1
Q
Neural tube defects are characteristically associated with increased in concentration of what substance in amniotic fluid or maternal serum
A
A-fetoprotein
2
Q
Neural tube defects is associated with decreased in intake of what substance
A
Folic acid/folate/ vit b9
3
Q
Failure of posterior vertebral arch to close
A
Spina bifida
4
Q
A type of spina bifida in which there is defect only of the vertebral arch with intact neural/meningeal tissue
A
Spina bifida occulta
*most common and mildest form
5
Q
Type of spina bifida that involves defect on the vertebral arch with cystic-like sac protrusion of meninges and/or neural tissue
A
Spina bifida cystica
6
Q
Herniated membranes consisting of meninges only
A
Meningocele
7
Q
Herniated tissue consisting of CSF, meninges, and neural tissue
A
Myelomeningocele
8
Q
TORCH complex
A
Toxoplasmosis
Rubella
Cytomegalovirus
Herpes Simplex Virus
9
Q
Torch syndrome characteristic findings
A
-microcephaly
-chorioretinitis
-CNS clacification
-petechial rash
-hepatosplenomegaly
-thrombocytopenia
10
Q
A type of hydrocephalus without obstruction or increased production of csf rather is cause by disorders characterized by decreased cerebral mass (eg Ischemic brain atrophy or advanced Alzheimers disease)
A
Hydrocephalus ex vacuo
11
Q
A form of hydrocephalus where increased volume of CSF is entirely within the ventricles
A
Internal hydrocephalus
12
Q
A form of hydrocephalus where increased volume of CSF is confined to the subarachnoid space
A
External hydrocephalus
13
Q
A form of hydrocephalus where there is free flow of CSF between the ventricles and the subarachnoid space
A
Communicating hydrocephalus
14
Q
A form of hydrocephalus where there is obstructed flow of CSF from the ventricles to the subarachnoid space
A
Non-communicating hydrocephalus
15
Q
This is a downward displacement of the cerebellar tonsils and medulla through the foramen magnum
A
Arnold-chiari malformation
16
Q
Thoracolumbar meningomyelocele is almost always a a characteristic of what disease
A
Arnol-chiari malformation
17
Q
Fetal alcohol syndrome characteristics
A
-facial abnormalities
-microcephaly
-atrial septal defect
-mental and growth retardation
-other anomalies.
18
Q
Seizures and mental retardation beginning in infancy is a characteristic of this autosomal dominant disease that presents with adenoma sebaceum of the skin and angiomyolipoma of the kidney
A
Tuberous sclerosis syndrome
19
Q
What is the most common site of thrombotic occlusion
A
Carotid bifurcation and MMA
20
Q
Most frequent site of embolic occlusion
A
MMA
21
Q
Pure sensory lacunar stroke most often results from lesions affecting what part of the brain
A
Thalamus
22
Q
Pure motor lacunar stroke most often results from lesions affecting the
A
Internal capsule
23
Q
Intracerebrak hemorrhage is associated with what aneurysm
A
Charcot-bouchard aneurysms
24
Q
Most common site of ICH
A
Basal ganglia
25
Subarachnoid hemorrhage is commonly associated with
Berry aneurysm
26
Causes of SAH
-AV malformations
-trauma
-hemorrhagic diatheses
27
Most frequent cause of ICH
- hypertension
28
Brain injury at the site of impact
Coup injury
29
Injury on the opposite of the brain from the site of impact
Countercoup injury
30
Portal of entry of infection into the CNS are via
-hematogenous spread (m/c)
-trauma
-local spread from paranasal sinuses,dental infections
-peripheral nerves
31
Triad of pyogenic meningitis
-fever
-headache
-nuchal rigidity
32
Resulting conditions from pyogenic meningitis
-reactive fibroblastic arachnoiditis
-obliteration of CSF flow
-hydrocephalus
33
What is characteristic finding of pyogenic meningitis
Purulent exudate in the subarachnoid space
34
CSF findings of pyogenic meningitis
-numerous neutrophils
-low glucose (less than 2/3 of s. Glucose conc)
-high protein
35
Toxoplasmosis in immunocompetent adults most often manifests as
Lymphadenitis
36
Toxoplasmosis in immunocompromised most often presnts with
CNS involvment
37
CSF findings in Viral meningitis
-lymphocyte
