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281 Cards in this Set

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In Mycotic Pneumonias, the fungi are ______ antigens and cause _________
Weak; tissue damage
Why do mycotic pneumonias cause tissue damage?
hypersensitivity reaction by the host against the fungal proteins
Histoplasmosis, coccidioidomycosis, North American blastomycosis, and cryptococcosis are example of what kind of mycoses?
deep mycoses
Deep mycoses induce what kind of reaction?
chronic granulomatous inflammatory reaction
what are the major opportunistic fungal organisms?
Candida, mucor, and aspergillus
What kind of inflammatory response is induced by Candida, mucor, and aspergillus fungi?
Acute inflammatory response in the debilitated patient.
opportunistic fungi invade and obstruct blood vessels which can cause _____________
hemorrhagic infarcts
What is the most common opportunistic agent that produces pneumonia in AIDs patients?
Pneumocystis carinii
Where do opportunistic agents, which cause mycotic pneumonia, invade the body and what kind of edema is created?
they proliferate in the alveolar spaces and cause "frothy-appearing" intra alveolar edema
True or False: Worldwide, tuberculosis kills more adults than any other infectious agent?
True
Why has the incidence of Pulmonary Tuberculosis been rising since 1985, despite effective anti-tuberculosis drugs?
increased numbers of immune-suppressed patients (AIDS, etc.) and emergence of drug-resistant strains of the myobacterium
name the 2 types of Pulmonary Tuberculosis
Primary Tuberculosis and Secondary Tuberculosis
For Primary Tuberculosis, what is the most common route of infection?
Inhalation of contaminated aerosol droplets from an infected individual
Where is the primary focus of infection (where in the body is the infection) for persons with Primary Tuberculous?
lung parenchyma, usually subpleural, most commonly in the upper portion of the lower lobe or the lower portion of the upper lobe
What is the primary focus called and what kind of necrosis takes place?
it is called Ghon focus and has central caseous necrosis
in primary tuberculosis, how do organisms spread through the body and what do they cause?
Organisms may spread through the lymphatics to produce caseous lymphadenditis in the hilar lymph nodes
what is the combo of subpleural lesion and hilar node involvement in primary tuberculosis called?
Primary Ghon Complex
what is the only residuum of the primary tuberculosis infection in about 90% of individuals?
a fibrotic or calcified Ghon complex
What can the progression of Primary Tuberculosis be in pre-school children?
sometimes it is not benign and can become tuberculous pneumonia, erode into a bronchus --> bronchogenic dissemination or -->disseminate by lymphatic and hematogenous routs to isolated organs or systemically throughout the body
What else is Secondary Tuberculosis called?
adult, reactive, postprimary TB
What is the cause of secondary TB?
previous exposure and development of hypersensitivity, reexposure to the organism or reactiviatoin of latent organisms
what does secondary TB cause?
prompt granulomatous tissue response with caseous necrosis
what type of areas do mycobacterium organisms prefer?
areas of high oxygen tension
what area of the body is reactivation (secondary TB) most likely to occur?
localized to the apices of one or both upper lobes of the lungs
what is the reactivation (sec. TB) in upper lobes of the lungs called?
Assmann focus
what is Cavitation?
the anatomic hallmark of secondary TB
what can happen to Granulomas in secondary TB?
coalesce to form larger foci of destruction most eventually become encapsulated by fibrous tissue and develop areas of dystrophic calcification
what happens if lesions of Secondary TB remain active?
they erode into neighboring airways and cavitate
what is the progression of apical cavitary birbocaseous TB?
it may heal, spread by direct extension, or be dissemiated through the tracheobronchial tree,the lymphatics, or the blood
What are some local complications of secondary TB?
massive hemoptysis due to erosion of a pulmonary vessel; sufficient fibrosis that leads to the loss of ventilating capacity; infection of the pleura leading to a tuberculous empyema
what is the most frequent cause of chemical pneumonia?
