Neuro Flashcards

(404 cards)

1
Q

`What do the wall and the lumen of the neural tube give rise to? What about neural crest cells, generally speaking?

A
  • wall: CNS tissue
  • lumen: ventricles
  • neural crest: PNS tissue
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2
Q

What compound is associated with neural tube defects?

A

low folate pre-conception

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3
Q

At what point in the process of gestation does low folate lead to neural tube defects?

A
  • pre-conception

- week 4 when neural pore closes

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4
Q

What are two ways to screen for neural tube defects in utero? What is the exception to this?

A
  • elevated alpha-fetoprotein in the amniotic fluid or maternal blood
  • elevated acetylcholinesterase in amniotic fluid
  • AFP won’t be elevated in cases of spina bifida occult
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5
Q

Anencephaly

A
  • a malformation of the anterior neural tube, which results in the absence of the forebrain and an open calvarium
  • associated with maternal type I diabetes and folate deficiency
  • presents with a “frog-like” appearance due to enlargement of the eyes but is incompatible with life
  • can detect in utero with elevated AFP and polyhydramnios since there is no swallowing center in the brain
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6
Q

Spina Bifida

A
  • failure of the posterior vertebral arch to close, resulting in a vertebral defect
  • most often due to failure of the caudal end of the neural tube to close as a result of low folate in the pre-conception period
  • spina bifida occulta is the mildest form and presents as a dimple or patch of hair overlying the vertebral defect with the dura still intact
  • true spina bifida can present with either a meningocele (protrusion of meninges alone) or meningomyelocele (protrusion of meninges and spinal cord)
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7
Q

What are the three degrees of spina bifida?

A
  • spina bifida occulta: mildest form, presenting as a dimple or patch of hair over the vertebral defect with the dura still intact
  • meningocele: moderate form, presenting as a cystic protrusion of just the meninges
  • meningomyelocele: severe form, presenting as a cystic protrusion of the meninges and spinal cord
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8
Q

What is the cerebral aqueduct?

A

the channel that drains CSF from the 3rd to the 4th ventricle

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9
Q

Cerebral Aqueduct Stenosis

A
  • a stenosis of the channel draining CSF from the 3rd to the 4th ventricle
  • results in the accumulation of CSF and is the most common cause of hydrocephalus in newborns
  • presents with enlarging head circumference due to dilation of the ventricles and the fact that the cranial suture lines are not yet fused
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10
Q

Describe the flow of CSF through the brain.

A
  • CSF is produced by ependymal cells of the choroid plexus lining the lateral ventricles
  • it flows into the 3rd ventricle via the interventricular foramen of Monro
  • then into the 4th ventricle via the cerebral aqueduct
  • from the 4th ventricle, it flows into he subarachnoid space via the foramina of Magendie and Luschka
  • finally, it drains into the venous sinuses through the arachnoid granulations
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11
Q

What are the foramina of Magendie and Luschka?

A
  • they are the foramen through which CSF flows out of the fourth ventricle and into the subarachnoid space
  • there are two lateral foramen of Luschka situated somewhat anteriorly and one medial foramen of Magendie posteriorly
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12
Q

Dandy-Walker Malformation

A
  • congenital failure of the cerebellar vermis to develop
  • presents as a massively dilated fourth ventricle (posterior fossa) with an absent cerebellum, typically accompanied by hydrocephalus
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13
Q

Type II Chiari Malformation

A
  • a congenital downward displacement of cerebellar vermis and tonsils through the foramen magnum
  • type II is defined as a displacement that obstructs the flow of CSF, resulting in hydrocephalus
  • presents with headaches and cerebellar symptoms
  • associated with meningomyelocele
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14
Q

What is the difference between a type I and a type II Chiari malformation?

A
  • type I doesn’t obstruct the flow of CSF and therefore doesn’t result in hydrocephalus like type II does
  • furthermore, type I has an associated with syringomyelia while type II has an association with meningomyelocele
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15
Q

Syringomyelia

A
  • a cystic degeneration of the spinal cord
  • arises with trauma or in association with a type I Chiari
  • usually occurs at C8-T1 and involves the anterior white commissure
  • presents as a loss of pain and temperature sensation in the upper extremities bilaterally with sparing of fine touch and position sense (said to have a “cape-like” distribution of deficits)
  • may expand to involve and damage the anterior horn (lower muscle signs) or lateral horn (Horner syndrome)
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16
Q

The anterior white commissure is where what fibers cross within the spinal cord?

A

it is where the second-order neurons of the spinothalamic tract, carrying pain and temperature, cross the midline

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17
Q

What structures will a syringomyelia typically involve once it expands beyond the white commissure? How does this present?

A
  • begins with involvement of the anterior white commissure, which affects the spinothalamic tract carrying pain and temperature
  • may expand to involve the anterior horn, which presents with lower motor neuron signs of flaccid paralysis, muscle atrophy, weakness, and impaired reflexes
  • may expand to involve the lateral horn, which is home to sympathetic neurons and presents as Horner syndrome
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18
Q

What is Horner syndrome?

A
  • results from disruption of sympathetic input to the face/head
  • presents with ptosis (droopy eyelid), miosis (constricted pupil), and anhidrosis (decreased sweating)
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19
Q

What is ptosis?

A

drooping of the eyelid

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20
Q

What is miosis?

A

constriction of the pupil; opposite of mydriasis

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21
Q

What are signs of damage to lower motor neurons?

A
  • flaccid paralysis
  • impaired reflexes
  • muscle weakness and atrophy
  • negative Babinski (down going toes)
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22
Q

What cells reside in the lateral horn of the spinal cord?

A

sympathetic preganglionic visceral motor neurons

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23
Q

What cells reside in the anterior horn of the spinal cord?

A

lower motor neurons

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24
Q

Polio damages what neuron population?

