Nervous System Examination App

Pediatric CNS Examination Proforma
Higher Mental Function Examination in Pediatrics
1. Level of Consciousness
Technique:
  • Observe the child's general alertness and responsiveness
  • Use age-appropriate stimuli to assess responsiveness
  • Apply the AVPU scale (Alert, Voice, Pain, Unresponsive)
  • For older children, use the Glasgow Coma Scale (GCS)
Normal Findings:
  • Alert and responsive to age-appropriate stimuli
  • Appropriate interaction with examiner and environment
  • GCS score of 15 (for older children)
Abnormal Findings:
  • Decreased level of consciousness
  • Fluctuating alertness
  • Inability to maintain wakefulness
  • GCS score below 15
Associated Conditions:
  • Intracranial pathologies:
    • Traumatic brain injury
    • Intracranial hemorrhage
    • Brain tumors
  • Metabolic disorders:
    • Hypoglycemia
    • Electrolyte imbalances
  • Infections:
    • Meningitis
    • Encephalitis
  • Toxin exposure or drug effects
2. Attention and Concentration
Technique:
  • For younger children:
    • Observe ability to focus on toys or activities
    • Assess response to calling their name
  • For older children:
    • Digit span test (forward and backward)
    • Serial subtraction (e.g., counting backward from 100 by 7s)
    • Spelling words backward
Normal Findings:
  • Age-appropriate attention span
  • Ability to complete tasks without significant distraction
  • Correct performance on attention tests (e.g., digit span of 5-7 for school-age children)
Abnormal Findings:
  • Short attention span relative to age
  • Easy distractibility
  • Inability to complete age-appropriate attention tasks
  • Perseveration (repetitive focus on a particular topic or task)
Associated Conditions:
  • Neurodevelopmental disorders:
    • Attention Deficit Hyperactivity Disorder (ADHD)
    • Autism Spectrum Disorder
  • Learning disabilities
  • Anxiety disorders
  • Depression
  • Sleep disorders
  • Certain medications (e.g., antihistamines, some antiepileptics)
3. Memory
Technique:
  • Immediate recall:
    • Ask the child to repeat a short list of words or numbers
  • Short-term memory:
    • Ask the child to recall the list after a few minutes
    • Use age-appropriate stories or pictures for recall
  • Long-term memory:
    • Ask about personal information (e.g., birthdate, address)
    • Inquire about recent events or learned information
Normal Findings:
  • Age-appropriate immediate recall (e.g., 3-5 items for school-age children)
  • Ability to remember recent events and learned information
  • Accurate recall of personal information
Abnormal Findings:
  • Impaired immediate recall
  • Difficulty remembering recent events (short-term memory loss)
  • Inability to recall well-established personal information
  • Confabulation (creating false memories to fill gaps)
Associated Conditions:
  • Traumatic brain injury
  • Epilepsy, especially temporal lobe epilepsy
  • Neurodevelopmental disorders:
    • Autism Spectrum Disorder
    • Intellectual disability
  • Psychiatric conditions:
    • Depression
    • Anxiety disorders
  • Certain medications (e.g., benzodiazepines, some antiepileptics)
  • Nutritional deficiencies (e.g., thiamine deficiency)
4. Language
Technique:
  • Assess receptive language:
    • Ask the child to follow simple and complex commands
    • Use age-appropriate questions to test comprehension
  • Evaluate expressive language:
    • Engage in conversation
    • Ask the child to name objects or describe pictures
    • For older children, assess reading and writing skills
  • Check for fluency and articulation
Normal Findings:
  • Age-appropriate understanding of spoken language
  • Ability to express thoughts clearly and coherently
  • Appropriate vocabulary for age
  • Normal fluency and articulation
Abnormal Findings:
  • Receptive aphasia: Difficulty understanding spoken or written language
  • Expressive aphasia: Difficulty producing speech or writing
  • Dysarthria: Impaired articulation
  • Dysfluency: Stuttering or cluttering
  • Echolalia: Repetition of words or phrases
Associated Conditions:
  • Developmental language disorders
  • Autism Spectrum Disorder
  • Specific learning disorders (e.g., dyslexia)
  • Hearing impairment
  • Neurological conditions:
    • Stroke (rare in children)
    • Brain tumors
    • Epilepsy
  • Genetic syndromes (e.g., Down syndrome, Fragile X syndrome)
5. Executive Functions
Technique:
  • Assess problem-solving skills:
    • Use age-appropriate puzzles or games
    • Present hypothetical situations for older children
  • Evaluate planning and organization:
    • Ask the child to describe how they would plan a simple task
    • For older children, use tasks like the Tower of London test
  • Test cognitive flexibility:
    • Use card sorting tasks (e.g., Wisconsin Card Sorting Test for older children)
    • Observe ability to switch between tasks
  • Assess impulse control and inhibition:
    • Use go/no-go tasks
    • Observe behavior during the examination
Normal Findings:
  • Age-appropriate problem-solving skills
  • Ability to plan and organize thoughts and actions
  • Cognitive flexibility in adapting to new rules or situations
  • Appropriate impulse control for age
Abnormal Findings:
  • Difficulty with problem-solving or abstract thinking
  • Poor planning and organizational skills
  • Cognitive rigidity or perseveration
  • Impulsivity or disinhibited behavior
  • Difficulty initiating or completing tasks
Associated Conditions:
  • Attention Deficit Hyperactivity Disorder (ADHD)
  • Autism Spectrum Disorder
  • Traumatic brain injury, especially frontal lobe injuries
  • Fetal Alcohol Spectrum Disorders
  • Certain genetic syndromes (e.g., Williams syndrome)
  • Childhood-onset schizophrenia (rare)
  • Pediatric stroke affecting frontal lobes
Note:

The assessment of higher mental functions in pediatrics should always be interpreted in the context of the child's age, developmental stage, and individual variability. Standardized neuropsychological tests may be necessary for a more comprehensive evaluation.

Cranial Nerve Examination
Olfactory Nerve (CN I)
Examination Technique:
  • Use age-appropriate, non-irritating scents (e.g., vanilla, peppermint, chocolate, coffee).
