path neuro formatted Flashcards
- 73.APRIL02 A 34 year old, mildly retarded woman is having a CT scan for presumed meningioma. She has some small nodules on her face, coloured nodules on her iris, and brown macules 2 – 3 cm in size on her hands and neck. Her parents, who accompany her, are both normal. The most likely diagnosis is
- Type I Neurofibromatosis
- Type II Neurofibromatosis
- Turcot’s syndrome
- Tuberous Sclerosis
- Trisomy 21
- 73.APRIL02 A 34 year old, mildly retarded woman is having a CT scan for presumed meningioma. She has some small nodules on her face, coloured nodules on her iris, and brown macules 2 – 3 cm in size on her hands and neck. Her parents, who accompany her, are both normal. The most likely diagnosis is Answer:
- Type I Neurofibromatosis
- Type I Neurofibromatosis
- Type II Neurofibromatosis
- Turcot’s syndrome
- Tuberous Sclerosis
- Trisomy 21
o 50% spontaneous / 50% AD
o Cutaneous Hyperpigmented Macules (Café au Lait) o Pigmented Nodules Of The Iris (Lisch Nodules)
- 84.APRIL02 Necrosis is a characteristic feature of
- Pilocytic astrocytoma
- Glioblastoma multiforme
- Acoustic schwannoma
- Craniopharyngioma
- Ependymoma
Answer:2. Glioblastoma multiforme
Usually large, heterogenous masses with central necrosis, thick, irregular “shaggy” walls, and increased vascularity with haemorrhage
• Marked mass effect & oedema
• Often find varying degrees of anaplasia within the same tumour
∴biopsy can be misleading
- 84.APRIL02 + APRIL2004 Necrosis is a characteristic feature of
- Pilocytic astrocytoma
- Glioblastoma multiforme
- Acoustic schwannoma
- Craniopharyngioma
- Ependymoma
- 85.APRIL02 A 12-year-old boy presents with a seizure and a solid mass involving the cortex of a temporal lobe is demonstrated on MRI. The most likely diagnosis is:
- DNET
- Pilocytic astrocytoma
- Anaplastic astrocytoma
- Mesial temporal sclerosis
- Pleomorphic xantho-astrocytoma
*LW: Difficult question, possibly poor recall given no perfect answer:
Preferred options of DNET vs PXA
1) DNET: multicystic bubbly cortical mass, commonly temporal lobe
2) Pilocytic astrocytoma: enhancing nodule with associated cyst, rarely cortical.
3) Anaplastic Astrocytoma may cause solid appearing cortical mass, however anaplastic occur in adulthood mean age - 40-50yrs.
4) Mesial sclerosis: neronal loss and gliosis
5) Pleomorphic xanthoastrocytoma: cortical enhancing mass with adjacent cyst, temporal lobe most common, older children.
Previous notes:
i thin it is 3 or 5: in the absence of stated associated cystic component astrocytoma possible, while age argues against. However question states age and temporal lobe, thus PXA felt most likely.
- 86.APRIL02 Which of the following statements concerning hypertensive haemorrhage IS LEAST correct?
- They are related to the lenticulo-striate arteries
- They are most common in the putamen
- They occur secondary to focal cerebral infarction
- Less than 1% occur in the posterior fossa
- They are not due to cerebral emboli
- 86.APRIL02 Which of the following statements concerning hypertensive haemorrhage IS LEAST correct?
Answer: - Less than 1% occur in the posterior fossa
- They are related to the lenticulo-striate arteries (lateral lenticulostriate arteries)
- They are most common in the putamen (Putamen / external capsule 60 – 65 %)
- They occur secondary to focal cerebral infarction (lacunar infarcts)
- Less than 1% occur in the posterior fossa
- They are not due to cerebral emboli (NOT due to emboli)
• Major sites o Predilection for areas supplied by penetrating branches of MCA & basilar artery: • 2/3rd's in basal ganglia • Putamen / external capsule 60 – 65 % • Thalamus 15 – 25 % • Pons 5 – 10% • Cerebellum 2 – 5 % • These behave like space occuping lesions leading to herniation and obstructive hydrocephalus • Subcortical lobar white matter 1 – 2 %
- 87.APRIL02 A 38 year old man with disseminated tuberculosis is having an MRl/MRA of their brain. Which of the followed would be the LEAST expected finding
- Exudate in the basal cisterns
- Hydrocephalus
- 4cm intraparenchymal cerebral mass
- Marked arterial irregularity
- Degeneration of the central portion /body of the corpus callosum
Answer:
5. Degeneration of the central portion /body of the corpus callosum This is a feature of Marchiafavia Bignami disease (Big Robbins p1329
- 87.APRIL02 A 38 year old man with disseminated tuberculosis is having an MRl/MRA of their brain. Which of the following would be the LEAST expected finding )
- Exudate in the basal cisterns (thick gelatinous exudate is found in the basal cisterns)
- Hydrocephalus (Both CT and MR can also document communicating hydrocephalus, a common sequela of tuberculous meningitis)
- 4cm intraparenchymal cerebral mass (tuberculoma)
- Marked arterial irregularity (TB meningitis - Progressive obliterative arteritis)
- Degeneration of the central portion /body of the corpus callosum This is a feature of Marchiafavia Bignami disease (Big Robbins p1329)
• CNS TB may take a variety of forms, including tuberculous meningitis, abscess, focal cerebritis, and tuberculoma.
• The most common radiographic findings associated with CNS TB include enhancement of the basal cisterns, granulomata, calcifications, hydrocephalus, meningeal enhancement, and infarction, most often of the basal ganglia.
• Coexistent pulmonary TB is often present, seen in 25% to 83% of cases of CNS TB
• Thick gelatinous exudate is found in the basal cisterns
• Arteries that course through this exudate can become directly involved by the inflammatory infiltrate, indirectly by reactive endarteritis obliterans, or by both processes, with consequent spasm and intimal changes resulting in thrombosis and infarction.
• Arteritis is present in approximately 28% to 41% of cases with basilar meningitis
• The most common parenchymal form of CNS TB is tuberculous granuloma (tuberculoma).
o Granulomata may be secondary to hematogenous spread of systemic disease or may evolve from extension of CSF infection into the adjacent parenchyma via cortical veins or small penetrating arteries
o Pathologically, the granuloma is composed of a central zone of solid caseation necrosis, surrounded by a capsule of collagenous tissue, epithelioid cells, multinucleated giant cells, and mononuclear inflammatory cells
• Tuberculomas may be found in the cerebrum, cerebellum, subarachnoid space, or subdural or epidural space.
• Parenchymal disease most often involves the corticomedullary junction and periventricular regions, as expected for hematogenous dissemination.
• Most tuberculomas are supratentorial
- 89.APRIL02 Which of the following statements IS LEAST correct concerning hypertensive changes in the brain?
- Severe hypertension may cause tentorial herniation due to diffuse cerebral edema
- The putamen is the most common site of hypertension related hemorrhage
- Hypertension is the principal cause of over 50% of primary cerebral hemorrhage
- The pons is a rare site of hypertensive hemorrhage (<1%)
- Histologically there is often a rim anoxic neural and glial change around an area old hemorrhage
answer: 4. The pons is a rare site of hypertensive hemorrhage (<1%) (5-10%)
6. 89.APRIL02 Which of the following statements IS LEAST correct concerning hypertensive changes in the brain?