A

lower motor neurons in the anterior horn

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25
Poliomyelitis
- damage incurred by lower motor neurons in the anterior horn due to poliovirus infection - polio is transmitted via the fecal-oral route and infects the oropharynx and small bowel, replicates in the Peyer's patches, and eventually spreads to the CNS - initially, it presents with fever, sore throat, n/v, and abdominal pain; later it presents with asymmetric lower motor neuron signs - CSF will demonstrate elevated WBC, a slight increase in protein, and no change in CSF glucose
26
Werdnig-Hoffman Disease
- an inherited, autosomal recessive degeneration of the anterior motor horn - presents as "floppy baby" with hypotonia and tongue fasciculations - death occurs within a few years
27
Amyotrophic Lateral Sclerosis
- a degenerative disorder of the upper and lower motor neurons of the corticospinal tract - presents with asymmetric upper and lower motor neuron signs; atrophy and weakness of the hands is usually the first indicator; importantly, there is no sensory impairment - most cases are sporadic and arise in middle age adults - however, a zinc-copper superoxide dismutase mutation (SOD1) is present in some familial cases and contribute to free radical injury - treat with riluzole
28
Louis Gherig (AML) disease affects which neuron populations?
only the upper and lower motor neurons of the corticospinal tract, meaning there are no sensory deficits
29
What mutation can be associated with familial cases of Amyotrophic Lateral Sclerosis?
a mutation in SOD1, a zinc-copper superoxide dismutase
30
What are signs of upper motor neuron damage within the corticospinal tracts?
- spastic paralysis - hyperreflexia - increased muscle tone - positive Babinski sign (upward facing toes) - clasp knife spasticity
31
What is the Babinski sign? What is a negative finding and what is a positive finding? What does a positive finding indicate?
- it is the plantar reflex to scraping the bottom of the foot - it is considered positive if the big toe points up - this is physiologic in neonates but indicative of upper motor neuron injury later
32
Friedreich Ataxia
- a degenerative disorder of the cerebellum and multiple tracts within the spinal cord - due to an autosomal recessive, unstable trinucleotide repeat of GAA in the frataxin gene on chromosome 9, which is essential for mitochondrial iron regulation - loss of this gene results in free radical damage - presents in early childhood with ataxia, loss of vibratory sense and proprioception, muscle weakness in the lower extremities, and loss of deep tendon reflexes - additional features include staggering gait, frequent falling, nystagmus, dysarthria, pes cavus, hammer toes, diabetes, and hypertrophic cardiomyopathy - patients become wheelchair bound within a few years
33
What mutation leads to Friedreich Ataxia?
an autosomal recessive, unstable trinucleotide repeat of GAA in the frataxin gene
34
What is the frataxin gene?
a gene important in mitochondrial iron regulation, which may incur an unstable, trinucleotide repeat of GAA, resulting in Friereich Ataxia
35
Describe the anatomy of the spinothalamic tract, what information it carries and at what level it decussates.
- it carries pain and temperature sensation - the first order neuron is located in the dorsal root ganglion and synapses on a second-order neuron in the posterior horn - this second-order neuron then decussates via the anterior white commissure near at the level where it enters - the second-order neuron then ascends via the spinothalamic tract to the thalamus where it synapses on the third-order neuron - this third-order neuron projects to the cortex
36
Describe the anatomy of the corticospinal tract, what information it carries and at what level it decussates.
- upper motor neurons descend and decussate in the medullary pyramids - they continue to descend and synapse on lower motor neurons in the anterior horn - these lower motor neurons then project to the NMJ
37
Describe the anatomy of the hypothalamospinal tract, what information it carries and at what level it decussates.
- first order neurons located in the hypothalamus synapse on the T1 lateral horn - from there, second-order neurons synapse on the superior cervical ganglion (sympathetic chain ganglion) - this third-order neuron then projects to the eyelids, pupil, and skin on the face carrying sympathetic input
38
Describe the anatomy of the dorsal column-medial lemniscus tract, what information it carries and at what level it decussates.
- first order neurons enter via the dorsal root and ascend within the ipsilateral dorsal column and project to second order neurons located in the medulla - second order neurons then decussate and ascend via the medial lemniscus to the thalamus where third order neurons are located - third order neurons finish carry pressure, touch, vibration, and proprioception information to the cortex
39
Meningitis is an inflammation of which layers of the meninges?
the arachnoid and pia, collectively known as the leptomeninges
40
Which layers of the meninges are included in the leptomeninges?
the arachnoid and pia
41
Which organisms are most commonly responsible for instances of meningitis in the following: - neonates - children/teenagers - adults/elderly - viral - immunocompromised - unvaccinated infants
- neonates are commonly infected by Group B Strep (agalactaie), E. coli, or Listeria monocytogenes - children and teenagers by N. meningitidis - adults by Strep pneumoniae - Coxsackievirus is the most common cause of viral meningitis in children - fungi affect the immunocompromised - H. influenza is common in unvaccinated infants
42
Meningitis
- inflammation of the leptomeninges (arachnoid and pia) - most often due to Group B strep, E. coli, Listeria monocytogeens, N. meningitidis, S. pneumoniae, H. influenza, Coxsackievirus, or fungi depending on the patient's age and immune competence - presents with a classic triad of headache, nuchal rigidity, and fever; photophobia, vomiting, or altered mental status are also possible - diagnosis is made by lumbar puncture and examining the CSF for neutrophils or lymphocytes and determine the CSF glucose level to determine whether it is fungal, viral, or bacterial - complications are usually seen in those with bacterial meningitis and include death secondary to edema and herniation, hydrocephalus, hearing loss, or seizures
43
Where is a lumbar puncture performed? What can help you identify this location?
between L4 and L5 at the level of the iliac crest
44
Why is a lumbar puncture performed between L4/L5?
because the spinal cord ends at L2 but the cauda equina and subarachnoid space continue to S2
45
Where does the spinal cord end? Where does the cauda equina end?
- spinal cord ends at L2 | - cauda equina ends at S2
46
What would you expect to find via lumbar puncture in someone with bacterial, fungal, or viral meningitis?
- bacterial: you would expect neutrophils and low CSF glucose - viral: you would expect lymphocytes and normal CSF glucose - fungal: you would expect lymphocytes and low CSF glucose
47
What does a lumbar puncture needle pass through on it's way to sample CSF?
- skin - supraspinous ligament - interspinous ligament - ligamentum flavum - epidural space - dura - arachnoid - ends in subarachnoid space
48
What is a normal or expected CSF glucose measurement?
⅔ that of the serum glucose
49
Which type of meningitis is most often associated with complications? What are those complications?
- bacterial more so than either viral or fungal | - includes death due to edema and herniation, hearing loss, hydrocephalus, and seizures
50
Through what pathway does N. meningitidis enter the subarachnoid space?
- it enters via the nasopharynx | - then spreads the blood and gets into the CSF
51
Neurons rely on what energy source?
glucose
52
Why are neurons particularly susceptible to ischemia?
because they rely almost exclusively on glucose for their energy needs
53
After what period of ischemia will neurons undergo necrosis?
between 3-5 minutes after initiation
54
What are four major etiologies for global cerebral ischemia?
- low perfusion (atherosclerosis) - acute decrease in blood flow (cardiac shock) - chronic hypoxia (anemia) - repeated episodes of hypoglycemia (insulinoma)
55
Mild global cerebral ischemia results in what presentation?
transient confusion with prompt recovery
56
Severe global cerebral ischemia results in what presentation?
diffuse necrosis and either a vegetative state or death
57
Moderate global ischemia is most likely to damage which structures and areas within the brain?
watershed areas are vulnerable as are three select groups of neurons: - pyramidal neurons in layers 3, 5, and 6 of the cortex - pyramidal neurons of the hippocampus - purkinje layer of the cerebellum
58
What is laminar necrosis in the brain?
- necrosis of the pyramidal neurons in layers 3, 5, and 6 of the cerebral cortex, which are particularly susceptible to ischemia - the result is "laminar" necrosis within the layers of the cortex
59
Global Cerebral Ischemia
- a state of global ischemia within the brain - most often due to low perfusion, acute decrease in blood flow, chronic hypoxia, or repeated episodes of hypoglycemia - presentation depends on the duration and magnitude of the insult - mild insult results in transient confusion with prompt recovery; severe results in diffuse necrosis and death or a persistent vegetative state; moderate damages watershed areas and particularly susceptible populations of neurons
60
What is the difference between an ischemic stroke and a TIA (transient ischemic attack)?
- TIAs have symptoms lasting less than 24 hours | - ischemic strokes have focal neurologic deficits lasting more than 24 hours
61
What are the three major subtypes of ischemic stroke?
- thrombotic - embolic - lacunar
62
Where are thrombotic strokes in the brain most likely to arise?
atherosclerosis usually develops at branch points such as the bifurcation of the internal carotid and the middle cerebral artery in the circle of Willis
63
What kind of infarct does a thrombotic stroke lead to? Why?
- it leads to a pale infarct in the periphery of the cortex - it is pale since attempts to lyse the thrombus are unsuccessful; if blood flow is momentarily re-established due to lysis, the ruptured plaque just forms a new one
64
Where is the most common source for emboli that contribute to an ischemic stroke?
the left side of the heart
65
Embolic strokes most often affect which part of the cerebral vasculature?
the middle cerebral artery
66
What kind of infarct does an embolic stroke lead to? Why?
- it leads to a hemorrhagic infarct at the periphery of the cortex - this is because the embolus causes necrosis but can be lysed and then the re-established blood flow fills the necrotic space
67
Ischemic strokes lead to what sort of necrosis within the brain?
liquefactive necrosis
68
What is the difference between a thrombotic stroke and an embolic stroke?
- the mechanisms are obviously different - thrombotic stroke leads to a pale infarct while an embolic stroke leads to a hemorrhagic infarct; this is because the embolus can be lysed and blood flow to the area restored, which isn't possible after a thrombotic stroke - thrombotic strokes most often occur at branch points, namely the bifurcation of the internal carotid and middle cerebral artery in the circle of Willis - embolic strokes most often occur in the middle cerebral artery from clots thrown from the left heart
69
Lacunar Stroke
- a small stroke that occurs secondary to hyaline arteriolosclerosis, a complication of benign hypertension - this most commonly involves the lenticulostriate vessels arising from the MCA and serving the deep structures of the brain - the result is small, cystic areas of infarction
70
Where are the lenticulostriate vessels?
they arise from the MCA and serve the deep brain structures such as the internal capsule and thalamus
