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Pediatric CNS History Taking and Examination - Detailed Proforma

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SECTION A: PATIENT IDENTIFICATION

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SECTION B: HISTORY TAKING

1. Chief Complaints

1. ______________ - since ______
2. ______________ - since ______
3. ______________ - since ______

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2. History of Presenting Illness (HPI)

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A. Symptom-Specific CNS Questions (Aruchamy's approach)

• Age at onset of first seizure
• Type: Focal / Generalized / Absence / Myoclonic / Infantile spasms
• Frequency, duration, and character of each episode
• Pre-ictal: Aura (visual / olfactory / gustatory / experiential), cry
• Ictal phase: eye deviation, head turning, limb posturing, automatisms, tongue bite, urinary/fecal incontinence, cyanosis, frothing
• Post-ictal: Todd's paralysis, drowsiness, confusion, headache, duration
• Precipitating factors: fever, sleep deprivation, stress, flickering lights, missed medication
• Treatment history and drug compliance

• Site (frontal, occipital, temporal, vertex, global), radiation
• Character (throbbing, pressure, burning)
• Severity (mild/moderate/severe, effect on activity)
• Timing (morning, nocturnal, awakening from sleep - suggests raised ICP)
• Associated: vomiting (projectile?), visual disturbances, photophobia, phonophobia
• Aggravating factors: bending forward, Valsalva, straining
• Relieving factors: rest, analgesics, lying down
• Frequency and duration

• Onset: sudden / gradual
• Level: drowsiness, stupor, coma
• Preceding fever, headache, seizures, trauma
• Associated: vomiting, rash, neck stiffness

• Onset (sudden = vascular/trauma; gradual = tumor/degenerative; subacute = inflammatory)
• Distribution: monoplegia, hemiplegia, paraplegia, quadriplegia, proximal, distal
• Progression: ascending (GBS), descending (botulism)
• Associated sensory complaints, sphincter involvement
• Preceding viral illness (ADEM, transverse myelitis)

• Type: tremor (resting/action), chorea, athetosis, dystonia, myoclonus, tics
• Distribution, onset, course
• Effect on daily activities, speech, swallowing
• Associated behavioral changes

• Milestones previously achieved
• Age at regression
• Rate: acute / gradual / stepwise
• Associated vision/hearing changes, seizures, behavioral disturbance

• Type: spastic, waddling, steppage, ataxic, antalgic
• Falls, balance difficulty
• Whether unilateral or bilateral

• Expressive vs. receptive
• Dysarthria, aphasia, dysphonia
• Onset and progression

• Onset, monocular or binocular diplopia
• Associated ptosis, ophthalmoplegia
• Visual field defects

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3. Antenatal History (VERY important in pediatric CNS)

• Mother's health during pregnancy (infections: TORCH, rubella, CMV, toxoplasma)
• Maternal fever, rash, drug intake (antiepileptics, thalidomide)
• Maternal hypertension, diabetes, thyroid disorder
• Exposure to radiation / toxins
• Antenatal USG abnormalities (hydrocephalus, neural tube defects)
• Polyhydramnios / oligohydramnios
• Fetal movements: when started, quality

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4. Natal (Birth) History

• Place of delivery (hospital/home), type of delivery (NVD/LSCS/forceps/vacuum)
• Gestational age at delivery (term/preterm/post-term)
• Birth weight
• APGAR score (if known)
• Complications: birth asphyxia, prolonged labor, meconium-stained liquor, cord around neck, shoulder dystocia
• Cry at birth: immediate / delayed / absent
• Resuscitation required? (mask O2, CPAP, ventilation)
• Admission to NICU - duration, reason
• Neonatal jaundice: age of onset, duration, severity, phototherapy/exchange transfusion
• Neonatal seizures, hypoglycemia, sepsis

