Exam 3: Motor Speech Disorders, Language, Higher-Order Functions

dysarthria

deficit in the control or EXECUTION of speech (related to strength, speed , range, accuracy of speech movements)

generally neurologic in orgin

can be categorized further into specific subtypes

apraxia of speech

deficit in PLANNING or programming speech movements

generally neurologic in origin

usually co-occurs with other conditions (aphasia or generic dysarthria)

flaccid dysarthria (what is it?, localization, neuromotor, etiology/cause of disease)

reduced ability to move —small, weak movements

localization/lesion: LMN (lower motor neuron)

• injury or malformation of the LMN “final pathway” to the neuromuscular junction

neuromotor: weakness

etiology/cause of disease = NMJ disease, Mysathenia Gravis, MS, Bell’s Palsy

phrenic nerves

nerves involved in respiration are spread from the cervical through thoracic divisions of the spinal cord
control the muscles needed for breathing

innervates diaphragm 3-5th cervical segments

• injuries to phrenic nerves —> can cause resp paralysis —> can lead to dysarthria

flaccid dysarthria clinical characteristics

clinical:

• weakness (paralysis)

• hypotonia

• diminished reflexes

• atrophy

• fasciculation (spontaneous muscles contractions)

• fibrillations (rapid, irregular heart rate)

flaccid dysarthria perceptual characteristics

perceptual:

• hyper nasality

• breathiness (continuous)

• nasal emission

• audible inspiration

• short phrases

spastic dysarthria (what is it?, localization, neuromotor, etiology/cause of disease)

• bilateral pyramidal UMN damage to pathways of CNS leading to LOSS OF INHIBITION

• loss of skilled movements, reduced range of motion

localization: bilateral UMN

• damage to the direct & indirect activation pathways+ pyramidal & extrapyramidal pathways

neuromotor: spasticity

etiology/causes of disease: Cerebral Palsy, infarcts of internal carotid/MCA/PCA, ALC, corticospinal or corticobulbar lesion

UMN: Pyramidal/Direct Pathways

consist of corticospinal & corticobulbar tracts

• corticospinal = control of limbs/trunk; decussation at the pyramids of the medulla

• corticobulbar = control of face, head, neck; decussation depending on CN

activation leads to discrete, skilled movements

lesions cause:

• weakness, loss of skilled movements

• babinski sign (loss of proper reflex, toes spread out instead of coming together)

UMN: Extrapyramidal/Indirect Pathways

major contributions from premotor areas

crucial connections with basal ganglia, cerebellum, retic form, vestibular nuclei

generally inhibitory— regulates reflexes for maintaining posture and tone

lesions cause:

• spasticity (inc resist to stretch)

• inc reflexes

• weakness

what patterns result from lesions to UMNs?

pyramidal & extrapyramidal damage (UMN)

• paralysis

• loss of skilled movement

• spasticity, loss of inhibition (specifcally extrapyramidal damage)

• increased and/or pathological reflexes

spastic dysarthria clinical characteristics

clinical/non- speech characteristics:

• dysphagia

• excessive, slow facial expressions

• pathological laughing and crying

• drooling

• facial posture fixed (unchanged)

• partial smile

*damage from pyramidal tract; bilateral

spastic dysarthria perceptual characteristics

speech/perceptual characteristics:

• harshness

• imprecise consonants

• low pitch

• slow rate

• strained-strangled quality

• short utterances

• pitch breaks

what is ataxia?

neurological condition characterized by the loss of coordination and control of muscle movement

due to damage in cerebellum

how does cerebellum modulate speech? how does damage to the cerebellum affect the body?

cerebellum:

• coordinates skilled movement (speed, range of muscular movements)

• part of the indirect pathway to LMNs (extrapyramidal support)

• receives input from the body and cortex; acts as an error controller (provides to the cortex)

impacts of damage:

• errors of force, speech, timing, and range —> difficulty in the coordination of movement

• IPSILATERAL control

  • R cortex, L cerebellum, L side of body

  • L cortex, R cerebellum, R side of body

ataxic dysarthria

results from cerebellar control circuit dysfunction; errors in coordination

localization: cerebellum

neuromotor: incoordination

etiology/causes of disease: cerebellar hemorrhage, Anoxia, Multiple Sclerosis

ataxic dysarthria clinical characteristics

clincal (non-speech):

