Reading...
Play button
Play button
Use LEFT and RIGHT arrow keys to navigate between flashcards;
Use UP and DOWN arrow keys to flip the card;
H to show hint;
A reads text to speech;
114 Cards in this Set
- Front
- Back
|
Contents of CNS myelin
|
Proteolipid protein (PLP)
Myelin basic protein (MBP) Myelin associated glycoprotein (MAG) |
|
|
Contents of PNS myelin
|
Myelin associated glycoprotein (MAG)
Protein Po No PLP like in the CNS |
|
|
Myelin is essentially compacted....
|
Plasma membrane
|
|
|
Number of internodes made by an oligodendrocyte in the CNS
|
30-50
|
|
|
Effect of demyelination on nerve
|
Conduction delay or block
Increased refractory time Hyperexcitability Spontaneous depolarization |
|
|
Name three immune mediated myelin disorders
|
Multiple Sclerosis (MS)
Acute disseminated encephalomyelitis (ADEM) Acute hemorragic leukoencephalitis |
|
|
Signs and symptoms of MS
|
Muscle weakness
Spasticity UMN signs Sensory Loss Ataxia Tremor Nystagmus Vision Loss Bladder/bowel dysfunction Fatigue |
|
|
Typical CSF findings in MS
|
Normal or slightly elevated white count
Mildly elevated protein (<100mg/dL) Oligoclonal bands (IGg) Myelin basic protein |
|
|
Four possible courses of MS
|
Benign MS
Relapsing remitting MS Primary progressive (Bad) Secondary progressive (stairstep) |
|
|
Chronic MS (Charcot's variant)
|
Periventricular plaques
Demyelination Astrocytosis |
|
|
Acute MS (Marburg variant)
|
"Tumefactive" MS
Mass effect exerted by inflammation |
|
|
Neuromyelitis optica
|
Variant of MS
Also called "Devic's disease" Optic neuritis Transverse myelitis |
|
|
Hyperacute form of ADEM
|
Acute hemorragic leukoencephalitis
Usually fatal Small vessel necrosis Hemorrage |
|
|
Progressive multifocal leukoencephalopathy (PML) is caused by...
|
JC virus
Associated with immunocompromise and is common in AIDS patients |
|
|
Subacute sclerosing panencephalitis (SSPE) is associated with what disese?
|
Measles
|
|
|
What is the biochemical pathogenesis seen in B12 deficiency?
|
No SAM (only methyl donor in CNS)
Unmethylated myelin causes subacute combined degeneration of the spinal cord (SCD) |
|
|
Elevated methylmalonic acid and homocysteine are indicative of...
|
a B12 deficiency
|
|
|
Rapid correction of hyponatremia can lead to...
|
Central pontine myelinolysis
(quadriparesis, locked in syndrome etc.) |
|
|
Central pontine myelinolysis
|
Quadriparesis
Pseudobulbar palsy Locked in syndrome |
|
|
Vascular myelin diseases
|
Arteriosclerosis
CADASIL |
|
|
Acquired cognitive impairment across multiple domains of cognitive function with no impairment of arousal.
|
Dementia
|
|
|
Locus of genes associated with Alzheimers
|
21 (Amyloid precursor protein gene)
14 (presenilin-1 gene) 2 (presenilin-2 gene) All are autosomal dominant mutations but are uncommon |
|
|
Pathological findings of Alzheimers
|
Neurofibrillary tangles
Neuritic plaques Hirano bodies Amyloid angiopathy |
|
|
CERAD score
|
Consortium to Establish Registry for Alzheimers Disease
0-A-B-C (C is bad) |
|
|
What is the disease known as FTDP-17?
|
Tauopathy (17q21-22)
Frontotemporal dementia Parkinsonism |
|
|
Picks disease
|
Tauopathy
Anterior limbic areas damaged Knife edge sparing of posterior 2/3 of superior temporal gyrus |
|
|
Progressive supranuclear palsy (PSP)
|
Tauopathy
|
|
|
What type of disorder is Postencephalitic parkinsonism?