Aspiration
what kinds of patients does chemical pneumonia most often occur?
unconscious patients, those with repeating vomiting episodes, and those with depressed cough reflexes (alcoholic intoxication, CNS malfunciton, acute drug intox. etc)
What are the clinical symptoms of chemical pneumonia?
they are depended upon the volume and nature of the aspirate
What does aspiration of liquid gastric contents?
extensive acute inflammatory reaction, pulmonary edema, and widespread destruction of epithelium with hemorrhage and hyaline membranes...if extensive the lung parenchyma may be almost completely destroyed
what happens if sufficient volume with low pH is aspirated?
changes can be seen clinically 2-5 hrs after aspiration. the onset of cyanosis, dyspnea, tachypnea, tachycardia and shock, bloody frothy sputum, marked pulmonary congestion and edema occurs.
What will the X-ray show after sufficient volume low pH aspiration and occurance of chemical pneumonia?
soft patchy mottling throughout both lung fields, indistinguishable from bronchopneumonia
what will occur if there is chronic or recurrent aspiration?
repeated bouts of tracheobronchitis and pneumonia
what are symptoms of chronic aspiration?
recurrent cough ad sputum production and, with time, fibrosis will ensue
What is the most common cause of lung abscess?
Aspiration
When lung abscess due to aspiration happens, what area of the lung is affected?
lower respiratory tract
Inoculation of what causes lung abscess of the lower respiratory tract?
Anaerobic organisms from the oral cavity
T or F: the abscesses tend to be solitary?
True
Why do abscesses occur most frequently on the right side?
due to the shallow angle of the right mainstem bronchus
What are some other causes of lung abscesses?
bacterial pneumonia (especially Staphylococcal and Klebsiella pneumonia), bronchial obstruction, septic emboli, cysts or bullae, and penetrating chest wound
What is it called if no underlying cause of lung abscess can be identified?
primary idiopathic or cryptogenic lung abscess
What is another name for lung abscess?
Pulmonary abscess
What is the pathology of an abscess?
consist of localized suppuration and liquefaction necrosis of lung parenchyma
What is considered chronic pathology of lung abscess?
>6 weeks
What happens to the lung wall in chronic abscess?
may be considerable fibroblastic proliferation
T or F: the abscesses can be solitary or multiple and can vary in diameter of few millimeters to large cavities of 5 -15 cm?
True
What are some clinical symptoms of pulmonary abscesses?
fever with prominent cough, accompanied by large amounts of foul smelling or bloody sputum, chest pain and weight loss.
If there is communication with an airway, what will the X-ray show?
homogenous density and air-fluid level
What can be suspected if there is no putrid sputum present?
bronchial obstruction, infected cavitated infarct
What is the fatality rate of a solitary lung abscess?
15-20%
What is the fatality rate of multiple hematogenously spread abscesses?
~50%
What are 2 main categories of neoplasia?
Bronchogenic Carcinoma & Metastatic Tumors
What are 3 types of bronhogenic carcinoma?
Squamous cell carcinoma (35%); Adenocarcinoma (35%); and small cell undifferentiated (oat cell) carcinoma (20%)
What is the leading cause of cancer deaths in the US?
bronchogenic carcinoma
What has replaced breast cancer as the most frequent cause of cancer deaths among women?
bronchogenic carcinoma
T or F: there is a greater incidence of bronchogenic carcinoma in men than in women
True
T or F: if discovered early, surgery is NOT effective treatment for bronchogenic carcinoma, except for the small cell undifferentiated type?
False, surgery IS effective if caught early except for the small cell undifferentiated type
What is the overall 5 year survival rate of bronchogenic carcinoma?
10 -15%
What is the single most important etiologic factor for bronchogenic carcinoma?
Smoking
Smoking is most closely associated with what types of bronchogenic carcinomas?
squamous cell carcinomas and small cell carcinomas
Risk is _____ to the number of cigarettes smoked daily, the duration of the habit, and tendency to inhale.
proportional
What also plays a role in bronchogenic carcinomas?
environmental/occupational exposure
From what cells do most bronchogenic carcinomas arise?
basal cells of the bronchial epithelium and differentiate into a variety of recognizable patterns
T or F: it is UNcommon for more than one histologic pattern to be present in the same tumor?
