Flashcards in Ch. 8: Neurologically Based Communicative Disorders & Dysphagia Deck (124):
A neurologically-based language disorder caused by various types of neuropathologies (most commonly stroke). Can be classified as fluent, nonfluent, and subcortical. May or may not be accompanied by alexia, agraphia, or agnosia.
Caused by a blocked or interrupted blood supply to the brain. Blockage or interruption may be caused by two kinds of arterial diseases: thrombosis or embolism.
A collection of blood material that blocks the flow of blood.
A traveling mass of arterial debris or a clump of tissue from a tumor that gets lodged in a smaller artery and thus blocks the flow of blood.
Caused by bleeding in the brain due to ruptured blood vessels. Ruptures may be intracerebral (within the brain) or extracerebral (within the meninges, resulting in subarachnoid, subdural, and epidural varieties).
Primary Intracranial Tumors
Tumors that grow from within the brain.
Aphasia characterized by limited, agrammatic, effortful, halting, and slow speech with impaired prosody. Includes Broca’s, Transcortical Motor Aphasia (TMA), Mixed Transcortical Aphasia (MTA), and Global Aphasia.
Nonfluent variety of aphasia caused by damage to Brodmann’s areas 44 and 45 in the posterior inferior frontal gyrus of the left hemisphere of the brain. Characterized by nonfluent, effortful, slow, halting, and uneven speech, limited word output, short phrases and sentences, misarticulated or distorted sounds, agrammatic or telegraphic speech, impaired repetition, impaired naming (especially confrontation naming), better auditory comprehension than production, difficulty in understanding syntactic structures, poor oral reading, poor comprehension of material that has been read, writing problems, and monotonous speech. Patients may also exhibit apraxia of speech and dysarthria. Patients may have right-sided paralysis or paresis. Some patients may be depressed or act emotionally when confronted with difficult tasks.
Transcortical Motor Aphasia (TMA)
Nonfluent variety of aphasia caused by lesions in the anterior superior frontal lobe, often below or above Broca’s area, which is not affected. Characterized by speechlessness, echolalia, perseveration, absent or reduced spontaneous speech, nonfluent, paraphasic, agrammatic and telegraphic speech, intact repetition skills (distinguishing characteristic of TMA), awareness of grammaticality, refusal to repeat nonsense syllables, unfinished sentences, limited word fluency, simple and imprecise syntactic structures, attempts to initiate speech with the help of motor activities, good comprehension of simple conversation, slow and difficult reading aloud, and seriously impaired writing. Patients tend to exhibit rigidity UE, akinesia, bradykinesia, buccofacial apraxia, and weakness of the legs. Patients with TMA may experience apathy, withdrawal, and little interest in communication.
Mixed Transcortical Aphasia (MTA)
Rare variety of nonfluent aphasia caused by lesions in the arterial border zone of the brain. Characterized by limited spontaneous speech, automatic, unintentional, and involuntary nature of communication, severe echolalia, severely impaired fluency, severely impaired auditory comprehension, marked naming difficulty and neologism, mostly unimpaired automatic speech, severely impaired reading, reading comprehension, and writing. Symptoms vary between patients. Patients may have bilateral upper motor neuron paralysis, quadriparesis, and visual field deficits.
Most severe form of nonfluent aphasia. Caused by extensive lesions affecting all language areas (perisylvian region). Characterized by profoundly impaired language skills, greatly reduced fluency, limited expressions, impaired repetition, impaired naming, auditory comprehension limited to single words, perseveration, and impaired reading and writing. Patients may have verbal and nonverbal apraxia, and strong neurological symptoms, such as right-sided paresis or paralysis, right-sided sensory loss, and neglect of the left side of the body.
Aphasias characterized by relatively intact fluency but generally less meaningful, or even meaningless, speech. Speech is generally flowing, abundant, easily initiated, and well-articulated with good prosody and phrase length. Includes Wernicke’s, Transcortical Sensory Aphasia (TSA), and Conduction Aphasia.
Common variety of fluent aphasia caused by lesions in the posterior portion of the superior temporal gyrus in the left hemisphere of the brain. Characterized by effortlessly produced fluent speech, rapid rate of speech with normal prosodic features and good articulation, intact grammatical structures, severe word-finding problems, semantic and literal paraphasias, extra syllables in words, creation of meaningless words (neologisms), circumlocution, empty speech, poor auditory comprehension, impaired conversational turn taking, impaired repetition, reading comprehension problems, writing problems, and overall poor communication despite fluent speech. Less frustrated with failed communication attempts. Sometimes mistaken for psychiatric patients. Generally free from neurological symptoms and paresis and paralysis are uncommon.
Transcortical Sensory Aphasia
A variety of fluent aphasia caused by lesions in the temporoparietal region of the brain. Characterized by fluent speech with normal phrase length, good prosody, normal articulation, and appropriate grammar and syntax, paraphasic and empty speech, severe naming problems and pauses due to those problems, good repetition, poor comprehension, echolalia, impaired auditory comprehension, difficulty pointing, difficulty answering yes/no questions, normal automatic speech (e.g., counting), tendency to complete poems and sentences started by the clinician, good oral reading but poor comprehension, and writing problems parallel to their expressive speech. Neglect may be common. Similar to Wernicke’s aphasia, but repetition is intact in this aphasia.
