NSG Comprehensive Board Review Flashcards

1
Q

What are Virchow-Robin spaces?

A

Perivascular potential space, between blood vessels and the surrounding sheath of leptomeninges entering the nervous tissue (brain and spinal cord).

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What ion(s) are increased in CSF compared to plasma?

A

Chloride.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What ion(s) are decreased in CSF compared to plasma?

A

Potassium, calcium, uric acid and glucose.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Describe Froin’s syndrome.

A

CSF xanthochromia and clotting (due to the presence of fibrinogen) occur when CSF is loculated, usually in the lumbar thecal sac.

CSF protein is increased (up to 1000mg/L).

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Sites of CSF production?

A

70% choroid plexus, 18% ultrafiltrate, 12% metabolic H20 production.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What nuclei controls CSF production?

A

Raphe nuclei send axons (serotonin) to the periependymal vessels.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Rate of CSF production.?

A

0.3-0.37mL/min or 20mL/hr.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

The BBB is formed by what?

A

Capillary endothelial tight junctions (mainly).

Pinocytic activity in endothelial cells.

Astrocytic foot processes.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What are the circumventricular organs?

A
  1. Organum vasculosum (lamina terminalis).
  2. Neurohypophysis.
  3. Median eminence of the hypothalamus.
  4. Subfornical organ.
  5. Subcommissural organ.
  6. Pineal gland.
  7. Area postrema.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Functions of the organum vasculosum (lamina terminalis).

A
  1. Outlet for hypothalamic peptides.

2. Detect peptides, amino acids, and proteins in the blood.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Functions of the neurohypophysis.

A

Outlet for hypothalamic hormones (oxytocin and vasopressin).

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Functions of the median eminence of the hypothalamus.

A

Release hypothalamic-releasing factors.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Functions of the subfornical organ.

A
  1. May be involved in body fluid regulation.
  2. Located between the foramina of Monro.
  3. Connected to the choroid plexus.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Functions of the subcommissural organ.

A
  1. Function unknown.
  2. Located under the posterior commissure.
  3. The only circumventricular organ with an intact BBB.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Functions of the pineal gland.

A
  1. Melatonin production.

2. Role in circadian rhythm.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Functions of the area postrema.

A
  1. A chemoreceptor that induces emesis when stimulated by digitalis or apomorphine.
  2. Located on the floor of the 4th ventricle.
  3. The only paired circumventricular organ.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

CBF in normal brain tissue.

A

50 mL/100g brain tissue per minute.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

CBF in the ischemic penumbra (reversible).

A

8-23mL/100g brain tissue per minute.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

CBF in irreversible neuronal death.

A

<8mL/100g brain tissue per minute.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What percentage of the population has a hypoplastic vertebral artery?

A

40%.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Intraosseus branches off the C3 segment of the ICA.

A
  1. Caroticotympanic artery.
  2. Vidian artery.
  3. Periosteal arterial branches.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Describe a persistent stapedial artery.

A

Embryonic stapedial artery that fails to involute (primitive hyoid branch of the ICA).

Courses from the vertical segment, exits through a bony canal on the cochlear promontory and traverses the footplate of stapes to terminate as the middle meningeal artery.

If present, the foramen spinosum is either small or absent with enlarged geniculate fossa (Y-shaped).

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Describe a persistent otic artery.

A

Primitive otic artery that fails to involute.

Embryonic carotid-basilar anastomoses.

Connects petrous ICA to embryonic dorsal longitudinal neural arteries.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Branches of the intracavernous (C4) ICA.

A
  1. Meningohypophyseal trunk (posterior trunk).
  2. Inferolateral trunk (artery of the inferior cavernous sinus).
  3. Medial trunk (McConnell’s capsular arteries).
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Branches off the meningohypophyseal trunk.

A
  1. Tentorial artery (of Bernasconi and Cassinari) - supplies tentorium.
  2. Inferior hypophyseal artery - supplies neurohypophysis.
  3. Dorsal meningeal artery (clival branch) - supples CN VI and part of the clivus.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Describe a persistent trigeminal artery.

A

Most common primitive internal carotid artery-basilar anastomosis.

Associated with higher incidence of vascular abnormalities (25%), aneurysms are most common.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Branches of the intracaverous ICA.

A
  1. Ophthalmic.
  2. Superior hypophyseal.
  3. PCOM.
  4. Anterior choroidal.
  5. Branches to the hypothalamus, optic nerve and chiasm.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Vascular territories of the superior hypophyseal arteries?

A

They course beneath the optic nerve to supply the pituitary (anterior lobe, pituitary stalk), tuber cinerum, and optic nerve and chiasm (inferior surface).

These arteries anastomose with the arteries of the contralateral as well as the inferior hypophyseal arteries to form the hypophyseal portal system.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

What is a fetal PCA?

A

When PCOM diameter is the same as PCA.

Failed regression of fetal PCA leads to dominant blood supply of occipital lobes from the ICA rather than from the vertebrobasilar system.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

Origin of the anterior choroidal artery.

A

Posteromedial surface of the ICA, 2-4 mm distal to the PCOMs origin.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

What are the two segments of the anterior choroidal artery?

A
  1. Cisternal segment - courses posteromedially within the suprasellar cistern beneath the optic tract, then turns posteromedially around the uncus.
  2. Intraventricular segment - continues from the cisternal segment. Prior to reaching the lateral geniculate body, it turns posterolaterally through the crural and ambient cisterns to enter the choroidal fissure (plexal point) of the temporal horn.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

What are the branches of the ACA?

A

A1 - horizontal/precommunicating segment.
A2 - vertical/postcommunicating segment.
A3 - distal ACA and cortical branches.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

Branches off the A1 segment?

A

1-12 perforating arteries called the medial lenticulostriate arteries (medial proximal striate arteries).

Course through the anterior perforated substance to supply the optic nerve (superior surface), optic chiasm, anterior hypothalamus, septum pellucidum, anterior commissure, and pillars of the fornix and anteroinferior striatum.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

Location of the anterior communicating artery?

A

Located in hte cistern of the lamina terminalis.

Two or more perforators arise from the Acomm and supply the infundibulum and optic chiasm.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Branches off the A2 segment.

A
  1. Recurrent artery of Heubner - arises just proximal (or more commonly just distal) to the ACOM and courses back towards A1.
  2. Orbitofrontal artery.
  3. Frontopolar artery.
  4. Anterior internal frontal artery.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

Vascular territory of the recurrent artery of Heubner?

A

Head of the caudate, anterior limb of the IC, anterior putamen and globus pallidus, septal nuclei, and inferior frontal lobe.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

What are the branches off the A3 segment?

A
  1. Callasomarginal.
  2. Pericallosal.
  3. Middle internal frontal artery.
  4. Posterior internal frontal artery.
  5. Paracentral artery.
  6. Superior parietal artery.
  7. Inferior parietal artery.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

What are the segments of the MCA?

A

M1 (horizontal segment).
M2 (insular segment).
M3 (opercular segment).
M4 (cortical segment).

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

What are the branches off the M1 segment?

A
  1. Uncal artery.
  2. Temporopolar artery.
  3. Anterior temporal artery.
  4. Lateral lenticulostriate arteries.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

What are the branches off the superior trunk of the M2 segment?

A
  1. Orbitofrontal branch.
  2. Prefrontal branch.
  3. Precentral branch.
  4. Central branch.
  5. Anterior parietal branch.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

What are the branches off the inferior trunk of the M2 segment?

A
  1. Posterior parietal branch.
  2. Angular branch.
  3. Temporooccipital branch.
  4. Posterotemporal branch.
  5. Middle temporal branch.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

What are the segments of the PCA?

A

P1 - precommunicating segment.
P2 - ambient segment.
P3 - quadrigeminal segment.
P4 - terminal cortical branches.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

What are the branches off the P1 segment?

A
  1. Posterior thalamoperforating arteries.
  2. Medial posterior choroidal arteries.
  3. Meningeal branches.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

What are the branches off the P2 segment?

A
  1. Lateral posterior choroidal artery.
  2. Thalamogenicular arteries.
  3. Cortical branches.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

What are the branches off the P3 segment?

A
  1. Posterior temporal artery.
  2. Internal occipital artery.
  3. Parietooccipital artery.
  4. Calcarine artery.
  5. Posterior pericallosal artery.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

Branches off the vertebral artery?

A
  1. Extracranial branches (segmental spinal branches, muscular branch anastomoses with muscular branch of ECA, and meningeal branch).
  2. Posterior spinal artery.
  3. Anterior spinal artery.
  4. PICA.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

What is the blood supply to the falx cerebelli?

A
  1. Posterior meningeal branches.
  2. Occipital.
  3. Ascending pharyngeal.
  4. PICA.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

Vascular territory of the posterior spinal artery?

A

Gracile and cuneate fasciculi, ifnerior cerebellar peduncle.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q

Vascular territory of the anterior spinal artery?

A

Pyramid, medial lemniscus, MLF, olive, vagal and hypoglossal nuclei.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
50
Q

What are the segments of PICA?

A
  1. Anterior medullary.
  2. Lateral medullary.
  3. Tonsillomedullary.
  4. Telovelotonsillar.
  5. Hemispheric branches.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
51
Q

What syndrome occurs with occlusion of PICA?

A

Lateral medullary syndrome (Wallenberg’s).

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
52
Q

What are the branches off AICA?

A
  1. Internal auditory artery.
  2. Recurrent perforating artery.
  3. Subarcuate artery.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
53
Q

Vascular supply to the striatum?

A

Mainly: MCA (lenticulostriate arteries).

Rostrally: recurrent artery of Heubner.

