Brain & Behavior Exam 2 - Embryo, Cerebellar, Ganglia, Limbic & Autonomic

Front 1

What is neural plate formed from?

Back 1

Neural ectoderm

Front 2

Where does neural groove form?

Back 2

Roofs over neural tube near 4th pair of somites and proceeds rostrally & caudally from there

Front 3

What does Neural crest give rise to?

Back 3

gives rise to DRG
sensory ganglia of V, VII, VIII, IX, and X
autonomic ganglia
Schwann cells
melanocytes
and chromaffin cells

Front 4

What is the order of formation of epiderm, endoderm and mesoderm?

Back 4

1st: Epi 2nd: Endo 3rd: Meso

1) Cells of epiblast convert from epithelium to mesenchyme and migrate to primitive streak and enter primitive streak, first cells push through midline and replace hypoblast and become endoderm
2) Next cells enter as intercalated layer and btw endoderm and epiblast and become mesoderm

Front 5

What closes first: anterior or posterior portion of the neural tube?

Back 5

Anterior closes first (24 days) vs posterior (26 days)

Front 6

What does Procephalon become?

Back 6

Telecephalon:Cerebral hemispheres

Diencephalon: Hyo/epi/thalamus & neurohypophysis (post. Pituitary; anterior pituitary = rafke’s pouch, not neural)

Front 7

What does mesencephalon become?

Back 7

mesencephalon (midbrain)

Front 8

What does Rhombocephalon become?

Back 8

Metacephalon – Pons & Cerebellum (V, VI, VII)

Myelencephalon – Medulla (V, VII, VIII, IX, X, XII)

Front 9

Which rhombomere does CN 4 arise from?

Back 9

R1

Front 10

Which rhombomere does CN XII arise from?

Back 10

R8

Front 11

What determines identity of all rhomban cephalic vessicles that form cranial nerves ?

Back 11

Hox coding

Front 12

What causes Neural tube defects?

Back 12

antifolate antibodies that inhibit folate binding protein

Front 13

What is Anancephaly ?

Back 13

most common malformation of nervous system; fatal, no brain

Front 14

What is Myelocele ?

Back 14

Open neural tube;

Front 15

What is Myelomeningocele ?

Back 15

very serious (second most worrisome after anancephaly) b/c prone to infections;

– forms in a sack, can lead to anacephaly or encephelosphere (hernation of brain outside)

Front 16

What is Cephalocele

Back 16

microcrania and a large herniated sack of brain. 80% Caucasians have occipital encephalocele, Asians and Africans are more likely to have frontal

Front 17

What is Atretic Parietal Meningocele ?

Back 17

– small “topknot” at vertex; attached by fibrous cord to plate - <5%

Front 18

Order of which cortical plate gives rise to cortex layers?

Back 18

1, 6,5432

Front 19

What is Arnold Chiari I malformation?

Back 19

Cerebellar tonsils grow super long and herniated through foramen magnum; then plugs up CSF;

Front 20

What is
Dandy Walker?

Back 20

Incomplete closure of the Vermis (midline of cerebellum); get outpouching and growing bulge of 4th ventricle; infants head just grows…

Front 21

What is Holoprosencephaly?

Back 21

Problem w/ Shh; Normally have different two sides, but now only have one hemisphere; things are not moving out laterally

Front 22

What is Tuberous sclerosis?

Back 22

a) Autosomal dominant
b) 1 per 10,000 to 50,000
c) Multi-system genetic disease that causes non-malignant tumors to grow in the brain and on other vital organs such as the kidneys, heart, eyes, lungs, and skin. A combination of symptoms may include seizures, developmental delay, behavioral problems, skin abnormalities, lung and kidney disease.
d) Grow until they turn off
e) TSC ACTS AS a TUMOR SUPPRESSOR GENE.
f) Mutations: two different mutations – on 9q and 16p (make different products) those two proteins dimerize make a trans-membrane channel, ergo if either is impacted, you get disease.
g) Clonal hamartomas –resembles neoplasm, but not malignant growth // surrounding tissue
h) Symptoms: ash leaf spots (white spots, rhabdomyomas of heart; renal angiomyolipomas,

Front 23

Where is marginal plate located?

Back 23

At top; ventricular zone at bottom; subplate above VZ and CP above SP

Front 24

What is Henson’s Node?

Back 24

One of those regions that expresses signaling molecule (retinoic acid)

Front 25

Benign Nodular Heterotopia

Back 25

random x inactivation; if half normal, & half abnormal; Half of the population of cells cannot migrate; half migrated

Front 26

What is Band Heterotopia ?

Back 26

mutation in Doublecortin; one band that migrates to there, and another band that does it right; see normal cortex and band of grey matter that didn’t migrate completely

Front 27

what is Diffuse Heterotopic Gray Matter?

Back 27

Messed up in such a way that the cells go everywhere, - globe in MRI, not a specific band and therefore everywhere

Front 28

Information from the Pontine nucleus goes where?

