CONGENITAL AND BRAIN MALFORMATIONS (based on Tagnawa T) PART 1 Flashcards

1
Q

What is the importance of a pediatric neurological examination?

A

It provides essential data for anatomical localization, reassures families, and helps evaluate disorders over time.

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2
Q

What are the main points to remember in pediatric neurological examination?

A

Adapt the exam to the child’s temperament and developmental level; know the expected neurodevelopmental milestones; observe the child’s behavior, walking, talking, and playing.

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3
Q

What are the elements of a complete neurological assessment?

A

Focused clinical history, physical examination, and complete neurological examination.

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4
Q

What is the first step in diagnosing a neurological problem?

A

Identify if there is a neurologic problem and localize where the lesion is.

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5
Q

What should be considered in the history of present illness for a neurological issue?

A

Duration of symptoms, whether they are constant/episodic, static/progressive/resolving, and the anatomical localization suggested by the history.

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6
Q

What are common red flags in pediatric neurology history?

A

Headache, changes in sensorium, weakness, changes in vision/hearing/response, and developmental milestone delays.

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7
Q

What is the difference between upper motor neuron and lower motor neuron lesions?

A

Upper motor neuron lesions involve the brain and spinal cord, while lower motor neuron lesions involve the anterior horn cell, nerve, neuromuscular junction, and the muscles it innervates.

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8
Q

What are the four basic questions to clarify the history of a current illness?

A

Is the process acute or insidious? Is it focal or generalized? Is it progressive or static? At what age did the problem begin?

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9
Q

What is the temporal profile of neurological disease?

A

Acute (seconds, minutes, hours), subacute (hours to 10 days), chronic (2 weeks or more), paroxysmal (episodic with returns to baseline).

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10
Q

What are examples of acute focal neurological conditions?

A

Vascular/infarct (e.g., ruptured aneurysm, stroke), hypoxic events, and trauma.

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11
Q

What are examples of subacute neurological conditions?

A

Inflammatory/infectious conditions, immune-mediated disorders, and toxic/metabolic issues.

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12
Q

What are examples of chronic neurological conditions?

A

Congenital abnormalities, degenerative diseases, and neoplastic conditions.

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13
Q

What are paroxysmal neurological conditions?

A

Seizures (focal or diffuse), vascular/syncope (diffuse), and pain/headache (focal or diffuse).

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14
Q

What is the difference between progressive and static neurological conditions?

A

Progressive conditions worsen over time (e.g., growing brain tumor), while static conditions remain unchanged (e.g., cerebral palsy).

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15
Q

What are examples of diagnostic tools in pediatric neurology?

A

Focused history, physical and neurological examination, developmental screening tests, and laboratory evaluations.

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16
Q

How can the age of onset help in diagnosing a neurologic condition?

A

Congenital problems are present at birth, while acquired problems develop later in life.

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17
Q

What is the most severe cause of an acute headache in the first 24 hours?

A

A vascular problem, such as a ruptured aneurysm or stroke.

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18
Q

What is the usual cause of an insidious or chronic headache?

A

A brain tumor, which progressively worsens over time.

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19
Q

What are common congenital anomalies of the CNS?

A

Conditions like neural tube defects, Chiari malformations, and agenesis of the corpus callosum.

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20
Q

What are the clinical features of cerebral palsy?

A

Motor impairments that are static over time, often involving spasticity, dyskinesia, or ataxia.

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21
Q

What are examples of neurocutaneous syndromes?

A

Neurofibromatosis, Tuberous Sclerosis, and Sturge-Weber Syndrome.

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22
Q

What are key features of seizures?

A

Episodic disturbances of movement, sensation, behavior, or consciousness caused by abnormal neuronal activity.

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23
Q

What conditions mimic seizures?

A

Syncopal events, migraines, and psychogenic nonepileptic seizures.

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24
Q

What are common pediatric CNS infections?

A

Meningitis, encephalitis, and brain abscess.

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25
Q

What are signs of a pediatric stroke?

A

Focal neurological deficits such as hemiparesis, aphasia, or visual disturbances.

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26
Q

What are examples of demyelinating disorders of the CNS?

A

Multiple sclerosis and acute disseminated encephalomyelitis (ADEM).

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27
Q

What are examples of neuromuscular disorders?

A

Muscular dystrophies, myasthenia gravis, and Guillain-Barré Syndrome.

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28
Q

What is Bell’s Palsy?

A

A condition characterized by sudden, unilateral facial paralysis due to facial nerve dysfunction.

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29
Q

What is the main purpose of a neurological examination?

A

It serves as the window through which the clinician views the nervous system.

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30
Q

What tools can be used to attract a child’s attention during a neurologic examination?

A

Tennis ball, small toys, bell, or any object that attracts attention.

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31
Q

Why should uncomfortable tasks in a neurologic exam be postponed until the end?

