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64 Cards in this Set
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Definition of Myasthenia gravis
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autoimmune disease characterized by autoantibodies against the neuromuscular junction, resulting in muscular weakness
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type of disease and where it takes place
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Clinical Presentation of Myasthenia Gravis
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Females > Males
Muscular weakness predominantly affecting hte facial muscles Extraocular muscle weakness may lead to ptosis and diplopia Weakness worsens with repeated contractions Respiratory muscle involvement may lead to death |
Gender Preference?
Predominantly affect what area? Causes what in this area? What worsens the ppresentation? Can it lead to death and how? |
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Mechanism of Myasthenia gravis
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autoantibodies against the acetylcholine (ACh) receptor
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What other pathologies are associated with Myasthenia Gravis?
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Associated with thymic hyperplasia and thymomas
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thymus
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What is the treatment for myasthenia gravis?
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anticholinesterase agents, steroids, thymectomy
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What is Eaton-Lambert syndrome asociated with?
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commonly a paraneoplastic syndrome of small-cell lung cancer
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what syndrome is associated with small cell lung cancer?
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Eaton-Lambert syndrome
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What is the mechanism of Eaton-Lanbert Syndrome?
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Production of antibodies directed against the calcium channel.
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What is the clinical presentation of Eaton-Lambert syndrome?
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Proximal muscular weakness
Weakness improves with repeated contraction |
Two things
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How does Duchenene Muscular Dystophy stand out from the other dystrophys?
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most common and severe form of muscular dystrophy.
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prevelence and severity
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Is duchenne muscular dystrophy inherited? What type of inheritance?
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X-linked inheritance
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What is the mechanism for duchenne muscular dystrophy?
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Mutation in dystrophin gene on the X chromosome (Xp21). Dystrophin is an important muscular protein and due to the mutation it is absent.
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What chromosome is the dystrophin gene on?
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X chromosome (Xp21)
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Why is the dystrophin protein important?
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it is an important muscle structural protein.
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How does the mutation in the dystrophin gene on on chromosome Xp21 effect the dystrophin protein?
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results in an absence in the dystrophin protein.
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What is the clinical presentation of duchenne muscular dystrophy?
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Normal at birth with onset of symptoms by age 5.
Progressive muscular weakness Proximal weakness of shoulder and pelvic girdles Calf pseudohypertrophy Heart failure and arrythmias may occur Respiratory insufficiency and pulmonary infections |
at what age do symptomes begin
progressive? where does the weakness most significantly occur where on the body do the muscles look extremely abnormal and what is this called can it be fatal name all 4 ways |
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What lab result will be found in Duchenne muscular dystrophy?
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elevated serum creatine kinase
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What are the micro characteristics associated with duchenne muscular dystrophy?
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muscle fibers of various sizes
necrosis, degeneration, and regeneration of fibers fibrosis fatty infiltration |
4 things
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How will you diagnose Duchenne muscular dystrophy?
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Muscle Biopsy: immunostains whaw decreased dystrophin protein
DNA analysis by PCR |
2 ways
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What is the other type of muscular dystrophy besides Duchenne muscular dystrophy?
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Becker Muscular dystrophy
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What is the prevalence and severity of Becker muscular dystrophy?
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less common and not as severe as Duchenne muscular dystrophy
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What is becker muscular dystrophy caused by?
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mutation in dystrophin protein
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What is the age on onset and progression of Becker muscular dystrophy as compared to duchenne muscular dystrophy?
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Later on set with variable progression
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Is there cardiac or respiratory involvement with becker muscular dystrophy?
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Cardiac involvement is rare
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What is the life span of someone with Becker muscular dystrophy?
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They may have a relatively normal life span.
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What is the clinical presentation of Guillain-Barre syndrome?
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Preceded by a viral illness
muscular weakness with an ascending paralysis Loss of DTRs |
3 things
preceded by.. type of paralysis DTRs |
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What is the actual pathology (mechanism) of Guillain-Barre syndrome?
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inflammation of demyelination of peripheral nerves and spinal nerve roots, resulting in muscular weakness
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How would you diagnose Guillain-Barre syndrome?
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Nerve conduction studies
lumbar puncture would have elevated protein |
2 tests
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What is the prognosis of someone with Guillain-Barre syndrome?
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Fatal in 5% because of respiratory paralysis.
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fatal?
what percent? |
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What are the two types of acute meningitis?
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Acute aseptic (viral) meningitis
Acute purulent (bacterial) meningits |
viurs and bacteria
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Whhere is the inflamation seen in acute aseptic meningits and what is the most frequent causative virus type
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leptomeningeal (arachnoid and pia mater) inflammation due to viruses (enterovirus most frequent)
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What type of infiltration is seen in acute aseptic meningitis and in what two areas is it found?
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Lymphocyti infiltration of leptomeninges and superficial cortex.
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What is the clinical appearance of acute aseptic meningitis?
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Fever, signs of meningeal irritation, depressed consciousness
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3 things
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What is the mortality rate of acute aseptic meningitis?
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Low mortality
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Where is the inflammation seen in acute purulent meningitis?
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purulent leptomeningeal inflammation due to bacteria.
