A 11 month old neurologically impaired baby is admitted to the pediatric ward. The child is under evaluation for cerebral palsy. The mother repos episodes of regurgitation, aspiration and frequent respiratory infection in child. A provisional diagnosis of GERD was made. After few days of being in hospital, mother complained that she noticed child’s back arched suddenly, with splaying of head and legs outwards which became stiff. The intern posted there rushed to the bedside where he noticed spasmodic torsional dystonia with opisthotonic posture. This episode lasted 2 minutes. Thereafter multiple episodes where noted. Electrolyte and biochemistry panel revealed normal findings. Which of the following is the most probable diagnosis?
Correct Answer: Sandifer syndrome
Description: Neurologically impaired children like those with cerebral palsy have increased risk of severe GERD due to multiple factors like low pressure of the lower esophageal sphincter Predominant supine position. Sandifer syndrome is a paroxysmal dystonic movement disorder occurring in association with gastro-oesophageal reflux, and, in some cases, hiatal hernia. Clinical findings: Onset : infancy or early childhood. The dystonic movements are characterised by abnormal posturing of the head and neck (toicollis) Severe arching of the spine. Episodes usually last for between 1-3 minutes can occur up to 10 times a day, although they are usually associated with the ingestion of food. Etiology Associated with gastro-oesophageal reflux but the pathophysiological mechanism is not clearly understood. Several studies have indicated that the dystonic posturing is a pathological reflex triggered in response to abdominal pain caused by gastroesophageal reflux and oesophagitis. Diagnostic methods Sandifer syndrome is diagnosed on the basis of the association of gastro-oesophageal reflux with the characteristic movement disorder. Neurological examination is usually normal. Management and treatment Early diagnosis of the syndrome is essential Effective treatment of the gastro-oesophageal reflux (by pharmacological therapy or surgical intervention) leads to resolution of the movement disorder. Prognosis The prognosis for patients is good
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