|
Tauopathy
|
|
|
Parkinsonism-ALS-Dementia complex of Guam
|
Tauopathy
|
|
|
Diagnostic criteria for dementia with Lewy bodies
|
one=possible two=probable
Fluctuating cognition with pronounced variation in attention and alertness Recurrent visual hallucinations Spontaneous motor features of parkinsonism |
|
|
HIV enecephalopathy
|
Damage to white matter, diencephalon and brainstem
Microglial nodules near blood vessels (macrophages, some multinucleated) |
|
|
Homozygosity for methionine on chromosome 20 codon 129 (PrP gene) increases susceptibility to....
|
Crutzfield-Jacobs disease
|
|
|
Bilateral pulvinar high signal on MRI
|
vCJD
|
|
|
Conformational change of PrP protein in Crutzfield Jacobs disease
|
Alpha helix mutates to beta pleated sheet
Can facilitate conformational change in other normal PrP molecules |
|
|
Radiological change indicative of arteriosclerosis
|
leukoaraiosis (white matter change)
|
|
|
Nutritional deficiencies that can cause dementia
|
B1 (thiamine)
B3 (Niacin)-pellagra B6 B12 Starvation |
|
|
An amnestic syndrome that damages the medial dorsal nucleus of the thalamaus
|
Korsakoff's syndrome
|
|
|
A type of dementia characterized by early memory loss
|
Alzheimers dementia
|
|
|
Signs of dementia with no early memory loss
Tau protein With or without pick changes |
Frontotemporal dementia
|
|
|
Cognitive changes with no early memory loss.
Lewy bodies |
Dementia with Lewy Bodies
|
|
|
Lisinopril HCTZ
|
HCTZ plus ACE inhibitor combo
|
|
|
Aggrenox
|
ASA and Dipyridamole combination
|
|
|
Clopidogrel (Plavix)
|
Irreversibly inhibits ADP induced exposure of platelet fibrinogen binding sites.
|
|
|
Risk of reocculusion of a carotid artery after endarterectomy in continued smokers.
|
9.8x (10x)
|
|
|
Third leading cause of death in the U.S
|
Stroke
|
|
|
Which is the only primary CNS tumor that is more common in women than men?
|
Meningioma
|
|
|
Which heritable conditions are associated with an increased incidence of CNS tumors?
|
Neurofibromatosis type 1
Neurofibromatosis type 2 Von Hippel-Lindau disease Li-Fraumeni syndrome Tuberous sclerosis Cowden Turcot Naevoid basal cell carcinoma syndrome |
|
|
Radiation induced tumors include...
|
Meningiomas
Sarcomas Gliomas |
|
|
Lymphomas are associated with the pathogen....
|
EBV
|
|
|
What is the most common source of metastatic brain cancer?
|
Lung cancer
|
|
|
Which CNS tumors are prone to Hemorrage?
|
Glioblastoma
Oligodendroglioma PNET Metastatic brain cancer |
|
|
The WHO tumor grading sytem is an important prognostic factor and is based on....
|
The histological appearance of the tumor.
Grade 1 is good Grade 4 is bad |
|
|
The TNM staging sytem which is NOT used for CNS tumors is based on the extent of the disease and takes into account....
|
the Tumor
the Nodes and the distant Metastases |
|
|
Subpial spread is a characteristic of ...
|
High grade tumors
|
|
|
Th most common CNS tumor in children, medulloblastoma , spreads by.....
|
Getting in the CSF and disseminating in the Subarachnoid space
|
|
|
What is the WHO grade of Glioblastoma Multiforme and what type of tumor is it?
|
Grade IV astrocytoma
|
|
|
Primary CNS lymphoma, despite its name, arises from which type of cells...
|
Microglia
|
|
|
Medulloblastoma, despite its name, arises from...
|
Embryonal cells in the cerebellum
|
|
|
Extra-axial with refernce to a CNS tumor means...
|
Outside the brain parenchyma
|
|
|
Supratentorial tumors tend to be more prevalent in ...whereas infratentorial tumors tend to be more prevalent in...
