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308 Cards in this Set
- Front
- Back
What is destroyed in multiple sclerosis?
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Oligodendrocytes are destroyed which causes demyelination of CNS axons.
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What is particularly interesting in the CSF of a MS patient?
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Oligoclonal bands. IgG/albumin ratio is also usually off.
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What are Schwann cells?
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Myelin producing cells for the PNS
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What are Dawson's fingers?
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Areas of demyelination surrounding end venules in a longitudinal section of the brain in MS patients.
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What cells mediate MS?
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T cell-mediated disease, particularly CD4 T cells.
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When does MS usually manifest?
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3-5th decades
|
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What is an essential part of the clinical presentation pattern of MS?
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Dissemination in TIME and SPACE
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What type of optic neuritis is a typical presentation of MS?
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Central scotoma due to retrobulbar optic neuritis (loss of central vision in 1 eye)
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What do T2 or Gadolinium lesions indicate?
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Recent plaques. The presence of some recent plaques and some older plaques can fulfill the dissemination need in the DX of MS.
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What is included in the DDx of MS?
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Acute Disseminated Encephalomyelitis
Idiopathic transverse myelitis Sarcoidosis SACD Infectious myelopathy (HTLV1) Tumor |
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What is used to treat acute exacerbations of MS?
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High-dose corticosteroids.
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What are some treatments for the prevention of MS exacerbations?
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Interferon-beta
Glatiramer acetat Mitoxantrone Natalizumab |
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How does natalizumab work?
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Natalizumab blocks VLA4 interaction in MS to impairs the binding of these molecules to VCAM so the immune cells cannot cross the BBB.
Used in MS |
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What is a S/E of mitoxantrone?
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Cardiotoxicity
|
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What are some causes of multiple mononeuropathies?
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Vasculitis
Diabetes mellitus Sarcoidosis Leprosy Hypothyroidism Hereditary neuropathy w/liability to pressure palsies |
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What is the mutation in DMD?
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Mutation in the dystrophin gene at Xp21
|
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What is the inheritance pattern of DMD?
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X-linked recessive lethal
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What is the clinical presentation of a px w/DMD?
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Toe-walking, waddling gait, excessive lumbar lordosis, and calf muscle hypertrophy
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What is the presentation of Fascioscapulohumeral dystrophy?
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In adulthood.
Inability to whistle (mild facial weakness) and weakness of scapulohumeral muscles. Slow progression. |
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What is the mutation associated with FSH dystrophy?
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Autosomal dominant of 4q35 chromosome
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What is the presentation of mytonic dystrophy?
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Congenital: respiratory failure & feeding difficulties, hypotonia and cognitive dysfxn
Adult: mild-moderate; slowly progressive weakness of face, anterior neck, and distal limbs w/difficulty relaxing mm. Frontal balding, temporal wasting, cataracts |
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What is the mutation associated w/myotonic dystrophy and its inheritance pattern?
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DMPK gene on chromosome 19.
CTG repeat disease. Autosomal dominant |
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What is oculopharyngeal dystrophy?
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Autosomal dominant disorder that appears in 4-5th decades.
Ptosis, dysphagia, possible mild weakness. GCG repeat on chromosome 14q11 |
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What is the inheritance patterns of mitochondrial myopathies?
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Maternal pattern of transmission w/variable linkage.
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What are the characteristics of MERRF?
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myoclonus, generalized seizures, ataxia, dementia, hearing loss, optic atrophy, muscle weakness
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What are the characteristics of MELAS?
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encephalopathy, high lactic acid level in serum or CSF, stroke-like episodes
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What are the characteristics of Kearnes-Sayres syndreom?
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Mitochondrial myopathy.
Progressive ophthalmoplegia, retintis pigmentosa, cardiac conduction abnormalities, myopathy |
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What are the general characteristics of glycolytic defects?
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Dynamic, related to exercise intolerance as exercise-induced cramps, pain or myoglobinuria.
Due to a defect that results in accumulation of a substrate or failure to produce an end product |
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What is type II glycogenosis?
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Acid maltase deficiency
Infantile form=Pompei's disease; presents w/hypotonia, failure to thrive in 1st 3mos Adult presents in 3rd-4th decade w/proximal weakness |
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What is type V glycogenosis?
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Myophosphorylase deficiency (McArdles)
Exercise intolerance w/muscle pain, cramps, myoglobinuria |
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What is CPT2 deficiency?
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CPT2 is needed to get carnitine into the mitochondria for lipid metaboilsm.
Autosomal recessive on 1p32 Presents w/myoglobinuria & renal failure |
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What is malignant hyperthermia?