Rare variety of fluent aphasia caused by lesions in the area between Wernicke’s and Broca’s areas of the brain. Characterized by disproportionate impairment in repetition (a distinguished impairment), variable speech fluency across patients, paraphasic speech, marked word-find problems (especially content words), empty speech because of omitted content words, efforts to correct speech, good syntax, good prosody, good articulation, severe to mild naming problems, near-normal auditory comprehension, variable reading problems, writing problems, and buccofacial apraxia. Similar to Wernicke’s aphasia, except patients with this aphasia have good to normal auditory comprehension. Some may have right-sided paresis or sensory impairment.
Variety of fluent aphasia characterized by a very debilitating and pervasive word-finding difficulty (most distinguished feature), pointing to named objects is unimpaired, generally fluent speech, normal syntax except for pauses, empty speech, verbal paraphasia (word substitutions), circumlocution, good auditory comprehension, intact repetition, good articulation, normal oral reading skills and good reading comprehension, and normal writing skills.
Aphasia caused by lesions to either the basal ganglia or thalamus within the left hemisphere of the brain. Symptoms depend on location of lesion.
Subcortical Aphasia (Left Basal Ganglia)
Type of subcortical aphasia characterized by fluent speech, intact repetition skills, normal auditory comprehension, articulation problems, prosodic problems, word-finding problems, semantic paraphasia, relatively preserved writing skills, and limb apraxia if the lesions extend posteriorly to deep white matter in the parietal lobe.
Subcortical Aphasia (Left Thalamus)
Type of subcortical aphasia characterized by hemiplegia, hemisensory loss, right visual field problems, sometimes coma, initial mutism which may improve, severe naming problems, good auditory comprehension, good repetition skills, and impaired reading and writing skills.
A loss of previously acquired reading skills due to recent brain damage.
Loss or impairment or normally acquired writing skills due to lesions in the foot of the second frontal gyrus of the brain, sometimes referred to as Exner’s writing area.
Impaired understanding of the meaning of certain stimuli even though there is no peripheral sensory impairment. Patients can see, feel, and hear stimuli but cannot understand their meaning. Impairment is often limited to one sensory modality. The meaning of stimuli may be grasped in another modality.
Associated with bilateral damage to the auditory association area. Characterized by impaired understanding of the meaning of auditory stimuli, normal peripheral hearing, difficulty in matching objects with their sound, and normal visual recognition of objects.
Auditory Verbal Agnosia
Associated with bilateral temporal lobe lesions that isolate Wernicke’s area. Often called pure word deafness. Characterized by impaired understanding of spoken words, normal peripheral hearing, normal recognition of nonverbal sounds, normal recognition of printed words, and normal or near-normal verbal expression and reading.
A rare disorder often associated with bilateral occipital lobe damage or posterior parietal lobe damage. Characterized by impaired visual recognition of objects, which may be intermittent, and normal auditory or tactile recognition of objects.
Associated with lesions in the parietal lobe. Characterized by impaired tactile recognition of objects when visual feedback is blocked (as with a blindfold), impaired naming objects clients can feel in their hands, and impaired description of objects clients can feel in their hands.
Apraxia of Speech
A neurogenic speech disorder characterized by sensorimotor problems in positioning and sequentially moving muscles for the volitional production of speech. Primarily an articulatory-phonologic disorder, although its etiology and characteristics are different from those of similar disorders in children. Adult patients will have acquired articulation normally, with current problems being due to recent neuropathology. Patients have unimpaired reflex and automatic acts. Difficulty is mostly in executing the voluntary movements involved in speech. May be associated with prosodic problems. Not caused by muscle weakness or neuromuscular slowness. Thought to be a disorder of motor programming.
A basic disorder of volitional movement in the absence of muscle weakness, paralysis, or fatigue.
Nonverbal Oral Apraxia
A disorder of nonverbal movement involving the oral muscles.
Neurologically based speech disorders, distinct from similarly based language disorders such as aphasia. Distinct from apraxia of speech, a neurogenic speech disorder, or motor planning/programming of speech movements with no muscular weakness or paralysis. Oral communication problems that accompany dysarthria include respiratory, articulatory, phonatory, resonatory, and prosodic disturbances that are caused by weakness, incoordination, or paralysis of speech musculature. Different types share certain characteristics. Impaired muscular control of the speech mechanism and peripheral or central nervous system pathology are common to all forms. Differences in the nature and loci of pathology create different forms of the disorder. Etiological factors include degenerative neuromuscular diseases and nonprogressive neurological conditions. Common lesion sites include the lower motor neuron, unilateral or bilateral upper motor neuron, cerebellum, and basal ganglia (extrapyramidal system). Problems include:
- Muscle weakness
- Variety of movement disorders (reduced or variable range and speed of movement, involuntary movements, reduced strength of movement, unsteady or inaccurate movement)
- Abnormal muscle tone (increased, decreased, or variable)
Dysarthria that results from damage to the cerebellar system. Characterized predominantly by articulatory and prosodic problems. Neuropathology includes cerebellar lesions, degenerative ataxia, cerebellar vascular lesions, tumors, TBI, toxic conditions, and inflammatory conditions. Major characteristics include:
- Gait disturbances (instability of the trunk and head; tremors and rocking motions; rotated or tilted head posture; hypotonia)
- Movement disorders (over- or undershooting of targets; uncoordinated, jerky, inaccurate, slow, imprecise, and halting movements)
- Articulation disorders (imprecise production of consonants; irregular articulatory breakdowns and distortion of vowels)
- Prosodic disorders (excessive and even stress; prolonged phonemes and intervals between words or syllables; slow rate of speech)
- Phonatory disorders (monopitch, monoloudness, and harshness)
- Speech quality (impression of drunken speech)
Dysarthria resulting from damage to the motor units of cranial or spinal nerves that supply speech nerves (lower motor neuron involvement). Neuropathology includes myasthenia gravis, botulism, vascular diseases, brainstem strokes, infections, demyelinating diseases, degenerative diseases, and surgical trauma to the brain, larynx, face, or chest. Characteristics include:
- Various muscular disorders
- Fasciculations and fibrillations
- Respiratory weakness, CN weakness
- Phonatory disorders (breathy voice, audible inspiration, short phrases)
- Resonance disorders (hypernasality, imprecise consonants, nasal emission)
- Phonatory-prosodic disorders (harsh voice, monopitch, monoloudness)
- Articulation disorders
Isolated twitches of resting muscles.
