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RANZCR Part 2 Exam > crack the core exam case companion > Flashcards

crack the core exam case companion Flashcards

1
Q

CASE 1

31 Year old female

  • What is this?
  • Classic History?
  • Classic distribution of WM involvement
  • Classic distribution of WM sparing?
A

CADASIL

  • Cerebral, autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy
  • Classic History?
    • Migraines
  • Classic distribution of WM involvement
    • temporal lobes
  • Classic distribution of WM sparing?
    • occipital lobes
  • Essential Trivia:
    • inherited condition.
    • presents between 30-40 yo.
    • hx of migrains with aura is classic
    • depression and FNDs can also occur
    • Lots of things involve the frontal WM.
    • However, involvement of the temporal WM esp when severe in a 30 yo should make you say CADASIL
    • The other classic finding is relative sparing of the occipital subcortical wm and U-Fibres.
    • The MRA is usually normal with minimal if any atherosclerosis.
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2
Q

CASE 2

  • HX: Developmental delay
  • What is the diagnosis?
  • What part of the CC forms first?
  • What conditions are classically a/w agenesis of the CC?
A
  • HX: Developmental delay
  • What is the diagnosis?
    • agenesis of the CC with corpocephaly
  • What part of the CC forms first?
    • Genu
  • What conditions are classically a/w agenesis of the CC?
    • midline lipoma
    • colpocephaly
    • hydrocephalys
    • chiari II malformations
    • DW spectrum.
  • ESSENTIAL TRIVIA
    • Three classic ways to demonstrate ACC
      • Wide spaced parallel ventricles
      • Colpocephaly
      • Steer Horn appearance on coronal.
    • Associations
      • Colpoephally
      • Intracranial Lipoma
      • Dandy walker
      • chiari II
      • Holoprosencephaly
      • Fetal Etoh syndrome.
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3
Q

CASE 3

ataxia and sleep apnea

  • What is the Dx
  • What is the Buzz word
  • what is the. most common association?
  • NAME THE 3 associations.
A

HX: Developmental delay

  • What is the diagnosis?
    • Joubert syndrome
  • What is the Buzz word
    • molar tooth sign
  • what is the. most common association?
    • retinal dysplasia
  • ESSENTIAL TRIVIA
    • AKA vermian aplasia
    • AKA molar tooth midbrain-hindbrain malformation
    • inherited condition AR
    • Lack of the normal decussation of the superior cerebrallar deduncular fibre tracts leads to the enlargement of the peduncles. All this together makes the MB look like a molar tooth.
    • ASSOCIATIONS
      • retinal dysplasia 50%
      • Mulicycstiv dysplastic kidney 30%
      • Polydactyly 15%
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4
Q

CASE 4

Notorious Drunk now with Quadriplegia

  • what is the dx?
  • What is the pathophysiology?
  • What is the earliest sign?
A
  • what is the dx?
    • Central pontile myelinolysis
    • T2 hyperintensity of the central pons
  • What is the pathophysiology?
    • rapid correction of Na
  • What is the earliest sign?
    • restricted diffusion in the lower pons.
  • ESSENTIAL TRIVIA
    • this is acute demyelination of the WM tracts of the pons. Its seen in the setting of acute osmotic changes, classically described with the rapid correction of hyponatremia.
    • the classic hx is a chronic alcoholic who had a rapidly corrected Na. He felt much better after the correction, then returns to the ED 3 days later with spastic quadriparesis and pseudobulbar palsy (hyperactive gag reflex and dysarthria)
    • The first sign is restricted diffusion in the lower pons
    • low signal on T1 and high signal on T2 in the same region may not show up for two weeks.
    • It can enhance like an acute MS plaque but doesn’t have to
    • Peripheral fibers are classically spared.
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5
Q

CASE 5

Hx witheld

  • What is the Dx?
  • What is the Classic Hx?
  • What is the tubercinerum?
A
  • What is the Dx?
    • Tuber cinerum hamartoma
  • What is the Classic Hx?
    • Gelastic seizures
  • What is the tubercinerum?
    • Part of the hypothalamus
  • ESSENTIAL TRIVIA
    • A hamartoma located within a specific location of the hypothalamus
    • AUNT MINNIE
    • Should be isointense to cortex on T! with no enhancement
    • Classic clinical stories
      • Gelastic Seizures
      • Precocious puberty
    • The Tubercinerum is part of the hypothalamus located between the optic chiasm and the mammillary bodies.
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6
Q

CASE 6

Hx: Altered mental status

  1. what is the diagnosis?
  2. What are ddx for bilateral thalamic hypodensities on CT?
  3. The artery of Percheron arise from ? and supplies?
A
  1. what is the diagnosis?
    • Artery of Percheron infarct
  2. What are ddx for bilateral thalamic hypodensities on CT?
    • internal cerebral vein thrombosis
    • Top of the basilar syndrome
    • Wernicke-Korsakoff.
  3. The artery of Percheron arises from ? and supplies?
    • arises from unilateral PCA and supplies bilateral thalamus and MB.

