Movement Disorders

Question 1

“Intention tremor” is another name for what condition?

Answer 1

Benign Essential Tremor

Question 2

What is affected in benign essential tremors and what are spared?

Answer 2

Postural tremors: Involves both hands, head, or both

Legs are spared (usually only diaphragm and up)

Question 3

What substance typically improves the benign essential tremor?

Answer 3

EtOH

Question 4

What typically worsens/enhances the benign essential tremor?

Answer 4

Emotional stress

Question 5

Inflammatory condition with an unknown cause, with multifocal demyelination of the white matter of the brain and spinal cord

Answer 5

Multiple Sclerosis

Question 6

What demographic do you typically see multiple sclerosis?

Answer 6

Primarily disease of Caucasians or northern European descent (strong genetic component)

Question 7

What age range is Multiple Sclerosis typically diagnosed/seen?

Answer 7

Typically found in the third decade (Ages 16-40)

Question 8

Describe the pattern of Multiple Sclerosis

Answer 8

Relapsing-remitting pattern with chronic progressive course (Flare up then resolve)

Overall slowly getting worse over time

Question 9

What are potential causes for relapse in MS?

Answer 9

Infection

Trauma

Pregnancy

Question 10

What symptom(s) may be the first manifestation of Multiple Sclerosis?

Answer 10

Blurred, double, or loss of vision in a single eye

Question 11

How is the diagnosis of Multiple Sclerosis made?

Answer 11

Clinical history, physical exam, and MRI findings

Question 12

What is a key presentation of Multiple Sclerosis?

Answer 12

New neurological symptoms in a young person

Question 13

List CSF Abnormalities seen in Multiple Sclerosis

Answer 13

Increased immunoglobulin levels (IgG)

Increased lymphocytes

Oligoclonal bands (Presence of bands are not specific as they are found in a variety of inflammatory neurological disorders)

Myelin basic protein may be elevated

Glucose usually normal

Question 14

What imaging is preferred in multiple sclerosis?

Answer 14

MRI more sensitive than CT (Demonstrates plaques = Dawson’s fingers)

CT scan can still show plaques

Question 15

Describe the MRI findings in multiple sclerosis

Answer 15

Hypointense “black holes” – permanent axonal damage (Black hole = dead tissue)

Hyperintense lesions are areas of inflammation - helps to identify new lesions

MS should not be diagnosed unless there is evidence of two or more
different regions of white matter that have been affected

Question 16

Describe the condition Clinical Isolated Syndrome

Answer 16

A single clinical event looking like MS

Patient does not meet the criteria for MS

These patients are at risk for developing MS

Question 17

How often do you monitor with MRI a patient with a clinical isolated syndrome event?

Answer 17

Follow up MRI every 6-12 months

Question 18

What condition was previously known as Devic disease and once regarded as MS variant?

Answer 18

Neuromyelitis Optica

Question 19

What finding can help tell apart Neuromyelitis Optica from Multiple Sclerosis?

Answer 19

white matter on MRI

MS will have white matter (Neuromyelitis Optica will not)

Question 20

What is the specific antibody marker for Neuromyelitis Optica?

Answer 20

NMO-IgG

Question 21

Adult onset neurodegenerative disorder of the extrapyramidal system

Dopamine depletion due to degeneration of dopaminergic nigrostriatal system leads to imbalance of dopamine and acetylcholine in the corpus striatum

Answer 21

Parkinsonism

Question 22

Parkinsonism is characterized by combination of these four cardinal signs:

Answer 22

Tremor at rest (pill rolling tremor)

Rigidity (cogwheeling)

Bradykinesia (difficulty initiating motion)

Progressive postural instability

Question 23

What is the treatment goal in Parkinsonism?

Answer 23

Get more dopamine to the brain

Question 24

What ethnic groups are most affected in Parkinsonism?

Answer 24

Occurs in all ethnic groups

Question 25

List the four potential causes of parkinsonism (category)

Answer 25

Idiopathic Genetic Toxin exposure Drugs

Question 26

How does Parkinsonism progess?

Answer 26

Usually upper extremities, then as it progresses you’ll start to see LE involvement (shuffling for example)

Question 27

What are the first and second line treatment for Parkinsonism?

Answer 27

First line treatment – dopaminergics Second line treatment - anticholinergics

Question 28

Chronic degenerative disorder of CNS that is an inherited autosomal dominant disorder

Answer 28

Huntington’s Disease

Question 29

What are the Characteristics (3 manifestations) of Huntington’s Disease?

Answer 29

Involuntary movements = chorea Progressive cognitive deterioration = dementia Psychiatric symptoms (Psychosis; suicide is common)

Question 30

What is the age of onset for Huntington’s Disease?

Answer 30

Onset age 30-50 Develops insidiously after age 30

Question 31

What is the prognosis for Huntington's Disease?

Answer 31

Fatal outcome – 15 to 20 years prognosis

Question 32

What chromosome is affected in Huntington's Disease?

Answer 32

Chromosome 4 affected Atrophic caudate nucleus GABA and Substance P decrease

Question 33

Inherited disorder with abnormal movements and postures that occurs sporadically or hereditary Dystonic movements of head and neck may take the form of torticollis, blepharospasm, facial grimacing, or forced opening and closing of mouth

Answer 33

Idiopathic Torsion Dystonia

Question 34

What is the genetic mutation in Idiopathic Torsion Dystonia?

Answer 34

Mutation of CAG deletion in dominantly inherited disorders

Question 35

What disorder is described below? Spasmodic torticollis – neck twisting to one side Onset 25-50 years of age

Answer 35

Focal Torsion Dystonia

Question 36

What is the treatment in Focal Torsion Dystonia?

Answer 36

Botulinum A toxin

Question 37

List some causes of Restless Leg Syndrome

Answer 37

Idiopathic Pregnancy Iron deficiency anemia Peripheral neuropathy Periodic leg movements of sleep

Question 38

Restless Leg Syndrome is a diagnosis of exclusion. True or False?

Answer 38

True Almost a diagnosis of exclusion – work patients up for underlying issues

Question 39

What is the clinical presentation of Restless Leg Syndrome?

Answer 39

Restlessness and curious sensory disturbances that lead to urge the limbs to move

Question 40

Disorder of multiple motor (80%) and phonic tics that is associated with OCD

Answer 40

Tourette’s Syndrome

Question 41

When do symptoms of Tourette's start?

Answer 41

Symptoms begin prior to age 21 years Tics first noted between ages 2-15

Question 42

What is the initial manifestation of Tourette's and the most commonly seen?

Answer 42

tics Most commonly of face

Question 43

Imitation of others’ movements is called what?

Answer 43

Echopraxia

Question 44

Repetition of speech of others is called what?

Answer 44

Echolalia

Question 45

What is the drug of choice in acute Tourette’s Syndrome?

Answer 45

Haldol

Question 46

What is the disorder of impaired motor functioning and posture that occurs prenatally, antenatally or during first year and is non-progressive?

Answer 46

Cerebral Palsy

Question 47

What is the most common type of Cerebral Palsy?

Answer 47

Spastic diplegia (Normal intelligence)