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Autosomal dominant disorder
Severe reaction to anesthesia Severe muscle rigidity, myoglobinuria, fever, tachycardia, arrhythmias. |
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What are periodic paralyses?
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Myopathies characterized by mutations in muscle membrane ion channels.
Hypokalemic=mutation in msucle membrane Ca channel Hyperkalemic=mutation in sodium channel |
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What is polymyositis?
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Adult onset.
Present w/weeks-months of proximal weakness & neck flexor weakness. |
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What is the cause of polymyositis?
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HLA restricted antigen specific cell mediate immune response against muscle fibers
|
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What is included in a WU for polymyositis?
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Muscle enzymes-->elevated CK, ANA, anti-Jo-1
EMG show irritable myopathy Bx=inflammation w/invasion of non-necrotic fibers by CD8 T cells and macrophages |
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What is the tx for polymyositis?
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immunomodulation
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What is the presentation of dermatomyositis?
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Weakness of neck flexors, shoulder girdle & pelvic girdle.
Rash precedes weakness-->heliotrope rash. Heliotrope rash=purple w/periorbital edema Gottrens sign over knuckles SubQ calcifications may be seen kids. |
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What is the pathophysiology of dermatomyositis?
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Vasculopathy of the muscle and other organs.
Humerally mediated microangiopathic disroder w/deposition of IgM C3 |
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What is seen in a WU for dermatomyositis?
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CK is elevated.
EMG=irritable myopathy Bx=perifascicular atophy & perivascular inflammation |
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What is tx of dermatomyositis?
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Immunomodulation w/IVIg & highdose steroids
|
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What is inclusion body myositis?
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Occurs in adults over 50
Slowly progressive weakness of both distal and proximal muscles w/prominent weakness of quadriceps & wrist & finger flexors |
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What is seen in a muscle Bx of inclusion body myositis?
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Invasion of non-necrotic fibers by CD8+ T cells. Eosinophilic cytoplasmic inclusions & deposition of amyloid.
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What is the tx for inclusion body myositis?
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No tx
|
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What can cause viral myositides & what is seen in the disease?
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CMV, influenza, coxsackie, EBV, mumps.
CK 500x NL |
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What bacteria can cause inflammatory muscle disease?
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Staph aureus in the tropics. Erythema, swelling, tenderness seen.
|
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What drugs cause necrotizing myopathies?
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Cholesterol-reducing drugs (statins, gemfibrozole, clofibrate)
Amphiphilic drugs (chloroquine & amiodorone) |
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What causes anti-microtubular myopathy?
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Colchicine.
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What causes drug-induced mitochondrial myopathy?
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AZT
|
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What causes drug-induced inflammatory myopathy?
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Tryptophan, D-penicillamine, procainamide (RARE)
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What causes myopathy secondary to impaired protein synthesis?
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Long-term steroid use (prednisone)
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What is the incidence distribution of myasthenia gravis?
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Peak 1: 2nd & 3rd decades, F>>M
Peak 2: 6th & 7th decade, M>>F |
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What is the pathophysiology of myasthenia gravis?
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Antibodies directed against AChR reduces the number of functional AChRs.
This results in EPP of diminshed amplitude. Fatigue results |
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What organ may be enlarged in myasthenia gravis?
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Thymus, some may have thymoma.
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What is used to test for myasthenia gravis?
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Repetitive nerve stimulation
Tensilon testing (edrophonium) Lab tests for anti-AChR antibodies CXR for thymus |
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What is the tx for myasthenia gravis?
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Anticholinesterase agents (pyridostigmine)
Immunosuppression Immunomodulation Thymectomy for px w/thymomas |
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What is Eaton-Lambert syndrome?
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Antibody mediated paraneoplastic disorder of the presynaptic junction. Autoantibodies directed against presynaptic Ca2+ channels-->reduce amt of ACh released.
Often related to small cell lung CA |
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What is the clinical presentation of Eaton-Lambert syndrome?
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Dry eyes & mouth
Constipation & impotence |
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How does botulinum toxin work?
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Interferes with transmitter release at motor nerve & at presynaptic cholinergic terminals. Appears to affect sensitivity to Ca2+
|
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What is the most common mutation related to DMD?
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Deletion of exons of dystrophin gene.
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What is the mutation associated with congenital muscular dystrophy?
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Mutations in laminin-2 (link to the basement membrane of muscle)
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What is the inheritance pattern seen in most cases of hereditary ALS?
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Autosomal dominant
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What genes are mutated in ALS?
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SOD1: missense mutations-->acquisition of toxic property
TDP-43 & FUS/TLS-->RNA binding proteins needed for correct splicing |
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What is SMA?
