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303 Cards in this Set
- Front
- Back
Atrophy of muscles of the face, ptosis, stiffness, difficulty in releasing hand grips, frontal balding, gonadal atrophy are frequent findings with this type of muscular dystrophy.
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Myotonic
|
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chromosome #5
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Spinal Muscular Atrophy
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Mutations involving voltage gated calcium channel
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hypocalemic
|
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Pseudohypertrophy of calf muscles
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Duchene
|
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Deficiencies involving mitochondrial dehydrogenase
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Lipid Myopathy
|
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blotchy red appearing muscle fibers in the form of ragged
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mitochondrial myopathies
|
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Rhabdomyolisis and accompanying myoglobinuria
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ethanol myopathy
|
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hyperphosphorylated tau protein aggregates are commonly found in the in the cytoplasm of myofibers of patients with?
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inclusion body myositis
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Rosenthal fibers are commonly seen in astrocytes of patients with
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Alexander Disease
|
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What astrocytes occur mainly in the gray matter of the brain?
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Protoplasmic
|
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Dorso-lateral aspect of the penis
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Pyronie Disease
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metabolic buffers, detoxifiers, suppliers of nutrients within the brain
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Astrocytes
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what type of herniation occurs with asymmetric expansion of of a cerebral hemisphere displaces the cingulate gyrus under the faulx cerebri
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Sublfacine
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Frequently arises in the mesentery or pelvic walls often in patients with Gardners' Syndrome.
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Intra-abdominal Desmoid Tumor
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Enlargement of a portion of of ventricular system due to a mass in the third ventricle will produce what type of hydrocephalus?
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Non-communicating
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Rupture of Charcot Bouchard Aneurysm is an important cause of this form of intra-cranial hemorrhage.
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Intra-Parenchymal
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These patients frequently have viral inclusions in the oligodendroglia cells
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Progressive multifocal leukoencephalopathy
JC Virus |
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This Fungus is frequently implicated in vasculitis within the central nervous system.
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Aspergillus Fumigatus
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muscle biopsy of the lesion in ______ type of of muscular dystrophy frequently shows a decrease of Dystrophin Molecules.
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Becker
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Frequently, patients with myasthenia gravis have abnormalities associated with___?
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Thymus Gland
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A translocation b/n chromosomes 2 and 13
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Alveolar Rhabdomyosarcomas
|
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Leads to increase in intracranial pressure
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All of the above:
1) Astrocytoma 2) Multiple cerebral ecchymotic hemorrhages 3) Cerebral Herniation |
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fast twitch muscle fibers
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Type II muscle fibers
|
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Female, 40, 0.5 cm soft tissue mass in left elbow, biphasic histological pattern consisting of cuboidal epithelial and spindle shaped mesenchymal cells
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Synovial Sarcomas
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Sustained involuntary contraction of a group of muscles is the cardinal symptom of this disease. patients complain of not being able to release their grip after a handshake
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Myotonic type of muscular dystrophy
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Cerebral infarcts associated with with ______ occlusions are considered to be hemorrhagic.
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Embolic
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The gene for prion protein (PRNP) is located on chromosome ____?
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20
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Female, 64, 0.5 cm mass, benign, and the most common kind of soft tissue tumor.
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Lipoma
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Parking lot inclusions
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Mitochondrial Myopathy
|
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Obliterative endocarditis, of meningeal blood vessels, and atrophy of the posterior columns of the spinal cord
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Tabes Dorsalis
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Non-productive infections of the CNS by a virus has been implicated in the genesis of:___?
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Subacute Sclerosing Panencephalitis
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Infection of CNS with ___ frequently leads to severe hemorrhagic necrotizing ventriculoencephalitis
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CMV
|
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the lesions in ________ are very common in the deep gray matter structures like the caudate and putamen.
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Creutzfeldt -Jacob - Disease
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rash like red patches on the knuckles, elbows, wrist, knees, and chest.
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Dermatomyositis
|
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What are the two types of secondary active transport?
