chapter 28- CNS Flashcards
is pilocytic astrocytoma benign or malignant
benign
how does craniopharyngioma usually present
supratentiral mass in kid or young adult with bitemporal hemiangiopsia
where does pilocytic astrocytoma typically occur
cerebellum
Describe the CSF with multiple sclerosis
Elevated protein, 1/3 pleocyotsis, increased IgG
What are 80% of adult primary brain tumors
Infiltrating astrocytomas
What is elevated in the serum of adrenoleukosystrophy
VLCFAs
how does cerebral aqueduct stenosis present
enlarging head circumference
Where do choroid plexus papilloma typically occur in kids vs adults
Kids in lateral ventricles; adults in 4th ventricle
what is the term for cystic degeneration of spinal cord
syringomyelia
what should you think of if a tumor is resenthol fiber positive and eosinophilic with GFAP positive
pilocytic astrocytoma
Where are infiltrating astrocytomas usually found
Cerebellum
what is the term for absence of skull and brain that has a frog-like appearance of the fetus
anencephaly
How does freidreich ataxia general begin
1st decade of life with gait ataxia, followed by hand clumsiness and dysarthria
What grade is a diffuse astrocytoma
2
what type of people are more likely to get CJD
those getting human growth hormone or that have received a corneal transplant
what is vascular dementia due to
HTN, atherosclerosis, or vasculitis
Where is brain lesion associated with toxoplasmosis most often found
Cerebral cortex and deep gray nuclei
what artery is often ruptured with epidural hematoma
middle meningeal artery
what is deficient in metachromatic leukodystrophy
aryl sulfatases
what is the disease defined as degeneration of GABAergic neurons in caudate nucleus of basal ganglia
huntington disease
what is the term for slow, involuntary snack like movements of fingers
athetosis
What type of astrocytoma is defined as poorly defined, gray, infiltrative tumors that expand and distort the invaded brain; GFAP positive
Diffuse astrocytoma
what is the term for inherited mutations in enzymes necessary for production or maintenance of myelin
leukodystrophies
what is the presentation of Werdnig-Hoffman disease
floppy baby
what does the CT look like for epidural hematoma
lens shaped lesion on CT
What is the most important histopathologic indicators of CNS injury
Gliosis
what does anencephaly result in
maternal polyhydramnios
what is deficient in Krabbe disease
galactocerebroside beta galactosidase
why are people with down syndrome more likely to get alzheimers
because APP is on chromosome 21
where does the galactocerebroside accumulate in Krabbe disease
macrophages
what type of infarct does thrombotic infarction produce
PALE at peripheral of cortex
what is also known as “talk and die” syndrome
epidural hematoma
are schwannomas S100 positive or negative
positive
where do thrombotic infarcts typically occur
at branch points
what disease should you think of with butterfly glioma
glioblastoma multiforme
what leukodystrophy is due to impaired addition of coenzyme A to long chain fatty acids
adrenoleukodystrophy
what is the most common benign tumor of CNS
meningioma
Where are medulloblastomas in kids vs adults
Kids in midline of cerebellum; in adults it is more lateral locations
what is control pontine myelinolysis due to
rapid IV correction of hyponatremia
What is the risk factor most commonly associated with lobar hemorrhages
Cerebral amyloid angioplasty
what distinguishes Amyotrophic lateral sclerosis from syringomyelia
lack of sensory impairment in ALS
what do subfalcene herniations compress
anterior cerebral artery
Who are lafora bodies found in
Cytoplasm of neurons in myoclonic epilepsy
what is the term for damage to the anterior horn due to poliovirus
poliomyelitis
what should you think of with whorled appearance of cells and psamomma bodies in CNS
meningioma
What is the term for minute aneurysms which may be at site of rupture
Charcot-Bouchard microaneurysms
What are the most common offending organisms in brain abscess
Streptococci and staphylococci
What is a tauopathy in affected individuals that commonly develop progressive truncal rigidity, disequilibrium with frequent falls and difficulty with voluntary eye movements
Progressive supranuclear palsy
what occurs on EEG with CJD
spike waves
What is the term for incomplete separation of cerebral hemispheres across the midline
Holoprosencephaly
What is diagnostic for multiple system atrophy
Glial cytoplasmic inclusions
What neuronal inclusion is associated with herpetic infection
Cowdry body
what is the most common cause of meningitis in kids and teenagers
N. meningitidis
what is the term for congenital failure of cerebellar vermis to develop
dandy-walker malformation
When are red neurons seen
Acute neuronal injury
what cranial nerve do uncal herniations compress
CN III
what is the term for degeneration of upper and lower motor neurons
amyotrophic lateral sclerosis
what lobe do oligodendrogliomas occur
frontal
what CNS tumor has fried egg appearance
oligodendroglioma
Where do most intracerebral/ intraparenchymal hemorrhage occur
Putamen
what is an early sign of amyotrophic lateral sclerosis
atrophy and weakness of hands
What is early sign of amyotrophic lateral sclerosis