Caudally: anterior choroidal.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
54
Q

Vascular supply to the internal capsule?

A

Anterior limb: ACA (Heubner, MCA lateral (lenticulostriate).

Genu: ICA perforators, MCA lateral (lenticulostriate)

Posterior limb: anterior choroidal and Pcomm.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
55
Q

Vascular supply to the thalamus?

A

PCA by way of posterior thalamoperforators, thalamogeniculate arteries, and the medial posterior choroidals.

Rostrally from Pcomm and basilar bifurcation.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
56
Q

Vascular supply to the medulla?

A

Anterior and posterior spinal arteries.

PICA and vertebral arteries.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
57
Q

Vascular supply to the pons?

A

Basilar paramedian branches.

Short and long circumferential branches of the basilar artery that anastomose with AICA at the middle cerebellar peduncle.

SCA.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
58
Q

Vascular supply to the midbrain?

A

Basilar artery, PCA, SCA, Pcomm, and anterior choroidal.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
59
Q

What is Weber’s syndrome?

A

CN III palsy with contralateral hemiplegia.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
60
Q

What is Benedikt’s syndrome?

A

Weber’s plus red nucleus lesion (coarse intentional tremor, hyperkinesias, and ataxia of the upper limb).

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
61
Q

What cistern does the basal vein of Rosenthal pass through?

A

Ambient cistern.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
62
Q

Excitatory neurotransmitters.

A

Glutamate and aspartate.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
63
Q

Inhibitory neurotransmitters.

A

GABA.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
64
Q

How many layers are in the neocortex?

A

The neocortex has 6 layers.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
65
Q

How many layers are in the allocortex?

A

The allocortex has 3 layers, located in the olfactory cortex, hippocampus, and dentate gyrus.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
66
Q

What are the 6 layers in the neocortex?

A
I. Molecular (most superficial).
II. External granular layer.
III. External pyramidal layer.
IV. Internal granular layer.
V. Internal pyramidal layer. 
VI. Multiform.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
67
Q

What layer of the neocortex is responsible for main sensory input?

A

Layer IV (internal granular layer).

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
68
Q

What layer of the neocortex has main efferents to the brainstem and spinal cord?

A

Layer V (internal pyramidal layer).

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
69
Q

What layer of the neocortex has efferent fibers to the thalamus?

A

Layer VI (multiform layer).

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
70
Q

Function of the premotor cortex (area 6a).

A

Voluntary motor control for responses dependent on sensory input.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
71
Q

Function of the supplemental motor cortex (area 6a).

A

Programming, planning, and initiation of motor movements.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
72
Q

Function of the frontal eye fields.

A

Initiates saccades - stimulation causes contralateral eye deviation.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
73
Q

Contents of the neostriatum (or striatum).

A

Caudate and putamen.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
74
Q

Contents of the paleostriatum.

A

Globus pallidus.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
75
Q

Contents of the corpus striatum.

A

Neostriatum + paleostriatum (caudate, putamen, GP).

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
76
Q

Contents of the archistriatum.

A

Amygdala.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
77
Q

Contents of the lentiform nuclei.

A

Putamen and globus pallidus.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
78
Q

Location of lateral medullary lamina.

A

Between putamen and globus pallidus.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
79
Q

Location of medial medullary lamina.

A

Between medial and lateral globus pallidus.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
80
Q

Origin of the adenohypophysis.

A

Ectoderm.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
81
Q

Origin of the neurohypophysis.

A

Diencephalic.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
82
Q

What are the three components of the adenohypophysis?

A
  1. Pars tuberalis
  2. Pars intermedia.
  3. Pars distalis.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
83
Q

What are the three components of the neurohypophysis?

A
  1. Pars nervosa (posterior lobe).
  2. Infundibulum.
  3. Nuclei (supraoptic and paraventricular).
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
84
Q

What arteries form the hypophyseal portal system?

A

The hypophyseal portal system is formed by the superior hypophyseal arteries (supraclinoid ICA) and the inferior hypophyseal arteries (branch of cavernous ICA meningohypophyseal trunk).

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
85
Q

What are the cholinergic areas of the CNS?

A
  1. Substantia innominata (nucleus basalis of Meynert).
  2. Pedunculopontine nucleus.
  3. Lateral dorsal tegmental nucleus.
  4. Medial habenular nucleus.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
86
Q

What is the archicerebellum?

A

Oldest component which includes the flocculus and nodulus.

Involved with vestibular function.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
87
Q

What is the paleocerebellum?

A

Includes the anterior lobe, rostral to the primary fissure.

Controls muscle tone with inputs from the stretch receptors.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
88
Q

What is the neocerebellum?

A

Newest component which includes the posterior lobe between the primary fissure and the lateral fissures.

Controls coordination with inputs from the contralateral cortex (via pontine relay nuclei).

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
89
Q

Function of dorsal spinocerebellar tract.

A

Uncrossed.

Conveys proprioception from the joints, muscle spindles, and Golgi tendon organs of the ipsilateral LEs and upper trunk to Clarke’s nucleus in lamina VII of the intermediate zone in the spinal cord.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
90
Q

Function of the ventral spinocerebellar tract.

A

Crossed.

Conveys efferent copies of motor commands that reach the alpha motor neurons and exteroceptive and proprioceptive information for the LEs.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
91
Q

What are the cerebellar nuclei and their functions.

A
  1. Fastigial nucleus - vestibular system.
  2. Globuse nucleus - tone.
  3. Emboliform nucleus - tone.
  4. Dentate nucleus - coordination.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
92
Q

What nuclei make up the nucleus interpositus?

A
  1. Globus and emboliform nuclei.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
93
Q

The olfactory tract carries secondary neurons to what three areas?

A
  1. Primary (lateral) olfactory area (via lateral olfactory stria).
  2. Anterior perforated substance (intermediate olfactory area).
  3. Medial olfactory area (septal area).
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
94
Q

What structures make up the primary (lateral) olfactory area?

A
  1. Uncus.
  2. Entorhinal area (anteiror portion of the hippocampal gyrus).
  3. Limen insula (insular and frontal lobe junction).
  4. Part of the amygdala.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
95
Q

Function of the medial olfactory area?

A
  1. Located in the subcallosal region of the medial frontal lobe, this region mediates emotional responses to odors with its limbic connections.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
96
Q

What is the diagonal band of Broca?

A
  1. Connects all three olfactory areas.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
97
Q

What is von Willebrand’s knee?

A
  1. Contains fibers from the contralateral optic nerve that travel across a short distance into the other optic nerve before continuing through the optic tract.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
98
Q

Function of the lateral subnucleus of CN III?

A
  1. Innervates ipsilateral inferior rectus, inferior oblique, and medial rectus.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
99
Q

Function of the medial subnucleus of CN III?

A
  1. Innervates contralateral superior rectus.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
100
Q

Function of the central subnucleus of CN III?

A
  1. Innervates levator palpebrae superioris bilaterally.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
101
Q

CN III passes through what cistern?

A
  1. Interpeduncular cistern at the midbrain/pons junction.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
102
Q

What division of CN III do parasympathetic fibers travel?

A
  1. Inferior division.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
103
Q

Function of the mesencephalic nucleus of CN V?

A
  1. Conveys proprioceptor info from the muscles of mastication.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
104
Q

Function of the chief sensory nucleus of CN V?

A
  1. Conveys light touch from face.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
105
Q

Function of the spinal nucleus of CN V?

A
  1. Conveys pain, temperature, and deep pressure info.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
106
Q

Location of the abducens nucleus?

A
  1. Ventral to the 4th ventricle in the pontine tegmentum.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
107
Q

Motor branches of CN VII?

A
  1. Ten Zebras Bit My Clock.

Temporal, zygomatic, buccal, mandibular, cervical.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
108
Q

Lesion of the lateral lemniscus causes what?

A
  1. Contralateral deafness.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
109
Q

Unilateral damage to the superior colliculus produces what?

A
  1. Contralateral visual field neglect.
  2. Impaired tracking.
  3. NO deficit with eye movements.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
110
Q

What is the main function of the rostral interstitial nucleus of the MLF?

A
  1. Main center for vertical eye movement (especially downward).
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
111
Q

What is the function of the inferior colliculus?

A
  1. Responsible for the tonotopic organization of auditory information and projects via the brachium of the inferior colliculus to the medial geniculate body.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
112
Q

What are some functions of the periaqueductal gray?

A
  1. Central analgesia.
  2. Vocalization.
  3. Control of reproductive behavior.
  4. Aggressive behavior.
  5. Upward gaze.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
113
Q

What happens when the red nucleus is stimulated?

A
  1. Increased tone in the contralateral flexors and decreased tone in the contralateral extensors.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
114
Q

What happens when the interposed nucleus is stimulated?

A
  1. Increased ipsilateral flexion.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
115
Q

What happens when the pedunculopontine nucleus is stimulated?

A
  1. Causes walking movements.

2. Controls locomotion.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
116
Q

What neurotransmitter is predominant in the locus ceruleus?

A
  1. NE.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
117
Q

What is the function of the locus ceruleus?

A
  1. Controls cortical activation and paradoxical (REM) sleep.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
118
Q

What are the functions of the raphe nuclei?

A
  1. Provided endogenous analgesia via the substantia gelatinosa.
  2. Controls deep sleep, mood, and aggression.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
119
Q

Features of Rexed lamina I?

A
  1. Mainly pain and temperature (fast pain, A-delta), that travel in the contralateral spinothalamic tract.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
120
Q

What is another term for Rexed lamina II?

A
  1. Substantia gelatinosa.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
121
Q

Function of Rexed lamina II?

