Sandifer Syndrome Clinical Presentation

Updated: Mar 15, 2019
  • Author: Pegeen Eslami, MD; Chief Editor: Carmen Cuffari, MD  more...
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Presentation

History

Sandifer syndrome is most commonly mistaken for seizures. [3, 5, 6]   [8] The infant or child typically appears to have an alteration in mental status associated with the tonic posturing.

  • A relationship with feeding suggests the diagnosis of Sandifer syndrome, which commonly occurs after feeding.

  • Posturing is typically brief, temporally associated with feeding, paroxysmal, absent during sleep

  • The child may have a sudden deviation of the head and neck to one side and the legs to the opposite side with a stretched out appearance. Typically, the back is arched posteriorly with hyperextension of the spine and elbows may be flexed and held posteriorly with hyperextended hips. Torticollis may be present. [9, 5, 6, 8]  Although the intermittent stiff tonic posture and periods of crying and apparent discomfort may suggest seizures, in many cases the rhythmic clonic component, which may be present in seizures, is not described.

  • Various stiff, bizarre postures can be observed.

    • Typically, the duration of the posture is 1-3 minutes.

    • This brief, paroxysmal pattern of posturing accounts for the fact that the movement observed in Sandifer syndrome may be mistaken for seizures.

    • During the posture, the infant may become very quiet or, less commonly, become very fussy. Fussiness and evident discomfort is most commonly observed as the posture abates

    • If a significant volume of gastroesophageal reflux is observed, even without actual vomitus, some infants and children may manifest evidence of respiratory tract irritation as well, including cough, wheezing, and stridor, depending on the degree and volume of reflux. [10]

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Physical

In children with Sandifer syndrome without mental impairment, the examination findings are normal. Children with Sandifer syndrome with mental impairment may have evidence of spasticity and may be diagnosed with cerebral palsy. [11]

  • Sandifer syndrome in infants is most commonly associated with normal examination findings.

  • Sandifer syndrome in older children may be associated with mental impairment and/or anatomic abnormalities of the GI tract ( eg hiatal hernia)

  • In infants with significant GERD and/or unsuspected milk protein allergy , physical exam may reveal evidence of poor nutrition, poor weight gain, occult blood on fecal guaiac testing 

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Causes

Dysfunction of the lower esophagus with resultant acidic reflux is thought to be the most common precipitating factor, with the episodes of posturing coinciding temporally with recent feedings.  Although gastro-esophageal reflux is common  and indeed physiologic in infants, gastro-esophageal reflux disease eg concerning symptoms or complications associated with the reflux is much less common. [12]  Concerning symptoms may include evidence of pain (presumed to be the cause of this posturing), irritatability, poor weight gain, sleep disturbance, respiratory symptoms Typically empiric treatment for GERD alleviates symptoms of Sandifer Syndrome.  

  • Gastroesophageal reflux disease (GERD) with varying degrees of esophageal inflammation

  • Hiatal hernia may be associated particularly ouside of infancy, and presumably exacerbates the GER

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Physical Examination

see above in Physical section

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Complications

Sandifer syndrome per se is not associated with specific complications.  However misdiagnosis eg as a neurologic problem may result in a significant delay in the diagnosis of associated GERD and timely intervention.  

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