Neuroanatomy Flashcards
What are the 6 layers of the cortex (superficial to deep)?
Molecular (plexiform) layer
External granular layer
External pyramidal layer
Internal granular layer
Internal pyramidal layer
Multiform (fusiform) layer
(MP EG EP IG IP MF)
What are the 3 FUNCTIONAL layers of the cortex?
- Supragranular layers (I-III)
- Internal granular layer (IV)
- Infragranular layers (V & VI)
Where do intracortical connections primarily originate and terminate (layer)?
Functional: supragranular layers (I-III which are molecular/plexiform, external granular, and external pyramidal)
What are the 2 main types of intracortical connections?
Associational (intrahemispheric)
&
Commissural (to contralateral hemisphere, usu thru corpus callosum)
What does the internal granular layer do, i.e. where do its connections come from?
Receives thalamocortical connections, esp from thalamic nuclei
Most prominent in primary sensory cortices
What do the infragranular layers mostly do?
Connect cortex & subcortical regions
Where are the infragranular layers most developed?
Motor cortex
Which part of the brain has extremely small or non-existent granular layers and is aka the “agranular” cortex?
Motor cortex
What layer gives rise to all of the principal cortical efferent projections to basal ganglia, brainstem, and spinal cord?
Layer V (internal pyramidal)
Where does layer VI go?
Mostly to the thalamus
aka the multiform or fusiform layer
What’s Nelson’s syndrome?
In the setting of Cushing’s disease (ACTH) s/p bilateral adrenalectomy, but the adenoma keeps growing and secreting ACTH
Does Nelson’s syndrome have Crooke’s cells?
Crooke’s cells are found in pituitaries of Cushing’s disease, but not in the pituitary surrounding the adenomas of Nelson’s syndrome (so no)
What structures make up the diencephalon?
- Thalamus
- Hypothalamus including the Neurohypophysis
- Subthalamus
- Epithalamus
What structures make up the epithalamus?
Ant & post paraventricular nuclei
Medial & lat Habenular nuclei
Stria medullaris thalami
Posterior commissure
Pineal Body
What are 3 changes you see in mammillary bodies in Wernicke’s?
- Soft granular parenchyma (& atrophy?)
- Abnormal capillaries with microhemorrhage (slides & grossly)
- Reactive astrocytes
What are the 3 hereditary motor & sensory neuropathies?
- Charcot-Marie-Tooth
- HMSN II
- Dejerine-Sottas neuropathy
What’s the disease with onion bulbs on EM and what are they?
Charcot-Marie-Tooth
Schwann cell hyperplasia i.t.s.o repetitive de- & remyelinations
What’s the protein you should telegraph for Charcot-Marie-Tooth?
PMP22
peripheral myelin protein 22
What’s the first test you do to eval for Charcot-Marie-Tooth? and why
Single-gene testing for PMP22 duplication/deletion
PMP22 duplication (a 1.5-Mb duplication at 17p11.2 that includes PMP22) accounts for as much as 50% of all CMT
what is the most common neoplasm family that happens in the pineal region?
germ cell tumors!
(germinoma > teratoma > choriocarcinoma»_space; yolk sac & embryonal)
describe the path of the gag reflex (lol WHAT)
Touch receptors in pharyngeal mucosa (CNIX)
Solitary nucleus
Nucleus ambiguus
CNX
Muscles of palate
Brown Sequard 3 main issues with ipsi or contralateral (NOT levels, NOT tracts)
- Ipsilateral flaccid paralysis/loss of cutaneous sensation
- Ipsilateral spastic paralysis & loss of vibration sense, proprioception, & fine touch
- Contralateral loss of pain & temp
Brown Sequard issue & why it happens bc of the tracts affected (NOT levels)
- Ipsilateral flaccid paralysis d/t LMN loss (anterior corticospinal tract)
- Ipsilateral spastic paralysis d/t loss of moderation by UMN of lateral corticospinal tract; loss of vibration sense, proprioception, & fine touch d/t posterior/dorsal columns (fasciculus Gracilis (leGs) &/or fasciculus cuneatus (arms))
- Contralateral loss of pain & temp d/t spinothalamic tract
Brown Sequard ipsi/contra consequences WITH LEVELS
- Ipsi flaccid + band of sensory loss AT level of lesion
- Ipsi spastic + contra loss of vibration/proprio/fine touch BELOW level of lesion
- Contra loss of pain & temp 1-2 levels below lesion
What are the ELEVEN (ugh) structures served by the anterior choroidal artery?
- Choroid plexus (LV & 3rd vent)
- Optic chiasm & tract
- Internal capsule
- LGN
- Globus pallidus
- Tail of the caudate
- Hippo
- Amygdala
- Substantia nigra
- Red nucleus
- Crus cerebri
Which structure served by the anterior choroidal artery also has partially redundant blood supply, and where does it come from?
