Neurological disorders

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Neurological disorders
2015-07-29 17:27:23
NBCOT exam prep
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  1. cerebral hemispheres (telencephalon)
    • convolutions of gray matter composed of gyri (crests) and sulci (fissures)
    • lateral central fissure (sylvian fissure)- separates the temporal lobe from frontal and parietal lobes
    • longitudinal cerebral fissure- separates the two hemispheres
    • central sulcus- separates frontal lobe from the parietal lobe
  2. paired hemispheres, consisting of 6 lobes on each side
    frontal, parietal, temporal, occipital, insular and limbic
  3. frontal lobe
    • precentral gyrus: primary motor cortex for voluntary muscle activiation
    • prefrontal cortex: control emotions, judgement and higher order cognitive functions
    • Premotor cortex: motor actions including Broca's area- controls motor aspects of speech
  4. Parietal lobe
    • postcentral gyrus- primary sensory cortex for integration of sensation
    • recieves fibers conveying touch, proprioception, pain, and temperature sensations from the opposite side of the body
  5. temporal lobe
    • primary auditory cortex: receives/processes auditory stimuli
    • associate auditory cortex: processes auditory stimuli
    • wenicke's area-language comprehension
  6. occipital lobe
    • primary visual cortex- recieves/processes visual stimuli
    • visual association cortex-processes visual stimuli
  7. insula
    deep within the lateral sulcus assiciated with visceral functions (involuntary functions)
  8. limbic system
    • consists of the limbic lobe (cinglaute, parahippocampal, and subcallosal gyri)
    • phylogenically the oldest part of the brain, concerned with instincts and emotions contributing to preservation of the individual
    • basic functions include feeding, aggression, emotions, endocrine aspects of sexual response and long-term memory formation
  9. white matter
    • myelinated nerve fibers located centally
    • tranverse (commissural) fibers - interconnect the two hemispheres, including the corpus collosum, anterior commisure and hippocampal commisure
    • projection fibers- connect cerebal hemispheres with other portions of the brain and spinal cord
    • association fibers- connect different portions of the cerebral hemispheres within the same hemisphere, allowing cortex to function as an integrated whole
  10. basal ganglia
    • masses of gray matter deep within the cerebral hemispheres
    • forms an associated motor system (extrapyramidal) with other nuclei in the subthalamus and midbrain
    • numerous fiber interconnections
    • caudate loop-functions in association cortex in the formation of motor plans
    • putamen loop- function in associateion with sensorimotor cortex to scale and adjust movements
  11. diencephalon
    • thalamus
    • sensory nuclei- integrate and relay sensroy information from body, face, retina, cochlea, and taste receptors to cerebral cortex and subcortical regions - smell (olfaction) is the exception
    • motor nuclei- relay motor information from cerebellum and globus pallidus to precenral motor cortex
    • other nuclei- assist in integration of visceral and somatic functions
    • subthalamus- involved in control of several functional pathways for sensory, moyot and reticular function
    • hypothalamus- integrates and controls the functions of the autonomic nervous system and neuroendocrine system; maintains homestasis (temp, eating, sexual functions, emotion)
    • epithalamus-
    • habenual nuclei-integrate olfactory, visceral, and somatic afferent pathways
    • pineal gland- secretes hormones that influence the pituitary gland and several other organs, influences circadian rhythm
  12. Brain stem
    • midbrain- connects pons to cerebrum; superior peduncle connects midbrain to cerebllum; contains cerebral peduncles each divided into two parts anterior (crus cerebri and substantia nigra) and posterior part (tegmentum); tegmentum contains all ascending tracts and some descending; substantia nigra is a large motor nulcues- important for motor control and muscle tone; superior colliculus is important for vision and visual reflexes; inferior colliculus is important for hearing and auditory reflexes; rubospinal tract important for coordination, contains CN nuclei oculomotor and trochler
    • PONS
    • connects the medulla oblongata to the midbrain, allowing passage of important ascending and descending tracts; midline rephe nuclei project wiely and are important for modulating pain and controlling arousal; tegmentation contains sereral CN nuclei- adbucens, trigeminal, facial, visubulocochlear
    • connects spinal cord with pons
    • contains relay nuclei of dorsal columns
    • contains important centers for vital functions (cardiac, repiratory, and vasomotor)
  13. cerebellum
    • located behind dorsal pons and medulla in posterior fossa
    • joined to brain stem by 3 pairs of peduncles (Superior, middle and inferior)
    • comprised of two hemispheres and midline vermis, have cerebellar cortex, unterlying white matter, and 4 paired deep nuclei
    • archicerebellum- connects with vestibular system and is concerned with equilibrium and regulation of muscle tone
    • paleocerebellum- receives input from proprioceptive pathways and is concerned with modifuing musle tone and syndergistic actions of muscles; it is important in maintenance of posture and voluntary movement control
    • neocerebllum- recieves input from corticopontocerebellar tracts and olivocerebellar fibers; it is concerned with smooth coordination of voluntary movements, ensures accurate force, direction and degree of movemement
  14. Spinal cord- general structure
    • cylindrical mass of nerve tissue extending from the foramen magnum in skull continuous with medulla to the lower border of first lumbar vertebra in the conus medullaris
    • divided into 30 segments- 8 cervical, 12 thoracic, 5 lumbar, 5 sacral, a few coccygeal segments
  15. spinal cord- central matter
    • central gray matter contains:
    • 2 anterior (ventral) horns and 2 posterior (dorsal) horns united by gay commissure with central canal
    • anterior horns- contrain cell bodies that give rise to motor (efferent) neurons- alpha motor neurons to effect muscles and gamma motor nuerons to muscle spindles
    • Posterior horns- contain afferent (sensory) neurons wth cell bodies located in the dorsal root ganglia
    • two enlargements, cervical and lumbosacral, for origins of nerves of upper and lower extremities
    • lateral horn is found to be thoracic and upper lumbar segments for preganglionic fibers of the autonomic nervous system
  16. white matter- anterior (ventral), lateral and posterior (dorsal) white columns or funiculi
    Ascending fiber systems (sensory)