  • Occlude one nostril and present the scent to the open nostril.
  • Ask the child to sniff and identify or describe the smell.
  • Repeat with the other nostril.
  • For infants and young children, observe for facial expressions, head turning, or changes in respiratory patterns in response to scents.
Interpretation of Findings:
  • Normal:
    • Child can detect and differentiate between various scents.
    • Infants may show subtle changes in facial expression or head turning towards pleasant smells.
  • Abnormal:
    • Anosmia: Complete inability to detect any odors in one or both nostrils.
    • Hyposmia: Reduced ability to detect odors.
    • Dysosmia: Distorted perception of odors.
    • Phantosmia: Perception of odors that are not present.
Associated Conditions:
  • Anosmia:
    • Congenital: Kallmann syndrome, familial anosmia
    • Acquired: Post-viral olfactory dysfunction (e.g., following COVID-19), severe head trauma with cribriform plate fracture
  • Hyposmia:
    • Chronic rhinosinusitis
    • Adenoid hypertrophy
    • Mild traumatic brain injury
  • Dysosmia:
    • Post-infectious olfactory dysfunction
    • Temporal lobe epilepsy
  • Phantosmia:
    • Migraine with aura
    • Olfactory hallucinations in certain psychiatric conditions
Optic Nerve (CN II)
Examination Technique:
  • Visual acuity:
    • Use age-appropriate methods: Picture charts (e.g., LEA symbols) for young children, Snellen chart for older children.
    • Test each eye separately, with proper correction if needed.
  • Visual fields:
    • Confrontation testing: Use finger counting or wiggling in each quadrant.
    • Toy perimetry for younger children: Present small toys in different areas of the visual field.
  • Color vision:
    • Ishihara plates for older children.
    • Sorting colored objects for younger children.
  • Fundoscopy:
    • Examine optic disc for color, margins, and cup-to-disc ratio.
    • Assess retinal vasculature and macular region.
  • Pupillary light reflex:
    • Check direct and consensual responses.
    • Assess for relative afferent pupillary defect (RAPD).
Interpretation of Findings:
  • Visual acuity:
    • Normal: Age-appropriate acuity (e.g., 20/20 to 20/40 for school-age children)
    • Abnormal: Reduced acuity in one or both eyes
  • Visual fields:
    • Normal: Full visual fields in all quadrants
    • Abnormal: Visual field defects (e.g., hemianopia, quadrantanopia)
  • Color vision:
    • Normal: Able to identify or sort colors correctly
    • Abnormal: Difficulty distinguishing certain colors, particularly red-green
  • Fundoscopy:
    • Normal: Clear disc margins, normal cup-to-disc ratio, no swelling or pallor
    • Abnormal: Papilledema, optic atrophy, increased cup-to-disc ratio
  • Pupillary light reflex:
    • Normal: Prompt, equal constriction to light in both pupils
    • Abnormal: RAPD, sluggish or absent response
Associated Conditions:
  • Reduced visual acuity:
    • Refractive errors (myopia, hyperopia, astigmatism)
    • Amblyopia
    • Optic neuritis
    • Retinopathy of prematurity
  • Visual field defects:
    • Bitemporal hemianopia: Craniopharyngioma, pituitary adenoma
    • Homonymous hemianopia: Occipital lobe lesions, stroke
    • Central scotoma: Optic neuritis, Leber's hereditary optic neuropathy
  • Color vision abnormalities:
    • Congenital color blindness (more common in males)
    • Acquired: Optic neuritis, Leber's hereditary optic neuropathy
  • Fundoscopic changes:
    • Papilledema: Idiopathic intracranial hypertension, brain tumors
    • Optic atrophy: Compressive lesions, multiple sclerosis, hereditary optic neuropathies
    • Increased cup-to-disc ratio: Juvenile glaucoma
  • Pupillary abnormalities:
    • RAPD: Unilateral or asymmetric optic nerve disease
    • Fixed, dilated pupil: Oculomotor nerve palsy, pharmacologic mydriasis
Oculomotor Nerve (CN III)
Examination Technique:
  • Pupillary response:
    • Assess direct and consensual light reflexes.
    • Check for anisocoria in light and dark conditions.
  • Eye movements:
    • Assess upward, downward, and medial gaze.
    • Check for nystagmus or restricted movement.
  • Eyelid position:
    • Observe for ptosis (drooping eyelid).
    • Compare eyelid position bilaterally.
  • Accommodation:
    • Ask the child to focus on a near object, then a distant one.
    • Observe for convergence and pupillary constriction.
Interpretation of Findings:
  • Pupillary response:
    • Normal: Equal, reactive pupils that constrict to light and accommodate.
    • Abnormal: Pupillary dilatation, non-reactivity, or anisocoria.
  • Eye movements:
    • Normal: Full range of eye movements in all directions.
    • Abnormal: Restricted movement, nystagmus.
  • Eyelid position:
    • Normal: Eyelids are symmetric and fully open.
    • Abnormal: Ptosis, asymmetry.
  • Accommodation:
    • Normal: Smooth accommodation with appropriate pupillary constriction.
    • Abnormal: Difficulty with near focus, impaired convergence.
Associated Conditions:
  • Pupillary abnormalities:
    • Myasthenia gravis
    • Third cranial nerve palsy (e.g., due to aneurysm, diabetes, trauma)
    • Adie's pupil (tonic pupil)
  • Eye movement disorders:
    • Oculomotor nerve palsy
    • Internuclear ophthalmoplegia
    • Thyroid eye disease
  • Eyelid abnormalities:
    • Horner's syndrome
    • Myasthenia gravis
  • Accommodation issues:
    • Accommodation insufficiency
    • Presbyopia
Trochlear Nerve (CN IV)
Examination Technique:
  • Eye movement:
    • Assess downward gaze, especially in adduction (looking down and in).
    • Observe for head tilt (compensatory mechanism).
  • Cover test:
    • Perform alternate cover test in primary gaze and downgaze.
    • Look for vertical misalignment.
  • Double vision assessment:
    • Ask older children about double vision, especially when looking down.
    • Use red glass test if appropriate for age.
