Chapter 7 - Neurological System Disorders part 3 Flashcards
Parkinson’s Disease
Symptoms: begins insidiously with resting “pill-rolling,” cardinal signs include: tremor, rigidity, resistance to passive motion is not velocity dependent (cog-wheel, lead-pipe), akinesia, postural instability, festinating gait, falling backwards (retropulsion), falling forwards (propulsion), mask face and micographia
Hoehn & Yahr 5 Stage Parkinson’s Scale
I - unilateral tremor, rigidity, akinesa, minimal or no functional impairment
II - bilateral tremor, rigidity or akinesia, with or without axial signs, Independent with ADL, no balance impairment
III - worsening of symptoms, first signs of impaired righting reflexes, onset of disability in ADL performance, can lead independent life
IV - requires help with some or all of ADL, unable to live alone w/o assistance, unable to stand/walk unaided
V - confined to bed/wheelchair, max A.
Tethered Cord Syndrome
Possible: hairy patch of skin, dimple, hemangioma (red birthmark), difficulties with bowel/bladder, gait disturbances, and or deformities of feet, low back pain and scoliosis
Duchenne’s
Most common type, diagnosed between 2 and 6 years, males, life expectancy early 20s
Symptoms: pseudohypertrophy (enlarged calves), possible enlarged forearms and thighs, weakness of proximal joints -> amb. with waddling gait (trendelenburg), Gower’s sign (walks up thighs)
Becker Muscular Dystrophy
A variant of Duchenne, but slower to progress, less severe, and less predictable, survive into late adulthood (normal lifespan if no cardiac involvement)
Symptoms: Loss of motor function of hips, thighs, pelvic area, and shoulders, enlarged calves, and cardiac system involvement.
Arthrogryposis Multiplex Congenita
Detected at birth and associated with loss of anterior horn cells
Presence of weakness, deformities, and associated joint contractures. Position of UE - IR of shoulders, Extension of elbows, flexion of wrists. Position of LE - Flexion and IR of hips, club feet.
May be stable, mild progressive or may improve
Related problems: congenital heart defects, spinal defects, torticollis, and involvement of diaphragm
Limb-Girdle Muscular Dystrohpy
Onset between 1st and 3rd decades
Slow progression with proximal muscles of pelvis and shoulders initially affected
Fascioscapulohumeral Muscular Dystrophy
Early adolescence; involves face, upper arms, scapular region, causing masking, weakness, decreased mobility of face, and inability to lift the arms above shoulder level.
With progression, can extend to abdominal and hip muscles, progresses slowly and rarely affects cardiac/resp. systems: therefore life expectancy relatively normal
Spinal Muscular Atrophy
decrease of motor neuron protein called Survival Motor Neuron, chromosome 5.
Weakness of voluntary muscles of shoulders, hips, thighs, and upper back causing spinal curvatures, breathing/swallowing may be affected.
Type I - birth/infancy (werding-hoffman disease)
Life expectancy 2 years
Type II - children, detected 6mo-3yr, life expectancy early childhood
Type III - older children, later onset, less severe form
Type IV - adolescent/adult, later onset, less severe form
Congenital Myasthenia Gravis
Onset near birth, males more frequently
Involves transmission of impulses in neuromuscular junction
Charcot-Marie-Tooth Disease
Teen years or earlier; Primary inperoneal (fibular) and distal leg muscles
Myopathies
Symptoms similar to dystrophies, but these progress slowly, resulting in a better prognosis - Weakness of the face, neck and limbs
Progressive Supranuclear Palsy
Manifested by loss of voluntary, but preservation of reflexive, eye movements bradykinesia, rigidity, axial dystonia, pseudobulbour palsy, and dementia. Occurs later middle life, death occurs approximately 15 years after onset.
Huntington’s Chorea
Autosomal dominant disorder, begins insidiously in middle age. Choreiform movements and progressive intellectual deterioration - psychiatric disturbance may come before movement disorder
Cerebellar/Spinocerebellar Disorders
Characterized by ataxia, dysmetria, dysdiadochokinesia, hypotonia, movement decomposition tremor, dysarthia, and nystagmus
Structural Cerebellar Lesions
Includes vascular lesions (stroke) and tumor deposits, producing symptoms and signs appropriate to their locus within the cerebellum
Spinocerebellar Degeneration
Friedrich’s Ataxia
Autosomal recessive inheritance, occurring in childhood/early adolescence.