Contractions of individual muscles.
Dysarthria resulting from damage to the basal ganglia (extrapyramidal system). Caused by degenerative, vascular, traumatic, infectious, neoplastic, and metabolic factors. Associated with involuntary movement and variable muscle tone. Prosodic disturbances are prominent. Characterized by:
- Movement disorders (abnormal and involuntary movements of orofacial muscles)
- Myoclonus, tics of the face and shoulders, tremor, chorea, abrupt and severe contractions of the extremities, writhing movements, spasms
- Dystonia, spasmodic torticollis, blepharospasm
- Communicative disorders depending on the dominant neurological conditions
- Phonatory disorders (voice tremor, intermittently strained voice, voice stoppage, vocal noise, harsh voice)
- Resonance disorders (intermittent hypernasality)
- Prosodic disorders (slower rate, excess loudness variations, prolonged interword intervals, equal stress)
- Respiratory problems (audible inspiration, forced and sudden inspiration and/or expiration)
- Inconsistent articulation problems, including imprecise consonant productions and distortion of vowels
Involuntary jerks of body parts.
Writhing, involuntary movements.
Sudden and involuntary contractions of a muscle or group of muscles.
Abnormal postures resulting from contractions of antagonistic muscles.
Intermittent dystonia and spasms of the neck muscles.
Forceful and involuntary closure of the eyes due to spasm of the orbicularis oris muscle.
Dysarthria resulting from damage to the basal ganglia (extrapyramidal system). Caused by degenerative diseases (commonly PD) and vascular disease (strokes), head injury, inflammation, tumor, drug toxicity, and hydrocephalus. Characterized by:
- Tremors in resting facial, mouth, and limb muscles that diminish when moved voluntarily
- Mask-like face with infrequent blinking and no smiling
- Micrographic writing
- Walking disorders
- Postural disturbances (involuntary flexion, difficulty changing positions)
- Phonatory disorders, such as monopitch, low pitch, monoloudness, and harsh and breathy voice
- Decreased swallowing (accumulation of saliva in mouth and drooling)
- Prosodic disorders, such as reduced stress, inappropriate silent intervals, short rushes of speech, variable and increased rate in segments, and short phrases
- Articulation disorders, such as imprecise consonants, repeated phonemes, resonance disorders, mild hypernasality
- Respiratory problems, including reduced vital capacity, irregular breathing, and faster rate of respiration
Dysarthria resulting from bilateral damage to the UMN (direct and indirect motor pathways). Typically from lesions in multiple areas, commonly the cortical areas, basal ganglia, internal capsule, pons, and medulla. Characterized by:
- Spasticity and weakness (bilateral face weakness), jaw strength may be normal and lower face weakness may be mild
- Movement disorders, including reduced range and slowness, loss of fine and skilled movements, increased muscle tone
- Hyperactive gag reflex
- Hyperadduction of VF, inadequate closure of VP port
- Prosodic disorders, including excess and equal stress, slow rate, monopitch, monoloudness, reduced stress, short phrases
- Articulation disorders, including imprecise production of consonants and distorted vowels
- Phonatory disorders, including continuous breathy voice, harshness, low pitch, pitch breaks
- Resonance disorders with a predominant hypernasality
A combination of two or more pure dysarthrias. All combinations are possible. Two most common forms:
- Flaccid-spastic dysarthria
- Ataxic-spastic dysarthria
Mixed Flaccid-Spastic Dysarthria
Mixed dysarthria characterized by imprecise production of consonants, hypernasality, harsh voice, slow rate, monopitch, short phrases, distorted vowels, low pitch, monoloudness, excess and equal stress or reduced stress, prolonged intervals, prolonged phonemes, a strained and strangled quality, breathiness, audible inspiration, inappropriate silences, and nasal emission.
Mixed Ataxic-Spastic Dysarthria
Mixed dysarthria characterized by impaired loudness control, harsh vocal quality, imprecise articulation, impaired emphasis, hypernasality, inappropriate pitch levels, and sudden articulatory breakdowns.