    dorsal columns/medial lemniscal system- convey sensations of proprioception, vibration and tactile discrimination; divided into faciculus cuneatus (UE tracts, laterally located) and faciculus gracilis (LE, medially located); neurons ascend to medulla where fibers cross to form medial lemniscus, ascend to the thalamus and then to somatosensory cortex

    spinothalamic tracts- convey sensations of pain and temperature (lateral spinothalamic tract) and curde touch (anterior spinothalamic); tracts ascend 1 or 2 ipsilateral spincal cord segments, synapse and cross in the spinal cord to opposite side and ascend in ventrolateral spinothalamic system

    spinocerebellar tracts- convey proprioception infromation from muscle spindles, goli tendon organs, touch and pressure receptors to cerebellum for control of voluntary movements; dorsal tract asccends to ipsilateral inferior cerebellar peduncle while ventrospinocerebellar tracts ascends to contralateral and ispilateral superior cerebellar peduncle

    spinoreticular tracts- convey deep and chronic pain to reticular fomration of breain stem via diffuse, polysynaptic pathways
  17. Descending fiber systems (motor pathways)
    corticospinal tracts- arise from primary cortex, descend in brain stem, cross in medulla via lateral corticospinal tract to ventral gray matter (anterior horn cells); 10 % or fibers do not cross and travel in anterior corticospinal tract to cervical and upper thoracic segments; important for voluntary motor control.

    vestibulospinal tracts- arise from vestibular nucleus and descend to spinal cord in lateral (uncrossed) and medial (crossed and uncrossed) vestibulospinal tracts; important for control of muscle tone, antigravity musles, and postural reflexes.

    rubrospinal tract- arises in contralateral red nucleus and descends in lateral white columns to spinal gray; assists in motor function

    reticulospinal system- arises in the reticular formation of the brain stem and desceneds (both crossed and uncrossed) in both venral and lateral columns, terminates on dorsal gray (modifies transmission of sensation, especially pain) and ventral gray (influences gamma motor neurons and spinal reflexes)

    tectospinal tract- arises from midbain and descends to ventral gray; assists in head turning responses in response to visual stimuli.
  18. CNS support structures
    skull (cranium)- contains brain and facial skeleton, with an opening (foramen magnum) at the base

    • meninges-three membranes that envelop the brain
    • dura mater- outer, tough, fibrous membrane attached to inner surgae of crainum- forms falx and tentorium
    • arachnoid-delicate vascular membrane
    • subrarachnoid space- formed by arachnoid and pia mater, contains cerebrospinal fluid and cisterns, major arteries
    • pia mater- thin, vasular membrane that covers the brain surface

    • ventricles -four cavities filled with CSF and communicate with each other and with the spinal cord canal
    • lateral ventricles- large, irregularly shaped, with anterior (frontal), posterior (occipital) and inferior (temporal) horns; communicates with third ventricle through foramen of monroe
    • third ventricle- located posteiror and deep between the two thalami; cerebral aqueduct communicates third with fourth ventricle
    • fourth ventricle- pyramid shaped vacitiy located in pons and medulla; foramina (openings) of Luschka and MAgnedi communicate fourth ventrical with subarachnoid space

    CSF- provides mechanical support (cushions brain), controls brain excitabiliey by redulation ionic composition, aids in exchange of nutrients and waste products; prodiueces in choroid plexus in ventricles; normal pressure is 70-180 mm/H20 and volum5 125-150cc.

    Blood brain barrier (BBB)- the selective restriction of blood borne substances from entereing the CNS; associated with capillary endothelial cells

    • blood supply- brain is 2% of body weight with a circulation of 18% of total blood volume
    • carotid system- internal carotid arteries arise off common carotids and branch to form anterior and middle cerebral arteries; supplies a larger area of brain and many deep structures
    • vertebrobasilar sustem- vertebral arteries arise off of subclavian arteries to unite and form basliar artiery; supplies brainstem, cerebellum, occipital lobe and parts of the thalamus
    • circle of willis- anterior communicating artery connects the anterior cerebral arteries and the posterior communicating artery connects each posterior and middle cerebral artery
    • venous drainage- includes cerebral veins, dural venous sinuses
  19. Neurons
    • vary in size and complexity
    • cell bodies with dendrites (receptive surface area to receive information via synapses)
    • axons conduct impulses away from the cell body
    • synapses- allow communcation between neurons
  20. neuron groupings and types
    • nuclei are compact groups of nerve cell bodies; in PNS these are called ganglia
    • projection neurons carry impulses to other parts of the CNS
    • axon bundles are called tracts of fasciculus
    • neurologia- support cells that do not transmit signals; important for myelin and neuron production
  21. LMNS (peripheral nerves)
    • Structures: cell bodies in the anterior horn of the spinal cord, spinal nerves, the crainal nerve fibers that travel to target muscles
    • symptoms of a lesion- flaccidity, decreased or absent deep tendon reflexes, atrophy.