71
What kind of lacunar stroke would result in only motor symptoms?
one that involves the internal capsule
72
What kind of lacunar stroke would result in a purely sensory stroke?
one that involves the thalamus
73
Describe the timeline and histologic changes seen after an ischemic stroke.
- the first thing to arise are eosinophilic, red neurons 12 hours after infarction - necrosis is seen after 24 hours - infiltration by neutrophils in 1-3 days - microglial cell infiltrate in 4-7 days - gliosis (scarring) in the 2-3 weeks that follow - the result is a fluid-filled cystic space surrounded by gliosis
74
What is the first change that can be seen after ischemic injury within the brain?
the presence of eosinophilic, red neurons
75
Describe the scar that forms after an ischemic stroke.
it is a fluid-filled cystic space surrounded by gliosis
76
What is a Charcot-Bouchard microaneurysm?
- an aneurysm of the lenticulostriate vessels arising due to hypertension, most often found in the basal ganglia - not visible on an angiogram
77
Intracerebral Hemorrhage
- a bleed into the brain parenchyma, classically due to rupture of a Charcot-Bouchard microaneurysm, amyloid angiopathy, vasculitis, or neoplasm - these aneurysms arise as a complication of hypertension and most often in the basal ganglia - presents with severe headache, n/v, and eventual coma - Charcot-Bouchard rupture typically involves the basal ganglia and internal capsule
78
How are Charcot-Bouchard micro aneurysms treated?
by treating the underlying hypertension
79
How would a subarachnoid hemorrhage appear on gross examination?
as a large blood clot/pool at the base of the brain
80
Subarachnoid Hemorrhage
- a bleed into the subarachnoid space - most frequently due to rupture of a berry aneurysm but it can also be the result of an AV malformation or anti-coagulated state - presents as a sudden headache, classically described as the worst headache of the patients life, with nuchal rigidity - lumbar puncture shows xanthochromia, a yellow hue due to bilirubin - inspection of the brain will reveal a large clot or bloody mass at the base of the brain - may be followed several days later by vasospasm and ischemic infarct, so provide nimodipine for prevention - increases risk for developing communicating or obstructive hydrocephalus - aw/ Marfan and AD-PCKD
81
Why would a lumbar puncture show xanthochromia?
it is a yellow hue of the CSF due to the presence of bilirubin, arising due to a subarachnoid hemorrhage
82
What is a berry aneurysm? Where are they most often located? What other disease are they associated with?
- it is an aneurysm lacking a media layer - most frequently located in the anterior circle of Willis at branch points of the anterior communicating artery; secondarily at the posterior communicating artery or MCA - associated with Marfan syndrome, Ehlers-Danlos syndrome, and ADPKD - other risk factors include advanced age, hypertension, smoking, and African American race
83
Epidural Hematoma
- a collection of blood between the dura and the skull - classically caused by a fracture of the temporal bone, which ruptures the middle meningeal artery - must be an artery because a vein doesn't have enough pressure to open a space between the dura and temporal bone - often presents with a lucid interval before the onset of neurologic signs and progression indicates an abrupt expansion - expansion may cause transtentorial herniation with CN III palsy - seen as a "lens-shaped" lesion on CT which doesn't cross suture lines - herniation is the feared, lethal complication
84
How does an epidural hematoma differ from a subdural hematoma?
- epidural appears lens-shaped on CT while subdural appears crescent-shaped - epidural is associated with trauma and fracture of the temporal bone, which injures the middle meningeal artery while subdural is associated with age-related cerebral atrophy and rupture of veins - epidural hematomas often have a lucid period after the injury followed by the onset of neurologic symptoms while subdurals have no associated lucid period
85
Subdural Hematoma
- a collection of blood beneath the dura - due to tearing of the bridging veins that lie between the dura and arachnoid, typically with trauma - presents with the immediate onset of progressive neurologic signs - crescent-shaped lesion on CT that crosses suture lines and which is hyperdense if acute and hypodense if chronic - more common in the elderly as a result of age-related cerebral atrophy stretching the veins that mediate disease - transtentorial herniation is the feared, lethal complication
86
Tonsillar Herniation
- displacement of the cerebellar tonsils into the foramen magnum - results in compression of the brainstem and cardiopulmonary arrest - the most common form of brain herniation
87
What is brain herniation?
displacement of brain tissue due to mass effect or increased ICP
88
Subfalcine Herniation
- displacement of the cingulate gyrus under the fall cerebri - results in compression of the anterior cerebral artery, leading to infarction --> lower extremity sensory and motor problems
89
Uncal Herniation
- displacement of the temporal lobe uncus under the tentorium cerebelli - compresses CN III, leading to a "down and out" positioning of the eye with dilated pupil - also compresses the PCA, leading to infarction of the occipital lobe with contralateral homonymous hemianopsia with macular sparing - Kernohan notch (indentation of the contralateral cerebral peduncle) results in a "false localization" sign with paralysis on the side ipsilateral the primary lesion/herniation
90
Metachromatic Leukodystrophy
- a autosomal recessive deficiency of arylsulfatase, which prevents normal degradation of sulfatides, allowing them to accumulate in oligodendrocyte lysosomes - leads central and peripheral demyelination with ataxia and dementia - the most common leukodystrophy
91
Krabbe Disease
- an autosomal recessive deficiency of galactocerebrosidase, leading to accumulation of galactocerebroside in macrophages - demyelination presents with peripheral neuropathy, developmental delay, optic atrophy, and globoid cells
92
Adrenoleukodystrophy
an X-linked defect, which impairs the addition of CoA to LCFAs (long-chain fatty acids), which then accumulate and damage the adrenal glands and white matter
93
Multiple Sclerosis
- autoimmune destruction of oligodendrocytes and CNS myelin - risk factors include women with the HLA-DR2 haplotype living far from the equator - primary symptoms are Charcot's triad of SIN: scanning speech, intention tremor (also incontinence, internuclear ophthalmoplegia), and nystagmus - diagnosed by MRI, which reveals plaques that vary in time and space, or a lumbar puncture, which demonstrates lymphocytes, an oligoclonal IgG expansion, and elevated myelin basic protein - gross examination finds gray-appearing plaques in the white matter - treatment for acute attacks is high-dose steroids; IFN-B is preferred for long-term treatment to slow progression
94
What are the major risk factors for MS?
- age 20-30 - women - HLA-DR2 genotype - living further from the equator
95
What are common symptoms of MS and in what region are the associated lesions?
- unilateral blurred vision - optic nerve - vertigo or scanning speech mimicking alcohol intoxication - brainstem - internuclear ophthalmoplegia - medial longitudinal fasciculus - hemiparesis or unilateral loss of sensation - cerebral white matter, most often periventricular - lower extremity loss of sensation or weakness - spinal cord - bowel, bladder, and sexual dysfunction - ANS
96
What procedures are used to diagnose MS? What do we expect to find using these methods?
- MRI is used to identify plaques in the white matter which vary across time and space - lumbar puncture is expected to reveal lymphocytes, an oligoclonal IgG expansion, and myelin basic protein
97
How is MS treated?
- long term treatment involves IFN-B to slow progression | - supplemented by high dose steroids during acute attacks
98
Subacute Sclerosing Panencephalitis
- a progressive encephalitis leading to death - due to persistent infection of the brain by measles - the infection occurs in infancy but neurologic signs arise many years later during late childhood - histology finds viral inclusions within neurons of the grey matter and in oligodendrocytes
99
Progressive Multifocal Leukoencephalopathy
- a JC virus infection of oligodendrocytes - arises from reactivation of latent virus in a newly immunosuppressed or immunocompromised patient - presents with rapidly progressive neurologic signs and death - there is an increased risk associated with use of natalizumab and rituximab
100
Central Pontine Myelinolysis
- aka osmotic demyelination syndrome - a focal demyelination of the pons caused by rapid IV correction of hyponatremia - results in an acute bilateral paralysis, commonly referred to as "locked in" syndrome because the patient is often only able to move his or her eyes
101
Degeneration of what part of the brain leads to dementia? Degeneration of what part leads to a movement disorder?
- the cortex leads to dementia | - degeneration of deep structures like the brainstem and in particular the basal ganglia lead to movement disorders
102
Alzheimer Disease
- a degenerative disease of the cortex, causing dementia - arises due to B-secretase degradation of the APP protein, which can't be processed like the A-secretase product and is deposited in the brain tissue instead - presents with slow-onset memory loss (beginning with short-term loss and then long-term), progressive disorientation, loss of learned motor skills and language, and changes in behavior or personality - patients often become mute and bedridden to die later of infection - may be complicated by cerebral amyloid angiopathy - degree of dementia correlates with the number of neurofibrillary tangles - most cases are sporadic and arises in the elderly (risk doubles every 5 years after age 60) but early onset AD is seen in association with presenilin 1 and 2 mutations as well as in those with Down syndrome - gross examination of the brain reveals central atrophy, narrowing of the gyri, widening of the sulci, and dilation of the ventricles - histology reveals neuritic plaques with an AB amyloid and entangled neuritic processes in addition to the presence of intracellular, hyperphosphorylated tau protein deposits - it is a clinical diagnosis and can only be confirmed by histology at autopsy
103
What is the most common cause of dementia?
Alzheimer's disease
104
What is the pathogenesis of Alzheimer's disease?
- normally a-secretase degrades the APP gene product to a structure that can be turned over by cells and properly handled - in those with Alzheimer's B-secretase has degraded the APP protein to a different product that can't be properly handled and is instead deposited as AB-amyloid
105
What genetic associations are there with Alzheimer's disease?
- the ApoE4 allele increases risk while the ApoE2 allele lowers it - the presenilin 1 and 2 genes are associated with familial, early onset Alzheimer's - most with Down syndrome have Alzheimer's by age 40
106
What is cerebral amyloid angiopathy?
a vascular disease of the brain seen in patients with Alzheimer's disease as AB amyloid is deposited around vessels, increasing the risk for hemorrhage
107
Vascular Dementia
- a dementia that arises from multifocal infarction and injury secondary to hypertension, atherosclerosis, or vasculitis - constituting the second most common cause of dementia
108
Why is it important to recognize a vascular dementia?
because you can treat the underlying condition and slow or stop the progression of the dementia
109
Pick Disease
- a degenerative disease of the frontal and temporal lobes - presents primarily with behavioral and language symptoms in the early stages and then progresses to dementia - characterized histologically by round aggregates of tau protein in neurons of the cortex
110
What is tau protein? Why is it clinically significant?