Unilateral Upper Motor Neuron Dysarthria
Dysarthria resulting from damage to the UMNs that supply cranial and spinal nerves involved in speech production. LH lesions → dysarthria + aphasia or apraxia. RH lesions → dysarthria + RH syndrome. Characterized by:
- Unilateral lower face weakness, unilateral tongue weakness, unilateral palatal weakness, and hemiplegia/hemiparesis
- Articulation disorders, including imprecise production of consonants and irregular articulatory breakdowns
- Phonatory disorders, including harsh voice, reduced loudness, and strained harshness
- Prosodic disorders, including slow rate, increased rate in segments, excess and equal stress, monopitch, monoloudness, low pitch, short phrases
- Resonance disorders, predominantly hypernasality
- Dysphagia, aphasia, apraxia, and right hemisphere syndrome
Treatment Goals for Dysarthria
Treatment goals include modification of respiratory, phonatory, articulatory, resonatory, and prosodic problems and increasing efficiency, effectiveness, and naturalness of communication. Also include increasing physiologic support for speech and teaching self-correction, self-evaluation, and self-monitoring skills. Teaching compensatory behaviors for lost or reduced functions is important, and teaching the used of AAC may be necessary.
Treatment Procedures for Dysarthria
Treatment procedures include intensive, systematic, and extensive drill, instruction, demonstration, modeling (followed by imitation), shaping, prompting, fading, differential reinforcement, and other proven behavioral management procedures. When necessary, phonetic placement and its variations can be taught. Instrumental feedback or biofeedback may be used when needed.
Modification of Respiration
- Training, with the help of a manometer or air pressure transducer, consistent production of subglottal air pressure
- Training maximum vowel prolongation
- Shaping production of longer phrases and sentences
- Teaching controlled exhalation
- Teaching the client to push, pull, or bear down during speech or nonspeech tasks
- Using a manual push on the client’s abdomen
- Modifying postures that promote respiratory support, including using neck and trunk braces if helpful
- Teaching the client to inhale more deeply and exhale slowly and with greater force during speech
Modification of Phonation
- Using biofeedback to shape desirable vocal intensity
- Training the client in the use of portable amplification systems if the voice is too soft
- Training aphonic clients in the use of an artificial larynx
- Teaching the client to initiate phonation at the beginning of an exhalation
Modification of Resonance
- Providing feedback on nasal airflow and hypernasality by using a mirror, nasal airflow transducer, or nasendoscope
- Training the client to open the mouth wider to increase oral resonance and vocal intensity
- Using a nasal obturator or nose clip
Modification of Articulation
- Training the client to assume to best posture for good articulation
- Using a bite block to improve jaw control and strength
- Using such methods as simplifying the target, instruction, demonstration, modeling, shaping, and immediate feedback in teaching correct articulation
- Using phonetic placement, slower rate, and minimal contrast pairs
- Providing instructions and demonstrations and teaching self-monitoring skills
- Teaching compensatory articulatory movements (e.g., use of a tongue blade to make sounds normally made with tongue tip)
Modification of Speech Rate
- Using delayed auditory feedback (DAF), a pacing board, an alphabet board, a metronome, hand or finger tapping
- Reducing excessive pause durations in speech
Modification of Prosody
- Reducing speech rate
- Teaching appropriate intonation
Modification of Pitch
- Instruction, modeling, differential feedback on pitch
Modification of Vocal Intensity
Modeling, shaping, and differential reinforcement of:
- Greater inhalation
- Increased laryngeal adduction
- Wider mouth opening
Caused by prolonged alcohol abuse. Characteristics include impaired learning, reduced memory, difficulty with visual-spatial integration, and difficulty processing abstract information. Often related to dementia.
An acquired neurological syndrome associated with persistent of progressive deterioration in intellectual functions (cognition, visuospatial skills), language, memory, emotion, and personality. Memory impairment must be evident in order to diagnose. Typically progressive, sometimes reversible. Classification is controversial.
Dementia of the Alzheimer Type (DAT)
Dementia associated with Alzheimer disease. Form of cortical dementia. Intellectual and language deterioration precedes motor deficits. Subcortical → motor deteroration before intellectual deterioration. Typical onset in 70s and 80s. Affects women more often than men. Associated with history of DAT, brain injury, or a family history of Down syndrome. Neuropathology includes:
- Neurofibrillary tangles
- Neuritic plaques
- Neuronal loss
- Neurochemical changes
Filamentous structures in the nerve cells, dendrites, and axons.
Occur with neurofibrils become thickened, twisted and tangled. Form unusual loops and tangles.
Minute areas of cortical and subcortical tissue generation. Cerebral cortex and hippocampus (concerned with memory) are very vulnerable. Result is destroyed cortical and subcortical synaptic connections.
Nerve cells are destroyed by fluid-filled cavities containing granular debris. Results in a shrunken brain. Shrinkage is most obvious in cerebral hemispheres, especially in temporal and parietal lobes.