Interpretation of Findings:
  • Eye movement:
    • Normal: Smooth downward movement in adduction.
    • Abnormal: Limited or absent downward movement in adduction.
  • Head position:
    • Normal: No head tilt.
    • Abnormal: Compensatory head tilt away from the affected side.
  • Cover test:
    • Normal: No vertical misalignment.
    • Abnormal: Hypertropia of the affected eye, worse in contralateral gaze and ipsilateral head tilt.
  • Double vision:
    • Normal: No diplopia.
    • Abnormal: Vertical diplopia, worse in downgaze and contralateral gaze.
Associated Conditions:
  • Congenital fourth nerve palsy:
    • Often associated with large vertical fusional amplitudes
    • May have long-standing head tilt
  • Acquired fourth nerve palsy:
    • Trauma: Most common cause in children
    • Intracranial tumors: Especially in the region of the tectal plate
    • Hydrocephalus: Due to compression of the nerve in its long intracranial course
  • Ophthalmoplegic migraine:
    • Rare condition with recurrent episodes of ophthalmoplegia associated with migraine headaches
  • Myasthenia gravis:
    • Can cause variable weakness of any extraocular muscle, including superior oblique
Trigeminal Nerve (CN V)
Examination Technique:
  • Sensory function:
    • Test light touch, pain, and temperature sensation in all three divisions (V1, V2, V3).
    • Use age-appropriate methods (e.g., soft brush, cotton swab, warm/cool objects).
  • Motor function:
    • Observe jaw movement during speaking and chewing.
    • Palpate masseter and temporalis muscles during clenching.
    • Test jaw opening against resistance (for older children).
  • Corneal reflex:
    • Gently touch the cornea with a cotton wisp.
    • Observe for blink reflex (afferent: CN V, efferent: CN VII).
  • Jaw jerk reflex:
    • Tap the chin lightly with a reflex hammer while the mouth is slightly open.
    • Observe for jaw closure.
Interpretation of Findings:
  • Sensory function:
    • Normal: Equal sensation in all divisions bilaterally.
    • Abnormal: Decreased or absent sensation in one or more divisions.
  • Motor function:
    • Normal: Symmetrical jaw movement, equal muscle strength.
    • Abnormal: Weakness, asymmetry, or deviation of jaw movement.
  • Corneal reflex:
    • Normal: Prompt bilateral blink reflex.
    • Abnormal: Absent or diminished blink on the affected side.
  • Jaw jerk reflex:
    • Normal: Slight jaw closure.
    • Abnormal: Exaggerated response (upper motor neuron lesion) or absent response (lower motor neuron lesion).
Associated Conditions:
  • Sensory abnormalities:
    • Trigeminal neuralgia: Rare in children, but can occur in multiple sclerosis
    • Herpes zoster ophthalmicus: Affects V1 distribution
    • Acoustic neuroma: Can affect V1 and V2 in large tumors
  • Motor abnormalities:
    • Acute bulbar palsy: Can affect jaw muscles
    • Möbius syndrome: Congenital facial paralysis, may involve trigeminal motor function
  • Corneal reflex abnormalities:
    • Acoustic neuroma: Can affect corneal reflex
    • Brainstem glioma: May impair corneal reflex
  • Jaw jerk abnormalities:
    • Exaggerated: Pseudobulbar palsy, amyotrophic lateral sclerosis (rare in children)
    • Absent: Bulbar palsy, Guillain-Barré syndrome
Abducens Nerve (CN VI)
Examination Technique:
  • Eye movement:
    • Assess lateral gaze in both eyes independently.
    • Observe for full abduction (outward movement) of each eye.
  • Cover test:
    • Perform alternate cover test in primary position and lateral gaze.
    • Look for esotropia (inward deviation) in the affected eye.
  • Head turn:
    • Observe for compensatory head turn toward the affected side.
  • Diplopia assessment:
    • Ask older children about double vision, especially when looking to the sides.
    • Use red glass test if age-appropriate.
Interpretation of Findings:
  • Eye movement:
    • Normal: Full abduction of both eyes.
    • Abnormal: Limited or absent abduction in the affected eye.
  • Cover test:
    • Normal: No deviation in primary or lateral gaze.
    • Abnormal: Esotropia, worse in ipsilateral gaze.
  • Head position:
    • Normal: No head turn.
    • Abnormal: Compensatory head turn toward the affected side to maintain binocular single vision.
  • Diplopia:
    • Normal: No diplopia.
    • Abnormal: Horizontal diplopia, worse in ipsilateral gaze.
Associated Conditions:
  • Increased intracranial pressure:
    • Due to the long intracranial course of CN VI, it's vulnerable to increased pressure.
    • Can be seen in hydrocephalus, pseudotumor cerebri, or space-occupying lesions.
  • Pontine lesions:
    • Brainstem gliomas or other tumors in the region of the pons.
    • Pontine hemorrhage or infarction (rare in children).
  • Infections:
    • Meningitis: Can cause CN VI palsy due to inflammation or increased intracranial pressure.
    • Petrous apicitis (Gradenigo's syndrome): Involves CN VI due to its course near the petrous apex.
  • Trauma:
    • Skull base fractures involving the petrous temporal bone.
    • Severe head trauma causing stretching of the nerve.
  • Neuromuscular junction disorders:
    • Myasthenia gravis: Can cause variable weakness of any extraocular muscle.
    • Miller Fisher syndrome: A variant of Guillain-Barré syndrome with ophthalmoplegia.
Facial Nerve (CN VII)
Examination Technique:
  • Facial movements:
    • Observe symmetry at rest.
    • Ask the child to perform: smile, show teeth, puff out cheeks, raise eyebrows, close eyes tightly.
    • For infants: observe facial movements during crying.
  • Taste sensation (anterior 2/3 of tongue):
    • Test with sugar, salt, and lemon juice on the anterior tongue (if age-appropriate).
  • Hyperacusis:
    • Ask about increased sensitivity to loud sounds (in older children).
  • Tear production:
    • Observe for excessive tearing or dry eyes.
    • Perform Schirmer's test if indicated.