    • motor (efferent) originate from anterior horn cells
    • sensory (afferent) originate from dorsal root ganglion
    • Cranial nerves: 12 pairs
    • Spinal nerves
  22. UMNS
    • Structures: any nerve cell body or nerve fiber in the spinal cord (except the anterior horn cells), all superior structures (gray and white matter affecting motor function and descending nerve tracts), cranial nerve nuclei
    • symptoms of a lesion: increased deep tendon reflexes, spasticity, clonus, emergence of primitive reflexes including Babinski's sign, exaggerated cutaneous reflexes, autonomic dysreflexia, flaccidity may occur at the level of the lesion
  23. Spinal level reflexes-
    involuntary response to stimuli; dependent upon intact, neural pathway (reflex arc), may be monosynaptic or polysynaptic; provides basis for unconcious motor function and basic defense mechansisms

    • stretch (myotatic) reflexes-
    • stimulus- muscle stretch
    • reflex arc- afferenct Ia fiber from muscle spindle to alpha motornueron projecting back to muscle of origin
    • functions to maintain muscle tone, support agonist muscle contraction, and to provide feedback about muscle length
    • tested by applying stretch to deep tendons
    • reciprocal inhibition- via an inhibitorty interneuron the same stretch stimulus inhibits the antagonist muscle
    • reciprocal innervation: describes the responses a stretch stimulus can have on agonist (facilitation), antagonist (inhibition), and synerigistic muscles (faciliation)

    • inverse stretch (myotic) reflex
    • stimuli-muscle contraction
    • reflex arc- afferent 1B fiber from golgi tendon organ via inhibitory internueorn to muscle of origin
    • functions to provide agonist inhibition, diminution of force of agonist contnnraction

    • gamma reflex loop
    • stretch reflex forms part of this loop
    • allows muscle tnsion to come under control of descending pathways
    • descending pathways excite gamma motor neurons, causing contraction of the muscle spindle, and in turn increased stretch sensitivity and increased rate of firing from spindle afferents; impulses are then conveyed to alpha motor neurons

    • flexor withdrawl reflex
    • stimulus- cutaneous sensory stimuli
    • reflex arc- cutaneous receptors vis interneurons to largely flexor muscles; mult segmental response involving groups of muscles
    • functions as a protective withdrawal mechanism to remove body part from harmful stimuli

    • crossed extension reflex
    • stimulus-noxious stimuli and reciprocal action of antagonists; flexors of one side are excited causing extensors on same side to be inhibited; opposite responses occur in opposite limb.
    • reflex arc- cutaneous and muscle receptors diverging to many spinal cord motor neurons on the same and opposite side
    • function- coordinates reciprocal limb activities such as gait
  24. stroke/cerebral vascular accident (CVA)
    • cerebral insufficiency- due to transient disturbances of blood flow
    • TIA - transient ischemic attack- transitory stroke that for the most part only lasts a few minutes.
    • TIAs occur when the blood supply to part of the brain is briefly inturrupted
    • TIA symptomes usually occur suddenly and are similar to those of a stroke, but disappear within an hour most often (Symptoms can include numbness or weakness in the face, arm or leg, espeically on one side of the body; confusion or difficulty in talking or understanding speech; trouble seeing in one or both eyes; difficulty walking, dizziness, and/or LOB and coordination. TIAs are often a warning sign that a person is at risk for a more serious and debilitating stroke.

    Cerebral infarction- due to either embolism  or thrombosis or the intra or extracranial arteries

    cerebral hemorrhage: bleed secondary to hypertension or aneurysm.

    cerebral arteriovenous malformation- abnormal, tangled collections of dilated blood vessels that result from congenitally malformed vascular structures.
  25. symptoms of CVA
    • abrupt onset of usually unilateral neurological signs (weakness, vision loss, sensory changes)
    • symptoms progress over several hours to two days
    • specific symptoms are determined by the site of the infarct and the involved artery
  26. Left hemisphere specialization
    • movement of the right side of the body
    • processing of sensory information from the right side of the body
    • visual reception from the right field
    • visual verbal processing
    • bilateral motor praxis
    • verbal memory
    • bilateral auditory reception
    • speech
    • processing of verbal auditory information
  27. Right hemisphere specialization
    • movement of the left side of the body
    • processing of sensory information on left side of body
    • visual reception from left field
    • visual spatial processing
    • left motor praxis
    • nonverbal memory
    • attention to incoming stimuli
    • emotion
    • processing of nonverbal auditory information
    • interpretation of abstract information
    • interpretation of tonal inflections
  28. risk factors

    hypertension, cardiac disease, atrial fibrillation, diabetes mellitus, smoking, alcohol abuse, hyperlipidemia

    nonmodifiable risk factors

    age (increased risk with older age), gender (more common in males), race (african-american and latino are at greater risk), heredity
  29. Traumatic Brain injury
    males are 2x more likely to incur a TBI (highest risk group being 15-29 years old)

    can result from a variety of occurrences (skull fractures, closed head injuries, penetrating wounds of the skull and brain, traumatic injury to extracranial blood vessels, nerve tissues, blood vessels, and meninges are sheared, torn, or ruptured, resulting in hemorhage, edema and ischemia
  30. TBI Symptoms and evaluation
    • concussion characterized by post-traumatic loss of conciousness
    • cerebral contusion/laceration/edema accompanied by surface wounds and skull fractures
    • VAriety of symptoms can result (hemiplegia, monoplegia, and abnormal reflexes, decorticate or decerebrate rigidity, fixed pupils, coma, changes in vital signs)

    • Diagnostic testing
    • Glascow coma scale (neurological scale which provides an objective method to record the concious state of a person; used for initial evaluation and continuing assessment to determine a person's level of conciouness after head injury; a client is assessed against the scale's criteria which delineate a range of points for three tests: eye, verbal and motor responses
    • score sum and three seperate values is considered
    • GCS total is 15 (fully concious) lowest possible total is 3 (deep coma or death), severe when <8, moderated 9-12, and minor when > 13