Posterior limb of internal capsule
Lenticulostriate arteries from MCA
What vessels can cause lateral medullary syndrome?
PICA (specifically lateral medullary segment)
Vertebral artery
What’s the eponym for lateral medullary syndrome?
Wallenberg
What are the 7 structures affected in lateral medullary syndrome?
- Vestibular nuclei
- Inferior cerebellar peduncle
- Central tegmental tract
- Lateral spinothalamic tract
- Spinal trigeminal nucleus & tract
- Nucleus ambiguus (incl vagus & glossopharyngeal nerves)
- Descending sympathetic fibers
LMS: what happens when the vestibular nuclei are infarcted?
Vomiting
Vertigo
Nystagmus
LMS: what happens when the inferior cerebellar peduncle is infarcted?
Ipsilateral ataxia, dysmetria (past pointing), dysdiadochokinesia
LMS: what happens when the central tegmental tract is infarcted?
Palatal myoclonus
LMS: what happens when the lateral spinothalamic tract is infarcted?
Contralateral loss of pain & temp from BODY (limbs & torso)
LMS: what happens when the spinal trigeminal nucleus & tract are infarcted?
ipsilateral loss of pain & temp from FACE
LMS: what happens when the nucleus ambiguus is infarcted?
Ipsilateral laryngeal, pharyngeal, & palatal hemiparalysis (dysphagia, hoarseness, diminished gag d/t efferent limb of CNX)
LMS: what happens when the descending sympathetic fibers are infarcted?
Ipsilateral Horner’s syndrome (ptosis, miosis, & anhidrosis)
What is Grinker’s myelinopathy?
Anoxic leukoencephalopathy or delayed post-hypoxic leukoencephalopathy
(white matter & BG)
Key finding in FCD Ia
Abnormal radial cortical lamination (a-b; r-t)
Key finding in FCD Ib
Abnormal tangential cortical lamination (a-b; r-t)
Key finding in FCD Ic
Abnormal radial AND tangential cortical lamination (i.e. Ia + Ib)
Key finding in FCD IIa
Dysmorphic neurons
Key finding in FCD IIb
Dysmorphic neurons AND balloon cells (b for balloon, IIb is for the 2 Ls in ballon)
Key finding in FCD IIIa
Temporal lobe, a/w hippo sclerosis
Key finding in FCD IIIb
Adjacent to glial or glioneuronal tumor
Key finding in FCD IIIc
Adjacent to vascular malformation
Key finding in FCD IIId
Adjacent to any other lesion acquired during early life (trauma, ischemic injury, encephalitis, etc)
What’s the Guillain-Mollaret triangle?
CONTRALATERAL dentate
Ipsilateral red
Ipsilateral inf olivary
What’s hypertrophic olivary degeneration (why do you get it and why is it weird)
Primary lesion in dentato-rubro-olivary pathway > Transneuronal degen of inf olivary nucleus > HYPERTROPHY of inf olivary nucleus (not atrophy!)
Where do parallel fibers come from?
Granule cells in cerebellar cortex
What do parallel fibers form?
Excitatory synapses onto dendrites of Purkinje cells & dendrites of inhib interneurons basket cells & stellate cells (molec layer)
Grumose degeneration
Degeneration of dentate nucleus of cerebellum: accumulation of granular eo material which is actually swollen/degen Purkinje axons (it looks like axonal spheroids on neurofilament or synapto IHC, just in the cerebellum
Name 3 things that can show grumose degeneration
- PSP
- Dentatorubropallidoluysian atrophy
- Spinocerebellar ataxia type 3
What are the 2 main (broad) categories of “disorders of forebrain induction”?
Holoprosencephaly
&
agenesis of the corpus callosum
Define holoprosencephaly.
Developmental defect of forebrain (prosencephalon which includes telencephalon and diencephalon) where you have incomplete separation of the cerebral hemispheres into R & L
What are the 3 types of holoprosencephaly?
- Alobar/complete (worst) (no separation, one ventricle)
- Semilobar/incomplete (some separation, usu posterior)
- Lobar (focal fusion along midline, either at OFC or cingulate)
What are the issues with noses/smell that you can get with holoprosencephaly?
- No olfactory bulbs
- Anosmia
- Congenital nasal pyriform aperture stenosis
What are 3 infections that have been a/w holoprosencephaly?
Toxoplasmosis
Syphilis
Rubella
What are 3 teratogens that have been a/w holoprosencephaly?
Ethanol
Retinoic acid
Cholesterol synthesis inhibitors
What is the frequency of genetic etiology of holoprosencephaly?
50% of cases have cytogenetic abnormalities
What is the most common genetic etiology of holoproscencephaly?