- Severe hypertension may cause tentorial herniation due to diffuse cerebral edema
- The putamen is the most common site of hypertension related hemorrhage (60-65%)
- Hypertension is the principal cause of over 50% of primary cerebral hemorrhage
- The pons is a rare site of hypertensive hemorrhage (<1%) (5-10%)
- Histologically there is often a rim anoxic neural and glial change around an area old hemorrhage
• Patients coming to postmortem examination may show an edematous brain weighing more than normal, with or without transtentorial or tonsillar herniation
- 90.APRIL02 Concerning CNS demyelination, which of the following statements is correct:
- Acute disseminated encephalomyelitis (ADEM) typically follows a bacterial infection
- Central pontine myelinosis is due to rapid correction of hyperkalaemia
- Multiple sclerosis lesions do not involve the corpus callosum
- Depletion of oligodendrocytes is a feature of MS lesions
- Multiple sclerosis increases in frequency with HIV
Answer:4. Depletion of oligodendrocytes is a feature of MS lesions (Loss of myelin & oligodendrocytes)
- 90.APRIL02 Concerning CNS demyelination, which of the following statements is correct:
- Acute disseminated encephalomyelitis (ADEM) typically follows a bacterial infection (viral)
- Central pontine myelinosis is due to rapid correction of hyperkalaemia (Caused by rapid correction of hyponatraemia or extreme serum hyperosmolarity)
- Multiple sclerosis lesions do not involve the corpus callosum (Corpus callosum in 50 – 90%)
- Depletion of oligodendrocytes is a feature of MS lesions (Loss of myelin & oligodendrocytes)
- Multiple sclerosis increases in frequency with HIV
- 91.APRIL02 Concerning central neurocytomas, which of the following statements IS INCORRECT:
- They arise in the region of the septum pellucidum
- Histologically (H&E stain) they resemble oligodendrogliomas
- They do not result in hydrocephalus
- They present in young adults
- Calcification is common
Answer:3. They do not result in hydrocephalus (100% have ventriculomegaly)
- 91.APRIL02 Concerning central neurocytomas, which of the following statements IS INCORRECT:
- They arise in the region of the septum pellucidum (Lateral ventricle adjacent to foramen of Monro and septum pellucidum, occasionally involves 3rd ventricle, rarely alone)
- Histologically (H&E stain) they resemble oligodendrogliomas (Mistaken for intraventricular oligodendroglioma on imaging & histologically – indistinguishable)
- They do not result in hydrocephalus (100% have ventriculomegaly)
- They present in young adults (Young adults (20 – 40 yrs))
- Calcification is common (> 60% have calcification)
- 23.02.25 Chronic alcoholic presents with nystagmus, ophthalmoplegia, ataxia, but with preserved speech, no facial droop. What is likely diagnosis?
- Wernicke’s encephalopathy
- Marchiafava Bignami syndrome
- Korsakoff’s syndrome
- Cerebellar infarct
- Osmotic pontine myelinosis
Answer:
1. Wernicke’s encephalopathy
- 23.02.25 Chronic alcoholic presents with nystagmus, ophthalmoplegia, ataxia, but with preserved speech, no facial droop. What is likely diagnosis? Rob p839
- Wernicke’s encephalopathy
- Marchiafava Bignami syndrome (Onset is usually insidious, with the most common symptom be¬ing nonspecific dementia)
- Korsakoff’s syndrome (Severe amnesia, causing confabulation, while patient remains alert & responsive)
- Cerebellar infarct (will have dysarthria)
- Osmotic pontine myelinosis (“locked-in” state)
- Wernicke encephalopathy is marked by ophthalmoplegia; nystagmus; ataxia of gait and stance; and derangement of mental function, characterized by global confusion, apathy, listlessness, and disorientation
- MRI findings are characteristic and include T2 hyperintensity within the medial thalamic nuclei (46%), the periaqueductal gray matter (40%), and the mamillary bodies. The acute lesions may enhance. Mamillary body abnormal signal intensity &/or enhancement may be the only finding of WE.
- 74.APRIL02 A 42-year-old alcoholic presents with confusion, nystagmus, opthalmoplegia ataxic gait but normal speech and facial movements. An MRI is ordered, These findings would be best be explained by;
- Cerebellar infarct
- Marchiafava-Bignami syndrome
- Central pontine myelinolysis
- Wernicke syndrome
- Korsakoff syndrome
- Wernicke syndrome
- 75.APRIL02 A 46-year-old man with non-Hodgkin’s lymphoma suffers increasing headache and drowsiness over 2 weeks. CSF examination shows small round cells with a thick gelatinous capsule, well appreciated on Indian ink suspension. This is most likely to represent:
- Cerebral involvement with candida
- Cerebral involvement with tuberculosis
- Cerebral involvement with cryptococcus
- Cerebral involvement with non-Hodgkin’s lymphoma
- Transformation of the lymphoma and secondary cerebral involvement with acute lymphoblastic leukaemia
- Cerebral involvement with cryptococcus
• May show encapsulated yeasts of Cryptococci on India Ink or Cryptococcal antigen, or in tissue sections by PAS, mucicarmine and silver stains (esp. frequent in debilitated or immunocompromised patients)
- 23.03.89 Pick’s disease, UNCOMMON FINDING ? Dan p314
- Assymetrical atrophy
- Predominant frontal lobes
- Cortical atrophy
- Involvement of posterior 2/3 superior temporal gyrus
- Putamen changes
answer:
4. Involvement of posterior 2/3 superior temporal gyrus
- 23.03.89 Pick’s disease, UNCOMMON FINDING ? Dan p314
- Asymmetrical atrophy
- Predominant frontal lobes
- Cortical atrophy
- Involvement of posterior 2/3 superior temporal gyrus
- Putamen changes
LJS opinion* I think putamen changes unlikely
Temporal lobes involved, anterior more than posterior
Can’t find involvement of deep structures anywhere. So I think 5 is uncommon finding
- 23.03.86 Mycotic aneurysms ? Rob p813
- Rarely bleed
- Peripheral arterial
- Circle of Willis
- Peripheral arterial in the brain anyway.
- 88.APRIL02 What are the most commonly involved organisms in cerebral abscesses?
- Gram negative anaerobes
- Streptococci and staphylococci
- Haemophilus and menigococcus
- Heemophilus and pneumococcus
- Staphylococci and mycobacteria
- Streptococci and staphylococci
- 23.02.24 What is the most common cause of brain abscess in adult?
- Streptococcus & Staph
- Staph & TB
- Staph & Toxo
- TB & Nocardia
- PML
- Streptococcus & Staph
- 23.02.74 CNS features NOT typical of TB are ?
- basal meningeal enhancement
- 4cm focal mass
- irregularity of vessels
all are feature of TB haha
- 23.03.07 In HSV I encephalitis which is TRUE
- Age 50-70 years
- Typically involves superior frontal lobes
- Commonest presentation is headache
- A subacute presentation with ataxia and seizures is recognized
- Oncocytic intracytoplasmic inclusions
Answer:3. Commonest presentation is headache (alterations in mood,memory and behavior is most common presentation)
- 23.03.07 In HSV I encephalitis which is TRUE Rob p828-829
- Age 50-70 years (most common in children and young adults)
- Typically involves superior frontal lobes (predilection for the limbic system (temporal lobes, cingulate gyri, subfrontal region))
- Commonest presentation is headache (alterations in mood,memory and behavior is most common presentation)
- A subacute presentation with ataxia and seizures is recognized
- Oncocytic intracytoplasmic inclusions (Cowdry Intranuclear inclusion bodies)
- 23.02.22 Creutz-Jakob disease –what would be UNUSUAL finding ?
- Cortical atrophy is rarely found
- Survival of 3-3½ years
- 23.02.22 Creutz-Jakob disease –what would be UNUSUAL finding ? Rob p830
Answer:2. Survival of 3-3½ years (uniformly fatal usually within 7 months) - Cortical atrophy is rarely found (brain appears grossly normal (no significant atrophy), as progression of dementia so rapid)
- Survival of 3-3½ years (uniformly fatal usually within 7 months)
- 23.02.36 Microscopic features of GBM DO NOT include ?
1. Necrosis and areas of different histology
incomplete questionRobins: 1308 The histologic appearance of glioblastoma is similar to anaplastic astrocytoma with the additional features of necrosis and vascular/endothelial cell proliferation.Necrosis in glioblastoma often occurs in a serpentine pattern in areas of hypercellularity.Tumor cells collect along the edges of the necrotic regions, producing a histologic pattern referred to as pseudo-palisadingThe vascular cell prolif- eration produces tufts of cells that pile up and bulge into thelumen; the minimal criterion for this feature is a double layer of endothelial cells. With marked vascular cell proliferation the tuft forms a ball-like structure, the glomeruloid body. VEGF, pro- duced by malignant astrocytes in response to hypoxia, contrib- utes to this distinctive vascular change. Since histologic features can be extremely variable from one region to another, small biopsy specimens may not be representative of the entire tumor.
- 23.02.42 Commonest sites for ependymoma are ?
- Fourth ventricle in children and spine in adults
- Lateral and third ventricle in infants…..