    • Administration of the Ranchos los Amigos Levels of Functioning
    • Level I- no response: total assistance
    • Level II- generalized response: total assistance
    • Level III- localized response: total assistance
    • Level IV- confused/agitated: Max A
    • Level V- Confused, inappropriate non-agitated: max A
    • Level VI- confused, appropriate: mod A
    • Level VII- automatic, appropriate: min A for daily living skills
    • Level VIII- Purposeful, appropriate: SBA
    • Level IX- purposeful appropriate: SBA on request
    • Level X- purposeful, appropriate: modified independent
  31. SCI
    etiology- trauma to the spinal cord as a result of compression, shearing force, contusion secondary to MVA, penetration wound, sports injury or fall.
  32. classification of SCI
    • degree of impairment and severity of injury is graded using the ASIA impairment scale
    • A- complete, no sensory or motor function is preserved in the sacral segments s4-s5
    • B- incomplete, sensory but no motor function preserved in sacral segments
    • C- incomplete, motor function is preserved below the nuerological level, and the majority of key muscle groups below the neurological level have a muscle grade of less than 3/5.
    • D-incomplete, motor function is preserved below the neurological level, and the majority of key muscle groups below the level have a muscle grade greater than or equal to 3/5.
    • E-normal, sensroy and motor function are normal
  33. Specific SCI symptoms
    • spinal shock (4-8 weeks), all reflex activity is obliterated below the level of the injury presenting a flaccid paralysis
    • sensory deficits may be partial or complete
    • loss of bowel/bladder control
    • loss of temp control below the leasion
    • decreased respiratory function
    • sexual dysfunction
    • changes in muscle tone (spasticity in UMN lesions and flaccidity in LMN lesions)
    • loss of motor function resulting in tetraplegia (quadraplegia) or paraplegia; may be complete or incomplete
  34. clinical syndromes
    central cord- resulting from hyperextension injuries and presenting as more UE deficits vs LE

    brown sequard (hemi-section of cord)- causes disruption of the descending lateral coriticospinal tracts, the ascending dorsal columns, and the ascesing latral spinothalamic tracts, which cross within one or two levels of the dorsal root entrance---- results in ipsilateral paralysis, ipsilateral loss of discriminative touch, contralateral loss of pain, and contralateral loss of thermal sense

    Anterior cord- cause by flexion injuries; motor function, pain. pinprick and temperature sensationare lost bilaterally beow the lesion while proprioception and light touch are preserved

    posterior cord- least frequent, injury to posterior columns results in proprioceptive loss. Pain, temp and touch are preserved; motor function is preserved to varying degrees

    conus medullaris- injury of the scaral cord and lumbarr nerve roots resulting in LE motor and sensory loss

    cauda equina sundrome- injury at the L1 level and beow resulting in a LMN lesion; flaccid paralysis with no spinal reflex activity
  35. complications of SCI
    • respiratory compications- decreased vital capacity, pneumonia
    • decubitus ulcer formation
    • orthostatic hypotension- an excessive fall in blood pressure upon assuming the upright position
    • deep vein thrombosis
    • autonomic dysreflexia- an abnormal response to a noxious stimulus that results in an extreme rise in blood pressure, pounding headache, and profuse sweating; complication is considered an emergency if not reversed by removing the irritating stimulus quickly- irritants could include constipation/impation, distention during bowl program, UTI, urinary retention, bocked catheter, overfilled urine collection bag, decubitus ucers, tight or restrictive clothing, ingrown toenails, burns, overstimulation during sex, heterotrophic ossification, skeletal fractures, appedicidtis, etc

    • management
    • identify the stimulus and relieve the underlying issue immediately
    • meds if no impact can be made by removing the stimuli

    • prevention
    • teache person.caregiver frequent pressure relief principles
    • ensure compliance with intermittent catheterization
    • practice well-balanced diet habits
    • ensure medication compliance
    • educate the person on how to use prevention methods, recognizing the cause, signs, and symptoms and intiating first aid to effectively deal with the situation
  36. Cerebral Palsy (CP)
    etiology- caused by an injury and/or disease prior to, during, or shortly after birth resulting in brain damage and secondary neurological and muscular deficits; detected by 12 months

    common causes include lack of oxygen, intracranial hemorrhage, menigitis, chronic alcohol abuse, toxicosis, infections, genetic factors, endorcrine and metablic disorders

    nonrogressive, but deformities and contractures can develop depending on level of involvement

    prognosis is dependent on severity and location of brain injury

    can be accompanied with seixure, intellectural and/or behavioral disroders
  37. Types of CP
    Spastic cerebral palsy- UMN lesion resulting in spasticity with flexor and extensor imbalance; presents as hypertonia (increased muscle tone) or hyperreflexia (increased intensity or reflex responses)

    dyskinetic cerebral palsy- lesion in the basal ganglia results in fluctuation in muscle tone; expresses self as dystonia (excessive or inadequate muscle tone), athetoisis (involuntary movement which are more distal than proximal), chorea (spamodic involuntary movements which are more proximal than distal and a lack of concontraction)

    • Ataxic CP- lesion of the cerebellum resulting in hypertonia and ataxic movements; characterized by lack of stabilty so coactivation is difficult, resulting in more primitive total patterns of movement. Classification is according to level of severity according to the GMFCS delineating five levels of functional performacnce fro children 6-12 years.
    • (Level I is greatest independence, vs Level V severe restrictions with walking and with handling objects)

    • monoplegia- affects one extremity
    • hemiplegia- involves the U and LE on the same side
    • paraplegia- involves BLEs
    • quadraplegia- involves all extremities
    • diplegia- involves less UE involvement and greater LE functional impairment.
  38. Complications of CP
    • seizures (about 50% of children with CP experience seizures)
    • language and cognitive deficits occur in 50-75 % of children with CP
    • visual impairments (40-50% of children)
    • strabismus-deviation of how one eye aligns with the other
    • nystagmus- reflexive response of the eyes triggered by head movement
    • refractive errors (myopia- nearsighted, hyperopia- farsigted, presbyopia- decreased elasiticty of the lens causing difficulty in accommodation when focusing on objects nearby and when shifting focus from near to far)
    • feeding disturbances- difficulty swallowing or chesing
    • diminished sensation is common in spastic hemiplegia
  39. classification of symptoms of movement/neuromuscular disease
    tremor- rhythmic, alternating, oscillary movements produced by repetitive patterns of muscle contraction and relaxation; classified by rate, rhythm, and distribution; tremors are identified as to whether they happen at rest (resting remor) or during activity (action or intention tremor)

    dyskinesias- involuntary, nonrepetitive but occasionally stereotyped movements affecting distal, proximal and axial musculature in varying combinations; representative of basal ganglia disorders

    myoclonus- a brief and rapid contraction of a muscle or group of muscles

    tics-brief, rapid, involuntary movements often resembling fragments of normal motor behavior. stereotyped and repetitive, but not usually rhythmic

    chorea- brief, purposeless, involuntary movements to the distal extremities and face (usaully related to basal ganglia)

    dystonia- resulted in sustained abnormal postures and disruptions of ongoing movement resulting from alterations of muscle tone

    ataxia- lack of coordination while performing voluntary movements; may appear as clumsiness, inaccuracy or instability, movements are not smooth and may appear disjointed or jerky.

