NEURO 3 Flashcards

Question

Cerebellopontine angle masses

Answer 1

schwannoma (75%) menignioma epidermoid

Answer 2

- AT/RT - medulloblastoma - ependymoma - JPA - Hemangioblastoma - ganglioglioma - Diffuse Pontine glioma

Answer 3

- Mets - Astrocytoma - Gliomatosis cerebri - oligodendroglioma - primary CNS lymphoma

Answer 4

soft tissue sarcoma that can mimic an aggressive meningioma because both enhance homogeneously - will not calcify or cause hyperostosis - will invade the skull

Answer 5

- hyperacute (\<24 hours): T1 iso, T2 bright - acute (1-3 days): T1 iso, T2 dark - early subacute (3-7 days): T1 bright, T2 dark) - late subacute (7-14 days): T1 bright, T2 bright) - chronic (\> 14 days): T1 dark, T2 dark)

Answer 6

star gazing fetus: upturned face, hypextended C-spine, short neck - deficit of occipital bones, resulting in an enlarged foramen magnum

Answer 7

- elongated superior cerebellar peduncles - small or aplastic vermis - absence of pyramdial decussation - strong assocaition with retinal dysplasia - association with multicystic dysplastic kidney

Answer 8

- occipital encephalocele - multiple renal cysts - polydactyly - strong association with holoprosencephaly

Answer 9

- single midline monster eye - solitary median maxillary incisor - pyriform aperture stenosis (From nasal process overgrowth)

Answer 10

- optic nerve hypoplasia - absent septum pellucidum - epilepsy

Answer 11

Klippel Feil syndrome

Answer 12

brachycephaly + fused fingers

Answer 13

brachycephaly + first arch (maxilla and mandible) hypoplasia

Answer 14

brachycephaly + wormian bones + absent clavicles Abstract Cleidocranial dysplasia: * AD skeletal dysplasia * abnormal clavicles, * patent sutures and fontanelles, * supernumerary teeth, * short stature, * and a variety of other skeletal changes. * The disease gene has been mapped to chromosome 6p21 within a region containing CBFA1, a member of the runt family of transcription factors. Typical clinical and radiological findings in CCD. (A) Facial appearance in a 6 month old boy. Note large, brachycephalic skull, frontal and parietal bossing with large anterior fonanelle, and the appearance of a small face. Other characteristic features include widely spaced eyes, low nasal bridge, reduced nasal length, but increased nasal width and protrusion. (B) Chest radiograph showing cone shaped thorax and left clavicular hypoplasia and aplasia on the right side. (C) Pelvic abnormalities in a 4 year old girl. Note hypoplasia of the iliac wings, broad femoral necks with large epiphyses, and unossified symphysis pubis. (D) Hand radiograph of a 2½ year old female showing hypoplastic distal phalanges, accessory epiphyses of the second metacarpal, and long second metacarpal. (E) Pantomographic view of the permanent dentition of a 16 year old female. Note multiple, unerupted, supernumerary teeth. https://jmg.bmj.com/content/36/3/177

Answer 15

mitochondrial encephalomyopathy, lactic acidosis, and stroke like episodes on MR spectroscopy: increased lactate; decreased NAA * CT * multiple infarcts * involving multiple vascular territories * may be either symmetrical or asymmetrical * parieto-occipital and parieto-temporal involvement is most common * basal ganglia calcification 1,2 * more prominent feature in older patients * atrophy 2 * MRI * acute infarcts * swollen gyri with increased T2 signal * may enhance * subcortical white matter involved * increased signal on DWI (T2 shine through) with little if any change on ADC: thought to represent vasogenic rather than cytotoxic oedema 3 * chronic infarcts * involving multiple vascular territories * may be either symmetrical or asymmetrical * parieto-occipital and parieto-temporal most common * MR spectroscopy: * may demonstrate elevated lactate in otherwise normal appearing brain parenchyma or in CSF 3,4. * Case courtesy of Dr Mustafa Takesh, Radiopaedia.org, rID: 78355