- Periventricular areas (permutations of adult/infant/children)
- Fourth ventricle in children and spine in adults
- 23.02.34 Ganglioglioma ? 1. Superficial location
(Most commonly superficial and supratentorial (temporal > frontal > parietal))
- 23.02.25 Alcoholic, treatment for Wernickes has rapid change in heart size over 1 wk. Most likely:
- Dehydration
- Resolution of pericardial effusion
- Projectional change on CXR
- Beri-beri heart disease
- Beri-beri heart disease this is caused by vit B1 (Thiamine) deficiency can cause acute heart failure with decreased LV systolic function and enlargement +/-
- 23.03.10 In a alcoholic with hyponatraemic and parietal paresis, which is MOST LIKELY:
- Central pontine myelinolysis
- Pontine myelinolysis, midbrain and supratentorial diseases
- Pontine lesions secondary to thiamine defiency
- Subacute combined degeneration of cord secondary to thiamine defiency
- Cord degeneration secondary secondary to folate defiency
*LW:
Correct terminology is osmotic demyelination syndrome / osmotic myelinolysis; which is a global term of both central pontine and extra pontine myelinolysis.
Central pontine myelinolysis clinical features:
dysarthria and dysphagia (secondary to corticobulbar fibre involvement), a flaccid quadriparesis (from corticospinal tract involvement) which later becomes spastic, all from involvement of the basis pontis.
if the lesion extends into the tegmentum of the pons - pupillary, oculomotor abnormalities may occur. There may be an apparent change in conscious level reflecting the “locked-in syndrome” that a large lesion in this site is particularly liable to produce.
Extra pontine myelinolysis clinical features:
Movement disorders
Mutism, parkinsonism, dystonia, and catatonia
- Central pontine myelinolysis
- 23.03.87 Patient with drug resistant Parkinsons and autonomic neuropathy ?
- Shy Drager Syndrome
- Drug resistant Parkinsons
- Striatnigeral degeneration
- Olviopontocerebellar atrphy
- Progressive supranuclear palsy
- Huntingtons
- Shy Drager Syndrome
• Parkinsonism with autonomic system failure, often manifesting as orthostatic hypotension
o orthostatic hypotension
o urinary incontinence
o inability to sweat
• Loss of neurones from intermediolateral column of spinal cord
o explains sympathetic dysfunction
• May be similar pathologically to Parkinson disease (Lewy bodies) or striatonigral degeneration (widespread neuronal loss)
• Shy Drager Syndrome
• Multiple system atrophy with autonomic failure; is a progressive disorder of the central and autonomic nervous systems. The disorder is characterized by postural hypotension
• There are 3 types of Shy-Drager syndrome:
o Parkinsonian-type which may include symptoms of Parkinson’s disease such as slow movement, stiff muscles, and mild tremors
o cerebellar-type which may include problems such as loss of balance and the tendency to fall; and
o combination-type
- 23.02.35 Schwannoma versus plexiform neurofibroma
Neurofibroma- schwann cell, fibroblast- NF1- uncommon - Involve cutaneous, spinal nn- 5-10% malignant degeneration- nerve fibre run through tumour- necrosis, cyst uncommon- histo: spindle cell, with intercellular collagen fibrils, myxomatous matrix- enhance- low T1, high t2
Schwannoma (5th -6th decade- schwan cell- NF2 (young patient)- common- involve CN8, spinal nn- no malignant degeneration- nerve eccentric from tumour- common to have cystic degeneration and haemorrhage- histo: spindle cell (Antoni A), looser mucoid stroma (Antoni B)- low 25% iso 75% T1, high T2
- 23.03.12 Child with post paravertebral mass, biopsy shows acute neural elements and schwann cells not attached to nerve – DIAGNOSIS ?
- Ganglioneuroma
- Ganglioglioma
- Neurofibroma
- Schwannoma
- Neuroblastoma
Answer : 1. Ganglioneuroma (Most well differentiated tumours comprised of ganglion cells and Schwann cells, without neuroblasts)
- 23.03.12 Child with post paravertebral mass, biopsy shows acute neural elements and schwann cells not attached to nerve – DIAGNOSIS ?
- Ganglioneuroma (Most well differentiated tumours comprised of ganglion cells and Schwann cells, without neuroblasts)
- Ganglioglioma
- Neurofibroma (Each fascicle is infiltrated by neoplasm – not possible to separate lesion from nerve)
- Schwannoma (most common in 5th to 6th decade except in NF-2)
- Neuroblastoma (appear as “small blue round cell tumour” - large nuclei, scant cytoplasm, solid sheets)
- 23.03.90 Periventricular mass in patient with renal transplant
- Lymphoma - primary
- GBM
- Lymphoma – 2°
- Lymphoma – 1° (Most common in immunosuppressed patients)
- 23.03.84 Cystic tumour in brain LEAST LIKELY:
- Haemangioblastoma
- JPA
- PNET
- Meningioma
- Schwannoma
- DNET
Answer:4. Meningioma (Cystic or necrotic change may be present - most often in parasagittal tumours (3 – 14%))
- 23.03.84 Cystic tumour in brain LEAST LIKELY is ?
- Haemangioblastoma (60% are cystic masses with mural nodule that usually abuts pial surface)
- JPA (Cerebeallar JPA 30% of total of JPA - Well-circumscribed mass with large cyst, and small reddish-tan mural nodule)
- PNET (commonly cystic)
- Meningioma (Cystic or necrotic change may be present - most often in parasagittal tumours (3 – 14%))
- Schwannoma (cystic change is common)
- DNET (Well-defined “pseudocystic” lesion (high water content)) BR 1348 – cystic changes are a common finding
- Sep03.17 CMV encephalitis (?WHICH IS FALSE)
- characteristic inclusions
- ependymal & subependymal involvement
- May cause haemorrhage
Probably 3??
• in some immunodeficient patients, reactivation results in disseminated infection and/or severe necrotizing meningoencephalitis and ependymitis
• CMV may involve the CNS and/or peripheral nervous system
• Neurologic manifestations of CMV thus include acute or chronic meningoencephalitis, cranial neuropathy, vasculitis, retinitis, myelitis, brachial plexus neuropathy, and peripheral neuropathy
• The pathologic hallmark of CMV is the “owl’s eye,” an enlarged cell with a distended nucleus containing eosinophilic viral inclusions, surrounded by a halo, resulting in the characteristic appearance
• The owl’s eye appearance can be seen in ependymal cells, subependymal astrocytes, oligodendroglia, endothelial cells, and neurons.
• Ependymal involvement is quite common.
• Infrequently, CMV infection may result in extensive destruction of gray and white matter
• Other typical histopathologic findings in the CNS include well-circumscribed microglial nodules, and CMV intranuclear inclusions may also be found in the spinal cord, spinal nerves, and retina
• CT is frequently insensitive in the imaging of CMV encephalitis
• The most common manifestation on CT is atrophy
• Infrequently, white matter hypodensity may be apparent
• Ring-enhancing lesions have also been described
• CT grossly underestimates the degree of involvement by CMV
o In addition to atrophy and white matter hypodensity, periventricular and subependymal enhancement may be seen and is best visualized with a double-dose delayed technique
• MR has far greater sensitivity than CT in the detection of CNS CMV infection
o In addition to atrophy, MR may demonstrate increased signal on T2-weighted images in the periventricular white matter, which may be patchy and is less often confluent
o Infrequently, subependymal enhancement is evident and, if present, is a valuable diagnostic clue o Fat-suppressed MR with gadolinium may reveal a thickened enhancing choroid/retina in patients with CMV retinitis, a hemorrhagic retinitis affecting 20% to 40% of the AIDS population
- Sep03.18 CJD & variant CJD
- caused by a slow virus
- CJD patients live for <12 months, vCJD live for a few years.
- Associated with frontal atrophy.
- CJD patients live for <12 months, vCJD live for a few years.