    hemiballismus- usually characterized by involuntary flinging motions of the extremities
  40. parkinson's disease
    • etiology- CNS movement disorder that is idiopathic, slowly progressive, and degenerative
    • onset- typically after 40 years, with increasing incidence with older age

    • symptoms-
    • begins with resting "pill rolling" tremor of one hand

    cardinal signs- tremor, rigidity, resistance to passive motion that is not velocity dependent (cogwheel or lead pipe), akinesia, postural instabiltiy, festinating gait, falling backwards, mask face, micrographia

    • stage and progerssion of the disorder is done using the Hoehn and Yahr's five-stage scale
    • stage i - imilater tremor, rigidity, akinesia, minimal functional impairments
    • stage V- confined to a wheelchair or bed, max assistance

    most commonly managed via medication to manage dopamine levels in the brain
  41. Spina bifida
    • etiology- unknown
    • failure of the spinal column to fully enclose and to protect the neural tube; defect could result in protrusion of the neural tube

    prognosis and degree of impaiment is dependent on the level of the lesion and extent of the neural tube defect; lesions typically occur in the thoracic or lumbar spine
  42. classification of spina bifida
    dependent on the level of the lesion and the extent of tissue involved

    spina bifida occulta- bony malformation with searation of vetebral arches with no external manifestations; may not be discovered until late childhood --- ocult spinal dysraphism (OSD)- when external manifestations such as a red birthmark, patch of hair, benign tumor or dimple cover the site

    • spina bifida cystica- an exposed pouch comprised of the spinal cord and meningies
    • spina bifida with meningocele- protrusion of a sace through the spine containing CSF and meninges but does not infled the spinal cord
    • spinabifida with myelomeningoes- sac protruding containing CSF meninges and the spincal cord or nerve roots

    most commonly locate d in the lumbar region but can occur at any point along the spinal column
  43. symptoms
    • spina bifida occulta- typically no symptoms, ocasional instability, and nueromuscular impairments such as gait and bowel/bladder impairments--- OCD could result in spinal cord being split or being tethered down with may lead to neurological damage and developmental abnormalities
    • spina bifida meningocele- does not present with sumptoms impacting on function, occasional instability and nueromuscular impairments

    • spina bifida with myelomeningocele resulting in sensory and motor deficis occurring below the level of the lesion and may result in lower extremity paralysis and/or deformites
    • lesion of S2-S4 result in bladder and bowel problems
  44. tethered cord syndrome
    tail end of the spinal cord - cord is stretched as a result of compression, being trapped with a fatty mass or scar tissue, developmental abnormality or an injury

    symotoms can include hairy patch of skin, dimple, difficulties with bowel/bladder control, gait disturbances, low back pain, scoliosis, symptoms can be exacerbated by prenanct or with age due to spinal stenosis
  45. med management
    protect the sac from rupturing- can be removed 24-48 hours after birth

    • shunit is indicated shold complication of hydrocephalus orccur (CSF is not reabsorbed ad results in an increased size of the ventricles in the infants head)
    • brain damage could occur as a result of the increased pressure and could affect development
    • increased pressue could also lead to arnold-chiari syndrome in which a portion of the cerebellum and the medulla oblongata slip through the foramen of magnum to the cervial spinal cord

    if shunts become blocked it can result in increased intra-cranial pressure, and there is typically extreme head growth, must monitor for infection of shunt as well (symptoms- vomitting, lethargy, fever, seizures, deterioration of cognitive function)
  46. Musculodystrophies/atrophies
    etiology-group of degenerative disorders resulting in muscle weakness and decreased muscle mass due to a hereditary disease process

    onset- can begin in infancy, childhood, and adulthood; average age of diagnosis is 5 years

    diagnosis- confirmed by blood tests for muscle enzymes or muscle proteins, nerve conduction velocity. elemctromyography and muscle/nerve biopsy if indicated
  47. Duchenne's muscular dystrophy
    • most common form; detected between 2 and 6 years, sex-linked recessive transmission (more common in mails)
    • symptoms include- pseudohypertrophy (enlargement of the calf muscles), giving an appearance the child is muscular and healthy; weakness of the proximal joints rogresses to functional mobility impairments such as trendelenberg (waddling gait) and gowers sign (using hands to crawl up the thighs to get to a standing position); weakness in muscles and heart and diaphragm; decreased life span (rarely past early 20s).
  48. Becker muscular dystrophy
    variant of DMD that is slower to progress, less severe, and less predictable.

