Introduction to Motor Speech Disorders in children PDF

Introduction to Motor Speech Disorders in children Niharika M K Lecturer JSSISH 05-08-2020  Motor Speech Disorders Speech disorders that disturb the natural ability to speak due to neurologic impairments These...

Introduction to Motor Speech Disorders in children Niharika M K Lecturer JSSISH 05-08-2020  Motor Speech Disorders Speech disorders that disturb the natural ability to speak due to neurologic impairments These neurologic impairments make it difficult for individuals with motor speech disorders to – plan, – program, – control, – coordinate, and – execute speech productions  Motor speech disorders - impairments in the systems and mechanisms - that control the movements necessary for the production of speech. They are a group of disorders resulting from disturbances in muscular control, weakness, slowness, or of the speech mechanism incoordination May affect the underlying sub-systems or processes of speech: Respiration, phonation, resonance, articulation, and prosody. Definition Motor speech disorders (MSDs) can be defined as speech disorders resulting from neurologic impairments affecting the motor planning, programming, neuromuscular control, or execution of speech. Major types of MSD: Dysarthria and Apraxia Prevalence of MSDs in children: Childhood dysarthria and childhood apraxia: 1 in every 1000 children (USA - 2019) Among developmental communication disorders, about 5% have MSDs Categorizing MSDs Age of onset Congenital Developmental Acquired Course Chronic or stationary Improving Progressive or degenerative Exacerbating-remitting Site of lesion Cranial nerves, brainstem, cerebellum, pyramidal, extrapyramidal tract, cereral cortex Neurologic Degenerative diagnosis Inflammatory Toxic-metabolic Neoplastic Traumatic Vascular Types of MSDs (Darley, Aronson, & Brown,1969, 1975) CEREBRAL PALSY  Cerebral Palsy (CP) is - a neurological disorder caused by - a non-progressive brain injury or malformation - that occurs while the child’s brain is under development (before birth, during birth, or immediately after birth). CP primarily affects body movement and muscle coordination. Childhood Dysarthria - when a child with cerebral palsy has difficulty coordinating the muscles they use for speaking and the coordination of their breathing as they talk.  Non life threatening Non- Permanent progressive CP Not Incurable communicabl e Manageable  First identified/described: John Little (1843) Term CP was coined by: Phelps (World War II) In 1947, group of specialists formed the AACP (American Academy for Cerebral Palsy) to form structured programs on diagnosis, treatment, technological development, perinatal care of cerebral palsied children  Definition Bobaths (1954) viewed cerebral palsy as a sensorimotor disorder which was not one condition, but a group of conditions occurring as a result of abnormal brain development or brain damage. Abbott (1956) defined CP are disorder of neurological disability caused by a lesion in the motor centers of the brain. This brain damage resulted not only in a loss of functional muscular control but also in sensory disturbances.  Perlstein (1961) defines CP as a condition, characterized by paralysis; weakness; Incoordination or any other aberration of motor function due to pathology of the motor control centers of the brain. Boone (1972) defines CP as a motor dysfunction secondary to CNS damage to the organism before, during or shortly after birth. Lord (1984) defined CP as a non-progressive disorder of motion and posture due to brain insult or injury occurring in the period of early brain growth, generally under 3 years of age.  According to American Academy for cerebral palsy and developmental medicine (1977), cerebral palsy refers to a nonprogressive CNS deficit. The lesion may be in a single or multiple locations of the brain, resulting in definite motor and possible sensory abnormalities. It occurs as a result of intrauterine factors, events at the time of labor and delivery (congenital CP) or a variety of factors in the early developing years (acquired CP). In addition to motor deficit, other associated disorders may be found.  According to National Trust Act, India (1999), CP refers to a group of non-progressive conditions of a person characterised by abnormal motor control and posture resulting from brain insult or injuries occurring in the pre- natal, peri-natal or infant period of development. According to the Surveillance of Cerebral Palsy in Europe (SCPE, 2000), CP is a group of permanent, but not unchanging, disorders of movement and/or posture and of motor function, which are due to a non- progressive interference, lesion, or abnormality of the developing brain.  