• hypotonia (low muscle tone)

• slow voluntary movements

• jerky movements

• wide-based gait

• tremors

• paucity of movement

• muscular incoordination

• impairments of equilibrium

ataxic dysarthria perceptual characteristics

perceptual characteristics:

• slurring/stumbling over words (lack of error correction)

• “drunk” sounding speech

• irregular articulatory breakdowns

• irregular vowel distortions

major inputs and outputs of the basal ganglia (circuit)

caudate & putamen: input from cortex, output to globus pallidus

globus pallidus: input from caudate & putamen, output to the thalamus

thalamus: input from globus pallidus, output to cortex

major features of Parkinson Disease

• bradykinesia (slowness of movement; difficulty initiating movement)

• postural instability

• tremor

• rigidity

• cognitive deficits

• hypokinetic dysarthria

hypokinetic dysarthria clinical characteristics

• tremor at rest

• rigidity “cogwheel”

• bradykinesia (slow movements)

• hypokinesia (small movements)

• masked faces: loss of facial expression

• akinesia (lack of voluntary movement)

• swallow infrequently, drooling

• dysphagia (difficulty swallowing food)

hypokinetic dysarthria perceptual characteristics/non-speech related

• monopitch

• reduced stress

• monoloudness

• inappropriate silences

• short rushes of speech

• increased rate overall

dyskinesia

a movement disorder characterized by involuntary, uncontrolled muscles movements (general term)

types of dyskinesia

• hyperkinetic dysarthria

  • with etiologies of: athetosis, chorea, myoclonus

akinesia

inner sense of restlessness but lack of movement/freezing, difficulty initating movement

• hypokinesia (dec amplitude or poverty of movement)

• facial rigidity (stiffness in facial muscles)

• delayed responses

ballism

movement disorder characterized by involuntary, flinging, and often violent movements of the extremities

• large amplitude movements

• flinging/ throwing movements

• repetitive and varying

• usually unilateral (affects one side of the body)

tremors

involuntary movement

rhythmic, oscillatory movement (shakiness, trembling)

tics

involuntary movements

sudden, brief, and repetitive movements or sounds (not necessarily rhythmic like tremors)

hyperkinesia

EXCESSIVE involuntary movements, characterized by an overabundance of muscular activity

(hypokinesia refers to decreased/reduced movements)

hyperkinetic dysarthria? perceptual characteristics?

associated with diseases of the basal ganglia circuit, cerebellar circuit, indirect (extrapyramidal) system

abnormal, rhythmic, or irregular and unpredictable (rapid or slow) involuntary movements

perceptual

• visible abnormal orofacial, head, and respiratory movements

• variable rate

• inappropriate silences

• excess loudness variation

• prolonged intervals & phonemes expiration/inspiration

• transient breathiness

etiologies/causes of hyperkinetic dysarthria

• chorea (Huntington’s or non-Huntington’s)

• tics

• dystonias

• myoclonus

Huntington’s Disease

one type of hyperkinetic dysarthria etiology

characterized by chorea, dementia

• constant jerky movements, including facial movements, restless/fidgety hands

Chorea (not Huntington’s)

one type of hyperkinetic dysarthria etiology

“dance"- like purposeless, unpredictable movement

• due to reduced GABA

• multiple etiologies (Wilson’s Disease, Sydenham’s Chorea)

• speech (excessive range of movement, sustained vowel fluctuations)

Gilles de la Tourette’s Syndrome

one type of hyperkinetic dysarthria etiology

example of vocal tics

brief involuntary movements or sounds that occur over a background of normal motor activity

• simple or complex (simple or complex)

• echolalia (repeating what is heard)

• palilalia (compulsive repetition of phrases)

under partial voluntary control

dystonias (meige, oromandibular, spasmodic dystonia)

one type of hyperkinetic dysarthria etiology

slow hyperkinesia characterized by involuntary abnormal postures resulting from excessive co-contraction of antagonistic muscles

• Meige Syndrome= oromandibular dystonia + forceful, spasmodic closure of the eyes

• spasmodic dysphonia= focal dystonia with strained voice quality & stoppages

• slow initiation of speech

palatopharyngolaryngeal myoclonus

abrupt RHYTHMIC unilateral or bilateral movements of the soft palate, pharyngeal walls and laryngeal muscles

(not like chorea which is more random)

speech:

• intermittent hypernasality

• tremor- like variations

• momentary rhythmic arrests

athetosis

condition where muscle contractions cause involuntary writhing (twisting/squirming) movements of the limbs, neck, tongue and others muscle groups

inability to maintain a body part in a single position

damage to basal ganglia can cause which movement related diseases?