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Cotransport/symport -- solutes move in same direction across the cm
Countertransport/antiport -- solutes move in opposite directions |
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Intra cellular vacuoles in neurons and glia cells are the most common histological finding in lesions associated with _____?
|
Prion Diseases
|
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which muscle fibers are high in myoglobulin and oxidative enzymes
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type 1
|
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The pathogenesis of this disease involves hypersensitivity type II reaction
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Myasthenia Gravis
|
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Papilledema is an important manifestation associated with __?
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Increased intracranial pressure
|
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Soft tissue tumors are often found at this location.
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Lower extremities, especially the thighs
|
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Pale or bland cerebral infarcts are usually associated with_____?
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Thrombotic occlusions
|
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the most common cause of acute Pyogenic (Bacterial) Meningitis among adolescents and young adults
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Neisseria Meningitis
|
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Area of Demyelination with destruction of White Matter in the brain are the most common finding in the brain of patients with?
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Progressive Multifocal Leukoencephalopathy
|
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The most common cause of primary brain intraparenchymal hemorrhage.
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Hypertension
|
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Epidural Hematomas are common due to traumatic lacerations or rupture involving what?
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Middle Meningeal Artery
|
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Giant Cell Tumor of the tendon sheath is commonly seen to occur at the
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wrists and fingers
|
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What is characterized by Myxoid Degeneration of the connective tissue?
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Ganglion
|
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The most common location where contusion injury occurs is ___________ in the CNS.
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The Frontal /Temporal lobe
|
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Often occurs in Adults on the Volar aspect of the arm.
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Nodular Fascitis
|
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In general, if the head is immobile,at the time of trauma, what type of injuries usually occur?
|
Coup Injuries
|
|
Tadpole or Strap Cells
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Rhabdomyosarcoma
|
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Papiledema is an important manifestation in what?
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Increased Intracranial pressure
|
|
Spindle Shaped Cells growing in a herringbone fashion.
|
Fibroma Sarcoma
|
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A degenerative Disease characterized by striking atrophy of the caudate nucleus
|
Huntington Disease
|
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Peyronie Disease is a type of?
|
Superficial Fibromatosis
|
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Mutations leading to abnormalities of the of the carnitine transport system.
|
Lipid Myopathies
|
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Cigar shaped nuclei.
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Leiomyoma
|
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Leiomyomas are common in?
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Sexually reproductive women
|
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Tumors of smooth muscle
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Leiomyoma - Benign
Leiomyosarcoma - |
|
Leiomyomas are most often seen in _____ and on the skin.
|
women
|
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painless and benign except for on the peritoneum
|
Leiomyoma
|
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5cm to 25cm in size
|
(Synovial Sarcoma)
Synovioma |
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Chromosomal translocation on #10 and #18
Malignant |
(Synovial Sarcoma)
Synovioma |
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Biphasic:
Epithelial and Spindle shaped cells |
(Synovial Sarcoma)
Synovioma |
|
Mutations leading to abnormalities of the carnitine transport system are involved in the pathogenesis of?
|
Lipid Myopathies
|
|
Mutations leading to abnormalities of the of the carnitine transport system.
|
Lipid Myopathies
|
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Cigar shaped nuclei.
|
Leiomyoma
|
|
Leiomyomas are common in?
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Sexually reproductive women
|
|
Tumors of smooth muscle
|
Leiomyoma - Benign
Leiomyosarcoma - malignant |
|
Leiomyomas are most often seen in _____ and on the skin
|
women
|
|
Spinal muscular atrophy AKA
|
Infantile motor neuron disease
|
|
Lesion in chromosome #5
|
Spinal muscular atrophy
|
|
Mutations leading to the abnormalities of the carnitine transport system are involved in the pathogenesis of
|
Lipid Myopathies
|
|
The phenomenon of Neurophalgia is associated with activities relating to ?
|
Microglial cells
|
|
leads to an increase in the intra cranial pressure
|
cerebral edema
cerebral herniation |
|
Dystrophin is needed in the function o?
|
striated muscle
|
|
Absence of Dystrophin
|
Duchene Muscular Dystrophy
|
|
Pelvic muscles first, then Shoulder and Calf
Atrophy/Pseudohypertrophy of Calf muscle |
Duchene Muscular Dystrophy
|
|
Locomotor Ataxia is an important finding in patients who have this form of Neuro
|
Tabes Dorsalis
|
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Reduced level of Dystrophin
|
Becker Muscular Dystrophy
|
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characterized by myotonia - a sustained involuntary contraction of a group of muscles.