According to Rosenbaum et al. (2007), CP describes a group of permanent disorders of the development of movement and posture, causing activity limitation, that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain. The motor disorders of CP are often accompanied by disturbances of sensation, perception, cognition, communication, and behavior, by epilepsy, and by secondary musculoskeletal problems.  Causes of CP CP etiology is complex A number of factors may cause damage to the central nervous system (CNS) at an early stage of its development The risk factors fall into the following categories: pre- conception, concerning the broadly defined health and living conditions of the mother; prenatal, which are related to the course of pregnancy; perinatal; as well as risk factors in the neonatal and infant period Pre-conception Mothers systemic diseases (high blood pressure, diabetes) Drugs and stimulants used Malnutrition Poisoning Infections Immune system disorders preceding pregnancy Physical and chemical factors Infertility Abortions Socio economic status Pre natal Vaginal bleeding Abnormalities of the placenta Multiple pregnancy Systemic disease during pregnancy Abnormal pulse of the fetus Intrauterine infections Intrauterine suppression of development Intrauterine hypoxia Premature rupture of membranes Assisted reproductive technology (in vitro fertilization) Peri natal Premature birth Vacuum assisted delivery Forceps delivery Delivery after due date Prolonged labor Asphyxia Aspiration syndrome CNS insults (hydrocephaly, microcephaly) Neonatal and infant period Respiratory distress syndrome Artificial respiratory support Infections (meningitis) Hyperbilirubinemia Hypoglycaemia Hypothyroxianemia Intracranial hemorrhages Neonatal convulsions  Associated problems Children with cerebral palsy generally have a number of medical conditions associated with the disorder Primary conditions – These are a direct result of the brain lesion or brain malformation. Primary conditions affect motor coordination, motor control, muscle tone, posture and balance. Gross motor, fine motor, and oral motor functioning are also considered primary conditions of Cerebral Palsy.  Secondary conditions – Primary conditions, in turn, may lead to secondary conditions such as inability to chew, inability to swallow, breathing difficulties, bladder and bowel control issues, and communication difficulties. If the brain lesion or malformation that caused Cerebral Palsy did not exist, these secondary conditions would not be present.  Associative conditions – Research has shown that individuals with Cerebral Palsy often have associative conditions not caused by the same brain injury or malformation which caused Cerebral Palsy, but proven to be common with individuals who have Cerebral Palsy. If the brain injury or malformation that caused the Cerebral Palsy did not occur, these conditions may still be present in the individual. Associative conditions include intellectual impairment, epileptic seizures, hearing impairment, and vision impairment.  Co-mitigating factors – An individual may also have health conditions separate from Cerebral Palsy. Unlike associative conditions, researchers have not concluded that these conditions have a high correlation to Cerebral Palsy. Co-mitigating factors exist regardless of Cerebral Palsy. Examples of co-mitigating conditions of Cerebral Palsy include autism, attention deficit hyperactivity disorder, and asthma. Physical and Mobility Issues Physical and mobility issues are synonymous with cerebral palsy. The issues can range from mild spastic movements to more severe issues such as being unable to walk without assistance or needing a wheelchair to get around. Physical and mobility issues also depend on the type of cerebral palsy the child has, such as spastic, athetoid, ataxic, etc. Gross motor and fine motor skills may be affected 07-09- Seizures Seizures are common among children with cerebral palsy because CP is caused by a brain injury occurring before, during or shortly after birth. Brain injuries increase the chance for abnormal nerve activity to occur within the brain, which can result in seizures. According to the Centers for Disease Control and Prevention (CDC), around 35% of all children with CP will experience seizures. There are numerous different types of seizures, such as: Absence seizures: Children with absence seizures may stare into space and have small and subtle body movements, such as eye blinking and lip-smacking. Atonic seizures: Children with atonic seizures have a temporary loss of muscle control and may collapse onto the floor without warning. Clonic seizures: Clonic seizures are marked by jerking muscle movement in the arms, face, and neck. Myoclonic seizures: Children with myoclonic seizures will have temporary jerking in the arms and legs. Tonic-clonic seizures: Tonic-clonic seizures are the most severe type of seizures, marked by body shakes, loss of consciousness, the body stiffening, and in some instances, loss of bladder control. Cognitive and Behavioral Issues Attention