Neuro Flashcards
Ddx intraaxial hemorrhage
HTN, tumor (primary or mets), vascular malformation (cavernoma, AVM), trauma, amyloid, venous infarct, transformation of ischemic stroke
gelatinous pseudocysts - who? describe? caused by?
HIV pts
perivascular spread of infection from
cryptococcus, toxo, TB, fungal, angiocentric lymphoma
Intraventricular mass in adult
glioma (astrocytoma, subependymoma, giant cell astrocytoma), meningioma, ependymoma, choroid plexus papilloma, metastasis, cystercosis, central neurocytoma (young adult)
intraventricular mass in child
choroid plexus papilloma, ependymoma, primitive neuroectodermal tumor (PNET), teratoma, and astrocytoma
Posterior dysmyelinating disease
Adrenoleukodystrophy (unless it’s a girl - very unlikely in this X-linked disease) - has a leading edge of enhancement
PRES (ask about HTN)
Anterior dysmyelinating disease
Alexander
Childhood onset Alexander disease is sporadic and typically presents with macrocephaly, rapid neuorological deterioration, seizures and spasticity, and retarded psychomotor development
Describe pattern of metachromatic leukodystrophy
Diffuse, sparing of subcortical U fibers, “tiger striping”
How do you grade an AVM?
Spetzler-Martin
Size of nidus (6cm)
location (eloquent?)
draining vein deep or superficial
What is a ganglioglioma
low grade, typically temporal lobe.
Solid or partially cystic mass.
Ddx temporal lobe mass with cystic component
Ganglioglioma PXA: pleomorphic xanthoastrocytoma hemangioblastoma (can have cystic component) pilocytic astrocytoma met (lung, breast, etc.) oligodendroglioma (look for calcs on CT) DNET (bubbly on T2) all cystic - neurocysticercosis
Name the CNS vascular malformations
capillary telangiectasia, AVM, cavernous hemangioma, developmental venous anomaly
periventricular cystic spaces in a child
periventricular leukomalacia from HIE (hypoxic ischemic encephalopathy) in former premie
hypothalamic/suprasellar masses - adult
craniopharyhgioma, pituitary adenoma (maybe with hemorrhage), teratoma, aneurysm, rathke cleft cyst, chordoma,
mets
Wernickes Encephalopathy - what is it? findings?
Thymine (B1) deficiency
symmetrical increased T2 signal in the thalami, mamillary bodies, tectal plate, and periaqueductal area
Ddx leptomeningeal enhancement
meningitis (bacterial, fungal, TB) leptomeningeal carcinomatosis (CNS) lymphoma/leukemia sarcoidosis (moya moya)
intracranial hypotension - what? findings?
CSF leak (usually in spine)
cerebellar tonsillar herniation
dural venous sinus distention
dural (pachymeningeal) enhancement
Ddx dural/pachymeningeal enhancement
infection: bacterial, fungal, TB intracranial hypotension post-op dural mets (breast and prostate) sarcoid lymphoma
What’s the difference between leptomeninges and dura/pachymeninges?
Lepto = pia and arachnoid (gyriform) Pachymeninges = dura (follows dural reflections)
Enlarged lateral and third ventricles, small 4th ventricle
aqueductal stenosis
Multiple hemosiderin deposits in brain (“black dot” differential)
DAI, mets, amyloid, cavernomas, neurocystercercosis (calcification internally)
ring-enhancing lesions in the CNS
GBM (thick rim) abscess (restricted diffusion) CNS lymphoma with immunocompromised host tumefactive MS (incomplete ring) sequestered subacute infarct resolving hematoma radiation necrosis Toxoplasmosis (neurocystercercosis - some enhance, some not)
lesion in 3rd ventricle
colloid cyst, aneurysm, neurocystercercosis, subependymoma
NF2 lesions
meningioma, schwannoma (esp 8th nerve), ependymoma
what is a phakomatosis? name some
neurocutaneous disorder characterised by involvement of structures that arise from the embryonic ectoderm (thus central nervous system, skin and eyes)
neurofibromatosis type 1 (NF1)
neurofribromatosis type 2 (NF2)
tuberous sclerosis
encephalotrigeminal angiomatosis: or Sturge-Weber syndrome
von Hippel-Lindau disease
ataxia telangiectasia (etc…)
lesions in Tuberous Sclerosis
cortical tubers subependymal nodules subependymal giant cell astrocytomas (SGCA) renal angiomyolipomas (AML) renal cysts cardiac rhabdomyoma(s) LAM (lungs)
lesions in NF1
neurofibromas, pheochromocytoma malignant peripheral nerve sheath tumour (MPNST) previously known as neurofibrosarcoma overal risk of developing a MPNST is ~10% *optic nerve glioma* Wilms tumour rhabdomyosarcoma renal angiomyolipoma glioma juvenile pilocytic astrocytoma diffuse brainstem glioma spinal astrocytoma and spinal pilocytic astrocytoma carcinoid tumour(s) ganglioglioma leukemia
Sturge-Weber syndrome in the brain: features?
