Epidemiology


Etiology


  • Males > Females (4:1 ratio).
  • Strong genetic component (high concordance in monozygotic twins).
  • Risk Factors: Advanced paternal age, maternal use of valproate, low birth weight.
  • Associations: Fragile X syndrome, Tuberous Sclerosis, Rett syndrome (though Rett is a distinct entity).

Pathophysiology


Clinical features


  • Social communication deficits: Poor eye contact, absent joint attention (no pointing by 18 mo), lack of social reciprocity. c
  • Restricted/repetitive behaviors: Hand-flapping, lining up toys, echolalia, rigid adherence to routines, sensory hypo-/hyperreactivity.
  • Exam: Early childhood macrocephaly (~20%), intellectual disability (~30–50%).

Diagnostics


  • Initial / Screening:
    • General developmental surveillance at all well-child checks (9, 18, and 30 months).
    • ASD-specific screening tool: M-CHAT-R/F (Modified Checklist for Autism in Toddlers) at 18 and 24 months.
  • Confirmatory / Gold Standard:
    • Comprehensive multidisciplinary clinical evaluation using DSM-5 criteria (supported by standardized assessment tools: ADOS-2, ADI-R).
  • Mandatory First Step in Workup:
    • Audiology / Formal Hearing Evaluation: Must be performed in all children with delayed speech or failure to respond to name to rule out sensorineural/conductive hearing loss before diagnosing ASD.
  • Adjunctive Testing:
    • Genetic testing: Chromosomal Microarray (CMA) and/or Fragile X (FMR1) DNA analysis for all unexplained ASD cases.
    • EEG: Indicated only if clinical seizures are suspected or if there is developmental regression (to rule out Landau-Kleffner syndrome).
    • Lead screening: If pica or environmental risk is present.

Treatment


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