Gen Path Exam 2 - CNS Disease Flashcards by A G

What category of disease?

Hydrocephalus

Primary disease

How well did you know this?

Not at all

Perfectly

What category of disease?

Epidermal hematoma

Vascular/traumatic

How well did you know this?

Not at all

Perfectly

What category of disease?

Intracerebral hemorrhage

Vascular/traumatic

How well did you know this?

Not at all

Perfectly

What category of disease?

Subarachnoid hemorrhage

Vascular/traumatic

How well did you know this?

Not at all

Perfectly

What category of disease?

Subdural hematoma

Vascular/traumatic

How well did you know this?

Not at all

Perfectly

What category of disease?

CVA/stroke

Vascular/traumatic

How well did you know this?

Not at all

Perfectly

What category of disease?

Acquired

How well did you know this?

Not at all

Perfectly

What category of disease?

Alzheimer’s

Acquired

How well did you know this?

Not at all

Perfectly

What category of disease?

Parkinson’s

Acquired

How well did you know this?

Not at all

Perfectly

What category of disease?

Huntington’s

Acquired

How well did you know this?

Not at all

Perfectly

What category of disease?

ALS

Acquired

How well did you know this?

Not at all

Perfectly

What category of disease?

Brain abscess

Infectious

How well did you know this?

Not at all

Perfectly

What category of disease?

Viral meningitis

Infectious

How well did you know this?

Not at all

Perfectly

What category of disease?

Bacterial meningitis

Infectious

How well did you know this?

Not at all

Perfectly

What category of disease?

Pilocytic astrocytoma

Neoplastic

How well did you know this?

Not at all

Perfectly

What category of disease?

Glioblastoma multiforme

Neoplastic

How well did you know this?

Not at all

Perfectly

What category of disease?

Meningioma

Neoplastic

How well did you know this?

Not at all

Perfectly

What category of disease?

Medulloblastoma

Neoplastic

How well did you know this?

Not at all

Perfectly

What category of disease?

Metastatic tumors

Neoplastic

How well did you know this?

Not at all

Perfectly

Which disease?

Symptomatic increase in the volume of CSF within the cerebral ventricles of the brain

Hydrocephalus

How well did you know this?

Not at all

Perfectly

In hydrocephalus, what does the increase in volume of CSF in the cerebral ventricles result in?

Expansion of ventricles due to increased CSF pressure

How well did you know this?

Not at all

Perfectly

What are the causes of hydrocephalus?

Obstructive
Impaired absorption of CSF at arachnoid villi (rare)
Overproduction of CSF by choroid plexus neoplasms (very rare)

How well did you know this?

Not at all

Perfectly

What is the MOST COMMON form of hydrocephalus?

Obstructive

How well did you know this?

Not at all

Perfectly

Obstructive hydrocephalus can be ___________ or __________

congenital; acquired

How well did you know this?

Not at all

Perfectly

What can happen if hydrocephalus is left untreated?

Permanent brain damage Mental impairment Death

CNS trauma often directly disrupts vessel walls, leading to what?

Hemorrhage

Depending on the affected vessel, the hemorrhage and hematoma formation may be one or a combination of what areas?

Epidural Subdural

In epidural hematomas, what vessels are vulnerable to traumatic injury?

Dural vessels (especially middle meningeal artery)

In adults with epidural hematoma, what do tears involving dural vessels almost always stem from?

Skull fractures

T/F: In infants with epidural hematoma, displacement of the easily deformable skull may tear a vessel, even in the ABSENCE of a skull fracture

True! (epidural hematomas in adults usually always stem from a skull fracture tho)

Once a vessel is torn, blood accumulating under arterial pressure can dissect the tightly applied dura away from the inner skull surface producing, a ___________ that compresses the brain surface

hematoma

How does a patient with an epidural hematoma appear clinically?

Lucid for hours btwn moment of trauma and development of neurologic signs

An epidural hematoma may expand rapidly and constitutes a neurosurgical ____________ necessitating prompt __________ and repair to prevent _______

emergency; drainage; death

What disease? Caused by tearing of the middle meningeal artery

Epidural hematoma

What disease? Rapid movement of the brain during trauma can tear the bridging veins that extend from the cerebral hemispheres through the subarachnoid and subdural space to the dural sinuses

Subdural hematoma

In subdural hematoma, the disruption of bridging veins that extend from cerebral hemispheres causes what to occur?

