CNS Tumors/ Basal Ganglia

Question 1

What is the difference between a primary and secondary tumor?

Answer 1

primary originate in the CNS

secondary– metastasize from sites outsides the CNS

Question 2

description of benign tumors

Answer 2

slow growing
does not spread
does not invade tissue

Question 3

description of malignant tumors

Answer 3

fast growing
spreads
invades tissues

Question 4

Grade I tumors

Answer 4

benign
slow-growing, low proliferative potential
associated with long-term survival

Question 5

Grade II tumors

Answer 5

benign or malignant
relatively slowly-growing, low proliferation, but sometimes recur as higher grade tumors
prognosis: typically survive more than 5 years post-diagnosis

Question 6

Grade III tumors

Answer 6

Malignant and often recur as higher grade tumors
treatment includes radiation or chemotherapy
prognosis: survive 2-3 years post-diagnosis

Question 7

Grade IV tumors

Answer 7

reproduce rapidly and are very aggressive malignant tumors
mitotically active disease
prognosis: is usually fatal (examples include glioblastoma); majority of individuals with glioblastoma succumb to disease within the year, whereas medulloblastoma with treatment has a 5-year survival rate

Question 8

Tumors of neuroepithelial tissue

Answer 8

astrocytomas
oligodendrogliomas
primitive neuroectodermal tumors (PNET)

Question 9

tumors of meninges

Answer 9

meningiomas

Question 10

Tumors of cranial and paraspinal nerves

Answer 10

Schwannoma (vestibular)

Question 11

Metastatic Tumors

Answer 11

Metastatic (secondary) tumors

Question 12

Astrocytomas

Answer 12

most common primary brain tumor in adults
young adults (most often frontal lobe) and Children (most often cerebellum)
Classified via 1-4 grading scale
-pilocytic astrocytoma (grade I): 30-40 y/o, benign, form cysts, slow growing
-low grade astrocytoma (grade II): 30-40 y/o, benign, form cysts, slow growing
-Glioblastoma Multiforme (Grade IV): highly malignant, rapid growth, <20% survival past 1 year: most common adult brain neoplasm)

Question 13

oligodendrogliomas

Answer 13

• majority of oligodendrogliomas present in young adulthood with onset of seizures and headaches due to electrical conductivity problems
• typically slow-growing, supratentorial
• can have long-term survival– median 9 years, 60-75% 5-year survival

Question 14

Primitive Neuroectodermal Tumors (PNET)

Answer 14

• arise from primitive, undifferentiated nerve cells from the gestational development of the nervous system
• most common malignant CNS tumor in children: Medulloblastoma (arise in 4th ventricle between brain stem and cerebellum)
• increased intracranial pressure (ICP):
  
  • typically first symptoms detected
  • classic triad: morning headache, vomiting , lethargy
• cerebellar impairments: progressively worsening ataxia
• brain stem impairments
  
  • multiple cranial nerve findings: diplopia, facial weakness, tinnitus, hearing loss, head tilt, stiff neck

Question 15

Schwannoma (vestibular) tumors

Answer 15

-formerly known as acoustic neuromas
-arise from vestibular nerve sheath of CN VIII
-benign, slow growing
-usually unilateral
-impairments arise from compression of CN VIII
-tinnitus
-relatively rapid progression of sensorineural hearing
loss
-facial numbness when tumor reaches ~ 2.5 cm diameter
-ataxia, if reaches cerebellum

Question 16

Meningioma

Answer 16

-most common primary, intracranial tumor of non-glial origin
-typically solitary, benign, slow-growing, highly vascular (arise from arachnoid granulations)
-most common in between 30s-60s
-women> men 2:1
common symptoms/impairments:
-focal weakness
-headache
-seizures (imbalance of peritumoral tissue pH, ion concentrations, amino acid balance)
-eventual increase of IP if untreated
-mental status changes
All symptoms depend on FOCAL area affected

