NeuroPathology - Lecture Eight Objectives - ALS and Neoplasms

Question 1

What is Amyotrophic Lateral Sclerosis?

Answer 1

a degenerative terminal disease affecting BOTH upper and lower motor neurons

Question 2

What clinically occurs with ALS?

Answer 2

muscle wasting and gliotic hardening of the anterior/lateral corticospinal tracts

Question 3

What percentage of ALS patients inherited it from family?

Answer 3

5-10% // familial ALS

Question 4

What structures are affected in ALS?

Answer 4

the cortex, corticospinal tract, brainstem nuclei of CN V, VII, IX, X, and XII, and anterior horn cells in spinal cord

Question 5

What are some possible causes of ALS?

Answer 5

excess glutamate, oxidative stress, impaired axonal transportation, protein aggregation, apoptosis, and lifestyle factors

Question 6

How is strength preserved early in ALS?

Answer 6

healthy, intact surrounding axons can sprout and re-innervate the partially denervated muscle

Question 7

How long can re-innervation occur/compensate in ALS?

Answer 7

Only up until motor unit loss is at about 50%. After that re-innervation cannot compensate faster than the rate of degeneration

Question 8

How is ALS diagnosed?

Answer 8

LMN and UMN lesions found by clinical examination + progression of disease within a region(s) AND an absence of any evidence that may indicate another disease

Question 9

What is the rarest form of ALS, and how is it characterized?

Answer 9

Primary Lateral Sclerosis // neuronal loss in cortex, hyperreflexia+spasticity, NO muscle atrophy or fasciculation(twitching)

Question 10

What are the two subtypes of ALS, and what % of the population do they affect?

Answer 10

Limb onset - 70-80% // Bulbar onset - 20-30%

Question 11

What are the clinical features of ALS Limb Onset?

Answer 11

extremities affected first, insidious LMN lesion onset, asymmetric weakness, distal to proximal, extensors weaker than flexors, head drooping, increased lumbar lordosis, clawhand, fasciculation, cramping/stiffness

Question 12

What are the clinical features of ALS Bulbar Onset?

Answer 12

voice/swallowing affected first, cognitive/executive function impairment, decreased verbal fluency, weakness in articulation, swallowing, facial muscle weakness, decreased tongue movements, and change in voice quality(hoarseness)

Question 13

What affects general prognosis of ALS survival rates?

Answer 13

younger age = better/longer survival rate, limb-onset has better prognosis, psychological well being, and less severe involvement/longer duration between onset and diagnosis/no dyspnea at onset

Question 14

What classifies a Primary brain tumor?

Answer 14

developed within the CNS

Question 15

What classifies a Secondary brain tumor?

Answer 15

metastasis of a cancer started elsewhere **commonly breast/lung cancer that metastasizes to brain

Question 16

How does the WHO classify a Grade I Tumor?

Answer 16

slow growing, cells almost look normal, rarely spreads and may be cured if completely removed

Question 17

How does the WHO classify a Grade II Tumor?

Answer 17

slow growing but may spready to nearby tissue and may recur/progress

Question 18

How does the WHO classify a Grade III Tumor?

Answer 18

quickly growing, likely to spread, looks very different from normal cells

Question 19

How does the WHO classify a Grade IV Tumor?

Answer 19

grows and spreads quickly and cells do not look normal at all, may have some dead cells and this tumor cannot be cured

Question 20

What are the symptoms of a brain tumor?

Answer 20

Depends on where in the brain it is / which lobe

Question 21

What are the brain tumor types?

Answer 21

Glioma, Meningioma, and Neurioma

Question 22

What are Gliomas subdivided into?

Answer 22

astrocytomas, oligodendrogliomas, and ependymomas

Question 23

What is the most common type of brain tumor?

Answer 23

Gliomas - 40-45% of all brain tumors

Question 24

What is a Glioblastoma?

Answer 24

A high grade astrocytoma that is rapidly growing, aggressive, and highly infiltrative that tends to invade both hemispheres via corpus callosum

Question 25

What percentage of oligodendrogliomas make up overall glioma cases?

Answer 25

2-3%

Question 26

What are the clinical characeristics of an oligodendroglioma?

Answer 26

solid and slow growing, predominantly located in cerebral hemispheres(often in frontal lobe), bleeds spontaneously and may manifest with stroke like symptoms

Question 27

What percentage of Ependymomas make up overall gliomas?

Answer 27

Low, only about 2% and arises from ependymal cells

Question 28

What are the characteristics of a Meningioma?

Answer 28

slow growing and usually benign in dural folds, cerebral convexities, spinal cord. Accounts for 27% of all intracranial neoplasms and second most common tumor

Question 29

What type of glioma arises from Schwann cells?

Answer 29

Neurinoma

Question 30

What are the characteristics of a Neurinoma?

Answer 30

unilateral sensorineural hearing loss, tinnitus, vertigo, unsteadiness