Presenting History

When evaluating a pediatric patient with Acute Infantile Hemiplegia or Childhood Stroke (arterial ischemic stroke vs cerebral venous sinus thrombosis), obtain an exhaustive chronological account of the onset, mode of progression, vascular territory clues, and underlying prothrombotic or cardioembolic predisposing factors.

  • Onset & Evolution: Inquire if the weakness was catastrophic/hyperacute (seconds to minutes, suggesting embolic occlusion), acute (hours, suggesting thrombosis), subacute (days to weeks, suggesting vasculitis/TBM), or fluctuating/stuttering (suggesting Moyamoya arteriopathy or transient ischemic attacks).
  • Paucity of Movements & Hemiplegia: Inquire about sudden weakness or loss of movement of one half of the body (face, arm, leg). In infants, ask if the mother noticed early hand preference (<1 year of age), fisting of one hand, or dragging of one leg.
  • Seizures at Onset (HHE Syndrome): Inquire about focal convulsions preceding or accompanying the hemiplegia. Note that Hemiconvulsion-Hemiplegia-Epilepsy (HHE) Syndrome classically presents with prolonged unilateral febrile or non-febrile status epilepticus followed by immediate dense flaccid hemiplegia.
  • Cranial Nerve & Speech Symptoms: Inquire about facial asymmetry during crying/smiling (UMN facial palsy), deviation of the mouth, slurred speech/aphasia (dominant MCA territory stroke), or swallowing difficulty.
  • Headache & Altered Consciousness: Ask about sudden severe "thunderclap" headache, vomiting, lethargy, or coma (suggesting cortical vein thrombosis, subarachnoid hemorrhage, or large MCA territory infarction with malignant edema).

Negative History (3C 1D Framework)

CategoryPertinent Negative QuestionRationale / Significance
Causes (Cardiac / Embolic)History of congenital cyanotic heart disease, squatting episodes, rheumatic fever, or prosthetic valve?Paradoxical embolism (via right-to-left shunt in Cyanotic CHD) or left atrial thrombus/vegetation (in IE/RHD) is the leading cause of cardioembolic stroke.
Causes (Infection / Vasculitis)History of recent varicella (chickenpox) in the past 6 months, chronic fever (TBM), or pharyngeal trauma?Post-Varicella Arteriopathy (Focal Cerebral Arteriopathy / FCA) is a classic cause of childhood stroke 2–6 months post-chickenpox. Retropharyngeal trauma (pencil/stick injury) causes internal carotid artery dissection.
Causes (Prothrombotic)History of acute dehydrating gastroenteritis, nephrotic syndrome, sickle cell crises, or family history of DVT?Severe dehydration and nephrotic syndrome predispose to Cerebral Venous Sinus Thrombosis (CVST); Sickle Cell Anemia causes large vessel vasculopathy.
Complaints (Progression)History of progressive bilateral weakness, cranial neuropathies, or fluctuating sensorium?Differentiates focal unilateral arterial ischemic stroke from acute disseminated encephalomyelitis (ADEM), viral encephalitis, or brainstem glioma.
ComplicationsHistory of recurrent seizures, limb length discrepancy, or speech arrest following the acute stroke?Evaluates post-stroke epilepsy, growth arrest of hemiplegic limbs, and acquired receptive/expressive aphasia.
Differentials (Mimics)History of rapid complete resolution of weakness within 24 hours following a single febrile seizure?Suggests Todd's Post-Ictal Paresis (benign transient hemiparesis resolving in <24 hours without structural infarction).
DifferentialsHistory of episodic alternating hemiplegia, paroxysmal ocular movements, or migraine headaches?Points to Alternating Hemiplegia of Childhood (AHC - ATP1A3 mutation) or Hemiplegic Migraine.
DifferentialsHistory of recurrent stroke-like episodes with lactic acidosis, short stature, or maternal family history?Suggests MELAS (Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-like episodes).

Other Relevant History

  • Antenatal & Birth History: Perinatal arterial ischemic stroke (PAIS) typically presents at birth or in early infancy with focal seizures or subtle asymmetry; maternal thrombophilia, preeclampsia, or birth asphyxia should be documented.
  • Developmental History: Assess milestones attained prior to the stroke event and document any acquired motor or language arrest.
  • Immunization & Contact History: Document chickenpox (varicella) and contact with open pulmonary tuberculosis.
  • Family History: Inquire about recurrent early strokes (<40 years), recurrent miscarriages, deep vein thrombosis, or consanguinity (homocystinuria, protein C/S deficiency, factor V Leiden, Moyamoya disease).

History Summary

Spoken Formulation: History Summary

"I would like to summarize the history of this Age in months / years old Male / Female child, Birth order order child born of Consanguineous / Non-consanguineous parentage from Geographic Location, who presented with sudden-onset acute right/left-sided hemiplegia following Focal convulsion / Fever / Head trauma of Duration in days / weeks, characterized by paucity of limb movements, facial asymmetry, and slurred speech, with No history of preceding developmental delay or neuroregression.

In view of the hyperacute onset, focal neurological deficit confined to the Middle / Anterior / Posterior Cerebral Artery territory, and presence of Cyanotic CHD / Post-varicella state / Prothrombotic risk factor, I would like to consider a clinical diagnosis of Acute Stroke in Childhood, most probably Acute Arterial Ischemic Stroke involving the Left/Right MCA territory (or Acute Infantile Hemiplegia), in the Acute Flaccid / Spastic Recovery stage, with No signs of raised intracranial pressure."