False, it is NOT UNcommon
What is squamous cell carcinoma?
central lesion and the cancer that is most closely associated with smoking
From where does the squamous cell carcinoma evolve?
preceding dysplastic squamous metaplasia of the bronchial epithelium
What are the characteristics of the squamous cell carcinoma?
it varies from well differentiated to poorly differentiated
Squamous cell carcinoma tends to infiltrate ______ before _______.
locally; metastasizing
On occasion, what can squamous cell carcinoma simulate the appearance of?
tuberculosis or infectious lung abscess on imaging studies
What are the majority of adenocarcinomas?
peripheral lesions, but central lesions can occur
What do adenocarcinomas tend to spread through?
submucosal lymphatics to hilar lymph nodes
What are the clinical represenations of the adenocarcinomas?
they may remain clinically silent until signs and symptoms of metastases appear
T or F: occasionally, adenocarcinomas arise from preexisting lung scars
True
Do women or men get adenocarcinomas more frequently?
They get them equally
Is adenocarcinoma closely related to smoking?
no
what is a rare varient of adenocarcinoma?
bronchioloalveolar carcinoma
From where does bronchioloalveolar carcinoma arise?
bronchiolar epithelium
How do bronchioloalveolar carcinomas present?
as peripheral mass lesion or irregular nodules ( i.e. multifocal independent tumors) scattered throughout one or both lungs
What can irregular nodules of bronchioloalvealar carcinoma simulate?
diffuse interstitial pneumonia
Is small cell undifferentiated (oat cell) carcinoma almost always a central lesion?
Yes
Oat cell carcinoma is predominately found in men or women?
men
What is oat cell carcinoma associated most closely with?
smoking and can progress rapidly with wide dissemination
What are the characteristics of the cells of small cell undifferentiated carcinoma?
small with little cytoplasm and no distinctive architectural growth pattern
From what type of cells do oat cell carcinomas arise?
an endocrine type cell
Small cell undifferentiated carcinoma produce what kind of substances?
hormone like substances (i.e. ADH, ACTH, gonadotrophins, etc)
What do these hormone like substances produced by oat cell carcinoma cause?
paraneoplastic syndromes
What is the effective treatment for oat cell carcinomas?
radiation and chemo is more effective than surgery, but overall prognosis is very poor
How can bronchogenic carcinoma present in a routine physcial exam?
patients with lung nodule may be free of symptoms
Chance for cure, of bronchogenic carcinoma, by surgical excision is greatest in what type of pateints?
Asymptomatic
What is the clinical presentation of patients who have symptoms associated with bronchogenic carcinoma?
chronic cough (often with hemoptysis), chest pain, anorexia and weight loss, and dyspnea
What does the Xray show in patients with bronchogenic carcinoma?
abnormal mass
What are clinical presentations of bronchogenic carcinomas that are of intrathoracic manifestation?
endobronchial obstruction with secondary atelectasis, pneumonia, abscess, or bronchiectasis; superior vena cava obstruction; extension to the pleura with or without effusion; extension to hilar and mediastinal lymph nodes; and mediastinal extension with involvement of the phrenic and recurrent laryngeal nerves causing a paralyzed diaphragm and paralyzed vocal cord
What are clinical presentations of bronchogenic carcinomas that are of extrathoracic manifestation?
generally the result of widespread metastases or systemic syndromes resulting from ectopic hormone production by the tumors.
What type of neoplasms are more common than primary neoplasms?
Metastatic
How do metastatic tumors typically present?
as mutiple nodules, usually in th elung periphery
Why is the Central Nervous System (CNS) encased by bone, membranes, and fluid?
Because of its importance and delicate nature
What bones encase the CNS?
skull and vertebral column
What membranes incase the CNS?
Dura mater, Pia mater, and arachnoid mater
What type of fluid surrounds the CNS?
Cerebrospinal Fluid (CSF)
What membrane is adherent to the bone of the cranium and follows the major fissures of the brain to secure it in place?
