Common Movement disorders - Melanie Flashcards
what is Tremor
rhythmical, involuntary and oscillatory movement of body part
what is rhythmic
regular and repetitive in timing (though the amplitude may vary)
what is Oscillatory
moves about an axis
what is involuntary
does not go away (or often enhanced) with distraction
what is the physiology of a tremor
arise from an overactive or unchecked basal ganglia system i which neurons with spontaneous oscillatory activity override the normal balance of inhibitory vs excitatory transmission
what is the epidemiology of a tremor
most common movement disorder
most common action tremor is essential tremor
most common rest tremor is parkinson’s disease
what are the types of tremors
essential, physiologic, dystonic, orthostatic, cerebellar, homes, functional, etc
what is an essential tremor
generally bilateral, symmetrical postural/kinetic tremor. mostly seen in hands/arms. it is visible and persistent. can spread to head but less common in the legs
what is a physiologic tremor
natural tremor that every person has as a result of several factors including cardiac vibrations transmitted throughout the body, stretch reflexes generated from muscle contraction and natural oscillatory action of neuronal bodies in the brain.
what makes physiologic tremors worse?
worse with exercise, anxiety, hyperthyroidism, certain medications
what is the typical history presentation for essential tremors
slow, progressive nature of the tremor
+ FH
tremor with action/posture but not at rest
should not have other neurologic signs
tremor can be reduced with alcohol consumption
what are exam findings for essential tremors
tremor is usually bilateral and absent at rest. will see mild tremor with postural movements or kinetics
typically not seen in legs (should have normal gait, balance, romberg testing, reflexes)
what are diagnostics for essential tremors
spiral drawings with both hands - may see unidirectional tremor axis
handwriting is normal or large, no micrographia
what should patients absolutely NOT have with essential tremors
bradykinesia or rigidity on exam
what is a differential diagnosis for essential tremors
mild bilateral dystonic tremor or cerebellar tremor
what are labs or diagnostics used for tremor
no labs absolutely necessary
MRI/CT
EMG
when might patients obtain labs for tremors and what labs
might be indicated depending on history
(B12, renal function, LFTs, Hypocalcemia, hypoatremia, hyperthyroidism, hyperparathyroidism, serum ceruloplasmin, toxicity/exposures)
when are MRI/CT indicated for tremor
if there is neurological symptoms exist.
when are EMGs indicated for tremor
for difficult to differentiate tremor syndromes
what is the mainstay treatment for tremors
propranolol or primidone (level A).
Can be used alone or together.
how is propranolol prescribed and for what
for essential tremors
starting at 10mg TID
Not used in patients with severe asthma or COPD
what is primidone used for and how much
used to treat tremors - anticonvulsant/barbiturates.
needs to be tapered over 7 weeks and cannot stop suddenly.
what are the primary side effects of primidone
sedation, dizziness and CNS depression
what are level B treatments for tremors
alprazolam, atenolol, gabapentin, sotalol, topiramate
what is Kayser-fleisher rings
iron metabolism disorder
what are red flags for tremors
Kayser-Fleisher rings, any significant nystagmus, ataxia, weakness/hemiparesis, difficulties with EOMI, particularly upward gaze, significant dementia/behavioral issues, hepatic dysfunction
what are exacerbating factors for tremors
caffeine, beta-agonists, dopaminergic drugs, stimulants, thyrotoxicosis, psych drugs, SSRIs, valproate, amiodarone and toxicities
what do you need to look for in a functional tremor
- sudden onset
- static course
- multiple/mixed tremors
- changing amplitude/frequency
- entrainment
- distractibility
- sudden remission
what can functional tremors be concomitant with
depression/anxiety/somatic disorders and responds to psychotherapy and physical therapy
what are physiologic tremors
faster, but lower amplitude than essential tremor. Usually not with intention. can be present in voice/hands but not neck.
Neck tremor is always pathologic. Can be seen with anxiety.
what is parkinsons disease
progressive, degenerative motor and non-motor neurological disease characterized by the loss of dopaminergic neurons in the substantia nigra pars compacta and the presence of Lewy bodies
what are the common associated causes of parkinsons
genetics, environmental toxins, free-radical damage, alpha-synuclein aggregation. Links to melanoma and diabetes
(military vets with agent orange and/or burn pits)
what are the diagnostic criteria for PD
Bradykinesia and either rigidity or a rest tremor
and postural instability
what is bradykinesia
slowness of movement AND decrement in amplitude and/or speed of repeated movements
what is the Hoehn and Yahr scale
severity of PD on a 0-5 scale
what are supportive findings of PD
clear, dramatic response to levadopa
olfactory loss
what are exclusionary findings of PD
cerebellar abnormalities (ataxia, nystagmus, supranuclear gaze palsy, etc)
early, severe dementia or behavioral distubances
strictly lower limb involvement >3 years
normal DaT imaging
what are red flags of ruling out PD
severe autonomic failure in first 5 years of diagnosis
rapid gait impairment, early bulbar dysfunction (swallowing/speech), bilateral symmetric Parkinsonism, limited disease progression for 5 years
what is important to ask about with PD
presenting symptom?
