Functional

Question 1

In the neurosurgical management of Parkinson disease, lesioning of the ventrolateral nucleus of the thalamus has proved especially beneficial in controlling which of the signs and symptoms of the disease?

Answer 1

Tremor

Question 2

Focal midbrain atrophy is most commonly seen on MR imaging of the brain in a patient with which of the following neurological disorders?

Answer 2

Focal midbrain atrophy with relative sparing of the pons, sometimes referred to as the “Hummingbird sign” or “Penguin sign” is characteristic of progressive supranuclear palsy (PSP).

PSP is a neurodegenerative disease characterized by Parkinsonian features, postural instability, and supranuclear vertical gaze palsy.

Pathologic specimens exhibit neurofibrillary tangles composed of misfolded tau protein.

Midbrain atrophy is specific to PSP and can be diagnostically useful in distinguishing PSP from idiopathic Parkinson’s disease and multiple systems atrophy, which may have overlapping symptoms.

Question 3

Enkephalins and GABA are the main neurotransmitter from:

Answer 3

putamen to GPe

Question 4

substance P and GABA are the main neurotransmitters from:

Answer 4

GPe to GPi

Question 5

Glutamate is the main neurotransmitter from:

Answer 5

cortex to putamen and STN

STN to GPe, GPi, and SN

Thalamus to cortex and putamen

Question 6

serotonin and cck are the main neurotransmitters from:

Answer 6

dorsal raphe nucleus to SN

Question 7

serotonin and enkephalins are the main neurotransmitters from:

Answer 7

dorsal raphe nucleus to caudate

Question 8

projections to and from the medial dorsal nucleus of the thalamus:

Answer 8

input to MD are: prefrontal cortex, GP, amygdala, temporal cortex and SN

output: prefrontal cortex

Question 9

DBS target for dystonia:

Answer 9

GPi

Question 10

DBS target for bradykinesia and rigidity (parkinsons):

Answer 10

STN

Question 11

input and projections of LP nucleus of thalamus:

Answer 11

LP nucleus of thalamus receives input from area 5 and projects to areas 5 and 7

Question 12

What is the main cortical input to the hippocampus?

Answer 12

entorhinal cortex through the alvear (to CA1) or perforant (to dentate gyrus and CA3) pathways

Question 13

What is the best treatment for drop attacks?

Answer 13

corpus callosotomy

prevents spread of seizures to the contralateral hemisphere

Risks of disconnection syndrome; therefore, the posterior part of the corpus callosum (splenium) should be preserved

Question 14

Inclusion bodies (all intracytoplasmic) type and disease:

Answer 14

hirano bodies: alzheimers

lafora bodies: lafora disease

pick bodies (stain with silver): picks disease

lewy bodies: parkinsons

bunina bodies: ALS

Question 15

type of inclusion bodies found in melanin cells?

Answer 15

marinesco bodies (normal)

contain ubiquitin

Question 16

Sodium valproate (AED) works by:

Answer 16

Increasing GABA

Question 17

benzodiazepine and barbiturates (AEDs) work by:

Answer 17

stimulating GABAa receptors

Question 18

phenytoin and carbemazepine (AED) work by:

Answer 18

decreasing sodium conductance by blocking sodium channels

Question 19

DBS of the periaqueductal gray can result in (side effects):

Answer 19

diplopia, oscillopsia, fear, and anxiety

Question 20

When targeting STN for Parkinson’s disease, if the DBS electrode is too:

Answer 20

Medial → III n → diplopia, ipsilateral eye adduction.

Lateral and anterior → internal capsule → contralateral contractions, contralateral gaze deviation.

Posterior → medial lemniscus → contralateral paresthesias.

Deep (ventral) → substantia nigra → fear, depression

Question 21

When targeting GPi with DBS for Parkinson’s disease or dystonia, if the electrode is too:

Answer 21

Medial and posterior → internal capsule → contralateral contractions.

Deep → optic tract → phosphenes (flashes of light) in the contralateral visual field.

Lateral and anterior → GPe, putamen → no effect.

Question 22

What forms the thalamic fasciculus (FFH1)? What is the main neurotransmitter?

Answer 22

thalamic fasciculus (FFH1) is formed by the lenticular fasciculus (FFH2) and the ansa lenticularis. It projects from the GPi to the thalamus using GABA. It also carries dentate nucleus projections to the thalamus

Question 23

Which thalamic nuclei project to the cingulate gyrus and what are the inputs?

Answer 23

Anterior nuclear group

receives input from the fornix and the mamillothalamic tract

Question 24

dorsal anterior cingulate cortex (dACC) is thought to be responsible for which type of pain?

Answer 24

affective

Question 25

What are the different targets for DBS and what are they treating?

Answer 25

Parkinsonism → STN

essential tremors → Vim

dystonia → GPi

nucleus accumbens septi → obsessive-compulsive disorder (OCD)

subcallosal cingulate gyrus → depression.

Question 26

What are the two neurotransmitters in the dorsal horn that transmit pain?