Back 28

Middle cerebellar peduncle

Front 29

Information from the Olivary nucleus goes where?

Back 29

Inferior cerebellar peduncle

Front 30

What are the layers of the cerebellar cortex and what cells can be found in them?

Back 30

1) Granule cell layer (Golgi cells that inhibit mossy fibers & GRANULAR CELLS --> excitatory!)

2) Purkinje layer (purkinje cells)

3) Molecular Layer (purkinge dendrites attached to purkinje cell bodies (which are in purkinje layer), also have stallate cells)

Front 31

Where do the climbing fibers originate from and are the excitatory or inhibitory?

Back 31

They originate from the Inferior olivary nucleus and travel to purkinje fibers.

they are EXCITATORY

Front 32

Information from the Pontine nucleus goes where?

Back 32

Middle cerebellar peduncle

Front 33

Information from the Olivary nucleus goes where?

Back 33

Inferior cerebellar peduncle

Front 34

What are the layers of the cerebellar cortex and what cells can be found in them?

Back 34

1) Granule cell layer (Golgi cells that inhibit mossy fibers & GRANULAR CELLS --> excitatory!)

2) Purkinje layer (purkinje cells)

3) Molecular Layer (purkinge dendrites attached to purkinje cell bodies (which are in purkinje layer), also have stallate cells)

Front 35

Where do the climbing fibers originate from and are the excitatory or inhibitory?

Back 35

They originate from the Inferior olivary nucleus and travel to purkinje fibers.

they also synapse directly on the deep cerebellar nuclear cell which then goes to the thalamus--> cortex --> etc

they are EXCITATORY

Front 36

What cells do the mossy fibers synapse on?

Back 36

Granular cells in granular layer of cerebellar cortex which give rise to the parallel fibers. They also synapse directly onto the deep cerebellar nuclei.

These cells are excitatory.

Front 37

are purkinje fibers excitatory or inhibitory

Back 37

INHIBITORY!!!!

Front 38

what causes hyperkinesia

Back 38

Knocking out either the STN or the D2 portion of the stiatum

Front 39

Progressive Supranuclear Palsy (PSP):

Back 39

• Poor responsiveness to levodopa

• supranuclear opthalmoplegia - this is when your eyes will move but you cannot control

• RECURRENT FALLS & POSTURAL PROBLEMS
• Dementia
• HALLMARK: cannot look up or down! Vertical gaze palsy!
• Gradually progressive, older than 40 at onset

Front 40

Multiple System Atrophy (MSA)

Back 40

• combination of realms of neurologic problems: parkinsonism, cerebellum system, and autonomic problem supranuclear opthalmoplegia
• UMN problems!!!
• ORTHOSTATIC HYPOTENSION & PARKINSONS or Parkinson’s and autonomic issues (sweating or digestive)
• Gate is broad – axial involvement (vs limbs)
• SYMMETRICAL SYMPTOMS!!! (Parkinson’s = unilateral)
• HOT CROSS BUN SYMBOL!
• Poorly responsive to levodopa

Front 41

Cortico-basal ganglionic degeneration

Back 41

• Clinical picture is a strikingly asymmetric akinetic-rigid parkinsonian syndrome associated with dystonia, myoclonus in combination with cortical signs including “alien limb” phenomena and cortical sensory loss
• VERY ASYMMETRIC
• Very jerky – myoclonus
• asymmetric cortical degeneration involving frontoparietal areas
`

Front 42

Dementia with lewbodies

Back 42

• visual hallucinations without treatment!
• Cognitive impairment is fluctuating (& earlier)
• Fluctuating cognition
• deficit in executive function
• unexplained syncope
• LEWY BODIES ARE FOUND ALL OVER BRAIN

Front 43

What is a feedback loop in the Cerebellar circuitry?

Back 43

Granule cells exciting Golgi cells that inhibit (feed back) the granule cells!

*granule cells are excitatory and Golgi are inhibitory so its a way of

Front 44

Alzheimer's disease structural change and neural anatomy

Back 44

Neurofibrillary tangle & neuritic plaque

Medial temporal structures

Front 45

Parkinson's Disease structural change and neural anatomy

Back 45

Lewy Body

Substantia nigra

Front 46

ALS structural change and neural anatomy

Back 46

Bunina body & skein

upper & lower motor neurons

Front 47

Multiple System Atrophy structural change and neural anatomy

Back 47

Glial cytoplasmic inclusion

Olivopontocerebellar;
Striatonigral and autonomic

Front 48

Huntington's disease structural change and neural anatomy

Back 48

Neuronal loss & gliosis

Basal ganglia atrophy

Front 49

Progressive Supranuclear Palsy

Back 49

Neurofibrillary tangle (straight filament type)

Multiple brainstem nuclei, subthalamus, cerebellar dentate

Front 50

Pick's disease

Back 50

Pick body

Lobar atrophy

Front 51

Is PSP a Tauopathy or amyloidoses?