Answer 16

* *Metachromatic - diffuse white matter involvement with tigroid apperance** Metachromatic leukodystrophy (MLD) is the most common hereditary (autosomal recessive) leukodystrophy and is one of the lysosomal storage disorders. It has characteristic imaging features including peri-atrial and to a lesser extent frontal horns leukodystrophy as well as periventricular perivenular sparing results in "tigroid pattern" on fluid-sensitive MRI sequences. Epidemiology Metachromatic leukodystrophy has an estimated prevalence of ~1:100,000 and typically manifests between 12 to 18 months of age. The disease can sometimes be classified according to the time of onset: late infantile: most common ~65% (range 50-80%) juvenile (onset between 3-10 years) adult (after age 16) https://radiopaedia.org/articles/metachromatic-leukodystrophy

Answer 17

Canavan's disease Canavan disease is rare genetic neurological disorder characterized by the spongy degeneration of the white matter in the brain. Affected infants may appear normal at birth, but usually develop symptoms between 3-6 months of age. Canavan disease, also known as spongiform degeneration of white matter (not to be confused with Creutzfeldt-Jakob Disease) or aspartoacylase deficiency, is a leukodystrophy clinically characterised by megalencephaly, severe mental and neurological deficits, and blindness. Epidemiology Canavan disease is particularly common in the Ashkenazi Jewish community 1. The carrier frequency among the Ashkenazi ranges from 1:37 to 1:57, with a corresponding prevalence of 1 in 6000-14000 in this high risk group1. In the general population the prevalence is 1 in 100000 11. Clinical features There are a wide range of clinical features, but generally there is a progression from lethargy and hypotonia, to macrocephaly (due to underlying megalencephaly) and spasticity, to blindness and seizures, to decerebrate posturing and eventual death 2. In the vast majority of patients, clinical onset is in infancy with death before 5 years of age, and often before 18 months, but juvenile-onset forms of the disease have also been reported 2. Juvenile-onset forms may have speech difficulty, mild intellectual impairment and suffer neurological regression 11. Pathology It is an autosomal recessive disorder due to a gene mutation on the short arm of chromosome 17 leading to deficiency of N-acetylaspartoacylase, a key enzyme in myelin synthesis, with resultant accumulation of N-acetylaspartate (NAA) in the brain, cerebrospinal fluid, plasma, and urine 3,4. Although its effects are widespread, it has a predilection for subcortical U-fibres and Alzheimer type II astrocytes in the gray matter 3,5. Markers Increased levels of NAA in the urine may be detected 11. Radiographic features In Canavan disease the neuroimaging findings are diagnostic of the condition 11. CT The oedematous sponginess of the white matter causes a characteristically low radiographic attenuation on CT so that it stands out from the relatively unaffected gray matter 4. Megalencephaly may also be also noted depending on the clinical stage 4. MRI MRI confirms the megalencephalic appearance and provides more detail of the white matter disease, which is typically diffuse, bilateral, and involving the subcortical U-fibres 4-8,11: T1: areas of low signal mainly within subcortical white matter generally with sparing of the corpus callosum, caudate nucleus, putamen and internal capsule as the condition progresses atrophy of the periventricular white matter may be seen with associated ventriculomegaly globi pallidi and thalami are usually affected as well T2/FLAIR: findings as above except for areas of high signal in the affected white matter DWI: restricted diffusion within the diseased white matter MR spectroscopy: markedly elevated NAA and NAA:creatine ratio are pathognomonic for the condition 11. this can be remembered using the mnemonic CaNAAvan There is no enhancement of affected regions on either CT or MRI 5-8. Treatment and prognosis The condition is fatal with death resulting at 2-5 years and treatment is generally supportive 4. No effective treatment is yet available 4. However, genetic therapies are being trialled and seem to reduce the level of NAA within the brain 11.