Bleeding into subdural space

Which types of people have a higher rate/are more susceptible of subdural hematomas?

Elderly with brain atrophy Infants

Why are elderly people with brain atrophy more susceptible to subdural hematomas?

Bridging veins are stretched out - brain has additional space to move

Why are infants more susceptible to subdural hematomas?

Their bridging veins are thin-walled

When do subdural hematomas become clinically evident?

Within 48 hrs after injury

Where are subdural hematomas most commonly found?

Over lateral aspects of cerebral hemispheres (may be bilateral)

In subdural hematoma, neurologic signs are attributable to what?

Pressure exerted on adjacent brain

What are the symptoms of a subdural hematoma?

Headache Confusion Slowly progressive neurologic deterioration

T/F: Symptoms of subdural hematoma are most often localized

FALSE, they are more often non-localized

How are symptomatic subdural hematomas treated?

Surgical removal of blood and associated reactive tissue

What commonly occurs during the healing process of a subdural hematoma? What does this result in?

Rebleeding Results in chronic subdural hematoma

An acute subdural hematoma appears as a collection of ________ _________ blood apposed to the contour of the brain surface, ___________ extension into the depths of ________.

freshly clotted; without; sulci

In subdural hematoma, what does the underlying brain look like? What does the subarachnoid space look like?

Underlying brain = flat Subarachnoid space = clear

In subdural hematoma, venous bleeding is ____________ with breakdown and organization of the hematoma taking place over time

self-limited

Describe the process of breakdown and organization of subdural hematoma over time

1. Hematoma organizes by lysis of clot in 1 week 2. Granulation tissue grows from dural surface into hematoma in 2 weeks 3. Fibrosis occurs in 1-3 months 4. Fibrosing lesions retract, leaving a thin layer of CT ("subdural membranes")

What disease? Characterized by injury to the brain as a consequence of altered blood flow

Cerebrovascular disease (CVD)

What disease? Clinical designation that applies to all the conditions of cerebrovascular disease, particularly when symptoms begin acutely

Stroke

What are the 2 mechanisms of the pathophysiology of cerebrovascular disease?

Hypoxia/ischemia/infarction Hemorrhage

Which pathophysiologic mechanism of CVD? Results from impairment of blood supply and oxygenation of CNS tissue

Hypoxia/ischemia/infarction

Which pathophysiologic mechanism of CVD? Results from rupture of CNS vessels

Hemorrhage

What are the common causes of hemorrhage in CVD?

Hypertension Vascular anomalies like malformation and aneurysm

What is the common cause of hypoxia/ischemia/infarction in CVD?

Embolism

T/F: In the brain, embolism is a more common etiology of hypxia/ischemia/infarction than thrombosis

True

What are the 2 most common causes of cerebral infarctions?

Atherosclerotic thrombi Emboli of cardiac origin

What is the cause of atherosclerotic thrombi, leading to cerebral infarction?

Carotid artery atherosclerotic disease (plaque rupture) at carotid bifurcation

What are the causes of emboli of cardiac origin, leading to cerebral infarction?

Valvular pathology (mitral) MI/congestive cardiomyopathy Cardiac arrhythmia (afib)

What is the extent of cerebral infarction determined by?

Site of occlusion Size of occluded vessel Duration of occlusion Collateral circulation

What do neurologic abnormalities from cerebral infarction depend on?

Artery involved + area of supply

Gross appearance of an ischemic cerebral infarct varies with ________

time

What is the gross appearance of an ischemic cerebral infarct during the first 6 hrs?

Little change

What is the gross appearance of an ischemic cerebral infarct during the first 48 hrs?

Tissue = pale, soft, swollen Corticomedullary junction = indistinct

What is the gross appearance of an ischemic cerebral infarct during days 2-10?

Brain = gelatinous, friable Boundary btwn normal and infarcted tissue is more distinct Edema resolves in viable adjacent tissue

What is the gross appearance of an ischemic cerebral infarct during days 10-3 weeks?

Liquefactive necrosis Fluid-filled cavity that expands until all dead tissue is gone

What risk factor is most commonly associated with clinically significant deep brain intracerebral hemorrhages?