|
Myotonic type of Muscular Dystrophy
|
|
What cells are considered to give rise to the rod cells seen in one of the forms of neurosyphilis?
|
Microglia
|
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Repeat expansion of CTG nucleotide on chromosome 19
|
Myotonic type of Muscular Dystrophy
|
|
O - Ring Appearance in the center of the muscle fiber
|
Myotonic type of Muscular Dystrophy
|
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atrophy of muscles of the face, ptosis, cataracts, balding in the front, decreased IgG in blood, testicular atrophy, abnormal glucose tolerance test, cardiomyopathy, and sometimes Dementia.
|
Myotonic type of Muscular Dystrophy
|
|
involved with mutations of the sarcoglycan complex disease
|
Myotonic type of Muscular Dystrophy
Lymph - girdle |
|
characterized by myotonia - a sustained involuntary contraction of a group of muscles.
|
Myotonic type of Muscular Dystrophy
|
|
What cells are considered to give rise to the rod cells seen in one of the forms of neurosyphilis?
|
Microglia
|
|
Repeat expansion of CTG nucleotide on chromosome 19
|
Myotonic type of Muscular Dystrophy.
Lymph - girdle |
|
O - Ring Appearance in the center of the muscle fiber
|
Myotonic type of Muscular Dystrophy
Lymph - girdle |
|
atrophy of muscles of the face, ptosis, cataracts, balding in the front, decreased IgG in blood, testicular atrophy, abnormal glucose tolerance test, cardiomyopathy, and sometimes Dementia.
|
Myotonic type of Muscular Dystrophy
Lymph - girdle |
|
itchy face
|
FascioScapuloHumeral Muscular Dystrophy
|
|
Necrosis of muscles
|
Myopathies
|
|
Inflammation of muscles
|
Myositis
|
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Inflammation and necrosis of muscles
|
Inflammatory Myopathies
|
|
Myopathies due to inborn errors of Metabolism
|
1) Lipid Myopathies
2) Mitochondrial Myopathy |
|
Derangement in the Carnitine transport system. Fatty acid cannot be broken down.
|
Lipid Myopathy
|
|
Deficiency of Mitochondrial dehydrogenase
|
Lipid Myopathy
|
|
Fribrillary Gliosis with few oligodendrocytes and no myelin are characteristic findings in the white matter of the of the white matter of the affected brain of patients with
|
multiple sclerosis
|
|
Stain for lipids
|
1) Oil Red O
2) Sudan Red Both produce a red/pink appearance |
|
Maternal problem 16-14 yrs of age affecting mitochondrial DNA.
Muscle fibers have a ragged red fiber appearance |
Oxidative phosphorylation disease
|
|
Parking lot inclusions
|
Oxidative phosphorylation disease
AKA Mitochondrial Myopathy |
|
Oxidative phosphorylation disease
AKA |
Mitochondrial Myopathy
|
|
fibrillary gliosis with few oligodendocytes and no myelin are characteristic findings in the white matter of the affected brain of patients with ?