- it is a microtubule-associated protein that normally helps organize microtubules - it is clinically important because when it becomes hyperphosphorylated, it is a marker or mediator of Alzheimer's disease - it is also the protein that forms Pick bodies
111
How do the tau tangles of Alzheimer's differ from the tau aggregates seen in those with Pick disease?
- in Alzheimer's they form whispy, flame-like intracellular structures - in Pick disease they form actual aggregates
112
Hyperphosphorylated tau proteins are a feature of which CNS disorders?
Alzheimer's disease and Pick disease
113
Parkinson Disease
- the degenerative loss of dopaminergic neurons in the substantia nigra of the basal ganglia, which is partly responsible for initiating movement - related to aging or in rare cases to MPTP exposure - presents with TRAP: tremor (pill rolling), rigidity (cog wheel), akinesia (or bradykinesia), and postural instability with shuffling gait - dementia is a feature of late disease - histology reveal the loss of the neuromelanin-pigmented neurons as well as Lewy bodies (eosinophilic inclusions of a-synuclein)
114
Which cell population is the focus of Parkinson disease?
the dopaminergic neurons in the substantia nigra of the basal ganglia
115
Exposure to MPTP brings about what sort of neurodegenerative disorder?
a disorder resembling Parkinson disease
116
Describe the tremor associated with Parkinson disease.
it is called a pill rolling tremor and it appears at rest, disappearing with movement
117
Describe the rigidity associated with Parkinson disease.
it is called cogwheel rigidity and it is a rigidity that can be observed with passive movement of the limbs
118
What do we call the pigment that is derived from catecholamines and stains the substantia nigra?
neuromelanin
119
What are Lewy bodies?
- cytoplasmic eosinophilic inclusions of a-synuclein that are visible on histology - seen in those with Lewy body dementia (cortical) and Parkinson disease (substantia nigra) - have a halo surrounding them
120
How can Lewy body dementia and Parkinson disease be differentiated?
dementia is a late feature of Parkinson disease whereas Lewy body has parkinsonian features with early onset dementia and hallucinations
121
Lewy Body Dementia
- a degenerative disorder most similar to Parkinson disease - presents with early-onset dementia (which distinguishes it from Parkinson disease), Parkinsonian features, and hallucinations - histology reveals cortical Lewy bodies
122
Huntington Disease
- a degeneration of GABAergic neurons in the caudate nucleus of the basal ganglia - due to an autosomal dominant trinucleotide expansion of CAG in the huntingtin gene on chromosome 4 - this repeat demonstrates anticipation, specifically in spermatogenesis - presents at age 40 with chorea, including athetosis, and can progress to dementia and depression - suicide is a common cause of death - grossly, you can identify atrophy of the caudate and putamen with hydrocephalus ex vacuo
123
What population of neurons are affected by Huntington disease?
GABAergic neurons in the caudate nucleus of the basal ganglia
124
What mutation is associated with Huntington disease?
a CAG trinucleotide repeat in the huntingtin gene on chromosome 4, which demonstrates anticipation in spermatogenesis
125
Normal Pressure Hydrocephalus
- an increase in CSF that results in dilated ventricles, which subsequently stretches the surrounding corona radiata - presents in the elderly with triad of urinary incontinence, gait instability, and dementia ("wet, wobbly, and wacky") - have a characteristic gait known as "magnetic gait" because their feet appear stuck to the floor - characteristically, lumbar puncture improves symptoms and treatment is ventriculoperitoneal shunting
126
Which form of dementia typically improves at the time of a lumbar puncture?
normal pressure hydrocephalus
127
Spongiform Encephalopathy
- a group of degenerative diseases due to prior protein - this protein is normally expressed in an a-helical configuration but disease arises with conversion to a B-pleated conformation - the resulting protein cannot be degraded and converts normal protein into the pathologic form, damaging neurons and glial cells - can be sporadic, inherited, or transmitted - Creutzfeld-Jakob disease is the most common but familial fatal insomnia is an inherited form - histology reveals spongy degeneration (intracellular vacuoles)
128
What is familial fatal insomnia?
an inherited form of prion disease/spongiform encephalopathy characterized by severe insomnia and an exaggerated startle response
129
Creutzfeldt-Jakob Disease
- the most common spongiform encephalopathy - usually sporadic but can arise from exposure to prion-infected human tissue via human GH or corneal transplant - presents as a very rapidly progressing dementia associated with ataxia and startle myoclonus - death arises within one year - "spike wave" complexes are seen on EEG - variant CJD is a special form known as "mad cow" disease which arises from exposure to prion-infected bovine tissue
130
What is the most rapidly progressing neurodegenerative disorder?
Creutzfeldt-Jakob Disease
131
How can metastatic brain tumors often be differentiated from primary tumors?
metastatic tend to be multiple, well-circumscribed lesions located at the gray-white junction
132
What are the most common origins for metastases to the brain?
lung, breast, and kidney
133
How do brain tumors differ between adult populations and child populations?
- adults tend to have supratentorial primary tumors | - children tend to have infratentorial primary tumors
134
Glioblastoma Multiforme
- a malignant, high-grade, GFAP-positive astrocyte tumor - characteristically crosses the corpus callosum, forming a "butterfly lesion" - histologically there are regions of pseudopalisading with tumor cells surrounding areas of necrosis - the most common primary malignant CNS tumor in adults and they have a poor prognosis
135
Meningioma
- a benign tumor of arachnoid cells - the most common benign CNS tumor in adults and more common in women than men - presents with seizures since the tumor compresses, but does not invade, the cortex - imaging reveals a round mass attached to the dura - histology shows a very distinctive whorled pattern with psammoma bodies
136
Schwannoma
- a benign tumor of Schwanna cells - can involve cranial nerves, spinal nerves, or CNS tissue - most frequently involves CN VIII at the cerebellopontine angle and presents with a loss of hearing and tinnitus - tumor cells stain S-100+ - bilateral tumors are a feature of neurofibromatosis 2
137
Oligodendroglioma
- a malignant tumor of oligodendrocytes - a slow growing, calcified tumor in the white matter, most often involving the frontal lobe - histology reveals cells with a "fried egg" appearance and a "chicken-wire" pattern of capillaries - may present with seizures
138
Pilocytic Astrocytoma
- a benign tumor of astrocytes - this is the most common CNS tumor in children and is typically located in the cerebellum - arises as a cystic lesion with a mural nodule, which is easily identifiable on imaging - histology shows Rosenthal fibers and eosinophilic granular bodies - the section will be GFAP positive
139
Medulloblastoma
- a malignant tumor derived from the granular cells of the cerebellum, which are neuroectoderm - usually arises in children - histology reveals small, round but cells forming Homer-Wright rosettes - the tumor grows rapidly and spreads via the CSF; therefore it has a poor prognosis - a metastasis to the cauda equina is known as a "drop metastasis"
140
Ependymoma
- a malignant tumor of the ependymal cells lining the ventricles - most often in the fourth ventricle of children - may present with hydrocephalus if the tumor forms an obstruction - histology reveals perivascular pseudorosettes
141
Craniopharyngioma
- a tumor arising from the epithelial remnants of Rathke's pouch (oral ectoderm) left behind on it's way to form the anterior pituitary - presents as a supratentorial mass in a child or young adult and may compress the optic chasm leading to a bitemporal hemianopsia - calcifications can be seen on imaging and cholesterol crystals are found in "motor oil-like" fluid within the tumor - most common childhood supratentorial tumor - although benign, it tends to recur
142
What is a "drop metastasis"?
a medulloblastoma metastasis to the cauda equina via the CSF
143
What are the most common primary CNS tumors seen in children?
- pilocytic astrocytoma - ependymoma - medulloblastoma
144
What are the most common primary CNS tumors seen in adults?
- glioblastoma multiforme - meningioma - Schwannoma
145
What are two possible causes for bitemporal hemianopsia?
- pituitary adenoma | - craniopharyngioma
146
What is the notochord?
a portion of mesoderm, which induces overlying ectoderm to differentiate into neuroectoderm and later gives rise to the nucleus pulposus
147
What are the alar and basal plates of the neural tube?
- alar: dorsal portion, which is associated with sensory information - basal: ventral portion, which is associated with motor activity
148
List the primary vesicles of the brain and the secondary vesicles they give rise to.
- prosencephalon gives rise to the telencephalon and diencephalon - mesencephalon to the mesencephalon - rhombencephalon to the metencephalon and myelencephalon
149
List the secondary vesicles of the developing brain and the adult structures to which they give rise.
- telencephalon: cerebral hemispheres and lateral ventricles - diencephalon: thalamus, hypothalamus, and third ventricle - mesencephalon: midbrain and aqueduct - metencephalon: pons, cerebellum, and upper part of 4th ventricle - myelencephalon: medulla and lower part of the 4th ventricle
150
What adult cell populations are derived from neuroectoderm?
- CNS neurons - ependymal cells - oligodendroglia - astrocytes
151
What adult neural cell populations are derived from neural crest?
PNS neurons and Schwann cells
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What adult neural cell population is derived from mesoderm?
microglia (macrophages)
153
Holoprosencephaly
- a failure of the left and right hemispheres to separate, usually during weeks 5-6 of gestation - thought to be related to mutations in the sonic hedgehog signaling pathway - moderate form presents with a cleft lip/palate and the most severe form results in cyclopia (failure of the eye to separate) - seen in associate with Patau syndrome (trisomy 13) and fetal alcohol syndrome
154
Describe development of the tongue, including the structures it arises from and it's innervation.
- the anterior tongue is derived from the 1st and 2nd arches, thus sensation is mediated by CN V3 and taste by CN VII - the posterior ⅓ is derived from the 3rd and 4th arches, thus sensation and taste are mediated by CN IX (and X in the most posterior aspect) - motor innervation is via CN X to the palatoglossus and via CN XII to the hyoglossus, genioglossus, and styloglossus
155
The tongue is derived from what embryonic structures?
- the anterior tongue from branchial arches 1 and 2 | - the posterior tongue from branchial arches 3 and 4
156
Which nerves are responsible for taste, sensation, and motor innervation of the tongue?
- taste: CN VII, IX, X - sensation: CN V3, IX, X - motor: CN X, XII
157
List the four muscles of the tongue, their innervation, and their function.
- hyoglossus (XII): retracts and depresses tongue - genioglossus (XII): protrudes tongue - styloglossus (XII): draws sides of tongue upward to create a trough for swallowing - palatoglossus (X): elevates posterior tongue during swallowing
158
What is a Nissl stain?
a stain for RER used to identify neuronal cell bodies and dendrites (the axon does not contain any RER)
159
What is Wallerian degeneration?
axonal degeneration distal to an injury with axonal retraction proximal to the injury, which allows for potential regeneration of the axon if in the PNS
160
