Movement disorders and functional neurological disorders

Question 1

Definition of FND

Answer 1

sensory, motor, cognitive, or speech problems not fully explained by known neurological diseases, wherein signs and symptoms are involuntary

Question 2

3 eras of evolution of FND

Answer 2

hysteria in the middle ages caused by a wandering uterus; conversion in the 20th century caused by past trauma; now FND with biopsychosocial origin

Question 3

Conversion (origin of FND)

Answer 3

past stress and psychological trauma so bad that people have to convert it into a more acceptable form (i.e. physical symptoms)

Question 4

Sex difference in FND

Answer 4

females > males

Question 5

Onset of FND

Answer 5

sudden onset following a trigger (e.g. stressor, injury, medical procedure) with frequent history of medically unexplained symptoms (e.g. IBS, chronic fatigue)

Question 6

Comorbidity of FND

Answer 6

high comorbidity with neurological and psychiatric disorders

Question 7

5 subtypes of FND

Answer 7

functional weakness, functional tremor, non-epileptic seizure, functional speech disorder, functional cognitive disorder

Question 8

Symptoms of functional weakness

Answer 8

face not affected; normal tone and reflexes; give-way weakness in arm; hoover’s sign in leg; dragging monoplegic gait

Question 9

Symptoms common in PNES (non-epileptic or functional seizure)

Answer 9

eyes closed; greater than 2 mins duration; asynchronous limb movement; stopping/starting; crying

Question 10

Symptoms rare in PNES

Answer 10

postictal disorientation; self-injury

Question 11

Symptoms of functional cognitive disorder

Answer 11

vivid memory of forgetting; complete amnesia of significant events; memory perfectionism

Question 12

Is FND a software or hardware problem?

Answer 12

software; due to a problem with functioning rather than lesions (e.g. hyperconnectivity between amygdala and PMC)

Question 13

Pathophysiological model of motor FND

Answer 13

psychosocial and biological vulnerability interact to develop brain network dysfunctions then FND symptoms

Question 14

Dysfunctions in FND

Answer 14

impaired emotional regulation (alexithymia); mismatch between predictions and sensory feedback (motor problems); impaired sense of agency

Question 15

Positive evidence of FND in a person

Answer 15

internal inconsistency, incompatibility, distractibility

Question 16

Neurophysiological ways of diagnosing FND

Answer 16

EEG for seizures and EMG coherence for tremor entrainment

Question 17

Hoover’s sign for functional weakness

Answer 17

unable to lift weak leg but displays an automatic compensatory contraction on the weak leg when the good leg is pushed down

Question 18

Tubular vision for functional blindness

Answer 18

same visual field size regardless of distance

Question 19

4 treatments for FND

Answer 19

delivery of the diagnosis as treatment; education about FND; psychological therapy (e.g. CBT, psychodynamic); physiotherapy (e.g. motor retraining)

Question 20

2 main kinds of movement disorders

Answer 20

parkinson’s and huntington’s

Question 21

Onset of parkinson’s

Answer 21

between ages 45 and 70

Question 22

Prevalence of parkinson’s

Answer 22

1% overall of people over age 65

Question 23

Which groups are of higher risk of getting parkinson’s?

Answer 23

males > females; white > black > asian; familial risk

Question 24

5 symptoms of parkinson’s

Answer 24

bradykinesia; rigidity; upper extremity resting tremor (initially unilateral then symmetric); postural instability with shuffling gait; expressionless or masked face

Question 25

Bradykinesia

Answer 25

slowing of speech, motor movement, thinking

Question 26

Rigidity

Answer 26

increased muscle tone/tightness at rest that stays fixed throughout range of motion (unlike spasticity); a sign of extrapyramidal motor dysfunction

Question 27

Prognosis of parkinson’s

Answer 27

insidious onset with a highly variable rate of progression and high risk of developing dementia after age 80 (>50%)

Question 28

Parkinsonism

Answer 28

conditions that share some symptoms and neuropathology as parkinson’s (e.g. Creutzfeldt-Jakob disease)

Question 29

Neuropathology of parkinson’s

Answer 29

substantia nigra degeneration (in basal ganglia); marked neuronal loss with remaining cells having lewy bodies

Question 30

3 ways to manage parkinson’s

Answer 30

L-dopa; dopamine agonists (e.g. bromocriptine); deep brain stimulation

Question 31

L-dopa

Answer 31

amino acid precursor that is converted to dopamine; very effective first dose but declines as disease progresses

Question 32

Epidemiology of huntington’s

Answer 32

affects 1-6 people per 100000 and is entirely hereditary (autosomal dominant)

Question 33

Autosomal dominant

Answer 33

if one parent has huntington’s and the other doesn’t, the child has a 50% chance of getting it too

Question 34

Which movement disorder is more rare?

Answer 34

huntington’s

Question 35

Prognosis of huntington’s

Answer 35

insidious onset of symptoms in 30s-50s with a progressive course over 10-15 years

Question 36

Clinical presentation of huntington

Answer 36

chorea, dystonia, subcortical dementia, early and prominent personality change

Question 37

Neuropathology of huntington’s

Answer 37

increased number of cytosine-adenine-guanine (CAG) trinucleotide repeats on chromosome 4; bilateral caudate degeneration; frontal-subcortical circuits

Question 38

Outcome of increased CAG on chromosome 4

Answer 38

protein misfolding then cell toxicity in the caudate

Question 39

Treatment for huntington’s

Answer 39

none

Question 40

How to diagnose huntington’s?

Answer 40

genetic testing