1. Good Morning Friday, July 19 th, 2013

4. Neurologic Exam in Children

5. Neurologic Exam Components – General Assessment – Mental Status – Cranial Nerves – Motor – Sensation – Reflexes – Cerebellum – Gait

6. General Assessment Vital Signs Developmental Stage General inspection of Patient – Dysmorphic features – Appropriate size, weight, head circumference – Voluntary or purposeful movement – Posture

7. Physical Exam Findings Skin – Hypopigmented patches, facial hemangiomas, café au lait spots, Spine – Visualize spine, gluteal folds Head – Size, sutures, fontanel, swelling

9. Mental Status Orientation Language Memory – Breakfast (short term) – Name of teacher/school (long term) – Counting or Alphabet Attention, Concentration, Executive Function, Calculations, Praxis – Spell world backwards – Serial 7s Level of Consciousness – One of the most important parts of the neuro exam – Glascow Coma Scale Mood, Thought Content

10. Infant Scale 5. Coos, babbles 4. Irritable cries 3. Cries to pain 2. Moans to pain 1. No response 6. Spontaneous, purposeful movement 5. Withdraws to touch 4. Withdraws to pain 3. Decorticate posture to pain (flexion) 2. Decerebrate posture to pain (extension) 1. No response

12. Cranial Nerves Infants: mainly observational I olfactory: rarely tested II optic: – Visual acuity: infant reaching for objects, child recognizing objects/letters/numbers – Visual fields: objects in the periphery – Pupillary light response: direct and consensual – Fundoscopy

13. Fundoscopy

15. Cranial Nerves III oculomotor: Superior/Inferior/Medial recti, inferior oblique, levator palpebrae superioris, autonomic muscles

16. Cranial Nerves IV trochlear: superior oblique, pulls down/in

17. Cranial Nerves VI abducens: lateral rectus, abducts

18. Corneal Light Reflex

19. Abnormal Eye Movements Nystagmus Tonic horizontal deviation Tonic downward gaze deviation Unilateral dilated, poorly reactive pupil Unilateral constricted pupil

20. Cranial Nerves V Trigeminal: – sensation of face, cornea, conjunctiva – Motor function of masseter, temporalis, pterygoids (muscles of mastication) – Lacrimation, taste

21. Cranial Nerves VII facial: – Symmetry of nasolabial folds – Eyelid muscle strength – Ability to wrinkle forehead – Infants Closes both eyes when crying Look for symmetric suck, spillage to one side (V, VII, IX, X, XII) – Taste

22. Cranial Nerves VII facial nerve palsy

23. Cranial Nerves VIII Vestibulocochlear: – Infants: alerting reponse to sound, localizes to sound – Children: whisper number or letter – Older children: Webber and Rinne – Vestibular Poor head control, truncal instability, gait ataxia, N/V, nystagmus (horizontal)

24. Cranial Nerves Weber Can detect – unilateral sensorineural (inner) – unilateral conductive (middle)

26. Weber without lateralization Weber lateralizes left Weber lateralizes right Rinne both ears AC>BC Normal/bilateral sensorineural loss Sensorineural loss in right Sensorineural loss in left Rinne left BC>AC Conductive loss in left Combined loss : conductive and sensorineural loss in left Rinne right BC>AC Combined loss : conductive and sensorineural loss in right Conductive loss in right Rinne both ears BC>AC Conductive loss in both ears Combined loss in right and conductive loss on left Combined loss in left and conductive loss on right

27. Cranial Nerves IX Glossopharyngeal and X Vagus: – Controls swallowing, soft palate movement, gag reflex – Drooling, pooling saliva can be a sign of dysfunction – Hoarseness can be a sign of CN X dysfunction – Dysarthria (IX, X and XII)

28. Cranial Nerves XI Spinal Accessory: trapezius and sternocleidomastoids – Elevation of shoulders, turning neck against resistance XII Hypoglossal: innervates the tongue – Tongue should be midline on protrusion – Deviation to affected side indicates palsy – Atrophy, fasiculations or oromotor apraxia can be a sign of dysfunction or serious illness

30. Motor - Infants Passive Tone – <28 wga: limbs passively extended – 34 wga: flexion of knee/hip, extended UE – 40 wga: strong flexion in all four extremities Active Muscle Activity Posture

31. Ballard Scale

33. Motor - Infants Active Muscle Activity – Symmetric, smooth, spontaneous movements (34 wga) – Small amplitude, choeo-athetoid movements of hands are normal – Jitteriness, tremulousness can occur, but should not be sustained – Reflexes – stepping reflex – Head Control – Vertical and Ventral Suspension