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Spinal muscular atrophy
Affects LMNs Destruction of motor neurons in anterior horn |
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What is the inheritance pattern of SMA?
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Autosomal recessive
|
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What is Type 1 SMA?
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Werdnig-Hoffman disease
Most severe. Onset before 6mos Death w/in 1st 2 years |
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What is Type II SMA?
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Intermediate in severity
Onset at 1.5 years Px never learn to walk |
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What is Type III SMA?
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Kugelberg-Welander
Onset after18 months Patients able to stand & walk |
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What is the gene mutated/lost in SMA?
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SMN1
There is both SMN1 & SMN2. Can be deletion of SMN1 or conversion of SMN1 to SMN2. SMN2 is not SMN1-->lacks exon 7-->not enough protein produces. More SMN protein-->milder phenotype |
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What are initial signs of CMT?
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stumbling gait, awkardness while running, ankle weakness in childhood.
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What is CMT?
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Motor sensory neuropathies
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What is the difference between CMT1 and CMT2?
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CMT1 has reduced nerve conduction velocity and demyelination
CMT2 has normal nerve conduction velocity and not obvious demyelination |
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What causes most cases of CMT1?
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duplication of PMP22
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What do mutations in MPZ gene cause?
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Similar to PMP22 mutations-->CMT1
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What does PMP22 code for?
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Protein in compact myelin
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What is connexin and what is it related to?
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CMT neuropathies
gap junction protein needed for nutrient entrance |
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What gene is mutated in Huntington's chorea?
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huntingtin
CAF expansion--->polyglutamate stretch produced-->protein inclusion. |
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What is affected in spinocerebellar ataxia?
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cerebellar purkinje cells-->uncoordinated walking.
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What is affected in spinobulbar muscular atrophy?
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motor neurons, polyglutamate expansion in androgen receptor
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What is the mutation in Fragile X syndrome?
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CCG repeat resulting in extensive methylation of 5' end of FMR gene-->shutdown of FMR expression
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What the mutation in myotonic dystrophy?
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expansion in 3' UTR of a gene resulting in RNA accumulation
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What is the mutation involved in Friedrich ataxia?
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GAA expansion in intron that affects splicing of Frataxin pre-mRNA
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What are the symptoms of dominant spinocerebellar ataxia?
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Gait ataxia, dysarthria.
Specific versions have ocular disorders, extrapyramidal signs, peripheral neuropathy, intellectual deterioration, seizures |
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What is accumulated in NFTs in AD?
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tau and ubiquitin
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What is a mutation associated w/AD?
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APP mutation causing missplicing to produce amyloid deposit.
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What are the dominant genes related to Parkinson's disease?
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alpha-synuclein (component of lewy bodies)
LRRK2 UCH1 (ubiquitin terminal ester) |
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What are some recessive genes related to Parkinson's disease?
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DJ1
PINK1 |
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What do lewy bodies consist of?
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synuclein
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What is Gerstmann-Straussler-Scheinker?
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Progressive degenerative disease w/prominent cerebellari nvolvement w/ataxia
Prion disease |
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What mutations are related to epilepsy?
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Mutations in ligand & voltage gaited channels.
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What is a myelinoclastic disorder?
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Disorder in which myelin breaks down due to some known or unknown exogenous factor.
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What is a leukodystrophy?
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Disorder in which myelin breaks down due to mutations in genes responsible for myelin metabolism or maintenance.
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What are the plaques seen in MS brains?
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Active plaques: densely cellular w/lots of lymphocytes, foamy macrophages, few astrocytes
Inactive plaques: paucicellular, densly gliotic, depletion of oligodendrocytes, reduced # of axons & axonal atrophy Shadow plaques: myelin reduced but not absent, thin myelin sheath on remyelinated axons |
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What is the clinical presentation of ADEM?
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Abrupt onset of neurologic syndrome w/persisent high fever & headache
Many have hx of febrile illness 2-14 days before |
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What is the neuropath of ADEM?
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Macro: edema in brain, possible herniation
Micro: small engorged vv. surrounded by foamy macrophages & lymphocytes, sleeve-like demyelination around vv. |
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What causes SACD?
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B12 deficiency.
Usually seen in the setting of atrophic gastritis. Paresthesiae of limbs early on. |
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What is the neuropath of SACD?
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Macro: shrunken, discolored posterior & lateral columns in spinal cord in lower C & T spine.
Micro: early lesions show symmetric, spongy vacuolation & degeneration of myelin sheaths in T spine in posterior columns then CST & SCTs. Myelin breakdown leads to axonal degeneration & gliosis. |
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What is central pontine myelinolysis?