|
Adults
Children |
|
|
The most common PRIMARY brain tumor in adults is...
|
Glioblastoma
|
|
|
The presence of necrosis or microvascular occlusion rules out a diagnosis of.....
|
diffuse astrocytoma
|
|
|
A diffusely infiltrating intraxial tumor which does not enhance on imaging studies is most likely a...
|
Grade 2 astrocytoma
|
|
|
An astrocytoma which has the features of hypercellularity and nuclear atypia goes from a grade 2 to a grade 3 anaplastic astrocytoma when we see....
|
Marked mitotic activity
and Enhancement on CT |
|
|
Poorly differentiated, pleomorphic astrocytic tumor cells with marked nuclear atypia, brisk mitotic activity and prominent vascular proliferation and/or necrosis are diagnostic of....
|
Glioblastoma Multiforme (grade IV)
|
|
|
The most common PRIMARY brain tumor in adults is...
|
Glioblastoma Multiforme
|
|
|
The presence of necrosis or microvascular occlusion rules out a diagnosis of.....
|
diffuse astrocytoma
|
|
|
A diffusely infiltrating intraxial tumor which does not enhance on imaging studies is most likely a...
|
Grade 2 astrocytoma
|
|
|
An astrocytoma which has the features of hypercellularity and nuclear atypia goes from a grade 2 to a grade 3 anaplastic astrocytoma when we see....
|
Marked mitotic activity
and Enhancement on CT |
|
|
Poorly differentiated, pleomorphic astrocytic tumor cells with marked nuclear atypia, brisk mitotic activity and prominent vascular proliferation and/or necrosis are diagnostic of....
|
Glioblastoma Multiforme (grade IV)
|
|
|
A secondary glioblastoma multiforme is one that has...
|
evolved from a lower grade tumor.
|
|
|
A mutation on 19p makes it more likely that....
|
A low grade Astrocytoma will progress into a secondary Glioblastoma.
|
|
|
A mutation or methylation silencing on 10q is...
|
a positive prognostic factor because it makes the likelihood of Temozolomide working higher.
|
|
|
The prognostic factors for Astrocytoma are...
|
Grade
Age (younger =better) Karnofsky score Location Treatment |
|
|
The standard of care treatment for Glioblastoma is...
|
Temozolomide
|
|
|
A white matter Oligodendroglioma is considered a grade...tumor and is characterized by chicken wire capillaries and fried egg appearance cells.
It can however progress to a .... |
WHO Grade II
WHO Grade III anaplastic oligodendroglioma. |
|
|
In the context of oligodendroglioma, mutations on 1p and 19 q mean...
|
increased sensitivity to chemotherapy with PCV
|
|
|
Meningiomas are highly associated with the genetic condition....
|
Neurofibromatosis type 2
|
|
|
Menigioma grades range from...
|
WHO grade I (benign)- WHO grade three (anaplastic meningioma)
|
|
|
WHO grade II (atypical) menigioma subtypes are....
|
Clear cell meningioma
Chordoid meningioma |
|
|
WHO grade III (anaplastic) menigiomas are...
|
Rhabdoid Meningioma
Papillary Meningioma |
|
|
Multiple periventricular tumors in an eledrly or AIDS patient are most likely...
|
Primary CNS Intermediate-to-high grade non-Hodgkin’s lymphomas, B-cell type.
Steroids should be withheld till biopsy. |
|
|
A cerebellar tumor in a middle aged patient with von Hippel-Lindau disease is most likely...
|
Hemangioblastoma, a vascular tumor arising from foamy stromal cells.
|
|
|
A finding of Rosenthal fibers in a child's cerebellum would suggest....
|
Juvenile pilocytic astrocytoma
The most common Glioma in children. Often cured by surgical excision alone (WHO grade I). |
|
|
A tumor in a child's fourth ventricle is most likely....
|
Ependymoma.