|
Multiple Sclerosis
|
|
Putamen is most commonly involved with
|
Wilson's Disease
|
|
Sustained involuntary contraction of a group of muscles is the cardinal neuromuscular symptom of this disease. Difficulty in releasing their grip following a handshake
|
Myotonic type of Muscular Dystrophy
|
|
Voltage gated calcium channel affected
|
Hypokalemia
|
|
Emboli in the central nervous system most often occlude the
|
middle cerebral artery
|
|
Thyrotoxic Myopathy
|
Exophthalmic ophthalmoplegia
|
|
Ethanol Myopathy
|
myoblobinuria,
increased LDH ISO IV |
|
Hormone or Steroid myopathy
Type II muscle fibers affected |
Cushing's syndrome
|
|
Type I Muscles affected by anti malarial drug
|
Chloroquine
|
|
Necrosis and inflammation together
|
Inflammatory myopathies
|
|
most common soft tissue tumor of adulthood
|
Lipoma
|
|
spongiform encelphalopathy
|
Kuru
|
|
Symmetric weakness of proximal muscles starting at the shoulder and moving down
|
Polymyositis
|
|
Symmetric Lesions on the body skin
|
Dermatomyositis
|
|
Assymetric lesions frequently involving the extensors of the knee, and flexors of the wrist and fingers
|
Inclusion body myositis
|
|
Myositis most commonly seen in adults
|
Polymyositis
|
|
Myositis seen over 50yrs of age
and similar to alzeimer's disease |
Inclusion body
|
|
cd8 involved
|
Polymyositsi
|
|
CD4 involved
|
Dermatomyositis
|
|
25 % have Jo-1 antibody
|
Dermatomyositis
|
|
Myositis most commonly seen in adults
|
Polymyositis
|
|
Myositis seen over 50yrs of age
and similar to alzeimer's disease |
Inclusion body
|
|
cd8 involved
|
Polymyositsis and Inclusion body myositis
|
|
CD4 involved
|
Dermatomyositis
|
|
25 % have Jo-1 antibody
|
Dermatomyositis
|
|
Intracytoplasmic inclusion bodies termed Hirano Bodies
|
Alzheimer's disease
|
|
CD8+ Lymphocytes under the Endomycium
|
Polymyositis
|
|
A bacterium is involved in the etiology of
|
Tabes
Dorsalis |
|
Heliotropic discoloration of the upper eyelid and Dusky Red Pathes on the knuckles, wrist and knees
|
Dermatomyositis
|
|
Groton Lesions
|
Dusky red pathces on knuckle, wrist, and knees in Dermatomyositis
|
|
CD4 under the Perimycium
|
Dermatomyositis
|
|
Congestion and hemorrhage in knees, knuckles, and elbows
|
Dermatomyositis
|
|
Vacuoles in muscles. muscle cells have hyperphosphorylated tau protein and beta amyloid proteins
|
Inclusion body Myositis
|
|
Selective injury of neurons of type III and Type V of the cerebral cortex is common in the patients with acute
|
Carbon Monoxide toxicity
|
|
Males are often most often affected in
|
Becker and Duchene Muscular Dystrophy
|
|
inflammation only
|
Myositis
|
|
Clostridium
|
Gas Gangrene
|
|
It is the toxin that produces inflammation
|
Botulinum
|
|
Diaphragm
|
Trichinella
|
|
Bacteria
|
Neutrophils
|
|
Parasites and Allergies
|
Eosinophils
|
|
Virus
|
Lymphocytes
|
|
Spirochete which produces Weil Disease
|
Leptosporidum
|
|
5 signs of inflammation
|
Calor - Heat
Dolor - Pain Rubor - Redness Tumor - Swelling Functio Laesa - Loss of Function |
|
Diseases of the NMJ
more common in women |
Myasethenia Gravis
Lambert Eaton Myasthenic Syndrome |
|
Hyperplasia or tumor of Thymus
<40 |
Myasthenia Gravis
|
|
Ab attach to receptors
|
Myasthenia Gravis
|
|
Type II hypersensitivity Rxn
|
Myasthenia Gravis
|
|
Type I muscle fibers involved
|
Myasthenia Gravis
|
|
muscles of the face, ones that are in constant use
|
Myasthenia Gravis
|
|
How many neurons?
|
10 to the 11th
|
|
Red degenaration
eosinophilic appearance |
Neuronal death
|
|
Death of a neuron how many hours after ischemia?
|
12 hrs
|
|
Folding of neurons
|
inclusion bodies
|
|
micro glia come from?
|
bone marrow
|
|
Seen in White Matter
|
Fibrillary astrocytes
|
|
Seen in gray matter
|
Protoploasmic astrocytes
|
|
Glial Fibrillary Acidic Protein
|
attached to blood vessels by Sucker feet or foot processes
|
|
No Fibroblast in
|
the brain
|
|
replacement of neurons
|
Fibrillary Gliosis or just Gliosis
by Astrocytes |
|
whenever there is hypoxia, hypoglycemia or presence of toxic stimuli
|
Swelling of astrocytes
|
|
contain a brightly eosinophilic structure in its stem which is abnormal
Rediish/pinkish color |
Rosenthal fibers
|
|
Rosenthal fibers are seen ion two conditions
|
1) Long standing Gliosis
2) Pilocytic Astrocytoma |
|
Basophilic structures within the astrocytes stem and body
Buish - purple color |
Corporea Amylacea
|
|
Coporea Replaceable seen in what conditions?
