Exam 2 Notes Flashcards
Acquired diseases are…
demyelinating disease, like MS
what makes up the defense of the CNS
Meninges
Blood-brain barrier
What are the three routes of infection
Direct implantation
Local spread
Hematogenous (most common)
Inherited diseases are
dysmyelinating, due to myelin synthesis (leukodystrophies)
What are the three most important factors in infection
Number of organisms
Host immune system
Type of organism
Acute infection process
Blood enters the brain, the immune system responds.
ends in either resolution or death
Chronic infection
third possible outcome infection, happens when acute infections worsens
What do viruses do, what type of infection is it an example of
provoke a lymphocytic response and may precede inflammation to myelin proteins causing demyelination
– found in Acute and chronic infection
what do Fungi cause, what type of infection is it an example of
granulomatous inflammation, found in chronic infection
What is the basic definition of MS?
demyelinating disorder in which episodes of neurologic deficits are separated by time.
Is MS a white or gray matter disorder?
White matter.
Name three symptoms of MS
(Varied): visual loss, paralysis, sensory loss, ataxia, brainstem signs, psychiatric disorders, dementia.
Name the classifications of MS
Benign MS
Relapsing remitting MS
Secondary chronic progressive
Primary progressive
What factor makes certain people more predisposed to MS?
Living in da cold
What is happening in Devic’s disease?
Within damaged white matter, there is necrosis. Also, many pts. show antibodies to aquaporins (used in astrocytic foot process and thus in integrity of BBB)
Bacteria causes what? what type of infection is it an example of?
cause purulent reactions with polymorphonuclear leukocytes and later necrosis – Acute infection
what permits chronic inflammation
Low-toxicity organisms
What cell is characteristic of a granuloma
Macrophages in the form of a giant cell
What responds to infectious organisms
astrocytes and microglia
Acute disseminated encephalomyelitis (ADEM) is most common in what group of people and how deadly is it?
Children. Favorable outcome if treated quickly.
Define Osmotic demyelination syndrome
Degeneration of a symmetric midline path of the basis pontis. Basically a severe loss of myelin and axons.
What causes Progrssive multifocal leukoencephalopathy (PML)?
JC virus, it causes infection of oligos
Define demyelinating
loss of myelin
Define Dysmyelinating
cannot form appropriate myelin
The little boy with discolored finger nails had what disease?
Adrenoleukodystrophy (ALD) AKA Addison’s
Name some neurologic manifestations of ALD
Normal until 3-8, progressive neurologic decline
Impaired auditory discrimination
Visual disturbances
What is one major pathological finding of Alexander’s disease?
Rosenthal fibers
What are some symptoms of infantile Krabbe’s disease
Normal for a few months, then irritability appears, along with spasticity, neurological regression and seizures
What are the cause of HIE in infants?
Placental abruption, cord accident, or cardiovascular stress during a difficult delivery.
What area of brain damage in term infants with HIE occurs?
border zone(watershed area) and basal ganglia and brainstem.
In mature infants, the basal ganglia, thalamus, and brainstem is affected more severely than other cortical region. Explain why.
they have higher energy demands and different neurotransmitters compared to the cortex
Periventricular leukomalacia cause what in low birth-weight and term infants?
Cerebral palsy
What pathology finding will be associated with PVL?
Ischemic white matter damage predominantly in the parieto-occipital lobes (watershed area).
What clinical signs are shown in degenerative diseases
progressive loss of neurologic function (dementia, loss of movement control), and pathologically: loss of neurons
What are the 6 common pathologic factors in neurodegenerative diseases
Deposition of fibrillar protein in matrix, Mitochondrial dysfunction, Excitotoxicity, Decreased proteasome activity, Decreased autophagy, and cell membrane alterations
Where are plaques found in Alzheimers
In deposits outside and around the neurons
What are Plaques
Beta amyloid fragments that clump together
What are Neurofibrillary tangles
paired helical filaments
What are Neurofibrillary tangles often referred to as and why
Ghost tangles, due to the death of the host neuron
What do Tau deposits cause
Impairment of axonal transport
What is the current opinion on how tau accumulates in Alzhiemers disease
Primary lesions are due to Aß deposition
Liquefaction, phagocytosis, cavitation, and gliosis are infacts-like process that evolve with which disease?
Periventricular leukomalacia (PVL)
What is the important cause of PVL?
Ischemia
Involving premyelinating oligodendrocyte that populate in the white matter
Clinical symptoms of PVL
Spastic diplegia or tetraplegia. Visual impairment, cognitive deficits, and seizure
Where does Germinal Matrix Hemorrhage start?
The germinal matrix is the layer under the ependymal lining of the ventricle. Hemorrhage starts between the thalamus and caudate
Why do patient surviving large grade IV hemorrhage often develop hydrocephalus?
Clots or gliosis of the aqueduct and subarachnoid space by clots and fibrous tissue and from obliteration of the foramina of Luschka.
Why does Kernicterus develop?
Inability of immature liver to conjugate billirubin, which crossess the BBB and enters neurons.
What pathological finding are in Status Marmoratus?
Marbled appearance of the thalamus and basal ganglia
What is the most common cause of Picks disease
Mutated tau protein
What clinical features are shown in picks disease
severe atrophy of frontal and temporal lobes
What are pick bodies
straight filaments and paired helical filaments
What are the 4 prominent protein storage diseases, and which proteins are associated with them
Parkinsons: synuclein
Huntingtons: Huntingtin
PSP: Tau
ALS: ubiquitin/TDP-43
What are some of the Parkinson symptoms
rigidity, small step gait, slow movement, tremors
What pathologically causes Parkinsons
alpha-synuclein is deposited in the neuronal body, forming Lewy bodies
what are the 5 possible ways to acquire parkinsonism
Drugs, vascular, toxins, infections, trauma
Describe dementia with Lewy bodies
Parkinsonism and dementia combined. causes cognitive deficits and hallucinations
What is Huntington disease
Degeneration of caudate, putamen, and the cortex
What is “anticipation” when dealing with Huntingtons
When the disease appears in younger ages and more severely
what is Progressive Supranuclear Palsy (PSP)
accumulation of tau and neuropil in pallidum, subthalamic nucleus, red nucleus, substantia nigra, oculomotor nucleus, medulla and dentate nucleus
What are the symptoms of PSP
postural instability and early falls, vertical gaze palsy, mild cognitive changes, progressive axial rigidity and bulbar palsy
What is ALS
Fatal degenerative disorder of upper and lower motor neurons
ALS clinical symptoms
affects Middle aged individuals, Muscle weakness, Spasticity Incontinence
What is Friedreich’s ataxia (FRDA)
Most frequent inherited ataxia, autosomal recessive disorder, begins usually before age 20 with ataxia
what is Neuromyelitis optica-NMO (Devic’s disease)
relapsing inflammatory demyelinative disease that causes transverse myelitis and optic neuritis
