1. Case Report: Paroxysmal Neck Dystonia
HADI FAKIH MD
NICU Director- SRHH
Case Report: Paroxysmal Neck Dystonia

2. 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

3. Video

4. 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

5. 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

6. Sandifer Syndrome (S S)
Final diagnosis:

7. 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.

8. 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

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

10. 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.

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

12. 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