• Parkinson’s Disease (HYPOkinesia)

• Huntington’s Disease (HYPERkinesia)

flaccid dysarthria is due to damage in what area?

lower motor neuron (LMN)

spastic dysarthria is due to damage in what area?

upper motor neuron; pyramidal (volitional mvmt)

ataxic dysarthria is due to damage in what area?

upper motor neuron; extrapyramidal support (cerebellum)

unilateral upper motor neuron (UUMN) dysarthria

unilateral innervation = when one side of the brain controls one side of the body

(usually contralateral hemisphere of brain controls the opposite part of body)

BUT usually gets compensated by the unaffected side of the brain except for CNs controlling lower half of face

what cognitive functions are affected by lesions to frontal lobes?

executive functions, working memory, planning, inhibition/impulse control, speech production (Broca’s Area)

what cognitive functions are affected by lesions to mesial/medial temporal lobes?

new long-term, declarative memories, emotional processing, language

what cognitive functions are affected by lesions to basal ganglia and cerebellum?

basal ganglia= regulating movement, planning/sequencing, motivation/reward

cerebellum= motor coordination, language/speech, visual-spatial memory

what cognitive functions are affected by lesions to thalamus?

sensory integration, memory (episodic memory b/c connects to hippocampus), regulation of attention

four features that brain disorders tell us about healthy brains

• localization of cognitive functions

  • shown by analyzing lesion sites

  • different regions of the brain are specialized for different functions)

• onset and clinical course of the brains’s capacity to adapt to changing circumstances

• individual variability in symptoms and outcome

• recovery and neuroplasticity in health and disease

coup-contrecoup

type of TBI

injury to one side of head (i.e during fall) or injury to opposite side of head as the brain hits against opposite side of injury

acceleration/decelertion

a sudden stop causes head to go forward and then back, as in a car accident

edema

type of brain injury

swelling of brain tissue; causes pressure

hemorrhage

type of brain injury

rupturing of blood vessels

focal injury/lesions

type of brain injury

lesions just beneath the point of direct impact

sheering

type of brain injury

twisting of axons that causes trauma & diffuse white matter/axonal injury

Complications associated with TBI

• polytrauma (other body parts also affected)

• emergent care (life-threatening)

• medications

• cognitive issues

• behavioral issues

Glasgow Coma Scale

assesses how conscious or in coma an individual is after TBI

tests motor response, verbal response, and eye opening

high test scores are associated with more consciousness

TBI classification with Glasgow coma scale

mild = concussion; minor head/brain injury

moderate= injury resulting in loss of consciousness from 20 min- 6 hr

severe= loss of consciousness > 6 hr

Rancho Los Amigos Scale

assesses individuals after a brain injury based on cognitive and behavioral presentations as they change level of consciousness

usually done after a coma

mild-moderate: fatigue, headaches, visual disturbances, nausea, mood changes

severe: cognitive deficits, speech + language, sensory

TBI mangement/treatment

• initial/ emergent

  • stabilization

  • treat other injuries

  • monitor for infection

• acute care

  • assess level of function, stabilize medically

• rehabilitation (inpatient)

  • restore function

  • restore lost functional abilities

  • enhance independence

• surgical treatment

Goals of Rehabilitation

• domains of higher-function are assessed for strengths and weaknesses

• appropriate goals are developed based on functional need and discharge plan

• patient and family education

dementia

a syndrome of acquired and persistent declines in several abilities, often both short and long-term memory

other cognitive functions that are impacted:

• language/aphasia

• apraxia

• visuospatial skills

• emotion

Types of Dementia

• Alzheimer Disease

• Fronto-temporal Dementia

  • Pick’s Disease

  • Primary progressive aphasia

  • Semantic dementia

• Cortical and Subcortical Dementia

• Lewy Body Disease

Alzheimer Disease

type of dementia

criteria:

• gradual onset of cognitive deficits

• progressive

• not due to other CNS problems or substance abuse

70% of dementia cases

risk factors: age, genetic

AD Neuropathology

1) neurofibrillary tangles in the cytoplasm of nerve cells (which creates intracellular deposits)

2) neuritic plaques (remains of degenerated nerve fibers/extracellular deposits)

3) neuronal atrophy (loss of synapses)

impairments from AD from onset vs later progression

onset:

• difficulty in remembering recent evens (episodic memory)

• working memory impaired

later progression

• semantic memory impaired (naming, category knowledge, verbal fluency)

Pick’s Disease

type of Frontotemporal Lobe Dementia

onset: impulsiveness or lack of inhibition

cause: buildup of proteins in frontal and temporal lobes; accumulation of abnormal brain cells (called Pick’s bodies)

speech deficits

• weakened, uncoordinated speech sounds, echolalia (repeating words), decrease ability to read (alexia) or write (agraphia)

behavioral/neuro deficits

• impulsivity

• sexual promiscuity

• lack of empathy

• lack of coordination

distinguishing features in Pick’s Disease vs AD

more likely Pick’s Disease if more than 3+ of the 6 below:

• onset before 65

• personality changes at first

• loss of normal controls, e.g gluttony

• hypersexuality

• lack of inhibition

• roaming behavior

Lewy Body Disease

type of cortical and subcortical dementias

cause: protein deposits (Lewy bodies) in neuronal cell bodies

major causes and signs of of subcortical dementia

multiple stokes

commonly co-occurs with AD

signs: heterogenous depending on the location of the lesions

mild/early stage of dementia

• newly learned info is more difficult to remember than a remote event memories

• anomia (difficulty finding the right words)

• decreased comprehension of complex info

• personality changes: indifference, anxiety, or irritability

moderate/middle stage of dementia

• memory for recent and remote events is more severely damaged

• vocab diminishes

• repeating ideas

• restlessness

• personality changes are more pronounced

severe/late stage of dementia

• memory of all other intellectual functions are severely impaired

• very little meaningful language

• muteness

• dec motor function: rigidity

what are the communication deficits with dementia?

language

• semantics worsens over time

• problems with comprehension

pragmatics

• incoherent jargon

• unresponsive mute

also visual-perceptual and executive function deficits

Categories of Autism Spectrum Disorder

1) Autistic Disorder

2) Asperger’s Disorder

3) Pervasive Developmental Disorder- Not Otherwise Specified (PDD-NOS)

4) Rett’s Syndrome

5) Childhood Disintegrative Disorder

Autistic Disorder/ Autism

• impairments in social interaction, communication, and imaginative play prior to 3 years of age

Asperger’s

• impairments in social interaction

• presence of restricted interests and activities (limited interests)

Rett’s Syndrome

• progressive disorder occuring only in FEMALES

• characterized by a period of normal development followed by a LOSS OF SKILLS

• loss of purposeful use of hands is replaced by repetitive hand movements

Childhood Disintegrative Disorder

• Normal development for the first two years of life

• Acquired skills are then lost

Pervasive Developmental Disorder- Not Otherwise Specified (PDD-NOS)

“atypical autism”

over-used diagnostic label

severe and pervasive impairments but does not meet criteria for other diagnoses

major features of ASD

diagnostic criteria:

1) deficits in social communication and social interaction (social- emotional recip, nonverbal communication, understanding relationships)

2) restricted, repetitive patterns of behavior, interests or activities

speech and language characteristics with ASD

• verbal abilities vary widely— from no speech/language to minimal impairment

• preserved abilities/strengths

  • speech generally intelligible

  • generally syntactically appropriate

  • can imitate

developmental and behavioral characteristics with ASD

• infants show less attention to social stimuli; toddlers do not engage well in pretend play

• social impairments, lacking the intuition to understand to others

• conversational challenges (limited range of communication functions)

• older children and adults perform worse on tests of face and emotion recognition

2 main brain findings with Autism

1) increased brain volume (macrocephaly)

• atypical rapid brain head growth 2-4 years old

2) under-connectivity in autism

• functional connectivity: how two areas of the brain fire together

• structural connectivity: how the white matter tracts connect to different brain areas