What are the functions of astrocytes?
- component of the BBB - supply glycogen fuel buffer - mediate reactive gliosis - provide physical support - repair - potassium metabolism - remove excess NT
161
What is GFAP?
an intermediate filament expressed by astrocytes and ependymal cells, which can be used for immunohistochemical purposes
162
What is the primary action of microglia?
they are phagocytic scavengers activated in response to tissue damage
163
What are multi-nucleated cells in the CNS indicative of? What cell type are they formed from?
they form when HIV infects microglia, which then fuse to form these cells
164
Which glial cell is not readily stained by Nissl?
microglia
165
What is concentrated at the nodes of Ranvier?
sodium channels
166
What do we call the conduction down a nerve that is myelinated?
saltatory conduction
167
What is the difference between a Schwann cell and an oligodendrocyte?
- oligodendrocytes myelinated multiple cells at one time in the CNS - Schwann cells myelinated a single axon in the PNS
168
What are the two functions of Schwann cells in the PNS?
- they myelinated axons | - they promote axonal regeneration
169
Which glial cell population is damaged in those with Guillan-Barre syndrome?
Schwann cells
170
What is the predominant type of glial cell in white matter?
oligodendrocytes
171
What neural cell type has a "fried egg" appearance?
oligodendrocytes
172
Describe free nerve ending sensory receptors. Where are they located, what information are they tuned to, how quickly do they adapt, and what size fiber are they linked to?
- they are located throughout all skin in the epidermis and in some viscera - they sense pain and temperature - they are carried by both C and Ad fibers
173
Describe Meissner corpuscle sensory receptors. Where are they located, what information are they tuned to, how quickly do they adapt, and what size fiber are they linked to?
- they are located in the dermal papillae of glabrous (hairless) skin, most concentrated in the finger tips - they sense dynamic, fine touch, specifically low frequency vibration - they adapt quickly and are linked to large, myelinated AB fibers
174
Describe Pacinian corpuscle sensory receptors. Where are they located, what information are they tuned to, how quickly do they adapt, and what size fiber are they linked to?
- they are located in the deep skin layers of thin skin - they sense higher frequency vibration and pressure - they adapt quickly and are linked to large, myelinated AB fibers
175
Describe Merkel disc sensory receptors. Where are they located, what information are they tuned to, how quickly do they adapt, and what size fiber are they linked to?
- they are located in the epidermis of glabrous skin - they sense pressure, deep static touch, and position sense - they adapt slowly and are linked to large, myelinated AB fibers
176
Describe Ruffini corpuscle sensory receptors. Where are they located, what information are they tuned to, how quickly do they adapt, and what size fiber are they linked to?
- they are located in the dermis of thin skin - they sense pressure on the skin in a specific direction (aka slippage of objects), joint angle change, and pressure - they adapt slowly and are innervated by AB fibers
177
What is endoneurium? Why are they clinically important?
- the delicate layer of connective tissue that invests a single myelinated nerve fiber layer - an inflammatory infiltrate is seen in this layer of tissue in those with Guillan-Barre
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What is the perineurium? Why is it clinically important?
- a layer of connective tissue that surrounds a fascicle of nerve fibers - they must be rejoined in microsurgery for limb reattachment
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What is epineurium?
a layer of dense connective tissue that surrounds an entire nerve and contains several fascicles and blood vessles
180
What is the principle site of acetylcholine synthesis in the brain?
the basal nucleus of Meynert
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What is the principle site of dopamine synthesis in the brain?
ventral tegmenjtum and substantia nigra pars compacta
182
What is the principle site of GABA synthesis in the brain?
the nucleus accumbens
183
What is the principle site of NE synthesis in the brain?
the locus ceruleus
184
What is the principle site of serotonin synthesis in the brain?
raphe nucleus
185
What NT changes are associated with disorders of anxiety?
low serotonin and GABA, excess NE
186
What NT changes are associated with disorders of depression?
low serotonin, dopamine, and NE
187
What NT changes are associated with schizophrenia?
increased dopamine
188
What NT changes are associated with Alzheimer's disease?
low acetylcholine
189
What NT changes are associated with Huntington's disease?
low acetylcholine and GABA, excess DA
190
What NT changes are associated with Parkinson's disease?
low DA, excess acetylcholine and serotonin
191
The blood brain barrier is formed by what three layers?
- tight junctions between non-fenestrated capillary endothelial cells - the basement membrane - astrocyte foot processes
192
Which substances readily diffuse across the BBB?
non polar, small, lipid-soluble substances
193
Which substances readily cross the BBB via carrier-mediated transport?
glucose and amino acids
194
How does glucose cross the BBB?
via carrier-mediate transport
195
How do amino acids cross the BBB?
via carrier-mediated transport
196
What is vasogenic edema?
a type of cerebral edema that results from disruption of the BBB, usually by infarction or neoplasms which damage endothelial tight junctions
197
List three areas of the brain with fenestrated capillaries and no BBB (circumventricular organs). Why is this critical for the functioning of these regions?
Sensory Organs - the area postrema is a medullary structure that must sample the blood because it controls vomiting - the organum vasculosum of the lamina terminalis sense osmotic changes Secretory Organs - neurohypophysis releases vasopressin and oxytocin
198
The hypothalamus regulates what?
TAN HATS - thirst and water balance - adenohypophysis control - neurohypophysis release of hormones - hunger - autonomic regulation - temperature regulation - sexual urges
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What is the organum vasculosum of the lamina terminalis?
a circumventricular organ that senses changes in osmolarity and communicates those changes to the hypothalamus
200
What is the area postrema?
a circumventricular organ in the medulla that responds to emetics via output to the hypothalamus
201
What is the role of the supraoptic nucleus of the hypothalamus?
make and release ADH
202
What is the role of the paraventricular nucleus of the hypothalamus?
make and release oxytocin
203
What is the function of the lateral area of the hypothalamus? What stimulates and inhibits it? What happens if it is lesioned?
- it mediates hunger and is therefore stimulated by ghrelin and inhibited by leptin - destruction leads to anorexia and failure to thrive
204
What is the function of the ventromedial area of the hypothalamus? What stimulates and inhibits it? What happens if it is lesioned?
- it mediates satiety and is therefore stimulated by leptin | - destruction leads to hyperphagia
205
Why would a craniopharyngioma present with hyperphagia?
because it could invade and destroy the ventromedial area of the hypothalamus
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What is the function of the anterior hypothalamus? What stimulates and inhibits it? What happens if it is lesioned?
- it senses temperature by monitoring the surrounding fluid temperature and receiving somatic input - it promotes cooling via activation of the parasympathetic system in response to heat
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What is the function of the posterior hypothalamus? What stimulates and inhibits it? What happens if it is lesioned?
- it senses temperature by monitoring the surrounding fluid temperature and receiving somatic input - it promotes heating via activation of the sympathetic system in response to cold
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What is the function of the suprachiasmatic nucleus of the hypothalamus? What stimulates and inhibits it? What happens if it is lesioned?
it mediates circadian rhythm via direct input from melanopsin-containing retinal ganglion cells along the retino-hypothalamic tract
209
Which portion of the hypothalamus regulates descending control of the sympathetic nervous system?
the paraventricular
210
What drives melatonin release to induce sleep?
- the suprachiasmatic nucleus receives input from the retinal ganglion cells to regulate the circadian rhythm - in responses to changes in light, the SCN releases NE onto the pineal gland, which releases melatonin
211
Melatonin is released from which structure? What stimulates release of melatonin from this structure?
the the pineal gland in response to norepinephrine released from projections originating in the suprachiasmatic hypothalamic nucleus
212
Extraocular movements during REM sleep are mediated by activity in which brain region?
the paramedian pontine reticular formation
213
Describe the neural pathway for voluntary eye movements.
- frontal eye field (area 8) - superior colliculus - paramedian pontine reticular formation - oculomotor nuclei - eye muscles & movement
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Describe the neural pathway for reflex saccades.
- retinal ganglion cells - superior colliculus - paramedian pontine reticular formation - oculomotor nuclei - eye muscles & movement
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Describe the neural pathway for smooth pursuit eye movements.
- retinal slip of image on retina - retinal ganglion cells - LGN - visual cortex - paramedian pontine reticular formation - oculomotor nuclei - eye muscles & movement
216
What is the vestibul-ocular reflex. Describe the neural pathway.
- it is used to keep the eyes still in response to head movements - begins with displacement of vestibular hair cells, AP along CN VIII to the vestibular nucleus, projecting to the oculomotor nucleus, which then controls eye movement
217
What happens within the 8th nerve when vestibular hair cells bend toward the kinocilium?
the rate of depolarization within the 8th nerve increases
218
Describe the response vestibular response to the Barany chair test.
- initially, during acceleration to the right, there is an increase in right CN 8 activity because the endolymph moves to the left relative to the head and bends the hair cells toward the more medial kinocilium - once rotation becomes constant, the endolymph is equilibrated and there is no signal - during deceleration, there is an increase in left 8 nerve activity compared to right because the individual stops but the endolymph continues flowing - this causes the patient to fall to the right or for their eyes to drift right and rapidly correct left
219
How does REM change throughout the course of the night?
it occurs every 90 minutes but the duration increases later in the night
220
The balance of which neurotransmitters is thought to mediate REM sleep?
REM is dependent on a rise in acetylcholine and fall in norepinephrine
221
What is the effect of alcohol, benzos, and barbiturates on sleep?
they decrease REM and slow wave sleep
222
How is bedwetting treated?
with oral desmopressin
223
How are night terrors and sleep walking treated?
benzodiazepines
224
What are beta brain waves and when are they seen?
they are high frequency (>12 Hz), low amplitude waves seen when awake with eyes open
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What is the difference between alpha and beta brain waves?
both are seen when awake but alpha waves are slower and larger in amplitude and are associated with having one's eyes closed
226
Describe stage 1 sleep.
- light sleep | - EEG demonstrates theta waves
227
