Chapter 7 - Neurological System Disorders part 3

Question 1

Parkinson’s Disease

Answer 1

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

Question 2

Hoehn & Yahr 5 Stage Parkinson’s Scale

Answer 2

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.

Question 3

Tethered Cord Syndrome

Answer 3

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

Question 4

Duchenne’s

Answer 4

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)

Question 5

Becker Muscular Dystrophy

Answer 5

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.

Question 6

Arthrogryposis Multiplex Congenita

Answer 6

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

Question 7

Limb-Girdle Muscular Dystrohpy

Answer 7

Onset between 1st and 3rd decades

Slow progression with proximal muscles of pelvis and shoulders initially affected

Question 8

Fascioscapulohumeral Muscular Dystrophy

Answer 8

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

Question 9

Spinal Muscular Atrophy

Answer 9

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

Question 10

Congenital Myasthenia Gravis

Answer 10

Onset near birth, males more frequently

Involves transmission of impulses in neuromuscular junction

Question 11

Charcot-Marie-Tooth Disease

Answer 11

Teen years or earlier; Primary inperoneal (fibular) and distal leg muscles

Question 12

Myopathies

Answer 12

Symptoms similar to dystrophies, but these progress slowly, resulting in a better prognosis - Weakness of the face, neck and limbs

Question 13

Progressive Supranuclear Palsy

Answer 13

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.

Question 14

Huntington’s Chorea

Answer 14

Autosomal dominant disorder, begins insidiously in middle age. Choreiform movements and progressive intellectual deterioration - psychiatric disturbance may come before movement disorder

Question 15

Cerebellar/Spinocerebellar Disorders

Answer 15

Characterized by ataxia, dysmetria, dysdiadochokinesia, hypotonia, movement decomposition tremor, dysarthia, and nystagmus

Question 16

Structural Cerebellar Lesions

Answer 16

Includes vascular lesions (stroke) and tumor deposits, producing symptoms and signs appropriate to their locus within the cerebellum

Question 17

Spinocerebellar Degeneration

Friedrich’s Ataxia

Answer 17

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

Question 18

Spinocerebellar Degeneration

Cerebella Corticol Degeneration

Answer 18

Pathological changes in cerebellum and inferior olives, onset between 30 and 50 years, cerebellar symptoms are only signs detectable

Question 19

Spinocerebellar Degeneration

Multiple Systems Degerenation

Answer 19

Onset young to middle life, spasticity, extrapyramidal, sensory, LMN and autonomic dysfunction

Question 20

Spina bifida Occulta

Answer 20

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)

Question 21

Occulta Spinal Dysraphism

Answer 21

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

Question 22

Spina Bifida Cystica

Answer 22

Exposed pouch comprised of spinal cord and meninges (spina bifida with meningocele or with myelomeningocele)

Question 23

Spina bifida with meningocele

Answer 23

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)

Question 24

Spina bifida with myelomeningocele

Answer 24

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

Question 25

Tonic-clonic/grand mal

Answer 25

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

Question 26

Myotonic-Akinetic Seizure

Answer 26

Myoclonic - brief involuntary jerking of extremities with or without LOC
Akinetic - includes a loss of tone

Question 27

Petit Mal Seizures

Absence seizures

Answer 27

Typically occur in ages 4-12, a LOC without a loss of muscle tone
Rapid blinking or staring into space

Question 28

Simple partial seizures

Answer 28

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

Question 29

Complex Partial Seizures

psychomotor seziure

Answer 29

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

Question 30

Infantile spasms
Infantile myoclonic seizure
West syndrome
Jackknife epilepsy

Answer 30

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

Question 31

Lennox-gastaut Syndrome

Answer 31

Intellectually disabled, begins during first 3 years of life

Associated with brain disorders, regression of developmental status can occur

Question 32

Status epilepticus

Answer 32

Prolonged seizures or in rapid succession, can be life threatening, sometimes triggered when meds stopped abruptly

Question 33

Simple febrile seizures

Answer 33

Can last 40 minutes and includes LOC and involuntary generalized jerking of grand/mal seizure.
Precipitated by a fever.

Question 34

Launda-Kleffer Syndrome

Acquired epileptic aphasia

Answer 34

Progressive encephalopathy, loss of language skills, auditory agnosia and behavioral disturbances