A
  1. Slow pain (C fibers).
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
122
Q

What is another term for rexed lamina III/IV?

A
  1. Nucleus proprius.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
123
Q

Function of rexed lamina III/IV?

A
  1. Contains interneurons that convey low intensity stimuli to the thalamus.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
124
Q

What is another term for rexed lamina VII?

A
  1. Zona intermedia.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
125
Q

What type of neurons are found in rexed lamina IX?

A
  1. Alpha and gamma motor neurons.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
126
Q

What is the function of the medial nuclear group of rexed lamina IX?

A
  1. Controls the axial muscles.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
127
Q

What is the function of the lateral nuclear group of rexed lamina IX?

A
  1. Controls the limbs appendicular muscles.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
128
Q

What is the function of the ventral nuclear group of rexed lamina IX?

A
  1. Controls extensors.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
129
Q

What is the function of the dorsal nuclear group of rexed lamina IX?

A
  1. Controls flexors.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
130
Q

Function of the posterior columns?

A
  1. Convey fine touch, vibration and proprioception.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
131
Q

Function of the anterior spinothalamic tract?

A
  1. Conveys mainly light touch.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
132
Q

Function of the lateral spinothalamic tract?

A
  1. Conveys pain and temperature.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
133
Q

Function of the spinotectal tract?

A
  1. May convey pain stimuli.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
134
Q

Function of the dorsal spinocerebellar tract?

A
  1. Conveys touch, pressure and proprioception from the lower limbs.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
135
Q

Function of the ventral spinocerebellar tract?

A
  1. Conveys lower limb posture and coordination information.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
136
Q

Function of the cuenocerebellar tract?

A
  1. Conveys touch, pressure, and proprioception from the upper limbs (upper limb equivalent of the dorsal spinocerebellar tract).
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
137
Q

Function of the rostral spinocerebellar tract?

A
  1. Provides efferent copies, enters the inferior peduncle (upper limb equivalent of the ventral spinocerebellar tract).
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
138
Q

Function of the spinoreticular tract?

A
  1. Modulates motor, sensory, behavior and awareness.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
139
Q

Function of the corticospinal tract?

A
  1. Conveys voluntary skilled movements.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
140
Q

Function of the tectospinal tract?

A
  1. Conveys reflex posture movements in response to visual and possibly auditory stimuli.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
141
Q

Function of the rubrospinal tract?

A
  1. Involved in the maintenance of flexor tone.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
142
Q

Function of the vestibulospinal tract?

A
  1. Involved in the maintenance of extensor tone.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
143
Q

Function of the pontine reticulospinal tract?

A
  1. Involved in the maintenance of extensor tone (antigravity muscles).
  2. Axial (especially the neck muscles) limb muscles.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
144
Q

Function of the medullary reticulospinal tract?

A
  1. Involved in inhibition of extensor tone.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
145
Q

What tracts are involved in flexor tone?

A
  1. Lateral reticulospinal (medullary tract).

2. Rubrospinal tract (to upper limbs only).

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
146
Q

What tracts are involved in extensor tone?

A
  1. Medial and lateral vestibulospinal tracts.

2. Medial (pontine) reticulospinal tract.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
147
Q

Damage to the anterior lobe of the cerebellum causes what?

A
  1. Removes tonic inhibition of the lateral vestibular nucleus resulting in increased extension.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
148
Q

Normal AP diameter of the spinal canal at C1-C2?

A
  1. 15 mm.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
149
Q

Normal AP diameter of the spinal canal at C3-T12?

A
  1. 12 mm.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
150
Q

Normal AP diameter of the spinal canal at L1-L5?

A
  1. 15-20 mm.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
151
Q

Structures within the cribiform plate?

A
  1. Olfactory nerves.
  2. Ethmoidal nerves.
  3. Ethmoidal arteries.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
152
Q

Structures within the optic canal?

A
  1. CN II

2. Ophthalmic artery.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
153
Q

Structures within the superior orbital fissure.

A
  1. CNs III, IV, and VI.
  2. CN V1 -all three branches (nasociliary, frontal, lacrimal).
  3. Sympathetic fibers from ICA plexus.
  4. Middle meningeal artery (orbital branch).
  5. Lacrimal artery (recurrent meningeal branch).
  6. Superior ophthalmic vein.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
154
Q

Structures within the inferior orbital fissure.

A
  1. CN V2.
  2. Zygomatic nerve.
  3. Maxillary nerve (pterygopalatine branch).
  4. Infraorbital artery and vein.
  5. Inferior ophtlamic vein.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
155
Q

Structures within the foramen rotundum.

A
  1. CN V2.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
156
Q

Structures within the foramen ovale.

A
  1. CN V3.

2. Lesser superficial petrosal nerve.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
157
Q

Structures within the foramen lacerum.

A
  1. Usually nothing, 30% with vidian artery.

2. ICA traverses upper portion.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
158
Q

Structures within the carotid canal.

A
  1. Sympathetic nerves.

2. ICA.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
159
Q

Structures within the internal acoustic meatus.

A
  1. CN VII, VIII.

2. Labyrinthine vessels.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
160
Q

Structures within the stylomastoid foramen.

A
  1. CN VII.

2. Stylomastoid artery.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
161
Q

Structures within the jugular foramen (pars nervosa).

A
  1. CN IX.

2. Jacobson’s nerve.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
162
Q

Structures within the jugular foramen (pars venosa).

A
  1. Posterolateral CN X, XI.
  2. Arnold’s nerve.
  3. Internal jugular vein.
  4. Inferior petrosal sinus.
  5. Posterior meningeal artery.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
163
Q

Structures within the hypoglossal canal.

A
  1. CN XII.

2. Posterior meningeal artery.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
164
Q

Structures within the foramen cecum.

A
  1. Emissary veins (SSS -> frontal sinus and nose).
  2. Anterior falcine artery.

Located between the frontal crest and crista galli.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
165
Q

Structures within the supraorbital foramen.

A
  1. CN V1.

2. Supraorbital vessels and nerve.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
166
Q

Structures within the infraorbital foramen.

A
  1. CN V2.

2. Infraorbital vessels and nerve.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
167
Q

Structures within the mandibular foramen.

A
  1. CN V3: inferior alveolar nerve.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
168
Q

Structures within the incisive foramen.

A
  1. CN V2: nasopalatine nerve.

2. Vessels to anterior hard palate.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
169
Q

Structures within the mental foramen.

A
  1. CN V3: mental nerve.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
170
Q

Structures within the greater palatine foramen.

A
  1. Greater palatine nerves.
  2. Vessels to the hard palate and gingiva.
  3. Located medial to the third molar.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
171
Q

Structures within the lesser palatine foramen.

A
  1. Lesser palatine nerves.

2. Vessels to the soft palate.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
172
Q

Structures within the pterygopalatine fossa.

A
  1. CN V2: maxillary nerve.
  2. Pterygopalatine ganglion.
  3. Vidian nerve.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
173
Q

Structures within the infratemporal fossa.

A
  1. CN V3.
  2. Chorda tympani nerve.
  3. Otic ganglion, inferior.
  4. Inferior alveolar nerve.
  5. Lingual nerve.
  6. Buccal nerve.
  7. Muscles: temporalis, medial and lateral pterygoids.
  8. Maxillary artery.
  9. Pterygoid venous plexus.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
174
Q

Structures within the vidian canal (pterygoid canal).

A
  1. Vidian nerve.
  2. Union between the greater superficial petrosal nerve (parasympathetic) and deep petrosal nerve (sympathetic).

Located at the base of medial pterygoid plate (sphenoid bone).

Connects the foramen lacerum to the pterygopalatine fossa.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
175
Q

Structures within the petrotympanic fissure.

A
  1. Transmits chorda tympani.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
176
Q

Structures within the greater petrosal foramen.

A
  1. Greater superficial petrosal nerve.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
177
Q

Structures within the lesser petrosal foramen.

A
  1. Lesser superficial petrosal nerve.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
178
Q

Description of the falciform ligament.

A
  1. Dura extending between anterior clinoid and planum sphenoidale, covering the optic nerve.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
179
Q

Description of Liliequist’s membrane.

A
  1. Two sleeves of arachnoid connecting the posteroinferior wall of the ICA cistern and the superior aspect of the interpeduncular cistern.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
180
Q

What structures pass above the annulus of Zinn?

A
  1. CN V1: lacrimal nerve.
  2. CN V1: frontal nerve.
  3. CN IV.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
181
Q

What structures pass through the annulus of Zinn?

A
  1. CN III: superior division.
  2. CN III: inferior division.
  3. CN VI: nasociliary nerve.
  4. CN VI.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
182
Q

What does the superior division of CN III innervate?

A
  1. Levator palpebrae superioris and superior rectus.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
183
Q

What does the inferior division of CN III innervate?

A
  1. Inferior rectus, medial rectus and inferior oblique.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
184
Q

Feature of the medial longitudinal fasciculus.

A
  1. Eye movements with vestibular input.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
185
Q

Feature of the dorsal longitudinal fasciculus.

A
  1. Periventricular hypothalamus and mammillary bodies to midbrain central gray.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
186
Q

Feature of the medial lemniscus.

A
  1. Posterior column continuation to the thalamus.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
187
Q

Feature of the lateral lemniscus.

A
  1. Part of the auditory pathway.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
188
Q

Feature of the commissure of Probst.

A
  1. Connects the nuclei of the lateral lemniscus.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
189
Q

Feature of the central tegmental tract.

A
  1. Connects the gustatory nucleus (rostral nucleus solitarius) to medial thalamic VPM (wakefulness).
  2. Connects red nucleus to inferior olive.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
190
Q

Feature of the medial forebrain bundle.