Trisomy 13
(75% of trisomy 13 cases have holoprosencephaly)
Gene for Smith-Lemli-Opitz syndrome
DHCR7 on chromosome 11
(functions in cholesterol synthesis)
What structure is usually missing in partial agenesis of the CC?
Splenium (posterior)
Name 3 syndromes a/w agenesis of the CC
- Aicardi
- Andermann
- Meckel
What are 3 broad categories of “malformations of cortical development”?
- Lissencephaly
- Heterotopias
- Cortical dysplasias with cytomegaly
Name 3 cortical malformations that occur due to abnormal neurogenesis.
- Microcephaly
- Hemimegalencephaly
- Focal cortical dysplasia
Name 3 cortical malformations that occur due to abnormal neuronal migration.
- Periventricular heterotopia
- Subcortical band heterotopia
- Lissencephaly
Name 1 cortical malformation that occurs due to abnormal arrest in neuronal migration
Cobblestone (type 2) lissencephaly
What gene is associated with autosomal recessive lissencephaly?
RELN (7q22)
What gene is a/w Miller-Dieker syndrome?
LIS1 & 14-3-3’ YWHAE continuous deletion (17p13.3)
targeted LoFxn alleles of Pafah1b1 & 14-3-3’
What gene is associated with autosomal dominant lissencephaly?
LIS1 & 14-3-3’ YWHAE continuous deletion (17p13.3)
targeted LoFxn alleles of Pafah1b1 & 14-3-3’
What is the mode of inheritance of Miller-Dieker syndrome?
Autosomal dominant (haploinsufficiency)
What is the mode of inheritance of isolated lissencephaly sequence?
Autosomal dominant
What gene is a/w isolated lissencephaly sequence?
LIS1 deletion alone (17p13.3)
targeted LoFxn alleles of Pafah1b1
What genes are a/w X-linked lissencephaly?
DCX (Xq22.3-q23) (DOMINANT)
ARX (Xp22.13)
What is the main difference in cortical morphology btwn LIS1 mutation and DCX mutation?
Both are 4 layer cortex, but
LIS1 is posterior
DCX is anterior
What is the main difference in morphology between LIS1, DCX, and ARX mutations?
ARX = 3 layer
LIS1 & DCX = 4 layer
Is ARX lissencephaly 3 layer or 4 layer?
Threeeeeeeeeeeeeeee
What does LIS1 encode?
non-catalytic subunit of plt activating factor acetyl hydrolase
(dynein > neuronal migration)
What are the facial features a/w Miller-Dieker? and are they d/t LIS1 or 14-3-3 mut?
microcephaly (lissencephaly)
bitemporal narrowing
micrognathia
vertical ridging in forehead
D/t 14-3-3 (or just not LIS1) bc LIS1 causes the lissencephaly
Mode of inheritance of DCX lissencephaly
X-linked dominant (males)
Mode of inheritance of ARX lissencephaly & the name of the syndrome
X-linked recessive
XLAG (X-linked lissencephaly with ambiguous genitalia)
mode of inheritance of cobblestone/type 2 lissencephaly
autosomal recessive
What does the cortex look like microscopically in cobblestone/type 2 lissencephaly?
Unlayered and totally disorganized
What are the 2 other organ systems that can be abnormal in cobblestone/type 2 lissencephaly?
Muscular
Ocular
why is it called cobblestone lissencephaly
the neurons? go to the MENINGES??? and they pooch the brain out and make the meninges super thick, so it looks cobblestoned
gene for Fukuyama congenital muscular dystrophy
FCMD (9q31)
Gene for muscle-eye-brain disease types
A, 5
B, 5
C, 5
FKRP (19q13.3)
Gene for Walker Warburg syndrome
POMT1 (9q31-33)
POMT2 (14q24.3)
2 genes a/w muscle-eye-brain disease (not the A, B, C)
POMGnT1 (1p34-33)
LARGE (22q12)
what is the underlying theme of all the types of cobblestone lissencephaly genes?
All result in hypoglycosylation
(issues with glycosyl transferases) (alpha dystroglycans)
What does Walker Warburg have clinically?
HARD + E
Hydrocephalus
Agyria (lissencephaly (cobblestone))
Retinal Dysplasia
Encephalocele (occipital)
also muscular dystrophy and also vermal agenesis/cerebellar dysplasia
Define grey matter heterotopia
Neurons and glia that form a region of grey matter in an abnormal location
What are the 2 main classes of grey matter heterotopia?
Nodular
Band/Laminar
Gene and inheritance pattern of familial subependymal heterotopia?
Gene: FLNA on Xq28 (Filamin 1)
X-linked dominance (happens in females, lethal in males)
Gene in periventricular nodular heterotopia with microcephaly (and inheritance i guess)
ARFGEF2
Auto recessive
What gene is a/w band heterotopia?