-moderately elevated protein
-normal glucose conc
38
Morphologic changes in the brain substance of persons with meningoencephalitis or encephalitis
-perivascular cuffing ( infiktrate of mononuclear cells in the Virchow-Robin spaces
-Inclusion bodies
-Glial nodules
39
Characteristic microscopic findings of CMV
Giant cells with eosinophilic inclusions involving both nucleus and cytoplasm
40
CMV causes what associated disease
Encephalomyelitis
Lesions of the kidneys,liver,lungs, and salivary glands
41
Caused by infectious protein particles, and are considered infectious and transmissible, devoid of DNA or RNA, and resistant to heating and other methods used for inactivation
Prion diseases
*long incubation period “ slow virus dses”
*progressive course
42
Prion disorders anatomical findings
Spongiform encephalopathy (clusters of small cysts in CNS gray matter) along with absence of inflammatory respose
43
44
Prion dses transmitted by ritual ingestion if human brain by cannibals in New Guinea
Kuru
45
Kuru characteristic findings
Morphologic:
-gliosis
-loss of neurons
-spongiosis of cerebrum, cerebellum and sc
-cerebellar atrophy
S&sx:
-cerebellar ataxia
-tremor
-slurred speech
-progressive mental deficience
-death after few mos
46
47
What are the prion diseases in humans
-kuru
-Creutzfeldt-jakob dse (subacute spongiform encephalitis)
-Gertsmann-straussler scheinker syndrome
-fatal familial insomnia
48
Prion disease that is hazardous to health workers working with brain specimen, those who had putative corneal transplantation, ingestion of beef products from cattle affected by mad cow disease
Creutzfeldt-Jacob disease (subacute spongiform encephalopathy)
49
Morphologic finding of Creutzfeldt-Jacob disease
-loss of neurons
-gliosis
-spongiosis (prominent)
50
Type of slow virus infection cause by altered measles virus and is usually fatal
Subacute Sclerosing Panencephalitis (SSP)
51
What characteristic of measles virus predisposes to SSP
-lack of M-protein (protein required for extracellular spread of virus)
52
What slow virus infections with CSF findings of (+) oligoclonal immunoglobulins against viral proteins but lacks “anti-M”
SSP
53
Slow virus infection cause by JC polyoma type of papovavirus
Progressive multifocal leukoencephalopathy (PML)
54
What structure is preferrentially infected by JC virus thus causing demyelination
Oligodendrocytes
55
PML is most associated with what disease
-leukemia
-lymphoma
-immunodeficiency
56
Epidemiology of Multiple Sclerosis
-most common demyelinating disease
-begins between 20-30
-women
-unknown cause: genetic and environmental
(1) increased CSF immunoglobulins (multiple oligloconal bands on electrophoresis)
(2) HLA haplotypes (A3,B7,DR2,DW2)
(3) northern European origin
(4) incidence directly proportional to the geographic distance from the equator
57
Morphologic changes in MS
-confined to CNS ONLY, PNS NOT AFFECTED
- depletion of oligodendrocyte
-multile focal areas of demyelination (plaques) irregularly scattered in the brain and SC
-favored sites: optic nerve,brain stem, paraventricular areas
-helper CD4+ and CD8+ T lymphocytes and macrophage invades the plaque
-reactive gliosis (later)
58
Manifestation of MS
Early
-weakness of LE
-visual disturbances
-retrobulbar pain
-sensory disturbances
-loss of bladder control
Charcot’s TRIAD (S-I-N)
1. Scannning speech
2. Intentional tremors
3. Nystagmus
59
Acute disseminated encephalomyelitis also known as
Post-infectious encephalitis
* follows a viral illness such as measles,mumps, rubella, chickenpox
60
Demyelinating diseases
-Multiple sclerosis
-Acute disseminated encephalomyelitis
-Guillain Barre Syndrome
61
Characteristic of GBS
-affects the PNS
-young adults
-often precede viral infection, immunization, or allergic reactions
-ascending muscle weakness
-respiratory failure and death
-albumin-cytologic dissociation (inc protein conc with only modest increase in cell count)
62
An important diagnostic finding of albumin-cytologic dissociation is seen in what demyelinating disease