Describe stage 2 sleep.
- deeper sleep - EEG demonstrates sleep spindles and K complexes - this is when bruxism (teeth grinding) occurs
228
What is bruxism? When during sleep does it occur?
it is teeth grinding and it occurs during stage 2 sleep
229
Describe stage 3 sleep.
- deepest non-REM sleep - EEG demonstrates delta waves, which are low frequency, high amplitude - this is the stage during which sleepwalking, night terrors, and bedwetting occur
230
When during sleep do night terrors and sleepwalking occur?
during stage 3
231
Describe control of wakefulness.
- the thalamic reticular nucleus receives collateral input from the thalamocortical and corticothalamic tracts - GABAergic neurons from the thalamic reticular nucleus then project to the thalamus as does the reticular activating system - the balance in the thalamus modulates the thalamocortical projections, which then synchronize the activity of the cortex
232
What changes are observed when someone is in REM sleep?
- beta waves - loss of motor tone - increase O2 demand from the brain - fluctuating pulse and blood pressure - this is also when dreaming and nightmares occur - penile/clitoral tumescence
233
What sort of EEG characterizes alert wakefulness, awake with eyes closed, stage 1 sleep, stage 2 sleep, stage 3 sleep, and REM sleep?
- alert: beta - eyes closed: alpha - stage 1: theta - stage 2: spindles and K complexes - stage 3: delta - REM: beta
234
What is the role of the ventral postero-lateral nucleus of the thalamus?
it is the location of tertiary neurons in the spinothalamic and DC/ML pathways
235
What is the role of the ventral postero-medial nucleus of the thalamus?
- it relays somatosensory information from the face, including taste - it receives input from the trigeminal and gustatory pathway (solitary nucleus) and projects to the somatosensory cortex
236
What is the role of the lateral geniculate nucleus of the thalamus?
- it is a relay for visual information | - it receives input from CN II and projects to the calcarine sulcus (aka primary visual cortex)
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What is the role of the medial geniculate nucleus of the thalamus?
- it is a relay for auditory information - it receives input from the superior olive and inferior colliculus - and projects to the auditory cortex of the temporal lobe
238
What is the role of the ventral lateral nucleus of the thalamus?
- it helps with coordination and planning of motor activity - it receives input from the basal ganglia and cerebellum - and projects to the motor cortex
239
Name five structures that are part of the limbic system.
- hippocampus - amygdala - fornix - mammillary bodies - cingulate gyrus
240
What are the functions of the limbic system?
- involved in emotion, long-term memory olfaction, behavior modulation, and ANS functioning - said to mediate the 5 F's: feeding, fleeing, fighting, feeling, and sex
241
What are the four dopaminergic pathways within the CNS?
- mesocortical - mesolimbic - nigrostriatal - tuberoinfundibular
242
A defect within the mesocortical pathway results in what?
a decrease in activity results in the negative symptoms of schizophrenia
243
A defect within the mesolimbic pathway results in what?
an increase in activity results in the positive symptoms of schizophrenia
244
The benefits of anti-psychotics arise from modulation of which dopaminergic pathway within the CNS?
they decrease activity in the mesolimbic pathway
245
A defect within the nigrostriatal pathway results in what?
- a decrease in activity leads to extrapyramidal symptoms such as those seen in Parkinson's disease - it is significantly affected by movement disorders and antipsychotic drugs
246
A defect in the tuberoinfundibular pathway results in what?
an increase in activity increases prolactin levels resulting in reduced libido, sexual dysfunction, galactorrhea, and gynecomastia
247
The tuberoinfundibular pathway regulates what?
it supplies the dopaminergic input to the anterior pituitary, which inhibits the release of prolactin
248
What is the function of the superior, middle, and inferior cerebellar peduncles?
- superior: output of deep nuclei of the cerebellum to the contralateral cortex - middle: input from the cortex to the contralateral cerebellum - inferior: propioceptive input from the ipsilateral spinal cord
249
What cell population serves as the primary output of the cerebellar cortex?
purkinje cells
250
List the deep nuclei of the cerebellum from lateral to medial.
- dentate - emboliform - globose - fastigial
251
Lateral cerebellar lesions have what results?
they affect primarily the neocerebellum and thus affect movement of the extremities; when injured, they increase the propensity for falling toward the ipsilateral side
252
Medial cerebellar lesions have what results?
such a lesion would involve the vermal cortex and fastigial nuclei, resulting in truncal ataxia (a wide-based cerebellar gait), nystagmus, and head tilting with bilateral motor deficits affecting axial and proximal limb musculature
253
What are the three functional divisions of the cerebellum?
- neocerebellum: receives input form the cortex and regulates initiation and programming of movement via the dentate nucleus - spinocerebellum: includes the vermis and paravermis; the paravermis is more lateral receives spinocerebellar input and then helps with correction of extremity movement; the vermis is more midline and receives spinocerebellar as well as vestibular input to help with correction of axial movement - vestibulocerebellum
254
Draw the connections within the basal ganglia.
Page 460 of FA
255
What is the net effect of the direct and indirect pathways within the basal ganglia.
- the direct is excitatory and promotes positive feedback to the motor cortex - the indirect is inhibitory and promotes negative feedback to the motor cortex
256
What is the basal ganglia striatum?
the putamen and caudate
257
What is the basal ganglia lentiform?
the putamen and globes pallidus
258
Describe the direct pathway within the basal ganglia.
- cortical inputs stimulate the striatum, stimulating release of GABA - this inhibits GABA release from the GPi, which disinhibits the thalamus - the thalamus then promotes further motor activity
259
Describe the indirect pathway within the basal ganglia.
- cortical inputs stimulate the striatum, stimulating release of GABA into the GPe - the GPe then releases less GABA itself, disinhibiting the subthalamic nucleus - the sub thalamic nucleus stimulate the GPi, which then released more GABA onto the thalamus - the thalamus is inhibited and sends fewer stimulatory signals to the cortex, inhibiting movement
260
Describe the effect of the substantia nigra pars compact on the basal ganglia.
- the SNc releases dopamine, which binds D1 receptors in the striatum of the direct pathway and D2 receptors in the striatum of the indirect pathway - as a result it up regulates the direct pathway and down regulates the indirect pathway, stimulating movement
261
What is an athetosis?
a slow, writhing movement, especially seen in the fingers, which arises from a basal ganglia lesion (e.g. Huntington's disease)
262
What is a chorea?
a sudden, jerky, purposeless movement that arises from a basal ganglia lesion (e.g. Huntington's disease)
263
What is a dystonia?
a sustained, involuntary muscle contraction that is often painful
264
Essential Tremor
- a high-frequency tremor that occurs with sustained posture and is worsened with movement or when anxious - often familial - often self-medicated with alcohol - treat with a nonselective beta-blocker or primidone (a barbiturate)
265
Hemiballismus
- a sudden, wild flailing of 1 arm with or without flailing of the ipsilateral leg - caused by a lesion in the contralateral subthalamic nucleus most often from a lacunar stroke
266
What is an intention tremor? Where does it suggest there is a brain lesion?
- it is a slow, zigzag motion that arises when pointing or extending toward a target - signifies cerebellar dysfunction
267
What is a myoclonus?
a sudden, brief, uncontrolled muscle contraction common in renal and liver failure which result in metabolic abnormalities
268
What is the function of the frontal eye field?
it is a region in the frontal cortex responsible for controlling visual attention and generating voluntary eye movements
269
What is the arcuate fasciclus?
a direct connection between Wernicke's area and Broca's area that allows for repetition without comprehension
270
What are the functions of Broca's and Wernicke's area and where is each located?
- Broca's is responsible for motor speech and is located in the inferior frontal gyrus of the frontal lobe - Wernicke's is responsible for speech comprehension and is located in the superior humoral gyrus of the temporal lobe
271
What is the purpose of the ventral and dorsal perceptual pathways?
- dorsal is for recognition of the relationship between objects - ventral is for recognition of objects
272
Which hemisphere is your dominant hemisphere?
that which is responsible for symbolic reasoning (including language) and is typically contralateral to your dominant hand
273
How do we test the non-dominant hemisphere of your brain?
with a test of "construction"
274
What is aphasia?
a higher-order language deficit (inability to understand, speak, read, or write)
275
What is dysarthria?
the motor inability to speak
276
Describe Broca's aphasia.
- results from a lesion to Broca's area - intact comprehension - non-fluent speech and impaired repetition
277
Describe Wernicke's aphasia.
- results from a lesion to Wernicke's area - impaired comprehension and impaired repetition - fluent speech
278
What is a conduction aphasia?
- caused by damaged to the arcuate fasciculus - speech comprehension is intact and the individual will have fluent speech - the only thing impaired is repetition
279
What is global aphasia?
- results from damage to the arcuate fasciculus, Broca's area, and Wernicke's area - presents with non-fluent speech, impaired comprehension, and impaired repetition
280
What is transcortical motor aphasia?
- results from a lesion in the area surrounding Broca's area but not Broca's area itself - speech comprehension and repetition are intact - speech is non-fluent
281
What is a transcortical sensory aphasia?
- results from a lesion to the temporal lobe around Wernicke's area but spares Wernicke's area itself - speech comprehension is impaired - speech is fluent and repetition is intact
282
What is a mixed transcortical aphasia?
- damage to the watershed areas surrounding Wernicke's and Broca's area but without damage to either area or the arcuate fasciculus - results in impaired speech comprehension and non-fluent speech - repetition is left in tact
283
What sort of lesion would cause alexia without agraphia?
one damaging the connection between the primary visual cortex and Wernicke's area
284
What sort of lesion would cause agraphia?
one damaging the connection between the motor cortex and Broca's area
285
Kluver-Bucy Syndrome
- a syndrome that arises from a lesion affecting the amygdala bilaterally - presents with disinhibited behavior such as hyper sexuality, hyperorality, and hyperplasia - associated with HSV-1 encephalitis
286
Syndrome of Neglect
- arises due to a lesion in the non dominant parietal cortex - characterized by an inability to respond to stimuli on the contralateral side of the body or in the contralateral visual field - contralateral apraxia and astereognosis are also seen as are errors in the accuracy of arm movements
287
What is the effect of a lesion to the non-dominant parietal cortex?
hemispatial neglect syndrome
288
What is the effect of a lesion to the dominant parietal cortex?
known as Gerstmann syndrome: - agraphia - acalculia - finger agnosia - left-right disorientation
289
What is the effect of a lesion to the reticular activating system?
reduced levels of arousal and wakefulness
290
Wernicke-Korsakoff Syndrome