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Monophasic demyelinatino of basis pontis.
Setting: rapid correction of hyponatremia, extensive skin burns, alcoholics, liver transplant px. Rapid onseto f confusion, limb weakness, gaze palsies, dysarthria, dysphagia, hypotension |
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What is the neuropath of central pontine myelinlysis?
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Basis pontis has central gray discolored region.
Active demyelination w/reactive astrocytes and macropahges. rare lymphocytes. |
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What causes PML?
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JC & BK viruses (polyomaviruses)
|
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What is the clinical presentation of PML?
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Focal neurologic deficits, dysarthria, limb weakness, visual disturbances, ataxia, personality changes, seizures. Death w/o immune reconstitution
|
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What is the neuropath of PML?
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WM has small foci of gray discoloration. Multifocal.
Areas of demyelination w/scant perivascular lymphocytes. Nuclear inclusions present |
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What is Krabbe's disease?
|
Galactocerebroside-beta-galactosidase deficiency
Lysosomal. Autosomal recessive Onset in infancy w/failure to thrive & vomiting. Death at 1-2 |
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What is the neuropath of Krabbe's disease?
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Mod-severe atrophy in narrow gyri & wide sulci (WM is gray & firm due to gliosis)
Extensive loss of myelin & oligodendrocytes. See globoid macrophages which tend to be multinucleated & cluster around blood vessels. |
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What is metachromatic leukodystrophy?
|
Arylsulfatase A def.(AR)
Present 1-2yo w/ataxia, spasticity, absent reflexes in LLs. Optic atrophy & seizures. Die at 3yo |
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What is the neuropath of metachromatic leukodystrophy?
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WM is firm & chalky.
Demyelination w/extensive axonal loss. Depletion-loss of oligodendrocytes w/severe gliosis. See brown metachromasia in froze nsections--->accumulation of sulfatide in tissue. |
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What is adrenoleukodystrophy?
|
ALDP mutation-->X-linked.
Infant: hypotonia, seizures, failure to thrive, deafness, retinal degeneration Childhood: loss of skills, dementia, cerebellar disorder Adulthood: clumsiness, spasticity, dementia. Very long chain fatty acids accumulate in tissue |
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What is the neuropath of adrenoleukodystrophy?
|
NL brain externally.
Firm gray WM w/symmetric caudal-->rostral gradient of severity (frontal better than caudal) U-fibers spared. Severe demyelination in central cerebral WM & commissures. Adrenals are atrophic & cortex is replaced by ballooned cells w/striated cytoplasm |
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What causes Tay-Sachs?
|
Autosomal recessive mutation in hexosaminidase A deficiency-->accumulation of GM2 ganglioside
|
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What is the clinical presentation of tay-sachs?
|
NL for first few months then fairulty to acquire coordinated skills, possible seizure dev.
Easily startled. Cherry red spotseen in macula & blindess at ~1yr Large heads at 2 years Death at 2-4 |
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What is the neuropath of taysachs?
|
Gyral atrophy w/loss of WM
Ballooned neurons through CNS & PNS. Foamy neurons. PAS positive |
|
What are the mucopolysaccharidoses?
|
Disorders due to defects in genes that metabolize glycosaminoglycans (GAGs) resulting in GAG accumulation in neurons
Hunters and Hurlers |
|
What are the inheritance patterns of Hunters & Hurlers?
|
Hunters: X-linked
Hurlers: AR |
|
What is the clinical presentation of Hunters?
|
Mental retardation, iduronidase sulfatase def., dermatan sulfate accumulates
|
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What is the clinical presentation of Hurlers?
|
Mental retardation, iduronidase sulfatase def., dermatan sulfate accumulates, dwarfism, corneal clouding, skeletal deformities (kyphosis, short neck, coarse facial features)
|
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What is the neuropath of mucopolysaccharidoses?
|
Macro: dural & meningeal thickening that can cause obstructive hydrocephalus. cut surface of WM can cause small perivascular cavities filled with foamy macrophages.
Micro: neuronal storage that varies with degree of mental retardation |
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What is the neuropath of AD?
|
Reduced brain weight with small, thin cerebral gyri & widened sulci
Enlarged third & lateral ventricles Extracellular amyloid plaques & NFTs |
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What is the neuropath of dementia w/lewy bodies?
|
More atrophy in limbic areas.
Lewy bodies in several areas. Neuronal loss in substantia nigra & locus cereleus |
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What is the neuropath of huntington's chorea?
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Cerebral atrophy & reduced brain weight. Atrophy of caudate, putamen, and globus pallidus.
