Movement disorders and aphasia

Question 1

Chorea

Answer 1

• hyperkinetic, Greek “to dance”
• Involuntary, abrupt, unsustained, irregular movements flow randomly from one body part to another.
• able to suppress partially and temporarily
• due to genetic disorder, immune, infection, toxic

Question 2

athetosis

Answer 2

is a slow form of chorea

characterized by more writhing, non-sustained and distal

Question 3

myoclonus

Answer 3

quick movement of muscle (sudden brief jerks): i.e. sleep jerks, hiccups
positive myoclonus: active mm contraction
negative myoclonus: brief lapse of mm contraction in active postural muscles followed by tightening of other muscles; different in nature than tic bcuz no associated “inner urge”
ex.)

hemi-facial spasm is a type of peripheral myoclonus characterized by frequent, involuntary muscle contractions on one side of the face caused by facial nerve injury, tumor, or nothing at all
(treat with botox or micro-vascular decompression (relieves pressure on the facial nerve)

Question 4

fasciculations

Answer 4

Fasciculation: Involuntary contractions or twitchings of groups of muscle fibers. Fasciculations can occur in normal individuals without an associated disease or condition, or as a result of illness, such as muscle cramps, nerve diseases, and metabolic imbalances

Question 5

dystonia

Answer 5

-sustained muscle contraction; frequently causes twisting, repetitive movement or abnormal posture
-the same group of muscles are repeatedly involved, simultaneous contraction of agonist and antagonist mm
-brought out by stress, voluntary movement, and fatigue
Jessica also added that if the pt is less than 26 years, probably a genetic disorder

five types: focal (affecting a single body part, treat with botox), segmental (affects two adjacent body parts), generalized (affects most of the body), multi-focal (involves two or more non-contiguous parts), and hemi-dystonia (Involves one-half of the body)

Tx: levodopa for young patients

Question 6

tremor

Answer 6

• is an unintentional, rhythmic oscillation of a body part in a fixed plane
• alternating or synchronous contractions of agonist and antagonist muscles.

Question 7

tic

Answer 7

• brief, intermittent movements(motor) or sounds (vocal/phonic tics)
• often coexist with ADHD and OCD

(vocal/phonic) motor in nature and involve respiratory, laryngeal, pharyngeal, oral and nasal musculature, and contraction of these muscles produce sounds thru nose, mouth or throat.

some movements commonly confused with tics:
tremors (rhythmic)
chorea (non-repetitive)
dystonia (non-repetitive, progressive in children), compulsions (inner urge)

Tx: behavioral intervention (habit reversal)

Question 8

bradykinesia

Answer 8

Bradykinesia is characterized as “slow, clumsy” and is one of the four key features of parkinson’s
rather than being a slowness in association, it is more a slowness in the execution of movement

Question 9

hyperkinesia

Answer 9

Hyperkinesia is an increase in muscle activity resulting in abnormal or excessive movements, or a combination of both

Question 10

tardive diskinesia

Answer 10

-delayed, abnormal movement induced by neuroleptics (need to get a list of current and past meds and discontinue any Rx typical antipsychotic, switch to atypical)

-facial, lingual, eyelid, and bulbar mm mostly involved (Jessica said a lot of times the tongue is involved)
symptoms will often improve over time after the drug is no longer taken

Question 11

asterixis

Answer 11

Asterixis is characterized by brief muscle contractions in active postural muscles followed by tightening of other muscles
also called the flapping tremor, or liver flap, is a tremor of the hand when the wrist is extended, sometimes said to resemble a bird flapping its wings.

Question 12

hemiballismus

Answer 12

-interrelated with chorea and may actually occur in the same patient
-characterized by more forceful, high-amplitude; flinging movements that are more proximal
more evident on one side than the other

Question 13

Tourette’s

Describe the etiology, diagnosis, treatment and prognosis f

Answer 13

-idiopathic
-critera to dx include:
onset before 18 yo
require both motor and vocal tics
have tics >1 year and may occur many times QD, every other day, or on and off

Question 14

Tremor types(4)

xplain how tremors are categorized as physiologic, pathologic, or functional (hysterical), and
provide examples of each.

• For benign essential (familial) tremor, discuss its prevalence, inheritance pattern, typical
clinical presentation, diagnosis, treatment, and prognosis.
• Describe findings typically associated with pathologic cerebellar tremors, and discuss tx options.