Gait unsteadiness, UE ataxia and dysarthia, tremor, areflexia, loss of large fiber sensory modalities, scoliosis, and cardiomyopathy common as disease progresses
Spinocerebellar Degeneration
Cerebella Corticol Degeneration
Pathological changes in cerebellum and inferior olives, onset between 30 and 50 years, cerebellar symptoms are only signs detectable
Spinocerebellar Degeneration
Multiple Systems Degerenation
Onset young to middle life, spasticity, extrapyramidal, sensory, LMN and autonomic dysfunction
Spina bifida Occulta
Boney malformation with separation of the vertebral arches of 1 or more vertebrea with no external manifestations (may not be discovered til late childhood)
Typically no symptoms, but possible instability and neuromuscular impairments (mild gait involvement and bladder/bowel problems)
Occulta Spinal Dysraphism
When external manifestations are present: dermal sinus (opening), red birthmark, patch of hair, benign tumor or dimple
May result in spinal cord being split (diplomyelia), or being tied down/tethered (diastemetomyelia) which may lead to neuro damage and developmental abnormalities
Spina Bifida Cystica
Exposed pouch comprised of spinal cord and meninges (spina bifida with meningocele or with myelomeningocele)
Spina bifida with meningocele
Protrusion of sac through spine, containing CSF and meninges
Typically no symptoms, but possible instability and neuromuscular impairments (mild gait involvement and bladder/bowel problems)
Spina bifida with myelomeningocele
Protrusion of sac through spine, containing CSF, meninges, spinal cord or nerve roots.
Results in sensory and motor deficits occuring below the level of lesion, may result in LE paralysis/deformities, bowel/bladder incontinence, DVT, and pressure sores.
Lesions of S2-S4 result in neurogenic bladder, incomplete emptying of bladder, and constipation
Tonic-clonic/grand mal
Includes brief aura
Tonic phase: LOC, stiffening of body, heavy and irregular breathing, drooling, skin paller, and occasional incontinence before clonic phase
Clonic phase: alt. rigidify and relaxation of muscles
Post-ictal phase: Follows clonic phase, includes drowsiness, disorientation, or fatigue
Myotonic-Akinetic Seizure
Myoclonic - brief involuntary jerking of extremities with or without LOC
Akinetic - includes a loss of tone
Petit Mal Seizures
Absence seizures
Typically occur in ages 4-12, a LOC without a loss of muscle tone
Rapid blinking or staring into space
Simple partial seizures
Abnormal electrical impulses occur in localized area of brain
Involuntary jerking of left hand and arm occurs, but can maintain interaction with environment
May become generalized -> LOC
Complex Partial Seizures
psychomotor seziure
Alterations of consciousness and unresponsiveness, may appear confused or dazed.
Automatic motions, such as lip smacking, chewing, swallowing, and nervous movement of hands/fingers, and repetitive movements occur.
Visual or auditory sensations occur just before seizure
Infantile spasms
Infantile myoclonic seizure
West syndrome
Jackknife epilepsy
Beings 3-9 months, dropping of head and flexion of arms, seizures may occur hundreds of times per day.
Prognosis is poor, spasms can decrease but are usually replaced by other seizure disorders
Lennox-gastaut Syndrome
Intellectually disabled, begins during first 3 years of life
Associated with brain disorders, regression of developmental status can occur
Status epilepticus
Prolonged seizures or in rapid succession, can be life threatening, sometimes triggered when meds stopped abruptly
Simple febrile seizures
Can last 40 minutes and includes LOC and involuntary generalized jerking of grand/mal seizure.
Precipitated by a fever.
Launda-Kleffer Syndrome
Acquired epileptic aphasia
Progressive encephalopathy, loss of language skills, auditory agnosia and behavioral disturbances