Hypertension

Hypertension can cause vessel wall ___________, making them weaker and more vulnerable to rupture. Name 3.

abnormalities 1. Accelerated atherosclerosis (large arteries) 2. Hyaline arteriosclerosis (small arteries) 3. Frank necrosis of arterioles

Where does hypertensive intracerebral hemorrhage originate in? (4)

Putamen (MOST COMMON) Thalamus Pons Cerebellar hemispheres

In intracerebral hemorrhage, rupture of an artery within brain tissue leads to ____________ of blood, which displaces ________ tissue and causes increased intracranial volume until the resulting tissue compression halts the bleeding

extravasation; brain

What is the MOST COMMON cause of clinically significant subarachnoid hemorrhage?

Rupture of saccular aneurysm in cerebral artery

What is the MOST COMMON type of intracranial aneurysm?

Saccular aneurysm

Which vessels are most often affected by saccular aneurysms?

Circle of Willis vessels

What disease? May result from: Extension of a traumatic hematoma, Rupture of a hypertensive intracerebral hemorrhage into the ventricular system Vascular malformation Hematologic disturbances CNS tumors

Subarachnoid hemorrhage

Which meningitis is the most serious form?

Bacterial meningitis

What disease? This is infection of the leptomeninges (pia and arachnoid mater), and the CSF, which diffusely affects the whole meninges and subarachnoid space

Bacterial meningitis

What are the most common organisms that cause bacterial meningitis?

Neisseria meningitidis Strep pneumoniae

How quickly do symptoms of bacterial meningitis develop?

24 hrs to 7 days

What are the following classic symptoms associated with? Headache Stiff neck Fever/chills Vomiting Photophobia Confusion Seizures Recent URI

Bacterial meningitis

Meningitis caused by ___________ may progress to a meningococcemia with multiple organ involvement, and may be associated with maculopapular, petechial, or purpuric _______ ______

N. meningitidis; skin rash

Meningococcemia is sometimes complicated by which syndrome?

Waterhouse-Friderichsen syndrome

What disease? Complications include ventriculitis, intracerebral abscess, cerebral infarction, subdural empyema (pus in subdural space)

Meningitis

How is bacterial meningitis diagnosed?

Cloudy CSF due to increased neutrophils Increase in CSF proteins Decrease in glucose

What is the tx for bacterial meningitis?

Vigorous IV antibiotics

How is bacterial meningitis caused by N. meningitidis prevented?

Vaccine

What disease? Mortality ranges from 3% for H. influenzae to 60% for Strep pneumoniae; highest in very young and elderly

Bacterial meningitis

What disease? Serious complications occasionally are observed including: Nerve deafness Cortical blindness Paralysis Muscular hypertonia Ataxia Complex seizure disorders Learning disabilities Obstructive hydrocephalus Cerebral atrophy

Bacterial meningitis

What is the most common cause of meningitis?

Viral meningitis

What type of meningitis? Benign and self-limiting, usually less severe

Viral meningitis

Viral meningitis may occur as a complication of what?

A viral infection (ex: mumps, measles)

What are the common causative organisms of viral meningitis?

Enteroviruses Mumps virus

What disease? Clinically presents with acute onset of headache, fever, nuchal rigidity; irritability, and rapid development of meningeal irritation

Viral meningitis

How is viral meningitis diagnosed?

CSF = clear/colorless Increased lymphocytes and protein Normal glucose

How is viral meningitis treated?

Control symptoms (palliative)

What disease? Severe focal infection of the brain and is typically 1 to 2 cm across

Brain abscess

What disease? Starts as an area of cerebritis and develops into a pus-filled cavity walled off by gliosis and surrounded by cerebral edema

Brain abscess

What does a brain abscess often result in?

Increased intracranial pressure

What are the 6 causes of brain abscesses?

Middle ear infection (60%) Frontal sinusitis infection (20%) Bacteremia/septicemia (10%) Penetrating skull trauma Meningitis Odontogenic infection

Which cause of brain abscess? Due to temporal lobe and cerebellar abscesses

Middle ear infection

Which cause of brain abscess? Due to frontal lobe abscess

Frontal sinusitis infection Bacteremia/septicemia

What are the common causative organisms of brain abscesses?