Dura Mater
What are the thinner inner membranes (pia and arachnoid) referred as?
leptomeninges and they follow the contours of the brain
What is beneath the arachnoid mater and what flows there?
subarachnoid space and the CSF flows in this space
What are the 6 main CNS cells?
neurons, astrocytes, microglia, ependyma, oligodendroglia, schwann cells
What are Neurons?
functional units of the NS and they populate both the CNS and PNS
What are the components of a neuron?
cell body, afferent dendritic processes, and efferent axon that is usually surrounded by a myelin sheath
What are astrocytes?
glial cells that act as stromal (supportive) cells of the NS and react to CNS injury like fibroblasts in other tissues
What are microglia?
glial cells that function as CNS macrophages
What are ependyma cells?
specialized ciliated glial cells that line the ventricular cavitities and central canal of the spinal cord
What are oligodendroglia?
glial cells that form myelin sheaths around nerve fibers in fetal CNS
What happens once the myelin is initially formed by oligodendroglia?
The myelin is necessary to maintain its integrity throughout life but cannot replace destroyed myelin
What are schwann cells?
cells that form and maintain myelin sheaths around PNS axons.
T or F: damage to myelin sheaths in the PNS can be regenerated by schwann cells
True, this is unlike the CNS where the myelin sheath cannot be fixed
What are general reactions to injury of the CNS?
cerebral edema (local or generalized)
What can cerebral edema cause?
increase intracranial pressure ( CSF pressure > 200 mm H2O)
What causes increased pressure due to cerebral edema?
when volume of material within cranium exceeds its physical capacity
What can cause localized cerebral volume increase?
tumor, abscess formation, hemorrhage, local edema, etc
What is the clinical presentation of cerebral edema, non specific CNS reaction to injury?
usually intermittent headache, mental slowness, confusion, and papilledema (swelling of the optic disc in the eye).
What is a major life threatening complication of cerebral edema?
herniation of the brain where the brain substance is displaced and compressed against a rigid structure (falx cerebri, tentorium cerebelli, foramen magnum)
What are the types of hernations associated with cerebral edema against rigid stuctures?
subfalcine herniations, uncal herniations, and tonsillar herniations...each has its own distinguishing neurological features and may be fatal
Where is CSF produced?
choroid plexus
Where does the CSF flow?
circulates throughout the ventricles of the brain and bathes the outer surface of the brain and spinal cord before reabsorption into venous circulation
What reabsorbs CSF?
arachnoid granulations
What can cause hydrocephalus with ventricular distention?
Obstruction of CSF flow, an increase in CSF production, or a decrease in reabsorption
What happens if hydrocephalus occurs before cranial bones fuse in infancy?
enlargement of the head
What happens if hydrocephalus occurs after fusion of the cranial bones?
acute hydrocephalus causes symptoms related to increased intracranial pressure (headache, nausea, vomiting, etc)
What is the clinical presentation of slow progressive hydrocephalus?
may not show elevated CSF pressures and may be clinically manifested by progressive dementia, gait disturbances, and incontinence
What is Hydrocephalus ex-vacuo?
compensatory ventricular dilation secondary to brain atrophy
A depressed fracture of the calvarium may cause what?
Tear vessels and dura mater and contuse or lacerate underlying brain tissue
What will happen if there is a fracture of the base of the skull?
Fracture may communicate with the sinuses or middle ear and result in leakage of CSF and increased risk of meningitis and intracranial infection
What is a concussion?
a clinical diagnosis characterized by transient loss of consciousness with retrograde and anterograde amnesia
What can happen due to repetitive concussions?
Permanent damage
What is a contusion?
Superficial areas of necrosis of the cortex resulting from crushing of CNS tissue by blunt force (coup lesions)
What are contrecoup lesions?
A more extensive contusion than the coup lesion that can be caused If the brain is set in motion it will strike the inside of the skull opposite the point of original contact
Where are contusions most often seen?
Inferior surface of the frontal lobes, anterior tip of the temporal lobes, and occipital lobe—these may act as foci of seizure activity, esp. the temporal lobe
What is epidural hematoma?
to hemorrhage into the potential space between the skull and dura mater
What is the most common cause of epidural hematoma?
tearing of the middle meningeal artery
Where are epidural hematomas commonly found?