GBS
63
Degenerative brain diseases
-Alzheimer disease
-Pick disease
-Huntington disease
-Idiopathic Parkinson Disease (paralysis agitans)
-Amyotrophic Lateral Sclerosis (ALS)
64
Anatomic changes in Alzheimers disease
-gen cerebral atrophy (frontal and hippocampus; widened sulci, narrowwing of gyri)
-neurofibrillary tangles
-amyloid angiopathy: Beta-amyloid
-neurotic (senile) plaques: (amyloid core)
-granulovacuolar degeneration
-Hirano bodies (prox dendritic eosinophilic inclusions consisting of actin)
-decrease # of neurons in the nucleus basalis of Meynert
65
Most frequent early sign of Alzheimer’s disease
-lost of recent memory
66
67
What gene mutation is linked to Familial Alzheimer disease
APP (amyloid precursor protein) gene mutation
68
Other genetic abnormalities associated with Alzheimer disease
-e4 allele of apoprotein E (chromosome 19)
-Presenilin-1(chromosome 14)
-Presenilin-2 (chromosome 1)
69
What neurotransmitter is deficient in Alzheimer disease
Acetylcholine
70
What is the most frequent cause of dementia
-Alzheimer disease
71
The second most frequent cause of dementia
Multi-infarction dementia
72
Triad of Alcohol encephalopathy (Wernicke disease)
-Confusion
-Ophthalmoplegia
-Ataxia
73
Morphologic features of alcohol encephalopathy
-marked atrophy or demyelination of cerebral cortex, pons, cerebellar vermis, mamillary bodies, and other paramedian masses of gray matter in the brain stem and diencephalon
74
Binswanger disease
-aka subcortical leukoencephalopathy
-associated with HTN
-presence of multiple lacunar infarcts and progressive demyelination limited to subcortical area
-cortex spared
75
Pick disease (Frontotemporal dementia) anatomic changes
-marked cerebral atrophy with gliosis and loss of cortical neurons (temporal and frontal)
- Pick bodies (round intracytoplasmic inclusions, silver-staining consisting of neurofilaments; abnormal tau proteins)
76
77
Pick disease difference from Alzheimer disease
-earlier onset (40-60)
-symptoms: personality and language changes; memory is spared
-lobes affected: frontal and temporal
-histo: pick bodies (tau)
78
Anatomic changes in Huntington dse
-degeneration and atrophy of striatum (caudate nucleus + putamen) and frontal cortex
-neuronal depletion
-gliosis
79
80
Box car ventricles
Huntington disease
81
What neurotransmitter is implicated in Huntington disease
↓ GABA + ↓ ACh + ↑ Dopamine = Chorea, agitation, psychosis
-GABA : decrease due to degeneration of GABAnergic neurons
-AcH: decrease due to degenration of cholinergic neurons
-Dopamine: increase
82
Autosomal dominant characterized by Trinicleotide (CAG) polyglutamine repeats on short arm of chromosome 4
Huntington disease
83
84
Histologic manifestation of Parkinson disease
-Depigmentation of the substantia nigra and locus ceruleus
-Damaged cells contain highly characteristic eosinophilic intracytoplasmic inclusions (lewy bodies).
85
Parkinsonism with autonomic dysfunction and orthostatic hypotension
Shy-Drager Syndrome
86
Degeneration of the upper and lower moror neurons
ALS
87
Morpholohic findings in ALS
-degenration and atrophy of LCST and anterior motor neurons of the cord
88
ALS manifestion
-symmetric atrophy and fasciculation (LMN sign)
-hyperreflexia
-spasticity
-pathologic reflex
89
Majority of intracranial tumors in adult is
Supratentorial
90
Most common primary intracranial tumor in adults
1.Glioblastoma multiforme
2. Meningioma
3. Acoustic neuroma
91
92
Most common primary intracranial tumors in children
-cerebellar astrocytoma
-medulloblastoma
93
Most common primary brain tumors in adult
Grade IV Astrocytoma/GBM
- Malignant astrocytic tumor (WHO grade IV)
- Very aggressive
- Commonly affects cerebral hemispheres (crosses corpus callosum → “butterfly glioma”)
- Histology: pseudopalisading necrosis, endothelial proliferation
94
Astrocytomas that do not infiktrate the brain
-pilocytic astrocytomas
-pleomorphic xanthroastrocytomas
-subependymal giant cell astrocytomas.