- a syndrome that arises most commonly in alcoholics - associated with a B1 (thiamine) deficiency; in particular, it can be precipitated by giving glucose without B1 to a B1-deficient patient - presents with confusion, opthalmoplegia, ataxia, memory loss (anterograde and retrograde), confabulation, and personality changes - associated with damage to the mammillary bodies
291
A lesion to the mammillary bodies will cause what syndrome?
Wernicke-Korsakoff syndrome
292
A lesion to the basal ganglia will result in what sort of deficit?
either a hypokinetic disorder like Parkinson's disease or a hyperkinetic disorder like Huntington's disease
293
Cerebellar lesions cause deficits on which side of the body?
ipsilateral
294
Lesions to the cerebellar hemispheres result in what?
ipsilateral intention tremor, limb ataxia, loss of balance
295
Lesions to the cerebellar vermis result in what?
truncal ataxia and dysarthria
296
What is the effect of a lesion to the subthalamic nucleus?
a contralateral hemiballismus
297
What is the effect of a bilateral hippocampal lesion?
anterograde amnesia
298
What is the effect of a lesion to the paramedian pontine reticular formation?
eyes look away from the side of the lesion
299
What is the effect of a lesion involving the frontal eye fields?
eyes look toward the lesion
300
What sort of lesion would cause the eyes to drift ipsilaterally? Contralaterally?
- ipsilateral: frontal eye field lesion | - contralateral: paramedian pontine reticular formation
301
Describe the orientation of the cerebral homunculus.
the feet are medial and with the head and hands more laterally
302
Autoregulation of cerebral perfusion is regulated mostly in response to what?
changes in the PCO2
303
What equation represents cerebral perfusion pressure?
CPP = MAP - ICP
304
What is therapeutic hyperventilation and when is it needed?
- hyperventilation is induced to lower PCO2, causing vasoconstriction in the cerebral arteries, limiting cerebral blood flow and reducing ICP - used to treat acute cerebral edema and quickly lower ICP
305
Middle Cerebral Artery Stroke
- affects the motor and sensory cortices of the upper limb and face including the region of the temporal lobe including Wernicke's area and the region of the frontal lobe including Broca's area and frontal eye fields - presents with contralateral paralysis and sensory loss more so in the face and upper limb than lower - aphasia if in dominant hemisphere, hemineglect if in non-dominant - ipsilateral deviation of eyes and superior quadrantanopsia - if only involves the superior division: there is greater damage to Broca's than Wernicke's - if only involves the inferior division: there is greater damage to Wernicke's
306
What is supplied by the superior and inferior divisions of the MCA?
- superior supplies the lateral aspect of the frontal and parietal lobes above the lateral sulcus, including frontal eye fields and Broca's area - inferior supplies the lateral aspect of the parietal and temporal lobes below the lateral sulcus, including Wernicke's area and Meyer's loop radiating from the LGN
307
Anterior Cerebral Artery Stroke
- affects the medial portions of the motor and sensory cortices as well as some frontal lobe areas involved in executive functioning - presents with contralateral paralysis and sensory loss more so in the leg than in the arm or face - may also present with frontal lobe syndrome and urinary incontinence
308
Lenticulostriate Artery Stroke
- affects the striatum and internal capsule - results in contralateral paralysis and/or sensory loss equally throughout the body (arms=legs=face) - absence of cortical signs - often in the setting of unmanaged hypertension as a lacunar infarct - can occur if a MCA is extremely proximal
309
ASA Stroke
- supplies the medial medulla - damages the lateral corticospinal tract, medial lemniscus, and hypoglossal nerve - defining feature is ipsilateral deviation of the tongue
310
PICA Stroke
- serves the lateral medulla - including the nucleus ambiguous (CN IX, X, XI), trigeminal, spinothalamic tract, vestibular, sympathetics, inferior cerebellar peduncle - defining feature is dysphagia, hoarseness, diminished gag reflex, and contralateral uvula deviation - other symptoms include vomiting and vertigo, loss of pain and temp on the contralateral body and ipsilateral face, ipsilateral horner syndrome, and ipsilateral ataxia
311
AICA Stroke
- damages the lateral pons (CN V, VI, VII, VIII) - including the facial nucleus, vestibular nuclei, spinothalamic tract, spinal trigeminal nucleus, sympathetics, and middle and inferior cerebellar peduncles - facial nucleus damage is unique and causes ipsilateral bell's palsy - other symptoms include vomiting and vertigo, loss of pain and temp on the contralateral body and ipsilateral face, ipsilateral horner syndrome, and ipsilateral ataxia
312
Basilar Artery Stroke
- damages the pons, medullar, lower midbrain - including the corticospinal and corticobulbar tracts, ocular cranial nerve nuclei, and paramedian pontine reticular formation - preserved consciousness, vertical eye movement, and blinking but nothing else
313
Posterior Cerebral Artery Stroke
- damages the occipital cortex and visual cortex | - leading to a contralateral hemianopia with macular sparing
314
The ophthalmic artery is a branch off what larger artery?
the internal carotid
315
Anterior Communicating Artery Berry Aneurysm
- may rupture and cause an anterior cerebral artery stroke | - may compress the optic chasm and cause a bitemporal hemianopia with visual acuity deficits
316
Posterior Communicating Artery Berry Aneurysm
compression may cause ipsilateral CN III palsy with mydriasis, ptosis, and "down and out" eye
317
Parasympathetic innervation to the eye travels through what cranial nerve?
CN III
318
Central Post-Stroke Pain Syndrome
- neuropathic pain due to a thalamic lesion - presents with an initial period of paresthesia followed in weeks to months by allodynia and dysesthesia - seen in 10% of stroke patients
319
Define allodynia and hyperalgesia.
- allodynia: an ordinarily painless stimuli causes pain | - hyperalgesia: a painful stimuli elicits more pain than it should
320
What imaging should be used to exclude hemorrhagic stroke before giving tPA to a stroke victim?
noncontrast CT
321
What are two imaging options for monitoring ischemic change in the brain? How long before these modalities can detect ischemia?
- CT detects ischemic changes in 6-24 hours | - diffusion weighted MRI can detect ischemia in 3-30 minutes
322
How should ischemic strokes be treated?
- tPA if within 3-4.5 hours of onset and there is no risk of hemorrhage - reduce risk with medical therapy, including control of BP, blood sugars, and lipids - treat conditions that increase risk such as atrial fibrillation
323
The dural venous sinuses drain into what larger vein?
the internal jugular vein
324
What is the main dural venous sinus into which CSF is returned?
the superior sagittal sinus
325
Venous Sinus Thrombosis
- signs and symptoms of increased ICP such as headache, seizures, and focal neurologic deficits - may lead to hemorrhage - associated with hypercoagulable states
326
Pseudotumor Cerebri
- also known as idiopathic intracranial hypertension, it is an increase in ICP with no apparent cause on imaging - presents with headache and diplopia (usually from CN VI palsy) without mental status change - fundoscopy reveals papilledema and lumbar puncture demonstrates an increase in opening pressure and provides headache relief - risk factors are women of childbearing age, excess vitamin A, danazol, ad tetracycline - treat with weight loss, acetazolamide, topiramate, and invasive procedures for refractory cases
327
Communicating Hydrocephalus
- an increase in CSF volume with ventricular dilation due to a decrease in reabsorption by arachnoid granulations - papilledema is seen on fundoscopy with increased ICP - danger is herniation
328
What is noncommunicating hydrocephalus?
an increase in CSF volume with ventricular dilation caused by a structural blockage of CSF
329
What is ex vacuo ventriculomegaly?
the appearance of increased CSF on imaging that is actually due to decreased brain tissue rather than an abnormal or pathologic increase in CSF
330
How many spinal nerves are there in each division of the spine?
there are 31 in total - 8 cervical - 12 thoracic - 5 lumbar - 5 sacral
331
Where do spinal nerves C1-C8 exit from?
C1 exits above the atlas (C1) while the rest exit below their corresponding vertebra
332
Spinal nerves tend to exit where in relationship to their vertebra?
below their vertebrae (except C1, which exits above)
333
What is a vertebral disc herniation?
herniation of the nucleus pulpous through the annulus fibrosus that normally surrounds it
334
Where are vertebral disc herniations most common?
posterolaterally at L4-L5 or L5-S1
335
Describe the medial-lateral organization within the dorsal column, lateral spinothalamic tract, and lateral corticospinal tract.
- in the dorsal column, more sacral input is organized more medially - in the lateral spinothalamic tract, more sacral input is organized more laterally - in the lateral corticospinal tract, more sacral output is carried more laterally
336
What sort of information is carried by the lateral corticospinal tract, dorsal column, and spinothalamic tract?
- corticospinal: descending motor information - dorsal column: ascending pressure, vibration, touch, and proprioception - spinothalamic tract: ascending pain and temperature
337
The dorsal column is separated into what two anatomic divisions?
- the fasciculus gracilis medially (lower body) | - the fasciculus cuneatus laterally (upper body)
338
Describe the dorsal column tract including where the neurons in the pathway reside, the medial-lateral organization within the tract, the information carried, and where it decussates.
- carries pressure, vibration, fine touch, and proprioception - first order neuron is located in the dorsal root ganglion and enters the spinal cord via the dorsal root - it ascends in the ipsilateral dorsal column with more sacral information organized more medially in the gracilis and more rostral information laterally in the cuneatus - second order neuron is located in the ipsilateral nucleus (gracilis or cuneate) of the medulla - decussate in the medulla as the internal arcuate fibers of the medial lemniscus - ascend in the contralateral medial lemniscus to the VPL of the thalamus where the third-order resides - third order neurons project from the VPL to the sensory cortex
339
Describe the spinothalamic tract including where the neurons in the pathway reside, the medial-lateral organization within the tract, the information carried, and where it decussates.
- carries pain and temperature - first order neuron is located in the dorsal root ganglion and enters the spinal cord via the dorsal root - it synapses on a second order neuron in the posterior horn at the level it entered - decussates via the anterior white commissure and ascends in the contralateral spinothalamic tract with sacral input more laterally - third order neuron is located in the VPL of the thalamus and projects to the sensory cortex
340
Describe the corticospinal tract including where the neurons in the pathway reside, the medial-lateral organization within the tract, the information carried, and where it decussates.
- carries descending motor commands - first order neuron (aka UMN) is located in the primary motor cortex and descends ipsilaterally through the internal capsule - then they decussate at the caudal medulla as the pyramidal decussation - from there they descend contralaterally to lower motor neurons in the anterior horn - the anterior horn projects via the ventral root to the NMJ
341
What is the significance of the gene of the internal capsule?
it contains the corticobulbar tract fibers and can be affected by lenticulostriate strokes
342
Clasp knife spasticity?
a sign of upper motor neuron injury in which there is a sudden, rapid decrease in resistance after enough force is applied at a joint
