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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.
Myotonic
chromosome #5
Spinal Muscular Atrophy
Mutations involving voltage gated calcium channel
hypocalemic
Pseudohypertrophy of calf muscles
Duchene
Deficiencies involving mitochondrial dehydrogenase
Lipid Myopathy
blotchy red appearing muscle fibers in the form of ragged
mitochondrial myopathies
Rhabdomyolisis and accompanying myoglobinuria
ethanol myopathy
hyperphosphorylated tau protein aggregates are commonly found in the in the cytoplasm of myofibers of patients with?
inclusion body myositis
Rosenthal fibers are commonly seen in astrocytes of patients with
Alexander Disease
What astrocytes occur mainly in the gray matter of the brain?
Protoplasmic
Dorso-lateral aspect of the penis
Pyronie Disease
metabolic buffers, detoxifiers, suppliers of nutrients within the brain
Astrocytes
what type of herniation occurs with asymmetric expansion of of a cerebral hemisphere displaces the cingulate gyrus under the faulx cerebri
Sublfacine
Frequently arises in the mesentery or pelvic walls often in patients with Gardners' Syndrome.
Intra-abdominal Desmoid Tumor
Enlargement of a portion of of ventricular system due to a mass in the third ventricle will produce what type of hydrocephalus?
Non-communicating
Rupture of Charcot Bouchard Aneurysm is an important cause of this form of intra-cranial hemorrhage.
Intra-Parenchymal
These patients frequently have viral inclusions in the oligodendroglia cells
Progressive multifocal leukoencephalopathy

JC Virus
This Fungus is frequently implicated in vasculitis within the central nervous system.
Aspergillus Fumigatus
muscle biopsy of the lesion in ______ type of of muscular dystrophy frequently shows a decrease of Dystrophin Molecules.
Becker
Frequently, patients with myasthenia gravis have abnormalities associated with___?
Thymus Gland
A translocation b/n chromosomes 2 and 13
Alveolar Rhabdomyosarcomas
Leads to increase in intracranial pressure
All of the above:
1) Astrocytoma
2) Multiple cerebral ecchymotic hemorrhages
3) Cerebral Herniation
fast twitch muscle fibers
Type II muscle fibers
Female, 40, 0.5 cm soft tissue mass in left elbow, biphasic histological pattern consisting of cuboidal epithelial and spindle shaped mesenchymal cells
Synovial Sarcomas
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
Myotonic type of muscular dystrophy
Cerebral infarcts associated with with ______ occlusions are considered to be hemorrhagic.
Embolic
The gene for prion protein (PRNP) is located on chromosome ____?
20
Female, 64, 0.5 cm mass, benign, and the most common kind of soft tissue tumor.
Lipoma
Parking lot inclusions
Mitochondrial Myopathy
Obliterative endocarditis, of meningeal blood vessels, and atrophy of the posterior columns of the spinal cord
Tabes Dorsalis
Non-productive infections of the CNS by a virus has been implicated in the genesis of:___?
Subacute Sclerosing Panencephalitis
Infection of CNS with ___ frequently leads to severe hemorrhagic necrotizing ventriculoencephalitis
CMV
the lesions in ________ are very common in the deep gray matter structures like the caudate and putamen.
Creutzfeldt -Jacob - Disease
rash like red patches on the knuckles, elbows, wrist, knees, and chest.
Dermatomyositis
What are the two types of secondary active transport?
Cotransport/symport -- solutes move in same direction across the cm
Countertransport/antiport -- solutes move in opposite directions
Intra cellular vacuoles in neurons and glia cells are the most common histological finding in lesions associated with _____?
Prion Diseases
which muscle fibers are high in myoglobulin and oxidative enzymes
type 1
The pathogenesis of this disease involves hypersensitivity type II reaction
Myasthenia Gravis
Papilledema is an important manifestation associated with __?
Increased intracranial pressure
Soft tissue tumors are often found at this location.
Lower extremities, especially the thighs
Pale or bland cerebral infarcts are usually associated with_____?
Thrombotic occlusions
the most common cause of acute Pyogenic (Bacterial) Meningitis among adolescents and young adults
Neisseria Meningitis
Area of Demyelination with destruction of White Matter in the brain are the most common finding in the brain of patients with?
Progressive Multifocal Leukoencephalopathy
The most common cause of primary brain intraparenchymal hemorrhage.
Hypertension
Epidural Hematomas are common due to traumatic lacerations or rupture involving what?
Middle Meningeal Artery
Giant Cell Tumor of the tendon sheath is commonly seen to occur at the
wrists and fingers
What is characterized by Myxoid Degeneration of the connective tissue?
Ganglion
The most common location where contusion injury occurs is ___________ in the CNS.
The Frontal /Temporal lobe
Often occurs in Adults on the Volar aspect of the arm.
Nodular Fascitis
In general, if the head is immobile,at the time of trauma, what type of injuries usually occur?
Coup Injuries
Tadpole or Strap Cells
Rhabdomyosarcoma
Papiledema is an important manifestation in what?
Increased Intracranial pressure
Spindle Shaped Cells growing in a herringbone fashion.
Fibroma Sarcoma
A degenerative Disease characterized by striking atrophy of the caudate nucleus
Huntington Disease
Peyronie Disease is a type of?
Superficial Fibromatosis
Mutations leading to abnormalities of the of the carnitine transport system.
Lipid Myopathies
Cigar shaped nuclei.
Leiomyoma
Leiomyomas are common in?
Sexually reproductive women
Tumors of smooth muscle
Leiomyoma - Benign

