Child's Nervous System

, Volume 35, Issue 1, pp 15–18 | Cite as

Early intraventricular baclofen therapy (IVB) for children with dystonic and dysautonomic storm

  • Claudio Ruggiero
  • Giulia Meccariello
  • Pietro SpennatoEmail author
  • Giuseppe Mirone
  • Stefania Graziano
  • Marco Gilone
  • Giuseppe Cinalli
Brief Communication



Intrathecal baclofen (ITB) is an effective treatment for managing primary and secondary dystonia. Intraventricular baclofen (IVB) was first developed to allow treating patients in which the use of ITB was difficult due to anatomic anomalies. After that, several studies indicate that intraventricular administration of baclofen, is more effective than ITB in refractory dystonia.

Clinical material

We report three cases of children with acute dystonic and dysautonomic storm, treated with IVB. The clinical outcome was satisfactory. The response to the treatment continued after the pump disconnection, suggesting that in this kind of cerebral dysregulations, short-term IVB is an effective treatment.


Early treatment with IVB may be an effective option in patients with post-anoxic dysautonomic and dystonic storm.


Intraventricular baclofen therapy Dystonia Baclofen 


Compliance with ethical standards

Conflict of interest

The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.


  1. 1.
    Albright AL (2006) Intraventricular baclofen infusion for dystonia. Report of two cases. J Neurosurg 105:71–74Google Scholar
  2. 2.
    Albright AL, Ferson SS (2009) Intraventricular baclofen for dystonia: techniques and outcomes. Clinical article. J Neurosurg Pediatr 3:11–14CrossRefGoogle Scholar
  3. 3.
    Albright AL, Barry MJ, Painter MJ, Shultz B (1998) Infusion of intrathecal baclofen for generalized dystonia in cerebral palsy. J Neurosurg 88:73–76CrossRefGoogle Scholar
  4. 4.
    Barry MJ, VanSwearingen JM, Albright AL (1999) Reliability and responsiveness of the Barry Albright Dystonia scale. Dev Med Child Neurol 41:404–411CrossRefGoogle Scholar
  5. 5.
    Becker R, Benes L, Sure U, Hellwig D, Bertalanffy H (2000) Intrathecal baclofen alleviates autonomic dysfunction in severe brain injury. J Clin Neurosci 7:316–319CrossRefGoogle Scholar
  6. 6.
    Bollo J, Gooch JL, Walker ML (2012) Stereotactic endoscopic placement of third ventricle catheter for long-term infusion of baclofen in patients with secondary generalized dystonia. J Neurosurg Pediatr 10:30–33CrossRefGoogle Scholar
  7. 7.
    Dachy B, Dan B (2004) Electrophysiological assessment of the effect of intrathecal baclofen in dystonic children. Clin Neurophysiol 115:774–778CrossRefGoogle Scholar
  8. 8.
    Elkaim LM, Alotaibi NM, Sigal A, Alotaibi HM, Lipsman N, Kalia SK, Fehlings DL, Lozano AM, Ibrahim GM, North American Pediatric DBS Collaboration (2018) Deep brain stimulation for pediatric dystonia: a meta-analysis with individual participant data. Dev Med Child Neurol.
  9. 9.
    Hoarau X, Richer E, Dehail P, Cuny E (2012) A 10-year follow-up study of patients with severe traumatic brain injury and dysautonomia treated with intrathecal baclofen therapy. Brain Inj 26:927–940CrossRefGoogle Scholar
  10. 10.
    Kofler M, Poustka K, Saltuari L (2008) Intrathecal baclofen for autonomic instability due to spinal cord injury. Auton Neurosci 146:106–110CrossRefGoogle Scholar
  11. 11.
    Rocque G, Albright AL, Brandon G, Rocque MD (2012) Intraventricular versus intrathecal baclofen for secondary dystonia: a comparison of complications. Neurosurgery 70:321–325CrossRefGoogle Scholar
  12. 12.
    Walcott BP, Nahed BV, Kahle KT, Duhaime AC, Sharma N, Eskandar EN (2012) Deep brain stimulation for medically refractory life-threatening status dystonicus in children. J Neurosurg Pediatr 9:99–102CrossRefGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Pediatric NeurosurgerySantobono-Pausilipon Children’s HospitalNaplesItaly
  2. 2.Department Pediatric NeurologySantobono-Pausilipon Children’s HospitalNaplesItaly

Personalised recommendations