A
  1. Connects septal area, hypothalamus, basal olfactory areas, hippocampus/subiculum to midbrain, pons and medulla.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
191
Q

Feature of the lamina terminalis.

A
  1. Closed rostral end of the neural tube.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
192
Q

Feature of the stria terminalis.

A
  1. Connects amygdala to hypothalamus.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
193
Q

Feature of the stria medullaris.

A
  1. Connects the septal area, hypothalamus, olfactory area and anterior thalamus to habenulum.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
194
Q

Feature of the fornix.

A
  1. Connects hippocampus to:
    A. Pre-commissural - septal nuclei, hypothalamus, mammillary bodies, an anterior thalamus.
    B. Post-commissural - cingulate gyrus.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
195
Q

Feature of the ansa lenticularis.

A
  1. GP interna to thalamus (goes AROUND the internal capsule).
196
Q

Feature of the lenticular fasciculus FFH2.

A
  1. GP interna to thalamus (goes THROUGH the internal capsule).
197
Q

Feature of the thalamic fasciculus FFH1.

A
  1. Combination of ansa lenticularis, lenticular fasciculus, and cerebellothalamic tract to VA and VL of the thalamus.
198
Q

Feature of the mammillothalamic tract.

A
  1. Connects mammillary body to anterior thalamic nucleus.
199
Q

Feature of the fasciculus retroflexus.

A
  1. Connects habenulum to midbrain and interpeduncular nuclei.
200
Q

Feature of the diagonal band of Broca.

A
  1. Connects septal nuclei to amygdala.
201
Q

What covers the corpus callosum?

A
  1. Covered with indusium griseum, which contains medial and lateral longitudinal striate that connects the medial olfactory area with hippocampus.
202
Q

Feature of the tapetum.

A
  1. Fibers in the corpus callosum that connect the temporal and occipital lobes.
203
Q

Feature of the uncinate fasciculus.

A
  1. Connects anterior temporal lobe to orbitofrontal gyrus.
204
Q

Feature of the arcuate fasciculus.

A
  1. Connects the frontal, parietal and temporal lobe (Wernicke’s area to Broca’s area).
205
Q

Feature of the anterior portion of the anterior commissure.

A
  1. Connects the two olfactory bulbs.
206
Q

Feature of the posterior portion of the anterior commissure.

A
  1. Connects the two inferior and middle frontal gyri, putamen, GP, external capsules and claustrum.
207
Q

Feature of the inferior collicular commissure.

A
  1. Connects the inferior colliculus.
208
Q

Feature of the posterior commissure.

A
  1. Crossing fibers from the pretectal nucleus for the light reflex.
209
Q

Feature of the trapezoid body.

A
  1. Connects ventral cochlear nuclei to contralateral superior olive.
210
Q

What is another term for the superior cerebellar peduncle?

A
  1. Brachium conjunctiva.
211
Q

What is another term for the middle cerebellar peduncle?

A
  1. Brachium pontis.
212
Q

What is another term for the inferior cerebellar peduncle?

A
  1. Restiform and juxtarestiform bodies.
213
Q

Functions of the medial geniculate body?

A
  1. Auditory relay nucleus.
214
Q

Functions of the lateral geniculate body?

A
  1. Visual relay nucleus.
215
Q

Functions of the superior colliculus?

A
  1. Coordination of head/eye movements with visual system.
216
Q

Functions of the inferior colliculus?

A
  1. Auditory relay and processing.
217
Q

Functions of the superior olive?

A
  1. Auditory relay and processing.
218
Q

Functions of the inferior olivary complex?

A
  1. Cerebellar input.
219
Q

Functions of the ciliary ganglion?

A
  1. Parasympathetic from oculomotor nerve.
220
Q

Functions of the gasserian ganglion (semilunar ganglion)?

A
  1. Trigeminal nerve.
221
Q

Functions of the geniculate ganglion?

A
  1. Sensation and taste from facial nerve.
222
Q

Functions of the sphenopalatine ganglion?

A
  1. Lacrimal and nasal glands from the facial nerve.
223
Q

Functions of the submandibular ganglion?

A
  1. Submandibular and sublingual glands from facial nerve.
224
Q

Functions of the spiral ganglion?

A
  1. Hearing to the cochlear nerve.
225
Q

Functions of the superior and inferior vestibular ganglion?

A
  1. Utricle - superior ganglion.

2. Saccule - inferior ganglion.

226
Q

Functions of Scarpa’s ganglion?

A
  1. Vestibular function.
227
Q

Functions of the otic ganglion?

A
  1. Parotid secretion from glossopharyngeal nerve.
228
Q

Functions of the inferior ganglion of CN IX (petrosal)?

A
  1. Taste.

2. Carotid body/sinus - CN IX.

229
Q

Functions of the superior ganglion of CN IX?

A
  1. Ear sensation.
230
Q

Functions of the inferior ganglion of CN X (nodose)?

A
  1. Taste and visceral sensation
231
Q

Functions of the superior ganglion of CN X (jugular)?

A
  1. Ear sensation.
232
Q

Mechanism of action of tetrodotoxin?

A
  1. Blocks voltage-gated Na channels.
233
Q

Mechanism of action of tetraethylammonium (TEA)?

A
  1. Blocks voltage-gated K channels.
234
Q

What are the four classes of small molecule neurotransmitters?

A
  1. Class 1 - ACh
  2. Class 2 - amines (NE, epi, dopamine, serotonin)
  3. Class 3 - amino acids (GABA, glycine, glutamate, aspartate)
  4. Class 4 - NO
235
Q

Function and locations of acetylcholine?

A
  1. Usually excitatory.
  2. Found in the motor cortex, skeletal muscle, pre-ganglionic autonomic nerves, post-ganglionic parasympathetic nerves, and post-ganglionic sympathetic nerves supplying sweat glands.
236
Q

Function and locations of norepinephrine?

A
  1. Usually excitatory.

2. Found in the pontine locus ceruleus and post-ganglionic sympathetic nerve fibers.

237
Q

Function and locations of dopamine?

A
  1. Inhibitory.

2. Found in the neurons of the substantia nigra that project to the putamen and caudate.

238
Q

Function and locations of glycine?

A
  1. Inhibitory.

2. Found in the spinal cord (Renshaw cells).

239
Q

Function and locations of GABA?

A
  1. Inhibitory.

2. Found in the cortex, basal ganglia, cerebellum (Purkinje cells), and spinal cord.

240
Q

Function and locations of glutamate?

A
  1. Excitatory.

2. Found in the cortex, dentate gyrus of the hippocampus, striatum, and cerebellar granule cells.

241
Q

Function and locations of serotonin?

A
  1. Inhibitory.
  2. Found in brainstem nucleu (median raphe nuclei) that project to the hypothalamus and spinal cord (dorsal horns) as well as the pineal gland.
242
Q

Locations of nicotinic ACh receptors?

A
  1. Located in the NMJ and pre-ganglionic endings of both sympathetic and parasympathetic fibers.
243
Q

Locations of muscarinic ACh receptors?

A
  1. Located in the post-ganglionic parasympathetic endings and post-ganglionic sympathetic endings innervating sweat glands.
244
Q

Function of the GABA-A receptor?

A
  1. Increases Cl channel permeability.
245
Q

Function of the GABA-B receptor?

A
  1. Increases K conductance.
246
Q

Mechanism of action of barbiturates?

A
  1. Prolong the duration of Cl channel opening.
247
Q

Mechanism of action of benzodiazepines?

A
  1. Increase the frequency of Cl channel opening.
248
Q

Function of free nerve endings?

A
  1. Respond to pain, touch and pressure.
249
Q

Function of Meissner corpuscles?

A
  1. Respond to touch.
250
Q

Function of Merkel disks?

A
  1. Respond to touch and pressure.
251
Q

Function of Pacinian corpuscles?

A
  1. Respond to vibration.
252
Q

Function of Ruffini end organs?

A
  1. Respond to heavy touch and pressure.
253
Q

What are the two types of anterior motor neurons?

A
  1. Alpha motor neurons - innervate skeletal muscle by sending alpha-type A fibers to large skeletal muscle fibers within the motor unit.
  2. Gamma motor neurons - relay gamma-type A fibers to intrafusal fibers of the muscle spindle.
254
Q

What is the difference between spasticity and rigidity?

A
  1. Spasticity is characterized by unidirectional resistance to change, velocity dependency, and increased reflexes.
  2. Rigidity is characterized by bidirectional resistance to change, is not velocity dependent, and is not associated with hyper-reflexia.
255
Q

When does the anterior neuropore close?

A
  1. 24 days.
256
Q

When does the posterior neuropore close?

A
  1. 26 days.
257
Q

What problems can occur if dysjunction occurs too early?

A
  1. Mesenchyme can enter the neural tube and form lipomas or lipomyelomeningoceles.
258
Q

Defects in secondary neurulation cause what?

A
  1. Spinal dysraphism below L1/2.
259
Q

Defects during ventral induction produce what conditions?

A
  1. Holoprosencephaly.
  2. Septo-optic dysplasia.
  3. Dandy-Walker malformation.
260
Q

Problems during cellular migration cause what conditions?

A
  1. Callosal agenesis.
  2. Schizencephaly.
  3. Heterotopias.
261
Q

At what age does the metopic suture close?

A
  1. Around the first year of life.
262
Q

At what age does the anterior fontanelle close?

A
  1. By 2.5 years of life.
263
Q

At what age does the posterior and sphenoid fontanelles close?

A
  1. By 2-3 months of life.
264
Q

At what age does the mastoid fontanelle close?