Leiomyosarcoma -
Leiomyomas are most often seen in _____ and on the skin.
women
painless and benign except for on the peritoneum
Leiomyoma
5cm to 25cm in size
(Synovial Sarcoma)

Synovioma
Chromosomal translocation on #10 and #18

Malignant
(Synovial Sarcoma)

Synovioma
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
Cigar shaped nuclei.
Leiomyoma
Leiomyomas are common in?
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
Reduced level of Dystrophin
Becker Muscular Dystrophy
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
O - Ring Appearance in the center of the muscle fiber
Myotonic type of Muscular Dystrophy
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
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
Degeneration of the lower brain stem cranial motor nuclei
Progressive Bulbar Palsy
Manifestations of Memory Disturbances and confabulation are associated in this Thiamine Deficient Patient.
Korsakoff Syndrome
Maintenance of the blood brain barrier

controlling ion transport and balance in the CNS
Astrocytes
Fungas frequently involved in the genesis of Parenchymal Invasion and injuries to the CNS
Cryptococcus Neoformans
Intra cytoplasmic inclusion bodies in ALS
Bunina Bodies
Lou Gherig's Disease
ALS
ALS
Amyptrophic Lateral Sclerosis
Degeneration of Lower Brain stem cranial motor nuclei
Progressive Bulbar
Deglutation
Problems Swallowing

Profressive Bulbar Palsy
Kennedy's Syndrome
Bulbo Spinal Atrophy
Sex Linked
Females transmit

Males Have
Distal Limb myoatrophy

Fasiculation in muscles of tongue

Degeneration of Lower Brain Stem and Spinal cord
Kennedy's Syndrome
CAG Repeats
Kennedy's Syndrome
Androgen receptor gene involved
Kennedy's Syndrome
Female Features

Gynecomastia
Kennedy's Syndrome
Genetic Metabolic diseases
1) Leukodystrophies
2)
Leukodystrophies
1) Krabbe Dystrophy
2) Metachromatic Leukodystrophy
3) Adrenoleukodystrophy
4) Pelzaes Mersbacher Disease
5) Canavan Disease
Galactosphingosine damages oligodendrioglial cells affecting motor neurons with little myeilin production. death 3-6 months after birth
Krabbe disease
Chromosome #14
Krabbe Diseasse
Chromosome # 22
Metachromatic Leukodystrophy
Sulfatides Accumulate
Metachromatic Leukodystrophy
Stain for Sulfatides
Toluidine Blue

Metachromatic appearance
Myelin loss in both peripheral and central nervous system

inability to break down VLCFA ( nvery long chain fatty acids)
Adrenoleukodystrophy
Protein Proteolipid - myelin prod stopped

"Tigroid Appearance"
Pelzaes Merzbacher disease
Chromosome # 17

Decreased aspartoacyclase
Canavan Disease
Subacute necrotizing encephalopathy
Mitochondrial Leigh Syndrome
decrease in Cytochrome C oxidase

Midbrain

Symmetric destruction of the midbrain
Mitochondrial Leigh Syndrome
Lactic Acidemia

Death 1 - 2 years
Mitochondrial Leigh Syndrome
Acquired Metabolic Diseases
Hypoglycemia
Hyperglycemia
B1 Deficiency
B12 Deficiency
Hepatic Encelipathy
Thiamine
B1
B1 Deficiency
Wernicke - Korsokoff Syndrome
Memory and Confabulation
Korsokoff
Psuchotic episodes
Wernicke
Any disease of the stomach can produce
B1 deficiency
Bulimics
Wernicke Korsokoff
Lesions in the
Wernicke Korsokoff
Parietal cells produce
intrinsic factor
Myelin production decreased
Subacute combined degen of spinal cord
Vacuolar myelopathy is similar to
B12 Deficiency
B12 Deficiency always have
Methylmalonic acid in urine
I. Resting tremor
Þ Parkinson’s