Interpretation of Findings:
  • Facial movements:
    • Normal: Symmetrical movement of all facial muscles.
    • Abnormal: Asymmetry, weakness, or paralysis of facial muscles.
  • Taste sensation:
    • Normal: Ability to identify different tastes on both sides of the anterior tongue.
    • Abnormal: Reduced or absent taste sensation on the affected side.
  • Hyperacusis:
    • Normal: No increased sensitivity to sounds.
    • Abnormal: Complaint of sounds seeming unusually loud.
  • Tear production:
    • Normal: Adequate and symmetric tear production.
    • Abnormal: Reduced tearing (dry eye) or excessive tearing on the affected side.
Associated Conditions:
  • Bell's palsy:
    • Most common cause of acute facial paralysis in children.
    • Typically unilateral and can involve all branches of CN VII.
  • Congenital facial palsy:
    • May be associated with other anomalies (e.g., Möbius syndrome, CHARGE syndrome).
    • Can be unilateral or bilateral.
  • Infectious causes:
    • Otitis media: Can cause facial palsy due to inflammation or direct pressure.
    • Herpes zoster oticus (Ramsay Hunt syndrome): Facial palsy with vesicular rash.
    • Lyme disease: Can cause facial palsy, often bilateral.
  • Traumatic injuries:
    • Temporal bone fractures: Can damage the facial nerve in its bony canal.
    • Birth trauma: Forceps delivery can cause temporary or permanent facial nerve injury.
  • Tumors:
    • Cerebellopontine angle tumors (e.g., acoustic neuroma): Rare in children but can affect CN VII.
    • Brainstem gliomas: Can involve the facial nerve nucleus or fascicles.
  • Guillain-Barré syndrome:
    • Can cause bilateral facial weakness as part of a more generalized polyneuropathy.
Vestibulocochlear Nerve (CN VIII)
Examination Technique:
  • Hearing assessment:
    • Perform age-appropriate hearing screening (e.g., behavioral observation, play audiometry).
    • Conduct whisper test or use tuning fork tests (Weber, Rinne) in older children.
  • Vestibular function:
    • Observe for spontaneous nystagmus.
    • Perform head impulse test (if age-appropriate).
    • Assess balance and coordination (e.g., Romberg test, tandem gait).
  • Nystagmus evaluation:
    • Observe eye movements in all directions of gaze.
    • Look for nystagmus, especially when looking laterally.
  • Symptom inquiry:
    • Ask about vertigo, dizziness, or balance problems (in older children).
    • Inquire about tinnitus or changes in hearing.
Interpretation of Findings:
  • Hearing assessment:
    • Normal: Age-appropriate responses to sounds, normal whisper test results.
    • Abnormal: Reduced or absent responses, asymmetry in hearing.
  • Vestibular function:
    • Normal: No spontaneous nystagmus, normal head impulse test, good balance.
    • Abnormal: Spontaneous nystagmus, catch-up saccade on head impulse test, impaired balance.
  • Nystagmus evaluation:
    • Normal: No nystagmus in any direction of gaze.
    • Abnormal: Presence of nystagmus, especially rotatory or vertical nystagmus.
  • Symptom inquiry:
    • Normal: No reports of vertigo, dizziness, or tinnitus.
    • Abnormal: Complaints of vertigo, dizziness, tinnitus, or hearing changes.
Associated Conditions:
  • Congenital hearing loss:
    • Genetic causes (e.g., connexin 26 mutations)
    • Congenital infections (TORCH infections)
  • Acquired hearing loss:
    • Otitis media: Common cause of conductive hearing loss in children.
    • Noise-induced hearing loss: Increasingly common in adolescents.
    • Ototoxicity: Due to certain antibiotics or chemotherapy agents.
  • Vestibular disorders:
    • Benign paroxysmal vertigo of childhood: Recurrent episodes of vertigo.
    • Post-traumatic vertigo: Following head injury.
    • Vestibular neuritis: Viral inflammation of the vestibular portion of CN VIII.
  • Tumors:
    • Acoustic neuroma (vestibular schwannoma): Rare in children but can cause progressive unilateral hearing loss.
    • Brainstem or cerebellar tumors: Can affect CN VIII.
  • Migraine-associated vertigo:
    • Can cause recurrent episodes of vertigo with or without headache.
  • Autoimmune inner ear disease:
    • Rare in children, can cause fluctuating hearing loss and vestibular symptoms.
Glossopharyngeal Nerve (CN IX)
Examination Technique:
  • Gag reflex:
    • Gently touch the posterior pharyngeal wall with a tongue depressor.
    • Observe for gag response (CN IX is afferent, CN X is efferent).
  • Taste sensation:
    • Test taste on the posterior third of the tongue (if age-appropriate).
    • Use solutions of sugar, salt, and lemon juice.
  • Swallowing assessment:
    • Observe the child drinking water.
    • Look for coughing, choking, or nasal regurgitation.
  • Palate elevation:
    • Ask the child to say "Ah" and observe palate movement.
Interpretation of Findings:
  • Gag reflex:
    • Normal: Symmetric gag reflex on both sides.
    • Abnormal: Absent or diminished gag reflex on the affected side.
  • Taste sensation:
    • Normal: Ability to identify tastes on the posterior tongue.
    • Abnormal: Reduced or absent taste sensation on the affected side.
  • Swallowing:
    • Normal: Smooth swallowing without difficulties.
    • Abnormal: Coughing, choking, or nasal regurgitation during swallowing.
  • Palate elevation:
    • Normal: Symmetric elevation of the soft palate.
    • Abnormal: Asymmetric or absent elevation on the affected side.
Associated Conditions:
  • Chiari malformation:
    • Can cause lower cranial nerve dysfunction, including CN IX.
  • Brainstem tumors or infarctions:
    • May affect the nucleus or fascicles of CN IX.
  • Skull base tumors:
    • Jugular foramen schwannomas or glomus tumors can affect CN IX.
  • Post-tonsillectomy complications:
    • Rare cases of CN IX injury during tonsillectomy.
  • Syringobulbia:
    • Can affect lower cranial nerves, including CN IX.