Strep viridans Staph aureus Klebsiella Fungus

What disease? Clinical presentation is similar to that of acute bacterial meningitis but focal neurological signs, epilepsy, and fever are common manifestations

Brain abscess

How are brain abscesses treated?

Aggressive antibiotics Surgical aspiration or excision

What is the overall mortality of a brain abscess?

10%

What are the 4 complications of brain abscess?

Meningitis Intracranial herniation Focal neurological deficit Epilepsy

What disease? Characterized by destruction of myelin with relative preservation of axons

Demyelinating diseases

What disease is by far the MOST COMMON of the demyelinating diseases?

When does MS begin? Is it more common in males or females?

20-30 yrs old; females

What disease? Characterized by relapsing and remitting episodes of immunologically mediated demyelination within the CNS

What disease? Recovery from each episode of demyelination is usually incomplete, leading to progressive deterioration

What is MS caused by?

Unknown Could be immune/viral/environmental/genetic

What disease? Characterized by irregularly scattered, focal areas of demyelination in the brain and spinal cord

What are the 3 favored sites for demyelination in MS?

Optic nerve Brain stem Periventricular areas

MS is typically characterized by ___________ with long asymptomatic __________ and often a progressive course, leading to invalidism w/ mental deterioration

exacerbations; remissions

What are these 5 early manifestations associated with? Weakness of legs Visual disturbances Retrobulbar pain Sensory disturbances Loss of bladder control

What is the classic manifestation of MS that is significant for diagnosis?

Charcot triad

Nystagmus, intention tremor, and dysarthria

Charcot triad (MS)

Heat-induced exacerbation of MS symptoms (especially optic neuritis). This lead the development of the 'hot bath test' in the 1950's.

Uhthoff's phenomenon (MS)

Remained the principal diagnostic tool used to determine if a patient had MS for 30+ years, with the practice ending in the 1980's when more accurate and safer testing methods became available

Uhthoff's phenomenon (MS)

During an acute MS exacerbation, a patient's face may develop ___________, and muscles of ___________ ___________ (especially the periorbital) can undulate in a wave-like manner

paresthesia; facial expression

______ manifestations of MS are reported to occur in 2–3% of those affected and may serve as the _____ presenting symptoms of MS

Oral; first

Unusual muscle movement that resembles movement of a “bag of worms”

Myokymia (MS)

If this symptom is observed, referral to a physician is advised if MS has not been diagnosed

Myokymia

What is 400x more likely among individuals with MS?

Trigeminal neurolgia

What are the 4 degenerative diseases?

Alzheimer's Parkinson's Huntington's ALS

Which disease is the prototype of cortical degenerative diseases?

Alzheimer's

Which disease accounts for the majority (50 – 75%) of all cases of dementia?

Alzheimer's

Currently, ~ 5.7 million Americans have this disease

Alzheimer's

The risk of which disease doubles every 5 yrs after the age of 65 up until age 90?

Alzheimer's

Which disease has a female predilicition (~2:1)?

Alzheimer's

The prevalence of Alzheimer's is expected to substantially increase in this century because it preferentially affects the _________, who constitute the fastest growing age group in the U.S

elderly

The number of persons affected by Alzheimer's in the U.S. is projected to nearly ________ by the year 2050

triple

T/F: Most cases of Alzheimer's are from familial cause (aka early-onset)

FALSE; most cases are sporadic (aka late-onset)

Alzheimer's disease affects the 3 processes that keep neurons healthy. Name them.

Communication Metabolism Repair

Which disease? Certain nerve cells in the brain stop working, lose connections with other nerve cells, and finally die

Alzheimer's

In Alzheimer's, what causes memory failure, personality changes, and problems with daily activities?

Destruction and death of nerve cells

What are the 2 major factors for pathogenesis of Alzheimer's?

Beta-amyloid senile plaques Neurofibrillary tangles

Which disease? Other factors like neuroinflammation, oxidative stress, cholinergic insufficiency, mitochondrial dysfunction, and autophagy dysfunction also play major role in the disease progression

Alzheimer's

The fundamental abnormality in Alzheimer's is the accumulation of which 2 proteins in the brain?