34. Motor - Infants Hypotonia – Most common motor abnormality – Hypotonia + preserved mobility + hyperreflexia Central nervous system origin (Down Syndrome) – Hypotonia + weakness + areflexia Anterior horn cell disorder Peripheral nerve or peripheral muscle disorder

35. Motor - Infants Hypertonia – Less common than hypotonia – Pyramidal tract dysfunction – Passive movement of the muscle  resistance – Associated with hypoxic-ischemic lesions – Difficulty determining spasticity vs. rigidity Spasticity (meaning to draw or tug) involuntary, velocity- dependent, increased muscle tone that results in resistance to movement Rigidity - Involuntary, bidirectional, non – velocity- dependent resistance to movement

36. Motor - Infants Opisthotonus – Arching of the neck and trunk – Decreased cortical inhibition – Associated with bilirubin encephalopathy, tetanus, TBI, CP, meningeal irritation/increased ICP

37. Motor - Children Observe them at play Muscle tone – Resistance felt upon passive movement – Hypotonia = decreased resistence + hyperextension – Hypertonia Spasticity (clasp knife) pyramidal Rigidity (lead pipe or cog-wheel feel) extrapyramidal

38. Motor - Children Weakness or Strength – Grades 0/5: no muscle movement at all 1/5: visible/palpable contraction, but No Movement 2/5: movement with gravity eliminated 3/5: movement against gravity 4/5: movement against gravity and some externally applied resistance 5/5: movement against gravity and full resistance

39. Motor - Children Distal Weakness – Can be symmetric or asymmetric – Seen in peripheral myopathies Proximal Weakness – Usually symmetric – Seen in myopathyies – Gower’s sign

40. Motor - Children Pronator Drift – extend UE with palms up, eyes closed – UMN weakness Arm pronates and falls, can pulls elbow down and in – Cerebellar disease One arm rises up or oscillates

41. UMN vs LMN Spasticity +/- weakness Stiffness +/- mild atrophy Increased Reflexes/Tone Spontaneous clonus Spontaneous spasms Gait – Slow, stiff, difficult to turn – Heavy legs Weakness – Foot drop, difficulty w/ stairs Decreased Reflexes/Tone Muscle atrophy Fasciculations Cramps Gait – Steppage, waddling

42. LMN signs http://www.uptodate.com/contents/clinical- features-of-amyotrophic-lateral-sclerosis-and- other-forms-of-motor-neuron- disease?detectedLanguage=en&source=searc h_result&search=upper+motor+neuron&selec tedTitle=1%7E150&provider=noProvider#H11 http://www.uptodate.com/contents/clinical- features-of-amyotrophic-lateral-sclerosis-and- other-forms-of-motor-neuron- disease?detectedLanguage=en&source=searc h_result&search=upper+motor+neuron&selec tedTitle=1%7E150&provider=noProvider#H11

44. Sensation

45. Child – Vibration (128 or 256 Hz tuning fork) – Proprioception (Romberg) – Light Touch (cotton swab) – Temperature and Pin Prick – Two point discrimination, stereognosis, graphesthesia Infant – difficult to assess – Perioral tactile stimulation  rooting reflex – Spinal cord lesion Only time pin prick testing is useful in infants

46. Romberg Sign

48. Reflexes - Infant

49. Other reflexes Babinski reflex Abdominal reflex Cremasteric reflex Perianal reflex

50. Reflexes - Children Jaw – tap chin with mouth slightly open Biceps – with elbow flexed, tap at antecubital fossa Brachioradialsis - tap above the wrist, on radial aspect  flexion of elbow Patellar – tap quadriceps tendon below patella  extension of knee Ankle (Achilles)

51. Reflexes - Children Absent or diminished – Can be loss of sensory fibers – Can be loss of muscle stretch reflex arc Exaggerated – Clonus – Knee jerk  contralateral hip muscle and plantar foot flexion

52. Reflexes Grading – 0 absent – 1 Decreased (Hypoactive) – 2 normal – 3 Increased (Hyperactive) – 4 Clonus

54. Cerebellar Dysmetria: Difficulty regulating rate and range of muscle contraction – Nystagmus – Intention tremor – Scanning speech – Truncal or gait ataxia – Rebound phenomenon

56. Gait Assess gait, heel walking, toe walking and tandem gait Circumduction (spasticity, hemiparesis) Broad-based, ataxic (cerebellar) High-steppage gait (peripheral neuropathy) Waddling gait (myopathy, DMD)

57. Noon Conference