A

Because once the child cries, it becomes difficult to elicit other symptoms or findings.

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32
Q

How can observation during play help in a neurologic examination?

A

It can reveal handedness, motor deficits, gait abnormalities, or lack of response to stimuli.

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33
Q

How should a younger child be positioned during the neurologic examination?

A

They should be examined in the parent’s lap.

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34
Q

What additional tools are useful for a comprehensive neurological exam?

A

Snellen chart, tongue depressor, cotton wisp, percussion hammer, sensory reflex hammer, and items like coins, paper clips, or substances with distinct smells and tastes.

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35
Q

What should be the first step in documenting a pediatric neurological examination?

A

Start with a general physical examination and mental status evaluation.

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36
Q

Why must the mental status assessment be age-appropriate?

A

You cannot describe behaviors or responses that are developmentally impossible for the child’s age.

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37
Q

What are the key components of a mental status examination?

A

Appearance, behavior, social interaction, motor activity, mood, affect, speech, thought processes, and intellectual functioning.

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38
Q

What is lethargy in terms of consciousness levels?

A

A state where the child has difficulty maintaining arousal and sleeps most of the time.

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39
Q

What does obtundation mean?

A

The child is responsive to stimulation like sound or touch but not to pain.

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40
Q

What is stupor in consciousness levels?

A

The child is only responsive to painful stimuli.

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41
Q

What is the definition of coma in terms of consciousness?

A

A state of unresponsiveness to pain or any stimuli.

42
Q

What are the primary signs of craniosynostosis?

A

Ridging of cranial sutures and early closure of the anterior fontanelle before 9–18 months.

43
Q

What is the significance of scalp vein prominence in children?

A

It may indicate increased intracranial pressure (ICP).

44
Q

What does a flattened occiput indicate?

A

It is often associated with hypotonia.

45
Q

What is the function of cranial nerve I (olfactory)?

A

It is responsible for the sense of smell.

46
Q

What tools can be used to test cranial nerve II (optic nerve)?

A

Snellen chart, Ishihara test for color vision, and fundoscopic examination.

47
Q

What is the function of cranial nerves III, IV, and VI?

A

They control eye movements and are tested through observing movements in various visual fields.

48
Q

How can cranial nerve V (trigeminal nerve) be tested?

A

Sensation over the forehead, cheeks, and chin, and motor functions like clenching teeth.

49
Q

What is the motor function of cranial nerve VII (facial nerve)?

A

It controls movements of facial muscles, such as raising eyebrows and smiling.

50
Q

What is the sensory function of cranial nerve VII (facial nerve)?

A

Taste from the anterior two-thirds of the tongue.

51
Q

How can cranial nerve VIII (vestibulocochlear nerve) be evaluated?

A

Through hearing tests like Rinne and Weber tests or observing balance and response to auditory stimuli.

52
Q

What is the function of cranial nerves IX and X?

A

They are responsible for swallowing, gag reflex, and functions of the soft palate, pharynx, and larynx.

53
Q

How can cranial nerve XI (accessory nerve) be tested?

A

By asking the child to turn their head or shrug their shoulders against resistance.

54
Q

What is the role of cranial nerve XII (hypoglossal nerve)?

A

It controls tongue movements and is tested by asking the child to stick out their tongue or speak.

55
Q

What is the normal head circumference for newborns?

A

33–35 cm.

56
Q

What is the significance of unusual odors in pediatric patients?

A

Urine smelling like maple syrup may indicate an inborn error of metabolism like maple syrup urine disease (MSUD).

57
Q

How is the motor function assessed in a pediatric neurologic exam?

A

By observing muscle strength, tone, and movements, and checking reflexes.

58
Q

What are the components of a sensory assessment in a neurologic exam?

A

Touch, pain, temperature, vibration, and position sense.

59
Q

What are some examples of developmental screening tests?

A

Denver Developmental Screening Test and Ages & Stages Questionnaires.

60
Q

How do you test balance and coordination in a pediatric patient?

A

Through activities like walking, hopping, or catching a ball.

61
Q

Why is documenting a neurological exam over time important?

A

It helps track the progression of disorders that evolve over time.

62
Q

What are examples of physical signs indicating neurocutaneous syndromes?

A

Abnormalities in skin pigmentation, hair texture, or scalp lesions.

63
Q

At what age does the anterior fontanelle usually close?

A

Between 9 and 18 months.

64
Q

What is the significance of the Macewen sign (cracked pot sound)?

A

It indicates increased intracranial pressure or separated cranial sutures.

65
Q

How do you test for extraocular muscle movement in infants?

A

By moving a colorful toy and observing their gaze.

66
Q

What is the caloric test used for?

A

To determine the status of cranial nerve VIII (vestibulocochlear) and brainstem function, often in brain death assessment.

67
Q

Why is the Snellen chart used in older children?