DCX (double cortin)
(also a/w lissencephaly)
Which layer of the cortex has the largest neurons?
Layer V
what genetic abnormalities are FCD IIb a/w?
somatic mutations in mTOR pathway
Genes a/w tuberous sclerosis
TSC1 chrom 9: hamartin
TSC2 chrom 16: tuberin
(tumor suppressors; when mutated, mTOR path is upregulated)
where does the recurrent artery of Heubner come from?
anterior cerebral artery
what does the recurrent artery of Heubner supply?
think Heubner’s HAMBUrgers
H: head of caudate + ant hypothalamus
A: anterior internal capsule + nucleus accumbens
M: Meynert + medial GP
B: diagonal band of Broca
U: parts of uncinate fasciculus
where does the anterior choroidal artery come from?
internal carotid (not quite MCA)
what does the anterior choroidal artery supply?
the Choroid is DEEP in the EYE (deep structures, optic pathways)
- optic radiations/posterior internal capsule
- optic tract
- LGN/lateral thalamus
LGN leads to everything near it: hippo + amygdala + tail of caudate + GPi + cerebral peduncle (lat) + choroid plexus in LV
what is anterior choroidal artery syndrome?
Hemiplegia
Hemianesthesia
Contralateral hemianopia
primary (only) efferent pathway from hippocampus
fornix
what cell type does Wernicke’s NOT affect?
Neurons
which is medial & which is lateral: fasciculus cuneatus and gracilis
medial: gracilis (when you say grace you put your hands together in the middle)
lateral: cuneatus
Somatotopy of dorsal columns. Go!
+
aff or eff?
cuneate: cervical and thoracic (lat to med) (CuneaTe has C & T)
gracilis: lumbar (lat) and sacral (med - bc it’s sacrament, cause you’re saying grace)
both are Afferent (Ascending)
somatotopy of lateral corticospinal tract
+
aff or eff?
medial to lateral: C > T > L > S
Efferent/dEscending
somatotopy of anterolateral system
medial to lateral: C > T > L > S
Afferent (Ascending)
how can you orient spinal cord based on BVs?
Posterior spinal arteries are Paired
Anterior is Alone
what other thing are Arnold Chiari type 2 malformations a/w?
lumbosacral myelomeningocele
&
hydrocephalus
what is a type 1 arnold-chiari
herniation of cerebellar tonsil thru foramen magnum
what is a type 2 arnold-chiari
herniation of cerebellar vermis & downward displacement of brainstem (+/- buckling)
what is a type 3 arnold-chiari
cerebello-encephalocele herniation thru occipitocervical or high cervical bony defect (like a big neck cyst with cerebellar herniation into it)
(this feels like a spina bifida but it’s like, maybe sometimes above the spine)
what is syringobulbia and what does it usually occur with?
a slit-like cavity in the brainstem
usu a/w syringomyelia
what is diastematomyelia?
a split in the spinal cord at midline by a spur of tissue (bony, cartilaginous, or fibrous)
(type of spina bifida)
what is the main function of the BG
tonically inhibit movement
what’s the direct pathway?
cortex > striatum > GPi > thalamus
(you inhibit the thalamus & brainstem, and when they are inhibited, THEIR inhibition decreases, which lets you move? i think? so double-inhibition)
has one synapse = direct
synapse is stratum > GPi
what’s the indirect pathway
indirect = multisynaptic
cortex > striatum > GPe > subthalamic nucleus > GPi + SN pars reticulata
allows for fine-tuned BG output = more controlled movement (same result, movement, just has more synapses bc subthal is involved)
which cranial nerve nuclei are in the midbrain?
III (has 2: Edinger-Westphal + Oculomotor)
IV (trochlear)
V (1 part: mesencephalic trigeminal)
which cranial nerve nuclei are in the pons?
V (2 parts: trigeminal motor & principal sensory trigeminal)
VI (abducens)
VII (facial motor)
VIII (1: vestibular)
what cranial nerves are in the salivatory nucleus (medulla)
superior: VII
inferior: IX & X
what cranial nerves are in the nucleus ambiguus?
IX & X
what cranial nerves are in the solitary nucleus?
VII
IX
X
what are the NUCLEI (not cranial nerves necessarily) in the medulla?
Cochlear (VIII)
Solitary (VII, IX, X)
Salivatory (sup VII & inf IX & X)
Nucleus ambiguus (IX & X)
Hypoglossal (XII)
Dorsal motor nucleus of vagus (X)
which cranial nerve nuclei are in the spinal cord?
accessory (XI)
spinal trigeminal (V, VII, IX, X)
Dural innervation (ugh???)
Supratentorial: small meningeal branches of trigeminal (V1, V2, and V3)
Infratentorial: upper cervical nerves