Over the temporal and parietal areas
What can happen if an epidural hematoma is not surgically drained?
they tend to expand rapidly in volume with ensuing brain herniation and death.
What is a subdural hematoma?
hemorrhage into the potential space between the dura mater and leptomeninges
What is the common cause of subdural hematoma?
blunt trauma but without overlying skull fracture
What is the clinical presentation of an acute subdural hematoma?
within a few days after the trauma and are manifested by fluctuating levels of consciousness and signs related to brain herniation
What is the clinical presentation of a chronic subdural hematoma?
clinically apparent weeks or months after often otherwise insignificant head trauma and are manifested by slowly developing confusion and inattention which may progress to signs and overt herniation
What is a subarachnoid hemorrhage?
Bleeding into the space between the brain and leptomeninges
What can be identified in the CSF of a subarachnoid hemorrhage?
Blood
What is the most common cause of a subarachnoid hemorrhage?
Trauma
What is hypoxic encephalopathy?
Condition produced by any process that reduces the effective oxygenation of the brain
What affects neurologic change due to hypoxic encephalopathy?
Extend and duration of hypoxia and the length of survival after the insult
What is more damaging, acute or chronic hypoxia?
Acute
What is the leading cause of “strokes” or cerebrovascular accidents and results from the loss of blood supply with necrosis of the brain tissue?
Infarction
What is vascular thrombosis?
underlying, pre-existing damage to the vessel wall (atherosclerosis, etc.) and tends to occur within the larger vessels (internal carotid, vertebral, basilar) of older individuals
What can precede infarcts due to thrombosis?
TIA- transient ischemic attacks
What is TIA – transient ischemic attacks?
neurologic deficits (blindness, paralysis, etc) that may be of sudden onset but resolve within a short period of time.
Where do most emboli arise?
thrombi within the left heart or carotid arteries
What are common characteristics of emboli?
Multiple and involve smaller vessels and usually have sudden onset of neurologic deficit that do not resolve
T or F: symptoms depend on location and size of infarct
True
What are 2 common causes of infarcts?
Vascular thrombosis and emboli
What causes approx 10% of clinical stroke syndromes?
Intracerebral hemorrhage
What accounts for more deaths, infarcts or hemorrhage?
Hemorrhage
What is the most common cause of hemorrhage?
Preexisting systemic hypertension
What does preexisting systemic hypertension do to the arteries?
Weakens small arteries in the deep white matter of the brain
T or F: hemorrhages are confined to an arterial distribution
False, they are not confined
What do hemorrhages do to the parenchyma?
Compresses and disrupts it
Where can a hemorrhage rupture?
Into the ventricular system
What can occur secondary to hemorrhage due to expanding volume of blood in the brain
Brain herniation
What is most likely to happen to a patient who survives a hemorrhagic episode?
regain some of their neurologic deficit than are patients with cerebral infarcts since there is not the extent of necrosis as is seen with infarction
What percent of the population presents with saccular (berry) aneurysms?
5-6%
Where do most saccular aneurysms occur?
Middle cerebral artery and its branches and tends to arise at the bifurcation of vessels
T or F: most saccular (berry) aneurysms remain asymptomatic throughout a persons life
True
What happens during spontaneous rupture and hemorrhage of a saccular aneurysm?
May lead to “stroke” or sudden death
What is the symptom of leakage of blood prior to a rupture due to saccular aneurysm?
An excruciating headache
Vascular malformation occurs in what % of the population?
5%
What are the 2 types of vascular malformations?
Venous and Arteriovenous
What vascular malformation is most common and usually asymptomatic?
Venous (67%)
What vascular malformation is less common and causes intracerebral hemorrhage by rupture?
Arteriovenous (12%)
What are 2 types of bacterial infectious disease?
Acute leptomeningitis and Intraparenchymal abscess
What is an acute leptomeningitis?
a secondary infection due to extension of a local ear or sinus infection, penetrating wounds, or blood-borne organisms from a distant site
What happens to the meninges during acute leptomeningitis?
They become congested and a clouded, purulent exudate is present
What are seen in the CSF if a patient has acute leptomeningitis?