A
  1. By 1 year of life.
265
Q

What is Meckel-Gruber syndrome?

A
  1. Associated with maternal hyperthermia on days 20-26 of gestation.
  2. Associated with cystic dysplastic kidneys, cardiac anomalies, orofacial clefting, and cephaloceles.
266
Q

What is arrhinencephaly?

A
  1. Absence of the olfactory bulbs and tracts with normal cortex and gray matter in place of the corpus callosum.
  2. It is associated with holoprosencephaly and Kallman syndrome (anosmia, hypogonadism, and MR).
267
Q

What is colpocephaly?

A
  1. Dilated occipital horns associated with agenesis of the corpus callosum and periventricular leukomalacia, MR and seizures.
268
Q

Features of Trisomy 13 (Patau syndrome)?

A
  1. Hypotelorism.
  2. Holoprosencephaly.
  3. Microcephaly.
  4. Micropthalmia.
  5. Cleft palate and lip.
  6. Polydactyly.
  7. Dextrocardia.
  8. Ocular abnormalities.
269
Q

Features of Trisomy 18 (Edward syndrome)?

A
  1. Gyral dysplasia.
  2. Callosal agensis.
  3. Chiari II.
  4. Dolichocephaly.
  5. Cerebellar hypoplasia.
  6. Hypertelorism.
  7. Microphthalmia.
  8. Syndactyly.
  9. Rocker bottom feet.
  10. Ventricular septal defects.
270
Q

Features of 5p deletion (cri-du-chat syndrome)?

A
  1. MR.
  2. Microcephaly.
  3. Hypertelorism.
  4. Congenital heart disease.
271
Q

Features of 15q deletion (Prader-Willi)?

A
  1. MR.
  2. Truncal obesity.
  3. Short stature.
  4. Hypogonadism.
272
Q

What locations are affected with kernicterus?

A
  1. Affects predominantly the GP, thalamus, subthalamus, and CNs III and VIII nuclei causing symmetric cell loss with extrapyramidal motor signs.
  2. This is caused by increased unbound unconjugated bilirubin staining the gray matter and causing neuronal necrosis.
273
Q

What is porencephaly?

A
  1. A cavity that extends from the leptomeninges to the ventricles or superficial white matter lined by white matter (as opposed to schizencephaly, which is a cleft lined by gray matter).
274
Q

What is hydranencephaly?

A
  1. Most of the cortex is replaced by CSF.
275
Q

Most common pathogen resulting in a brain abscess?

A
  1. Streptococcus.
276
Q

What are the pathologic states of a cerebral abscess?

A
  1. Early cerebritis (up to 5 days).
  2. Late cerebritis (5 days to 2 weeks).
  3. Early capsule formation (2-3 weeks).
  4. Late capsule formation (> 3 weeks).
277
Q

What is Gradenigo syndrome?

A
  1. Petrous apex osteomyelitis with CN VI palsy and retroorbital pain and may occur in children from extension of severe otitis.
278
Q

Characteristics of lepromatous form of leprosy?

A
  1. Lesions are located in the skin on cooler parts of the body (hands, feet, head, peripheral nerves, anterior eyes, upper airways and testes).
279
Q

Characteristics of tuberculoid form of leprosy?

A
  1. Areas of hypesthetic skin, inflamed swollen nerves, caseating granulomas.
280
Q

What are the 4 types of neurosyphilis?

A
  1. Meningovascular syphilis (lues).
  2. General paresis of the insane.
  3. Tabes dorsalis.
  4. Congenital syphilis.
281
Q

Neurologic complications of Lyme disease?

A
  1. Erythema migrans (70%).
  2. Aseptic meningitis.
  3. Cranial neuritis (especially CN VII).
  4. Encephalitis.
  5. Myelopathy.
  6. Radiculopathy.
  7. Peripheral neuropathy.
282
Q

Most common CNS fungal infection?

A
  1. Candidiasis.
283
Q

Histological features of HSV infections?

A
  1. Cowdry type A inclusions (intranuclear eosinophilic masses with a surrounding halo) found in neurons, oligodendrocytes, and astrocytes.
284
Q

Histological features of CMV infections?

A
  1. Also produces Cowdry type A inclusions (as in HSV) and intracytoplasmic inclusions.
285
Q

What is Ramsay Hunt syndrome?

A
  1. Herpes infection of the geniculate ganglion.
  2. May cause dysfunction of CN VII with altered sensation of taste, facial weakness, and vesicular eruptions on the pinna and in the external auditory canal.
286
Q

Histological feature of rabies infections?

A
  1. Negri bodies (intracytoplasmic eosinophilic collections of ribonucleoproteins) are seen in 80% of cases and are especially predominant in the cerebellum (Purkinje cells), brainstem and hippocampus.
287
Q

What is Gerstmann-Strasuller syndrome?

A
  1. Prion disease that can be autosomal dominant or sporadic.
  2. Kuru plaques are seen in the cerebellum.
  3. Presents with ataxia, dysarthria, hyporeflexia, and cognitive decline without myoclonus.
288
Q

What is fatal familial insomnia?

A
  1. Prion disease characterized by thalamic atrophy.

2. Presents with sleep disturbance, agitation, mild cognitive changes, and dysautonomia.

289
Q

What is the most frequent congenital CNS infection?

A
  1. CMV.
290
Q

Presentation of congenital CMV?

A
  1. Hepatosplenomegaly, jaundice, thrombocytopenia, chorioretinitis, seizures, MR, optic atrophy, impaired hearing, and hydrocephalus.
291
Q

Radiographic findings of CMV?

A
  1. May demonstrate microcephaly and periventricular eggshell calcifications.
292
Q

What is the classic triad of congenital toxoplamosis infection?

A
  1. Hydrocephalus.
  2. Bilateral chorioretinitis.
  3. Cranial calcifications.
293
Q

Presentation of congenital rubella?

A
  1. Chorioretinitis, cataracts, glaucoma, micropthalmia, microcephaly, MR, and deafness.
294
Q

Presentation of congenital syphilis?

A
  1. Hutchinson triad of dental disorders, bilateral deafness, and interstitial keratitis.
295
Q

What tumors stain positive for chromogranin?

A
  1. Pituinary adenoma.
296
Q

What tumors stain positive for common leukocyte antigen?

A
  1. Lymphoma.

2. Germinoma.

297
Q

What tumors stain positive for cytokeratin?

A
  1. Carinoma.
  2. Craniopharyngioma.
  3. Chordoma.
298
Q

What tumors stain positive for desmin?

A
  1. Rhabdosarcoma.

2. Teratoma.

299
Q

What tumors stain positive for immunoglobulins kappa and lambda chains?

A
  1. Lymphomas.
300
Q

What tumors stain positive for neurofilament and synaptophysin?

A
  1. Ganglioglioma.

2. PNET.

301
Q

What tumors stain positive for S100?

A
  1. Schwannoma.
  2. Neurofibroma.
  3. PNET.
  4. Chordoma.
  5. Melanoma.
  6. Renal cell carcinoma.
302
Q

What tumors stain positive for synpatophysin?

A
  1. Tumors with neurons (ganglioglioma, central neurocytoma, etc).
303
Q

What tumors stain positive for transthyretin?

A
  1. Choroid plexus tumors.
304
Q

What tumors stain positive for vimentin?

A
  1. Meningioma.
305
Q

Characteristics of limbic encephalitis?

A
  1. Associated with testicular or lung cancer and anti-Ma protein antibodies.
  2. Affects medial temporal lobes, cingulate gyrus, and insula.
  3. Usually bilateral hyperintense lesions on T2.
  4. Presents with memory impairment and AMS.
306
Q

What condition is associated with anti-Yo antibodies?

A
  1. Cause cerebellar degeneration and are associated with ovarian and breast cancer.
307
Q

What condition is associated with anti-Hu antibodies?

A
  1. Associated with oat cell pulmonary carcinoma or lymphoma.

2. Cause sensory neuropathy, encephalitis, and cerebellar degeneration.

308
Q

What condition is associated with anti-Ri antibodies?

A
  1. Associated with breast cancer.

2. Causes opsoclonus.

309
Q

What condition is associated with antibodies to glutamic acid decarboxylase?

A
  1. Stiff man syndrome - involuntary muscles spasms and rigidity.
310
Q

What is the second most common pediatric brain tumor?

A
  1. Juvenile pilocytic astrocytoma.
311
Q

Common locations for juvenile pilocytic astrocytomas?

A
  1. Cerebellum.
  2. Brainstem.
  3. Optic pathway.
  4. Infundibulum.
312
Q

Histologic buzzword for pilocytic astrocytomas?

A
  1. Rosenthal fibers.
313
Q

Common presentation and location of pleomorphic xanthoastrocytomas?

A
  1. Usually superficial and involve the cortex and leptomeninges but the dura (temporal lobe predominance).
  2. Seizures are frequent.
314
Q

Common presentation and location of subependymal giant cell astrocytomas?

A
  1. Located near the foramen of Monro.
  2. Present with hydrocephalus and seizures.
  3. Seen in 15% of patients with tuberous sclerosis.
315
Q

Treatment of optic gliomas?

A
  1. If distal to the chiasm, remove optic nerve and attached globe.
  2. If the chiasm is involved, resect up to the chiasm preserving vision in the better eye and consider radiation if tumor progression is noted.
316
Q

What factor is associated with a better response to chemotherapy in oligodendrogliomas?

A
  1. Combined loss of heterozygosity of chromosomes 1p and 19q (the most common genetic alteration in oligodendrogliomas) is associated with a better response to chemotherapy and improved progression free and overall survival in patients with anaplastic oligodendroglioma.
317
Q

Common locations for ependymomas?

