Background

Severe episodes of generalized dystonia have rarely been reported in the literature. Status Dystonicus (SD) is a life threatening disorder requiring prompt diagnosis and intervention.

We herein describe the clinical presentation and treatment outcome of SD in pediatric patient

Case Description:

 A 4-year-old girl with mixed-type cerebral palsy (GMFCS V). She was the product of a breach delivery at 26 weeks gestation for a non-consanguineous marriage. At the outset, she displayed abnormally painful stretching of her legs which promptly spread to all other limbs

A combination of Baclofen and a titrated Benzahexole was used but worsening of her episodes necessitated admission. Midazolam and Diazepam tried with partial response. Added Chloral Hydrate had no effect.

A brain MRI, Sepsis Screen, Electrolyte, Barium test and gastroscopy were requested. Result revealed a severe gastric reflux, growth of H. Pylori and a normal scan.

Use of anti-reflux and antibiotics brought significant improvement and patient remained clinically stable on maintenance medication.

Discussion

SD first recognized by Jankovic and Penn in 1982. Patients may develop serious metabolic, renal and respiratory complications.

Our case has proved challenging to treat. We were interested in the growth of H. Pylori and its related symptoms as the main triggers in our patient and how common these will be in others with a similar diagnosis. 

Implication

 Pediatricians should be aware of the condition so that it is recognized and treated early.  It is a rare condition and to date, no definite data exists about the optimal treatment strategy