Neutrophils
What are common symptoms associated with acute leptomeningitis?
agitation, irritability, headache, photophobia, and stiff neck
Why is it important to recognize and treat meningitis in the early stages?
To avoid fatal consequences
What is a viral meningitis?
may be a complication of many systemic viral diseases but usually is not as clinically relevant as bacterial meningitis
What can be seen in the CSF during viral meningitis?
Lymphocytes
What is an uncommon but important complication of systemic viral disease?
Viral encephalitis
Portals of entry for viruses to cause viral infectious disease primarily include?
Hematogenous spread and spread along peripheral nerves
T or F: many viruses are site specific
True
What do symptoms of viral infection depend on?
Virulence of virus and site affected
What are the most common cause of viral meningitis?
Enterovirus
What causes poliomyelitis?
Enterovirus
What are symptoms of enterovirus infection?
nonspecific upper respiratory tract infection, GI symptoms, and fever which may progress to headache and stiff neck
T or F most cases of enterovirus infection do not resolve without sequelae
False, they do resolve without sequelae
What happens if the enterovirus infection is not resolved and progresses?
It involves the large motor neurons in the spinal cord and brain stem and cause permanent lower motor neuron paralysis (i.e. symptom o f polio)
When infected with herpes virus, where do the viruses live?
In the nervous system ganglia
What symptoms are associated with herpes virus infection?
Painful skin vesicles and ulcers along the nerve distribution of those ganglia
T or F: involvement of the CNS with herpes virus infection is fatal
True
What are babies born by vaginal delivery to women with genital herpes at risk for?
CNS infection and being immunocompromised patients
What does transplacental rubella infections during the 1st trimester of pregnancy cause?
Reduction in cell growth and cell division of the fetus
If infected with rubella during pregnancy, what happened to many fetuses?
They are stillborn or aborted; live birth fetuses may have slow cardiovascular and pulmonary defects, blindness, deafness, mental retardation, and low birth weight
What is primarily a disease of animals?
Rabies
How can man be infected with rabies?
Direct inoculation or less commonly inhalation
What is the incubation period of the rabies viruse?
1-3 months
What are the characteristic onset symptoms of rabies infection?
Fever, malaise, and headache which progresses to profound CNS sensitivity, coma, and death
What used to be most common cause of meningitis in children but is now more common in adults?
Tuberculosis meningitis
What does tuberculosis meningitis arise from?
Hematogenous spread from a pulmonary focus
What is the result if tuberculosis infection is not treated?
Death
What is the mortality rate of tuberculosis if treated?
30%
What occurs in about 30% of untreated cases of syphilis and a component of the tertiary stage?
Neurosyphilis
How does syphilis enter the CNS?
hematogenously during the secondary stage
What does cerebral invovlement of syphilis cause?
psychotic dementia that begins about 15 yrs after initial infection
What is Tabes dorsalis?
lack of coordination of voluntary movements and sensory disorders due to syphilis infection that involves the spinal cord and onset of 10-25 yrs after initial onset
What is the most common fungal infection of the CNS?
Cryptococcus
What are the characteristics of cryptococcus?
insidious onset with headache or behavioral changes and death if untreated
What is dementia?
impairment of orientation, memory, intellect, and judgement with associated alterations in mood and behavior
What are dementia associated with?
degenerative diseases and may also be seen in disorders due to other causes
T or F: degenerative diseases are curable and do not play a part in patient death
False. they are incurable and do influence patient death
What disease occurs in the later stages of life and has a slow insidious onset with behavioral changes (anxiety, depression, insomnia, visual hallucinations, paranoia), recent memory loss, and progressive intellectual impairment with eventual inability to comprehend, communicate, or care for oneself
Alzheimers
T or F: with proper care patients with alzheimers can lead a long life
True
What is secondary to other causes such as respiratory infection in patients with alzheimers?
death
What happens to the brain in patients with alzheimers?
the brains hows marked diffuse cortical atrophy due to loss of neurons and results in dilation of ventricles (hydrocephalus ex-vacou)
What is huntington disease?
an autosomal dominant inherited disease with the initial onset of involuntary muscle movements (choreiform movement) and facial grimaces occurring in mid-adult life. This is followed by personality changes, emotional disturbances, and dementia ultimately leading to death within 10-15 years of diagnosis
What disease has been traced to a genetic defect of chromosome 4?