Neuronal loss & gliosis in areas of basal ganglia. |
|
What is the neuropath of Parkinson's dsease?
|
Midbrain & pons have loss of pigment in substantia nigra & locus cereleus, which both also show neuronal loss.
Lewy bodies must be found for dx. |
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What is the neuropath of SCA?
|
Atrophy of cerebellum, pons, and inferior olivary nuclei. Neuronal loss in SC.
|
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What is the clinical presentation of Friedrich's ataxia?
|
Onset before 15
Ataxia of gait followed by limb ataxia, dysarthria, loss of proprioception & vibration sense in LL, generalized areflexia, exensor plantar response. |
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What is the most common mutation in Friedrich's ataxia?
|
GAA repeat
|
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What is the neuropath of Friedrich's ataxia?
|
SC: degeneration & gliosis in posterior columns, distal degeneration of CST & SCT
Medulla: degeneration & neuronal loss in accessory cuneate & gracile nuclei. Cerebellum: gliotic WM, cortex usually spared, dentate nucleus has marked neuronal loss & SCPs are atrophied. DRG have neuronal loss. |
|
What is the neuropath of ALS?
|
Shrunken, gray anterior nerve roots.
SC can be atrophic Loss of motor neurons & gliosis in spinal cord & brainstem (LMNs) and motor cortex (UMNs) SC has degeneration of CST in lateral funiculus. Skeletal muscle atrophies. |
|
What is a small, linear hemorrhage?
|
Nerve fiber layer hemorrhage
|
|
What is a small, round hemorrhage?
|
Deep intraretinal hemorrhage
|
|
What is a large, red hemorrhage with vessels anterior to it?
|
Subretinal hemorrhage
|
|
What is a large, red hemorrhage anterior to the blood vessels?
|
Preretinal hemorrhage
|
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What is a large, dark hemorrhage?
|
Choroidal
|
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What hemorrhages come from choroidal vessels?
|
Subretinal or choroidal
|
|
What vessels do preretinal hemorrhages come from?
|
Disc & retinal vessels
|
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What vessels do nerve fiber layer hemorrhages come from?
|
Disc & retinal vessels
|
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What vessels do deep intraretinal hemorrhages come from?
|
Retinal vessels
|
|
Where do disc vessels bleed?
|
Only near the disc.
|
|
What is the etiology of a hemorrhage from disc vessels?
|
Neurologic
|
|
What is the etiology of a hemorrhage from retinal vessels?
|
Systemic disease
|
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What is the etiology of a hemorrhage from choroidal vessels?
|
Inflammatory or degenerative disease
|
|
What is a flat black disc shaped pathology?
|
Choroidal nevus
|
|
What is an elevated black disc shaped pathology?
|
Melanoma
|
|
What is an angular black pathology?
|
Retinitis pigmentosa
|
|
What is a doughnut-shaped black pahtology?
|
Chorioretinal scar
|
|
What is a diffuse balck pathology indicative of?
|
Inflammatory or degenerative disease
|
|
What are the topical beta-blockers?
|
timolol, levobunolol, carteolol
|
|
What are the S/E of topical beta blockers?
|
Bradycardia, shortness of breath, masked hypoglycemia, increased lipid panel
|
|
What are the S/E of topical prostaglandins?
|
Redness, longlong eyelashes, iris color change (permanent), macular edema, reactive HSV keratitis
|
|
What are the S/E of carbonic anhydrase inhibitors?
|
kidney stones, numbness, malaise, metabolic acidosis
|
|
How do fluoroquinolone work?
|
DNA gyrase inhibitors
|
|
What is hypopyon?
|
layer of WBCs at the inferior aspect of the anterior chamber
|
|
What is keratic precipitates?
|
deposits of inflammatory debris on the endothelial surface of the cornea
|
|
What is anterior synechiae?
|
Inflammatory adhesions between the peripheral iris and the peripheral cornea
|
|
What are posterior synechiae?
|
Inflammatory adhesions between posterior iris and lens
|
|
What is arteritic ischemia optic neuropathy related to?
|
temporal arteritis
|
|
What is nonarteritic ischemic optic neuropathy related to?
|
diabetes and HTN
|
|
What are the most common benign ocular tumors in children?
|
Dermoid
|
|
What is the most common benign ocular tumor in adults?
|
Cavernous hemangioma
|
|
What is dacryocystitis?
|
Infection of the nasolacrimal duct
|
|
What is the most common malignant orbital tumor in children?
|
Rhabdomyosarcoma
|
|
What do you see with Horner's syndrome?