|
1) Advancing age
2) Degenerative changes within the astrocytes |
|
AKA Polyglucosan Bodies
|
Corporea Amylacea
|
|
Alzheimer type II astrocytes are frequently seen in the _____ whenever there is a high level of ammonia e.g. Cirrhosis in Wilson's Disease?
|
Gray Matter
|
|
Mitochondria is not working right
|
citruline cycle stopped - urea is not produced. Ammonia instead
Arginine, citruline, |
|
Multiple internodes are produced by O
|
ligondendroglial cells
|
|
single nodes are produced by
|
Schwann cells
|
|
Sattelite cells gather around myelin sheath
|
Satelitosis
|
|
Ependymal cells lining Ventricular system
|
CMV
|
|
MPS
Look like Lymphocytes but do not have too many markers like lymphocytes |
Mononuclear Phagocytic System
|
|
Microglial cells replace dead neurons
|
Neuronophagia
|
|
In a state of Paretic form of neurosyphillis, Microglial cells also become
|
Rod Cells
|
|
Selective injury of neurons of type III and V of the Cerebral Cortex is common in patients with what type of acute toxicity/exposure?
|
Carbon monoxide
|
|
Males are mostly affected in what type of Muscular Dystrophy?
|
1) Duchene
2) Becker |
|
What type of Rhabdomyosarcoma occurs in individuals who are 10 - 25 years of age?
|
Alveolar
|
|
which muscles are most often involved with myasthenia gravis?
|
Muscles involved in eye movement
|
|
Cavernous angiomas are found in
|
the cerebellum
|
|
Arteriovenous
|
Tortuous
|
|
Capillary elangiectasis are seen in the
|
Pons
|
|
Angiomas are
|
Stenosis of capillaries
|
|
inflammation of CNS is primaqrily from
|
infection
|
|
inflammation of meninges
|
menangitis
|
|
inflammmation of brain
|
encephalitis
|
|
inflammation of meninges and brain
|
meningioencephalitis
|
|
encephalomyelitis
|
inflammation of brain and spinal cord
|
|
meningioencephalomyelitis
|
inflammation of the brain, meninges, and spinal cord
|
|
bacterial infections to the brain, meninges, and spinal cord are primarily
|
hematogenous
|
|
acute Meningitis
(Septic, Pyogenic, aseptic [virus]) |
due to bacteria or virus
|
|
meningeal infections always have
|
bacteria or virus in the csf
|
|
CSF is always cloudy with decrease in glucose and lots of protein and PMN
|
Bacterial infection
|
|
CSF has SOME cloudiness with normal protein and lots of Lymphocytes
|
Viral infection
|
|
csf infectionsvery young and very old
|
neisseria monocytogenes
Streptococcus |
|
localized bacterial growth
|
localized abcess formation
|
|
klebsiella?
|
neisseria?
|
|
Fibrosis always leads to hydrocephalus
|
fibrosis of the meninges
|
|
acute viral meningitis
|
enterovirus
coxsackie echovirus non paralytic form of polio |
|
acute focal supporrative
|
Staph or Streptococcus
|
|
frontal lobe most often affected
|
brain abcesses
|
|
Seen in Tuberculosis with
|
Obliterative Enarteritis
|
|
Calcification and hydrocephalus therefore Increased intracranial pressure
|
Tuberculosis
|
|
cupping of the lymphocytes
|
Syphilis
|
|
3 stages
neurological state in stage 3 |
Syphilis
|
|
Neurological Syphilis takes how many years
|
3-5
|
|
3 stages of Syphilis in the Brain:
|
1) Meningovascular
2) Paretic 3) Locomotor Ataxia |
|
Seen in temporal and frontal lobes and also in the base of the brain
|
Meningovascular
|
|
Thickening of the meninges, paravascular cupping in the form of Obliterative Endarteritis aka: Heubner Arteritis common
|
Meningovascular
|
|
Gumma formation is seen in the meninges
|
Meningovascular
|
|
Dementia Sets in
|
Paretic form
|
|
Few Neurons Left, Gliosis has taken place
|
Windblown cortex or appearance in Paretic Syphilis
|
|
Microglial cells produce what in Paretic Syphilis
|
Rod Cells in Paretic Syphilis
|
|
Atrophy of the posterior column of the Dorsal roots
|
Tabes Dorsalis - 3rd stage of Neurosyphyilis
|
|
Pupil Does Not Dilate
|
Argyle Robertson Pupils - Tabes Dorsalis
|
|
Charcot Joints
|
Tabes Dorsalis
|
|
3 stages of Lyme disease, Neurological component in stage?