Vagus Nerve (CN X)
Examination Technique:
  • Voice quality:
    • Listen to the child's voice for hoarseness or nasal quality.
    • Ask older children to say "Ah" for an extended period.
  • Palate and uvula movement:
    • Ask the child to open their mouth and say "Ah".
    • Observe palate and uvula elevation and symmetry.
  • Swallowing:
    • Observe the child drinking water.
    • Look for coughing, choking, or nasal regurgitation.
  • Gag reflex:
    • Gently touch the posterior pharyngeal wall.
    • Observe for symmetric contraction (CN X is efferent).
Interpretation of Findings:
  • Voice quality:
    • Normal: Clear voice without hoarseness or nasal quality.
    • Abnormal: Hoarse, breathy, or nasal voice quality.
  • Palate and uvula movement:
    • Normal: Symmetric elevation of soft palate and uvula.
    • Abnormal: Asymmetric elevation or deviation of uvula to the unaffected side.
  • Swallowing:
    • Normal: Smooth swallowing without difficulties.
    • Abnormal: Coughing, choking, or nasal regurgitation during swallowing.
  • Gag reflex:
    • Normal: Symmetric contraction of the pharynx.
    • Abnormal: Absent or asymmetric contraction.
Associated Conditions:
  • Vocal cord paralysis:
    • Can be congenital or acquired (e.g., post-surgical, trauma).
  • Arnold-Chiari malformation:
    • May cause lower cranial nerve dysfunction, including CN X.
  • Brainstem tumors or infarctions:
    • Can affect the nucleus ambiguus or vagal fascicles.
  • Guillain-Barré syndrome:
    • May involve CN X, causing bulbar symptoms.
  • Postviral vagal neuropathy:
    • Can cause voice changes and swallowing difficulties.
Accessory Nerve (CN XI)
Examination Technique:
  • Sternocleidomastoid muscle:
    • Inspect for muscle bulk and symmetry.
    • Ask the child to turn their head against resistance.
    • Palpate for contraction of the opposite sternocleidomastoid.
  • Trapezius muscle:
    • Inspect for muscle bulk and symmetry.
    • Ask the child to shrug their shoulders against resistance.
    • Observe and palpate for trapezius contraction.
  • Functional assessment:
    • For older children, ask them to perform functional tasks (e.g., combing hair).
Interpretation of Findings:
  • Sternocleidomastoid muscle:
    • Normal: Symmetric muscle bulk and strength, able to turn head against resistance.
    • Abnormal: Atrophy, weakness, or inability to turn head against resistance on affected side.
  • Trapezius muscle:
    • Normal: Symmetric muscle bulk and strength, able to shrug shoulders against resistance.
    • Abnormal: Atrophy, weakness, or inability to shrug shoulder on affected side.
  • Functional assessment:
    • Normal: Able to perform age-appropriate functional tasks without difficulty.
    • Abnormal: Difficulty or inability to perform tasks involving shoulder elevation or head turning.
Associated Conditions:
  • Iatrogenic injury:
    • Most common cause, often due to neck surgeries (e.g., lymph node biopsy).
  • Birth trauma:
    • Can occur during difficult deliveries, especially with large birth weight.
  • Skull base tumors:
    • Jugular foramen schwannomas or glomus tumors can affect CN XI.
  • Neuromuscular disorders:
    • Muscular dystrophies or motor neuron diseases can affect CN XI-innervated muscles.
  • Chiari malformation:
    • Can cause lower cranial nerve dysfunction, including CN XI.
Hypoglossal Nerve (CN XII)
Examination Technique:
  • Tongue inspection:
    • Observe the tongue at rest for any atrophy or fasciculations.
    • Note any asymmetry or deviation.
  • Tongue movements:
    • Ask the child to stick out their tongue.
    • Request tongue movements in all directions (up, down, side-to-side).
  • Strength testing:
    • For older children, ask them to push their tongue against the inside of each cheek while you provide counter-pressure externally.
  • Speech assessment:
    • Listen for articulation difficulties, especially with lingual sounds (e.g., "la", "ta", "na").
Interpretation of Findings:
  • Tongue inspection:
    • Normal: Symmetric tongue without atrophy or fasciculations.
    • Abnormal: Atrophy, fasciculations, or asymmetry of the tongue.
  • Tongue movements:
    • Normal: Tongue protrudes straight and moves freely in all directions.
    • Abnormal: Deviation of the tongue toward the affected side when protruded, limited range of motion.
  • Strength testing:
    • Normal: Equal strength when pushing against cheeks bilaterally.
    • Abnormal: Weakness noted on the affected side.
  • Speech assessment:
    • Normal: Clear articulation of lingual sounds.
    • Abnormal: Slurred speech, particularly with difficulty pronouncing lingual sounds.
Associated Conditions:
  • Congenital hypoglossal nerve palsy:
    • Can be isolated or part of syndromes (e.g., Möbius syndrome).
  • Space-occupying lesions:
    • Brainstem gliomas or medullary tumors affecting the hypoglossal nucleus.
    • Skull base tumors near the hypoglossal canal.
  • Inflammatory conditions:
    • Multiple sclerosis (rare in young children but can occur in adolescents).
    • Guillain-Barré syndrome with bulbar involvement.
  • Vascular disorders:
    • Medullary infarctions (rare in children).
    • Vertebral artery dissection affecting the hypoglossal nerve.
  • Craniocervical junction abnormalities:
    • Chiari malformation can cause lower cranial nerve dysfunction, including CN XII.
    • Basilar invagination or other bony abnormalities compressing the nerve.
  • Iatrogenic injury:
    • Rare complication of tonsillectomy or other neck surgeries.
Motor System Examination
1. Muscle Bulk
Technique:
  • Visual inspection:
    • Observe the patient in a well-lit room.
    • Have the patient wear clothing that allows visualization of major muscle groups.
    • Compare symmetrical areas on both sides of the body.
  • Palpation:
    • Gently palpate major muscle groups to assess bulk and consistency.
    • Compare the feeling of muscle groups on both sides.
  • Measurement (if indicated):
    • Use a tape measure to quantify muscle circumference at standardized points (e.g., mid-thigh, mid-bicep).
