Movement Disorders Flashcards
“Intention tremor” is another name for what condition?
Benign Essential Tremor
What is affected in benign essential tremors and what are spared?
Postural tremors: Involves both hands, head, or both
Legs are spared (usually only diaphragm and up)
What substance typically improves the benign essential tremor?
EtOH
What typically worsens/enhances the benign essential tremor?
Emotional stress
Inflammatory condition with an unknown cause, with multifocal demyelination of the white matter of the brain and spinal cord
Multiple Sclerosis
What demographic do you typically see multiple sclerosis?
Primarily disease of Caucasians or northern European descent (strong genetic component)
What age range is Multiple Sclerosis typically diagnosed/seen?
Typically found in the third decade (Ages 16-40)
Describe the pattern of Multiple Sclerosis
Relapsing-remitting pattern with chronic progressive course (Flare up then resolve)
Overall slowly getting worse over time
What are potential causes for relapse in MS?
Infection
Trauma
Pregnancy
What symptom(s) may be the first manifestation of Multiple Sclerosis?
Blurred, double, or loss of vision in a single eye
How is the diagnosis of Multiple Sclerosis made?
Clinical history, physical exam, and MRI findings
What is a key presentation of Multiple Sclerosis?
New neurological symptoms in a young person
List CSF Abnormalities seen in Multiple Sclerosis
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
What imaging is preferred in multiple sclerosis?
MRI more sensitive than CT (Demonstrates plaques = Dawson’s fingers)
CT scan can still show plaques
Describe the MRI findings in multiple sclerosis
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
Describe the condition Clinical Isolated Syndrome
A single clinical event looking like MS
Patient does not meet the criteria for MS
These patients are at risk for developing MS
How often do you monitor with MRI a patient with a clinical isolated syndrome event?
Follow up MRI every 6-12 months
What condition was previously known as Devic disease and once regarded as MS variant?
Neuromyelitis Optica
What finding can help tell apart Neuromyelitis Optica from Multiple Sclerosis?
white matter on MRI
MS will have white matter (Neuromyelitis Optica will not)
What is the specific antibody marker for Neuromyelitis Optica?
NMO-IgG
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
Parkinsonism
Parkinsonism is characterized by combination of these four cardinal signs:
Tremor at rest (pill rolling tremor)
Rigidity (cogwheeling)
Bradykinesia (difficulty initiating motion)
Progressive postural instability
What is the treatment goal in Parkinsonism?
Get more dopamine to the brain
What ethnic groups are most affected in Parkinsonism?
Occurs in all ethnic groups
List the four potential causes of parkinsonism (category)
Idiopathic
Genetic
Toxin exposure
Drugs
How does Parkinsonism progess?
Usually upper extremities, then as it progresses you’ll start to see LE involvement (shuffling for example)
What are the first and second line treatment for Parkinsonism?
First line treatment – dopaminergics
Second line treatment - anticholinergics
Chronic degenerative disorder of CNS that is an inherited autosomal dominant disorder
Huntington’s Disease
What are the Characteristics (3 manifestations) of Huntington’s Disease?
Involuntary movements = chorea
Progressive cognitive deterioration = dementia
Psychiatric symptoms (Psychosis; suicide is common)
What is the age of onset for Huntington’s Disease?
Onset age 30-50
Develops insidiously after age 30
What is the prognosis for Huntington’s Disease?
Fatal outcome – 15 to 20 years prognosis
What chromosome is affected in Huntington’s Disease?
Chromosome 4 affected
Atrophic caudate nucleus
GABA and Substance P decrease
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
Idiopathic Torsion Dystonia
What is the genetic mutation in Idiopathic Torsion Dystonia?
Mutation of CAG deletion in dominantly inherited disorders
What disorder is described below?
Spasmodic torticollis – neck twisting to one side
Onset 25-50 years of age
Focal Torsion Dystonia
What is the treatment in Focal Torsion Dystonia?
Botulinum A toxin
List some causes of Restless Leg Syndrome
Idiopathic
Pregnancy
Iron deficiency anemia
Peripheral neuropathy
Periodic leg movements of sleep
Restless Leg Syndrome is a diagnosis of exclusion. True or False?
True
Almost a diagnosis of exclusion – work patients up for underlying issues
What is the clinical presentation of Restless Leg Syndrome?
Restlessness and curious sensory disturbances that lead to urge the limbs to move
Disorder of multiple motor (80%) and phonic tics that is associated with OCD
Tourette’s Syndrome
When do symptoms of Tourette’s start?
Symptoms begin prior to age 21 years
Tics first noted between ages 2-15
What is the initial manifestation of Tourette’s and the most commonly seen?
tics
Most commonly of face
Imitation of others’ movements is called what?
Echopraxia
Repetition of speech of others is called what?
Echolalia
What is the drug of choice in acute Tourette’s Syndrome?
Haldol
What is the disorder of impaired motor functioning and posture that occurs prenatally, antenatally or during first year and is non-progressive?
Cerebral Palsy
What is the most common type of Cerebral Palsy?
Spastic diplegia (Normal intelligence)