It may eventually disseminate via the CSF. |
|
|
A tumor with Homer Wright (neuroblastic) rosettes, densely packed cells with round to oval or carrot-shaped nuclei and scanty cytoplasm in a child's cerebellum is most likely....
|
Medulloblastoma (WHO grade IV)
Can spread via the CSF. |
|
|
An epithelial tumor derived from Rathke’s cleft epithelium in a child age 5-14 that presents with Symptoms related to compression of the optic chiasm, pituitary gland, and 3rd ventricle is most likely.
|
Benign Craniopharyngioma (WHO grade I)
Can cause increased ICP Prognosis depends on surgical resectability. |
|
|
in the spinal cord, Extramedullary refers to something that is located...
|
Between the dura and the cord.
Medullary is the spinal cord equivalent of Axial. |
|
|
Spinal cord origin pain that is worse at night and better with ambulation is most likely...
|
Spinal cord tumor rather than a herniated disc.
|
|
|
Facial numbness (C2-C3) lesion has a high likelihood of being..
|
A spinal cord tumor.
Bowel and Bladder dysfunction can also be a sign of spinal cord tumor. |
|
|
The main categories of intradural-extramedullary tumors are...
|
Meningioma
Schwannoma Neurofibroma |
|
|
an intradural – intramedullary tumor in the cervical or upper thoracic region is most likely....
|
Astrocytoma
|
|
|
An intradural-intramedullary tumor in the lower thoracic or lumbar area is most likely....
|
Ependymoma
|
|
|
Schwannoma is...
|
Proliferation of Schwann cells that cann sometimes be dissected from the cell.
|
|
|
Neurofibroma is....
|
Proliferation of Schwann cells, fibroblasts, and perineurial cells that is intrinsic to the nerve.
|
|
|
Tumors with a proclivity to spread to the brain include...
|
melanoma
renal cell carcinoma choriocarcinoma |
|
|
Neurofibromatosis type 1 is charcterized by....
|
Neurofibromas
Optic nerve Gliomas Lisch nodules (in the iris) Cafe au lait spots |
|
|
Neurofibromatosis type 2 is charcterized by....
|
Bilateral acoustic Schwannomas
Multiple meningiomas Gliomas and ependymomas of the spinal cord. |
|
|
Tuberous Sclerosis is associated with ....
|
Cortical tuber-like hamartomas
|
|
|
Von Hippel-Lindau disease is associated with...
|
Capillary Hemangioblastomas in the cerebellum, retina and less commonly in the brainstem and spinal cord.
|
|
|
What are the human prion diseases?
|
CJD
vCJD Gertsmann-Straussler-Scheinker Familial fatal insomnia Kuru |
|
|
Alzheimers phase 1 includes...
|
Memory disturbances
Poor judgement Carelessness Irritability |
|
|
Alzheimers phase 2 includes...
|
Aphasia
Urinary incontinence Restlessness with pacing Acalculia Apraxia |
|
|
Alzheimers phase 3 includes...
|
All intellectual functions affected.
Language disturbances. |
|
|
Best definitive diagnosis of Alzheimers.
|
Postmortem examination.
|
|
|
What is the classic presentation of normal pressure hydrocephalus?
|
Urinary incontinence.
Dementia. Gait disturbance. |
|
|
What is the classic presentation of Wernicke's encephalopathy?
|
Nystagmus
Delerium. Ataxia. |
|
|
Binswanger disease
|
Small vessel disease of the white matter.
|
|
|
Lacunar infarcts are usually found in...
|
Putamen and adjacent internal capsule.
Central white matter. Thalamus. Cerebellum. Pons. |
|
|
A seizure preceeding a stroke usually indicates an...
|
AVM rupture.
|
|
|
Most common place for a berry aneurysm is...
|
Anterior communicating artery.
|
|
|
What are the common sources of brain emboli?
|
Cardiac mural thrombus
Valve vegetation Carotid plaque Infarct distal to embolus. |
|
|
What are the inherited Leukodystrophies?
|
Krabbe-autosomal recessive
Metachromatic-Autosomal recessive Adrenoleukodystrophy-X linked |
|
|
What are the acquired leukodystrophies?
|
PML-JC virus
SSPE-Measles |
