Neurology VI

Question 1

Wilson disease is characterized by accumulation of copper, especially in the […], brain, cornea, and kidneys.

Answer 1

Wilson disease is characterized by accumulation of copper, especially in the liver, brain, cornea, and kidneys.

copper-mediated production of hydroxyl free radicals leads to tissue damage

Question 2

Wilson disease is characterized by […] serum ceruloplasmin and […] urinary copper.

Answer 2

Wilson disease is characterized by decreased serum ceruloplasmin and increased urinary copper.

Question 3

Wilson disease may be treated with oral […] or chelating agents (e.g. penicillamine, trientine).

Answer 3

Wilson disease may be treated with oral zinc or chelating agents (e.g. penicillamine, trientine).

Question 4

Wilson disease often presents with […] due to copper deposits in Descemet’s membrane of the cornea.

Answer 4

Wilson disease often presents with Kayser-Fleisher rings due to copper deposits in Descemet’s membrane of the cornea.

Kayser-Fleisher rings may be visualized on slit lamp examination if not seen on visual inspection

Question 5

[…] syndrome is caused by hemisection of the spinal cord.

Answer 5

Brown-Sequard syndrome is caused by hemisection of the spinal cord.

Question 6

[…] syndrome presents after a stroke with initial parethesias followed in weeks to months by abnormal, unpleasant sense of touch (dysesthesia) and hypersensitivity to pain (allodynia).

Answer 6

Central post-stroke (thalamic pain) syndrome presents after a stroke with initial parethesias followed in weeks to months by abnormal, unpleasant sense of touch (dysesthesia) and hypersensitivity to pain (allodynia).

due to thalamic lesions (occurs in 10% of stroke patients); also known as Dejerine–Roussy syndrome

Question 7

[…] necrosis is a hallmark of prolonged seizures and can lead to persistent neurologic deficits and recurrent seizures.

Answer 7

Cortical laminar necrosis is a hallmark of prolonged seizures and can lead to persistent neurologic deficits and recurrent seizures.

occurs due to excitatory cytotoxicity; appears as cortical hyperintensity on diffusion-weighted imaging

Question 8

[…] hematoma often presents with a loss of consciousness followed by a lucid interval for up to 48 hours.

Answer 8

Epidural hematoma often presents with a loss of consciousness followed by a lucid interval for up to 48 hours.

“talk and die syndrome”

Question 9

[…] is characterized grossly by gray-appearing plaques in the white matter.

Answer 9

Multiple sclerosis is characterized grossly by gray-appearing plaques in the white matter.

Question 10

[…] is characterized by increased CSF opening pressure with no apparent cause on imaging (e.g. no hydrocephalus, obstruction of CSF outflow).

Answer 10

Pseudotumor cerebri is characterized by increased CSF opening pressure with no apparent cause on imaging (e.g. no hydrocephalus, obstruction of CSF outflow).

> 200 mm H2O in a non-obese patient or > 250 mm H2O in an obese patient; also known as idiopathic intracranial hypertension

Question 11

[…] is a glutamate inhibitor that is approved for use in patients with amyotrophic lateral sclerosis (ALS).

Answer 11

Riluzole is a glutamate inhibitor that is approved for use in patients with amyotrophic lateral sclerosis (ALS).

extends survival time and/or time to tracheostomy

Question 12

Riluzole is a […] inhibitor that is approved for use in patients with amyotrophic lateral sclerosis (ALS).

Answer 12

Riluzole is a glutamate inhibitor that is approved for use in patients with amyotrophic lateral sclerosis (ALS).

extends survival time and/or time to tracheostomy

Question 13

Riluzole is a glutamate inhibitor that is approved for use in patients with […].

Answer 13

Riluzole is a glutamate inhibitor that is approved for use in patients with amyotrophic lateral sclerosis (ALS).

extends survival time and/or time to tracheostomy

Question 14

[…] is a cystic cavity within the central canal of the spinal cord (yellow arrows).

Answer 14

Syringomyelia is a cystic cavity within the central canal of the spinal cord (yellow arrows).

Question 15

Central retinal artery occlusion is characterized by a pale retina with a […] at the fovea.

Answer 15

Central retinal artery occlusion is characterized by a pale retina with a “cherry red” spot at the fovea.

the fovea is thin, allowing for visualization of the choroid blood vessels

Question 16

[…] occlusion is characterized by a pale retina with a “cherry red” spot at the fovea.