deficit hyperactivity disorder (ADHD) Challenges with behavior Emotional problems (inability to connect with others emotionally) Psychological issues Problems with comprehension and decision-making skills Memory issues Learning disabilities Intellectual disabilities Communication difficulties Difficulty in expression and comprehension May turn non-verbal Dysarthria Apraxia of speech Difficulty in developing gesture and facial expression Sensory issues Hyposensitivity or hypersensitivity Difficulties feeling light touch, pain, pressure on the skin, temperature, position of the body Vision Problems Children with CP are at risk of vision problems such as Cerebral visual impairment (caused by brain damage), strabismus (caused by eye issues), and hyperopia (long- sightedness). Hearing issues An estimated 10-20 percent of children with cerebral palsy have some form of hearing impairment, and five percent are deaf Mostly congenital, sensorineural hearing loss Dysphagia Children with cerebral palsy are at risk of dysphagia due to poor muscle and motor function control. Oral Health Issues Excessive gagging, problems with drinking and eating, gingivitis, involuntary cheek and tongue biting, and more Tooth decay and cavities Teeth grinding while sleeping Drooling Abnormal alignment in the upper teeth and lower teeth Oro-motor function issues Oro-motor dysfunction may be seen in 90% of the children with CP Difficulty with speaking, swallowing, feeding/chewing, sucking, blowing Drooling – inadequate lip seal Articulatory imprecision Inadequate intra oral breath pressure Reduced strength, range and speed of articulatory movements Reduced oral sensation Diminished gag reflex Digestive Issues Children with cerebral palsy may have long-term digestive issues. Common digestive issues include: Constipation Incontinence Issues with swallowing and/or sucking Unusual weight gain or weight loss Bladder infections Vomiting Unusual fatigue Aspiration Respiratory Issues Difficulties in swallowing, low activity levels, excessive drooling, blocked airways, and the inability to cough are among a few of the various reasons that children with cerebral palsy may experience respiratory issues. Around 30% of children with cerebral palsy present respiratory complications There are numerous respiratory conditions associated with cerebral palsy, including: Bronchitis Pneumonia Asthma Aspiration Pneumonia Chronic lung disease Sleep Issues and Disorders Sleep issues are a common occurrence among children with cerebral palsy, usually due to associated health problems The most common health issues that contribute to sleep issues include muscle spasms, pain, drooling, skin ulcers, epilepsy, and gastrointestinal reflux disorder (GERD) Skin Conditions Skin conditions often affect children with cerebral palsy due to excessive drooling and the inability to wash their hands and faces correctly, change their bedsheets, and perform other tasks to stay hygienic  Classification of CP The clinical presentation of cerebral palsy is varied. Children with CP differ clinically in the following characteristics: level of motor damage, type/nature of motor disorder, distribution of motor impairment, etiology, presence/number of accompanying impairments, structural brain abnormalities on neuroimaging, degree of severity of impairments, and individual therapeutic needs. 10-09-2020 Traditional classifications of CP – Physiological – Topographic – Etiologic – Neuroanatomic (radiologic) – Therapeutic – Functional Physiologic classification (1) This is based on the type/nature of motor or movement disorder (quality and changes in tone) and classifies CP into two types: – spastic (pyramidal) and – non-spastic (extrapyramidal) Spastic Non-spastic Tone Increased (hypertonia) Decreased (hypotonia) Deep tendon Increased Normal or mildly increased reflexes Movement Decreased Disordered (2) Hardy’s classification Disorders of muscle tone: Hypertonic (spasticity and rigidity) and hypotonic (flaccidity and atonia) Disorders of movement: Hyperkinetic and hypokinetic Disorders of coordination: Ataxia Topographic classification Relies on the localization/limb distribution of neuromotor impairment It subdivides spastic CP into: Quadriplegia (symmetric/equal and severe spasticity of all four limbs), Diplegia (involvement of the four limbs but greater spasticity and weakness in the lower limbs) Hemiplegia (involvement of the upper and lower limbs on one side of the body) Tripegia (three-limb spasticity) and Monoplegia (one-limb spasticity) Etiologic classification Based on the actual cause (etiology) and timing of insult The etiology of CP is multifactorial, and the causal pathways are (mechanisms) multiple and complex The risk factors or associated etiological factors in CP include genetic abnormalities, cerebral dysgenesis, multiple gestation, intrauterine/congenital infection (TORCHS), maternal