Neurochemical Changes in Dementia
Depletion of neurochemicals that help transmit messages across brain structures. Severely depleted neurochemicals include:
Symptoms of Early-Stage DAT
- Subtle memory problems (especially for remote events)
- Somewhat pronounced difficulty with new learning and visuospatial problems (e.g., copying 3-D figures)
- Poor reasoning and judgment in social situations
- Behavior changes, including self-neglect and avoidance of routine tasks (e.g., cooking, asking for a partner to keep the score while playing tennis)
- Slight disorientation in new surroundings
- Subtle language changes that are not evident to family members
Symptoms of Later-Stage DAT
- Intensified early-stage symptoms
- Severe problems recalling remote and recent events
- Intensified visuospatial problems
- Widespread intellectual deterioration
- Hyperactivity, restlessness, agitation, meaningless handling of objects
- Profound disorientation to place, time, and person
- Problems with self-care (e.g., dressing, bathing)
- Difficulty managing daily routines
- Lack of affect, tact, and judgment
- Loss of initiative, indifference
- Paranoid delusions and hallucinations
- Aggressive or disruptive behaviors
- Inappropriate humor and laughter
- Seizures, myoclonic jerks (sudden muscular contractions), incontinence, and physical deterioration in very late stage
Language Problems Associated with DAT
- Naming problems, verbal and literal paraphasias, and circumlocution
- Problems comprehending abstract meanings
- Impaired picture description
- Difficulty generating a list of words that begin with a specific letter
- Echolalia, palilalia, logoclonia
- Empty speech, jargon, and hyperfluency
- Incoherent, slurred, and rapid speech
- Pragmatic language problems, including inattention to social conventions (e.g., greetings), difficulty initiating conversation, and difficulty maintaining topics
- Reading and writing problems
- In final stages, no meaningful speech, mutism, and complete disorientation to time, place, people, and self
Repeating one’s own utterances.
Repeating the final syllable of words.
Frontotemporal Dementia (Including Pick’s Disease)
A group of heterogeneous diseases that includes Pick’s disease (PiD). Onset between ages 40 - 60. Often underdiagnosed and sometimes confused with DAT. Neuropathology includes:
- Degeneration of nerve cells in the left and right frontal lobe, temporal lobe, or both the lobes in both hemispheres
- In the classic PiD, atrophy may be focal, involving the anterior frontal and temporal lobes, the orbital frontal lobe, and the medial temporal lobe
- Presence of Pick bodies and Pick cells
- Absence of Pick bodies and Pick cells in some variants of this form of dementia, but with the presence of atrophied, gliosed, and swollen brain cells
- Notable behavioral changes as initial symptoms in patients with marked right-sided atrophy, notable language changes in those with marked left atrophy
- Behavior disorders, including uninhibited and inappropriate social behaviors, compulsive behaviors, excessive eating and weight gain, delusions
- Emotional disturbances, including depression, withdrawal, irritability, mood fluctuations, occasional euphoria, excessive jocularity, exaggerated self-esteem
- Impaired judgment and reasoning and lack of insight
Language problems include:
- Dominant language problems with somewhat better preserved memory and orientation (contrasted with patients who have DAT)
- Progressive loss of vocabulary and consequent paraphasia and circumlocution
- Difficulty defining common words and problems in reciting category-specific words (e.g., animals)
- Limited spontaneous speech, echolalia, and nonfluent speech
- Impaired comprehension of speech and printed material
Dense intracellular formations in the neuronal cytoplasm.
Ballooned and inflated neurons.
Dementia Associated with Parkinson’s Disease
Neuropathology of Parkinson’s disease includes:
- Brainstem degeneration
- Presence of abnormal structures called Lewy bodies
- Frontal lobe atrophy resulting in widened sulci
- Reduced inhibitory dopamine due to loss of cells in the substantia nigra
- Neurofibrillary tangles and plaques of the kind found in Alzheimer’s disease
Neurologic symptoms of Parkinson’s disease include:
- Slow voluntary movements (bradykinesia)
- Tremors in resting muscles (exacerbated during stress)
- Muscle rigidity (increased tone and resistance to movement)
- A masklike face
- Reduced eye blinking, festinating gait, disturbed posture, frequent falls, freezing during movement
- Swallowing disorders
- Sleep disturbances
Speech, language, and related problems associated with Parkinson’s disease include:
- Reduced speech volume
- Voice problems that include monopitch and monoloudness
- Long and frequent pauses in speech
- Slow, fast, or festinating speech rate
- Dysarthric speech
- Serious memory problems, problems in abstract reasoning and problem solving
- Impaired visuospatial perception
- Impaired word-list generation
- Severe naming and language comprehension problems in later stages
- Apathy, confusion, hallucinations, delirium
Refers to a group of neurological disorders that include hypokinesia, tremor, and muscular rigidity.
Small pathologic spots typically found in the substantia nigra in patients with Parkinson’s disease.
