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Sandifer Syndrome

Sandifer Syndrome

Sandifer syndrome is a rare paroxysmal movement disorder characterized by spasmodic torsional dystonia with posturing of the head and neck, primarily associated with gastroesophageal reflux disease (GERD) and feeding disorders in infants and young children.

Key Points

  • Peak age: 18-24 months
  • Strong association with GERD (>90%)
  • Often misdiagnosed as seizures
  • More common in neurologically impaired children
  • Episodes typically occur during or after feeding

Epidemiology

  • Prevalence: Unknown (likely underreported)
  • Male:Female ratio - approximately equal
  • Higher incidence in infants with:
    • Hiatal hernia
    • Developmental delays
    • Feeding difficulties

Clinical Features

Characteristic Movements

  • Sudden onset dystonic posturing
    • Head and neck extension or rotation
    • Back arching (opisthotonus)
    • Torticollis-like movements
    • Upper body spasms
  • Episode Characteristics
    • Duration: seconds to minutes
    • Frequency: multiple times per day
    • Temporal relation to feeds
    • Resolution during sleep

Associated Symptoms

  • Feeding-related
    • Regurgitation
    • Feeding difficulties
    • Irritability during feeds
    • Poor weight gain
  • GERD symptoms
    • Heartburn
    • Vomiting
    • Esophagitis
    • Food refusal

Distinguishing Features

  • Preserved consciousness
  • No post-ictal phase
  • Normal EEG during episodes
  • Improvement with anti-reflux measures

Diagnostic Approach

Initial Evaluation

  • Detailed history
    • Feeding patterns
    • Episode characteristics
    • Developmental history
    • Family history
  • Physical examination
    • Growth parameters
    • Neurological examination
    • Observation during feeds
    • Signs of complications

Diagnostic Studies

  • GERD evaluation
    • 24-hour pH monitoring
    • Impedance testing
    • Upper GI series
    • Endoscopy (if indicated)
  • Neurological evaluation
    • EEG (to rule out seizures)
    • Brain imaging (if indicated)
    • Video recording of episodes

Differential Diagnosis

  • Neurological conditions
    • Infantile spasms
    • Dystonia
    • Seizure disorders
  • Gastrointestinal disorders
    • Isolated GERD
    • Cow's milk protein allergy
    • Eosinophilic esophagitis

Management Approaches

GERD Management

  • Lifestyle modifications
    • Feeding position
    • Thickened feeds
    • Small, frequent feeds
    • Post-feed positioning
  • Medications
    • Proton pump inhibitors
    • H2 receptor antagonists
    • Prokinetics (if indicated)

Supportive Care

  • Feeding therapy
    • Positioning techniques
    • Feed modification
    • Parent education
  • Physical therapy
    • Positioning support
    • Muscle strengthening
    • Movement therapy

Surgical Intervention

  • Indications
    • Refractory symptoms
    • Severe GERD
    • Failure to thrive
  • Procedures
    • Fundoplication
    • Gastrostomy tube placement

Complications & Prognosis

Potential Complications

  • Growth-related
    • Failure to thrive
    • Malnutrition
    • Feeding aversion
  • GERD-related
    • Esophagitis
    • Barrett's esophagus
    • Aspiration
  • Psychosocial impact
    • Parent anxiety
    • Feeding stress
    • Social isolation

Prognosis

  • Generally good with appropriate treatment
  • Resolution factors
    • Age of onset
    • Severity of GERD
    • Underlying conditions
    • Treatment compliance
  • Long-term outcomes
    • Most resolve with GERD treatment
    • May persist in neurologically impaired children
    • Regular monitoring needed
Further Reading


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