- Sep03.28 Pilocytic Astrocytoma
- Associated with NF2
- 50% are solid
- Prognosis is less than 70% 5 year survival rate
- Rosenthal fibres
4. Rosenthal fibres • Rosenthal fibres often present = eosinophilic bodies within astrocyte processes • up to 100% 5YS • Rosenthal fibers present in o Pilocytic astrocytoma o glioma o pineal tumor o Alexander’s disease o long-standing gliosis
- Sep03.64 DNET , which one is right
- Intracortical
- degeneration of an anaplastic astrocytoma
- cystic change and haemorrhage
- Intracortical
- Sep03.65 Least common site for meningioma:
- adjacent to hippocampus
- parietal lobes
- between cerebrum & cerebellum
- adjacent to nose
- adjacent to nose
• Most common
o Hemispheric convexity (20%)
o Parasagittal (25%) → may occlude SSS
• Very common
o Sphenoid ridge, wing (15 – 20%) → may involve optic canal, wing meningiomas often en plaque Yock p26 Case 42
o Olfactory groove (5 – 10%)
• Common o Parasellar (5 – 10%) CR p79 Case 63 o Cavernous sinus CR p11 Case 7 • 66% partially or totally encase carotid artery • 33% narrow the artery
• Less common
o CPA, along clivus (posterior fossa – 10%) Yock p38 Case 63 TA p3 Case 1
o Tentorium cerebelli Yock p36 Case 59
o Foramen magnum Yock p38 Case 64
• Rare
o Optic nerve sheath (<2%) CR p155 Case 119
o Extracranial (nose, sinuses, skull (intraosseous))
o Intraventricular
• usually trigone of (L) lateral ventricle Yock p40 Cases67-70 • most common trigonal mass in adults
o Spinal canal (M : F = 1:10 )
• mostly thoracic region
o Sylvian fissure Yock p31 Case 51 • Paediatric age group
- Sep03.81 Berry aneurysm, most correct statement:
- majority occur at branch points
- 40% posterior circulation
- majority occur at branch points
• About 90% of berry aneurysms occur in the anterior circulation and are found near major arterial branch points.
• Multiple aneurysms exist in 20-30% of autopsy series.
- Sep03.88 Least likely site for hypertensive bleed in the brain is:
- hippocampus
- cerebellum
- basal ganglia
- thalamus
- hippocampus
- Necrosis is a characteristic feature of:
a. Pilocytic astrocytoma
b. Glioblastoma multiforme
c. Acoustic schwannoma
d. Craniopharyngioma
e. Ependymoma
Answer: GBM.
a. Pilocytic astrocytoma F - WHO grade I; bipolar cells with elongated hairlike processes; Rosenthal fibers; may have microscopically infiltrative margin; mural nodule may be highly vascular; often calcifications. Rarely malignant degeneration with hypercellularity, mitotic figures and necrosis.
b. Glioblastoma multiforme T - high grade astrocytoma (anaplastic, fibrillar astrocytes) with either coagulation necrosis or microvascular proliferation; with thickened vascular walls due to endothelial cell hyperplasia and hypertrophy; usually hypercellular with mitotic figures (some atypical), multinucleated tumor cells, bizarre nuclei, karyorrhectic cells; may have perinecrotic pseudopalisading of tumor cells around necrotic tumor.
c. Acoustic schwannoma F - interlacing fascicles; biphasic with Antoni A (cellular) and B (myxoid) patterns and Verocay bodies (palisading nuclei); no/rare mitotic figures. Cystic change may be due to intratumoral hemorrhage.
d. Craniopharyngioma F - Adamantinomatous (kids): relatively poorly circumscribed, nests and trabeculae of epithelium in fibrocollagenous stroma; nuclear palisading; often “wet” keratin, may undergo cystic degeneration, calcification, xanthogranulomatous reaction; cyst fluid contains cholesterol crystals, cholesterol clefts, reactive giant cells; variable necrosis, inflammation and Rosenthal fibers; no keratohyaline granules Papillary (adults): well-circumscribed, cores of fibrovascular stroma lined by well-diffd squamous epithelium that may form pseudopapillae.
e. Ependymoma F - solid or papillary, small blue fibrillar to epithelioid cells with granular chromatin; form perivascular rosettes and less commonly ependymal rosettes. [Path outlines]
- Solid mass involving the cortex of a temporal lobe is demonstrated on MRI. The most likely diagnosis is:
- DNET
- Pilocytic astrocytoma
- Anaplastic astrocytoma
- Mesial temporal sclerosis
- Pleomorphic xantho-astrocytoma
ANSWER:
1. DNET T - nodular intracortical lesion +/- cortical dysplasia, temporal lobe (62%).
- Solid mass involving the cortex of a temporal lobe is demonstrated on MRI. The most likely diagnosis is: (GC)
- DNET T - nodular intracortical lesion +/- cortical dysplasia, temporal lobe (62%).
- Pilocytic astrocytoma F - usually cystic with mural nodule, may be solid or diffusely infiltrative (eg. optic n.). Usually midline - cerebellum, 3rd V, thalamus-hypothalumus.
- Anaplastic astrocytoma F - found in hemispheres, usually frontal & temporal lobes.
- Mesial temporal sclerosis F -
- Pleomorphic xanthoastrocytoma F - most common in temporal lobe, superficial cortical location is typical, involves (attached to) leptomeninges. But is usually cystic (48%) with mural nodule; occasionally more diffuse with gyral infiltration. [Adelaide path notes, Dahnert]
- Least likely site for hypertensive bleed in the brain is:
- hippocampus
- cerebellum
- basal ganglia
- thalamus
- brainstem
ANSWER:1. Hippocampus
- Least likely site for hypertensive bleed in the brain is: (GC)
- Hippocampus
- Cerebellum F - 10%; behave like SOL’s - herniation & obstructive hydrocephalus.
- Basal ganglia F - with thalamus account for 65% of hypertensive bleeds
- Thalamus F - see above
- Brainstem F - 10%
- BBB – which is incorrect:
- H2O soluble medium cannot pass if normal BBB
- Fat soluble medium cannot pass if normal BBB
- Capillaries are continuous
- Continuous capillaries have no fenestrations
- Tight junctions are important
ANSWER:
2. Fat soluble medium cannot pass if normal BBB F - lipophilic solutes do not have a significant affinity for plasma proteins and are able to pass into the luminal plasma membrane lipid with ease, and hence into the brain.
- H2O soluble medium cannot pass if normal BBB T - solutes with high affinity for plasma water, low affinity for plasma proteins, and high extremely low partition coefficients do not penetrate normal BBB (eg. contrast media).
- Fat soluble medium cannot pass if normal BBB F - lipophilic solutes do not have a significant affinity for plasma proteins and are able to pass into the luminal plasma membrane lipid with ease, and hence into the brain.
- Capillaries are continuous T - this is the basis of the BBB. Other types are fenestrated (circumventricular organs), and sinusoidal (liver, spleen, bone marrow).
- Continuous capillaries have no fenestrations T - wall is composed of a single layer of endothelial cells connected by tight junctions, surrounded by a continuous basement membrane. It is the continuity of the interendothelial tight junctions, lack of pinocytosis and absence of fenestrations that contribute to the restricted permeability of the BBB.
- Tight junctions are important T - see above.
- Cortically based lesion, which is most likely:
a. Oligodendroglioma
b. Low grade glioma
c. Meningioma
d. Ependymoma
ANSWER:
a. Oligodendroglioma T - characteristically involves cortex and subcortical WM.
- Cortically based lesion, which is most likely: (GC)
a. Oligodendroglioma T - characteristically involves cortex and subcortical WM.
b. Low grade glioma F
c. Meningioma F - extra axial. Rarely atypical meningiomas may show sarcomatous transformation with spread over entire hemisphere and invasion of cerebral parenchyma (leptomeningeal supply).
d. Ependymoma F - supratentorial tumours frequently grow into brain parenchyma extending to the cortical surface, esp in frontal and parietal lobes. May see scalloping of inner table or invasion of overlying meninges/bone. [Dahnert]
- Which is least likely to involve the corpus callosum:
a. GBM
b. Marchiafava Bignami
c. DAI
d. Dandy Walker
e. Lymphoma
ANSWER: d. Dandy Walker F - assocd with dysgenesis of the CC in 20-25% (cf. primary involvemt)
- Which is least likely to involve the corpus callosum: (GC)
a. GBM T - most commonly spread via direct extension along WM tracts, including the CC - classic butterfly pattern.
b. Marchifava Bignami T - primarily affects the CC - acute form affects the genu & splenium, chronic form affects the body.
c. DAI T - classic triad of GW junction, dorsolateral brainstem, and CC (most commonly eccentrically and in the splenium).