Beta-amyloid Tau

What protein forms senile plaques in Alzheimer's?

Beta-amyloid

What protein forms neurofibrillary tangles in Alzheimer's?

Tau

What is the primary critical initiating event for the development of Alzheimer's and precedes the clinical (symptomatic) onset of Alzheimer's?

Accumulation of senile plaques

Besides accumulation of senile plaques, what 3 things accompany the progression of cognitive decline in Alzheimer's?

Neurofibrillary tangles Neuronal dysfunction/death Inflammatory rxns

What is formed from the transmembrane protein amyloid precursor protein (APP)?

Beta-amyloid

What readily aggregates and eventually forms into large aggregates and fibrils in the brain called senile plaques?

Beta-amyloid

What can be directly neurotoxic, meaning even small aggregates can result in neuronal synaptic dysfunction?

Beta-amyloid

What is an example of neuronal synaptic dysfunction due to beta-amyloid?

Blocking of long-term potentiation

What elicits an inflammatory response from microglia and astrocytes in the brain?

Senile plaques

What stimulates the secretion of mediators from microglia and astrocytes that cause damage to neurons?

Senile plaques

What are neurofibrillary tangles composed of?

Neuronal filaments of hyperphosphorylated tau protein

What does tau protein normally do?

Bind/stabilize microtubules Support axonal transport of organelles, glycoproteins, neurotransmitters

What does tau do once it is hyperphosphorylated (abnormal)?

Can't bind to microtubules Forms neurofibrillary tangles -> accumulate in pyramidal neurons

What are the 2 suggested mechanisms of neurofibrillary tangle injury to neurons?

1. Tau protein aggregates elicit stress response -> neuronal death 2. Tau protein can't stabilize microtubules -> neuronal toxicity & death

The brain content of the enzyme ______ and its product, ____________, is decreased in patients with AD, in the cerebral cortex, hippocampus and especially the ________ ___________

ChAT; acetylcholine; basal forebrain

What does ChAT stand for?

Choline acetyltransferase

What is located in the basal forebrain and is severely lost in Alzheimer's disease?

Cholinergic neurons

What contributes to memory and attention deficits seen in Alzheimer's pts?

Loss of cholinergic neurons

What disease? Slow, progressive intellectual deterioration during the course of several years

Alzheimer's

Who usually notes insidious memory impairment in pts with Alzheimer's?

Spouse/family member

What do pts with Alzheimer's have difficulty with?

Learning/retaining new info Handling complex tasks Impaired reasoning, judgment, spatial ability, orientation

Behavioral changes such as what may accompany memory impairment in Alzheimer's?

Mood changes Apathy

What may pts develop in later stages of Alzheimer's?

Agitation Psychosis

In Alzheimer's, the brain shows a variable degree of ________ __________ marked by widening of the cerebral sulci that is most pronounced in the frontal, temporal, and parietal lobes

cortical atrophy

With significant atrophy in Alzheimer's, there is compensatory __________ enlargement (hydrocephalus ex vacuo) secondary to loss of parenchyma and reduced ______ volume

ventricular; brain

In Alzheimer's, structures of the medial temporal lobe, including hippocampus, entorhinal cortex and amygdala, are involved early in the course and are usually severely _________ in the later stages

atrophied

What are the characteristics of cerebral atrophy in Alzheimer's?

Narrowed gyri Widened sulci

Hydrocephalus ex vacuo

Compensatory dilation of ventricles (Alzheimer's)

Composed of an extracellular core of beta-amyloid protein surrounded by microglial cells and reactive astrocytes

Senile plaques

Occur most frequently in the hippocampus, amygdala and neocortex

Senile plaques

Abnormal tangles of insoluble cytoskeletal-like hyperphosphorylated tau (paired helical) filaments that form within the cytoplasm of cortical neurons, especially the pyramidal cells of the hippocampus, the amygdala, the basal forebrain.

Neurofibrillary tangles

Intraneuronal cytoplasmic granule-containing vacuoles occurring within the pyramidal cells of the hippocampus

Granulovacuolar degeneration (Alzheimer's)

Elongated, glassy, eosinophilic bodies consisting of paracrystalline arrays of beaded filaments, with actin as their major component

Hirano bodies (Alzheimer's)

Found most commonly within hippocampal pyramidal cells

Hirano bodies (Alzheimer's)

Distorted, twisted and dilated dendritic processes and axons of cerebral cortex found around beta-amyloid plaques

Neuropil threads (Alzheimer's)

What are the biological markers that have been correlated with the degree of dementia?

