Sandifer Syndrome

Updated: Nov 10, 2016
  • Author: Pegeen Eslami, MD; Chief Editor: Carmen Cuffari, MD  more...
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Overview

Background

Sandifer syndrome involves spasmodic torsional dystonia with arching of the back and rigid opisthotonic posturing, mainly involving the neck, back, and upper extremities, associated with symptomatic gastroesophageal reflux, esophagitis, or the presence of hiatal hernia. [1, 2]

Pediatric neurologists may be the first to see patients with Sandifer syndrome because the primary care provider and the parents may believe that the spasms represent seizures. Case reports of this syndrome are available; however, pediatric emergency department physicians, pediatric neurologists, and gastroenterologists see patients with this complex with some frequency. The syndrome is most certainly underrecognized, and delays in diagnosis are due to atypical presentations or cases in which the diagnosis is not part of the differential. [3, 4, 5, 6]

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Pathophysiology

The true pathophysiologic mechanisms of this condition remain unclear.

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Epidemiology

Frequency

International

The incidence is unknown, although some suggestion indicates that in clinical practice it occurs in less than 1% of children with gastroesophageal reflux.

Mortality/Morbidity

Mortality is not typically associated with Sandifer syndrome.

Morbidity consists of the discomfort associated with this syndrome. [7] Infants may lose weight if persistent or severe gastroesophageal reflux disease (GERD) is present. Associated morbidities may also include the presence of a hiatal hernia and esophagitis.

Race

Race does not seem to influence incidence.

Sex

No sex predilection is recognized.

Age

Typically, Sandifer syndrome is observed from infancy to early childhood. Peak prevalence is in individuals younger than 24 months. Children with mental impairment or spasticity may experience Sandifer syndrome into adolescence.

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