Answer 16

Central retinal artery occlusion is characterized by a pale retina with a “cherry red” spot at the fovea.

the fovea is thin, allowing for visualization of the choroid blood vessels

Question 17

Creutzfeldt-Jakob disease is associated with increased […] protein in CSF.

Answer 17

Creutzfeldt-Jakob disease is associated with increased 14-3-3 protein in CSF.

Question 18

[…] disease is associated with increased 14-3-3 protein in CSF.

Answer 18

Creutzfeldt-Jakob disease is associated with increased 14-3-3 protein in CSF.

Question 19

Epidural hematoma is characterized by a hyperdense […]-shaped collection of blood on CT.

Answer 19

Epidural hematoma is characterized by a hyperdense lens-shaped collection of blood on CT.

also may be described as a biconvex disk

Question 20

[…] hematoma is characterized by a hyperdense lens-shaped collection of blood on CT.

Answer 20

Epidural hematoma is characterized by a hyperdense lens-shaped collection of blood on CT.

also may be described as a biconvex disk

Question 21

Frontotemporal dementia is characterized by […] and/or […] symptoms early in the disease course.

Answer 21

Frontotemporal dementia is characterized by behavioral and/or language symptoms early in the disease course.

other characteristic features include personality changes, earlier age of onset, and a strong hereditary component

Question 22

[…] dementia is characterized by behavioral and/or language symptoms early in the disease course.

Answer 22

Frontotemporal dementia is characterized by behavioral and/or language symptoms early in the disease course.

other characteristic features include personality changes, earlier age of onset, and a strong hereditary component

Question 23

Glioblastoma multiforme is a CNS tumor that usually arises in the cerebral hemispheres and characteristically crosses the […].

Answer 23

Glioblastoma multiforme is a CNS tumor that usually arises in the cerebral hemispheres and characteristically crosses the corpus callosum (“butterfly glioma”).

classically visualized as a butterfly appearance with central necrosis

Question 24

[…] is a CNS tumor that usually arises in the cerebral hemispheres and characteristically crosses the corpus callosum (“butterfly glioma”).

Answer 24

Glioblastoma multiforme is a CNS tumor that usually arises in the cerebral hemispheres and characteristically crosses the corpus callosum (“butterfly glioma”).

classically visualized as a butterfly appearance with central necrosis

Question 25

Hemispatial neglect syndrome is characterized by inability to process sensory information (agnosia) of the [...]-lateral half of the world.

Answer 25

Hemispatial neglect syndrome is characterized by inability to process sensory information (agnosia) of the **contra**-lateral half of the world. occurs with lesions of the non-dominant parietal cortex

Question 26

[...] syndrome is characterized by inability to process sensory information (agnosia) of the contra-lateral half of the world.

Answer 26

**Hemispatial neglect** syndrome is characterized by inability to process sensory information (agnosia) of the contra-lateral half of the world. occurs with lesions of the non-dominant parietal cortex

Question 27

Lacunar strokes often occur secondary to microatheroma formation and [...] in the small penetrating arteries of the brain.

Answer 27

Lacunar strokes often occur secondary to microatheroma formation and **lipohyalinosis** in the small penetrating arteries of the brain. ultimately leads to thrombotic small-vessel occlusion; related to hypertension, diabetes, hyperlipidemia, and smoking

Question 28

[...] strokes often occur secondary to microatheroma formation and lipohyalinosis in the small penetrating arteries of the brain.

Answer 28

**Lacunar** strokes often occur secondary to microatheroma formation and lipohyalinosis in the small penetrating arteries of the brain. ultimately leads to thrombotic small-vessel occlusion; related to hypertension, diabetes, hyperlipidemia, and smoking

Question 29

Lateral medullary (Wallenberg) syndrome is typically caused by occlusion of the [...] artery.

Answer 29

Lateral medullary (Wallenberg) syndrome is typically caused by occlusion of the **posterior inferior cerebellar** artery.

Question 30

[...] syndrome is typically caused by occlusion of the posterior inferior cerebellar artery.

Answer 30

**Lateral medullary (Wallenberg)** syndrome is typically caused by occlusion of the posterior inferior cerebellar artery.

Question 31

Metastatic CNS tumors characteristically present as multiple, well-circumscribed lesions at the [...] junction.