when did it start?
how long has it occurred?
anosmia, constipation, urinary retention, acting out dreams, restless legs, increased saliva, hypophonia, no strong family history, exposure to pesticides, decreased hand dexterity, depression, sweating, seborrheic dermatitis, slowness, rigidity, dystonia
what is the exam for PD
observe the patient….
Masked face, slowed movements, resting tremor, mental status, Romberg/tandem walk, test extremities for rigidity, finger taps, hand opening, RAM, heel/toe taps, decreased decrements, decreased amplitude, standing from chair, gait, arm swing, tremors during walking, freezing of gait, festination, pull test
what are DaT scans used for
ruling in or ruling out parkinsonian diseases (not just idiopathic Parkinson’s disease)
how is PD diagnosed
clinical diagnosis
no labs or imaging diagnoses PD
what are the mainstay treatments for PD
carbidopa/levodopa
what is the best treatment to slow the progression of PD
Exercise and Monitoring symptoms
what are other treatment options for PD
MAOB inhibitors
Amantadine
Dopamine Agnoists
Anti-cholinergics
COMT inhibitors
DBS
what are the MAOB inhibitors and how to they work
treatment for PD
Selegine, Rasagiline
increase dopamine by interfering with dopamine re-uptake
good for any age, mild symptoms. can dose once/twice daily
what is Amantadine used for
treatment of PD
acts as dopamine agnoist - antiviral
works well for tremors
what are the dopamine agonists that are used to treat PD
Pramipexole, Ropinirole
what are anti-cholinergics used to treat PD
Trihexyphenidly (Artane), Benztropine - patients <65yo
what COMT inhibitors are used to treat PD
entacapone - taken with levodopa to maintain higher serum levels of dopamine
what needs to be watched for with any treatments of PD
hallucinations, impulse control problems or obsessive behaviors, motor fluctuations, dyskinesias, withdrawal (mental status change)
what are non-motor complications of PD
autonomic dysfunctions (constipation, urinary retention, sexual dysfunction, rhinorrhea, orthostatic hypotension)
depression/apathy/anxiety
psychosis
memory problems
sleep/fatigue
what are atypical parkinsonisms
MSA, PSP, CBD
Vascular parkinsonism
Drug induced parkinsonism
what is huntingtons disease
progressive, inherited neurodegenerative disease in an autosomal dominant patter (only need gene from one parent)
Caused by CAG trinucleotide repeat expansion
when is CAG considered abnormal
36-120 repeats
what is the epidemiology of Huntingtons disease
can be found in familial clusters. in US 6 per 100,000
what is the presentation of Huntingtons disease
movement, psychiatric and behavioral
most common movement is chorea
can be unaware of their own movements
hypotonia, hyperreflexia and dystonia common along with bradykinesia
inability to sustain prolonged muscle movements
progresses to spasticity, clonus and rigidity
what is chorea movements
dancing movement.
may look fidgety or restless and can include limbs, trunk and face
what is the psychiatric presentation of Huntingtons
can precede motor symptoms year prior to motor involvement
depression, irritability, apathy, withdrawn
hallucinations and paranoia
OCD and psychosis
high risk of suicide among patients as well as carriers
what is the cognitive presentations of huntingtons
usually present after motor and psychiatric symptoms
most common is executive dysfunction - problem with multi–tasking, making decisions.
lack insight into their condition and limitations
weight loss
what is a typical history presentation of hutningtons disease
unless adopted patient will have family member with known Huntingtons or an unknown movement, psychiatric problem
family/caregiver history collaboration vital as patients can lack insight
may report restlessness, dysarthria, dyphagia
short term memory dysfunction
what is seen on exam with Huntington’s disease
observation of movements
eye movements - delay
motor impersistence with tongue protrusion
tone
reflexes
gait
tics, myoclonus
how is huntingtons disease diagnosed
proven family hisotyr and exam
genetic testing for CAG repeats
what are the treatments for huntingtons diease
symptomatic management
hallucinations/psychosis
mood/depression
spasticity/hyperkinesia
dopamine depleting agens for chorea/ballism