A
  1. Usually infratentorial (may grow from the 4th ventricle out through the foramen of Luschka and Magendie).
  2. Also account for 60% of intramedullary spinal cord tumors, occurring mostly at the filum.
318
Q

Common locations for subependymomas?

A
  1. Usually located in the floor of the inferior 4th ventricle or the lateral ventricle.
319
Q

Common locations for choroid plexus papillomas?

A
  1. 50% are located in the lateral ventricle (more commonly left atrium in children).
  2. 40% are in the 4th ventricle (more commonly in adults).
320
Q

Characteristics of desmoplastic infantile ganglioglioma?

A
  1. Usually occur before 18 months.
  2. Frontal, cystic lesions adherent to the dura with a desmoplastic reaction.
  3. Can be differentiated from meningioma because it is GFAP positive and EMA negative.
321
Q

Common location of central neurocytomas?

A
  1. Usually originates at the septum pellucidum and occurs in the lateral and third ventricles near the Foramen of Monro.
322
Q

What tumors have true “ependymal” rosettes?

A
  1. Most commonly seen in ependymomas.
323
Q

What tumors have Homer Wright rosettes?

A
  1. Most commonly seen in medulloblastoma and neuroblastoma as well as pineocytoma.
324
Q

What tumors have Flexnor-Wintersteiner rosettes?

A
  1. Most commonly seen in retinoblastomas and pineoblastomas.
325
Q

Common locations for hemangioblastomas?

A
  1. The most common locations are cerebellar hemispheres or vermis (80%), cervical spinal cord (10%) and brainstem (3%).
326
Q

What tissue are craniopharyngiomas derived from?

A
  1. Derived from squamous cells from the Rathke cleft.
327
Q

What is trilateral retinoblastoma?

A
  1. Bilateral retinoblastomas with a pineoblastoma.
328
Q

What is the most common cause of a tumor in the suprasellar and pineal regions?

A
  1. Germinoma.
329
Q

Most common pineal tumor?

A
  1. Germinoma.
330
Q

Second most common pineal tumor?

A
  1. Teratoma.
331
Q

What tumor has elevated serum AFP and Schiller-Duval bodies?

A
  1. Yolk sac tumor (endodermal sinus tumor).
332
Q

Common locations for chordoma?

A
  1. 40% occur in the clivus and 60% in the sacrum.
333
Q

What tissue are chordomas derived from?

A
  1. Derived from notochord remnants (as is the nucleus pulposus).
334
Q

What stains are positive in chordomas?

A
  1. Positive for cytokeratin and EMA and S100 (mesenchymal, neural crest).
335
Q

What tumors have Russell bodies?

A
  1. Plasmacytoma.
336
Q

Common location for colloid cysts?

A
  1. Normally in the anterior roof of the third ventricle between the columns of the fornices and is frequently attached to the stroma of the choroid.
337
Q

Common locations for arachnoid cysts?

A
  1. Middle fossa (60%).
  2. Suprasellar (10%).
  3. Quadrigeminal cistern (10%).
  4. Posterior fossa (10%, CPA and cisterna magna).
  5. Convexity (5%).
338
Q

What is cavum septum pellucidum?

A
  1. At the level of the caudate head.

2. CSF is in between the sheets of the septum pellucidum in the lateral ventricles.

339
Q

What is cavum vergae?

A
  1. A posterior continuation of the cavum septum pellucidum.
340
Q

What is cavum velum interpositum?

A
  1. In the third ventricle because of failure of fusion of the tela choroidea.
341
Q

Schwann cells are derived from what tissue?

A
  1. Neural crest cells.
342
Q

Positive stains for cutaneous neurofibroma?

A
  1. Vimentin, Leu7, S100, and occasionally GFAP.
343
Q

DDx for posterior third ventricle tumors?

A
  1. Meningioma.
  2. Choroid plexus papilloma.
  3. Metastatic tumor.
344
Q

DDx for tectal tumors?

A
  1. Low grade astrocytoma.
345
Q

DDx for primary septum pellucidum tumors?

A
  1. Astrocytoma.
  2. Lymphoma.
  3. Germinoma.
346
Q

DDx for frontal horn/septum pellucidum tumors?

A
  1. Central neurocytoma.
  2. Giant cell astrocytoma.
  3. Subependymoma.
347
Q

DDx for frontal horn tumors in children?

A
  1. Low grade astrocytoma.

2. Giant cell astrocytoma.

348
Q

DDx for tumors in the body of the ventricle in children?

A
  1. PNET.

2. Astrocytoma.

349
Q

DDx for tumors in the atrium of the ventricle in children?

A
  1. Choroid plexus papilloma.
  2. Ependymoma.
  3. Astrocytoma.
350
Q

DDx for tumors in the occipital and temporal horns in children?

A
  1. Meningioma (rare).
351
Q

DDx for foramen of Monro tumors in children?

A
  1. Giant cell astrocytoma.
  2. Colloid cyst.
  3. Craniopharyngioma.
352
Q

DDx for anterior third ventricle tumors in children?

A
  1. Astrocytoma.
  2. Histiocytosis (hypothalamic/infundibular).
  3. Germinoma.
  4. Craniopharyngioma (extrinsic).
353
Q

DDx for fourth ventricular tumors in children?

A
  1. Pilocytic astrocytoma.
  2. Medulloblastoma.
  3. Ependymoma.
  4. Exophytic brainstem glioma.
354
Q

DDx for frontal horn tumors in adults?

A
  1. High grade astrocytoma.
  2. Giant cell astrocytoma.
  3. Central neurocytoma.
  4. Subependymoma.
355
Q

DDx for ventricular body tumors in adults?

A
  1. Astrotycoma.
  2. Central neurocytoma.
  3. Oligodendroglioma.
  4. Subependymoma.
356
Q

DDx for ventricular atrium tumors in adults?

A
  1. Meningoima.
  2. Metastatic tumor.
  3. Lymphoma.
357
Q

DDx for occipital and temporal horn tumors in adults?

A
  1. Meningioma.
358
Q

DDx for foramen of Monro tumors in adults?

A
  1. High grade astrocytoma.
  2. Central neurocytoma.
  3. Oligodendroglioma.
  4. Subependymoma.
  5. Colloid cyst.
359
Q

DDx for anterior third ventricle lesions in adults?

A
  1. Colloid cyst.
  2. Suprasellar extension of pituitary tumor.
  3. Aneurysm.
  4. Glioma.
  5. Sarcoid.
  6. Germinoma.
360
Q

DDx for CPA lesions?

A
  1. Vestibular Schwannoma.
  2. Meningioma.
  3. Epidermoid.
  4. Other (vascular lesions such as dolichoectasia of the basilar artery, aneurysm, AVM, and metastatic tumors.)
361
Q

DDx for internal auditory canal masses?

A
  1. Vestibular schwannoma.
  2. Post-operative fibrosis.
  3. Neuritis (Bell palsy and Ramsay-Hunt zoster otitis).
362
Q

DDx for temporal bone lesions involving the CPA?

A
  1. Gradenigo syndrome (osteomyelitis of the petrous apex with CN VI palsy, otorrhea, and retroorbital pain).
  2. Malignant external otitis.
  3. Cholesteatoma.
  4. Paraganglioma (globus tympanicum tumor).
363
Q

Presentation of Wyburn-Mason syndrome?

A
  1. Unilateral cutaneous vascular nevi of the face and trunk with retinal, optic nerve, visual pathway and midbrain AVMs.
364
Q

Presentation of Klippel-Trenaunay-Weber syndrome?

A
  1. Angioosteohypertrophy (overgrowth of vessels and bones).
  2. One limb is usually enlarged.
  3. It is associated with leptomeningeal AVMs and dermatomal cutaneous hemangiomas.
365
Q

Presentation of epidermal nevus syndrome?

A
  1. Ipsilateral nevus and bone thickening associated with MR, seizures, hemiparesis, and gyral malformations.
366
Q

What is Marchiafava-Bignami disease?

A
  1. Demyelination and necrosis of the genu and body of the corpus callosum.
  2. Symptoms include dementia, depression, apathy and delusions.
367
Q

Symptoms of arsenic toxicity?

A
  1. Encephalopathy, peripheral neuropathy, abdominal pain, nausea, vomiting, diarrhea, shock.
368
Q

What nerve is commonly affected in lead toxicity?

A
  1. Radial nerve leading to wrist drop.
369
Q

Symptoms of manganese toxicity and what location is affected?

A
  1. Causes Parkinonsian symptoms, psychologic disorders and headache.
  2. Neuronal loss and gliosis in the pallidum and striatum.
370
Q

Symptoms of Wernicke’s encephalopathy?

A
  1. Conjugate gaze and lateral rectus palsies, nystagmus, gait ataxia, and confusion.
371
Q

What locations are affected in Wernicke’s encephalopathy?

A
  1. It affects the mamillary bodies, mediodorsal nucleus of the thalamus, periaqueductal gray, floor of the fourth ventricle (dorsal motor nuclei of X and the vestibular nuclei), and superior cerebellar vermis.
  2. The gaze palsies are related to CNs III and VI nuclei lesions, nystagmus to vestibular nuclei lesions, and ataxia to superior cerebellar vermian lesions.
372
Q

Symptoms of Korsakoff psychosis?

A
  1. Deterioration in retrograde and anterograde memory, unintentional confabulation.
373
Q

What locations are affected in Korsakoff’s psychosis?

A
  1. Lesions are in the dorsomedial thalamus.
374
Q

What is the MOA of tetanus toxin?

