CNS Pathology Flashcards
What are Down syndrome pt. more at risk for?
1) Early onset dementia presenting with senile plaques and neurofibrillary tangles
2) Acute leukemias
3) Congential heart disease
4) GI defects (duodenal atresai and Hirschsprung dx)
Spontaneous contractions of a single lower motor unit; occur at rest
Fasiculations
How can you differentiate between a benign positional vertigo and a posterior fossa disease using the Baranay maneuver?
1) Latency will be immediate with posterior fossa dx; 2-20 seconds for benign positional vertigo
2) Duration will be persistent with a posterior fossa dx; < 1 min for a benign positional vertigo
3) Adaptation with benign positional vertigo
Treatment for tension headache?
1) NSAID
2) NSAID + Caffeine
3) NSAID + Barbiturate
4) All together
What is the barbiturate used for tension headaches? What are its effects?
1) Butalbital
2) Causes sedation by facilitating GABA neurotransmission
3) Can become dependent and will have rebound headaches after removal of substance
Why is caffeine used in the treatment of tensions headaches?
Caffeine is a competitive antagonist at the adenosine receptor
Headache that has abrupt onset, occurs bilaterally, PE and all other tests are normal; most common cause of headache
Tension Headache
What are aura symptoms?
1) Presence of Zig-zag lines that flash
2) Occur before migraine
3) May also have olfactory hallucinations
What is the difference between communicating and non-communicating hydrocephalus?
1) Communicating = symmetric enlargement of ventricular system
2) Non-communicating = asymmetric enlargement of ventricular system
Herniation that occurs when unilateral expansion of a cerebral hemisphere displaces the cingulate gyrus under the falx cerebri
Subfalcine herniation
Herniation that occurs at the medial aspect of the temporal lobe where it is compressed against the tentorium
Transtentorial herniation
Displacement of the cerebellar tonsils through the foramen magnum
Tonsillar herniation
What are the complications of Transtentorail herniation?
1) Compromise of CN III
2) Compression of posterior cerebral artery
3) Kernohan’s notch- contralateral cerebral peduncle compression (causes ipsilateral paresis)
4) Secondary brainstem (Duret) hemorrhages
What is a complication of a tonsillar hernation?
1) Death due to compression of essential components of the medulla oblongota
What does the presence of alpha-fetoprotein in maternal serum indicate
Neural tube defect
Absence of cortical gyri
Lissencephaly
Small, numerous and irregularly formed cerebral convolutions
Polymicrogyria
Spectrum of malformations characterized by incomplete separation of the cerebral hemispheres across the midline
Holoprosencephaly
What occurs in Dandy-Walker Malformation
1) Agenesis of cerebellar vermis
2) Cystic enlargement of 4th ventricle
Small posteiror fossa with downward extension of the vermis through the foramen magnum
Arnold Chiari malformation (Chiari II malformation)
Low lying cerebellar tonsils that extend down into the vertebral canal
Chiari I malformation
Vertigo caused by free-floating otoconia that have been dislodged form the utricular macula and have been moved into one of the semicircular canal
Benign positional vertigo
What is the location of an acoustic neuroma?
1) Cerebellopontine angle
Headache that presents in young males, is unilateral, and usually retro-orbital, autonomic dysfunctions; duration of 30 min to 2 hours
Cluster Headache
Headache with unilateral lightning bolt pain; 2-20 episodes dilay; normal neurologic exam
Trigeminal Neuralgia
What must occur in order to classify a headache as a migraine?
At least four attack must have occured
What time period in someone’s life are they more likely to get a migraine?
In the first four decades of life
Unilateral throbbing pain associated with photophobia and phonophobia; usually associated with nausea and vomitting; duration: 4-72 hours
Migraine
What is the main cause of an epidural hematoma?
1) Rupture of middle meningeal artery
2) Generally occurs in children with temporal bone displacement
3) Neurosurgical emergency
Main cause of subdural hematoma?
1) Tearing of the bridging veins in elderly or infants
What region of the brain is at greatest risk of a border zone infarct in a hypotensive state?
1) edges of the cerebral vascular supply that mainly receives adequate supply due to overlap
What are the stages of global cerebral ischemia?
1) Presentation of red neurons (12-24 hrs)
2) Infiltration of neutrophils (1-3 days)
3) Influx of macrophages (3-5 days)
4) Reactive gliosis (1-2 weeks)
5) Scar formation/repair (months)
How does hypertension affect the brain?
1) Lacunar infarcts
2) Slit Hemorrhages
3) Hypertensive Encephalopathy
What are the two major causes of intracranial hemorrhage?
1) Hypertension
2) Cerebral amyloid angiopathy (CAA)
What mendelian disorders are at greater risk for a berry aneurysm?
1) Autosomal dominant polycystic kidney disease
2) Ehlers-Danlos syndrome
3) Neurofibromatosis type 1
4) Marfan syndrome
Extension of only the meninges herniating into the spinal canal defect
Meningocele
Meninges and spinal cord herniate through the spinal canal defect
Meningomyelocele
Malformation of the anterior neural tube resulting in no forebrain and an open calvarium; polyhydramnios and frog-like appearance
Anencephaly
Lake-like spaces that occur in the lenticular nucleus, thalamus, internal capsule, caudate nucleus, and pons? What is the most common cause?
1) Lacunar Infarct
2) Caused by hypertension
Most common location of a berry aneursym?