Answer 14

• rest tremor: occurs in a body part that is not activated and supported by gravity
• action tremor: occurs with voluntary muscle contraction (postural; isometric - against a rigid stationary object; kinetic - during action)
• physiologic tremor: benign, high frequency, and of low amplitude; amplified by fatigue, anxiety fear, stimulant use and medical conditions (hyperthyroidism)
• essential tumor: autosomal dominant; most common tremor in adults, unknown pathophysiology; exercaerbated by intentional activity (drinking from a cup) and inhibited by alcohol. approx. 60% of pts report (+) fmhx (check slide 35 for dx criteria); impairment of voluntary activities, mostly teens and 50s.
• cerebellar: slow tremor of extremities that occurs at the end of intentional movements caused by lesions in the cerebellum from stroke, tumor, disease, or excessive alcohol use. This tremor is improved with rest.
• parkinsonian: “pill rolling action” caused by damages to the brain that control movement. This typical arises around age 60, starts in one side of the body and slowly travels to the other. at rest

Question 15

Huntington’s disease

Answer 15

• Huntington’s chorea involves a loss of GABA-producing neurons in the basal ganglia and cortex.
• symptoms worsen steadily with 15 years being the typical duration from onset to death.
• Early presentation: change of personality; subtle abnormal movements (fidgety, restless, bradykinetic) and become more pronounced
• Advanced stage ( seldom still for more than a few seconds; voluntary movemnts slow, dementia)

Tx: chorea, depression, psychosis

involves face, head and neck, tongue, trunk and extremities
altered behavior (this one will apparently get you thrown in jail), depression, obsessive-compulsive features, and/or psychosis
dementia (90% demented before age 50)
irregular and unsteady gait
Huntington’s disease is due to a loss in GABA-producing neurons in the basal ganglia and cortex. HD is dx with MRI showing atrophy of the head of caudate nuclei. DNA testing confirms (I believe based on the number of CAG tri-nucleotide repeats in the Huntingtin gene. Treatment is directed at symptoms: Amantadine, tetrabenazine is used to treat chorea, typical/atypical neuroleptics are used to treat psychosis, and tricyclic antidepressants and SSRIs are used to treat depression. Symptoms worsen steadily, with 15 years typically being the length of time between onset and death.

Question 16

Essential tremor dx

Answer 16

-bilateral tremor of arms and forearms > 3 y
-no other neuro signs + family hx
may have head tremor with NO abnormal posture, respone o alcohol

Question 17

Parkinson
Describe the location and neurotransmitters of the neurons involved in Parkinsonism

List essentials in the diagnosis of primary idiopathic Parkinsonism (“Parkinson’s Disease”).

• Describe the etiology, differential diagnosis and stages of Parkinson’s Disease.
• .
• Explain how physical therapy and speech therapy can help a patient with Parkinsonism.

Explain how each of the following medications exerts a beneficial effect in treating
Parkinsonism: dopamine precursors (carbidopa:levodopa), dopamine agonists (bromocriptine,
pergolide), anticholinergic agents (benztropine, trihexyphenidyl), amantadine, and selegiline.

Answer 17

Parkinson’s results from a loss of dopamine-containing neurons located in the pigmented substantia nigra and locus coerulus in the midbrain. Imbalance od DA and AcH, leads to disfunction in basal ganglia and subsequently disruption in motor function

Dx:
1.bradykinesia 2.rigidity 3.tremor(resting tremor commonly) 4. postural instability. **bradykinesia is the hallmark of the disease and only one of the other three in addition to bradykinesia is necessary to be considered “PD

PD and and NOT other parkinsonism: if unilateral onset, response to levodopa, development of dyskineasia

Meds:
-dopamine precursors (levodopa): metabolic precursor of dopamine that crosses the BBB (unlike dopamine); must gradually increase dose to maintain therapeutic levels which has strong accompanying motor side effects

Cabidopa: dopamine decarboxylase inhibitor that does not cross BBB. Reduces peripheral metabolism of levodopa creating a relative/indirect increase in the amount of levodopa reaching the brain

Amantadine: early and mild disease

-dopamine agonists (bromocriptine, pergolide): used in Parkinsons treatment because although the dopamine releasing neurons have disappeared, postsynaptic dopamine receptors are present and functional

Anticholinergic agents (benztropine, trihexyphenidyl): less commonly used; muscarinic antagonist; side effects are dry mouth, constipation, urinary retention and confusion.