A
  1. It produces presynaptic excitation of agonist and antagonist muscles (especially the masseter causing lockjaw or trismus) by inhibiting the neurotransmitter release of glycine from inhibitory interneurons such as Renshaw cells in the spinal cord.
  2. Tetanus toxin specifically cleaves synaptobrevin.
375
Q

What other conditions are similar to tetanus?

A
  1. Strychnine poisoning.
  2. Black widow spider bite.
  3. Stiff-man syndrome.
376
Q

Symptoms of diptheria toxin?

A
  1. Causing ascending paralysis and cardiomyopathy.
  2. It is distinguished by early bulbar and ciliary dysfunction and delayed symmetric sensorimotor peripheral neuropathy with demyelination.
377
Q

MOA of the botulism exotoxin?

A
  1. Causes presynaptic inhibition at the neuromuscular junction by decreasing ACh release (similar to Lambert-Eaton).
  2. It cleaves target synaptosome associated protein (SNAP) and N-ethylmaleimide-sensitive factor (NSF) attachment receptors (t-SNAREs) and vesicle SNAREs (v-SNAREs).
378
Q

Symptoms of botulism toxin?

A
  1. Blurred vision, unreactive pupils, diplopia, bulbar paralysis, and then respiratory suppression and quadriparesis in a descending pattern that evolves over 2-4 days.
  2. No sensory changes.
379
Q

MOA and symptoms of black widow spider bite?

A
  1. Depletes the presynaptic ACh stores into the NMJ.

2. Cramps and spasms followed by weakness.

380
Q

Pathophysiology of phenylketonuria?

A
  1. Deficiency of phenylalanine hydroxylase.

2. Phenylalanine accumulates and causing defective myelination.

381
Q

Genetics of PKU?

A
  1. Autosomal recessive on chromosome 12.
382
Q

Pathophysiology of homocystinuria?

A
  1. Defect in methionine metabolism.

2. Increased homocysteine in the blood, urine and CSF.

383
Q

Genetics of homocystinuria?

A
  1. Autosomal recessive with a deficiency of cystathionine beta-synthase on chromosome 21.
384
Q

Presentation of homocystinuria?

A
  1. Physically similar to Marfan’s (tall and thin), but with mental retardation and increased incidence of stroke, lens dislocation, and arachnodactyly.
385
Q

Pathophysiology of maple syrup urine disease?

A
  1. Decreased branch-chain amino acid catabolism caused by a deficiency of branched-chain alpha-keto acid dehydrogenase.
386
Q

Pathophysiology of Hartnup disease?

A
  1. Defect in amino acid transport in the proximal tubule of the nephron with inability to reabsorb neutral amino acids.
387
Q

Presentation of Hartnup disease?

A
  1. Niacin deficiency with pellagra like symptoms.
388
Q

Pathophysiology of Niemann-Pick disease?

A
  1. Deficiency in sphingomyelinase with accumulation of sphinomyelin and cholesterol.
389
Q

Genetics of Niemann-Pick disease?

A
  1. Autosomal recessive with chromosomal abnormalities on 11 and 18.
390
Q

Presentation of Niemann-Pick disease?

A
  1. Death within 2 years.
  2. Cherry red spot (50%).
  3. Supranuclear paresis of vertical gaze.
  4. Pyschomotor retardation.
  5. Hepatosplenomegaly.
391
Q

Histological characteristics of Niemann-Pick disease?

A
  1. Niemann-Pick cells or “foam cells” are large vacuolated histiocytes and lymphocytes.
392
Q

Pathophysiology of Gaucher disease?

A
  1. Deficiency of glucocerebrosidase with accumulation of glucocerebrosides.
393
Q

Histological characteristics of Gaucher disease?

A
  1. Gaucher cells have wrinkled tissue paper appearance from stored glucocerebrosidase.
394
Q

Pathophysiology of Fabry disease?

A
  1. Deficiency of alpha-galactosidase with accumulation of ceramides.
395
Q

Genetics of Fabry disease?

A
  1. X-linked recessive with onset in adolescence.
396
Q

Presentation of Fabry disease?

A
  1. Painful dysesthesias.
  2. Hypertension.
  3. Renal failure.
  4. CHF.
  5. Death by MI or stroke usually in the 6th decade.
397
Q

Pathophysiology of Tay-Sachs disease?

A
  1. Deficiency of hexosaminidase A with accumulation of GM2 gangliosides.
398
Q

Presentation of Tay-Sachs disease?

A
  1. Cherry red spots with macrocephaly without visceromegaly.
  2. Hypotonia.
  3. Seizures.
399
Q

Pathophysiology of Sandhoff disease?

A
  1. Deficiency of hexosaminidase A and B with accumulation of GM2 gangliosides.
400
Q

Presentation of Sandhoff disease?

A
  1. Similar clinical picture to Tay-Sachs disease, but has visceral storage in the liver, spleen, kidney, and heart.
401
Q

Pathophysiology of GM1 gangliosidosis?

A
  1. Deficiency of acid beta-galactosidase with accumulation of GM1 gangliosides.
402
Q

Presentation of GM1 gangliosidosis?

A
  1. Both CNS and visceral involvement with dysmorphic face, cherry red macula, hepatosplenomegaly, bone abnormalities, and contractures.
403
Q

Pathophysiology of Hurler disease?

A
  1. Deficiency of alpha-l-iduronidase with accumulation of mucopolysaccharides.
  2. Urine contains heparin and dermatan sulfate.
404
Q

Presentation of Hurler disease?

A
  1. Gargoyle face, mental retardation, dwarfism, corneal opacities, conduction deafness, hepatosplenomegaly, cardiac dysfunction, skeletal abnormalities, and thick meninges that may cause spinal cord compression.
405
Q

Histological characteristics of Hurler disease?

A
  1. Zebra bodies.
406
Q

What is Scheie syndrome?

A
  1. Milder, rare form of Hurler disease with autosomal recessive inheritance.
  2. There is no MR or neuronal storage.
  3. May produce spinal cord compression from thickened dura, corneal opacities, and carpal tunnel syndrome.
407
Q

Pathophysiology of Hunter syndrome?

A
  1. Deficiency of iduronate sulfatase.

2. Urine contains heparin and dermatan sulfate.

408
Q

Genetics of Hunter syndrome?

A
  1. X-linked recessive.
409
Q

Presentation of Hunter syndrome?

A
  1. Similar to Hurler disease but milder, with no MR, less corneal clouding, and slower progression.
  2. Characteristic skin pebbling and peripheral nerve entrapment.
410
Q

Pathophysiology of Sanfilippo syndrome?

A
  1. Deficiencies of heparin sulfate pathways.

2. Urine contains heparin sulfate.

411
Q

Presentation of Sanfilippo syndrome?

A
  1. MR, ataxia, dysostosis multiplex, and seizures but less corneal clouding and dwarfism.
412
Q

Pathophysiology of Morquio syndrome?

A
  1. Deficiency of galactose 6-sulfatase and beta-galactosidase.
  2. Urine contains keratin sulfate.
413
Q

Presentation of Morquio syndrome?

A
  1. Severe skeletal abnormalities with ligamentous laxities, odontoid hypoplasia, and thick cervical dura with cervical myelopathy, dwarfism, and osteoporosis.
414
Q

Pathophysiology of Maroteau-Lamy disease?

A
  1. Deficiency of sulfatase B.

2. Urine contains dermatan sulfate.

415
Q

Presentation of Maroteau-Lamy disease?

A
  1. No MR but there may be carpal tunnel syndrome and valvular heart disease.
416
Q

Pathophysiology of Sly syndrome?

A
  1. Deficiency of beta-glucuronidase.

2. Urine contains dermatan sulfate, heparan sulfate and chondroitin sulfate.

417
Q

Presentation of Sly syndrome?

A
  1. Moderate MR, corneal clouding, hydrocephalus, hepatosplenomegaly, and bony changes.
418
Q

Pathophysiology of Krabbe disease?

A
  1. Deficiency of galactocerebrosidase beta-galactosidase on chromosome 14 with accumulation of galactocerebroside from myelin sheaths in lysosomes.
419
Q

Presentation of Krabbe disease?

A
  1. Pyschomotor delay.
  2. Microcephaly.
  3. Death by 2 years.
420
Q

Most common leukodystrophy?

A
  1. Metachromatic leukodystrophy.
421
Q

Pathophysiology of metachromatic leukodystrophy?

A
  1. Deficiency of arylsulfatase A with accumulation of sulfatides in lysosomes.
422
Q

Pathophysiology of Pelizaeus-Merzbacher disease?

A
  1. Defective synthesis of proteolipid protein, a myelin protein, required for oligodendrocyte differentiation and survival.
  2. Atrophic brain with demyelination that spares perivascular white matter.
423
Q

Presentation of Pelizaeus-Merzbacher disease?

A
  1. Abnormal eye movements, spasticity, ataxia and MR.

2. Only leukodystrophy with 100% incidence of nystagmus.

424
Q

Pathophysiology of Canavan disease?

A
  1. Caused by a deficiency of N-acetyl-aspartoacylase.
425
Q

Presentation of Canavan disease?

A
  1. Brain actually increases in size, psychomotor regression, blindness, spasticity.
426
Q

Pathophysiology of Alexander disease?

A
  1. Defect in the GFAP gene.

2. Hemispheric demyelination with macrocephaly and mitochondrial dysfunction.

427
Q

Pathophysiology of Refsum disease?

A
  1. Autosomal recessive disease caused by a mutation in the gene encoding phytanic acid alpha-hydroxylase.
428
Q

Presentation of Refsum disease?