    • Record measurements for both sides.
Normal Findings:
  • Symmetric muscle bulk appropriate for age and body habitus.
  • No visible or palpable atrophy or hypertrophy.
  • Muscle consistency firm but not hard.
  • Measurements (if taken) should be within 1 cm between sides for most muscle groups.
Abnormal Findings:
  • Atrophy:
    • Visible decrease in muscle size.
    • May be focal (affecting specific muscles) or generalized.
    • Palpation may reveal a softer consistency.
  • Hypertrophy:
    • Visible increase in muscle size.
    • May be symmetric (e.g., in athletes) or asymmetric (pathological).
    • Palpation may reveal increased firmness.
  • Pseudohypertrophy:
    • Apparent enlargement of muscles, but with decreased strength.
    • Often feels softer on palpation due to fat infiltration.
  • Fasciculations:
    • Visible, fine, rapid, and spontaneous contractions of muscle fibers.
Associated Conditions:
  • Atrophy:
    • Lower motor neuron disorders (e.g., spinal muscular atrophy, peripheral neuropathies)
    • Disuse atrophy (e.g., prolonged immobilization, casting)
    • Muscular dystrophies (in later stages)
    • Spinal cord injuries
  • Hypertrophy:
    • Physiological (e.g., athletics, weight training)
    • Myotonia congenita
    • Hypothyroidism (Hoffman's syndrome)
    • Acromegaly (in adolescents)
  • Pseudohypertrophy:
    • Duchenne muscular dystrophy (especially calf muscles)
    • Becker muscular dystrophy
  • Fasciculations:
    • Benign fasciculation syndrome
    • Spinal muscular atrophy
    • Amyotrophic lateral sclerosis (rare in children)
    • Peripheral neuropathies
2. Muscle Tone
Technique:
  • Passive range of motion:
    • Gently move major joints through their full range of motion.
    • Assess resistance to passive movement.
    • Perform quick stretches to detect spasticity.
  • Palpation:
    • Feel muscle consistency at rest.
  • Observation:
    • Note posture and resting position of limbs.
Normal Findings:
  • Appropriate resistance to passive movement.
  • Full range of motion in all joints.
  • No catch or clasp-knife response during quick stretches.
  • Muscles feel soft and relaxed at rest.
Abnormal Findings:
  • Hypotonia:
    • Decreased resistance to passive movement.
    • Increased range of motion ("floppy" appearance).
    • Muscles feel soft and doughy on palpation.
  • Hypertonia:
    • Spasticity:
      • Increased resistance to passive movement, velocity-dependent.
      • Clasp-knife phenomenon on quick stretch.
    • Rigidity:
      • Increased resistance throughout range of motion, not velocity-dependent.
      • "Lead-pipe" rigidity or "cogwheel" rigidity.
Associated Conditions:
  • Hypotonia:
    • Central causes: Down syndrome, cerebral palsy (hypotonic type), Prader-Willi syndrome
    • Peripheral causes: Spinal muscular atrophy, congenital myopathies, Guillain-Barré syndrome
    • Metabolic disorders: Hypothyroidism, rickets
  • Spasticity:
    • Cerebral palsy (spastic type)
    • Spinal cord injuries
    • Hereditary spastic paraplegia
  • Rigidity:
    • Juvenile Parkinson's disease (rare)
    • Wilson's disease
    • Neurodegeneration with brain iron accumulation (NBIA)
3. Muscle Strength
Technique:
  • Manual muscle testing:
    • Test major muscle groups against resistance.
    • Grade using the Medical Research Council (MRC) scale (0-5).
  • Functional testing (for younger children):
    • Observe age-appropriate activities (e.g., climbing stairs, rising from floor).
Normal Findings:
  • MRC grade 5/5 in all muscle groups.
  • Symmetrical strength bilaterally.
  • Age-appropriate performance in functional tasks.
Abnormal Findings:
  • Weakness:
    • MRC grade 4/5 or below in affected muscle groups.
    • May be focal, regional, or generalized.
  • Patterns of weakness:
    • Proximal weakness: Difficulty rising from floor, waddling gait.
    • Distal weakness: Foot drop, hand grip weakness.
    • Hemiparesis: Weakness on one side of the body.
    • Paraparesis: Bilateral lower limb weakness.
    • Quadriparesis: Weakness in all four limbs.
Associated Conditions:
  • Upper motor neuron lesions:
    • Cerebral palsy
    • Stroke (pediatric)
    • Spinal cord injury
  • Lower motor neuron disorders:
    • Spinal muscular atrophy
    • Guillain-Barré syndrome
    • Charcot-Marie-Tooth disease
  • Muscular disorders:
    • Duchenne muscular dystrophy
    • Becker muscular dystrophy
    • Congenital myopathies
  • Neuromuscular junction disorders:
    • Myasthenia gravis
    • Congenital myasthenic syndromes
4. Deep Tendon Reflexes
Technique:
  • Use a reflex hammer to test:
    • Biceps reflex (C5-C6)
    • Triceps reflex (C7-C8)
    • Knee reflex (L3-L4)
    • Ankle reflex (S1-S2)
  • Ensure the patient is relaxed and the limb is in the correct position.
  • Use reinforcement techniques if needed (e.g., Jendrassik maneuver).
Normal Findings:
  • Reflexes present and symmetric bilaterally.
  • Typical grading is 2+ on a scale of 0 to 4+.
  • No clonus (unless a beat or two in young children or anxious patients).
Abnormal Findings:
  • Hyperreflexia:
    • Exaggerated reflexes (3+ or 4+).
    • May be associated with clonus.
  • Hyporeflexia:
    • Diminished (1+) or absent (0) reflexes.
  • Asymmetry:
    • Difference in reflex intensity between sides.
Associated Conditions:
  • Hyperreflexia:
    • Upper motor neuron lesions (e.g., cerebral palsy, spinal cord injury)
    • Hyperthyroidism
    • Anxiety or physiological in some children
  • Hyporeflexia:
    • Lower motor neuron disorders
    • Peripheral neuropathies
    • Spinal muscular atrophy
    • Guillain-Barré syndrome
5. Plantar Reflex
Technique:
  • Stroke the lateral aspect of the sole from heel to toe.