Answer 31

Metastatic CNS tumors characteristically present as multiple, well-circumscribed lesions at the **gray-white** junction. typically manifests as headache, focal neurologic dysfunction, cognitive change, or seizure

Question 32

[...] CNS tumors characteristically present as multiple, well-circumscribed lesions at the gray-white junction.

Answer 32

**Metastatic** CNS tumors characteristically present as multiple, well-circumscribed lesions at the gray-white junction. typically manifests as headache, focal neurologic dysfunction, cognitive change, or seizure

Question 33

Pick disease is a degenerative disease of the [...] and [...] cortex.

Answer 33

Pick disease is a degenerative disease of the **frontal and temporal** cortex. also known as frontotemporal dementia; parietal and occipital lobe are spared (vs Alzheimer's)

Question 34

[...] disease is a degenerative disease of the frontal and temporal cortex.

Answer 34

**Pick** disease is a degenerative disease of the frontal and temporal cortex. also known as frontotemporal dementia; parietal and occipital lobe are spared (vs Alzheimer's)

Question 35

Primary CNS lymphoma is typically characterized by [...] in the CSF and a single ring-enhancing lesion on MRI in an HIV-infected patient.

Answer 35

Primary CNS lymphoma is typically characterized by **EBV DNA** in the CSF and a single ring-enhancing lesion on MRI in an HIV-infected patient. versus toxoplasmosis, which typically presents as multiple ring-enhancing lesions on MRI

Question 36

[...] is typically characterized by EBV DNA in the CSF and a single ring-enhancing lesion on MRI in an HIV-infected patient.

Answer 36

**Primary CNS lymphoma** is typically characterized by EBV DNA in the CSF and a single ring-enhancing lesion on MRI in an HIV-infected patient. versus toxoplasmosis, which typically presents as multiple ring-enhancing lesions on MRI

Question 37

Subacute combined degeneration is a spinal cord lesion most commonly seen with vitamin [...] deficiency.

Answer 37

Subacute combined degeneration is a spinal cord lesion most commonly seen with vitamin **B12** deficiency. causes ataxic gait, paresthesia, impaired position/vibration sense

Question 38

[...] is a spinal cord lesion most commonly seen with vitamin B12 deficiency.

Answer 38

**Subacute combined degeneration** is a spinal cord lesion most commonly seen with vitamin B12 deficiency. causes ataxic gait, paresthesia, impaired position/vibration sense

Question 39

Subarachnoid hemorrhage is characterized by a [...] spinal tap.

Answer 39

Subarachnoid hemorrhage is characterized by a **yellow (xanthochromic)** spinal tap. due to bilirubin breakdown from hemoglobin; usually seen 6 hours after symptom onset

Question 40

[...] hemorrhage is characterized by a yellow (xanthochromic) spinal tap.

Answer 40

**Subarachnoid** hemorrhage is characterized by a yellow (xanthochromic) spinal tap. due to bilirubin breakdown from hemoglobin; usually seen 6 hours after symptom onset

Question 41

Subdural hematoma is characterized by a [...]-shaped hemorrhage on CT.

Answer 41

Subdural hematoma is characterized by a **crescent-**shaped hemorrhage on CT.

Question 42

[...] hematoma is characterized by a crescent-shaped hemorrhage on CT.

Answer 42

**Subdural** hematoma is characterized by a crescent-shaped hemorrhage on CT.

Question 43

Wilson disease often presents with neuropsychiatric manifestations, due to copper deposition in the [...].

Answer 43

Wilson disease often presents with neuropsychiatric manifestations, due to copper deposition in the **basal ganglia**. e.g. dysarthria, dystonia, tremor, parkinsonism, behavioral changes, dementia, chorea; copper deposition results in atrophy of the basal ganglia

Question 44

[...] disease often presents with neuropsychiatric manifestations, due to copper deposition in the basal ganglia.

Answer 44

**Wilson** disease often presents with neuropsychiatric manifestations, due to copper deposition in the basal ganglia. e.g. dysarthria, dystonia, tremor, parkinsonism, behavioral changes, dementia, chorea; copper deposition results in atrophy of the basal ganglia

Question 45

Huntington disease is characterized by degeneration of [...] neurons in the caudate nucleus of the basal ganglia.

Answer 45

Huntington disease is characterized by degeneration of **GABAergic** neurons in the caudate nucleus of the basal ganglia.

Question 46

Huntington disease is characterized by degeneration of GABAergic neurons in the [...] of the basal ganglia.

Answer 46

Huntington disease is characterized by degeneration of GABAergic neurons in the **caudate nucleus** of the basal ganglia.