infection, prematurity, low birth weight, perinatal asphyxia (HIE), bilirubin encephalopathy, postnatal CNS infections, etc. These associated etiological factors can be classified according to the timing of insult as prenatal (commonest), perinatal and postnatal Neuroanatomic (neuropathologic) classification Correlates specific radiologic findings (brain structural alterations) with types of CP Neuropathologic classification relies on neuroimaging studies such as magnetic resonance imaging (MRI) and computed tomography scan (CT scan) Abnormal radiological findings and diagnoses can be classified into five categories namely: malformations, gray matter damage, white matter damage, ventriculomegaly, atrophy or CSF space abnormalities and miscellaneous findings. Therapeutic classification Based on treatment needs Four types – Non-treatment, – Modest treatment, – Need for a CP treatment team, and – Pervasive support groups Functional capacity Functionally, CP is classified into levels of severity based on functional (motor) abilities and/or limitation of activity Class I – no limitation of capacity practically Class II – mild to moderate limitation Class III – moderate to severe limitation Class IV – no capacity Clinical classifications of CP Balf and Ingram (1955) Hagberg et al. (1976) SCPE (Surveillance of Cerebral Palsy in Europe), 2000  Dysarthria Childhood Dysarthria is a motor speech disorder in which the muscles that are used to produce speech are damaged, paralyzed, or weakened. Dys – impaired or dysfunctional Arthr – joint or vocal articulation The area of the nervous system that is affected determines the type of the dysarthria and how seriously it affects the speech. Can be congenital, developmental,or acquired  As a child grows and and begins to talk, dysthraria may become more noticeable via slurred speech and an abnormal speaking pattern Their speech can range from talking too fast or talking extremely slow Because of the damage to the mouth and facial muscles, children with dysarthia will have limited movement of the jaw, tongue, and lips In addition, they may also have swallowing and feeding problems, as well as excessive drooling and saliva buildup Most common cause – CP CP is not only the cause of dysarthria in childhood Definition According to Darley, Aronsen and Brown (1969) Dysarthria is collective name for a group of speech disorders, resulting from disturbances in muscular control over the speech mechanism due to damage of the central/ peripheral nervous system. It designates problems in oral communication due to paralysis, weakness/ Incoordination of the speech musculature  Duffy (2005) defined dysarthria as a collective name for a group of neurologic speech disorder resulting from abnormalities in the strength, speed, range, steadiness, tone or accuracy of movements required for control of the respiratory, phonatory, resonatory, articulatory and prosodic aspect of speech production. The responsible pathophysiologic disturbances are due to central or peripheral nervous system abnormalities and most often reflect weakness, spaticity, incoordination, involuntary movements or excessive or reduced or variable muscle tone.  Childhood Dysathria can be classified based on several factors such as – Age of onset – Etiology – Site of lesion – Underlying neurological conditions – Clinical features Age of onset: Childhood dysarthrias are broadly classified as Congenital or developmental Acquired Congenital: These are associated with diseases present at birth and are therefore also labeled developmental dysarthrias. Examples of this type include those seen with CP, Moebius syndrome Acquired: result from some disease or event (traumatic brain injury, CVA, tumor etc) with an onset during the paediatric period (0 – 15 years of age) but usually following a period of normal speech and language development. Etiology Dworkin and Hartman, 1988, used the classification system for adult neurogenic communication disorders to classify the causes of childhood dysarthria. It includes eight categories. – Vascular accidents – Infectious process – Traumatic insults – Allergic or anoxic conditions – Metabolic disorders – Idiopathic disorders – Neoplasms – Degenerating and demyelinating diseases These 8 categories is commonly known by the mnemonic VITAMIN D.  Vascular accidents: Cerebro vascular accidents (CVA) or strokes will disrupt the normal blood flow within the brain by rupturing the blood vessels.This leads to the intracranial bleeding to the surrounding areas. This may damage those areas that are undernourished as a result of this. This prolonged ischemia causes infarction in which the affected areas of the brain die and replaced with scar tissue. Infectious process: Infectious organisms which enters to the brain with the help of the circulatory system through the fractures of the cranial and facial bones. Eg. Meningitis, encephalitis, and poliomyelitis etc  Traumatic insults: Head injuries result in diffuse neuropathologic changes including shearing, swelling and hemorrhaging of brain tissue. The pathophysiologic and neuropsychologic effects are usually dependent upon the areas of the brain that are damaged. Allergic or anoxic conditions: Negative reactions to allergens that may invade the nervous system may cause temporary or permanent neurologic disturbances. Anoxia, in which oxygen supply to the brain is disrupted for a period of time will also cause neurological disturbance.  