ESSENTIAL TRIVIA

  • The artery of Percheron is a rare vascular variant in which a single common trunk arises from one of the PCAs to supply both thalami and the MB.
  • Occlusion of the AoP will cause a bilateral infarction of the thalamus and a V-shaped infarct of the rostral midbrain.
  • on CT, Occlusion of the bilateral cerebral veins, occlusion of the tip of the basilar artery or even Wernicke’s can give a similar appearance.
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7
Q

CASE 7

Hx Withheld

  • What is the Dx?
  • What is the Classic presentation
  • What is the Classic Hx?
  • What is the Next Step?
A
  • What is the Dx?
    • Superficial siderosis
  • What is the Classic presentation
    • SEMSPROMEIRA; JEAROMG ;PSS
  • What is the Classic Hx?
    • AVM OR ANEURYSM
  • What is the Next Step?
    • CTA/Conventional Angiogram

SUPERFICIAL SIDEROSIS

  • Deposition of hemosiderin along the leptomeninges
  • Essential trivia
    • the etiology is typically recurrent or extensive SAH. This can be from any cause. Trauma, bleeding brain tumor, AVM, or aneurysm.
    • CTA is the Next step
    • Sensorineural hearing loss is found in 95% of patients and is the most common clinical presentation.
    • It is typically bilateral and gradual in onset.
    • The second most common symptom 88% is ataxia.
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8
Q

CASE 8

Hx Withheld.

  • What is the Dx?
  • Fibrous dysplasia vs Pagets?
  • What if the patient had a cafe au lait spot?
  • What if the pt had intramuscular myxomas?
A
  • What is the Dx?
    • Fibrous Dysplasia
  • Fibrous dysplasia vs Pagets?
    • Pagets involves the INNER TABLE
  • What if the patient had a cafe au lait spot?
    • McCune Albright sundrome
  • What if the pt had intramuscular myxomas?
    • Mazabraud syndrome
  • ESSENTIAL TRIVIA
    • FD is a benign tumour like process that occurs as a result of screwed up osteoblastic function.
    • The result is the progressive replacement of normal bone with immature woven bone.
    • Lion Face/leontiasis Ossea is the historic term used for craniofacial FD
    • Ground glass is the classic buzzword
    • Pagets is the primary DDX
    • The difference is that FD spares the inner table, and pagets classically involves it.
    • McCune Albright syndrome
      • precocious puberty
      • cafe au lait spots
      • polyostotic FD
    • Mazabraud syndrome
      • polyostotic fibrous dysplasia
      • multiple soft tissue myxomas (usually in large muscle groups)
      • Increased risk of malignant transformation.
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9
Q

CASE 9

Hx: Headache

  • what is the Dx?
  • Unlike NF1, NF 2 is not associated with?
  • What is A/W NF 1
  • NF1 patients get optic nerve gliomas, these are typically ____
A
  • what is the Dx?
    • NF 2
    • Multiple Meningiomas and BILATERAL SCHWANNOMAS
  • Unlike NF1, NF 2 is not associated with?
    • Neurofibromas
  • NF 1 Associations
    • Sphenoid wing dysplasia
    • Gliomas
    • Cafe Au Lait spots >6
    • LATERAL THORACIC MENINGOCELE
  • NF1 patients get optic nerve gliomas, these are typically LOWER GRADE THAN THOSE THAT OCCUR SPORADICALLY.

ESSENTIAL TRIVIA:

  • Rare AD neurocutaneous disorder
  • MISME
    • multiple inherited schwannomas, meningiomas and ependymomas
  • Neurofribromas are NOT part of the NF-2 Spectrum, making the making the name a misnomer
  • The finding of a meningioma in a child should raise the question of NF 2.
  • Ependymomas a/w NF2 are typically SPINAL (not intracranial.
  • Schwannomas a/w NF 2 usually involved the vestibular branch of CN 8.
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10
Q

NF 1 ASSOCIATIONS

6

A
  1. Sphenoid wing dysplasia
  2. Plexiform neurofibromas
  3. Bad Scoliosis
  4. Renal vascular hypertension
  5. Lateral thoracic Meningioceles ***
  6. Pseudarthrosis of the fibula

The Optic nerve gliomas seen with NF1 are typically WHO Grade 1 JPAs (juvenile Pilocyctic astrocytomas).