General & Head-to-Toe Examination

Pre-Examination Child Behavioral State

  • Child State: Document mental status and state (Prechtl State 3: quiet wakefulness / alert and interactive).

Vitals & Anthropometry

  • Pulse & Blood Pressure: Check all four limb pulses for symmetry, radio-femoral delay (Coarctation of Aorta), or pulse asymmetry (Takayasu arteritis). Measure blood pressure in both upper and lower limbs.
  • Anthropometry: Measure limb lengths bilaterally:
    • Limb Length Discrepancy: A shortening $>1\text{ cm}$ in the hemiplegic arm/leg indicates a congenital or long-standing perinatal insult rather than a recent acquired stroke.
    • Measure thumb and fingernail size (contralateral parietal lobe lesion produces hypoplastic nails).

Head-to-Toe Markers

  • Neurocutaneous Markers: Port-wine stain (Sturge-Weber syndrome causing leptomeningeal angiomatosis and contralateral hemiparesis), café-au-lait macules (Neurofibromatosis type 1 with vascular stenosis), or ash-leaf spots (Tuberous sclerosis).
  • Head & Cranium: Auscultate skull and globes with stethoscope bell for cranial bruits (Arteriovenous Malformation / Vein of Galen malformation).
  • Oral Cavity: Inspect posterior pharynx for tonsillar injury, pencil/foreign body trauma, or peritonsillar abscess (causes internal carotid artery thrombosis).
  • Cardiovascular Markers: Look for clubbing and central cyanosis (Cyanotic CHD), splinter hemorrhages/Janeway lesions/Osler nodes (Infective Endocarditis), or murmurs.

Systemic Examination: Central Nervous System

1. Higher Mental Functions & Speech

  • Consciousness: Alert (GCS 15/15) vs drowsy/stuporous.
  • Speech: Evaluate expressive dysphasia (Broca's area / left frontal operculum) vs receptive dysphasia (Wernicke's area / left superior temporal gyrus) in right-handed children.

2. Cranial Nerves

  • CN II (Optic): Confrontation visual field testing for Homonymous Hemianopia (contralateral visual field loss in MCA/PCA stroke). Fundoscopy for papilledema or emboli.
  • CN III, IV, VI: Conjugate eye deviation:
    • Cortical (Hemispheric) Stroke: Eyes look towards the lesion (away from the hemiplegic side) due to unopposed frontal eye field action.
    • Brainstem (Pontine) Stroke: Eyes look away from the lesion (towards the hemiplegic side).
  • CN VII (Facial): Contralateral Upper Motor Neuron (UMN) Facial Palsy:
    • Flattening of nasolabial fold and drooping of mouth angle on the hemiplegic side, with normal forehead wrinkling and eye closure (preserved bilateral cortical supply).
  • CN IX, X, XII: Swallowing competence; tongue deviation towards the hemiplegic side on protrusion (due to weakness of contralateral genioglossus).

3. Motor System Examination

  • Stage of Weakness:
    • Acute Stage (Days 1–7): Flaccid Stage (hypotonia and hyporeflexia due to cerebral shock).
    • Chronic Stage (>1–2 Weeks): Spastic Stage (hypertonia, hyperreflexia, clonus, extensor plantar response).
  • Distribution of Weakness:
    • MCA Territory: Predominantly affects Face and Upper Limb > Lower Limb; distal hand muscles more severely affected than proximal girdle.
    • ACA Territory: Predominantly affects Lower Limb > Upper Limb.
    • Internal Capsule (Dense Capsular Hemiplegia): Face, Arm, and Leg equally and densely paralyzed.
  • Reflexes:
    • DTRs: Brisk/exaggerated (3+ to 4+) on the hemiplegic side with patellar/ankle clonus in the chronic phase.
    • Plantar: Extensor (Babinski positive) on the hemiplegic side; normal flexor on the healthy side.
  • Gait (if ambulant): Hemiplegic / Circumduction Gait (pelvis tilted upward, leg swung outward in a semi-circle with arm held flexed, adducted, and pronated).

4. Sensory & Cortical Sensations

  • Cortical Sensory Loss: Astereognosis (inability to recognize objects placed in the hand), two-point discrimination deficit, and sensory extinction/neglect (contralateral parietal lobe lesion).

Final Summary & Diagnosis

Spoken Formulation: Final Presentation

"On examination of this Age in years old Male / Female child who is conscious and alert, with normal vitals and no cranial bruits, neurological examination reveals:

  • Normal higher mental functions / Expressive / Receptive Aphasia,
  • Left / Right Homonymous Hemianopia on confrontation testing,
  • Left / Right Upper Motor Neuron Facial Palsy,
  • Left / Right Spastic Hemiparesis (Upper limb > Lower limb / Dense capsular pattern) with hypertonia, hyperreflexia (3+), sustained ankle clonus, and positive Babinski extensor response,
  • Normal sensory modalities and absence of cerebellar signs.

Final Diagnosis: I formulate my diagnosis as a case of Acute Arterial Ischemic Stroke in Childhood (Acute Infantile Hemiplegia), localizing to the Left / Right Middle Cerebral Artery territory (Internal Capsule / Cortical MCA branch), currently in the Spastic Recovery Phase, likely secondary to Cardioembolism (Cyanotic CHD/RHD) / Focal Cerebral Arteriopathy (Post-Varicella) / Prothrombotic state, with No evidence of active raised intracranial pressure."