|
TRIAD
Ptosis Miosis Anhidrosis |
|
What is a 1st order Horner syndrome?
|
Central lesion
Could be stroke, tumor or demyelination |
|
What is a 2nd order Horner syndrome?
|
Preganglionic
Pancoast tumor Carotid dissection Surgery Trauma |
|
What is a 3rd order Horner syndrome?
|
Cavernous sinus mass
Cluster headache ICA disease |
|
What does a CNIII palsy indicate?
|
Aneurysm at junction of PCoA & ICA until proven otherwise
|
|
What is caused by giant cell arteritis?
|
Sudden, profound vision loss
AION |
|
What is this and what is it evidence of?
|
Hypopyon
Evidence of anterior uveitis or iritis. |
|
What is the white pathology seen here?
|
Hard exudate
|
|
What is the pathology seen here?
|
Hard exudate
|
|
What is the pathology seen here?
|
Chorioretinal scar
|
|
What is seen here?
|
Keratic precipitate
|
|
what is the white pathology here?
|
Drusen
|
|
What is the white pathology seen here?
|
Soft exudate
|
|
What is the white pathology here indicative of?
|
Retinitis
|
|
What disease is seen here?
|
HSV keratitis
|
|
What is seen here?
|
Multifocal chorioretinal scarring indicating ocular histoplasmosis
|
|
What is this?
|
Vitritis: inflammatory cells in the vitreous behind the lens!
|
|
What is this?
|
Retinal vasculitis
|
|
What is this?
|
Toxoplasmosis
|
|
What is this?
|
Choroidal nevus
|
|
What is this red pathology?
|
Choroidal hemorrhage
|
|
What is this red pathology?
|
Deep intraretinal hemorrhage
|
|
What is this red pathology?
|
Nerve fiber layer hemorrhage
|
|
What is this red pathology?
|
nerve fiber layer hemorrhage
|
|
What is this red pathology?
|
Nerve fiber layer & preretinal hemorrhage
|
|
What is this red pathology?
|
Preretinal hemorrhage
|
|
What is this red pathology?
|
Preretinal hemorrhage
|
|
What is this red pathology?
|
Subretinal choroidal hemorrhage
|
|
What is this red pathology?
|
Subretinal hemorrhage
|
|
What is this black pathology?
|
Malignant melanoma
|
|
What is this black pathology?
|
Choroidal nevus
|
|
What is this black pathology?
|
Pigment crescent
|
|
What is this black pathology?
|
Retinitis pigmentosa
|
|
What is the C/D ratio?
|
0.1
|
|
What is the C/D ratio?
|
0.7
|
|
What is the C/D ratio?
|
0.8
|
|
What optic disc pathology is seen here?
|
Optic atrophy
|
|
What is seen here?
|
Scleral crescent, a normal variant
|
|
What pathology is seen here?
|
0.7 C/D ratio
Disc edema |
|
What pathology is seen here?
|
0.7
Optic atrophy |
|
What is this white pathology?
|
Soft exudates/cotton wool spots
|
|
What is this?
|
Central retinal artery occlusion
|
|
What is this?
|
Flare and/or cell
|
|
What is this white pathology?
|
Soft exudate/cotton wool spots
|
|
|
|
|
What is this?
|
Ischemic optic neuropathy
|
|
What is this?
|
PDR
|
|
What is this?
|
Posterior synechiae
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What is this?
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Retinal artery occlusion
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What is this white pathology?
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Soft exudate/cotton wool spot
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What is this?
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Retinitis pigmentosa
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What is this?
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Retinitis pigmentosa
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The ankle jerk reflex is related to what cord levels?
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S1, S2
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The patellar reflex is related to what cord levels?
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L3, L4
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The biceps reflex is related to what cord levels?
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C5, C6
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The triceps reflex is related to what cord levels?
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C7, C8
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Describe upper brachial plexopathy.
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C5-C6
weakness of shoulder abduction internal rotation of shoulder elbow flexion waiter's tip position |
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Describe lower brachial plexopathy.
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Ulnar and median weakness affects intrinsic mm. of hand & wrist flexion
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Describe a preferential lumbar plexopathy.
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Proximal muscle weakness, loss of patellar reflex.
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Describe a preferential sacral plexopathy.
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weakness below knee & loss of achilles reflex
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LMN signs.
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Loss of reflexes
Decreased tone Muscle atrophy Fasciculations Flexor plantar response |
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UMN signs.
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Hyperreflexia
Increased tone/spasticity Extensor plantar response |
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Positive Romberg indicates what?
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Sensory ataxia
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UMN vs. LMN in face.
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UMN spares forehead.
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Describe cerebellar ataxia.