The optic nerve gliomas seen in the wild are typically WHO grade 4 GBMs.

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11
Q

TUBEROUS SCLEROSIS

4 sites of TS associations.

A
  • BRAIN
    • Cortical Tubers
    • Subependymal Tubers
    • SEGA
  • Kidney
    • AMLs
      • tend to be large, multiple and bilateral
  • Lungs
    • LAM
      • Lymphangioleiomyomatosis
  • Heart
    • Cardiac rhabdomyomas
      • Typically involve the ventricular septum and nearly all occur before 1 year of age.
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12
Q

VHL

Assoications

2 sites and the associations in those sites.

2 cancers

A
  • BRAIN
    • Think hemangioblastomas (cyst and nodule)
    • Endolymphatic sac tumours (temporal bone thing)
  • ABDOMEN
    • LOTS OF CYSTS
      • liver
      • kidney
      • pancreas
      • Serous cyst adenomas in pancreass
  • TUMOURS
    • Pheochromocytomas
    • Bilateral Clear cell RCCs.
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13
Q

Cowden Syndrome Associations

3

A
  • Lhermitt Duclos
    • dysplastic cerebellar Gangliocytoma
    • striated non-enhancing cerebellar tumour that does not cross the midline
  • Bowel hamartomas
  • Breast Ca
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14
Q

GORLIN SYNDROME Associations

A
  • Multipple odontogenic cysts
  • Massive flax cerebri Calcifations
  • Medulloblastoma
  • The reason there is any clinical utility to knowing this at all is that when they get radiation for their medulloblastoma, they develop lots of basal cells.
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15
Q

CASE 10

Hx: Stuffy nose

  • What is the dx?
  • This Ca originates from?
  • Classical Findings for this tumour?
  • Typical age?
A
  • What is the dx?
    • Esthesioneuroblastoma
  • This Ca originates from?
    • the olfactory cells
  • Classical Findings for this tumour?
    • peritumoural cysts
    • dumbbell appearance
  • Typical age?
    • bimodal 20s and 50s
  • ESSENTIAL TRIVIA
    • Essentially a neuroblastoma of the olfactory cells
    • bimodal distribution
    • Starts growing at the fribriform plate, then usually grows down and up, waisting in the middle. Creatinga dumbell appearance.
    • The characteristic finding I speritumoural cysts at the top.
    • If they want a single diagnosis, they have to show you this, otherwise is a squmaous cell, which is far more common.
    • They enhance avidly
    • They take up MIBG bc they are basically a neuroblastoma.
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16
Q

CASE 11

Hx: Stuffy nose

  • What is the dx?
  • What is the classical Finding for this Tumour?
  • What is the classic buzz word finding?
  • What is hidden within 10-15% of these tumours?
A
  • What is the dx?
    • Inverting papilloma
  • What is the classical Finding for this Tumour?
    • middle turbinate?
  • What is the classic buzz word finding?
    • Cerebriform Patter on alternating high and low signal
  • What is hidden within 10-15% of these tumours?
    • SCC
  • ESSENTIAL TRIVIA
    • This is a benign sinonasal tunour, with distinctive imaging features making it testible
    • 10-15% conversion to SCC
    • The typical location is the lateral wall of the nsal cavity, associated with the middle turbinate.
    • On MRI they have a classic cerebriform pattern of alternating high and low signal. This is seen on both T2 and T1 + c. This sign is found in at least 50% of these and is fairly unique to the tumour.
    • On CT these look like lobulated masses containing fragments of destroyed bone.
  • WHEN I SAY - frequent local recurrence despite adequate resection
  • YOU SAY:
    • Schneiderian Ca
    • this is a rare form of malignancy that has histo indistinguishable from the usual inverting papilloma.
      • you say
17
Q

What are the 4 types of Fungal Sinusitis?

A
  • acute invasive
  • non invasive
  • chronic invasive
  • allergic

Allergic and Invasive are the most commonly tested.

Allergic:

  • is the most common
  • IgE response to fungal antigents
  • tends to affect all the sinuses at the same time
  • Mucin which contains concentrated protein, fungal elements and heavy metals, results in a hy per dense look on T and a DARK look on both T1 and T2.
  • This mimics Pneumatized sinus.
  • Trying to get you to call a fungus laden sinus as pneumatised sinus on MRI is the oldest trick in the book.

Angio-Invasive:

  • Think about mucor eating the diabetic dues face off.

ESSENTIAL TRIVIA:

  • CT is best for bony change.
  • MRI is best for intracranial or orbital spread.
  • The black turbinate sign, an ischaemic (non-enhancing_ middle turbinate mucosa is an early indicator of invasive fungal.

RANZCR Part 2 Exam (64 decks)

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