Back 51

Tauopathy

Front 52

Is parkinson disease Tauopathy, amyloidoses or Synuclieopathies?

Back 52

Synucleinopathy

Front 53

Right sided Midbrain lesion symptoms

Back 53

Right side
Drooping of the eyelid (ptosis)
Defects in adduction and
vertical movements of the eye
Double vision (diplopia) when
eyelid is opened passively
Dilated pupil (mydriasis)
Impaired direct and consensual
pupillary reflexes in the
affected eye

Left side
Hemiplegia (paralysis)

Front 54

Pontine lesion on right corticospinal tract? Millard Gubler syndrome

Back 54

Right:

Inability to wrinkle the brow, elevate the
eyebrow, close the eye, retract the
corner of the mouth, move the lips
and the platysma
Loss of nasolabial fold
Drooling on the side of the palsy
Slurred speech
Hyperacusis - stapedius muscle
Direct and consensual corneal reflex
impaired (only eye globe moves up)
Decreased tear secretion


Left:
hemiplegia

Front 55

Pontine lesion (raymond)

Back 55

Right side:
Internal squint
Horizontal diplopia increasing on
gaze to the affected side
Rotation of the head to the side of
the palsy

Left:
Hemiplegia

Front 56

Medullary lesion (lateral medullary syndrome)

Back 56

Right:
Loss of pain and temperature
sensation above the neck
Loss of direct corneal reflex and
preservation of consensual response

Left:
Loss of pain and temperature
sensation below the neck

Front 57

Medullary lesion (anterior) - jackson's syndrome

Back 57

Left:
Hemiatrophy of the tongue
Deviation of the tip of the
tongue toward the affected
side on protrusion

Right:
Hemiplegia

Front 58

What are the four sections of the hypothalamus?

Back 58

1) Preoptic
2) Supraoptic
3)Tuberal
4) Mamillary

Front 59

What nuclei are in the preoptic region?

Back 59

Suprachiasmatic nucleus & preoptic nucleus

Front 60

What nuclei are in the supraoptic region?

Back 60

superoptic nucleus & paraventricular nucleus

Front 61

What nuclei are in the tubular region?

Back 61

Tuberomamillary nucleus

Front 62

What are the limbic system inputs to the hypothalamus

Back 62

Hippocampus-fimbria-fornix -->mamillary body (memory)


Amygdala --> preoptic-anterior hypothalamus (emotion)

Front 63

Sensory inputs into the hypothalamus

Back 63

Visual --> suprachiasmatic nucleus (circadian rhythms)

Smell and taste preoptic-anterior hypothalamus (pleasant or unpleasant quality)


Somatic --> tuberal region (from erogenous areas, also pain)


Visceral -->CN 10 most regions (from nucleus of solitary tract)

Vestibular --> supraoptic nucleus (surge of vasopressin in motion sickness)

Front 64

Humoral inputs into the hypothalamus

Back 64

glucose, hormones, leptin

Front 65

What does activation of the anterior and medial hypothalamus lead to?

Back 65

parasympathetic effects, leading to energy conservation

Front 66

What does activation of the lateral and posterior hypothalamus result?

Back 66

sympathetic effects, leading to expenditure of energy like in stress

Front 67

How does activation of the lateral hypothalamus impact sleep?

Back 67

Excites tuberomamillary nucleus (histaminergic) which in turn activates the thalamus and other areas in wakefulness

Front 68

How does Activation of groups in the preoptic hypothalamus effect sleep

Back 68

It inhibits the tuberomamillary nucleus leading to sleep

Front 69

What do people w/ narcolepsy lack?

Back 69

hypocretin-secreting neurons

Front 70

What do neurons in the anterior hypothalamus respond to and what do they do?

Back 70

Thermosensitive neurons in anterior hypothalamus respond to increases in blood temperature setting off mechanisms for heat dissipation: vasodilation, sweating.

Front 71

What do neurons in the posterior hypothalamus respond to and what do they do?

Back 71

• Thermosensitive neurons in posterior hypothalamus respond to decreases in blood temperature setting off mechanisms for heat conservation and production: vasoconstriction, shivering

Front 72

How do lesions in the medial hypothalamus effect saity?

Back 72

Lesions result in obesity, usually together with sexual infantilism (adiposogenital dystrophy)

Front 73

Wilson's disease symptoms

Back 73

30-35% have neurologic symptoms – if see YOUNG person w/ movement disorder, MUST RULE OUT WILSON’S DISEASE!!!!

Psychiatric symptoms more common than movement disorders in younger people;

Sardonic smile – separates from Huntington’s

Front 74

Wilson's disease pathophysio

Back 74

Autosomal recessive
ATPB7 GENE

COPPER ACCUMULATION ALL OVER - involves GPi and putanem

Front 75

How do you treat Wilson's disease?

Back 75

Liver transplant!

Front 76

limbic chemical system inputs

Back 76

serotonergic, cholinergic, dopaminergic and noradrenergic