Chapter 22 - CNS Part 3 Flashcards
What is the result of cerebellar degeneration?
Ataxia
What is the result of motor neuron degeneration?
Weakness
What is the result of degeneration of the cortex?
Memory, language, insight/planning issues
What is dementia?
Impaired memory and cognition along with personality changes accompanied with decreased functioning
What is the most common cause of dementia?
Alzheimer’s disease
After the age of 85, what is the frequency for Alzheimer’s disease?
50%
What kind of plaques are associated with Alzheimer’s disease?
Beta-amyloid
What is the resulting substance from the neuronal degeneration of Alzheimer’s disease?
Tau proteins
What happens to the brain in Alzheimer’s disease?
Neurotoxicity, decreased neurotransmission, cortical atrophy
How long does it usually take to be diagnosed with Alzheimer’s disease?
5-10 years
What chromosome abnormality based condition is associated with the 10% genetic predisposition of Alzheimer’s disease?
Down Syndrome
Is Alzheimer’s disease widely genetic?
No (only 10%)
What is the culprit for CNS degeneration?
Abnormal protein accumulation
What is the most common cause of Parkinsonism?
Parkinson’s disease
What is Parkinsonism?
Abnormal motor function, tremor, rigidity, bradykinesia, instability
What neurons are damaged in Parkinsonism?
Dopaminergic neurons
What specific CNS areas are affected by Parkinsonism?
Substantia nigra, cortex, medulla, pons
What CNS neural activity is altered in Parkinsonism?
Synaptic transmission (most commonly involving dopamine)
Is Parkinsonism always caused by Parkinson’s disease?
No (just most commonly)
A Lewy body is a cluster of what protein?
Alpha-synuclein
What type of inclusion is associated with Parkinson’s disease?
Lewy bodies
Pallor of the substantia nigra is associated with what neurological disease?
Parkinson’s
What is the usual age of onset for Parkinson’s disease?
45-65 years
How long does it typically take for Parkinson’s to become immobilizing?
10-15 years
What type of gait is associated with Parkinson’s disease?
Festinating gait
Pill-rolling of the thumb and index fingers along with cogwheel rigidity is associated with what neurological disease?
Parkinson’s disease
What is usually the cause of death of those with Parkinson’s?
Frequent falls, infection (pneumonia)
What is the condition when dementia is present within one year of motor symptoms (Parkinson’s)?
Lewy body dementia
What is the treatment for Parkinson’s disease?
L-DOPA (increases dopamine), deep brain stimulator
What is the genetic pattern of Huntington disease?
Autosomal dominant
What is the genetic factor involved in Huntington disease?
Huntington gene, trinucleotide repeat: CAG (anticipation generation by generation)
What inclusion is involved with Huntington disease?
Huntingtin protein
Cerebral atrophy due to Huntington disease is prominent at what CNS locations?
Caudate and putamen nuclei
What is the typical age of delayed onset of Huntington disease?
30-40 year old (don’t forget it is still an inherited disease
Chorea of the entire body is associated with what condition?
Huntington disease
Huntington disease is most commonly lethal after how many years?
About 15
How do those with Huntington disease typically die?
Pneumonia, injuries, choking, suicide
What neurological condition is uniquely associated with both lower and upper motor neuron death?
Amyotrophic lateral sclerosis
What locations and tracts are affected with the lower motor neuron death seen in ALS?
Corticospinal and corticobulbar tracts, anterior horn cells, ventral nerve roots
What general locations of the CNS are affected with lower motor neuron death seen in ALS?
Spinal cord and brainstem
What specific cells are associated with upper motor neuron death seen in ALS and in what location?
Betz cells in the posterior frontal lobe
What gender is more likely to get ALS?
Males
What is the usual age range for ALS?
40-50 years
What muscular effects are seen in ALS?
Fasciculations, asymmetric distal extremity weakness, progressive muscle atrophy and decreased strength
What specific muscles are unaffected by ALS?
Extraocular muscles
What is usually the cause of death with those who have ALS?
Respiratory paralysis (pneumonia)
What is the genetic pattern of ALS?
10% autosomal dominant
Are cases of ALS usually sporadic or genetically inherited?
Sporadic (90%)
What is the frequency of cranial CNS tumors?
15 per 100,000
What is the frequency of spinal CNS tumors?
2 per 100,000
Which are more frequent: cranial or spinal CNS tumors?
Cranial
Are CNS tumors common overall?