    symptoms include- loss of motor unction of the hips, thighs, pelvic area, and shoulders, enlarged calves, cardiac system can be involved, survival can be into late adulthood (if limited cardiac involvement, normal lifespan can be attained).
  49. Arthrogryposis multiplex congenita
    detected at bith and associated with loss of anterior horn cells

    weakness, deformities and associated joint contractures

    position of rest for UEs tends to be IR of the shoulders, extension of the elbows and flexion of the wrists; LEs flexion and IR of the hips and clubfoot

    may be stable, mildly progressive or may improve

    related problems include congenital heart defects, spinal defects, torticollis, and involvement of the diaphragm
  50. limb-girdle muscular dystrophy
    onset begins between first and third decade of life. proximal muscles of the pelvis and shoulder are initially affected, typically progresses slowly
  51. fascioscapulohumeral muscular dystrophy
    occurs in early adolescence, invovles face upper arms, and scapular region, causing masking, weakness, decreased mobility of the face and inabiltiy to lift arms above soulder level

    as it progressed weakness can extend to abdominal muscles and sometimes the hip muscles

    progresses slowly and rarely affects the cardiac or respiratory systems
  52. spinal muscular atrophy
    caused by a decrease of motor neuron protrein called Survival of Motor Neuron chromosome 5

    waeness of the voluntary muscles of the shoulders, hips, thighs, and upper back which result in spinal curvatures

    muscles for breathing and swallowing can be affected

    the earlier age of diagnosis, the more severe functional deficits are and the shorter the life expectancy
  53. congenital myasthenia gravis
    disorder involving transmission of impusles in the nueromuscular junction; onset starting near birth and more common in males
  54. myopathies
    symptoms similar to distrophies but myoptathies progress slowly resulting in a better prognosis; weakness of face neck and limbs is characteristic
  55. progressive supranuclear palsy
    loss of voluntary, but preservation of reflexive eye movements, bradykinesia (slow movement, impaired abilty to adjust body movements), rigidity, axial dystonia, dementia
  56. huntingtons chorea
    autosomal dominant disorder characterized by choreiform movements (brief, purposeless, involuntary movements of distal extremities and face), psychiatric disorder (personality change manic-depressive symptoms)
  57. cerebellar/spinocerebellar disorders
    characterized by ataxia, dysmetria, dysdiadochokinesia, hypotonia, movement decomposition tremor, dysarthia, and nystagmus
  58. Disorders of the PNS
    Amyotrophic Lateral Sclerosis (ALS)
    motor neuron disease of unknown etilogy characterized by progressive degeneration of corticospinal tracts and anterior horn cells or bulbar efferent neurons

    symptoms- muscle weakness and atrophy, cramps and fasciculations precede weakness, signs usually begin in hands, LMN signs soon accompanied by spasticity, hyperactive deep tendon reflexes, dysarthria and dysphagia evident; sensory systems, eye movemes, and urinary sphincters are often spared

    symptom severity is documented by scores on the ALS Functional Raring Scale

    • no specific treatment to slow the disease process.
    • treatmen aimed at treating secondary complications such as spasticity, prevention of aspirations, prevention of ulcers and contractures, and pain management
  59. brachial plexus disoder
    etiology- secondary to traction during birht, invasion of matastic cancer, after radiation treatment, or traction injury

    symptoms- mixed motor and sensory disorders of the corresponding limb

    • erbs palsy- paralysis of the upper brachial plexus C5-C6(7 sometimes)
    • muscles paralused include the supraspinatus and infraspinatus as well as the deltoid, biceps, brachialis and subscapularis
    • marm cannont be raised, elbow flexion is weakend and weakness in retraction and protraction of the scalupa
    • arm grossly presents with the arm straigth and wrist fully bent (waiter's tip position); after 6 months old contractures can form; positioning and ROM exercises are essential to retain shoulder ER, abduction and flexion at the shoulder)

    Klumpke's palsy- paralysi of the lower brachial pledus including C7, C8 and T1; paralysis of the hand and wrist presents, often with ipsilateral horner's syndrome; characteristic signs are that the hand is limp and fingers do not move.
  60. peripheral neuropathy
    may result of trauma, pressure paralysis, forcible overextension of a join, hemorrhage into a nerve, exposure to cold or radiation or ischemic paralysis

    symotoms- pain, weakness, and paresthesias in distribution of the affected nerve
  61. Guillain-barre syndrome
    etiology-may occur after infectious disorder, surgery or an immunization afecting both sexes at any age

    symtoms- acute, rapidly progressive from of polyneuropathy characterized by summetric muscular weakness and mild distal sensroy loss/parasthethias; weakness is more apparant than sensory loss.
  62. myasthenia gravis
    etiology-autoimmune attack on the acetylcholine receptor of the postsynaptic neuromuscular junction (disorder of nueromuscular transmission)

    symptoms- ptosis, diploplia, muscle fatigue after exercise, dysarthria, dysphagia, and prixmial limb weakness

    symptoms fluctuate, and there can be periods of relapse (quadriparesis can occur)
  63. post polio syndrome (PPS)
    motor nuerons breaking down causing new muscle weakness after recovered motor neurons ddevelop new terminal axon sprouts

    symptoms- new onset of weakness, easily fatigued, muscle pain, joint pain, cold interoleracne, atrophy, loss of functional skills
  64. Multiple Sclerosis
    etiology-exact cause is unknown; characterized by demylination; diagnosis is largely based on symptoms

    slow progressive CNS disease characterized by demyelination in the brain and spinal cord

    • symptoms-multiple and varied symptoms, usually with remissions and exacerbations; paresthesia in one or more extremiites, trunk or face; weakness or clumsiness in the leg of hand is common; visual disturbance (diploplia, partial blindness, nystagmus, eye pain)
    • emotional disturbances; nalance loss or vertigo; bladder dysfunction; sensroit motor findings

    • types:
    • relapsing remittin
    • secondary progressive
    • primary progressive
    • progressive relapsing
  65. Evaluation and intervention for neurological system disorders
    create an occupational profile

    analyze performance in areas of occupation (ADL, IADL, Education, Work, Play and leisure, rest and sleep, social participation

    • Evaluation of client factors and performance skills:
    • determine sensory and motor dysfunction and strengths
    • extent of paralysis/weakness, severity and distribution of spasticity, gross and fine motor coordination loss, evaluation of sensory modalities (light touch, pain, pressure, proprioception, kinesthesia, temp, gustatory, olfactory, auditory); postural control eval; range of motion testing; manual muscle testing; skin integrity