Amount of decreased ChAT Beta-amyloid burden in brain

What disease? Slowly progressive, debilitating, degenerative disorder of the basal ganglia

Parkinson's

What disease? Characterized by resting tremor, bradykinesia, rigidity, postural changes, and often mental changes

Parkinson's

What is another name for Parkinson's?

Paralysis Agitans

Are males or females more commonly affected by Parkinson's?

Males

What disease? Predominately seen in persons between 50 and 65 years of age, with an average age of onset at 55 years

Parkinson's

What disease? Can be primary (idiopathic) and accounts for ~75% of cases, or secondary (acquired) and accounts for ~ 25% of cases

Parkinson's

T/F: The specific etiology of primary Parkinson's is unknown and is believed to be due to a combination of genetic and environmental factors

True

What are the 4 causes of secondary Parkinson's?

Familial Trauma Drugs/toxins Shy-Drager syndrome

Describe the inheritance/familial pattern of secondary Parkinson's

Autosomal dominant or recessive

Which drug/toxin is correlated to secondary Parkinson's?

MPTP (dopamine antagonist)

Autonomic degeneration and orthostatic hypotension with Parkinson's

Shy-Drager syndrome

Parkinson's shows degeneration of pigmented _____________ neurons of the __________ ________, the ________ __________, and several other brainstem nuclei

dopaminergic; substantia nigra; locus ceruleus

Degeneration of dopaminergic neurons and other brainstem nuclei causes Parkinson's by reducing the amount of ___________ in the __________ ___________

dopamine; corpus striatum

In Parkinson's, what are surviving cells containing eospinophilic spherical inclusions in the substantia nigra called?

Lewy bodies

Lewy bodies contain fine filaments composed of what?

a-synuclein

Protein involved in synaptic transmission

a-synuclein

When do clinical symptoms of Parkinson's become evident?

Loss of 60% of dopaminergic neurons

What disease has the following clinical presentation? Resting tremor Bradykinesia Autonomic dysfunction Sensory dysfunction Personality changes Dementia

Parkinson's

What symptom of Parkinson's? Seen in the hand as a “pill rolling” tremor (thumb and forefinger); can also involve the leg and lip

Resting tremor

What does a resting tremor seen in Parkinson's improve with?

Purposeful movement

What symptom of Parkinson's? General slowing of movements; difficulty in initiating movements and freezing of motion

Bradykinesia

What symptom of Parkinson's? Shuffling gait with short steps, lack of facial expression (mask-like facies), impaired swallowing causing drooling (sialorrhea)

Bradykinesia

What symptom of Parkinson's? Orthostatic hypotension

Autonomic dysfunction

What symptom of Parkinson's? Flushing and excessive sweating (“drenching sweats”)

Autonomic dysfunction

What symptom of Parkinson's? Constipation

Autonomic dysfunction

What symptom of Parkinson's? Paresthesias, pain, akathisia

Sensory dysfunction

What symptom of Parkinson's? Apathy, lack of confidence, fearfulness, anxiety, emotional lability/inflexibility, social withdrawal, dependency

Personality changes

What symptom of Parkinson's? Develops in 10-20% of patients

Dementia

An autosomal dominant, fatal, progressive movement disorder associated with degeneration of the striatum (caudate/putamen)

Huntington's

Huntington's especially affects which 2 neurons?

Cholinergic and GABA-ergic

Characterized by the delay of clinical abnormalities until age 30 to 40

Huntington's

Course extends 15 to 20 years, beginning with athetoid movements, followed by progressive deterioration leading to hypertonicity, fecal and urine incontinence, anorexia and weight loss, and eventually dementia and death

Huntington's

Huntington's is characterized by increased copies of what within the HD gene?

CAG trinucelotide repeats

Where is the HD gene located?

Short arm of chromosome 4

What does the HD gene encode for?

Huntingtin protein

Subject to ubiquitination and proteolysis yielding fragments that can form intranuclear aggregates

Huntingtin protein

Huntingtin protein intranuclear aggregates are suspected to be what?