Amantadine: an anti-viral med that has been shown to stimulate the release of dopamine from neurons of the nigra striatum

Selegiline: Inhibits MOA-B resulting in increased levels of dopamine in the presynaptic neuron; used as an adjunct treatment for Parkinson’s disease

• Discuss newer surgical treatment options for patients with severe Parkinsonism.
Deep brain stimulation

Question 18

List the major signs of basal ganglia dysfunction

Answer 18

Damage to the basal ganglia cells may cause problems with one’s ability to control speech, movement, and posture. This combination of symptoms is called parkinsonism.

A person with basal ganglia dysfunction may have difficulty starting, stopping, or sustaining movement. Depending on which area is affected, there may also be problems with memory and other thought processes.

Question 19

List and briefly discuss examples of some focal dystonias.

Answer 19

Focal dystonia is a neurological condition that affects a muscle or group of muscles in a specific part of the body causing involuntary muscular contractions and abnormal postures. For example, in focal hand dystonia, the fingers either curl into the palm or extend outward without control. In musicians, the condition is referred to as Musician’s Focal Dystonia, or simply musician’s dystonia. In sports, it is commonly referred to as (the) yips.

Question 20

Describe causes of secondary Parkinsonism

Answer 20

medications, esp neuroleptics

Question 21

List several groups of medications that can produce a drug-induced tremor.

Answer 21

Mainly caused by neuroleptics(typical and atypical although atypical are less likely to cause tardive dyskinesia. Some anti-cholinergics aka anti-spasm medications commonly prescribed for COPD. Anti-eleptic drugs ex: phenobarbital, and “anti-parkinsons” aka levodopa

Question 22

Describe the role of the cerebellum in speech production.

Answer 22

Describe the role of the cerebellum in speech production.
The cerebellum coordinates and refines the planned motor impulses sent from the cortex based on the sensory information about the positions and conditions of the articulators and on prior practice on what the skilled target movement should be.
Adjustment in the timing and force of the movements to compensate for unexpected change in the circumstances of a movement may be influenced by connections of the cerebellum to the extrapyramidal system.
Therefore the cerebellum is able to coordinate and modify both planned and ongoing speech movements.

Question 23

Describe the primary characteristics of Wernicke’s aphasia.

Answer 23

Describe the primary characteristics of Wernicke’s aphasia.
Verbal comprehension of both written and verbal language is severely impaired. Those affected have difficulty understanding meaning of individual words and may not be able to follow any command consisting of more than one step. They exhibit fluent speech but it is marred by paraphasia (production of unintended syllables, words, or phrases during the effort to speak) and anomia (inability to produce names or people or objects). These pts typically produce jargon or nonsensical words.

Question 24

Differentiate between sensory aphasia and Wernicke’s aphasia.

Answer 24

Differentiate between sensory aphasia and Wernicke’s aphasia.
Transcortical sensory aphasia is similar to Wernicke’s aphasia with the exception of a strong ability to repeat words and phrases, such as repeating questions instead of answering them (echolalia). Think “Rainman” when repeats things that are said to him.

Question 25

Describe the symptoms of global aphasia.

Answer 25

Describe the symptoms of global aphasia.
Occurs when both expressive and receptive problems are present, as damage encompasses both Broca’s and Wernicke’s areas. Often appears acutely after a major infarction, hemorrhage, or TBI involving the dominant hemisphere. Pts are unable to say or understand more than a few words, are unable to read or write. Most severe form, as nearly all aspects of speech and language are affected.

Question 26

Differentiate between cortical, subcortical, primary progressive aphasia.

Answer 26

Differentiate between cortical, subcortical, primary progressive aphasia.
Cortical - Fluent: anomic, conduction, transcortical sensory, Wernicke; Non-fluent: Broca, global, mixed non-fluent, mixed transcortical (isolation syndrome), transcortical motor affect the cortex
Subcortical - striatal aphasia w/wo white matter extension, thalamic aphasia, anterior capsular/putaminal aphasia, posterior capsular/putaminal aphasia issues within cortico-cortical pathways, thalamus
Primary progressive aphasia - a degenerative brain condition; deterioration of brain tissue affecting areas of brain tissue affecting areas of the brain responsible for speech & language