95
Most common intracranial primary malignancy
Gliobastoma multiforme (WHO GRADE IV)
96
97
Microscopic findings of Glioblastoma Multiforme
Area of necrosis and hemorrhage surrounded by “pseudopalisade” arrangement of tumor cells
98
When intracranial, what is the most frequent site of shcwannoma
CN VIII
99
Morphologic finding: closely packed cells with large round nuclei surrounded by clear halo of cytoplasm (“fried egg” appearance)
Oligodendroglioma
“Fried-egg” appearance → round nuclei with clear (perinuclear halo) cytoplasm
- Chicken-wire capillary pattern
- Often calcifications present
- IDH1/IDH2 mutation, 1p/19q co-deletion
100
Ependymoma most frequently occurs in what ventricle
Fourth ventricle
101
Histologic characteristics include tubules or rosettes with cells encircling vessels or pointing toward a central lumen; tumor cells characteristically demonstrate blepharoplasts, rod-shaped structures near the nucleus representing basal bodies of cilia.
Ependymoma
Perivascular pseudorosettes (classic,hallmark) → tumor cells arranged around blood vessels with an intervening zone of fibrillary processes
- True ependymal rosettes (tumor cells form lumen-like structures) may also be seen
- May cause hydrocephalus due to obstruction of CSF flow
- Rod-shaped blepharoplasts (basal ciliary bodies) seen on EM
102
Second most common primary intracranial neoplasm
Meningioma
103
Meningioma originates in the
Arachnoidal cells of the meninges
104
Meningioma occurs most frequently in what brain area
-Convexities of cerebral hemispheres
-Parasagittal region
105
Meningioma treatment
Surgical removal
106
107
108
-Whorled pattern of spindle cells
- Psammoma bodies (concentric calcifications)
- Often expresses estrogen receptor (grows in women)
- Well-circumscribed, dural-based mass compressing but not invading brain
Meningioma
109
A highly malignant tumor of the cerebellum with histologic characteristic of Homer Wright rosettes (tumor cells arranged around neuropil but not true lumen)
Medulloblastoma
110
Hemangioblastoma most frequently occurs in
Cerebellum
111
Hemangioblastoma is associated with what disease
Von Hippel-Lindau disease
112
2 histologic pattern of schwannoma
a. antoni A: interlacing bundles of elongated cells with palisading nuclei
b. antoni B: looser, less cellular pattern than Antoni A
113
Mutation in what gene is associated with primary Glioblastoma Multiforme (GBM)
Priamry GBM >> EFGR and PTEN mutation
*secondary GBM >>> p53 mutation
114
2 types of cerebral edema
-vasogenic edema
-cytotoxic edema
115
Cause of cytotoxic edema
Neuronal and glial cell injury
Due to:
-hypoxia
-hypoglycemia
-toxins
-ischemia
116
Cause of vasogenic edema
-increase vascular permeability >> plasma leakage into the interstitial space
Causes:
- tumors
- abscesses
- trauma
- inflammation
117
Vasogenic edema definition
ECF accumulation due to disruption of BBB; reversible; predominantly affect the white matter
118
Cytotoxic edema definition
-ICF accumulation due to failure of Na+/K+ -ATPase pump; irreversible; affects both white and gray matter
119
Extrusion of malformed brain tissue through a midline cranial defect
Encephalocele
120
Most common location of encephalocele
Occiput
121
Classic triad of normal pressure hydrocephalus
“Wet, Wobbly, Wacky”
-Urinary incontinence
-Gait disturbance (magnetic gait)
-Dementia
122
SAH and meningitis causes what type of hydrocephalus
Communicating
123
Aqueductal stenosis and tumor compressing 4th ventricle causes what type of hydrocephalus
Noncommunicating
124
125
Arnold-chiari type 1
- herniation of cerebellar tonsils into the foramen magnum
126
Arnold-chiari tyoe II
Herniation of vermis, brainstem(medulla) + meningomyelocele
127
Triad of Dandy Walker malformation
1. Cystic dilation of the 4th ventricle
2. Hypoplasia or agenesis of cerebellar vermis
3. Enlarged posterior fossa (upward displacement of tentorium and torcula)
128
Most common site of lacunar infarct
Putamen
129
Most common area of borderzone (Watershed) infarct
ACA-MCA borderzone
130
Deep parenchymal ICH is caused by
Hypertension
131
Lobar ICH is caused by
Cerebral amyloid angiopathy
132
Most common sites of Hypertensive Intraparenchymal Hemorrhage
- putamen (most common)
- thalamus
- pons
- cerebellar hemispheres
133
134
Most common type of intracranial aneurysm
Saccular type
135
Most common location of intracranial aneurysm
ACA-ACoA junction
136
Morphologic finding of aneurysmal wall and neck
-absent smooth muscle and internal elastic lamina
137
Most common site of AVM
Posterior br of MCA
138
AVM
AVM = Arteries + Veins Mixed, with intervening brain tissue.