Toxic to neurons

In Huntington's, ___________ transmission results in an increased number of CAG repeats and respectively earlier onset of disease manifestations in successive generations

paternal

In Huntington's, the brain is small and shows atrophy of the ________ _________ and _________

caudate nucleus; putamen

Which structure is atrophied secondarily in Huntington's?

Globus pallidus

What structures are dilated in Huntington's?

Lateral + 3rd ventricles

What is frequently seen in the frontal lobe, less often in the parietal lobe, and occasionally in the entire cortex in a pt with Huntington's?

Atrophy

Where is there severe loss of neurons and gliosis in Huntington's?

Affected parts of corpus striatum

In Huntington's, what is present in the remaining striatal neurons and in the cortex? What do they contain?

Intranuclear inclusions Contain aggregates of huntingtin protein

What is a synonym of ALS?

Lou Gehrig's

Progressive, fatal motor neuron neurodegenerative disease of unknown etiology

ALS

Characterized by the dysfunction and death of lower motor neurons in anterior horn of the spinal cord, and upper motor neurons (Betz cells) in the motor cortex

ALS

What does the loss of lower motor neurons in anterior horn of spinal cord lead to in ALS?

Muscle atrophy

T/F: Majority of cases of ALS are familial

FALSE, majority are sporadic, but ~10% are familial

What disease? If familial, caused by mutation of superoxide dismutase gene

ALS

What is the age range for clinical onset of ALS?

50-70 yrs old

Does ALS affect men or women more?

Men

What are the 3 symptom groups of ALS?

Limb Bulbar Respiratory

Which symptom group of ALS? Painless muscle weakness Clumsiness (e.g., dropping things, stumbling, falling) Impaired fine motor skills (e.g., writing) Limb stiffness Gait disturbances Wristdrop or footdrop

Limb

Which symptom group of ALS? Dysarthria or voice change Dysphagia and/or weight loss Drooling Choking during swallowing

Bulbar

Which symptom group of ALS? Dyspnea on exertion or at rest Orthopnea

Respiratory

When does ALS typically lead to death after onset of symptoms?

1-6 yrs

What factors are most important in the different mechanisms of ALS?

Genetic Exogenous (toxins, viruses, metals)

In ALS, the final mechanism of motor neuron death is likely to be via a process resembling what?

Apoptosis

Most CNS tumors are _________; tumors of the spinal cord are much less frequent

intracranial

What are the 2nd most common form of malignancy in children? (leukemia is 1st)

CNS tumors

Primary malignant CNS tumors rarely do what?

Metastasize

Metastatic tumors to the brain are found more frequently than what?

Primary intracranial neoplasms

What are the most common primary intracranial tumors in adults?

Glioblastoma Meningioma Acoustic neuroma

What are the most common primary intracranial tumors in children?

Medulloblastoma Cerebellar astrocytomas

What do the types of neurological symptoms that result from CNS tumor development depend on?

Site Extent Rate of growth

T/F: Even benign intracranial tumors can result in devastating clinical consequences due to tumor compression of the brain parenchyma

True

What disease? Characterized by a history of: -New-onset partial or generalized seizures -Focal neurologic deficit (visual disturbances, cranial nerve palsy, motor impairment, hemiplegia, ataxia) -Altered mental status -Cognitive impairment

CNS tumor

Tumors that arise from glial supportive tissue of brain

Gliomas

They are the most common primary brain tumors, accounting for 50% - 60% of all newly diagnosed CNS tumors

Gliomas

What type of tumor are astrocytomas?

Glioma

What are astrocytomas derived from?

Astrocytes

Where are astrocytomas found?

Cerebral hemispheres

What CNS tumor? Most frequent in the 4th-6th decades of life

Astrocytoma

What CNS tumor? Account for 80% of gliomas in adults

Astrocytoma

What CNS tumor? Regional effects are compression, invasion, destruction of parenchyma

Astrocytoma

What are the 4 groups of astrocytomas based on?

Histology

What are the 2 groups of astrocytomas we need to know for exam?

Grade I = Pilocytic (juvenile) Grade IV = Glioblastoma multiforme

Most common pediatric brain tumor