Not really (17 per 100,000)
Do CNS tumors usually metastasize outside of the CNS?
Not usually
Are CNS tumors usually primary or secondary?
Primary
What is significant about the stages of CNS tumors?
No premalignant (in situ) stages
What effect do CNS tumors usually have on intracranial pressure?
Increase
What is the term for a tumor derived from glial cells?
Glioma
What are the three broad categories of gliomas?
Astrocytoma, oligodendroglioma, ependymoma
Which categories of gliomas are known to be diffuse/infiltrative?
Astrocytoma and oligodendroglioma
What kind of CNS tumor makes up 80% of adult gliomas?
Diffuse astrocytomas
What is the usual age range of diffuse astrocytomas?
30-60
Are diffuse astrocytomas benign or malignant?
Always malignant
What is the prognosis for diffuse astrocytomas?
Poor: 1-5 years
What is the primary location for diffuse astrocytomas?
Mostly frontal lobe
What are the three grades of diffuse astrocytomas?
A: well-differentiated
B: anaplastic
C: glioblastoma
What is the worst grade of a diffuse astrocytoma?
Glioblastoma (close to just a 1 year prognosis)
What is the general location for diffuse astrocytomas?
Cerebral cortex (rarely brainstem or cerebellum)
What kind of astrocytoma is benign, cystic, and commonly affects children or young adults?
Pilocytic
What is the usual location of pilocytic astrocytomas?
Cerebellum and spinal cord (rarely cerebral cortex)
Which is has a poorly circumscribed lesion: a diffuse or pilocytic astrocytoma?
Diffuse
Which is has a well-circumscribed lesion: a diffuse or pilocytic astrocytoma?
Pilocytic
Why does cerebral edema possibly occur with glioblastomas?
Leaky angiogenesis
What is the age range of oligodendrogliomas?
30-50
What type of tumor makes up about 5-15% of all gliomas?
Oligodendroglioma
What is the usual location for oligodendrogliomas?
Frontal or temporal lobes of the cortex
What is the prognosis for oligodendrogliomas?
5-20 years
A history of neurological problems, especially years of seizures, is associated with what kind of CNS tumor?
Oligodendroglioma
What CNS tumor is most commonly seen among pediatrics?
Ependymomas
What is the most common location for ependymomas in pediatrics?
Periventricular regions
What is the most common location for ependymomas in adults?
Spinal canal
Which type of CNS tumor presents a microscopic fibrillary appearance with rosettes/canals?
Ependymoma
Which CNS tumor presents a uniform, dark chromatin, and clear halo microscopic appearance?
Oligodendroglioma
Embryonal (primitive) neoplasms are derived from what cells?
Neuroectodermal cells (small round cells)
Is a medulloblastoma benign or malignant?
Malignant
What is the location for a medulloblastoma?
Exclusively in the cerebellar region (specifically midline and infratentorial)
What is the difference between a medulloblastoma and a primitive neuroectodermal tumor?
PNETs are located outside of the CNS
Medulloblastoma are similar to what other kind of tumor?
Primitive neuroectodermal tumors
What kind of tumor make up 20% of all pediatric tumors?
Medulloblastoma
What type of malignant tumor is highly radiosensitive, well-circumscribed, and hyperchromatic nuclei?
Medulloblastoma
Homer-Wright rosettes with a central neutrophil are commonly seen in which pediatric malignant tumor?
Medulloblastoma
What is the most common CNS tumor in immunosuppressed patients, most uniquely with AIDS patients?
Primary CNS lymphoma
What is a primary CNS lymphoma made of?
Diffuse large B cells
Testing positive for what virus can lead to extensive CNS necrosis and possibly a primary CNS lymphoma?
EBV (which targets B cells possibly leading to their transformation)
How does primary CNS lymphoma spread?
Via ventricles
What is the age range for primary CNS lymphomas?
Any age but increases with age after the age of 60
What is the usual location for primary CNS lymphoma?
Any brain tissue (multifocal)
Are meningiomas benign or malignant?
Benign
What are the transformed cells of meningiomas?
Transformed arachnoid cells
Meningiomas most commonly affect what population?
Adult females
Why don’t CNS tumors usually metastasize to other areas of the body?
Dural protection
Tumors can metastasize to the CNS from what kinds of cancers?
Lung, breast, melanoma, kidney, GI
What kind of tumor makes up 25-50% of intracranial tumors?
Tumors metastasizing to the CNS from other locations