    • determine cognitive perceptual dysfunction
    • evaluate foundational visual skills--
    • evaluate pervasive impairments (decreased arousal, alertness, sustained attention, concrete thinking, decreased insight, judgement etc)

    impact of deficits on functional activities

    determine psychosocial dysfunction and strengths, coping mechanisms, adaptation to change in occupational role functioning or to difficulty assuming occupational roles.
  66. General intervention/treatment guidelines
    • positioning (seating and wheeled mobility prescription, bed positioning, pressure reduction and pressure relief techniques);
    • postural control training for seated and standing activities
    • motor learning approaches
    • motor control retraining/relearning for functional integration of affected limbs
    • specific ADL training/retraining/adaptation
    • prescription of AT
    • family/caregiver education
    • cognitive-perceptual retraining compensation in the context of functional activities
    • visual skills retraining
    • intervention for sexual dysfunction
    • bowel and bladder training
    • skin care education
    • DME
    • sensory re-education, compensation, and safety
    • assistance with the development of coping strategies
    • community re-integration
    • return to work or work hardening programs for adults
    • collaboration with educational team for children
  67. Pain
    sensory and emotional response associated with actual or potential tissue damage

    • acute- pain provoked by noxious stimulation; associated with an underlying pathology (injury or acute inflammation/disease)
    • signs: sharp pain and sympathetic changes (increased heart rate, increased blood pressure, pupillary dilation, hyperventilation, anxiety, protective/escape behaviors)

    • chronic pain: pain that persists beyond the usual course of healing
    • signs- symptoms present for >6 months for which an underlying pathology is no longer indentifiable or may never have been present
  68. pain syndromes
    • neuropathic pain-pain as a result of lesions in some part of the nervous system (central or peripheral); usually accompanied by some degree of sensory deficit
    • thalamic pain- continuous, intense pain occuring on the contralateral hemiplegia side; the result of a stroke involving the ventral posterolateral thalamus; poor rehabilitation potential.

    • complex regional pain syndrome (CRPS)
    • type i- formally known as reflex sympathetic dystrophy (RSD); pain maintained by efferent activity of sympathetic nervous system; characterized by abnormal burning pain (causalgia), hypersensitivity to light touch, and sympathetic hyperfunction (coldness, sweating); usually associated with traumatic injury.

    • CRPS Type II- formerly known as neuralgia; pain occurring along the branches of a nerve; frequently paroxysmal; radiculalgia- neuralgia of nerve roots; paresthesias, allodynia

    phantom limb pain- pain in a limb following ampuation of that limb; differentiated from far more common phantom limb sensation

    musculoskeletal pain

    psychosomatic pain-the origin of the pain experience is due to mental or emotional disorders

    headache and cranialfacial pain (i.e. TMJ)

    referred pain- pain arising from deep visceral tissues that is felt in a body region remote from the dite of pathology, resulting in tenderness and cutaneous hyperalgesia (eg medial left arm pain with hear attack, right subscapular pain from gallbladder attack)
  69. Assessment of chronic pain
    • history- determine chief complaints, description of onset, and mechanism of injury
    • determine localization- chronic pain is poorly localized, not well defined.
    • identify nature of pain- constant/intermittent
    • determine  subjective assessment using pain intensity scales
    • physicalexamination-identification of underlying pathology; objective physical findings are usually not readily identified.
  70. OT intervention for chronic pain
    • educate the individual about contributing factors
    • assist the inidividual in identifying and responding adaptively to pain behaviors

    remove behvaioral reinforcers, establish a behavior contract, provide positive reinforcers and educational support, demonstrate change, practice well behaviors

    • assist the individual in developing strategies and using techniques to manage pain
    • teach coping skills/stress managment/assertive communication;provide relazation techniques, deep breathing exercises.

    refer to other professional for direct pain/symptom control interventions

    establish a realistic daily activity program; improve conditioning(walking program); functional capacity; prescribe AT as necessary; teach energy conservation

    provide family education
  71. sensory processing disorders
    etiology- unknown; subtle, primarily subcorticala, neura dysfunction with impaired processing of sensory information and modulation of multisensory systems

    • Symptoms classificatiion
    • classified with various tools:
    • Ayre's Sensory Integration

    • Dunn's Model- symptoms are classified according to the interaction of sensory stimuli that are needed to stimulate a
    • behavioral response
    • Two types- 
    • high neurological threshold: failure to register or respond to routine environmental sensation, or sensation must be experienced over a prolonged time period to elicit a behavioral response.

    low neurological threshold: minimal stimulous facilitates a behavioral over-response

    • two types of behavioral responses
    • passive behavioral response: the individual makes no attempt to change the intensity or duration of the sensory input

    active behvaioral response: the individual avoids or seeks to avoid sesnsory stimuli

    • These combine to form four categories
    • poor registration: high neurological thresholds with passive behavioral response
    • sensory seeking: high neurological threshold with active behavioral response
    • Sensory sensitivity: low neurological threshold, passive behavioral response
    • sensory avoiding: low neurological threshold, active behavioral response

    • ecological model of sensory modulation disorder described individual's unique response to interactions between external and internal dimensions of sensory processing (external- culture, relationships, chosen task; internal- sensation, emotion, and attention)
    • difficulty with modulation  of either can result in difficulties with social and environmental interactions; difficulties with self-regulation due to a mismatch between internal capabilities and external  environmental activities)

    • Sensory modulation disorder
    • sensory overresponsitivy (SOR), sensory underresponsitivity (SUR), sensory seeking (SS)

    • sesnosry based motor disorder
    • dyspraxia; sensorry-based postural disorders

    Sensory discrimination disorder (SDD)- visual, auditory, tactile, vestibular, proprioceptive, taste/smell
  72. SPD signs and symptoms
    • fluctuating or extreme responsiveness while engaging in everyday activities
    • difficulties in interacting with the environment in play,learning, and social situations,  and while engaging in other developmental and health promoting activities
    • difficulty with conceiving,planning or sequencing executing novel actions (dyspraxia)--tends to avoid or reject simlpe motor challenges