Metabolic disorders: Abnormal metabolism of the nervous system can cause pathologic effects such as intracranial hypertension, brain tissue degeneration, and disturbances in the transmission and uptake of neurotransmitter substances. A major source of such adverse metabolic reactions is dysfunction of one or more endocrine glands. Idiopathic disorders: If the neurological signs and symptoms presented by the patient cannot be attributed to the an apparent cause their origin may be labeled idiopathic. However the identity of the actual etiology often unfolds over the course of time and with further diagnostic study.  Neoplasms: Any type of abnormal growth such as tumor, cyst, or vascular malformation is considered a neoplasm. When this occur within the nervous system they may interfere with normal blood flow dynamics, and they can cause increased intracranial pressure by blocking the circulation of the CSF within the ventricular system. These effects may result in widespread neuropathologic changes including those under vascular lesions. Degenerating and demyelinating diseases: these include cerebral palsy, Huntingtons disease, muscular dystrophies etc. Site of lesion Espir and Rose, 1983 have presented a site of lesion classification for the childhood dysarthria that is based on classic sites of lesion presented in clinical literature of neurology. Five major sites are included – Muscle – Lower motor neuron – Upper motor neuron – Extrapyramidal – Cerebellar Clinical features At present the most useful clinical classification for childhood dysarthria is Spastic, Dyskinetic, Ataxic, Flaccid, Mixed Classification system by Darley, Aronson and Brown(1975) classified dysarthria into 6 subtypes: Flaccid, Spastic, Ataxic, Hypokinetic, hyperkinetic, Mixed Darley, Aronson, & Brown (1969, 1975)  General characteristics of Childhood dysarthria Feeding problems Oro motor impairments Sensory deficits Delayed speech and language development Slurred and effortful speech  SYNDROMES LEADING TO DYSARTHRIA Juvenile progressive bulbar palsy Congenital supranuclear palsy Guillain-Barre syndrome Worster-drought syndrome Duchenne Muscular dystrophy 12-09-2020  Juvenile progressive bulbar palsy Also called as Fazio-Londe Disease Disorder of the cranial nerve motor neurons (brain stem) and anterior horn cells (spinal cord) Usually considered a rare motor neuron disease limited to the bulbar muscles (Muscles of the mouth and throat responsible for speech and swallowing) Progressive paralysis of muscles innervated by cranial nerves All of the lower cranial nerve nuclei maybe affected in juvenile progressive bulbar palsy, but the ocular motor nerve is spared The first symptoms depend on which region in the nervous system is affected Commonly, the first abnormality is in the hands, where the patient may be conscious of weakness, stiffness of movements of the fingers or may experience fascicular twitching When degeneration begins in the bulbar motor nuclei, the first symptom noticed is usually Dysarthria or Dysphagia Initial symptoms include Facial paralysis Dysphagia Flaccid dysarthria Signs Progressive weakness of tongue, face and pharyngeal muscles Progressive hypernasality (initially) Later stage – dysphagia Facial and tongue weakness Bulbar atrophy is sometimes severe but often is not present Fasciculations are not always observed  Onset of first symptom has been reported between 1–12 years, with a mean age of onset at 8 years Clinical course can be divided into early (< 6 yrs age, predominance of respiratory symptoms) and late course (6–20 years of age, predominance of motor symptoms on superior limbs) Progression to involve other cranial nerve muscles occurs over a period of months or years Course of the disease is fluctuating, but the dysarthria is typically flaccid (Lowery & Miller, 1983) The disease may progress to patient's death in a period as short as 9 months or may have a slow evolution or may show plateaus  Congenital supranuclear palsy Steele-Richardson-Olszewski syndrome Progressive supranuclear palsy Supranuclear: Located above the level of the motor neurons of the spinal or cranial nerves. Used to indicate disorders of movement caused by destruction or functional impairment of brain structures, such as the motor cortex, pyramidal tract, or corpus striatum. Degenerative disease involving the gradual deterioration and death of specific volumes of the brain  Deterioration of cells in the brainstem, cerebral cortex, cerebellum and basal ganglia — a cluster of cells deep within your brain — is what causes the coordination and movement issues of progressive supranuclear palsy.  