Study Neuromuscular and Nervous Systems Pediatric Pathology Flash Cards

 
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Neuromuscular and Nervous Systems Pediatric Pathology

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Spinal muscular atrophy?
A CONDITION OF PROGRESSIVE DEGENERATION OF THE ANTERIOR HORN CELL
Characteristics of spina bifida?
*MOTOR LOSS BELOW THE LEVEL OF DEFECT IN THE SPINAL CORD
*SENSORY DEFICITS
*HYDROCEPHALUS
*ARNOLD-CHIARI TYPE II MALFORMATION SYNDROME
*LATEX ALLERGY
*BOWEL AND BLADDER DYSFUNCTION
*LEARNING DISABILITIES
Meningocele?
HERNIATION OF MENINGES AND CSF INTO A SAC THAT PROTRUDES THROUGH THE VERTEBRAL DEFECT. THE SPINAL CORD REMAINS INTACT.
Myelomeningocele?
*A SEVERE FORM OF SPINA BIFIDA
*HERNIATION OF MENINGES, CSF, AND SPINAL CORD EXTENDING THROUGH THE DEFECT IN THE VERTEBRAE.
*THE DEFECT MAY OR MAY NOT BE COVERED BY THE SKIN
What are the two forms of spina bifida cystica?
*MENINGOCELE
*MYELOMENINCOCELE
Presents with a cyst like protrusion through the non-fused vertebrae?
SPINA BIFIDA CYSTICA
Spina Bifida Cystica?
*CYST-LIKE PROTRUSION THROUGH THE NON-FUSED VERTEBRAE.
Spina Bifida Occulta?
*NON FUSION OF SPINOUS PROCESSES OF A VERTEBRAE
*SPINAL CORD AND MENINGES REMAIN INTACT
*NO ASSOCIATED DISABILITY
There is usually no associated disability in this form of spina bifida?
SPINA BIFIDA OCCULTA
What are the causative factors of spina bifida?
*GENETIC PREDISPOSITION
*ENVIRONMENTAL INFLUENCE
*LOW LEVELS OF MATERNAL FOLIC ACID
*MATERNAL HYPERTHERMIA
*CERTAIN CLASSIFICATIONS OF DRUGS
What are the different forms of Spina Bifida?
*SPINA BIFIDA OCCULTA
*SPINA BIFIDA CYSTICA (MENINGOCELE, MYELOMENINGOCELE)
Low levels of maternal folic acid can lead to what defect?
SPINA BIFIDA
What systems does spina bifida typically effect?
*CENTRAL NERVOUS SYSTEM (CNS)
*MUSCULOSKELETAL SYSTEM
*URINARY SYSTEM
In a patient with spina bifida, where does the defect usually occur?
LOW THORACIC, LUMBAR, OR SACRAL REGIONS
True/False

Spina bifida is a developmental abnormality due to insufficient closure of the neural tube by the 26th day of gestation?
FALSE

28TH DAY OF GESTATION
What are the characteristics of Prader-Willi Syndrome?
*SMALL HANDS, FEET, AND SEX ORGANS
*HYPOTONIA
*ALMOND SHAPED EYES
*OBESITY
*CONSTANT DESIRE FOR FOOD
*COORDINATION IMPAIRMENTS AND MENTAL RETARDATION
What is the causative factor of Prader-Willi Syndrome?
PARTIAL DELETION OF CHROMOSOME 15
When do the characteristics of Duchenne Muscular Dystrophy usually start to show?
BETWEEN 2 AND 5 YEARS OF AGE
When does death typically occur with Duchenne Muscular Dystrophy?
PRIOR TO AGE 25

(USUALLY TEENAGE YEARS)
Cell membranes weaken, myofibrils are destroyed, and muscle contractility is lost. This is a description of?
DUCHENNE MUSCULAR DYSTROPHY
What muscle proteins are missing with Duchenne Muscular Dystrophy?
*DYSTROPHIN
*NEBULIN
True/False

Duchenne Muscular Dystrophy is a non progressive disorder?
FALSE

PROGRESSIVE
Treatment of trisomy 21 include?
*EXERCISE AND FITNESS
*STABILITY
*MAXIMIZING RESPIRATORY FUNCTION
*EDUCATION FOR CAREGIVERS
What are the characteristics of Down Syndrome?
*MENTAL RETARDATION
*HYPOTONIA
*JOINT HYPERMOBILITY
*FLATTENED NASAL BRIDGE
*NARROW EYELIDS
*SMALL MOUTH
*FEEDING IMPAIRMENTS
*FLAT FEET
*SCOLIOSIS
*CONGENITAL HEART DISEASE
*VISUAL AND HEARING LOSS
True/False

Trisomy 21 is a genetic abnormality?
TRUE
Down Syndrome is AKA?
TRISOMY 21
Athetosis?
SLOW, INVOLUNTARY, WRITHING, TWISTING, "WORMLIKE" MOVEMENTS OCCUR
True/False

Arthrogryposis Multiplex Congenita is a non-progressive disorder?
TRUE
What are the characteristics of Arthrogryposis Multiplex Congenita?
*CYLINDER-LIKE EXTREMITIES
*MULTIPLE CONTRACTURES
*DISLOCATION OF JOINTS
*MUSCLE ATROPHY
Gryposis?
ABNORMAL CURVATURE

(ARTHROGRYPOSIS MULTIPLEX CONGENITA)
An abnormal curvature of the joints during utero leading to contractures in the muscles?
ARTHROGRYPOSIS MULTIPLEX CONGENITA
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