    • poor initiation of activities as demonstrated by some children due to difficulty generating ideas (ideation)
    • difficulty with goal-directed action on the environment
  73. Tactile
    • deficits in modulation (regulation and organization)
    • tactile defensiveness- over-responsivity to ordinary touch sensations; individual may demonstrate irriation and discomfort from textures such as clothing, sand, grass, glue, water, paint and/or food;may demonstrate various behavioral responses including distractibility, anger, hostility, temper tantrums,fear, and distress

    Under-responstivity: demonstrates diminished sensory registration and responsiveness; individual may not respond to normal levels of tactile input;individual may not respond to normal levels of tactile input and may seek disproportionate amounts of stimuli to gain environmental information (i.e. excessive touching of people and objects)

    • deficits in tactile discrimination
    • difficulty interpreting tactile information in a precise efficient manner; contributes to impaired body scheme, awkwardness in fine and gross motor tasks; hinders ability to learn about properties and substances

    difficulty localizing tactile stimuli: impaired streognosis and decreased fine motor and visual motor integration
  74. Proprioceptive processing disorder
    • deficits in modulation
    • poor awareness of position of body, body parts and body schema
    • clusiness and awkward
    • distractibility
    • motor planning and movement difficulties
    • reliance on visual cues or other cognitive strategies to motor  plan, guide movements and perform tasks
    • poor awareness of personal space
    • seeks heavy resistance and pressure
    • use of too much or too little force
  75. vestibular processing disorder manifestations
    • deficits in modulation
    • hypersensitivity to movement, charcterized by aversion to movement,impacting on the sympathetic nervous system
    • hyposensitivity to movement characterized by individual seeking intense vestibular stimulation without complaints of feeling dizzy- tendency of a thrill seeker to be unaware of potential danger
    • gravitational insecrity characterized by excessive fear during typical activities, especially when the child's feet are off the ground,moving backwards or upwards, walking on uneven terrain, jumping, getting on/off elevators,using any playground equipment.

    • vestibular discrimination deficits characterized by the above symptoms;however symptoms are demonstrated on a subtle level
    • low muscle tone
    • postural-ocular deficts
    • decreased balance and equilibrium reactions
    • deficits in bilateral coordination
    • low endurance
    • deficient motor planning and sequencing
    • behavioral responses include difficulty with attention, organization and communication

    • sensory based motor disorder
    • deficits in proprioceptive, vestibular systems
    • dyspraxia: difficulty planning movements particularly those that are complex or new
    • postural disorders: decreased muscle tone impacting on stability
  76. OT eval for SPD
    • parent/caregiver interview regarding medical and developmental history
    • teacher interview regarding school and behaviors (Sensory Processing Measure)
    • formal assessment of sensory processing (Sensory profile)
    • informal observations of performance and behavior and formal clinical observations
    • specific things to observe include: crossing midline,bilateral coordination and muscle tone
  77. seizure disorders
    • must be differentiated from epilepsy
    • epilepsy-chronic state of recurrent seizures
    • seizure disorder- temporary disturbance in brain activity causing a group of nerve cells to fire excessively, interferring with normal brain function

    often associatied with other conditions such as- oxygen deprivation, TBI, CP, CVA, brain tumors, neurological conditions, hydrocephalus,metabolic disroders, infections, rubella
  78. seizure classifcation
    two broad groups
    • primary generalized seizures: seizures being with widespread involvement of both sides of the brain
    • partial seizures begin with involvement of a smaller localized area-can spread which is known as secondary generalized seizure
  79. generalized seizures
    • tonic-clonic seizures/grand mal
    • most common type in children
    • tonic phase:loss of conciousness, stiffening of body, heavy and irregular breathing,drooling, skin pallor,
    • clonic phase- alternating rigidity and relaxation of the muscles
    • post-tictal stat follows clonic phase, preiod of drowsinees, disorientation, or fatigue

    • myoclonic-akinetic seizures
    • myoclonic seizures:brief involuntary jerking of the extremities with or without loss of conciousness
    • akinetic seizures: include loss of tone
    • myoclonic akinietic seizure- are difficult to control

    • petit-mal seizures, also called absence seizures
    • typicallyoccur between 4-12 years, loss of conciousness without loss of muscle tone occurs; rapid blinking and staring into space;child does not fall down but does not recall the episode or lapse in time
  80. partial focal seizures
    • simple partial seizures- abnormal electrical impulses occur in a localized part of the brain
    • involuntary, repetitive jerking of the left hand and arm occurs but the individual can maintain interaction with environment

    complex partial or psychomotor seizure:symptoms vary, alterations in conciousness and unresponsiveness
  81. seizure disorders
    infantile spasms: begins at 3-9months, dropping of head and flexion of arms occur, may occur hundreds of time per day; prognosis is generally poor; spasms sometimes decrease but are typically replaced by other seizure disorders

    • lennax-Gastaut syndrome
    • children with severe seizure, mental retardation and a specific EEG pattern
    • associated with various brain disorders from structural abnormalities to birth asphyxia (deprived of oxygen)
    • regression of developmental status can occur in some cases

    • landay Kleer syndrom
    • progressive encephalopathy; loss of language skills; auditory agnosia (inability to distinguish different sounds); behavioral disturbances

    • simple febrile seizures
    • mot common type of seizure (5-10% of children under 5yrs), precipitated by a fever
    • seizure lasts less than 10 minutes; loss of conicousness and involuntary jerking-- do not cause damage

    status elipticus- prolonged seizure or seizures in rapid succession; can be life threatening; tyically occurs when tonic-clonic seizures are not well controlled
  82. intervention of seizure disorder
    first aid, remove dangerous objects, protect individual from harm, raise bed rails (if applicable), turn individual on side if at risk for aspiration, protect head, sidelying is the recovery position, call for medical attention

    post seizure care- rest, notify others

    • OT eval and intervention:
    • observe all medical and safety considerations; document activity, side effects and behvaioral changes