A
  1. Motor and sensory loss, ataxia, blindness, cardiac abnormalities, and deafness.
429
Q

Symptoms of Kearns-Sayre syndrome?

A
  1. Pigmentary retinopathy, cardiac abnormalities with heart block or cardiomyopathy, and progressive ophthalmoplegia.
430
Q

Symptoms of Leber hereditary optic neuropathy?

A
  1. Progressive painless loss of central vision.
431
Q

Pathophysiology of Menke kinky hair disease?

A
  1. Defect in copper absorption in the GI tract.
432
Q

Symptoms of Menke kinky hair disease?

A
  1. Secondary hair growth is brittle, twisted and colorless.

2. Seizures and MR.

433
Q

Pathophysiology of Leigh disease?

A
  1. Mitochondrial dysfunction by multiple metabolic defects.
434
Q

What is Lowe syndrome?

A
  1. An X-linked recessive disease causing bilateral cataracts, large eyes, nystagmus, psychomotor retardation, and death by renal failure.
435
Q

What is Zellweger syndrome?

A
  1. Autosomal recessive disease with decreased liver peroxisomes and accumulation of long chain fatty acids.
  2. Presents with cortical dysgenesis and white matter degeneration with hepatorenal dysfunction.
436
Q

What is Fahr disease?

A
  1. Primary basal ganglia and cerebellar blood vessel calcification associated with renal disease or decreased parathyroid hormone.
437
Q

What is Hallervorden-Spatz disease?

A
  1. Occurs in late childhood with death in early adulthood.

2. Symptoms include extrapyramidal and corticospinal dysfunction with dementia.

438
Q

What is Shy-Drager syndrome?

A
  1. Multiple system atrophy with predominant autonomic dysfunction (orthostatic hypotension, urinary incontinence, impotence.
439
Q

Pathophysiology of Friedreich ataxia?

A
  1. Trinucleotide repeat that affects the frataxin gene on chromosome 9.
440
Q

Pathophysiology of Werdnig-Hoffman disease?

A
  1. Autosomal recessive inheritance on chromosome 5q.

2. Causes “floppy baby syndrome”.

441
Q

Pathophysiology of ALS?

A
  1. Degeneration of motor neurons (including Betz cells) and the corticospinal tracts.
442
Q

What are Bunina bodies?

A
  1. Intracytoplasmic, anterior horn cells seen in ALS.
443
Q

What diseases can cause strokes in children?

A
  1. Fabry disease.

2. Homocystinuria.

444
Q

What diseases can cause Parkinson symptoms in adolescents?

A
  1. Wilson disease.

2. Hallervorden-Spatz disease.

445
Q

What diseases can cause cerebellar degeneration?

A
  1. Ethanol.
  2. Dilantin.
  3. Paraneoplastic syndromes.
  4. Down syndorme.
  5. Friedriech ataxia.
446
Q

What is Devic disease?

A
  1. A variant of acute MS in adults, primarily affects the optic nerves and spinal cord (acute necrotizing transverse myelitis).
447
Q

What is Schilder disease?

A
  1. Affects children.

2. Aggressive bilateral acute MS-type demyelination with hemispheric involvement that may also affect axons.

448
Q

What cells in the brain are most vulnerable to ischemia?

A
  1. Hippocampus, cortex (parietooccipital deep sulci), basal ganglia (caudate and putamen), and cerebellum (Purkinje cells).
449
Q

What areas in the hippcampus are most susceptible to ischemia and which is most resistant?

A
  1. CA1 (Sommer area) and CA3 (endplate) are most susceptible.
  2. CA2 is the most resistant.
450
Q

What is Benedikt syndrome?

A
  1. Caused by a lesion in the tegmentum of the midbrain.

2. Causes CN III palsy with contralateral hemiplegia and cerebellar ataxia and tremor.

451
Q

How does moyamoya present in children?

A
  1. Ischemia and transient weakness.
452
Q

How does moyamoya present in adults?

A
  1. Hemorrhage.
453
Q

What genetic mutation is responsible for CADASIL?

A
  1. Mutation of the Notch 3 gene on chromosome 19.
454
Q

How does CADASIL present?

A
  1. Recurrent infarcts with subsequent dementia and leukoencephalopathy with U fiber sparing.
455
Q

Presentation of Raeder syndrome?

A
  1. Unilateral headache and face pain of the V1 and V2 distributions and Horner syndrome.
456
Q

What is Terson syndrome?

A
  1. Retinal hemorrhages associated with aSAH.
457
Q

What is the hemorrhage risk for AVMs?

A
  1. 2-4% per year.
458
Q

If multiple AVMs exist, what conditions should you consider?

A
  1. Wyburn-Mason and hereditary hemorrhagic telangiectasia.
459
Q

Presentations of vein of Galen malformations?

A
  1. Neonates: cyanotic heart disease.
  2. Infant: hydrocephalus and seizures.
  3. Children: SAH.
460
Q

What is a type 1 vein of Galen malformation?

A
  1. AV-fistula of the anterior and posterior choroidal arteries to the median venous sac (embryonal precursor of the vein of Galen).
461
Q

What is a type 2 vein of Galen malformation?

A
  1. Parenchymal AVM in the thalamus/midbrain with thalamoperforate feeders draining into the vein of Galen.
462
Q

What genetic loci are associated with cavernous malformations?

A
  1. CCM1.
  2. CCM2.
  3. PDCD10.
463
Q

What is the most common vascular malformation?

A
  1. Venous malformations.
464
Q

What syndrome is associated with multiple venous malformations?

A
  1. Blue rubber nevus syndrome.
465
Q

What is sinus pericranii?

A
  1. Large communication between intracranial and extracranial veins.
466
Q

Imaging characteristics of DAI?

A
  1. Hyperintense lesions on T2.
467
Q

Describe subfalcine herniation.

A
  1. Cingulate gyrus moves under the free edge of the falx and the ipsilateral foramen of Monro becomes trapped.
  2. ACA may also be compressed.
468
Q

Describe transtentorial herniation.

A
  1. Uncus and parahippocampal gyrus is pushed over the tentorial edge.
  2. Obliteration of the suprasellar cistern and inferomedial displacement of the anterior choroidal, PCOM, and PCA.
  3. The PCA may become compressed causing occipital strokes.
  4. The anterior choroidal artery and perforators may be compressed causing midbrain Duret hemorrhages and basal ganglia infarction.
469
Q

Pathophysiology of fibrous dysplasia.

A
  1. Expands and replaces normal bony medullary spaces with vascular fibrocellular tissue.
470
Q

Definition of platybasia?

A
  1. Flattened skull base with an increased angle of the clivus to the spine or clivus to the anterior fossa > 135 degrees.
471
Q

What is caudal regression syndrome?

A
  1. Consists of lumbosacral agenesis, imperforate anus, genital malformations, renal dysplasia, and sirenomelia (fused legs).
472
Q

What is terminal myelocystocele?

A
  1. Consists of posterior spina bifida or partial sacral agenesis with tethered cord and hydromyelia.
473
Q

What is Foix-Alajouanine syndrome?

A
  1. Subacute necrotizing myelitis, esepcially in the gray matter, usually with a type 1 AVM and caused by venous hypertension.
474
Q

How does Foix-Alajouanine syndrome present?

A
  1. Spastic and then flaccid paraplegia with an ascending sensory loss and loss of sphincter control.
475
Q

What is Klippel-Trenaunay-Weber syndrome?

A
  1. Spinal cord AVM with a cutaneous vascular nevus and an enlarged finger or upper limb (if cervical).
476
Q

What is the pathophysiology of Charcot-Marie-Tooth disease?

A
  1. Atrophy of the peroneal muscle and degeneration of anterior horn cells, posterior columns, DRG, axons and myelin.
477
Q

How does Charcot-Marie-Tooth present?

A
  1. Sensory ataxia and weakness without autonomic dysfunction.
  2. Distal muscle atrophy occurs in the feet and then the hands (develop claw hand).
  3. Patients develop pes cavus and hammertoe deformities.
478
Q

How does Dejerine-Sottas disease present?

A
  1. Slow, progressive development of claw feet and hands, symmetric weakness, wasting of distal limbs, foot pain, and paresthesias, without autonomic dysfunction.
479
Q

Pathophysiology of Refsum disease?

A
  1. Deficiency of phytanic acid oxidase with accumulation of phytanic acid.
480
Q

How does Refsum disease present?

A
  1. Distal symmetric sensorimotor loss in lower limbs, and is associated with retinitis pigmentosum, cardiomyopathy, and hearing loss.
481
Q

How does posterior interosseus syndrome present?

A
  1. Weakness of the radial-inervated forearm and hand muscles (supinator, extensor digitorum, extensor carpi ulnaris, and abductor pollicis longus).
  2. No sensory loss.
  3. Causes a finger drop without a wrist drop because of sparing of the extensor carpi radialis longus.
482
Q

How does anterior interosseus syndrome present?

A
  1. Pure weakness without sensory loss caused by compression of the anterior interosseus branch of the median nerve in the deep forearm.
  2. Unable to form the “okay” sign and demonstrate the “pinched” sign.
483
Q

How does tarsal tunnel syndrome present?

A
  1. Compression of the tibial nerve with paresthesias of the sole of the foot without motor changes.
484
Q

What is Ramsay-Hunt syndrome?

A
  1. Herpes zoster infection of the geniculate ganglion with CN VII dysfunction, possibly CN VIII dysfunction, and vesicular lesions of the ear.
485
Q

What are possible causes of bilateral CN VII nerve palsies?

A
  1. Guillan-Barre and Lyme disease.