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Incoordination
Hypotonia Oculomotor abnormalities Dysarthria |
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Parietal lobe damage results in what?
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Either side: impairment of sensation on contralateral side of body, decreased 2 pt discrimination, sensory inattention, extinction, sensory agnosia
Dominant side: apraxias (Gerstmanns syndrome=dyscalculia, L-R agnosia, finger agnosia, dysgraphia) Nondominant side: neglect of opposite side, impaired constructional abilities |
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What is the result of bilateral temporal lobe lesions?
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Profound memory loss
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What is the result of lesions to the dominant temporal lobe?
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decreased verbal learning
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What is the result of lesions to the nondominant temporal lobe?
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decreased visual learning
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What is the result of frontal lobe lesions?
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Hemiparesis
Personality changes Motor difficulties (motor cortex) |
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Describe Broca's aphasia.
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Damage to dominant inferior frontal gyrus.
Nonfluent speech but good comprehension Associated w/contralateral hemiparesis |
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Describe Wernicke's aphasia.
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Lesion in dominant superior temporal gyrus.
Fluent speech with poor comprehension. Poor awareness of problem. |
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Describe conduction aphasia.
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Lesion to arcuate fasciculus, which connects Wernickes & Brocas areas.
Difficulty with repetition. |
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What is a typical presentation of polyneuropathy?
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Stocking-glove loss.
Usually symmetrical |
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What is a sensory neuropathy?
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Loss of neurons at DRGs.
profound sensory loss in extremities, can be painful. |
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Pes cavus is related to what disease?
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CMT
Motor sensory neuropathy |
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What is Guillain-Barre?
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An acquired demyelinating polyneuropathy associated with recent infection.
Results in slowed NCVs Normal CSF w/increased protein |
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What is the diagnostic criteria for Guillain barre?
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1. Weakness that is ~symmetric in all limbs
2. Paresthesias in feet & hands 3. Areflexia or hyporeflexia by 1 wk 4. Progression over days-1 month 5. Elevated CSF protein w/in 3wks 6. Slowed NCVs |
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What is seen in dying back distal axonopathy?
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Longest & largest axons affected @ nerve terminal
Due to metabolic abnormality Can be associated w/diabetes, uremia, alcohol, vitamin def., drug tox. |
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What are the lab features of dying back distal axonopathy?
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Normal NCVs-->weakness & sensory loss due to nerve fiber loss
CSF normal |
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What disorders are associated with a CAG repeat expansion?
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Huntingtons
SCA Spinal bulbar muscular atrophy |
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What disorders are associated with CGG repeat?
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Fragile-X
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What disorders are associated with CTG repeat?
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Myotonic dystrophy
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What disorders are associated with GAA repeat?
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Friedrich ataxia
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What is seen here?
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Active MS plaque with accumulation of T cells near vessels.
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What disease could this be?
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ADEM
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What is seen here?
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Adrenoleukodystrophy.
See ballooned neurons. |
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What is seen here?
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Perivascular lymphocytes.
Adrenoleukodystrophy |
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What is this?
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ALS
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What is this an image of?
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Amyloid plaques of AD
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What is the disease seen here?
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Central pontine myelinolysis.
Loss of myelin in basis pontis. |
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What disease is this?
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Friedrich's ataxia with posterior column loss (gracile>cuneate)
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What is seen here?
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Atrophy of caudate & putamen related to Huntingtons
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What is seen here?
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Paucicellular, densely gliotic inactive MS plaque
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What are these related to?
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Globoid macrophages related to Krabbe's disease.
Pathognomic. |
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What are these?
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Lewy bodies
DLB |
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What is this indicative of?
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metachromatic dystrophy
Should be violet, but is brown to sulfatide accumulation. |
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What is seen here?
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Accumulation of ganglioside in neurons related to mucopolysaccharidoses.
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What is seen here?
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Nuclear inclusions seen in PML
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What are the possible causes of distal axonopathies?
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Diabetes
Uremia Amyloidosis Alcohol Thiamine deficiency Phenytoin toxicity |
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What conditions can affect DRG cell bodies causing neuronopathies?
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Cancer (paraneoplastic)
Sjogrens Vitamin B6 toxicity Cisplatin drugs HIV (rare) |
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Drusen indicate what?
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May be age-related macular degeneration.
Unrelated to systemic disease. |
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Hard exudates indicate what?
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HTN, diabetes, venous occlusion, macular degeneration
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Soft exudates indicate what?
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Severe systemic occlusive microvascular disease
HTN, collagen disease, AIDS |
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Myelinated nerve fibers indicate what?
