Movement Disorders Lecture Flashcards
Seen when there is NO muscle engagement/activity
(must hold up the pts affected body part to really determine)
Resting tremor
Tremor that is produced by voluntary muscle contraction
Action tremor
Tremor that is present during visually guided, target directed motion
ie..going to pick up a water bottle
Intention tremor
“normal” finding
high frequency, low amplitude postural tremor
enhanced by hyper-adrenergic states
fine, fast tremor. low amplitude. worse with anxiety
Physiologic tremor
MC movement disorder wordwide
aka benign familial tremor
generally seen in elderly (60+)
Benign essential tremor
Insidious development, slow progression
95% start with postural distal arm tremor
*unilateral, progresses to bilateral
almost ALWAYS upper extremity!!!
Benign essential tremor
Elder pt, slow progression, upper extremity (starts unilateral, moves bilateral)
..think of?
Benign essential tremor
Worsens with:
Stress, fatigue, CNS stimulants, voluntary activity
Better with:
ETOH, beta blockers, rest
Benign essential tremor
Treatment for benign essential tremor
contraindicated in asthma
SE: sedation, dizziness, nausea, mood changes
barbiurate, anticonvulsant
Primidone
Do you use beta blockers to treat benign essential tremor?
YES.
What will the neurological exam look like in a benign essential tremor?
Normal
Neurodegenerative disorder resulting from decreased dopaminergic transmission in basal ganglia
Parkinsons
(think dopamine when you hear Parkinsons)
4 cardinal signs:
- tremor
- rigidity
- bradykinesia
- postural impairment
Parkinsons
Tremor is present in 85% of cases
“pill rolling” 4-6 Hz resting tremor
one limb or one side of body for months to years
spares head
Parkinsons
Rigid gait
Flat affect/facial expressions
Tremor with rest and action
Parkinsons
Increased resistance to passive movements
“cogwheel rigidity”
NO weakness (5/5 in all extremities)
NO change in DTRs
Parkinsons
Bradykinesia (slowness of movements)
Effects voluntary movements and speech
*start hesitation..trouble taking that first step
Parkinsons
Parksinson pts have difficulty with balance and gait
..at what point in the disease process do you see this?
later in disease course
(worrisome if seen early. normally this takes long time)
What is “festinating gait” referring to?
The way a Parkinson’s pt walks…
short fast steps, trying to keep up with center of gravity. shuffling
What does turn “en bloc” refer to?
The way a Parkinson’s patient turns
freezing with direction change or when entering small space
“mask like” face
decreased blinking
dementia 6x normal
micrographia
hypophonia
depression
akinesia
Parkinsons
(non specific, just other findings)
Peak onset 60 (35-85)
Course 10-25 years
Males>Females
Some genetic predisposition
Parkinsons
Loss of melanin-containing, dopaminergic neurons in substantia nigra
Lewy bodies***- Pathological hallmark of PD when in basal ganglia (not specific)
Pathophysiology of Parkinsons
At the time Parkinsons symptoms appear, what levels might be depleted by 70%?
Dopamine
Gold standard tx of Parkinsons?
Levodopa
(converted to dopamine in the brain. dopamine itself cant cross BBB)
When do you start treating Parkinsons?
When functional disability starts
*medication benefit only works for a few years
What is a surgical tx option for Parkinsons?
Surgically implanted deep brain stimulation
blocks abnormal signals that cause PD symptoms
Autosomal dominant neurodegenerative disorder
Classic triad: motor, cognitive and psychiatric symptoms
insidious onset, no cure
age of onset: 30-50
Fatal in 15-20 years*
Huntington’s disease
Primary involuntary movement in Huntington’s Disease
“the dance”
Chorea
What is athetosis? (seen in Huntington’s)
Proximal limb writhing
Involuntary, irregular, rapid, uncontrolled, excessive movement
*often not noticed by, nor disturbing to, the patient
Chorea
Abnormal eye movements
Slow or uncoordinated fine motor control
Dyarthria
Dysphagia
Gait disturbance
Bradykinesia and rigidity late in course (as chorea peaks and wanes)
Huntington’s
Psychiatric symptoms:
Depression
Mania
OCD
Apathy
Anxiety
Huntington’s disease
Cognitive symptoms:
Dysfunction of executive functions
Problems with plannig, judgement, emotion, regulation, learning, attention, etc.