|
2
|
|
Borrelia Burgdorferri
|
Lyme Disease
|
|
Bullseye Lesions
|
Lyme Disease
|
|
Lymphocytes are commonly seen in what type of infections?
|
Viral
|
|
Inclusion bodies are also seen in?
|
Viral Infections
|
|
Perivascular Cuffing
|
Tabes Dorsalis
|
|
Arbovirus Infection
|
Arthropod Borne infections
(Western Nile, Western Equine, WEE, Lymphocytic Polio Virus etc. |
|
Are Arbovirus' DNA or RNA?
|
RNA
|
|
Always have necrosis of the red neurons, Neuronophagia, Gliosis,
Clinical: Confusion, Delerium, Headache, Stupor, Coma... |
Arbovirus
|
|
affects Frontal and Temporal Lobes
Children and young adults |
Herpes Simplex type I
|
|
Inclusions in the nucleus of the affected neurons in Herpes simplex type I
|
Cowdry Bodies
|
|
Meningitis,
60% - 70% on neonates will be infected. Eye Lesions and Herpetic Encephalitis Inclusions not common |
Herpes Type II
|
|
Immunodeficient patients often end up having
|
Acute Hemorrhagic Encephalitis
|
|
Cytomegalovirus is what kind of Virus?
|
DNA
|
|
Common in children, extremely common in HIV patients
|
CMV
|
|
Produces Necrosis around the ventricles and affects the empendymal cells of the ventricles. Produces acute hemorrhagic necrotizing ventriculoencephalitis.
|
CMV
|
|
Inclusions are intracytoplasmic and in the nucleus
|
CMV
|
|
Polio virus is what kind of virus
|
Retrovirus (RNA)
|
|
frequently seen in the anterior horn cells of the motor neurons
|
Polio
|
|
Motor Cranial nuclei are also affected
|
Poliuo
|
|
Photophobia
|
Rabies
|
|
Oligodendrioglial Cells are commonly found in the?
|
White matter
|
|
(PML)
|
Progressive Multifocal Leukoencephalopathy
|
|
JC Virus is associated with?
|
PML
|
|
SSPE
|
Subacute Sclerosing Panencephalovirus
|
|
SSPE is Associated with patients who have had?
|
Measles Virus
|
|
Virus intranuclear inclusions are seen
|
SSPE
|
|
Abnormal hand movements of the hands and feet, coma, death
|
SSPE
|
|
Nonproductive infection involved with measles.
antivirus does not get produced |
SSPE
|
|
Fungus Media
|
Saboror's agar
|
|
Fungal agents involved in the brain
|
1) Candida Albicans
2) Mucor 3) Aspergillus Fumigatus 4) Cryptococus Neoformans 5) Histoplasma Capsulatum 6) Blastomyces Dermatidis |
|
Chronic Meningitis
|
Cryptococcus Neoformans
|
|
Vasculitis
|
1) Mucor and
2) Aspergillus Fumigatus |
|
Parenchymal Invasion
No Inflammation |
Cryptococus Neoformans
|
|
Fungus frequently seen in HIV
|
Cryptococus Neoformans
|
|
The Phenomenon of Satellitosis around the affected neurons is seen due to the accumulation of these cells.