  • Observe the response of the great toe and other toes.
  • Test bilaterally.
Normal Findings:
  • In children over 2 years: Plantar flexion of the great toe (negative Babinski sign).
  • In infants and young children: Dorsiflexion of the great toe may be normal.
Abnormal Findings:
  • Babinski sign:
    • Dorsiflexion of the great toe with fanning of other toes.
    • Abnormal in children over 2 years.
Associated Conditions:
  • Upper motor neuron lesions:
    • Cerebral palsy
    • Spinal cord injury above lumbar level
    • Brain tumors
  • Metabolic disorders:
    • Kernicterus
  • Neurodegenerative disorders:
    • Adrenoleukodystrophy
    • Metachromatic leukodystrophy
Sensory System Examination
1. Light Touch
Technique:
  • Use a cotton wisp, soft brush, or finger.
  • Lightly touch various dermatomes.
  • Ask the child to indicate when they feel the touch.
  • Compare symmetrical areas on both sides of the body.
Normal Findings:
  • Consistent and symmetric perception of light touch in all dermatomes.
  • Ability to localize the site of touch accurately.
Abnormal Findings:
  • Hypoesthesia: Reduced sensation to light touch.
  • Anesthesia: Complete loss of sensation to light touch.
  • Hyperesthesia: Increased sensitivity to light touch.
  • Allodynia: Pain elicited by normally non-painful stimuli.
Associated Conditions:
  • Peripheral neuropathies:
    • Charcot-Marie-Tooth disease
    • Diabetic neuropathy (in type 1 diabetes)
  • Spinal cord disorders:
    • Transverse myelitis
    • Spinal cord tumors
  • Demyelinating disorders:
    • Multiple sclerosis (rare in young children)
    • Acute disseminated encephalomyelitis (ADEM)
  • Vitamin deficiencies:
    • Vitamin B12 deficiency
2. Pain Sensation
Technique:
  • Use a sterile pin or neurotip.
  • Apply brief, light pricks to various dermatomes.
  • Ask the child to distinguish between sharp and dull.
  • Compare symmetrical areas on both sides of the body.
Normal Findings:
  • Consistent and symmetric perception of sharp sensation.
  • Ability to distinguish between sharp and dull stimuli.
Abnormal Findings:
  • Hypoalgesia: Reduced sensation to painful stimuli.
  • Analgesia: Complete loss of pain sensation.
  • Hyperalgesia: Increased sensitivity to painful stimuli.
Associated Conditions:
  • Hereditary sensory and autonomic neuropathies (HSAN):
    • Congenital insensitivity to pain with anhidrosis (CIPA)
  • Spinal cord disorders:
    • Syringomyelia (dissociated sensory loss)
    • Spinal cord tumors
  • Peripheral neuropathies:
    • Leprosy (in endemic areas)
    • Fabry disease
  • Neurotoxic exposures:
    • Lead poisoning
    • Mercury toxicity
3. Temperature Sensation
Technique:
  • Use two test tubes, one with warm water and one with cool water.
  • Alternatively, use the flat sides of tuning forks at room temperature (cool) and after warming.
  • Touch various dermatomes and ask the child to identify "warm" or "cool".
  • Compare symmetrical areas on both sides of the body.
Normal Findings:
  • Consistent and symmetric perception of temperature differences.
  • Ability to distinguish between warm and cool stimuli accurately.
Abnormal Findings:
  • Thermohypoesthesia: Reduced sensitivity to temperature changes.
  • Thermoanesthesia: Complete loss of temperature sensation.
  • Altered perception: Inability to distinguish between warm and cool.
Associated Conditions:
  • Spinal cord disorders:
    • Syringomyelia
    • Brown-Séquard syndrome
  • Peripheral neuropathies:
    • Diabetic neuropathy
    • Familial dysautonomia
  • Congenital disorders:
    • Hereditary sensory and autonomic neuropathy (HSAN)
  • Thalamic lesions:
    • Thalamic stroke (rare in children)
    • Thalamic tumors
4. Vibration Sense
Technique:
  • Use a 128 Hz tuning fork.
  • Apply the base of the vibrating fork to bony prominences (e.g., great toe, medial malleolus, tibial tuberosity).
  • Ask the child to indicate when they feel the vibration start and stop.
  • Compare symmetrical areas on both sides of the body.
Normal Findings:
  • Perception of vibration at all tested sites.
  • Symmetric sensation between left and right sides.
  • Ability to feel vibration for at least 10-15 seconds at the great toe in older children and adolescents.
Abnormal Findings:
  • Reduced vibration sense: Shortened duration of perceived vibration.
  • Absent vibration sense: Inability to perceive vibration.
  • Asymmetry: Difference in vibration perception between sides.
Associated Conditions:
  • Peripheral neuropathies:
    • Charcot-Marie-Tooth disease
    • Guillain-Barré syndrome
  • Vitamin deficiencies:
    • Vitamin B12 deficiency
    • Vitamin E deficiency
  • Spinal cord disorders:
    • Friedreich's ataxia
    • Subacute combined degeneration
  • Neurotoxic exposures:
    • Cisplatin-induced neuropathy
    • Lead poisoning
5. Proprioception
Technique:
  • Hold the sides of the child's finger or toe.
  • Demonstrate "up" and "down" positions.
  • Make small movements of the digit and ask the child to identify the direction.
  • Start with larger movements and progress to smaller ones.
  • Test joints more proximally if distal joint proprioception is impaired.
Normal Findings:
  • Accurate identification of joint position and movement direction.
  • Ability to detect small movements (1-2 degrees) at distal joints.
Abnormal Findings:
  • Impaired proprioception: Inability to accurately identify joint position or movement.
  • Loss of proprioception: Complete inability to perceive joint position or movement.