leptomeningeal hemangioma results in a vascular steal affecting the subjacent cortex and white matter producing localised ischemia. The majority of cases (~ 80%) have unihemispherical involvement
Look for port wine stain on face
later get calcification starting subcortically
Hemangioblastoma - where? who gets them?
posterior fossa, spine. ~young adults. Consider vHL (25% are vHL, so always raise this possibility).
posterior fossa mass in child
medulloblastoma, JPA, ependymoma
posterior fossa mass in adult
mets, hemangioblastoma, meningioma, astrocytoma, epidermoid
Findings in ADEM? what does it mean?
Acute Disseminated Encephalomyelitis
monophasic acute inflammation and demyelination of white matter typically following a recent (1 - 2 weeks prior) viral infection or vaccination. Grey matter, especially that of the basal ganglia, is also often involved, albeit to a lesser extent, as is the spinal cord
PML - what does it stand for? Look like?
Progressive multifocal leukoencephalopathy
Demyelinating disease which results from the JC virus. strongly associated with immunosuppressed states, esp AIDS but also transplant patients and leukemia.
Asymmetric focal zones of low attenuation involving the peri-ventricular and sub-cortical white matter (including U fibers). This is in distinction to the more symmetrical hypo-attenuation seen in HIV encephalopathy.
What’s subacute sclerosing panencephalitis? Who gets it? Caused by?
Sub acute sclerosing panencephalitis (SSPE) (also known as Dawson disease)
- rare chronic, progressive and fatal encephalitis
- children and young adults
- caused by persistent infection of immune resistant measles virus
Appearance of HIV encephalitis
diffuse, symmetric cerebral atrophy, out of keeping with age
symmetric periventricular and deep white matter hypoattenuation and T2 hyperintensity
confluent or patchy
no mass effect
no enhancement
infant with large cyst and nodule - Ddx
DIG - desmoplastic infantile ganglioglioma
Ddx - supratentorial ependymoma, PNET, pilocytic astrocytoma
PNET - stands for? Includes what tumors?
Primative neuroectodermal tumor
aggressive brain tumor in child
medulloblastoma : by far the most common
supratentorial PNET : 15% of all CNS PNET
pineoblastoma : most common supratentorial PNET
spinal PNET : rare
hemangiopericytoma - what is it? where does it occur?
Think aggressive variant of meningioma that metastasizes
Helpful features include :
lobulated contour
absence of calcification and hyperostosis
invasion of the skull (common)
multiple flow voids on MRI (need to distinguish from spoke-wheel appearance of meningioma)
corkscrew arteries
may have a narrow base of dural attachment
Ddx of skull-base extra-axial masses
Hemangiopericytoma meningioma lymphoma dural mets plasmacytoma
Leigh disease - what? who? looks like?
also known as sub acute necrotizing encephalomyelopathy). Progressive (mitochondrial) neurodegenerative disorder and invariably leads to death in childhood
High T2 in:
brainstem
periaqueductal gray matter
medulla
midbrain
putamen : characteristic but not always present
Ddx of high T1 in basal ganglia
*TPN (hyperalimentation)
hepatic encephalopathy
NF1
Ddx of high T2 in basal ganglia
hypoxic event CO (globus pallidus) NF1 CJD (also cortex) Leigh dz (more generalized) Wilson's dz (also more generalized)
Finding in CO poisoning:
low density / high T2 in globus pallidus
What is Dandy Walker malformation?
triad of:
hypoplasia of the vermis and cephalad rotation of the vermian remnant
cystic dilatation of the fourth ventricle extending posteriorly
enlarged posterior fossa with torcular-lambdoid inversion (torcular lying above the level of the lambdoid due to abnormally high tentorium)
It is at the severe end of the spectrum denoted by the term Dandy-Walker continuum.