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5. Postnatal History

• Feeding: breast / bottle, any feeding difficulties (sucking, swallowing)
• Weight gain pattern
• Hospitalizations, surgeries
• Serious illnesses: meningitis, encephalitis, head injury, near-drowning

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6. Developmental History (Core for Pediatric CNS - Aruchamy emphasis)

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7. Immunization History

• Vaccination status: up-to-date / incomplete / not vaccinated
• BCG, OPV/IPV (especially relevant for acute flaccid paralysis)
• MMR, DPT
• Any adverse events following immunization (AEFI)
• JE / Meningococcal / Varicella (if given)

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8. Nutritional History

• Exclusive breastfeeding (up to 6 months?)
• Weaning age and diet
• Current diet - type, frequency, variety
• Suspected nutritional deficiencies: Vitamin B12 (subacute combined degeneration), Vitamin D (rickets + hypocalcemic seizures), Thiamine (Wernicke's), iron (breath-holding spells)

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9. Past History

• Similar episodes in the past
• Meningitis, encephalitis, febrile seizures, head injury
• Metabolic disorders, neurocutaneous syndromes
• Surgeries (VP shunt, brain tumor, craniofacial)
• Chronic medications (antiepileptics - note dose, duration, compliance)

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10. Family History

• Seizure disorder in parents or siblings
• Neurodegenerative diseases, metabolic storage disorders
• Consanguinity (relevant for autosomal recessive metabolic/genetic CNS conditions)
• Neurocutaneous syndromes: NF1, TSC, Sturge-Weber (family members with café-au-lait spots, seizures, skin lesions)
• Hearing or vision loss in family members

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11. Socioeconomic and Environmental History

• Socioeconomic class
• Lead exposure (paint chips, dust) - relevant for lead encephalopathy
• Infection exposures: TB contacts, meningococcal clusters
• School performance and behavioral changes

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SECTION C: GENERAL EXAMINATION

• General appearance: well/ill-looking, consciousness, posture, spontaneous movements
• Nutrition and hydration status
• Dysmorphic features (suggestive of chromosomal/genetic syndromes)
• Anthropometry:
  • Weight: ___ kg (centile: ___)
  • Height/Length: ___ cm (centile: ___)
  • Head circumference: ___ cm (centile: ___) - microcephaly / normocephaly / macrocephaly
• Vital signs: Temp / Pulse / RR / BP (4-limb BP if CoA suspected)
• Skin: café-au-lait spots, ash-leaf macules, adenoma sebaceum, port-wine stain, neurofibromas, shagreen patch (neurocutaneous syndromes)
• Pallor, icterus, cyanosis, clubbing, lymphadenopathy, edema
• Gait (if ambulatory): observe before formal exam

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SECTION D: CNS EXAMINATION (Systematic)

1. Higher Mental Functions

• Time: day, date, year
• Place: where they are, which city
• Person: name, age, recognition of family

• Immediate memory: digit span, repeat 3 objects
• Recent memory: recall of 3 objects after 5 minutes
• Remote memory: past events, school name

• Serial 7 subtraction (older children)
• Reverse digit span

• Age-appropriate knowledge
• Fund of general knowledge
• Abstract reasoning (older children): similarities, differences, analogies (e.g., "a cat and a dog are both ___")

• Fluency, articulation, comprehension
• Dysarthria: cerebellar (scanning), UMN (spastic), LMN/bulbar (nasal, breathy)
• Aphasia: expressive (Broca's), receptive (Wernicke's)

• Cooperative, hyperactive, autistic features, emotional lability

• Right-left orientation
• Copy geometric figures (older children)

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2. Head Examination

• Shape: normocephalic / brachycephalic / dolichocephalic / plagiocephaly / scaphocephaly
• Circumference: measure and plot on chart
• Fontanelles (infants):
  • Anterior fontanelle: open/closed, size (___ x ___ cm), tense/bulging/sunken/pulsatile
  • Posterior fontanelle: open/closed
• Sutures: fused / open / diastasis of sutures (raised ICP)
• Transillumination (infants with suspected hydranencephaly)
• Scalp veins: dilated (raised ICP, venous occlusion)
• Skull percussion: "cracked-pot" sound (Macewen's sign) = raised ICP with suture separation
• Cranial bruits (over temporal region and eyes): AVM