139
Cavernous malformation
Cavernoma = Cavern-like vascular spaces, with no brain tissue in between.
Histologic hallmark: Popcorn or Mulberry appearance vascular spaces
Most common site: Cerebellum
140
141
142
Pathogenesis of ALS
SOD1 (Ch21) mutation
143
Trinucleotide repeats in Friedrich ataxia
-GAA in (Ch9)
144
Bunina bodies: PAS (+) cytoplasmic inclusion
ALS
145
Histology: whorled cells, psammoma bodies. What brain tumor?
-Meningioma
- Common benign tumor in adults
- Arises from arachnoid cap cells
- Often parasagittal; attached to dura
146
146
Histo: Rosenthal fibers (eosinophilic corkscrew fibers). What brain tumor?
Pilocystic astrocytoma
- Usually benign (WHO grade I)
- Arises in cerebellum
147
"Red neurons" observed 12-24 hours after global ischemia are diagnostic of:
Acute hypoxic-Ischemic neuronal injury
Time after insult Findings
0–12 hours No visible change on light microscopy (but ultrastructural changes begin).
12–24 hours Red neurons appear: shrunken cell bodies, intensely eosinophilic cytoplasm, pyknotic nuclei — hallmark of acute neuronal necrosis.
24–72 hours Neutrophilic infiltration peaks
3–5 days Microglial infiltration (macrophages remove debris).
1–2 weeks Reactive gliosis and vascular proliferation.
>2 weeks Cystic cavity formation surrounded by gliosis (chronic infarct).
148
Alzheimer type Il astrocytes-large, clear nuclei-are classically seen in:
Hepatic encephalopathy
Alzheimer type II astrocytes are reactive glial cells (not related to Alzheimer disease) seen in metabolic or toxic encephalopathies, especially hepatic encephalopathy due to hyperammonemia.
149
TDP-43
Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD)
150
α-synuclein
Parkinson disease, Lewy body dementia (intracellular Lewy bodies)
151
A 65-yesr-old man develops progressive memory loss and personality change. Brain shows f-amyloid plaques and neurofibrillary tangles. The key abnormal protein is:
Tau
The patient presents with progressive memory loss and personality change, and the brain shows β-amyloid plaques and neurofibrillary tangles (NFTs) — classic Alzheimer disease (AD) pathology.
NFTs key protein: Tau
152
Patient: 25-year-old woman → young adult
Lesion: Cerebellar, cystic with mural nodule
Histology: Rosenthal fibers (eosinophilic corkscrew-shaped protein aggregates)
Pilocytic astrocytoma: low-grade (WHO grade I) astrocytoma
153
Familial early-onset AD (<65 y). Gene mutation
APP on chromosome 21
* also linked to Down syndrome
154
Sporadic late-onset Alzheimers Disease (>65 y). Gene mutation?
APOE ε4 allele
155
155
A 65-yesr-old man develops progressive memory loss and personality change. Brain shows f-amyloid plaques and neurofibrillary tangles. The key abnormal protein is:
Tau
The patient presents with progressive memory loss and personality change, and the brain shows β-amyloid plaques and neurofibrillary tangles (NFTs) — classic Alzheimer disease (AD) pathology.
NFTs key protein: Tau
PATHOLOGY
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