Dementia Associated with Huntington’s Disease
Classified as subcortical. Typical onset is 35 – 40 years. Affects males and females equally. Genetic etiology is supported. Half the offspring of the patient may have the disease. A malformed protein called huntingtin kills the brain cells that control movement. Neuropathology of Huntington’s disease includes:
- A loss of neurons in the basal ganglia, caudate nucleus, putamen, substantia nigra
- Atrophy may be found in prefrontal and parietal lobes
- Reduced levels of inhibitory neurotransmitters, especially GABA and acetylcholine
Symptoms of Huntington’s disease include:
- Increasingly uncontrollable tic-like movement disorders
- Gait distrubrances in the advanced stages, leading to little or no voluntary movement
- Behavioral disorders, including excessive complaining, nagging, eccentricity, irritability, emotional outbursts, a false sense of superiority, depression or euphoria, schizophrenic-like behaviors (delusions and hallucinations), suicide attempts
Speech, language, and cognitive-linguistic problems include:
- Deterioration of intellectual functions
- Impaired word-list generation
- Naming problems
- Incontinence, sleep disturbances, sleep reversal, dysphagia
- Muteness in final stages
Irregular, spasmodic, involuntary movement of the neck, head, and face.
Several infectious diseases, such as HIV and Creutzfeldt-Jakob disease, can lead to dementia.
AIDS Dementia Complex/HIV Encephalopathy
HIV infection and opportunistic brain infections can cause dementia. Slow onset, but rapid deterioration in final stages. Neurologic symptoms include disturbed gait, tremor, headache, seizures, ataxia, rigidity, motor weakness, facial nerve paralysis, and incontinence. Dementia symptoms include forgetfulness, poor concentration, slow or impaired thinking, apathy and loss of interest in word, depression, mania, confusion, hallucinations, delusions, and memory loss. Language problems are less predominant until mutism occurs in the final stage.
Dementia due to Creutzfeldt-Jakob Disease
Dementia caused by an unconventional infectious agent called prion. Features of the disease include widespread spongiform state in the brain, neuron loss, fatigue, sleep disturbances, cerebellar ataxia, tremor, rigidity, chorea, athetosis, visual problems, memory problems, reasoning impairments, depression, anxiety, euphoria, hallucinations, and delusions. Final stage is characterized by stupor, mutism, seizures, and pneumonia that often lead to death.
Vascular diseases may cause bilateral cortical, subcortical, or mixed damage resulting in dementia. More common in men than in women, and more common in African Americans than in whites, this form of dementia is associated with chronic hypertension. Distinguishing feature is sudden onset.
Dementia Associated with Multiple CVAs
Dementia can be caused by multiple CVAs or infarctions within the deep structures of the brain. Atrophy of subcortical white matter caused by repeated infarcts is associated with long-standing hypertension.
Dementia Associated with TBI
Repeated brain injuries, especially those that cause prolonged periods of unconsciousness, are a cause of dementia. Cortical, subcortical, and mixed types of damage may be associated with this type of dementia.
Management Skills of Daily Activities
- Establishing a simple routine
- Using various reminders (alarms, written instructions, staff reminders, self-monitoring devices, signs, etc.)
- Writing down a list of things to do every morning
- Always keeping phone numbers and possessions in a specific place
- Writing a checklist of things to do before leaving the house
- Always carrying a card with names, addresses, and phone numbers of caregivers
- Writing down important information when memory begins to fail
Interacting with a Patient with Dementia
- Approach the patient slowly, touch the patient gently, establish eye contact, and speak clearly and slowly
- Use gestures, smiling, posture, and other cues
- Talk about simple and concrete events and talk in simple, short sentences
- Point out the topic, person, or thing before speaking about it
- Ask yes/no questions
- Restate important information
- Structure the client’s room and the living environment to establish a routine
- Always say goodbye or give other departing signals
- Reduce emotional outbursts by analyzing the conditions under which they occur and eliminating those conditions
- Minimize demands made on the client
Right Hemisphere Functions
- Understanding holistic gestalt stimuli, visual perception, geometric and spatial information
- Facial recognition, drawing, and copying
- Arousal, attentional, and orientation
- Emotional experience or expression
- Perception of musical harmony
- Certain prosodic features of communication, understanding ambiguous meanings, expressing and understanding the emotional tone of speech, understanding discourse, and pragmatic language skills (e.g., appropriate communication, turn taking, and topic maintenance)
Right Hemisphere Syndrome
Varies in symptom complex. Perceptual and attentional deficits dominate the symptom complex. Affective and communicative deficits accompany them, but pure linguistic deficits (of the kind seen in agrammatic aphasia) are not typical. Attentional and perceptual deficits include:
- Left neglect
- Denial of illness (anosognosia)
- Confabulation regarding disability
- Facial recognition deficits
- Constructional impairment
- Attentional deficits
- Visuoperceptual deficits
Affective deficits include:
- Difficulty understanding emotions other people express
- Difficulty describing emotions expressed on printed faces in storybooks
- Difficulty recognizing emotions expressed in isolated verbal productions
- Difficulty understanding emotional tone of voice
- Expressing emotions (not necessarily experiencing emotions)
Communicative deficits include:
- Prosodic deficits
- Impaired discourse and narrative skills
- Confabulation and excessive speech
- Difficulty understanding implied, alternate, or abstract meanings
- Pragmatic deficits
- Other communicative deficits, such as naming problems, difficulty comprehending complex verbal material, impaired oral reading of sentences
Reduced awareness of the left side of the body and generally reduced awareness of stimuli in the left visual field.