Question 47

[...] disease is characterized by degeneration of GABAergic neurons in the caudate nucleus of the basal ganglia.

Answer 47

**Huntington** disease is characterized by degeneration of GABAergic neurons in the caudate nucleus of the basal ganglia.

Question 48

Lower motor neuron lesions of the facial nerve result in ipsi-lateral paralysis of the [...] muscles of facial expression.

Answer 48

Lower motor neuron lesions of the facial nerve result in ipsi-lateral paralysis of the **upper and lower** muscles of facial expression. e.g. destruction of the facial nucleus or CN VII anywhere along its course

Question 49

Lower motor neuron lesions of the facial nerve result in [...]-lateral paralysis of the upper and lower muscles of facial expression.

Answer 49

Lower motor neuron lesions of the facial nerve result in **ipsi-**lateral paralysis of the upper and lower muscles of facial expression. e.g. destruction of the facial nucleus or CN VII anywhere along its course

Question 50

[...] motor neuron lesions of the facial nerve result in ipsi-lateral paralysis of the upper and lower muscles of facial expression.

Answer 50

**Lower** motor neuron lesions of the facial nerve result in ipsi-lateral paralysis of the upper and lower muscles of facial expression. e.g. destruction of the facial nucleus or CN VII anywhere along its course

Question 51

Parkinson disease is characterized histologically by [...], which are round, eosinophilic inclusions composed of α-synuclein.

Answer 51

Parkinson disease is characterized histologically by **Lewy bodies**, which are round, eosinophilic inclusions composed of α-synuclein.

Question 52

Parkinson disease is characterized histologically by Lewy bodies, which are round, eosinophilic inclusions composed of [...].

Answer 52

Parkinson disease is characterized histologically by Lewy bodies, which are round, eosinophilic inclusions composed of **α-synuclein.**

Question 53

[...] disease is characterized histologically by Lewy bodies, which are round, eosinophilic inclusions composed of α-synuclein.

Answer 53

**Parkinson** disease is characterized histologically by Lewy bodies, which are round, eosinophilic inclusions composed of α-synuclein.

Question 54

Upper motor neuron lesions of the facial nerve result in contra-lateral paralysis of the [...] muscles of facial expression.

Answer 54

Upper motor neuron lesions of the facial nerve result in contra-lateral paralysis of the **lower** muscles of facial expression. e.g. destruction of motor cortex or connection between motor cortex and facial nucleus; forehead is spared due to bilateral UMN innervation

Question 55

Upper motor neuron lesions of the facial nerve result in [...]-lateral paralysis of the lower muscles of facial expression.

Answer 55

Upper motor neuron lesions of the facial nerve result in **contra-**lateral paralysis of the lower muscles of facial expression. e.g. destruction of motor cortex or connection between motor cortex and facial nucleus; forehead is spared due to bilateral UMN innervation

Question 56

[...] motor neuron lesions of the facial nerve result in contra-lateral paralysis of the lower muscles of facial expression.

Answer 56

**Upper** motor neuron lesions of the facial nerve result in contra-lateral paralysis of the lower muscles of facial expression. e.g. destruction of motor cortex or connection between motor cortex and facial nucleus; forehead is spared due to bilateral UMN innervation

Question 57

Normal pressure hydrocephalus classically presents with a triad of [...], gait instability, and dementia.

Answer 57

Normal pressure hydrocephalus classically presents with a triad of **urinary incontinence**, gait instability, and dementia. "wet, wobbly, and wacky"; sometimes reversible

Question 58

Normal pressure hydrocephalus classically presents with a triad of urinary incontinence, [...], and dementia.

Answer 58

Normal pressure hydrocephalus classically presents with a triad of urinary incontinence, **gait instability,** and dementia. "wet, wobbly, and wacky"; sometimes reversible

Question 59

Normal pressure hydrocephalus classically presents with a triad of urinary incontinence, gait instability, and [...].

Answer 59

Normal pressure hydrocephalus classically presents with a triad of urinary incontinence, gait instability, and dementia. "wet, wobbly, and wacky"; sometimes reversible

Question 60

[...] hydrocephalus classically presents with a triad of urinary incontinence, gait instability, and dementia.

Answer 60

**Normal pressure** hydrocephalus classically presents with a triad of urinary incontinence, gait instability, and dementia. "wet, wobbly, and wacky"; sometimes reversible