity/hypointen sity delimitated by affected optic nerve sheath • Useful in differentiation from optic nerve gliomas
- 23. Quiz 15: face of the giant panda • Wilson's disease--genetic disorder of the copper metabolism • Hepatocellular degeneration • Accumulation in liver and brain • T2w hyperintensity-- pontine tegmentum • T2w hypointensity • Periaqueductal gray matter • Partial red nuclei • Lateral aspect of the substantia nigra pars reticulata • Upper colliculus
- 24. Panda sign of sarcoidosis • Gallium-67 citrate scan finding • Bilateral involvement of parotid and lacrimal glands • Superimposed on the normal uptake in nasopharyngeal mucosa
- 25. Osmotic demyelination syndrome • Pons • Basal ganglia • Thalami • Cerebral peduncles • Subcortical white matter • Cerebellum • Cervicomedullary junction
- 26. Quiz 16: Empty orbit sign • Neurofibromatosis type 1 • Orbit on skull film or CT • Lack of the innominate line due to dysplasia of the greater wing of the sphenoid, shortening of the lateral wall of the orbit and flattening of the orbital angle
- 27. Quiz 17: TS • Bourneville-Pringle syndrome • Calcified subependymal nodules (hamartomas) • Subependymal giant cell astrocytomas • Foramen of Monroe (Most) • Cortical/subcortical tubers • Thickened cortex, enlarged gyri • White matter radial migration lines • Cyst-like white matter lesions
- 28. Quiz 18: Sturge-Weber • Encephalotrigeminal angiomatosis • Congenital low-flow vascular malformation • Port-wine stain on face in trigeminal distribution • Leptimeningeal angioma primarily parieto-occipital distribution • Seizure, severe metal retardation • Intracranial gyral calcification • Tram track distribution • Occipital location • Ipsilateral enlargement and enhancement of choroid plexus
- 29. Quiz 19: Spoke wheel • Typical angiographic appearance found in meningioma • Multiple small arteries radially distributed from a dominant feeding artery • Extra-axial, slow-growing, well- vascularized lesions with a benign behavior • Dural tail and hyperostosis of the adjacent bone
- 30. Quiz 20: Stripe/tigroid • Linear hypointensities radiating from ventricular margins within hyperintense white matter • No enhancement • A specific pattern of demyelination sparing perivascular white matter • Dark spots/linear areas against a bright affected white matter >> leopard skin • Metachromatic leukodystrophy (MLD)
- 31. References • http://www.scielo.br/scielo.php?pid=S0100- 39842011000200013&script=sci_arttext&tlng=en • http://www.scielo.br/scielo.php?pid=S0100- 39842011000200014&script=sci_arttext&tlng=en • http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2765 171/ • pdf.posterng.netkey.at/download/index.php?module =get_pdf • Diagnostic neuroradiology; Anne G. Osborn • Diagnostic imaging: brain • Diagnostic imaging: head and neck • Head and neck imaging; Peter M. Som • http://pubs.rsna.org/doi/full/10.1148/radiographics. 19.6.g99no251605 • http://pubs.rsna.org/doi/full/10.1148/radiol.239305 0459 • http://pubs.rsna.org/doi/full/10.1148/rg.294085205 • http://www.ajnr.org/content/16/8/1653.full.pdf • http://www.slideshare.net/fernferretie/the-orbit • http://radiopaedia.org/articles/hot-cross-bun-sign • http://radiopaedia.org/articles/panda-sign-of- sarcoidosis • http://radiologykey.com/toxic-and-metabolic-brain- disease • http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4365 670/pdf/BJBMS-1-1.pdf • http://saraswatastar.weebly.com/radiological- signs.html • https://www.urmc.rochester.edu/medialibraries/ur mcmedia/imaging/education/educational- resources/documents/paragangliomas_000.pdf
Editor's Notes
- Associated with cavernomas (30% of cases)
- On MRI lobulated appearance due to multiple locules which show variable signals depending on blood degradation products Seizures 50% Neuro deficit 25% Asymptomatic 20%
- Neuroaxonal dystrophy
- Hypo to iso-signal intensity on T1w
- Periventricular (Dawson’s finger) ตั้งฉาก ภ้า small vv disease จะ spare callososeptal interface Spinal cord—postlat; short segment ไม่เกิน 3 vertebral bodies unlike NMO ถ้า small vv จะอยู่กลาง At least 2 of 5 areas Three or more periventricular lesions One or more infratentorial lesion One or more spinal cord lesion One or more optic nerve lesion One or more cortical/juxtacortical lesion
- neurologic disorder from chronic thiamine deficiency--ocular abnormalities, ataxia, and confusion Patho: areas of congestion and petechial hemorrhage in the subependymal regions around the third ventricle and aqueduct, floor of the fourth ventricle, pulvinar and dorsomedial thalamic nuclei, hypothalami, superior cerebellar vermis, and mammillary bodies CT is generally not helpful. Rarely, low density may be seen in thalamus MRI: T2W hyperintensities. Restricted DWI.+-Enhancement. enhancement of the mammillary bodies is pathognomonic. Microhemorrhages.