Treatment Targets of Right Hemisphere Syndrome
Treatment focuses on:
- Denial and indifference (clinician gives feedback on errors)
- Impaired attention
- Impulsive behavior
- Pragmatic impairments
- Impaired reasoning
- Impaired inference
- Impaired comprehension of metaphors and idioms
- Visual neglect
Traumatic Brain Injury (TBI)
Injury to the brain sustained by physical trauma or external force. Does not include brain damage due to strokes, tumors, and progressive or transient neuropathologies. Affects populations of 0 – 4, 15 – 19, and elderly the most. Affects more males than females. Falls are the most common cause. Can also be caused by car accidents, crashes, assaults, alcohol and drug abuse, etc. Consequences include immediate or subsequent death, loss of consciousness, coma, destruction of brain tissue, diffuse axonal injury, diffuse vascular injury, brainstem injury, focal lesions, infection, penetration of foreign objects, reduced cerebral blood flow, increased blood pressure, swelling of the brain tissue, hydrocephalus, increased intracranial pressure, ischemic brain damage, seizures, long-term physical, language, and cognitive deficits, and various types of hematomas. Consequences could also include altered consciousness, confusion, disorientation, posttraumatic amnesia, dysphagia, behavior changes (hallucinations, delusions, confabulations), poor emotional control, social withdrawal, irritability, childishness, and unreasonable behaviors. Communication disorders associated with TBI include an initial mutism, confused language, dysarthria, naming difficulties, perseveration of verbal responses, reduced word fluency, difficulty initiating conversation, lack of turn taking in conversation, problems in topic initiation and maintenance, lack of narrative cohesion, impaired prosody, imprecise language, difficulty with abstract language, reading and writing problems, auditory comprehension deficits, and generally disturbed social interaction.
Brain injuries that involve a fractured or perforate skull, torn or lacerated meninges, and an injury that extends to brain tissue. Bullets and blows to the head are the most frequent causes.
Brain injuries that involve no open wound in the head, no penetration of a foreign substance into the brain, and a damaged brain within the skull. Classified this way even if the skull is fractured. Can be of the acceleration-deceleration or nonacceleration types.
Acceleration-Deceleration Closed-Head Injuries
A head is set into motion by physical forces. When the head begins to move, the brain inside is still static. Soon, the brain begins to move. When the head stops moving, the brain keeps moving inside the skull and thus strikes the skull on the opposite side of the initial impact. The moving brain is lacerated or torn because of the bony projections on the base of the skull. Common in car accidents.
Nonacceleration Closed-Head Injuries
Occurs when a restrained head is hit by a moving object. May fracture the skull in most cases.
Brain injury at the point of impact.
Injury at the opposite side of the impact cause by the moving brain striking the skull.
Accumulation of blood.
Method of treatment for TBI. Clinicians train attention, visual processing, and memory, which may not result in improved communication. Attempts to improve memory, reasoning skills, and other cognitive functions may be better integrated with communication treatment. Preferred method of treatment for TBI by some clinicians.
Method of treatment for TBI. Involves direct behavioral procedures. Systematic reinforcement of attending behaviors, appropriate discourse, topic maintenance, self-correction, and so forth, will result in their increase and a concomitant decrease in many inappropriate behaviors.
TBI Communication Treatment Goals
- Increasing the patient’s orientation and attention to place, person, and time by asking questions about the patient’s whereabouts, using written signs to help the patient remember the day of the week or the name of the hospital
- Increasing the patient’s memory for daily routines through written signs that remind the patient of routines and lists of daily activities
- Increasing the patient’s memory for the names of significant people by asking the patient to name pictures of family members
- Decreasing the variability in activities and schedules by initially creating a simple, structured routine with few activities for the patient
- Increasing the patient’s attention to communication partners and topics to promote better comprehension by giving such signals as, “Listen carefully now.”
- Improving communicative attention by introducing new topics and warning about topic changes in conversation
- Teaching the client to ask questions when something said is not clear to him or her by modeling such questions and statements as “What do you mean?” and “I don’t understand that”
- Withholding attention from irrelevant, inappropriate, or tangential responses to reduce their frequency
- Teaching narrative skills in graded steps by initially telling a brief and simple story, asking the patient to retell it, and eventually having the client retell more complex stories
- Integrating such pragmatic skills as topic maintenance and topic initiation into narrative skills teaching by prompting with “say more” and “give details”
- Integrating work- or school-related words, phrases, and narratives into communication
- Selecting client-specific work or school-related vocabulary and expressions into treatment sessions
- Using tangible reinforcers when necessary because some patients with TBI may not respond to verbal reinforcers in the early stages of recovery
- Teaching self-monitoring skills by including them at all levels of training
- Teaching compensatory strategies to handle residual deficits (e.g., writing down instructions and important information, requesting information, requesting people to speak slowly or repeat, requesting others to write down messages, establishing simple an invariable routines, reducing environmental distractions, and self-cueing)
- Training family members and others to recognize prompt, model, and reinforce appropriate communication and general behavior
- Promoting community reentry by preparing the patient for reentry to school or work
- Educating family members, teachers, and supervisors about the patient’s strengths and weaknesses, teaching family members, teachers, colleagues, and supervisor to change their style of communication if needed, and modifying teacher or supervisor demands (if necessary)
Involves impaired execution of the oral, pharyngeal, and esophageal stages of swallowing. The patient may experience problems in chewing food, preparing it for swallow, initiating the swallow, propelling the bolus through the pharynx, and passing food through the esophagus. Possible causes include strokes, tumors, neurologic diseases, surgical or radiation treatment of cancer, upper body surgery, TBI, cervical spine disease, polio, COPD, CP, genetic factors, and side effects of prescription drugs.