Answer 18

``` intermediate TE (140) at long TE (280) - lipid and lactate double peak seen ```

Answer 19

Alzheimer's disease

Answer 20

meningiomas

Answer 21

hepatic encephalopathy

Answer 22

- elevated choline, lactate, and lipids - decreased NAA

Answer 23

- decreased choline, NAA - elevated inositol

Answer 24

- decreased choline and NAA - increased lactate

Answer 25

complication of Apical petrositis, when Dorello's canal is involved triad of: 1) otomastoiditis 2) facial pain (trigeminal neuropathy) 3) lateral rectus palsy (CN 6)

Answer 26

sphenopalatine artery (terminal internal maxillary artery)

Answer 27

odontogenic abscess

Answer 28

inferior rectus --\> Medial rectus --\> superior rectus --\> lateral rectus --\> superior/inferior obliques (IM SLO)

Answer 29

- stop Plavix 7 days prior - stop coumadin 4-5 days prior - stop LMW heparin 24 hours prio - hold heparin for 2-4 hours and document normal PTT - ASA and NSAIDS are fine

Answer 30

- sacrococcygeal osseous defect (scimitar sacrum) - anorectal malformation - anterior sacral meningocele

Answer 31

myelopathy associated with a dural VF

Answer 32

* A spectrum of amyloid disorders patholgoically defined as deposition of amyloid within the brain vessels. * CEREBRAL AMYLOID ANGIOPATHY (CAA) * without inflammation * AB - RELATED ANGIITIS (ABRA) * vascular angiodestructive inflammation * CEREBRAL AMYLOID ANGIOPATHY RELATED INFLAMMATION * CAA-RI * Perivascular inflammatory change without angiodestructive process.

Answer 33

* Deposition of Amyloid in vessel walls * Not Synonymous with AD, can be an exclusive dx. * Increased Risk for sig Hx * Peripheral Microhx * Lobar Hemorrhage * superficial siderosis.

Answer 34

* Leptomeningeal enhancement common * 10x greater likelihood that simple CAA * CAARI: Confluent subcortical +/- cortical edema * parietal and temporal lobes more frequent * May be unifocal or multifocal. Often asymmetric * Microhemorrhages tend to be more frequent in areas of edema * Much less likely to have lobar hemorrhage than CAA

Answer 35

Circumferential vessle wall enhancement with surrounding micro hemorrhages. https://www.asnr.org/education/neurocurriculum-live/

Answer 36

Leptomeningeal enhancement within the central sulcus on post contrast flare and post contrast T1W imaging. SWI shows evidence of prior lobar hx in the right frontal libe and superficial siderosis and peripheral micro hx https://www.asnr.org/education/neurocurriculum-live/

Answer 37

* Alzheimers' Dementia * Amyloid + Tau disorder * Amyloid alone is insufficient to cause significant neuronal damage * Tau deposition is likely the culprit for neuronal loss https://www.asnr.org/education/neurocurriculum-live/

Answer 38

1. Cog impairment increases over time 2. Amyloid deposition proceeds CI by many years, and in many may be asymptomatic 3. Tau deposition occurs 4. around the same time FDG hypometabolism occurs 5. MRI positive findings of atrophy then occur https://www.asnr.org/education/neurocurriculum-live/

Answer 39

TOP LEFT: diffuse/generalised Amyloid deposition TOP RIGHT and BOTTOM LEFT: more regional deposition of Tau, temporal/parietal BOTTOM RIGHT: temporal lobe and parietal lobe atrophy on MRI https://www.asnr.org/education/neurocurriculum-live/

Answer 40

* T1W sequences * Coronal: * profound mesial temporal lobe volume loss * hippocampus * entorhinal cortex * Asymmetry of the left sylvian fissure * Parasagittal * Pre-cuneus atrophy disproportionate to the rest of the brain

Answer 41

* Marked atrophy over two years with a striking posterior predominance. ? AD vs DLB. * FDG PET * Asymmetric primary posterior hypo metabolism * Amyloid PET (FLORBETPIR) * Showed amyloid deposition * Ioflupane scan done * Reduced striatal binding * OVERALL: * This patient has dementia with Lewy bodies * DLB is often amyloid positive * So the amyloid scan may not be very useful https://www.asnr.org/education/neurocurriculum-live/