Answer 26

substance P (chronic pain, burning, slow) and glutamate (acute pain, sharp, fast) via C and Adelta fibers, respectively

Question 27

Marchiafava–Bignami disease: It was first described in Italian men drinking inexpensive red wine. Symptoms include dementia, depression, apathy, and delusions. What are the CNS manifestations?

Answer 27

It is rare, and causes demyelination and necrosis of the genu and body of the corpus callosum.

Question 28

CNS manifestations and symptoms of central pontine myelinolysis?

Answer 28

demyelination and necrosis with preserved axons and neurons and absence of inflammatory cells. Symptoms include quadriparesis, hyperreflexia, seizures, coma, and pseudobulbar palsy.

Question 29

Black widow spider venom: symptoms, mechanism, treatment

Answer 29

This depletes the presynaptic ACh stores into the neuromuscular junction, causing cramps and spasms followed by weakness. Treatment is with Ca2+ gluconate and MgSO4.

Question 30

Huntington’s disease: onset, inheritance/chromosome, mutation, symptoms, treatment

Answer 30

Onset in the 30s, with survival 15–30 years more.

autosomal dominant on chromosome 4 with no sex predilection

trinucleotide repeat disorder of CAG

Symptoms begin with personality changes followed by subcortical dementia (no aphasia, agnosia, or apraxia) and choreiform movements (affects hands and face first)

atrophy mostly of the caudate (with boxcar ventricles) more than the putamen, GP, and cortex. Medium spiny type 1 neurons are affected first and aspiny neurons are spared. There is decreased GABA and ACh and increased NE and somatostatin.

Haloperidol (2–10 mg/day) may be used to treat the athetosis, with low doses and frequent drug holidays to decrease the risk of tardive dyskinesia

Question 31

primary basal ganglia and cerebellar blood vessel calcification (sometimes also dentate nuclei) associated with renal disease or decreased parathyroid hormone.

Answer 31

Fahrs disease

Question 32

Striatonigral degeneration: symptoms, CNS manifestations

Answer 32

Symptoms include rigidity and akinesia (Parkinson-like symptoms) with syncope. There are atrophic and brown putamen and depigmented SN as well as decreased neurons in the putamen, caudate, and SN. There are no Lewy bodies or neurofibrillary tangles.

Question 33

Progressive supranuclear palsy: onset, symptoms, response to L-DOPA, CNS manifestations, type of neurofibrillary tangles

Answer 33

Onset of 50–60 years and death within 1–12 years with male predominance

Symptoms include deterioration of intellect, vision, speech, and gait, with vertical gaze palsy, loss of voluntary eye movements and opticokinetic nystagmus, decreased oculocephalic reflexes, pseudobulbar palsy, and axial rigidity without tremor

poor response to L-DOPA

atrophy of the midbrain, superior colliculus, and subthalamic nuclei with ventricular dilation and decreased neurons in the GP, SN, and various brainstem nuclei

neurofibrillary tangles are different than the Alzheimer type (i.e., globose versus flame-shaped).

Question 34

Olivopontocerebellar atrophy: onset, inheritance, symptoms, CNS manifestations

Answer 34

Onset is at 15 years with sporadic, recessive, and dominant inheritance

Ataxia of the lower limbs occurs first

atrophy of the pons, middle cerebellar peduncle, inferior olive, and cerebellar cortex. It is associated with Parkinson’s syndrome.

Question 35

Strokes in children are caused by:

Answer 35

Fabry disease, homocystinuria.

Question 36

Caudate subcomponents:

Answer 36

Head: contiguous with the anterior perforated substance.

Body: separated from the thalamus by the stria terminalis and the terminal vein.

Tail: in the roof of the temporal horn near the amygdala.

Nucleus accumbens septi: located where the caudate and putamen meet anteriorly.

Question 37

striatum definitions:

Answer 37

Lentiform nuclei = putamen + GP.

Corpus striatum = neostriatum + paleostriatum (caudate, putamen, GP).

Neostriatum (or striatum) = caudate + putamen.

Question 38

Striatum input fibers:

Answer 38

Corticostriate fibers (most important input) which travel from:

Area 4 → bilateral putamen.

Premotor cortex → ipsilateral caudate and putamen.

Prefrontal cortex → caudate.

Amygdalostriate fibers:

Amygdala → caudate and putamen.

Thalamostriate fibers, which travel from:

Intralaminar CM nucleus (the largest intralaminar nucleus) → putamen.

Parafascicular nucleus (PF) → caudate.

Nigrostriatal fibers, which travel from:

SN → caudate and putamen (inhibitory dopamine).

Raphe nucleus sends inhibitory fibers (serotonin).

Question 39

Striatum ouput:

Answer 39

Striatonigral fibers: head of caudate → substantia nigra pars reticulata (SNpr).

Striatopallidal fibers: caudate/putamen → GP interna (inhibitory, GABA).

Nigrothalamic fibers: terminate in VA, VLm, MD (GABA).

Question 40

Pallidosubthalamic fibers:

Answer 40

LGP → ST.

MGP → stria medullaris → lateral habenula.

GP → VA, VL, and CM.

Question 41

Answer 41