d. Dandy Walker F - assocd with dysgenesis of the CC in 20-25% (cf. primary involvemt)
e. Lymphoma T - differ from GBM as usually less peritumoral oedema, less often necrotic, highly radiosensitive, frequently respond dramatically to steroids. [AJR 2002] Completed using stems from Aug 2006, previously only had DWS [Eric]
Associated anomalies include: o Chiari II malformation o Dandy-Walker complex o Neuronal migration disorders o Cephalocoeles o Schizencephaly o Interhemispheric lipoma – tubulonodular type o Azygous anterior cerebral arteries
- GBM, what is the most important feature in Dx:
- Necrosis
- Angiogenesis
- Cystic change
- Mitosis
- Vasogenic oedema
ANSWER:1. Necrosis T - high grade astrocytoma (anaplastic, fibrillar astrocytes) with either coagulation necrosis or microvascular proliferation
- GBM what is the most important feature in Dx: (GC)
- Necrosis T - high grade astrocytoma (anaplastic, fibrillar astrocytes) with either coagulation necrosis or microvascular proliferation
- Angiogenesis ? - microvascular proliferation is also a Dx feature
- Cystic change F - non specific feature
- Mitosis F - abnormal mitoses are also present in grade III (anaplastic) astrocytoma
- Vasogenic oedema F - non specific feature
WHO grading & Histological criteria:
I Pilocytic astrocytoma -
II Diffuse astrocytoma - 1 criterion, usually nuclear atypia
III Anaplastic astrocytoma - 2 criteria, usually nuclear atypia and mitoses
IV Glioblastoma - 3 criteria, usu nuclear atypia, mitoses, microvasc proliferation +/or necrosis
- Cavernous Angioma – which is is not typical:
- detectable at angiography
- Bleeding tendency
- No intervening brain tissue
- Pseudocapsule + surrounding hemosiderin laden macrophages
- Associated venous angioma
- Detectable at angiography F - most are angiographically occult due to slow flow.
- Cavernous Angioma – which is is not typical: (GC)
- Detectable at angiography F - most are angiographically occult due to slow flow.
- Bleeding tendency T - nearly all show evidence of recent and remote hemorrhage, as suggested by the presence of hemosiderin-laden macrophages, cholesterol crystals, and hemosiderin-stained parenchymal tissues.
- No intervening normal brain - this is the only histopathologic characteristic that distinguishes these lesions from capillary telangiectasias.
- Pseudocapsule + surrounding hemosiderin laden macrophages T - clots and blood products of various stages of evolution within the lesion, as well as calcification and gliosis surround the cavernoma, creating the appearance of a pseudocapsule.
- Associated venous angioma T - persistent elevations in venous pressures, as may be seen locally within a venous malformation, may promote a pathologic reactive angiogenesis or angiogenic proliferation, resulting in abnormal blood vessel growth and coalescence. [eMedicine]
- Which of the following is not a congenital CNS infection:
- Chickenpox
- Rubella
- CMV
- Toxoplasmosis
- Herpes
ANSWER:1. Chickenpox Dahnert includes it in “Other”, p232. ? T - Congenital varicella syndrome occurs in 2% of children born to women who develop varicella during the 1st or 2nd trimester of pregnancy. Manifests as IUGR, cortical atrophy, microcephaly, microphthalmia, cataracts, limb hypoplasia, skin scarring.
- Which of the following is not a congenital CNS infection: see Aug 2007 (GC)
- Chickenpox Dahnert includes it in “Other”, p232. ? T - Congenital varicella syndrome occurs in 2% of children born to women who develop varicella during the 1st or 2nd trimester of pregnancy. Manifests as IUGR, cortical atrophy, microcephaly, microphthalmia, cataracts, limb hypoplasia, skin scarring.
- Rubella T
- CMV T
- Toxoplasmosis T
- Herpes In utero infection is rare; 3% of these will have severe symptoms of skin vesicles/scarring, eye disease, microcephaphaly/hydranencephaly. HSV-2 is most commonly acquired during vaginal delivery (intrapartum), can also occur postnatally (horizontal transmission).
- Hashimoto’s – FNA findings
- Hurthle cells
- Fibrosing nodules
- Psammoma bodies
ANSWER:1. a. Hurthle cells -T - mononuclear inflammatory infiltrate containing small lymphocytes, plasma cells, and well-developed germinal centers. The thyroid follicles are small and are lined in many areas by epithelial cells with abundant eosinophilic, granular cytoplasm, termed Hurthle cells.1.
- Hashimoto’s – FNA findings (TW)
a. Hurthle cells -T - mononuclear inflammatory infiltrate containing small lymphocytes, plasma cells, and well-developed germinal centers. The thyroid follicles are small and are lined in many areas by epithelial cells with abundant eosinophilic, granular cytoplasm, termed Hurthle cells.
b. Fibrosing nodules - F - Reidel’s thyroiditis.
c. Psammoma bodies - F - in papillary thyroid carcinoma.
Concentrically calcified structures.= o ns = Hurthle cells seen in:
o Hashimotos
o Follicular adenoma
- Which is not a feature of Alzheimer’s:
- Hirano bodies
- Lewy bodies
- Senile Plaques
- Neurofibrillary tangles
- Granulovacuolar degeneration
- Amyloid
ANSWER:2. Lewy Bodies F - eosinophilic intracytoplasmic inclusions found in some neurones in Parkinson’s disease
- Which is not a feature of Alzheimer’s: (GC).
- Hirano bodies T - elongated glassy eosinophilic bodies of paracrystallline arrays of beaded filaments predominantly of actin, found mostly in hippocampal pyramidal cells in AD.
- Lewy Bodies F - eosinophilic intracytoplasmic inclusions found in some neurones in Parkinson’s disease.
- Senile Plaques T - aka neuritic plaques; focal spherical collections of dilated tortuous silver-staining neuritic processes (dystrophic neurites) surrounding a central amyloid core.
- Neurofibrillary tangles T - bundles of filaments in cytoplasm of neurons that displace or encircle the nucleus, ‘flame-shaped’, seen well with silver staining (especially in cortical neurones). Characteristic but not specific to AD; also seen in PSNP, postencephalitic PD, ALS-parkinsonian-dementia complex of guam (!)
- Granulovacuolar degeneration T - small clear intraneuronal cytoplasmic vacuoles, each containing a granule.
- Amyloid T - almost an invariable accompaniment of AD. Beta-amyloid and its precursor protein (APP) are implicated in the aetiopathogenesis.
- PNET, which is the most typical appearance:
- Cortical
- Angiogenesis
- Cystic
- Vasogenic oedema
- Astrocytoma
ANSWER:2. Angiogenesis T - WHO grade IV. Supratentorial PNETs had highly branched capillaries with extensive endothelial cell hyperplasia. Glomeruloid arrays of microvessels extended from the capillaries. Small fragments of endothelial tubes were scattered throughout the tumor. [Different vascular patterns of medulloblastoma & supratentorial PNETs. Internat J of Dev Neuroscience 1999]
- PNET, which is the most typical appearance: (GC) Eric had ‘cystic’ for this answer
- Cortical F - deep cerebral white matter, most commonly frontal lobe.
- Angiogenesis T - WHO grade IV. Supratentorial PNETs had highly branched capillaries with extensive endothelial cell hyperplasia. Glomeruloid arrays of microvessels extended from the capillaries. Small fragments of endothelial tubes were scattered throughout the tumor. [Different vascular patterns of medulloblastoma & supratentorial PNETs. Internat J of Dev Neuroscience 1999]
- Cystic F - large (hemispheric) heterogeneous mass with tendency for necrosis (65%), cyst formation, calcification (71%), haemorrhage (10%).