Asymmetric weakness of hands
what does the lumbar puncture for multiple sclerosis show
increased lymphocytes, increased Ig with oligoclonal IgG bands, and myelin basic protein
What is a X-linked polyglutamine repeat expansion disease characterized by distal limb amyoptrophy and bulbar signs associated with degeneration of LMNs in the spinal cord and brainstem
Bulbospinal atrophy (Kennedy syndrome)
what is the term for inherited degeneration of anterior motor horn
Werdnig-Hoffman disease
what is found within the cels with glioblastoma multiform
GFAP
What syndrome has synchronous B/L optic neuritis and spinal cord demyelination
Neuromyelitis optics
What is the term for depressed, retracted, yellowish-brown patches involving crests of gyri, most commonly those that are located at site of contrecoup injury
Plaque Jayne
what is the most common leukodystrophy
metachromatic leukodystrophy
what is the most common source of thromboembolic that causes embolic ischemic stroke
left side of heart
what are early finding in liquefactive necrosis caused by ischemic stroke
red neurons
What is a rare progressive clinical syndrome characterized by cognitive decline, spasticity of limbs and seizures
Subacute sclerosing panencephalitis
what should you think of in a kid with a tumor that has small, round blue cells as well as homer-wright rosettes
medulloblastoma
what is the presentation of dandy-walker malformation
massively dilated 4th ventricle with absent cerebellum; often accompanied by hydrocephalus
What is the clinical pattern associated with Multiple system atrophy and what is the inclusion seen
Parkinsonism, cerebellar ataxia, autonomic failure; alpha-synuclein
what is the most common cause of meningitis in adults and elderly
strep pneumonia
What is known as the batwing deformity
Agenesis of corpus callosum
what are the most common causes of meningitis in neonates
GBS, E. coli and L. monocytogenes
What is shown on the external exam of a brain of someone who has rabies encephalitis
Intense edema and vascular congestion
What grade is a pilocytic astrocytoma
1
what is the most common CNS tumor in kids
pilocytic astrocytoma
What is the hallmark finding in progressive supranuclear palsy
Presence of tau-containing inclusions in neurons and glia
What grade is a anaplastic astrocytoma
3
Where do ependymomas typically occur
Near 4th ventricle
what is the triad that is associated with normal pressure hydrocephalus
urinary incontinence, gait instability, dementia
What do mutations in DCX involved in neuronal heterotopias cause
Lissencephaly in males and subcritical band heterotopias in females
what sensory loss occurs with syringomyelia
pain and temperature
What should you think of with Lisch nodules and cafe au lait spots
Neurofibromatosis type 1
who does hyaline arteriolosclerosis occur in
benign HTN and DM
What disease should you think of with Kuru plaques
Variant CJD
What is the term for tangled network of wormlike vascular channels with prominent, pulsation arteriovenous shunting with high blood flow
Arteriovenous malformation
What do the inclusions associated with subacute sclerosing panencephalitis contain
Nucleocapsids characteristic of measles
What is the common presentation with adrenoleukosystrophy
Involves ABCD1; young males presenting with behavioral changes and adrenal insufficiency
What grade of lesion is gliomatosis cerebri
Grade 3/4
what disease is athetosis associated with
huntington disease
what is a benign tumor of arachnoid cells
meningioma
What should you think of with candle-guttering
Tuberous sclerosis
Who is korsakoff syndrome common in
Chronic alcoholics
how does huntington disease present
chorea; progress to dementia and depression
What disease is a leukodystrophy associated with mutations in the gene encoding GFAP, abundant rosenthal fibers found in periventricular, perivbascular and subpial locaitons
Alexander disease
What grade is glioblastoma
Grade 4
What is a disease of infancy characterized by lactic academia, arrest of psychomotor development, feeding problems, seizures, extraocular palsies and weakness with hypotonia
Leigh syndrome (subacute necrotizing encephalopathy
what is the classic triad with meningitis
headache, neck stiffness, fever
What does methanol preferentially affect
Retina
what is the term for congenital extension of cerebellar tonsils through foramen magnum
arnold-chiari malformation
What is mutated in fatal familial insomnia
Specific mutation in PRNP gene (aspartate substitute for asparagine at residue 178)
What is the typical presentation associated with HSV-1 encephalitis
Alterations in mood, memory and behavior
what is subdural hematoma due to
tearing of bridging veins
what is subarachnoid hemorrhage most commonly due to
rupture of berry aneurysm
What is the clinical presentation of tuberous sclerosis
Seizures, autism, mental retardation
what are the 3 most common metastasis to brain
lung, breast, kidney
what is the presentation of central pontine myelinolysis
acute paralysis; “locked in syndrome”
what are neural tube defects associated with low levels of prior to conception
folate
What are premature infants with cerebral palsy at increased risk of
Intraparenchymal hemorrhage
What mutation increase the risk of kids getting HSV-1 encephalitis