Holoprosencephaly - describe the types:
Incomplete separation of the two hemispheres
Alobar: single midline monoventricle, absent midline structures
Semilobar: fused thalami, absence of septum pellucidum
monoventricle with partially developed occipital and temporal horns
rudimentary falx
Lobar: fusion of the frontal horns of lateral ventricles
wide communication of this fused segment with the third ventricle
fusion of the fornicies
absence of septum pellucidum
agenesis or hypoplasia of the corpus callosum
(Septo-optic dysplasia is in the ddx for lobar)
Schizencephaly - what do you have to have? Two types:
grey matter-lined cleft extending from the ependyma to the pia mater
Open lip: CSF seen between
Closed lip: cleft walls touch
rhombencephalosynapsis
absent or small cerebellar vermis and fused cerebellar hemispheres
abnormally shaped 4th ventricle
what does herpes encephalitis look like? What do you need to do?
T2 hyper, maybe enhancement, of medial temporal lobes, insular cortex and inferolateral frontal lobes (cingulate gyrus). The basal ganglia are typically spared
Call the clinician for immediate treatment!
Vein of Galen malformation: appearance? consequences? Rx?
AV fistula of the median prosencephalic vein (MPV) (a precursor of the vein of Galen) occurring at 6-11 weeks gestation. The MPV fails to regress and becomes aneurysmal.
Can cause high-output heart failure, hydrocephalus/aqueductal stenosis, ischemia
Rx: embolization
Signs of carotid-cavernous fistula
proptosis enlarged superior ophthalmic veins extra ocular muscles may be enlarged orbital edema may show SAH / ICH from ruptured cortical vein
fat going through dorsal spinal defect
lipomyelocele (no meningocele in the case shown)
Ddx intramedullary spinal cord lesion
astrocytoma (extensive, kids more)
ependymoma (bleeding and cysts, adults more)
hemangioblastoma (often cystic)
lymphoma
sarcoidosis
ADEM (expanding, continuous lesions affecting multiple levels)
MS
transverse myelitis - what is it? appearance
Inflammatory condition affecting BOTH HALVES of the spinal cord and associated with rapidly progressive dysfunction
Cause: idiopathic or e.g. MS, ADEM (post viral/vaccine), SLE
Usually thoracic
Usually 3-4 segments of cord, usually 2/3 of cross sectional area of cord
Nonenhancing cyst in conus
Ventriculus terminalis (normal structure)
CSF lesion displacing spinal cord
arachnoid cyst (rx fenestration)
What is a snake-eye or owl-eye appearance in the spinal cord?
compressive myelopathy from compression and venous infarction - means pt won’t totally recover after surgery
Spine: dorsal columns are involved, demonstrating high signal on T2 - what is it? cause?
Subacute combined degeneration (B12 deficiency)
or Tabes Dorsalis from tertiary Syphilis
name for enhancing lesion in or around conus
myxopapillary ependymoma
Name for spinal defect containing CSF? Containing neural elements as well?
Meningocele contains meninges
myelomeningocele contains neural elements
named for direction (dorsal, ventral, lateral) and location (thoracic, sacral, etc.)
widened neural foramen
Peripheral nerve sheath tumor (schwannoma), neurofibromas (NF1), synovial cyst, arachnoid cyst
Ddx ivory vertebral body - child
lymphoma
osteosarcoma
osteoblastoma
metastatic disease (eg neuroblastoma)
Ddx ivory vertebra - adult
osteoblastic mets (prostate, breast)
lymphoma
Paget disease of bone
What’s the Currarino triad?
Hereditary: anorectal malfomation or congenital anorectal stenosis sacrococcygeal osseous defect pre sacral mass e.g anterior sacral meningocoele
describe a hangman’s fracture. is it stable?
involves both pars interarticularis of C2, and is as a result of hyperextension and distraction
unstable
what’s os odontoideum
tip of dens is separate, not an acute fracture
Fracture in pt with ankylosing spondylitis
Anderson fracture
Defect and scelrosis at anterio-superior corner of a vertebral body
Limbus vertebra
Caused by herniation of a portion of the nucleus pulposus underneath the ring apophysis before its fusion to the body
More central is a Schmorl’s node
epidural spinal mass
epidural metastasis lymphoma epidural abscess herniated disk epidural hematoma meningioma