343
Atrophy is a sign of what sort of motor neuron damage?
lower motor neuron
344
Brown-Sequard Syndrome
- results from a hemisection of the spinal cord - presents with ipsilateral UMN signs below the lesion; ipsilateral loss of tactile, vibration, proprioception below the lesion; contralateral loss of pain and temp below the lesion; ipsilateral loss of all sensation at the level of the lesion; ipsilateral LMN signs at the level of the lesion - potential for ipsilateral Horner syndrome if the lesion occurs above T1
345
What sort of spinal lesion would result in ipsilateral Horner syndrome?
damage to the descending hypothalamospinal tract above T1
346
How can poliomyelitis be differentiated from Werdnig-Hoffmann disease?
- they both affect the anterior horns and LMNs | - but poliomyelitis presents with asymmetric weakness and Werdnig-Hoffmann disease presents with symmetric weakness
347
What is Lissauer's tract?
the tract at the tip of the posterior horn, which contains projections from first order neurons of the spinothalamic tract
348
Upper Thoracic Anterior Spinal Artery Occlusion
- damages everything except the dorsal columns and Lissauer's tract - common because the upper thoracic ASA territory is a watershed area since the artery of Adamkiewicz supplies the ASA below T8
349
Tabes Dorsalis
- degeneration of the dorsal columns and roots due to tertiary syphilis - results in progressive sensory ataxia, charcot joints, shooting pain, and Argyll Robertson pupils - exam with demonstrate the absence of DTRs and a positive Romberg sign
350
What is the Romberg test?
- it is based on the idea that you need at least two of the following to maintain balance: vision, proprioception, or vestibular function - asks patient to close their eyes and walk - a positive sign is when they lose their balance and it indicates a sensory ataxia (problem with proprioception)
351
What are Argyll Robertson pupils?
- small pupils that constrict in response to a nearby object but not in response to light - indicative of neurosyphilis
352
What sort of spinal cord lesion and presentation are found in those with a B12 deficiency?
- called subacute combined degeneration - causes demyelination of spinocerebellar tracts, lateral corticospinal tracts, and dorsal columns - presents with ataxic gait, paresthesia, and impaired position/vibration sense
353
The bicep DTR is a test of what nerve root?
C5
354
The tricep DTR is a test of what nerve root?
C7
355
The patella DTR is a test of what nerve root?
L4
356
The achilles DTR is a test of what nerve root?
S1
357
What is the cremasteric reflex and what level does it test?
- a reflexive contraction of the cremasteric muscle (elevation of testicles) in response to brushing the inner thigh - tests L1-L2
358
What are two causes of an absent cremasteric reflex?
- testicular torsion disrupting the innervation | - L1-L2 injury
359
What is the anal wink reflex and what level does it test?
- contraction of the external sphincter in response to touching the skin around the anus - tests S3, S4
360
What landmark denotes the C6 dermatome?
includes the thumb
361
What landmark denotes the T4 dermatome?
the nipple
362
What landmark denotes the T7 dermatome?
xiphoid process
363
What landmark denotes the T10 dermatome?
umbilicus
364
What landmark denotes the L1 dermatome?
the inguinal ligament
365
What landmark denotes the L4 dermatome?
includes the kneecaps
366
What is included in the S2-S4 dermatome?
genitals and anal zones
367
Where does the diaphragm refer pain?
the right shoulder via the phrenic nerve
368
Where does the gallbladder refer pain?
the right shoulder via the phrenic nerve
369
When should primitive reflexes disappear?
within the first year of life
370
What causes primitive reflexes to disappear in infancy and what would cause them to return in adulthood?
- disappear due to inhibition by a mature frontal lobe | - reappear following frontal lobe damage
371
Describe the moro reflex.
abduction of the arms when startled and then drawing together after feeling like they're falling
372
Describe the rooting reflex.
move head toward one side if cheek or mouth is stroked
373
Describe the sucking reflex.
sucking in response to contact with the roof of the mouth
374
Describe the palmar reflex.
curling of fingers if the palm is stroked
375
Describe the plantar reflex.
- aka the Babinski sign | - dorsiflexion of large toe and fanning of others with plantar stimulation
376
Describe the galant reflex.
stroking along one side of the spine while a newborn is face down causes lateral flexion of the lower body toward the ipsilateral side
377
Which cranial nerves exit above the pons, from the pons, and from the medulla?
- above pons: I, II, III, IV - pons: V, VI, VII, VIII - medulla: IX, X, XI, XII
378
Transtentorial (central) Herniation
- herniation caused by a supratentorial mass - results in caudal displacement of the brain stem - potential for rupture of the paramedian artery leading to a brainstem hemorrhage (aka Duret hemorrhage) - usually fatal
379
Hemangioblastoma
- an adult primary brain tumor most often found in the cerebellum - may be capable of producing EPO and causing a secondary polycythemia - histology reveals closely arranged, thin-walled capillaries with minimal intervening parenchyma - associated with VHL when found with retinal angiomas
380
Pinealoma
- a tumor of the pineal gland - can cause parinaud syndrome (vertical gaze palsy) if it compresses the tectum, obstructive hydrocephalus, or precocious puberty in males (via B-hCG production) - histology is similar to germ cell tumors
381
In contrast to central pontine myelinolysis syndrome, what happens when hypernatremia is corrected too quickly?
there is cerebral edema and possible herniation
382
Acute Inflammatory Demyelinating Polyradiculopathy
- the most common subtype of Guillain-Barre syndrome - associated with an earlier infection and mediated by autoimmune attack of peripheral myelin due to molecular mimicry (specifically, destruction of Schwann cells) - results in symmetric, ascending muscle weakness or paralysis beginning in the lower extremities; may also see autonomic dysregulation or sensory abnormalities - exam findings may include papilledema and increased CSF protein with normal cell count - complete recovery is expected after weeks to months but respiratory support is critical until that time; additional therapy includes plasmapheresis and IVIG but there is no role for steroids
383
Acute Disseminated (Post-Infectious) Encephalomyelitis
- a multifocal periventricular inflammation and demyelination after infection or vaccination - presents with rapidly progressive multifocal neurologic symptoms and altered mental status
384
Charcot-Marie-Tooth Disease
- also known as hereditary motor and sensory neuropathy - a group of, primarily autosomal dominant, progressive hereditary nerve disorders - caused by defective production of proteins critical for the structure and function of peripheral nerves or myelin sheath - associated with foot deformities (pes caves), lower extremity weakness, and sensory deficits
385
What are seizures?
synchronized, high-frequency neuronal firing
386
Partial Seizures
- seizures affecting a single area of the brain - most commonly originating in the medial temporal lobe and often preceded by an aura - include simple partial (consciousness remains intact) and complex partial (impaired consciousness) - can secondarily generalize
387
What is the difference between a simple and complex partial seizure?
simple refers to a partial seizure in which consciousness remains intact while complex refers to a case in which consciousness is impaired
388
What is epilepsy?
a disorder of recurrent seizures, excluding febrile seizures
389
What is status epilepticus?
a continuous or recurring seizure that may result in brain injury, typically defined as those lasting longer than 5 minutes
390
What are the most common causes of seizures in children, adults, and geriatrics?
- children: genetic, infection, trauma, congenital, and metabolic - adults: tumor, trauma, stroke, infection - geriatrics: stroke, tumor, trauma, metabolic, infection
391
What are myoclonic seizures and what are tonic-clonic seizures?
- myoclonic: generalized seizure characterized by quick, repetitive jerks - tonic-clonic: generalized seizure characterized by alternating stiffening and movement
392
What is a tonic seizure?
a generalized seizure characterized by stiffening
393
What is an atonic seizure?
a generalized seizure in which the individual drops to the floor, commonly mistaken for fainting
394
What is vertigo?
the sensation of spinning while actually stationary
395
Peripheral Vertigo
- the more common form of vertigo - caused by an inner ear etiology such as semicircular canal debris, vestibular nerve infection, etc. - positional testing will reveal delayed horizontal nystagmus
396
Central Vertigo
- that arising from a brain stem or cerebellar lesion - findings include directional change of nystagmus, skew deviation, diplopia, and dysmetria - positional testing reveals immediate nystagmus in any direction and may change directions
397
Von Hippel-Lindau Disease
- a syndrome of tumors due to a deletion of VHL on chromosome 3, which normally ubiquitinates hypoxia-inducible factor 1a - syndrome includes HARP: Hemangioblastomas of the retina, brain stem, cerebellum, and spine; Angiomatosis; bilateral Renal cell carcinomas; and Pheochromocytomas
398
Neurofibromatosis Type 1
- a neurocutaneous disorder arising from a mutation of the NF1 tumor suppressor gene on chromosome 17, which encodes a negative regulator of RAS called neurofibromin - presents with cafe-au-lait spots, Lisch nodules (pigmented iris hamartomas), cutaneous neurofibromas, optic gliomas, and pheochromocytomas - the neurofibromas are derived from neural crest cells
399
Tuberous Sclerosis
- a neurocutaneous disorder due to a TSC1 mutation on chromosome 9 or TSC2 mutation on chromosome 16 - features follow the HAMARTOMASS acronym: Hamartomas in CNS/skin, Angiofibromas, Mitral regurgitation, Ash-leaf spots, cardiac Rhabdomyoma, (Tuberous sclerosis), autosomal dOminant, Mental retardation, renal Angiomyolipoma, Seizures, and Shagreen patches - also have an increased incidence of subependymal astrocytomas and ungal fibromas
400
Sturge-Weber Syndrome
- a somatic mosaicism for an activating mutation of GNAQ causes congenital, non-inherited neural crest anomalies - affects small blood vessels to cause a port-wine stain of the face in a CN V1/V2 distribution - also presents with ipsilateral leptomeningeal angioma causing seizures and epilepsy, intellectual disability, and episcleral hemangioma causing increased IOP and early onset glaucoma - STURGE: Sporadic, port-wine Stain, Tram track calcifications (opposing gyri), Unilateral, Retardation, Glaucoma, GNAQ gene, Epilepsy
401
Cluster Headaches
- repetitive brief headaches with excruciating periorbital pain with lacrimation and rhinorrhea - unilateral and may also present with Horner syndrome - last 15min-3hr and occur in clusters - treat with sumatriptan acutely and verapamil for prophylaxis
402
Tension Headaches
- those with constant, steady pain, no photophobia, no photophobia, and no aura - tend to bilateral and lasting more than thirty minutes - treat with analgesics, NSAIDs, and acetaminophen; use amitriptyline for chronic pain
403
Migraine Headaches
- those with pulsating pain and nausea, photophobia, or phonophobia; many are preceded by an aura - arise from irritation of CN V, meninges, or blood vessels - mediated by substance P, CGRP, and vasoactive peptides - unilateral and lasting between 4-72 hours - treat acutely with NSAIDs, triptans, and dihydroergotamine - use lifestyle changes, beta-blockers, CCBs, amitriptyline, topiramate, and valproate for prophylaxis
404
What are some pathologic causes of headache?
- meningitis - hydrocephalus - subarachnoid hemorrhage - neoplasia - arteritis