1) Bifuraction of the anterior communciating artery and anterior cerebral artery
Describe the bacterial cause of meningitis according to age. Neonate (0-6 mos)? Children (6mos-6years)?
6 years to 60 years? Elderly (>60 years)?
1) Group B streptococcus agalactae, E. coli, Listeria
2) Streptococcus pneumonaie, Neisseria meningitidis, Haemophilus influenzae type B
3) Streptococcus pneumoniae, Neisseria meningitidis
4) Streptococcus pneumoniae and Listeria monocytogenes
What are the findings of CSF for an acute purulent meningitis?
1) Increased neutrophils (>90,000)
2) Increased protein
3) Decreased glucose
4) Findings on gram stain
What are the findings of CSF for an acute aseptic meningitis?
1) Lymphocytosis
2) Moderately increased protein
3) Normal glucose level
4) No findings on gram stain
Describe the progression of an infarct in the brain.
1) At 12 hours red neurons with cytotoxic and vasogenic edema
2) After 24 hours the tissue appears pale, soft and swollen
3) 1-3 days infiltration of neutrophils
4) 3-7 days infiltration of macrophages (microglia)
5) Liquefaction occurs at 2-3 weeks
6) After several months there is separation from the cavity forming a gliotic cyst
Dilation of the ventricular system with a compensatory increase in CSF volume due to loss of a brain parenchyma
Hydrocephalus ex vacuo
What are the causes of Hydrocephalus ex vacuo?
1) Parkinson’s
2) Alzheimer
What are Duret Hemorrhages caused by? What is their appearance?
1) Transtenorail (uncal) hernia
2) Linear, flame shaped lesions caused by tearing of arteries supplying the upper brain stem
Failure of bony spinal canal to close, but no structural herniation
Spina Bifida Occulta
Diverticulum of malformed CNS tissue extending through a defect in the cranium
Encephalocele
Collection of neurons in inapporpirate locations. How does this present?
1) Neuronal heterotopias
2) epilepsy
Infarct at the supratentorial periventricular white matter that consists of a chalky yellow plaque
Periventricular Leukomalacia
What are Charcot-Bouchard microaneurysm associated with?
1) Intracerebral hemorrhage
2) Caused by hypertension
Sensory loss of pain and temperature with sparing of fine touch and position in the upper extremities in a cape like distribution
Syringomyelia
Formation of a fluid filled cleft-like cavity in the inner portion of the cord that occurs from C8-T1
Syringomyelia
Discontinuous multi-segmental expansion of the ependymal lined central canal of the cord
Hydromyelia
What is the difference between Creutzfeldt-Jakob Disease and vCJD?
1) vCJD has behavioral changes early in onset
2) vCJD has plaques in the cortical regions with surrounding spongiform change
3) No alterations in PRNG gene
What has been found to be connected genetically to CJD?
1) Homozygous state at the 129 codon of the PRNG gene
What are the two microscopic findings in CJD?
1) Spongiform transformation
2) Presence of uniform empty vacuoles within the neuropil and perikaryon of neurons
Autoimmune attack on the myelin sheath; results in plaques within the white matter; characterized by distinct episodes of neurologic deficits separated in time
Multiple Sclerosis
What is HLA-DR2 associated with?
Multiple Sclerosis
What is used to diagnose MS?
1) MRI - shows plaques in white matter
2) Lumbar puncture- shows increased lymphocytes and oligoclonal IgG bands
Bilateral optic neuritis and spinal cord demyelination
Neuromyelitis Optica
Diffuse, monophasic demylinating disease that follows a viral infection; there is myelin loss with relative preservation of axons
Acute Disseminated Encephalomyelitis (ADEM)
Fulminant syndrome of CNS demyelination affecting younger adults and children; preceded by a URI
Acute necrotizing hemorrhagic encephalomyelitis
Spherical collections of dilated, neuritic processes around a central amyloid core; found in Alzhemier disease
Neuritic plaque
What is the abnormal protein found within the amyloid core of a neurtic plaque?
1) A-beta
What are the two major microscopic findings in Alzheimer disease?
1) Neuritic plaques
2) Neurofibrillary tangles
Bundles of filaments in the cytoplasm of the neurons that displaces the nucleus; elongated flame shape
Neurofibrillary tangles
Elongated, glassy, eosinophilic bodies consisting of paracrystaline arrays of beaded filaments; what is this associated with?
1) Hirano bodies
2) Alzheimer disease
What is the major protein deposited in Alzheimer disease derived from? How does it come about?
1) Aβ is derived from Amyloid precursor protein (APP)
2) Aβ is only formed when APP undergoes beta cleavage
What cleave must APP undergo to make Aβ?
Beta cleavage
What chromosome is APP found on?
Chromosome 21
What is another complication of the abundant amyloid protein found in Alzheimers?
1)Cerebral amyloid angiopathy which could cause intracerebral hemorrhage due to weakening of the wall
What protein is hyperphosphorylated in the neurofibrillary tangles found in Alzheimer
1) Tau protein is hyperphosphorylated
Group of disorders that have progressive degeneration of temporal and frontal lobes resulting in deterioration of language and changes in personality
Frontotemporal Dementias
Frontotemporal dementia that occurs progressively; Atrophy is so severe that it causes wafer thin gyri; presence of cytoplasmic round filamentous inclusions that are weakly basophilic
Pick Disease