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Normal variant
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List 3 mechanisms used by antiseizure drugs.
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Enhance GABAergic transmission
Inhibit glutamatergic transmission Block voltage-gated Na+ and Ca2+ currents |
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List the drugs that work by slowing the rate of recovery of voltage-gated sodium channels from inactivation.
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Hydantoins
Iminostilbenes Carboxylic acids Lamotrignine |
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What are the iminostilbenes?
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carbamazepine
oxcarbezpine |
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List the drugs that work by allowing more Cl- to enter thus hyperpolarizing the neuron.
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Barbiturates
Benzodiazepines |
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What are the barbiturates?
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Phenobarbital
Primidone |
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What are the benzodiazepines?
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Diazepam
Clonazepam |
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What is primidone metabolized to?
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Phenobarbital and PEMA
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What drugs reduce the current through T-type Ca2+ channels in thalamic neurons?
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Succinimide (ethoximide)
Carboxylic acids |
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What drugs inhibit the metabolism of GABA?
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Vigabatrin
Gabapentine Tiagabine |
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What is a side effect of zonisamide and what is this drug used for?
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Antiseizure drug.
Also causes weight loss. |
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How does topiramate work?
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Blocks some Glu receptors
Enhances GABA activity Blocks sodium channels Also causes weight loss |
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What is a drug specific for absence seizures?
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Ethosuximide
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What is used to treat LGS seizures?
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Valproate
Benzos |
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What is the treatment for status epilepticus?
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Diazepam followed in 30min by phenytoin or fosphenytoin.
May need to use phenobarbital. |
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What antiseizure drug can cause skin rashes?
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Lamotrignine
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What drugs increase phenytoin metabolism?
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Phenobarbital
Carbamazepine |
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What drugs decrease phenytoin metabolism?
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Chloramphenicol
Dicumarol Cimetidine Sulfonamide Isoniazid |
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What drugs inhibit the metabolism of carbamazepine?
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Erythromycin
Isoniazid Propoxyphene |
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What are some unique S/E of phenytoin?
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Gingival hyperplasia
Hirsutism Megaloblastic anemia Inhibition of antidiuretic hormone release Birth defects: clefts! |
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What antiseizure drug causes spina bifida?
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Valproate
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What drugs do NOT work in absence seizures?
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Phenytoin
Carbamazepine |
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What antiseizure drugs are also used to treat mania?
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Carbamazepine
Oxcarbazepine Gabapentin Valproate Topiramate |
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What are the nondepolarizing neuromuscular blocking drugs?
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-iums
Tubocurarine Metocurine Gallamine |
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What is a depolarizing neuromuscular blocking drug?
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Succinylcholine
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What are some S/E of neuromuscular blocking drugs?
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Do not enter CNS-->no anesthesia
Depolarizing: fasciculations Nondepolarizing: release of histamine-->vasodilation, increased secretions |
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What is dantroline?
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Myotropic spasmolytic
Interferes w/Ca2+ release from sarcoplasmic reticulum |
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What are centrally acting spasmolytics?
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Baclofen
Diazepam |
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How does baclofen work?
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GABAb agonist
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What is tizanidine?
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Centrally acting spasmolytic
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How do local anesthetics work?
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Reversibly block conduction along axons by blocking Na+ channels
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What are the local anesthetics?
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-caines=esters
i before -caines=amides |
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How are esters metabolized?
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Plasma ChE
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How are amides metabolized?
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Liver
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What are S/E of local anesthetics?
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CNS effects-->agitation, confusion
Cardiovascular depression |
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What are the anticholinergic dilating agents?
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Atropine
Scopolamine Homatropine Cyclopentolate Tropicamide |
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What is an advantage of anticholinergics over adrenergic agonists as dilating agents?
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Anticholinergics dilate w/cycloplegia.
Cycloplegia paralyzes the ciliary muscle |
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How do beta blockers treat glaucoma?
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Decrease aqueous production.
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How do alpha-2 agonists treat glaucoma?
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Decrease aqueous production & increase aqueous outflow
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How do prostaglandins treat glaucoma?
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Increase uveoscleral outflow.
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How do carbonic anhydrase inhibitors treat glaucoma?
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Decrease aqueous production.
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What is an S/E of amiodarone?
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Corneal deposits
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What is an S/E of placquenil?
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Macular toxicity
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What is an ocular S/E of topiramate?
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Bilateral ACG
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What are ocular S/E of topical steroids?
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Increased IOP
Cataract Increased susceptibility to infection Activation of HSV Corneal perforation |
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What are cholinergics used for in the eye?
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OAG-rare
ACG Miosis Myesthenia |