Huntington Disease
What are the pathological presentations of Huntington Disease?
atrophy of specific areas within the deep white matter of the cerebral hemispheres
What is paralysis agitans?
Parkinsons Disease
What is characteristic of resting "pill-rolling" tremors and slow movements (bradykinesia) which progresses to rigidity and postural changes, poor balance, shuffling gate, and a "blank" facial expression?
Parkinsons Disease
Less than half of patients with Parkinsons will develop what?
progressive dementia
What is the cause of Parkinsons?
unknown, but there is degeneration and depigmentation of the substantia nigra region of the brain stem indicating loss of neurons which release the neurotransmitter dopamine
What is aparkinsonism?
symptoms identical to Parkinsons produced by other diseases and some drugs
What is Amyotrophic Lateral Sclerosis (ALS)?
a degenerative disease of older adults characterized by gradual progressive motor weakness leading to total paralysis and respiratory failure within 3-5 years. Sensory function and mental function remain intact. Basically, there is progressive degeneration of upper motor neurons in the spinal cord, brain stem, and cortex
What are 2 demyelinating diseases?
Multiple Sclerosis and Acute Idiopathic Polyneuritis (Guillian-Barre)
What is multiple sclerosis?
The most common demyelinating disease
How does MS represent?
autoimmune reaction against the oligodendroglia that may be triggered by a viral infection with prolonged latent period
What is the onset of MS?
young adults (20-40 years) with highly variable clinical symptoms which may include paresthesias, visual disturbances, muscle weakness, incoordination, and speech difficulties
What are characteristics of MS?
episodic exacerbations and remissions and the disease may progress to blindness, incontinence, ataxia, and paraplegia. Intelligence is usually not impaired
What are brain characteritiscs of MS?
multiple irregular gray sclerotic plaques involving both the white and gray matter. They are also almost always present in brain stem, spinal cord, and optic nerves
What is ALS?
post-infectious autoimmune disorder where there is bilateral progressive ascending motor paralysis affecting both spinal and cranial nerves often preceded by "viral" respiratory or gastrointestinal disease. It may progress to involve the phrenic nerve with paralysis of the diaphragm
How are patients who have ALS treated?
must be supported by assisted respiration until the paralysis begins to recede
What is the outcome of ALS?
usual outcome is gradual recovery without residual defect, but it may become chronically progressive
What are Metabolic Storage Diseases?
Tay-Sachs and Niemann-Pick
T or F metabolic storage diseases are rare?
True
What are the characteristics of Metabolic Storage Diseases?
genetic origin that result in various enzyme deficiencies which interrupt normal metabolic pathways and cause accumulation of precursor substances in the neural tissues and systemic organs
How do Metabolic Storage Diseases present clinically?
vary but may involve severe retardation or early death. Diagnosis and identification is important for genetic counseling
T or F there are specific signs and symptoms of neoplastic disease?
False
What are the clinical presentations of Neoplastic Diseases?
They depend on local effects (irritative, compressive, or destructive effects on the particular sites involved) and generalized effects (increased intracranial pressure, edema, hemorrhage, etc.)
What are potentially biologically malignant but has rare extraneural metastases?
all primary CNS neoplasms (arising in the neural parenchyma)
What represents as the 2nd most common type of tumor in childhood, despite overall incidence increasing in age?
CNS neoplasms
What is a Meningioma?
slow growing, extraneural, benign neoplasm which occurs most frequently in middle-aged adults and may or may not be symptomatic
From what do Meningioas arise?
cells within the leptomeninges and therefore grows as firm, lobulated, masses on the surface of the brain which compress and indent the underlying brain parenchyma
What are symtoms of Meningiomas?
headache, focal seizures and visual disturbances. The prognosis is excellent provided that the tumor is accessible to surgical removal.