- CT is non-specific, demonstrating white matter regions of low attenuation. MRI: widespread confluent white matter hyperintensities. More circumscribed hyperintense lesions are also seen in the basal ganglia, thalamus and pons 3. Although the subcortical white matter can be diffuse; initial course involve anterior temporal lobe (86%) and external capsule (93%) are classical. Relative sparing occipital and orbitofrontal subcortical white matter, subcortical U-fibers and cortex. Cerebral microhaemorrhages Eventually cerebral atrophy
- Highly sensitive and specific sign
- Glomus valgale--intravagal paraganglia dispersed within the perineurium, below the nerve sheath, or between the nerve fiber fascicles อยู่หลัง ICA—displace ICA anteriorly Carotid body at the common carotid artery (CCA) bifurcation splays the bifurcation of the CCA does not narrow the caliber of the ECA and ICA may involve the lower cranial nerves and pharynx Superior extension to the skull base 3. Glomus jugulare tumors arising within the jugular foramen follow the paths of least resistance: mastoid air cell tracts, vascular channels, eustachian tube, and neural foramina Tumor spread via the air cell tracts or the Haversian canal system results in moth-eaten pattern of destruction of the temporal bone Dehiscence of the inferior wall of the tympanic cavity and involvement of the mesotympanum and the ossicles Medial spread from the middle ear results in osteonecrosis 4. Glomus tympanicum--Within middle ear
- gray matter–white matter junctions, findings indicative of a hematogenously disseminated process (c)Unenhanced T1-weighted MR image shows hyperintense borders, a somewhat irregular and “shaggy” and low-signal-intensity centers of the lesions. (e) Contrast-enhanced T1-weighted MR image shows no internal enhancement of the lesions
- DDx CNS lymphoma, tuberculosis, aspergillosis, progressive multifocal leukoencephalopathy, cryptococcosis, and bacterial abscesses Lesions in lymphoma tend to be more locally infiltrative Butterfly-like pattern of spread and enhancement is more suggestive of lymphoma than toxoplasmosis Larger More often have a periventricular distribution
- DDx CNS lymphoma, tuberculosis, aspergillosis, progressive multifocal leukoencephalopathy, cryptococcosis, and bacterial abscesses Lesions in lymphoma tend to be more locally infiltrative Butterfly-like pattern of spread and enhancement is more suggestive of lymphoma than toxoplasmosis Larger More often have a periventricular distribution
- AR Abnormal eye movements, nystagmus, difficulty in following a moving object with eyes, episodes of taquipneia and apnea, besides motor developmental delay
- degeneration of pontine neurons and myelinated transverse pontocerebellar fibers in a cruciform pattern features of Parkinsonism and/or cerebellar ataxia along with autonomic dysfunction may have predominant Parkinsonian features (MSA-P) or predominant cerebellar features (MSA-C) Preservation of pontine tegumentum and corticospinal tract
- Other findings in Parkinsonism hypointensity of the putamina, slit-like hyperintensity of the lateral putaminal border, and atrophy of the cerebellar vermis or hemispheres
- AMEN A: acoustic schwannoma (~80%) M: meningioma (~10%) E: ependymoma (~5%) N: neuroepithelial cyst (arachnoid/epidermoid) (~5%)
- The primary vitreous is supplied by the embryonal hyaloid circulation, which regresses at birth
- women between their third and fifth decade of life
- neurologic disorder --chronically malnourished alcoholics. rapid changes in serum osmolality --aggressive iatrogenic correction of hyponatremia. extensive demyelination within the pons and other sites CT: low density in affected areas, reflecting edema MRI: ovoid/trident areas of T2-weighted (T2W) hyperintensity in the central portion of the pons with sparing of the ventrolateral aspect of the pons and the corticospinal tracts. No enhancement In the subacute phase (1-2 wks), involve the entire pons. Restrion ได้ MR spectroscopy: decreased N-acetyl-aspartate (NAA)/creatine (Cr) and increased choline (Cho)/Cr ratios MR perfusion: increased on cerebral blood volume (CBV)
- In addition to the absence of the innominate line, there is egg-shaped enlargement of the anterior orbital rim, a bony defect in the posterior orbit, and anteroposterior enlargement of the middle cranial fossa Sphenoid wing dysplasia associated with pulsatile exophthalmos, expansion of the temporal fossa, and herniation of the temporal lobe into the orbit have been described as the ‘cranio-orbital-temporal’ subtype of NF1
- SEN<1.3 VS SEGA>1.3cm T1 ขาว restricted DWI, enhanced Focal lacune-like Cyst (vascular etiology)
- The leptomeningeal angiomas cause abnormal venous drainage with chronic ischaemia, leading ultimately to cortical atrophy and calcification, the latter feature being usually very prominent. By 2 years of age, skull radiographs may reveal ‘tramline calcifications’ within the cortices.
- Another remarkable and very common characteristic of meningiomas is the presence of a dural tail and, in 25% of cases, hyperostosis of the adjacent bone
- Symmetric increased signal in the periventricular white matter Initial sparing of subcortical U fibers No enhancement --Unlike in adrenoleukodystrophy AR--Deficiency of lysosomal enzyme arylsulfatase