|
Oligodendroglia
|
|
This illness is preceded by a recent episode of upper respiratory or Mycoplasma infection
|
Acute Necrotizing Hemorrhagic Encephalomyelitis
|
|
Cardiac Abnormalities are frequent in patients with?
|
Friedreich Ataxia
|
|
Glial cytoplasmic inclusions typically within the cytoplasm of Oligodendrocytes are common in patients with
|
Multiple System trophy
|
|
The CSF pressure in Pick Disease is?
|
Normal
|
|
Loss of Both Upper and Lower Motor Neurons
|
Amyotrophic Lateral Sclerosis
|
|
the nigro striatal dopaminergic system is selecteively involved.
|
Parkinson's Disease
|
|
Argyll Robertson Pupil
|
Tabes Dorsalis
|
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Degeneration of the lower brain stem cranial motor nuclei
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Progressive Bulbar Palsy
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Manifestations of Memory Disturbances and confabulation are associated in this Thiamine Deficient Patient.
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Korsakoff Syndrome
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Maintenance of the blood brain barrier
controlling ion transport and balance in the CNS |
Astrocytes
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Fungas frequently involved in the genesis of Parenchymal Invasion and injuries to the CNS
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Cryptococcus Neoformans
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Intra cytoplasmic inclusion bodies in ALS
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Bunina Bodies
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Lou Gherig's Disease
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ALS
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ALS
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Amyptrophic Lateral Sclerosis
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Degeneration of Lower Brain stem cranial motor nuclei
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Progressive Bulbar
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Deglutation
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Problems Swallowing
Profressive Bulbar Palsy |
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Kennedy's Syndrome
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Bulbo Spinal Atrophy
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Sex Linked
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Females transmit
Males Have |
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Distal Limb myoatrophy
Fasiculation in muscles of tongue Degeneration of Lower Brain Stem and Spinal cord |
Kennedy's Syndrome
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CAG Repeats
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Kennedy's Syndrome
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Androgen receptor gene involved
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Kennedy's Syndrome
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Female Features
Gynecomastia |
Kennedy's Syndrome
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Genetic Metabolic diseases
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1) Leukodystrophies
2) |
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Leukodystrophies
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1) Krabbe Dystrophy
2) Metachromatic Leukodystrophy 3) Adrenoleukodystrophy 4) Pelzaes Mersbacher Disease 5) Canavan Disease |
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Galactosphingosine damages oligodendrioglial cells affecting motor neurons with little myeilin production. death 3-6 months after birth
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Krabbe disease
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Chromosome #14
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Krabbe Diseasse
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Chromosome # 22
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Metachromatic Leukodystrophy
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Sulfatides Accumulate
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Metachromatic Leukodystrophy
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Stain for Sulfatides
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Toluidine Blue
Metachromatic appearance |
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Myelin loss in both peripheral and central nervous system
inability to break down VLCFA ( nvery long chain fatty acids) |
Adrenoleukodystrophy
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Protein Proteolipid - myelin prod stopped
"Tigroid Appearance" |
Pelzaes Merzbacher disease
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Chromosome # 17
Decreased aspartoacyclase |
Canavan Disease
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Subacute necrotizing encephalopathy
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Mitochondrial Leigh Syndrome
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decrease in Cytochrome C oxidase
Midbrain Symmetric destruction of the midbrain |
Mitochondrial Leigh Syndrome
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Lactic Acidemia
Death 1 - 2 years |
Mitochondrial Leigh Syndrome
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Acquired Metabolic Diseases
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Hypoglycemia
Hyperglycemia B1 Deficiency B12 Deficiency Hepatic Encelipathy |
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Thiamine
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B1
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B1 Deficiency
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Wernicke - Korsokoff Syndrome
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Memory and Confabulation
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Korsokoff
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Psuchotic episodes
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Wernicke
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Any disease of the stomach can produce
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B1 deficiency
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Bulimics
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Wernicke Korsokoff
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Lesions in the
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Wernicke Korsokoff
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Parietal cells produce
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intrinsic factor
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Myelin production decreased
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Subacute combined degen of spinal cord
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Vacuolar myelopathy is similar to
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B12 Deficiency
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B12 Deficiency always have
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Methylmalonic acid in urine
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I. Resting tremor
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Þ Parkinson’s
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