What are astrocytomas?
most common glial tumors arising in the brain. There are four different grades of astrocytoma depending on the cellular differentiation.
What is a grade 1 astrocytoma?
well differentiated and arises in the subcortical white matter with a peak incidence in 3rd and 4th decades
What is the symptomatology of Grade 1 Astrocytomas?
seizures, increased intracranial pressure, motor deficits, and mental changes
Do Grade 1 astrocytomas grow fast or slow?
Slow and patients may survive ~3-10 yrs after diagnosis
What is Grade IV Astrocytoma?
Glioblastoma Multiforme
T or F: Glioblastoma Multiforme astrocytoma is a highly malignant neoplasm
True
What is the most common primary brain tumor in adults?
Glioblastoma Multiforme (Grade IV)
What is the peak incidence of Grade IV astrocytomas and where do they arise?
5th or 6th decade; arise in frontal or temporal lobes and enlarge rapidly
What is the symptomatology of the grade IV astrocytomas?
same as other astrocytomas but with more rapid progression and deterioration. Untreated, the average survival is 14 weeks. With radiation, survival may be extended up to 10-11 months
What compromises 15-30% of intracranial tumors?
Metastatic Tumors
What are symptoms of metastatic tumors?
(headache, seizures, motor deficits, mental changes, etc.) are the result of local expansion and surrounding edema
What are the primary sites of metastatic tumors (in order of frequency)?
lung, breast, malignant melanoma, kidney, and gastrointestinal tract
Where in the cortex do most metastatic lesions appear?
grey-white junction and may be multiple.
What is muscle contraction dependent on?
nervous innervation
What will have an adverse effect on muscular activity?
Any lesion to the anterior horn cell, its peripheral axon, or the neuromuscular junction of the muscle fiber itself
T or F: Muscle has a limited way of responding to changes in the environment or injury - atrophy, hypertrophy, necrosis, or contracture
True
What are are genetically determined myopathies characterized by regressive alterations in individual muscle fibers resulting in weakness of the affected muscles?
Muscular Dystrophies
How are muscular dystophies clinically divided?
various types depending on the distribution of muscle involvement, but the microscopic pathology is essentially the same in each type
What type of dystrophy is characterized by: sex-linked recessive disease (expressed in males only) which usually does not become apparent until after one year of age, affects primarily the pelvic girdle muscles causing difficulty walking and disturbances of gait?
Duchenne Type
What will cause the gastrocnemius to appear enlarged with the Duchenne Type of dystrophy?
pseudohypertrophy (fatty infiltration between muscle fibers)
How does the Duchenne dystrophy influence respiration?
There is a slow progression to involve the muscles of respiration and death usually occurs in the 20-30 year range from pulmonary complications
This is an autosomal dominant disease which characteristically first appears in adolescence
Facioscapulohumeral dystrophy type
What muscles are affected by the facioscapulohumeral type?
muscles of the face and shoulder girdle and over a long period of time affects the trunk and legs
T or F there is a significant decrease in life expectancy of those with the facioscapulohumeral type?
false, there is NO decrease in life expectancy
This is an autosomal dominant disease which appears early in adult life and tends to affect facial muscles and the distal muscles of the extremities. It is characterized by an inability to relax muscles after contraction
Myotonic Dystrophy
What are common associated signs of myotonic dystrophy?
cataracts, frontal baldness, ptosis of eyelids, cardiac abnormalities and testicular atrophy
This is an autoimmune disease that usually becomes evident in early adulthood and is characterized by abnormal muscular fatigue and weakness which is initially manifested in the most active muscles (extraocular, face, tongue, upper extremities) and may progress to involve the respiratory muscles
Myasthenia Gravis
What is the basic defect associated with Myasthesia Gravis?
destruction of the acetylcholine receptors of the muscle fiber at the neuromuscular junction which interferes with the initiation of muscle fiber contraction
Myasthenia gravis is often associated with tumors of what?
The Thymus (thymoma) or thymic hyperplasia
What is the prognosis of Myasthenia gravis?
unpredictable, there is often a chronic course with spontaneous remissions and exacerbations