Cant follow instructions
Temper outbursts
Difficulty with spatial perception
Huntington’s
decreased GABA
decreased Ach
increased dopamine
Huntingtons
Excessive number of “CAG” sequence repeats
Huntingtons
How do you treat Huntingtons?
Symptomatically
(Neuroleptics, Benzos, Dopamine depleting agents)
MAJOR potential complication of Huntingtons?
Rhabdomyolysis
(breakdown of muscle tissue that releases damaging protein into blood)
Inherited neurobehavioral disorder characterized by sudden involuntary, repetitive muscle movements and vocalizations
Tourette’s
For a diagnosis of Tourette’s…
Tic episodes several times a day, almost every day, or periodically during period longer than….
1 year
Where does Tourette’s start/originate?
Head and face
(ie blinking, face twitch, head jerk, shrug, neck stretch, sniffing)
Meaningless repitition or imitation of movements
*seen in Tourette’s patients
Echopraxia
Palilalia
repeating one’s own words
Repeating heard words-last words spoken by someone else
Echolalia
Coprolalia
the involuntary and repetitive use of obscene language
movement disorder in which involuntary sustained or intermittent muscle contractions cause twisting and repetitive movements, abnormal postures or both
diagnosed in the young!
Cerebral palsy
Non-traumatic fractures can occur due to extreme hyper flexion or hyper extension
Cerebral Palsy (CP)
“burning” sensation
generally starts in distal extremities (MC feet) and moves proximally
Neuropathy
Hallmark= VERY painful with light touch
Hypersensitive
pain out of proportion
Neuropathy
Compression at the superior thoracic outlet resulting from excess pressure places on a neurovascular bundle passing between the anterior scalene and middle scalene muscles
Thoracis outlet syndrome
Positive Adson’s sign
Costoclavicular movement
seen in…
Thoracis Outlet Syndrome
Intermittent, progressive chronic disorder involving aberrant firing of trigeminal nerve-
usually 2nd and 3rd branches
Unilateral lancing paroxysmal facial pain
Trigeminal neuralgia
Triggers= light touch!!
but NOT firm pressure
Neuropathy
What will a neuro exam look like in trigeminal neuralgia?
NORMAL! except for illicit pain with light touch
Drug class for treatment of trigeminal neuralgia?
Anti-convulsants
Highest risk location of shingles?
Trigeminal (especially ophthalmic division..can cause blindess)
Brachial plexus
A term applied to a number of genetic disorders that produce progressive degeneration and necrosis of skeletal muscle fibers and eventual replacement with fat and connective tissue
Muscular dystrophy
Does muscular dystrophy involve the nervous system?
NO..disease of muscle tissue!
A disorder of the neuromuscular junction that affects impulse transmission between the motor neuron and the innervated muscle cell
Myasthenia Gravis
An acute life threatening polyneuropathy
rapid progressive limb weakness and LOSS of tendon reflexes
MC cause of acute, flaccid paralysis in developed countries
Guillain-Barre syndrome
An acute onset immune-mediated demyelinating neurpathy
progressive ascending muscle weakness of limbs, producing a symmetric flaccid paralysis
Symptoms: parasthesia and numbness, often accompany the loss of motor function
Guillain-Barre syndrome
What is the Myerson Sign and what disorder is it seen in?
Sustained eye blinking in response to taping above nasal bridge
seen in Parkinson’s
Can you tx trigeminal neuralgia with Carbamazepine?
YES