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3. Spine Examination

• Inspection: midline defects, hair tuft, dimple, lipoma, sinus (spina bifida occulta, tethered cord)
• Gibbus, scoliosis, kyphosis, lordosis
• Tenderness over vertebral spines
• Range of motion of spine

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4. Cranial Nerve Examination

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5. Motor System Examination

• Muscle bulk: normal / wasting / hypertrophy (pseudohypertrophy of calves - DMD)
• Symmetry
• Fasciculations (LMN), involuntary movements
• Posture: decerebrate / decorticate
• Asymmetric limb use

• Hypotonia: floppy baby - check head lag, ventral suspension, axillary suspension, horizontal suspension
• Hypertonia: spasticity (clasp-knife) vs rigidity (lead pipe / cogwheel)
• Assess at: wrist, elbow, knee, ankle (clonus)
• Ankle clonus: >3 beats = abnormal (UMN lesion)

• Abdominal reflexes: upper (T8-T10), lower (T10-T12); absent in UMN lesion
• Cremasteric reflex (males): L1-L2
• Plantar response (Babinski): Flexor (normal in >2 years) / Extensor (UMN lesion or normal in <2 years)

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6. Involuntary Movements

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7. Cerebellar Examination

• Wide-based, unsteady, lurching to affected side (Romberg's test: positive in sensory ataxia, negative in cerebellar ataxia)

• Finger-nose test: intention tremor, past-pointing (dysmetria)
• Heel-shin test: ataxia of lower limbs
• Disdiadochokinesis: rapid alternating movements (pronation-supination) - dysdiadochokinesis = cerebellar lesion
• Rebound phenomenon

• Nystagmus (horizontal/vertical - direction, fast phase toward lesion in cerebellar)

• Scanning/staccato speech (cerebellar dysarthria)

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8. Sensory Examination (as far as possible in children)

• Light touch (cotton wool)
• Pain (pinprick)
• Temperature (hot/cold test tubes)
• Vibration sense (128 Hz tuning fork over bony prominences)
• Proprioception / joint position sense (move finger/toe up or down)

• Two-point discrimination
• Stereognosis (identify object in hand)
• Graphesthesia (number written on palm)
• Point localization

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9. Meningeal Signs (essential in febrile child)

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10. Autonomic Nervous System

• Pulse rate and rhythm (bradycardia / tachycardia)
• Blood pressure (postural hypotension)
• Sweating: anhidrosis (Horner's), hyperhidrosis
• Bladder: retention / neurogenic bladder / incontinence
• Bowel: constipation / incontinence
• Skin: flushing, livedo reticularis, trophic changes
• Horner's syndrome: ptosis, miosis, anhidrosis, enophthalmos

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SECTION E: NEUROCUTANEOUS MARKERS (Aruchamy emphasis)

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SECTION F: SYSTEMIC EXAMINATION (CNS-Relevant)

• Cardiac murmurs (cardioembolic stroke, SBE with neurological sequelae)
• Blood pressure (hypertensive encephalopathy)

• Respiratory pattern (Cheyne-Stokes, Kussmaul, ataxic - brainstem localization)

• Hepatosplenomegaly (lysosomal storage diseases: Gaucher's, Niemann-Pick, GM1 gangliosidosis)
• Signs of portal hypertension (Wilson's disease)

• Kayser-Fleischer rings (Wilson's disease - slit-lamp)
• Cherry-red spot at macula (Tay-Sachs, Niemann-Pick)
• Cataracts (metabolic disorders, rubella embryopathy)

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SECTION G: FUNCTIONAL ASSESSMENT