Answer 42

Posterior cortical atrophy (PCA), also called Benson's syndrome, is a rare, visual variant of Alzheimer's disease. It affects areas in the back of the brain responsible for spatial perception, complex visual processing, spelling and calculation.

Answer 43

Persons with logopenic variant primary progressive aphasia (lvPPA) have increasing trouble thinking of the words they want to say. As time goes on, people with lvPPA have more trouble getting the words out, and they eventually begin to speak slower and slower. What Causes lvPPA? The cause of lvPPA is unknown. Scientists know that in lvPPA there is a large build-up of proteins called amyloid and tau within brain cells, which are the same proteins that build up in Alzheimer’s disease. These proteins occur normally, but we do not yet understand why they build up in large amounts. As more and more proteins form in brain cells, the cells lose their ability to function and eventually die. This causes the affected parts of the brain, most often the left posterior temporal cortex and inferior parietal lobe, to shrink. https: //memory.ucsf.edu/dementia/primary-progressive-aphasia/logopenic-variant-primary-progressive-aphasia https: //www.asnr.org/education/neurocurriculum-live/

Answer 44

* Possibly the second most common form of dementia * may be difficult to distinguish from AD in early disease * Autopsy study: * 50% of patients with living dx of DLB had AD on Autropsy * A-synucleinopahty * majority also show amyloid deposition https://www.asnr.org/education/neurocurriculum-live/

Answer 45

* Cingulate Island * posterior hypometabolism with sparing of the cingulate cortex. * Occipital Tunnel * sparing of the calcarine cortex * DatScan * reduced striatal binding

Answer 46

* Primary progressive aphasia * Heterogenous group of neurodegenerative disorders characterised by progressive language deficit * Subtypes * Non-fluent or agrammatic (navPPA) * Semantic (svPPA) * Logopenic (lvPPA) * Although grouped together, the clinical symptoms, imaging and pathology are very different.

Answer 47

Nonfluent navPPA Logopenic Lv PPA Semantic Sv PPA

Answer 48

1. navPPA * nonfluent variant * Dominant hemisphete frontal lobe hypometabolism and atrophy which frequently extends to involve the temporal lobe later in the course of the disease 2. SvPPA * Asymmetric dominant lobe atrophy and hypometabolism in the temporal pole 3. LvPPA * dominate hemisphere hypometabolism temporal lobe/ posterior para sylvian and temporal lobe distribution

Answer 49

* Patient 3 * Profound hippocampal volume loss * Relative Preservation of the entorhinal cortex * Frontal lobe atrophy slightly more prominent than that of the paramedic parietal lobe * Patient cognitively intact despite profound volume loss * Amyloid pet scan (left picture) * Showed normal white matter binding * Right picture shows abnormal cortical binding * LATE: * Profound hippocampus damage (CA1) as well as in the subiculum, entorhinal cortex and amygdala * ? Sunomymous with hippocampal sclerosis of aging. * Hipoocampal sclerosis unilateral in 40-50% * Hippocampal atrophy usually greater than those with Pure AD. Often asymmetric * Combo of AD and LATE had greater hippocampal atrophy than those with only AD. * Also atrophy in the frontal anterior temporal and insular cortices. * Corresponds with the distribution of TDP-Proteinopathy on autopsy * Typically \>75 years * profound mesial temporal atrophy, even greater with coexistent AD pathology. * May have little cognitive impairment, MCI or severe demented (typically with +AD) * Often asymmetric MTL atrophy (L\>R in my experience) https://www.asnr.org/

NEURO 3 Flashcards

(75 cards)