Neurodegenerative disease

Question 1

How does the tremor of Parkinson’s change when a patient is asleep?

Answer 1

It disappears (paradoxical despite being a resting tremor). Most tremors caused by damage to the basal ganglia will disappear during sleep

Question 2

What diseases cause choreiform movements?

Answer 2

Wilson and Huntington disease

Question 3

Damage to what part of the brain leads to intention (or kinetic) tremors?

Answer 3

The cerebellum (can also result from damage to cerebrospinal tract)

Question 4

What are risk factors for Alzheimer’s disease?

Answer 4

Age, female sex, history of severe head trauma, and Down syndrome.
ApoE4 (Chr 19) is a genetic risk factor.
Early-onset AD is associated with amyloid precursor protein (APP) (Chr 21), presenilin 1 (PS1) (Chr 14), and presenilin 2 (PS2) (Chr 1)

Question 5

What area of the brain is primarily affected by progressive supranuclear palsy (PSP)?

Answer 5

Primarily the frontal lobe

Question 6

What are the primary characteristics of progressive supranuclear palsy (PSP)?

Answer 6

Limitations in vertical gaze, extrapyramidal rigidity, gait ataxia, dementia, and frequent or early falls

Question 7

What gene abnormality is found in Huntington disease?

Answer 7

More than 40 CAG repeats in the HD gene on chr 4

Question 8

What genes have been implicated in Parkinson disease?

Answer 8

SNCA (alpha-synuclein), PARK2 (E3 ubiquitin ligase), UCHL1 (ubiquitin carboxyterminal esterase L1) PINK1 (PTEN-induced putative kinase 1), and LRRK2 (leucine-rich repeat kinase 2)

Question 9

What PD patients are good candidates for deep brain stimulation?

Answer 9

Patients without significant active cognitive or psychiatric problems who have medically intractable motor fluctuations, intractable tremor, or intolerance of medication adverse effects

Question 10

What are hereditary causes of chorea?

Answer 10

Huntington disease, neuroacanthocytosis, and Wilson disease

Question 11

What is Marchiafava-Bignami syndrome?

Answer 11

Frontal-type dementia, seizures, and pyramidal signs; focal demyelination and necrosis of the corpus callosum. Due to alcohol consumption.

Question 12

What are typical signs of cerebellar degeneration?

Answer 12

Gait ataxia greater than limb ataxia, dysarthria, typically no nystagmus. Due to alcohol consumption.

Question 13

What is Lhermitte’s sign?

Answer 13

A tingling, electric shock sensation of the spine when the patient flexes the neck - seen in MS

Question 14

What areas of the brain does MS most commonly affect?

Answer 14

Periventricular white matter, juxtacortical regions, corpus callosum, and the cerebellar peduncles

Question 15

What change in visual evoked potentials can be used in suspected MS to document evidence of old optic neuritis?

Answer 15

Increased latency of the P100 wave on the affected side

Question 16

What is the cause of neuromyelitis optica (Devic disease)?

Answer 16

Antibodies to the aquaporin-4 channel. Characterized by transverse myelitis and optic neuritis. Poor prognosis with most patients developing paralysis and blindness in the long term

Question 17

What is posterior reversible encephalopathy syndrome (PRES)?

Answer 17

A leukoencephalopathy caused by rapidly developing hypertension and eclampsia or is due to immunosuppressants used in organ transplant (e.g. tacrolismus and cyclosporine). Characterized by acute confusional state and cortical visual loss (blindness with preserved pupillary reactivity).

Question 18

What is Wallerian degeneration? vs neuronal (axonal) degeneration?

Answer 18

Wallerian: Disintegration of axons and myelin distal to the site of injury.
Neuronal: distal dying of the axons and loss of myelin after damage to the cell body of the neurons

Question 19

What type of urinary incontinence is most commonly seen in MS?

Answer 19

Urge incontinence due to a spastic bladder

Question 20

What are common symptoms of progressive supranuclear palsy?

Answer 20

Parkinsonism, supranuclear impairment of eye movements (vertical gaze typically affected more prominently than horizontal gaze), and impaired postural reflexes

Question 21

Are the extrapyramidal features of idiopathic PD (e.g. resting tremor, rigidity) usually symmetric or asymmetric?

Answer 21

Asymmetric

Question 22

What language deficits and common to Alzheimer disease?

Answer 22

Decreased fluency, dysnomia, and transcortical sensory aphasia (reduction in ability to understand complex linguistic structures). Repetition remains intact

Question 23

What is general paresis?

Answer 23

It is a severe neuropsychiatric disorder caused by chronic meningoencephalitis that leads to cerebral atrophy in late-stage syphilis. Degenerative changes are associated primarily with the frontal and temporal lobar cortex. It is characterized by dementia, delusions, dysarthria, tremor, myoclonus, seizures, spasticity, and Argyll Robertson pupils

Question 24

What are Lewy bodies?

Answer 24

Intracytoplasmic, eosinophilic inclusions with poorly staining halos surrounding them

Question 25

How does speech change in Parkinson disease?

Answer 25

The clarity and volume decrease (develop hypophonia). Handwriting also becomes increasingly smaller and less legible (micrographia)