First symptom: impairs voluntary eye movements (difficulty looking up or down) Movements are slowed, muscles become rigid Difficulty to control gait and balance PSP gets progressively worse but is not itself directly life- threatening It predisposes patients to serious complications such as pneumonia secondary to difficulty in swallowing (dysphagia) The most common complications are choking and pneumonia, head injury, and fractures caused by falls  Guillain-Barre Syndrome Disorder of peripheral and cranial nerve axons Described as an idiopathic demyelinating polyneuropathy The cell bodies in the spinal cord and the brain stem are the anatomic sites for maintaining the variability of the axons of the spinal nerves and the cranial nerves. In this condition the lesion will be in the peripheral and the cranial nerve axons. In GBS, the body’s immune system begins to attack the body itself, causing what is known as autoimmune disease.  The nerves in your peripheral nervous system connect your brain to the rest of your body and transmit signals to your muscles. The muscles won’t be able to respond to signals they receive from your brain if these nerves are damaged. The first symptom is usually a tingling sensation in your toes, feet, and legs. The tingling spreads upward to your arms and fingers. Both children and adults are affected; but onset is usually in adulthood. Peak incidence in children occurs between 4 and 10 years. Characteristics – specific pattern manifestation Diffuse lower motor unit paralysis Either rapid/gradual in onset Sensory loss may be less severe than motor weakness Bilateral paralysis of the facial nerve (VII) Dysphagia with particular involvement of cranial nerve IX and X Respiratory function may be impaired because of weak breathing muscles General prognosis is good with 65% of the patients recovering completely; the rest shows persistent weakness and atrophy of the feet and leg muscles More serious conditions may be life-threatening  Worster-drought syndrome Also called the bilateral perisylvian or opercular syndrome or congenital suprabulbar paresis Worster-Drought syndrome (WDS) is a type of cerebral palsy (movement disorder) that affects the muscles around the mouth and throat (perisylvian region or operculum) It is caused by inadequate development of the corticobulbar tracts This abnormality appears to occur in the 12 to 16 week period of pregnancy. Main symptoms Drooling Excessive salivation Swallowing difficulties Increased reflexes Speech impairment – severe dysarthria Mild spastic diplegia is common Epilepsy Cognitive and behavioral impairment  Duchenne Muscular dystrophy Disorders of muscles Dystrophies are hereditary myopathies characterized by progressive muscle degeneration and weakness Most common and most severe type It is also called as Pseudohypertrophic muscular dystrophy Onset is between 2 and 5 years; affects boys almost exclusively and progresses rapidly It was reported that there individuals lack the protein dystrophin – which helps to trigger muscular contraction; and this absence results in a series of events in the muscle cells leading to their destructions and loss of muscle strength. Characteristics Muscle weakness starting in the muscle of the pelvis; and trunk and progressing to involvement of all striated muscles including those used for speech Enlargement of the calf muscle as well as other muscle groups Infiltrations of fat and connective tissue replaces muscle fibres and gives the illusion of increased muscle bulk called Pseudohypertrophy Delayed independent walking and an awkward and often tiptoe gait  Flaccid dysarthria (during last stages) Articulation disorder is usually mild – errors in one or two phonemes Labial phonemes are produced more correctly than tongue – lip reduced oral breath pressure and vocal intensity. Inability to sustain phonation Strength of the tongue and rate of tongue movement are poor; pointing and narrowing of tongue is often limited; and pursing and retraction of lips is difficult. As disease progresses – respiratory, laryngeal and pharyngeal muscles weakens. To conclude… Cerebral palsy is a neurological syndrome and refers to a combination of signs and symptoms; it is not a disease. It encompasses a group of non-progressive, but often changing, motor impairment syndromes secondary to lesions or anomalies of the brain, arising in the early stages of its development. A number of symptoms and signs are common to both CP and neurodegenerative/metabolic disorders, particularly in infancy and early childhood. All children with features of CP should be carefully evaluated for an underlying cause.