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What is the most common cause of acute purulent meningitis in the following age groups:
neonates infants and children adelescents and yound adults elderly |
neonates: group B streptococci, Escherichia Coli
infants and children: haemophilus influenzae Adolescents and young adults neisseria meningitidis Elderly: Streptococcus pneumoniae and listeria monocytogenes |
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In acute purulent meningits what type of infiltration and what precific areas of the leptomeninges is the infiltrate found.
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neutrophilic infiltration of the leptomeninges, extending variably to cortex
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In acute purulent meningitis will the leptomeninges have any distinct appearance?
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Opaque leptomeninges
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What color
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In acute purulent meningitis there is Diffuse _____ _____ which creates a risk of _____ ______
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Diffuse cerebral edema: risk of fatal herniations
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What is the clinical presentation of Acute bacterial meningitis?
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Headache, fever, nuchal rigidigy, cloudy sensorium (level of consciousness), coma, and death
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6 things
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What is the result of the organization of purulent exudate and fibrosis in acute purulent meningitis?
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hydrocephalus
cranial nerrve impairment (neural deafness) |
2 things
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What is the cause of mycobacterial meningoencephalitis?
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Can be caused by mycobaterium tuberculosis or atypical mycobacteria
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What area of the brain does mycobacterial meningoencephalitis involve.
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Usually involves the basal surface of the brain
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What is a main characteristic finding in mycobacterial meningoencephalitis?
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characteristic tuberculomas within the brain and dura mater.
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Mycobacterial meningoencephalitis is often found in ____ patients, particularly by _______ ______.
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AIDS, mycobacterium avium-intracellulare (MAI)
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CSF Parameter in Different Forms of Meningitis
What are the normal CSF values for: Cells, gulcose, proteins, and pressure |
Cells: <5 lymphocytes
Glucose: 45-85 (50-70% glycemia) Proteins: 15-45 Pressure: 70-180 |
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CSF Parameter in Different Forms of Meningitis
In bacterial meningitis what are the CSF values for: Cells, gulcose, proteins, and pressure |
Cells: up to 90,000 neutrophils
Glucose: decreased (<45) Proteins: Inceased (>50) Pressure: Markedly elevated |
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CSF Parameter in Different Forms of Meningitis
In viral meningitis what are the CSF values for: Cells, gulcose, proteins, and pressure |
Cells: 100-1,000 mostly lymphocytes
Glucose: normal Proteins: Increased (>50) Pressure: Slightly elevated |
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CSF Parameter in Different Forms of Meningitis
In granulomatous (mycobacterial/fungal) meningitis what are the CSF values for: Cells, gulcose, proteins, and pressure |
Cells: 100-1,000 lymphocytes
Glucose: Decreased (<45) Proteins: Increase (>50) Pressure: Moderately elevated |
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What are the common features of viral encephalitis?
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Perivascular cuffs, microglial nodules, neuron loss, and neuronophagia
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4 things
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What are the clinical manifestations of viral encephalitis?
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variable (mental status change, fever, and headache, often progressive to coma)
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What are the 4 specific forms of viral encephalitis?
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Arthropod-Borne
Herpes Simplex Type 1 Rabies HIV |
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What is characteristic of herpes simplex type 1 viral encephalitides?
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characteristic hemorrhagic necrosis of temporal lobes
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What is characteristic of rabies viral encephalitis?
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charaacteristic negri bodies in hippocampal and purkinje neurons
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What is characteristic of the HIV form of viral encephalitides.
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Cerebral involvement is common and leads to AIDS-dementia complex.
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What is included in the AIDS-dementia complex and what is it's histopathology?
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Dementia and other neurological abnormalities.
histopathology: microglial nodules and diagnostic multinucleated giant cells |
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What does spinal involemnet of HIV viral encephalitis lead to?
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spinal involvement of HIV viral encephalitis leads to "vacuolar myelopathy": similar to vitamin B12 deficiency-associated "subacute combined degeneration"
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What is "progressive multifolal leukoencephalopathy " (PML)?
cause? micro? |
Related to JC virus (a polyomavirus)
JC virus causes PML in immunocompromised patients (especially AIDS) Histopathology: demyelination, lymphohistiocytic, and astrogliosis astrocytes acqure bizarre shapes Oligodendrocytes in acive lesions contain intranuclear inclusions. |
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What patient population does "progressive multifocal leukoencephalopathy" occur in?
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AIDS
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What are the most frequent causative agent of fungal meningoencephalitides?
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Candida, Aspergillus, Cryptococcus, and Mucor species are hte most frequent agents
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In fungal meningoencephalities, two causative agents have a marked tropism for blood vessels. What are these agents and what does this cause?
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Aspergillus and mucor have ea marked tropism for blood vessels: vasculitis, rupture of blood vessels, and hemorrhage are the results.
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Which agent causes diffuse meningoencephalities and the common characteristic of this?
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Cryptococcus causes diffuse mentingoencephalitis and "soap bubble lesions" are the characteristic of cryptococcus.
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For toxoplasmosis,
what is the most common pat. pop., what is the result of the infxn., and the is the diagnostic characteristic of of toxoplasmosis? |
AIDS patients
Cerebral abscess with central necrosis and chronic inflammation mRI/CT scan: characteristic ring-enhancing lesions |
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What are the clinical manifectations and diagnostic characteristic of a cerebral abcess?
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CT/MRI: ring-enhancing lesion
Signs of increasd intracranial pressure (headache, vomiting, and papilledema) Focal neurological deficits |
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