- Vasogenic oedema F - thin rim of oedema
- Astrocytoma [Dahnert] (–)Location - Supratentorial, most commonly located in frontal, parietal lobes next to lateral ventricle - Can be intraventricular - May include olfactory neuroblastoma (esthesioneuroblastoma) Morphology - Large bulky hemispheric mass with necrosis, cyst formation, haemorrhage & prominent vessels, densely cellular - Histol: small undifferentiated cells, Homer Wright pseudorosettes rarely Psammoma bodies: a form of dystrophic calcification. A necrotic cell forms a focus for crystal deposition and layers form around it. Associated with: Papillary carcinoma Renal cell carcinoma Meningioma Female genital tract tumours Breast cancer
- In HSV I encephalitis, which is least correct
- Age 50-70 years
- Typically involves superior frontal lobes
- Commonest presentation is headache
- May present with seizures, ataxia and lethargy
ANSWER:2. Typically involves superior frontal lobes
- In HSV I encephalitis, which is least correct
- Age 50-70 years
- Typically involves superior frontal lobes
- Commonest presentation is headache
- May present with seizures, ataxia and lethargy
- In alcoholic with hyponatremia and partial paresis, which is most likely
- Central pontine myelinosis
- Pontine myelinosis, midbrain and supratentorial changes
- Pontine lesion secondary to Thiamine deficiency
- Subacute combined degeneration of cord secondary to thiamine deficiency
- Cord degeneration secondary to folate deficiency
- Hashimoto’s thyroiditis
Some people say 1, some 2
1. Central pontine myelinosis - F - central symptoms ALOC, pseudobulbar palsy, dysarthria, dysphagia.
- Pontine myelinosis, midbrain and supratentorial changes - T - probably best answer :Extrapontine myelinolysis (now known as - along with CPM - osmotic demyelination syndrome). Movement problems, along with the CPM problems of pseudobulbar palsy, dysarthria, dysphagia, ALOC.
- Pontine lesion secondary to Thiamine deficiency - F - wernickes encephalopathy tends to be symmetrical distribution in structure surrounding 3rd ventricle, aqueduct, and 4th ventricle. Typically mamillary bodies, periaqueductal gray matter, hypothalamus, and thalami adjacent to 3rd ventricle.
- Subacute combined degeneration of cord secondary to thiamine deficiency - F - this would be best answer if was cobalamin (Vit B12) deficiency and not thiamin (B1) in regards to subacute combined degeneration. Cobalamin deficiency can cause paraesthesia of lower limbs, ataxia, to the extent of complete paraplegia. Realistically, the clinical presentation is probably related to the PFO + head trauma + coagulopathic + bleed.
- Cord degeneration secondary to folate deficiency - F - deficiency of both cobalamin (B12) and folic acid produce megaloblastic anemia, but only B12 deficiency produces neurologic changes.
- Hashimoto’s thyroiditis - F
- Child with posterior paravertebral mass, biopsy shows mature neural elements and Schwann cells not attached to nerve – diagnosis is
- Ganglioneuroma
- Ganglioglioma
- Neurofibroma
- Schwannoma
- Neuroblastoma
ANSWER:1. Ganglioneuroma - T - The most well-differentiation lesions (in the neuroblastoma spectrum) - see ganglion cells and Schwann cells, and neuroblasts are no longer present.
- Child with posterior paravertebral mass, biopsy shows mature neural elements and Schwann cells not attached to nerve – diagnosis is (TW)
- Ganglioneuroma - T - The most well-differentiation lesions (in the neuroblastoma spectrum) - see ganglion cells and Schwann cells, and neuroblasts are no longer present.
- Ganglioglioma - F - tumor of neoplastic astrocytes (rarely oligodendrocytes) and ganglion cells.
- Neurofibroma - F - PNST arise from cells of the peripheral nerve (Schwann cells, perineural cells, fibroblasts) - attached to nerve, but can be separated from it
- Schwannoma - F - PNST - see ans 3.
- Neuroblastoma - F- small, primitive-appearing cells with dark nuclei, scant cytoplasm, and poorly defined cell borders growing in solid sheets. Certain NBs may have some degree of differentiation with clusters of larger cells resembling ganglion cells.
- Child with mass FNA shows small round blue cells- least likely diagnosis is
- Neuroblastoma
- Ewing’s sarcoma
- Rhabdomyosarcoma
- Wilms tumour
*LW:
hopefully incomplete recall, as technically all of the 4 listed options can be classified as small round blue cells.
Wilms tumour can be included within this category, with blastemal cells representing small round blue cells.
ANSWER:4. Wilms tumour - F - classic Wilms tumor comprised of 3 cell types - Blastemal cells (undifferentiated cells), Stromal cells (immature spindle cells and heterologous skeletal, cartilage, osteoid, or fat), and epithelial cells (Glomeruli and tubules).
- Child with mass FNA shows small round blue cells- least likely diagnosis is (TW)
- Neuroblastoma - T - small, primitive-appearing cells with dark nuclei, scant cytoplasm, and poorly defined cell borders growing in solid sheets.
- Ewing’s sarcoma - T - monotonous sheets of small round blue cells with hyperchromatic nuclei and scant cytoplasm.
- Rhabdomyosarcoma - T - subtypes Botryoid and spindle cell (leiomyomatous / Embryonal / Alveolar / Undifferentiated. Histo of embryonal and alveolar types - cells have scant cytoplasm and a centrally placed round nucleus that occupies the majority of the cell.
- Wilms tumour - F - classic Wilms tumor comprised of 3 cell types - Blastemal cells (undifferentiated cells), Stromal cells (immature spindle cells and heterologous skeletal, cartilage, osteoid, or fat), and epithelial cells (Glomeruli and tubules).
- Retinoblastoma - T - sheets, trabeculae and nests of small blue cells with scant cytoplasm.Ewing’s sarcoma family of tumors (EFT) includes Ewings sarcoma, extraosseous Ewing’s sarcoma, more differentiated neuroectodermal tumors (PNET: previously AKA neuroepithelioma, adult neuroblastoms, Askin’s tumor of chest wall).
- Definition of Hamartoma is
- Abnormal disorganised tissue in abnormal position
- Abnormal disorganised tissue in normal position
- Normal disorgansied tissue in normal position
- Normal disorganised tissue in abnormal position
ANSWER:3. Normal disorganised tissue in normal position - T - cellular elements are mature and identical to those found in remainder of organ, but do not reproduce the normal architecture of the surrounding tissue. Tumor-like malformation with tissues of particular part of body arranged haphazardly, usually with excess of one or more of its components.
- Definition of Hamartoma is (TW)
- Abnormal disorganised tissue in abnormal position
- Abnormal disorganised tissue in normal position
- Normal disorganised tissue in normal position - T - cellular elements are mature and identical to those found in remainder of organ, but do not reproduce the normal architecture of the surrounding tissue. Tumor-like malformation with tissues of particular part of body arranged haphazardly, usually with excess of one or more of its components.
- Normal disorganised tissue in abnormal position
Examples of hamartomas: hamartoma of lung, vascular hamartomas (hemangioma, lymphangiomas), melanotic hamartoma (melanocytic naevus, spitz naevus, halo naevus, dysplastic naevus, blue naevus), skeletal hamartoms (exostoses), generalised hamartomatous dysplasias (NF, TS).
- Cystic tumour in brain, least likely is
- Hemangioblastoma
- JPA
- PNET
- Meningioma
- Schwannoma
ANSWER:4. Meningioma - F - extraaxial. Necrosis, cysts, haemorrhage may be seen in 8-23%. Can have assoc trapped CSF clefts
- Cystic tumour in brain, least likely is (TW).
- Hemangioblastoma - T - typically adult with intra-axial posteior fossa mass with cyst, enhancing mural nodule abutting pia.
- JPA - T - common cystic lesion with mural nodule in children
- PNET - T - PNET-MB - most common PF tumor in children (debatable vs JPA). Small intratumoral cysts / necrosis 40-50%.
- Meningioma - F - extraaxial. Necrosis, cysts, haemorrhage may be seen in 8-23%. Can have assoc trapped CSF clefts.
- Schwannoma - T - peripheral nerve sheath tumor which can be cystic and intraparenchymal. 1-2% intracerebral (superficial or periventricular location, cerebral hemisphere is most common site). Intracerebral schwannoma = cyst with nodule.Osborn.
- Multiple sclerosis, least likely finding is
- Cerebellar white matter
- Cerebellar vermis
- Corpus callosum
- Peri venular
ANSWER:2. Cerebellar vermis - F
- Multiple sclerosis, least likely finding is (TW
)1. Cerebellar white matter - T - cerebellar peduncles are classic. - Cerebellar vermis - F
- Corpus callosum - T - typically mutliple perpendicular callososeptal T2 hyperintensities.
- Peri venular - T - perpendicular lesions in perivenular distribution. >85% lesions periventricular / perivenous. 50-90% callososeptal interface. Perivenular extnsion “Dawson finger” along path of deep medullary veins.
- Mycotic aneurysms, most correct answer
- Rarely bleed
- Peripheral arterial
- Circle of Willis
- Peripheral arterial - T - peripheral to first bifurcation of major vessel (45%); often located near surface of brain especially over convexities.