Transportation of food from the plate to the mouth. May be accomplished by self-feeding or feeding by others.
Transportation of food from the mouth to the stomach.
Oral Preparatory Phase
Food placed in the mouth is first masticated. Bolus is formed.
Disorders of the Oral Preparatory Phase
- Problems in chewing food because of reduced range of lateral and vertical tongue movement, reduced range of lateral mandibular movement, reduced buccal tension, and poor alignment of the mandible and maxilla.
- Difficulty in forming and holding the bolus, abnormal holding of the bolus, slippage of food into anterior and lateral sulcus, aspiration before swallow due mostly to weak lip closure, reduced tongue movement, and inadequate tongue and buccal tension.
This phase begins with the anterior-to-posterior tongue action that moves the bolus posteriorly. Phase ends as the bolus passes through the anterior faucial pillars when the swallowing reflex is initiated.
Disorders of the Oral Phase
- Anterior, instead of posterior, tongue movement and generally weak tongue movement
- Reduced range of movement and elevation
- Tongue thrust
- Reduced labial, buccal, and tongue tension and strength
- Food residue in various places (e.g., anterior and lateral sulcus and the floor of the mouth), suggesting incomplete swallow
- Premature swallow of solid and liquid food and aspiration before swallow, caused by apraxia of swallow
- Piecemeal swallow (attempts at swallowing abnormally small amount of the bolus)
This phase consists of reflex actions of the swallow. Reflexes are triggered by the contact food makes with the anterior faucial pillars. Involves CP closure, laryngeal closure by an elevated larynx to seal the airway, reflexive relaxation of the CP muscle for the bolus to enter, and reflexive contractions of the pharyngeal contractors to move the bolus down and eventually into the esophagus.
Disorders of the Pharyngeal Phase
- Difficulties in propelling the bolus through the pharynx and into the pharyngoesophageal sphincter (PE) segment
- Delayed or absent swallowing reflex
- Nasal and airway penetration of food
- Food coating on the pharyngeal walls
- Food residue in the valleculae, on top of the airway, in the pyriform sinuses, and throughout the pharynx
- Delayed pharyngeal transit
- Reduced pharyngeal peristalsis
- Pharyngeal paralysis
Inadequate closure of the airway
- Aspiration before or after swallow
- Reduced movement of the base of the tongue
- Reduced laryngeal movement
- CP dysfunctions
This swallowing phase is not under voluntary control. It begins when the food arrives at the orifice of the esophagus. Food is propelled through the esophagus by peristaltic action and gravity into the stomach. Bolus entry into esophagus results in restored breathing and a depressed larynx and soft palate.
Disorders of the Esophageal Phase
- Difficulty passing the bolus through the CP muscle and past the 7th cervical vertebrae
- Backflow of food from esophagus to pharynx
- Reduced esophageal contractions (due to surgery, neurologic damage, or radiation therapy)
- Formation of diverticulum (a pouch that collects food)
- Development of a tracheoesophageal fistula (a hole)
- Esophageal obstruction (e.g., by a tumor)
Direct Treatment of Swallowing
Food or liquid is placed in the patient’s mouth to shape appropriate swallowing. Designed to reduce problems that are evident in the different stages of the swallow.
Indirect Treatment of Swallowing
Treatment of swallowing that does not involve food. Instead, various exercises designed to improve muscle strength are prescribed and practiced.
Patient is asked to hold food in the mouth, take a deep breath, and hold it soon after initiating a slight exhalation, swallow while holding the breath, and cough soon after the swallow. Closes the airway at the level of the VF to prevent aspiration.
Super Supraglottic Swallow
Patient is asked to inhale and hold the breath tightly by bearing down and swallow while holding breath and bearing down. Closes the airway before and during the swallow. Promotes false VF closure.
Patient is asked to squeeze as hard as possible while swallowing. Increases the posterior motion of the tongue and increases pharyngeal pressure.
Patient is first educated about the laryngeal elevation, then asked to palpate the laryngeal elevation when swallowing saliva, and finally taught to hold laryngeal elevation during swallowing for progressively longer durations. Elevates the larynx, widening the CP opening.
The CP muscle is split from top to bottom to create a permanently open sphincter for swallowing. Fibers of the inferior constrictor above and the esophageal musculature below may also be split. Eating may be resumed within a week. Surgical procedure. Recommended for patients with Parkinson’s, ALS, and oculopharyngeal dystrophy.
Involves inserting a feeding tube into the esophagus and stomach through a hole (stoma) that has been surgically created through the cervical esophagus. Surgical procedure. For patients who cannot tolerate oral feeding.
Involves insertion of a feeding tube into the stomach through an opening in the abdomen. Blended table food is directly transported to the stomach. Surgical procedure. For patients who cannot tolerate oral feeding.
A tube that is inserted through the nose, pharynx, and esophagus into the stomach, feeds the patient. Surgical procedure. For patients who cannot tolerate oral feeding.
A tube is inserted into the esophagus and stomach through a sole that has been surgically created through the pharynx. Non-oral, surgical feeding.