Associated Conditions:
  • Sensory ataxias:
    • Friedreich's ataxia
    • Vitamin B12 deficiency
  • Peripheral neuropathies:
    • Chronic inflammatory demyelinating polyneuropathy (CIDP)
    • Diabetic neuropathy
  • Spinal cord disorders:
    • Posterior column lesions
    • Tabes dorsalis (rare in children)
  • Parietal lobe lesions:
    • Stroke (rare in children)
    • Brain tumors affecting the parietal lobe
Cerebellar Examination
1. Gait and Stance
Technique:
  • Observe normal walking:
    • Ask the child to walk across the room normally.
    • Observe for balance, symmetry, and arm swing.
  • Tandem gait:
    • Ask the child to walk heel-to-toe in a straight line.
  • Romberg test:
    • Ask the child to stand with feet together and arms by their sides.
    • Ask them to close their eyes for up to 30 seconds.
Normal Findings:
  • Normal walking: Smooth, coordinated gait with normal arm swing.
  • Tandem gait: Able to walk heel-to-toe without losing balance.
  • Romberg test: Maintains balance with eyes closed.
Abnormal Findings:
  • Ataxic gait: Wide-based, unsteady, or staggering gait.
  • Inability to perform tandem gait.
  • Positive Romberg sign: Significant swaying or falling when eyes are closed.
Associated Conditions:
  • Cerebellar disorders:
    • Cerebellar ataxia (various etiologies)
    • Cerebellar tumors
  • Sensory ataxias:
    • Friedreich's ataxia
    • Vitamin B12 deficiency
  • Vestibular disorders:
    • Vestibular neuritis
    • Labyrinthitis
  • Neurometabolic disorders:
    • Ataxia-telangiectasia
    • Wilson's disease
2. Coordination Tests
Technique:
  • Finger-to-nose test:
    • Ask the child to touch their nose with their index finger, then touch the examiner's finger.
    • Move your finger to different positions.
  • Heel-to-shin test:
    • Ask the child to place one heel on the opposite knee and slide it down the shin to the ankle.
  • Rapid alternating movements:
    • Ask the child to pat their thighs with both hands, alternating between palm up and palm down, as quickly as possible.
Normal Findings:
  • Finger-to-nose: Smooth, accurate movements without tremor or past-pointing.
  • Heel-to-shin: Smooth, straight line movement down the shin.
  • Rapid alternating movements: Quick, rhythmic, and accurate alternating movements.
Abnormal Findings:
  • Dysmetria: Inaccurate targeting, overshooting, or undershooting.
  • Intention tremor: Tremor that worsens as the target is approached.
  • Dysdiadochokinesia: Irregular, slow, or uncoordinated rapid alternating movements.
Associated Conditions:
  • Cerebellar disorders:
    • Cerebellar ataxia
    • Cerebellar tumors or hemorrhage
  • Genetic ataxias:
    • Spinocerebellar ataxias
    • Ataxia-telangiectasia
  • Metabolic disorders:
    • Wilson's disease
    • Maple syrup urine disease
  • Multiple sclerosis (rare in young children)
3. Nystagmus Assessment
Technique:
  • Observe eyes in primary position.
  • Ask the child to follow your finger to the extreme lateral gaze positions.
  • Hold lateral gaze for a few seconds.
  • Check for vertical nystagmus by having the child look up and down.
Normal Findings:
  • No nystagmus in primary position.
  • Smooth pursuit movements.
  • No or minimal end-point nystagmus on extreme lateral gaze.
Abnormal Findings:
  • Cerebellar nystagmus: Bilateral, multidirectional, worse on lateral gaze.
  • Vertical nystagmus: Suggests brainstem or cerebellar vermis involvement.
  • Pendular nystagmus: Equal velocity in both directions.
Associated Conditions:
  • Cerebellar disorders:
    • Cerebellar ataxia
    • Chiari malformation
  • Brainstem disorders:
    • Brainstem glioma
    • Multiple sclerosis (rare in young children)
  • Vestibular disorders:
    • Benign paroxysmal vertigo of childhood
    • Vestibular neuritis
  • Metabolic disorders:
    • Gaucher disease
    • Niemann-Pick disease type C
4. Speech Assessment
Technique:
  • Ask the child to repeat words or phrases, focusing on:
    • Multi-syllabic words (e.g., "Mississippi", "hippopotamus")
    • Tongue twisters (e.g., "She sells seashells by the seashore")
  • Observe for fluency, rhythm, and articulation.
Normal Findings:
  • Clear, fluent speech
  • Proper rhythm and intonation
  • Accurate articulation of complex words
Abnormal Findings:
  • Scanning speech: Slow, measured speech with separation between syllables
  • Dysarthria: Slurred or unclear speech
  • Intention tremor affecting speech
Associated Conditions:
  • Cerebellar disorders:
    • Cerebellar ataxia
    • Cerebellar tumors or hemorrhage
  • Genetic disorders:
    • Friedreich's ataxia
    • Spinocerebellar ataxias
  • Neurodegenerative disorders:
    • Multiple system atrophy (rare in children)
  • Metabolic disorders:
    • Wilson's disease
    • Mitochondrial disorders
5. Tremor Assessment
Technique:
  • Observe for resting tremor.
  • Check for postural tremor:
    • Ask the child to hold arms outstretched.
  • Assess for intention tremor:
    • During finger-to-nose test.
    • While reaching for objects.
Normal Findings:
  • No visible tremor at rest.
  • Minimal or no tremor with arms outstretched.
  • Smooth movements without tremor during targeted tasks.
Abnormal Findings:
  • Intention tremor: Tremor that worsens as the target is approached.
  • Postural tremor: Visible shaking when maintaining a posture.
  • Resting tremor: Tremor present when the body part is relaxed (uncommon in cerebellar disorders).
Associated Conditions:
  • Cerebellar disorders:
    • Cerebellar ataxia
    • Cerebellar tumors
  • Essential tremor (can occur in children)
  • Metabolic disorders:
    • Hyperthyroidism
    • Wilson's disease
  • Toxin exposure:
    • Mercury poisoning
    • Lead toxicity
  • Medication side effects:
    • Valproic acid
    • Stimulants
Remember

Cerebellar signs in children can be subtle and may require careful observation and comparison with age-appropriate norms. Any persistent or progressive cerebellar signs warrant further investigation and possibly neuroimaging studies.

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