- Patient with drug resistant Parkinson’s and autonomic neuropathy, most correct option
- Shy Drager
- Drug resistant Parkinson’s
- Striatonigral degeneration
- Olivopontocerebellar atrophy
- Progressive supranuclear palsy
ANSWER:1. 1. Shy Drager - T - known as multiple system atrophy. Sky-Drager syndrome still used sometimes for MSA when primary symptoms are autonomic failure. MSA - striatonigral degeneration is clinically similar to idiopathic PD in presentation, but is relatively resistent to L-dopa treatment (due to pattern of neuronal degeneration - ie both dopaminergic projections and its target neurons are absent, therefore L-dopa cannot bulster neurotransmission as occurs in parkinsons disease).
- Patient with drug resistant Parkinson’s and autonomic neuropathy, most correct option (TW)ANSWER:1. 1. Shy Drager - T - known as multiple system atrophy. Sky-Drager syndrome still used sometimes for MSA when primary symptoms are autonomic failure. MSA - striatonigral degeneration is clinically similar to idiopathic PD in presentation, but is relatively resistent to L-dopa treatment (due to pattern of neuronal degeneration - ie both dopaminergic projections and its target neurons are absent, therefore L-dopa cannot bulster neurotransmission as occurs in parkinsons disease).
- Drug resistant Parkinson’s - F - autonomic neuropathy too.
- Striatonigral degeneration - F - can see in PD and MSA
- Olivopontocerebellar atrophy - F
- Progressive supranuclear palsy - F
- Pick’s disease, uncommon findings
- Asymmetrical atrophy
- Predominant frontal lobes
- Cortical atrophy
- Involvement of post ⅔ superior temporal gyrus & parietal lobe
ANSWER:4. Involvement of post ⅔ superior temporal gyrus & parietal lobe - F - spared posterior aspect of superior temporal gyrus and pre- and postcentral gyri. Unremarkable parietal and occipital lobes.
Picks disease / Frontotemporal dementia - nonspecific songioform degneration, with gliosis and neuronal loss, sometimes with Pick cells and bodies. 25-40% of FTD is familial. 10-30% of patients with positive family history have tau mutations (Tauopathy).
- Pick’s disease, uncommon findings (TW)
- Asymmetrical atrophy - T - worse atrophy of dominant hemisphere
- Predominant frontal lobes - T - anterior frontotemporal atrophy.
- Cortical atrophy - T - thin cortex. Gliosis of corticl gray matter. Soft, retracted subcortical white matter. Almost complete loss of large pyramidal neurons, diffuse spongiosis and gliosis.
- Involvement of post ⅔ superior temporal gyrus & parietal lobe - F - spared posterior aspect of superior temporal gyrus and pre- and postcentral gyri. Unremarkable parietal and occipital lobes.Picks disease / Frontotemporal dementia - nonspecific songioform degneration, with gliosis and neuronal loss, sometimes with Pick cells and bodies. 25-40% of FTD is familial. 10-30% of patients with positive family history have tau mutations (Tauopathy).
- Periventricular mass in patient renal transplant
- Primary lymphoma
- GBM
- Secondary lymphoma
- Primary lymphoma - T - most notable risk factor for development of primary CNS lymphoma is immunodeficiency. Solid organ transplant patients have 30-50x higher incidence of lymphoproliferative disorder cf general population.
- 40 year old female with stroke, underlying cause least likely is
- Atherosclerosis
- Dissection
- Coarctation of aorta
- Giant cell arteritis
- Mitral valve prolapse
ANSWER:4. Giant cell arteritis - F - GCA is a chronic vasculitis of large and medium sized vessels. Mean age at Dx is approx 72yo, and the disease essentially never occurs in individuals younger than 50yo (UpToDate).
- 40 year old female with stroke, underlying cause least likely is (TW)
- Atherosclerosis - T
- Dissection - T
- Coarctation of aorta - T- congenital or acquired (eg inflammatory diseases of aort such as Takayasu). Previously undiagnosed adults - classic presenting sign is hypertension.
- Giant cell arteritis - F - GCA is a chronic vasculitis of large and medium sized vessels. Mean age at Dx is approx 72yo, and the disease essentially never occurs in individuals younger than 50yo (UpToDate).
- Mitral valve prolapse - T - most common congenital cause of MR in adults. Natural history of MVP is generall benign, but serious complications do occur; the most common are infective endocarditis, CVAs, need for MV surgery, death.
September 2003 1. Sep03.26 Atypical Scenario
- Craniopharyngioma in a 42 year old
- Anaplastic thyroid cancer in a 29 year old
- Bowel cancer in a 32 year old
- Cholangiocarcinoma in a young adult with emphysema
ANSWER:2. Anaplastic thyroid cancer in a 29 year old - F - older patients, mean age 65yo.
- Sep03.26 Atypical Scenario (TW)1. Craniopharyngioma in a 42 year old - T - Bimodal age distribution (peak 5-15yo; papillary craniopharyngioma >50y).
- Anaplastic thyroid cancer in a 29 year old - F - older patients, mean age 65yo.
- Bowel cancer in a 32 year old - T - peak incidence for CRC is 60-70yo. CRC in a young person, preexisting UC or one of teh polyposis syndromes must be suspected.
- Cholangiocarcinoma in a young adult with emphysema - T - a-1-antitrypsin deficency predisposes to cholangiocarcinoma.
- Sep03.28 Pilocytic Astrocytoma
- Associated with NF2
- 50% are solid
- Prognosis is less than 70% 5 year survival rate
- Multipolar cells with microcysts, and bipolar cells with Rosenthal fibres
ANSWER:4. Multipolar cells with microcysts, and bipolar cells with Rosenthal fibres - T - classic “biphasic” pattern of two astrocyte populations: compacted biplar cells with Rosenthal fibers (electron dense GFAP staining cytoplasmic inclusions); Loose-textured multipolar cells with microcysts, eosinophilic granular bodies.Expanded option 4. Previous said “…… fibers (this is correct ans)”
- Sep03.28 Pilocytic Astrocytoma (TW)
- Associated with NF2 - F - NF1. 15% of NF1 patients develop PAs (most commonly in optic pathway). Upt o 1/3 of patients with optic pathway PAs have NF1.
- 50% are solid - F - 40% solid with necrotic center, heterogeneous enhancement. 10% solid, homogeneous. 50% non enhancing cyst with enhancing mural nodule.
- Prognosis is less than 70% 5 year survival rate - F - median survival rates at 20y >70%
- Multipolar cells with microcysts, and bipolar cells with Rosenthal fibres - T - classic “biphasic” pattern of two astrocyte populations: compacted biplar cells with Rosenthal fibers (electron dense GFAP staining cytoplasmic inclusions); Loose-textured multipolar cells with microcysts, eosinophilic granular bodies.Expanded option
- Previous said “…… fibers (this is correct ans)”
- Sep03.41Retinoblastoma, which is the least likely ?
- Very radio sensitive. Excellent prognosis even if it extends retro-orbitally
- Carriers of RB gene have a 90% risk
- Can get extraocular Retinoblastomas
ANSWER:1. Very radio sensitive. Excellent prognosis even if it extends retro-orbitally - F - enuleation usually is indicated for large tumors with not visual potential, blind, painful eyes, and/or tumors that extend into the optic nerve. External beam XRT was original globe-sparing treatment for Rb. Risk of tumor recurrence following ext XRT 7%, occurring within 40months. Also risk of secondary cancers with XRT. UpToDate
- Sep03.41Retinoblastoma, which is the least likely ? (TW).
- Very radio sensitive. Excellent prognosis even if it extends retro-orbitally - F - enuleation usually is indicated for large tumors with not visual potential, blind, painful eyes, and/or tumors that extend into the optic nerve. External beam XRT was original globe-sparing treatment for Rb. Risk of tumor recurrence following ext XRT 7%, occurring within 40months. Also risk of secondary cancers with XRT. UpToDate.
- Carriers of RB gene have a 90% risk - T - If a mutant RB allele arises in the germ line, it can be transmitted as a dominant trait, and carriers are at high risk (>90% risk for most mutations) for retinoblastoma. Robbins.
- Can get extraocular Retinoblastomas - T - trilateral RB = bilateral RB with neuroectodermal pineal tumor. Quadrilateral RB = trilateral RB with 4th focus in suprasellar cistern. Dahnert 6th.