TLR3 mutation
what is a degenerative disease of frontal and temporal cortexes; spares parietal and occipital lobes
pick disease
What are rosenthal fibers found with
Regions of long standing gliosis; characteristic of pilocytic astrocytoma
What is the histology associated with cerebral amyloid angiopathy
No fibrosis present; instead dense and uniform deposits of amyloid are present
where does syringomyelia typically occur
C8-T1
what are the 3 ways to develop ischemic stroke
thrombotic, embolic, lacunar
What is holoprosencephaly associated with
Trisomy 13 and SHH mutations
what disorder has the presentation of ataxia with loss of vibratory sense and proprioception, muscle weakness in LE, and loss of DTR
Friedrich ataxia
What is altered in most CJD
PRNP
What is the term for compensatory increase in ventricular volume secondary to loss of brain parenchyma
Hydrocephalus ex vacuo
What is the term for a gray matter cell with a large nucleus, pale staining central chromatin, an intranuclear glycogen droplet and a prominent nuclear membrane and nucleoli
Alzheimer’s type 2 astrocyte (not associated with Alzheimer’s disease)
What is the diagnostic marker for Parkinson’s disease
Lewy body
what virus can cause subacute sclerosing pan encephalitis
measles virus
what is the most common cause of dementia
Alzheimers disease
What is the clinical pattern associated with amyotrophic lateral sclerosis
Weakness with upper and lower motor neuron signs
what vessels are most commonly involved in lacunar ischemic stroke
lenticulostriate vessels
what artery is usually involved in embolic ischemic stroke
middle cerebral artery
what is a lethal complication of epidural hematoma
herniation
what is the most common malignant CNS tumor in adults
glioblastoma multiforme
What is the term for perinatal ischemic lesions of cerebral cortex, resulting in depths of sucking bear the brunt of injury and result in thinned-out, gliotic gyri
Ulegyria
What is the term for congregation around cell bodies of dying neurons
Neuronophagia
What is the term for non-progressive neurologic motor deficit characterized by combinations of spasticity, dystopia, ataxia/athetosis and paresis
Cerebral palsy
What is the term for aberrant and irregular myelinatization gives rise to marble like appearance of deep nuclei
Status marmoratus
What should youthink of with bunina bodies
Amyotrophic lateral sclerosis
what is the presentation of CJD
rapidly progressive dementia associated with ataxia and startle myoclonus
What is the presentation of infiltrating astrocytomas
Seizures, headaches, focal neurologic deficits
What is the term for infarcts in supratentorial periventricular white matter
Periventricular leukomalacia
what are the lewy bodies associated with parkinson disease composed of
alpha- synuclein
what is a high grade malignant tumor of astrocytes
glioblastoma multiforme
What is a foci of hermitage and necrosis in mammary bodies and walls of 3rd and 4th ventricles associated with Thiamine
Wernicke encephalopathy
what HLA is associated with multiple sclerosis
HLA-DR2
what virus is needed for progressive multifocal leukoencephalopathy
JC virus infected oligodendrocytes
what does lacunar ischemic stroke occur secondary to
hyaline arteriolosclerosis
what is the most common site of intracerebral hemorrhage
basal ganglia
what does imaging of meningioma reveal
round mass attached to dura (will press on cortex but does NOT invade)
What is the term for thick, elongated, brightly eosinophilic, irregularly structures that occur within astrocytic process and contain alphaB-crystallin and hsp27
Rosenthal fibers
What astrocytoma is a predominant neoplastic astrocyte that shows brightly eosinophilic cell body from which emanate abundant, stout processes
Gemistocytic astrocytoma
what is the clinical presentation associated with parkinson disease
TRAPS: tremor, rigidity, Akinesia/bradykinesia, Postural instability, shuffling gait
What antibodies are common in neuromyelitis optics
Antibodies against aquaporin 4 (the major water channel in astrocytes)
What disorder is associated with molar tooth sign on imaging
Joubert syndrome
What genetic thing is most associated with friedreich ataxia
Frataxin mutations, GAA trinucleotide repeat, cardiomyopathy
how are neural tube defects detected during prenatal care
by increased AFP in amniotic fluid and maternal blood
What is the common site for arteriovenous malformations
Territory of middle cerebral artery
what is central pontine myelinolysis
focal demyelination of pons
What is the disease that is venous angiomatous malformation of spinal cord and overlying meninges
Foix-alajouanine disease
What does Waterhouse-Friderichsen syndrome result from
Meningitis–associated septicemia with hemorrhagic infarction of adrenal glands and cutaneous petechiae
What neuronal inclusion is associated with rabies
Negri body
What is the most striking alteration in fatal familial insomnia
Neuronal loss and reactive gliosis in anterior ventral and dorsomedial nuclei of thalamus. Neuronal loss is also prominent in inferior olivary nuclei
What is the clinical pattern associated with spinal bulbar muscular atrophy
Lower motor neuron weakness, diminished androgen