Question 17

Metastatic tumors

Answer 17

Occur is 20-40% of people with a primary CA elsewhere in the body
Cancers that frequently spread to the brain (lung, breast, colon, kidney)
treatment of solitary lesions is surgical resection followed by radiation therapy

Question 18

Components of Basal Ganglia

Answer 18

Caudate nucleus
putamen
globus pallidus
subthalamic nucleus
substantia nigra

Question 19

Basal Ganglia

Answer 19

Act as “way station” taking information, integrating it for complex modulation of motor behaviors and projecting back out to cortex for motor output

Question 20

Where is Dopamine produced?

Answer 20

substantia nigra

Question 21

What are the two main feedback systems for refining motor system output?

Answer 21

basal ganglia

cerebellum

Question 22

Function of motor loop

Roles of motor loop

Answer 22

links putamen, globus pallidus, and ventral lateral thalamic nucleus to the motor and premotor cortex

Roles: movement selection and action, regulating muscle contraction, force, multi-joint movements, and sequencing

Question 23

Basal ganglia motor circuit regulates…

Answer 23

muscle contraction
muscle force
multi-joint movements
sequencing movements
*No direct output to LMNs, so works via motor thalamus, pedunctulopontine nucleus, and midbrain locomotor region*

Question 24

Hypokinetic disorders

Answer 24

= excessive inhibition

Parkinson’s Disease

Question 25

Hyperkinetic disorders

Answer 25

= inadequate inhibition
Huntington's 
Dystonia
Tourette's
Dyskinetic cerebral palsy

Question 26

indicators of a diagnosis other than PD

Answer 26

early postural unsteadiness
rapid progression of signs
respiratory dysfunction
abnormal postures
emotional lability 
cerebellar signs
corticospinal dysfunction
voluntary gaze dysfunction

Question 27

symptoms of progressive supranuclear palsy

therapy options for progressive supranuclear palsy

Answer 27

• early onset gait unsteadiness with tendency to fall backward
• axial rigidity
• freezing of gait
• apathy
• depression
• slowed thinking
• psychosis
• rage attacks
• involuntary eye closing
• dysarthria
• dysphagia
• supranucelar gaze palsy

prism glasses, strength, flexibility, posture, balance, mobility, weighted walkers and heel wedges

Question 28

multiple system atrophy

therapy options for multiple system atrophy

Answer 28

progressive degenerative disease affecting basal ganglia, cerebellar, and autonomic systems, peripheral nervous system, and cerebral cortex.

• akinetic/rigid syndrome
• cerebellar signs (dysarthria, truncal/gait ataxia
• autonomic dysfunction (hyoptension, B&B, decrease sweating/tears/saliva, impotence
• corticospinal tract dysfunction (motor function, UMN signs)

therapy: strength, fitness, education (offset hypotension w/ slow position changes, avoid prolonged standing, use compression, avoid warm temps)

Question 29

Lewy Body Dementia

therapy options for LBD

Answer 29

lewy bodies = abnormal accumulations of proteins within neurons

• early, generalized cognitive decline
• visual hallucinations
• motor signs indistinguishable from postural instability gait difficulty subtype of PD

therapy: exercise for strengthening, fitness, balance, etc. as with PD

Question 30

drug-induced parkinsonism

Answer 30

bilateral onset with rapid progression, early postural tremor, involuntary facial movement

Question 31

chronic traumatic encephalopathy (CTE)

Answer 31

victims of abuse, epilepsy, military, athletes (repeated head trauma)

Question 32

huntington’s disease

Answer 32

characterized by chorea (involuntary, jerky movements) and dementia

• inherited, progressive
• onset 40-50 years of age
• causes degeneration in the brain, decreasing signals from the basal ganglia
• disinhibition of the motor thalamus and the PPN -> excessive output from motor areas of the cerebral cortex

therapy: education, fall prevention, assistive technology, HEP