A

To assess visual acuity in children who can identify letters or pictures.

68
Q

What are motor skills observed in a pediatric neurological examination?

A

Ability to walk, sit, raise hands, squeeze fingers, hop, skip, or jump.

69
Q

How can balance be checked in a pediatric patient?

A

By assessing how the child stands and walks or having them stand with eyes closed while being gently pushed to one side.

70
Q

What is Romberg’s test used for?

A

It tests the dorsal column and balance by having the child stand with eyes closed (support required to prevent falls).

71
Q

How is muscle strength graded on a scale of 0 to 5?

A

0: No contraction;
1: Slight contraction, no movement;
2: Full range of motion, not against gravity;
3: Full range of motion against gravity;
4: Full range of motion against some resistance;
5: Full range of motion against full resistance.

72
Q

How is motor strength described in younger children?

A

By observing movements like flexion, extension, or grasping, since they cannot follow instructions for resistance tests.

73
Q

Why is sensory examination challenging in children under 7 years old?

A

They cannot reliably quantify or describe sensations.

74
Q

What tools are used in a sensory examination?

A

Dull needles, tuning forks, alcohol swabs, or objects to test sensations like hot, cold, sharp, or dull.

75
Q

What does cerebellar examination assess?

A

Coordination, timing of muscle contractions, and smoothness of movements.

76
Q

What are key tests for cerebellar function?

A

Finger-to-nose test, dysdiadochokinesia test, and observing gait for ataxia or drunken gait.

77
Q

What is dysmetria?

A

A condition where movements are clumsy and overshoot the target, indicating cerebellar dysfunction.

78
Q

What does an intention tremor indicate?

A

It appears toward the end of a movement, suggesting cerebellar disease.

79
Q

What is the function of reflex testing in pediatric neurology?

A

It helps identify normal, hyperreflexic, or hyporeflexic responses to assess upper or lower motor neuron problems.

80
Q

How are reflexes tested in children?

A

By using a reflex hammer on points like the knee or elbow while supporting the area with fingers to reduce discomfort.

81
Q

What are meningeal signs assessed during a neurologic exam?

A

Nuchal rigidity, Kernig’s sign, and Brudzinski’s sign.

82
Q

At what age are meningeal signs like Kernig’s and Brudzinski’s appreciable?

A

Beyond 18 months; lumbar puncture is recommended for younger children.

83
Q

What does nuchal rigidity indicate?

A

A sign of meningeal irritation, such as in meningitis.

84
Q

What is neuroembryology?

A

The process of nervous system development from the neural tube to the central and peripheral nervous systems.

85
Q

Why is the CNS the last to mature postnatally?

A

Because the nervous system continues to develop and refine connections even after birth.

86
Q

What is the approach to diagnosing congenital anomalies?

A

Recognize abnormalities, make an accurate diagnosis, provide a realistic prognosis, and discuss management options with the family.

87
Q

How does understanding neuroembryology aid in discussing congenital anomalies?

A

It helps explain when and how anomalies occurred during fetal development.

88
Q

What common misconceptions exist about congenital anomalies?

A

Myths and misinformation about their causes persist despite modern resources and internet access.

89
Q

How can congenital anomalies be identified prenatally?

A

Through congenital screening during pregnancy.

90
Q

What are the steps to manage congenital anomalies postnatally?

A

Accurate diagnosis, discussing prognosis, managing associated medical problems, and delivering appropriate care.

91
Q

What questions should be answered when assessing congenital anomalies?

A

Where are the problems? What are the problems? What is the diagnosis? What are the associated problems?

92
Q

What does overshooting a target during hand movement suggest?

A

Dysmetria, often caused by cerebellar dysfunction.

93
Q

How do cerebellar lesions differ from cerebral lesions?

A

Cerebellar lesions cause ipsilateral symptoms, while cerebral lesions cause contralateral symptoms.

94
Q

What is the importance of explaining the timing of congenital anomalies to parents?

A

It helps them understand when the problem occurred and dispels myths about the causes.

95
Q

What role does gait observation play in a pediatric neurological exam?

A

It helps identify motor deficits, ataxia, or other abnormalities related to cerebellar function.

96
Q

What should be noted when assessing reflexes in pediatric patients?

A

Whether the reflexes are normal, hyperreflexic, or hyporeflexic, and if they indicate an upper or lower motor neuron issue.

97
Q

Why is it essential to support a child during balance tests?

A

To prevent falls and ensure the safety of the child.

98
Q

How is cerebellar function tested in younger children?

A

By introducing small objects for the child to reach and observing the smoothness of movements.

99
Q

What are the key signs of cerebellar disease?

A

Clumsy, unsteady movements, variable speed and force, and intention tremors.

100
Q

What is the importance of accurate prognosis in congenital anomalies?

A

It helps families set realistic expectations and plan for management and care.