- Sep03.64 DNET:
- Intracortical
- Cystic
- Angiogenesis
- WHO grade II
- Frontal lobe
ANSWER:1. Intracortical T - well-demarcated “bubbly” cortical mass
- Sep03.64 DNET: (TW & GC)
- Intracortical T - well-demarcated “bubbly” cortical mass
- Cystic F - pseudocystic imaging appearance due to hypodensity. Histology may show “floating neurons” + mucinous degeneration.
- Angiogenesis F - pathological hallmark is the “specific glioneuronal element”: columns of heterogeneous cells oriented perpendicular to cortex. Also glial nodules and cortical dysplasia.
- WHO grade II F - grade I
- Frontal lobe F - temporal lobe 62%, frontal lobe 31%[Adelaide notes, Dahnert, RG 2001]
- Sep03.65 Least common site for meningioma:
- adjacent to hippocampus
- parietal lobes
- between cerebrum & cerebellum
- adjacent to nose
- adjacent to nose - Atypical location.
Ectopic = extradural meningioma: <1% cases, intradiploic space, outer table of skull, scalp, paranasal sinus, parotid gland, parapharyngeal space, mediastinum, lung, adrenal gland.
Types of meningioma:
- Globular meningioma (most common): compacted roundd mass with invagination of brain; flat at base; contact to falx / tentorium / basal dura / convexity dura.
- Meningioma en plaque: pronounced hyperostosis of adjacent bone particularly along base of skull; difficult to distinguish hyperostosis from tumor cloaking the inner table
- Multicentric meningioma (2-9%): tendency to localize to a single hemicranium; present clinically at earlier stage. CSF seeding is exceptional; in 50% assoc with NF2.
- Sep03.74 Paragangliomas. Which is false
- paraganglioma, chemodectoma and carotid body tumors can be used interchangeably
- often adherent to vessels resulting in incomplete excision and recurrence of 10%
- glomus jugulare and carotid body paraganglioms are the most common head and neck paragangliomas
- paraganglioms have biphasic or biphenotypic pattern and composed of chief cells and sustentacular cells
ANSWER: 1.1. paraganglioma, chemodectoma, and carotid body tumors can be used interchangeably - F - multiple names: glomus tumor, chemodectoma, endothelioma, perithelioma, sympathoblastoma, fibroangioma, sympathetic nevi. Paragangliomas are classified based on their location, innervation, and microscopic appearance. Would need to specify location for paraganglioma / chemodectoma to be able to use interchangeably with carotid body tumor.
- Sep03.74 Paragangliomas (TW)ANSWER:
- paraganglioma, chemodectoma, and carotid body tumors can be used interchangeably - F - multiple names: glomus tumor, chemodectoma, endothelioma, perithelioma, sympathoblastoma, fibroangioma, sympathetic nevi. Paragangliomas are classified based on their location, innervation, and microscopic appearance. Would need to specify location for paraganglioma / chemodectoma to be able to use interchangeably with carotid body tumor.
- carotid body tumors often adherent to vessels resulting in incomplete excision and recurrence of 10% - T - Shamblin classification - Type I are localized and easily removed; type II adherent and partially surround carotid vessels; type III adherent and completely surround carotid vesels and extremely difficult to resect often requiring resection of ICA and vein graft interposition. Prevalence of local recurrence and local invasion - 40-50% of glomus jugulare tumors, 17% for vagal paragangliomas, and about 10% for carotid body tumors.
- glomus jugulare and carotid body paragangliomas are the most common head and neck paragangliomas - T - conflicting reports regarding the prevalence of these 2 subtypes some saying one is more prevalent, some saying the other is.
- paragangliomas have biphasic or biphenotypic pattern and composed of chief cells and sustentacular cells - T
- Sep03.17 CMV encephalitis: which is false?
- characteristic inclusions
- ependymal & subependymal involvement.
- may cause haemorrhage
- majority of newborns hae systemic signs of disease, of which about half have CNS involvement
ANSWER:4. majority of newborns have systemic signs of disease, of which about half have CNS involvement - F - most infected newborns appear normal. 10% have systemic signs of disease (hepatosplenomegaly, petechiae, chorioretinitis, jaundice, and IUGR). 55% with systemic disease have CNS involvement (microcephaly, parenchymal Ca+, SNHL, seizures, hypotonia or hypertonia).
- Sep03.17 CMV encephalitis: which is false? (TW)
- characteristic inclusions - T - CMV inclusion-bearing cells. Prominent cytomegatic cells with intranuclear and intracytoplasmic inclusions can be readily identified. Hallmark is cytomegaly with viral nuclear and cytoplasmic inclusions.
- ependymal & subependymal involvement - T - may affect any cell type by striking tendency for virus to localise in teh epehdymal and subependymal regions of brain. Replicates in ependyma, germinal matrix, and capillary endothelia.
- may cause haemorrhage - T - results in severe necrotising ventriculo-encephalitis with massive necrosis, haemorrhage, ventriculitis and choroid plexusitis
- majority of newborns have systemic signs of disease, of which about half have CNS involvement - F - most infected newborns appear normal. 10% have systemic signs of disease (hepatosplenomegaly, petechiae, chorioretinitis, jaundice, and IUGR). 55% with systemic disease have CNS involvement (microcephaly, parenchymal Ca+, SNHL, seizures, hypotonia or hypertonia).
- Sep03.18 CJD & variant CJD
- caused by a slow virus
- CJD patients live for <12 months, vCJD live for a few years.
- Associated with marked frontal atrophy.
CJD patients live for <12 months, vCJD live for a few years - T - mean duration illness for vCJD 14months, for CJD 4-5months. Both diseases are progressive and uniformly fatal
- Sep03.18 CJD & variant CJD (TW)
- caused by a slow virus - F - rapidly progressing, fatal, potentially transmissible dementing disorder caused by a prion (proteinaceous infectious particle devoid of DNA and RNA)
- CJD patients live for <12 months, vCJD live for a few years - T - mean duration illness for vCJD 14months, for CJD 4-5months. Both diseases are progressive and uniformly fatal
- Associated with marked frontal atrophy - F - get abnormal signal in predominantly gray matter - Basal ganglia (caudate nuclei, putamina, and to lesser extend globi pallidi) thalami. Cerebral cortex (most commonly frontal and temporal lobes). Mild cortical atropy - diffuse or confined to affected structures. Ventricular enlargement.
- Sep03.63 IV contrast, 4 -12 hrs afterwards, it results in skin necrosis. It’s a repeatable response. What type of reaction is it?
- Type 1 hypersensitivity
- Type 2
- Type 3
- Type 4
- Non-immune reaction
ANSWER: 3. Type 3 antigen antibody complex
- Sep03.63 IV contrast, 4 -12 hrs afterwards, it results in skin necrosis. It’s a repeatable response. What type of reaction is it? (TW)
- Type 1 hypersensitivity - F- Anaphylactic type. Rapidly developing immunologic reaction developing within minutes after combination of antigen with antibody bound to mast cells or basophils in individuals previously sensitized to antigen
- Type 2 - F - Cytotoxic type. Mediated by antibodies directed toward antigens present on surface of cells or other tissue component (subtypes: complement-dependent reactions; antibody-dependent cell mediated cytotoxicity; antibody mediated cellular dysfunction)
- Type 3 - T - Immunocomplex disease. Induced by antigen-antibody complexes that produce tissue damage as a result of their capacity to active the complement system. Can be generalized (immune complexes formed in circulation adn deposited in many organs etc), or localized to particular organs such as kidney (GN), joints (arthritis) or the small blood vessels of the skin if the complexes are formed an deposited locally (the local Arthus reaction). See below.
- Type 4 - F - Cell mediated (delayed). Initiated by specifically sensitized T lymphocytes.
- Non-immune reaction - F - unless someone is repeatedly extravasating a lot of contrast! Maybe fun, but not ethical.Local immune complex disease (Arthus reaction)
- type III hypersensitivity reaction. Localized area of tissue necrosis resulting from acute immune complex vasculitis, usually elicited in the skin. Reaction can be produced by intracutaneous injection of antigen in an immune patient having circulating antibodies against the antigen. Unlike IgE mediated type I reactions, which appear immediately, the Arthus lesion develops over a few hours and reaches a peak 4-10hrs after injection, when it can be seen as an area of visible oedema with severe hemorrhage followed occasionally by ulceration.
