Case Report: Paroxysmal Neck Dystonia HADI FAKIH MD NICU Director- SRHH hadifakih75@gmail.com https://www.researchgate.net/profile/Hadi_Fakih3 Case Report: Paroxysmal Neck Dystonia

PRESENTATION AN 8 mo girl, reported during a vaccination visit: Recurrent episodic Neck Deviation & abnormal head posturing Lasting hours No cyanosis No LOC No fever

Video

History: No post ictal phase No family hx of epilepsy or such a problem No current medication Multiple admissions Variety of consultants Lab investigations: chemistries + NST  nl

Progression Very Concerned Mom C spine X-Ray: normal Hip : normal CXR: normal CT scan of Brain: normal EEG: normal Video EEG: normal At 8 mo., still having the same paroxysmal dystonic neck deviation

Sandifer Syndrome (S S) Final diagnosis:

INCIDENCE Sandifer syndrome (SS) is a rare cause of torticollis in infants that results from gastroesophageal reflux disease (GERD)  SS occurs in less than 1% of children with GERD The abnormal posture is considered to result from a reflex mechanism that aims To protect the air passages from reflux material or To relieve the abdominal discomfort caused by acid reflux.

CLINICAL APPROACH Diagnosis may be difficult and often requires multiple evaluations by specialists Infants are often misdiagnosed due to paroxysmal neurobehaviors and receive unnecessary medication and treatment Analysis of the torticollis may help establish a suitable strategy for diagnosis Symptoms of SS usually promptly respond to antireflux medications and/or fundoplication Lehwald N, et al. Sandifer syndrome—a multidisciplinary diagnostic and therapeutic challenge. Eur J Pediatr Surg. 2007

Herman MJ. Torticollis in infants and children: common and unusual causes. Instr Course Lect. 2006

S&S Lehwald N, et al. Sandifer syndrome—a multidisciplinary diagnostic and therapeutic challenge. Eur J Pediatr Surg. 2007 SS is most commonly associated with normal examination findings, especially in infants Irritability, crying, eye deviation with head version Paroxysmal dystonic posture are common symptoms. symptoms normally occur during or shortly after feeding.

WHEN TO SUSPECT The intermittent occurrence of torticollis with alternating directions Normal sternocleidomastoid muscle, and Normal cervical radiographic findings  Probable Diagnosis

DIAGNOSTIC TESTS Requires upper gastrointestinal studies. PH metering, which is inexpensive and quick Plays a significant role in the diagnosis of SS Allow the clinician to avoid other invasive and expensive investigations