• Activities of daily living (ADL): dressing, feeding, toileting, mobility
• School performance: grade, repeating year, learning difficulties
• Behavioral issues: attention deficit, hyperactivity, conduct disorder
• Social interaction: eye contact, peer relationships, play
• Communication: level of language, whether verbal/non-verbal

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SECTION H: PROVISIONAL DIAGNOSIS AND DIFFERENTIAL DIAGNOSIS

1. Provisional diagnosis: __________________________
2. Differentials:
   • a. __________________________
   • b. __________________________
   • c. __________________________

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SECTION I: INVESTIGATIONS PLANNED

• CBC, ESR, CRP
• Blood glucose (BGL), serum electrolytes (Na, K, Ca, Mg, phosphorus)
• Renal function tests, liver function tests
• Urine routine and microscopy

• CSF analysis: (after ruling out raised ICP by fundoscopy/CT):
  • Appearance, opening pressure
  • Cell count and differential
  • Protein, glucose (CSF:blood glucose ratio)
  • Culture, GRAM stain, AFB, India ink
  • PCR (HSV, EV, TB as indicated)
• Neuroimaging:
  • MRI brain (with/without contrast): preferred in most CNS conditions
  • CT brain: acute trauma, hemorrhage, emergency setting
  • MRI spine: if myelopathy / cord lesion suspected
• EEG:
  • Routine / Sleep / Video-EEG / Ambulatory EEG
  • Indication: seizures, encephalopathy, suspected non-epileptic events
• Nerve conduction studies (NCS) / EMG: peripheral neuropathy, NMJ disorders, myopathy
• Metabolic workup (if regression or storage disease suspected):
  • Plasma lactate, ammonia, amino acids
  • Urine organic acids, mucopolysaccharides
  • Lysosomal enzyme assay
  • VLCFA (adrenoleukodystrophy)
• Genetic / Molecular:
  • Chromosomal karyotype, FISH, microarray
  • Specific gene panel (e.g., SCN1A for Dravet syndrome, MECP2 for Rett syndrome, DMD gene)
• Others as indicated:
  • Thyroid function tests
  • Vitamin B12, folate
  • Serology: TORCH, measles, JE, HSV
  • Autoimmune: ANA, anti-NMDAR antibodies, MOG antibodies, AQP4 (neuromyelitis optica)
  • Ophthalmologic evaluation (fundoscopy, BAER, VEP)
  • Audiometry

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SECTION J: SUMMARY

A ___-year-old _____ (M/F) presented with ______________ for _______ duration. On examination, GCS was __/15, with ________________ (key neurological findings). Relevant positive findings include _______________. Relevant negative findings include _______________.

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KEY POINTS FROM ARUCHAMY'S APPROACH

1. Developmental history is the cornerstone of pediatric CNS evaluation - always construct a developmental timeline before examining.
2. Regression of milestones is a red flag - implies acquired or progressive neurological disease.
3. Birth history must be detailed - perinatal asphyxia is the single most common cause of cerebral palsy and neonatal seizures.
4. Neurocutaneous markers should be actively sought in every child with seizures, intellectual disability, or developmental delay.
5. Fontanelle assessment in infants - bulging indicates raised ICP; sunken indicates dehydration.
6. Primitive reflexes persisting beyond expected age are indicators of upper motor neuron dysfunction.
7. CNS localization - always attempt to localize the lesion (UMN vs LMN vs cerebellar vs extrapyramidal vs neuromuscular) before ordering investigations.
8. Consanguinity and family history must be explicitly asked - autosomal recessive metabolic conditions are common in India and present as progressive neurological disease.
9. Meningeal signs may be absent in neonates - high index of suspicion required for neonatal meningitis.
10. In febrile seizures, differentiate simple vs complex: simple = age 6 months-5 years, generalized, <15 min, no recurrence within 24 hours, no post-ictal focal deficit.

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