Single intraventricular baclofen bolus for dystonic storm: case report

Review

Dystonia is characterized by involuntary sustained or intermittent muscle contractions

causing repetitive twisting movements, abnormal postures, or both (1). While Dystonic
storm, also known as status dystonicus, is a severe and acute deterioration of dystonia
primary or secondary characterized by continuous contractures and spasms with
important morbidity and mortality (2,3). The most affected subgroups are children, sex
male and etiology of cerebral palsy (7). In general, there are precipitates like infection,
trauma, drugs adjustment and failure of deep brain stimulation (DBS) or intrathecal
baclofen pump (ITB) (4). This condition is frequently refractory to pharmacological
therapy (5,6).

The diagnosis of dystonic storm must be identified as soon as possible because the risk
of complications depends on early treatment initiation (8). Dystonia severity action plan
(DSAP) has become an important tool for that objective with grade 4 and 5, besides that
it has been used for patient’s evolution (9). Also, milestones for diagnosis are at least
one sign of life-threatening as bulbar weakness, metabolic derangements, respiratory
failure and exhaustion and pain (2).

Management of dystonic storm begins with the association of principles such as address
precipitants, supportive care, calibrate sedation and dystonia specific medications (7,9).
Looking for triggers is essential because up to 65% of cases experience fever, secondary
pain due to a previous trauma or surgery, gastroesophageal reflux, failure of DBS or of
ITB and drug withdrawal or onset drug such as haloperidol and metoclopramide, which
must be treated aggressively. Supportive measures such as protection of the airway by
dystonic spasms and drowsiness for drug side-effects, early nasogastric tube indication
to avoid potential aspiration, intravenous (i.v.) hydration and metabolic and
hidroeletrolitic correction are necessary. The remission of dystonia should be achieved
by sleeping without respiratory depression. It starts with chloral hydrate enteral for
sedation and i.v. clonidine in severe cases. If there is no response, it may scale with
orotraqueal intubation, continuously i.v. midazolam and nondepolarizing paralyzing
agents, with intermittent reductions of the sedative and anaesthetic for serial
evaluations (7,9,12). In parallel, the early and fast escalation of antidystonic medications
until high doses with common polypharmacy is very important (9). Drugs combination
with the best results include trihexyphenidyl, haloperidol, tetrabenazine and sometimes
gabapentin (7).

As the Farmacological intervention have overall poor response, approximately 10%, the
discussion about invasive therapies need to happen just after rule out infection by
maintenance of medical emergency. The neurosurgical procedures are the best option
to treat distonic storm(7,10). Intrathecal baclofen (ITB) has been used only in fewer
cases that merge good results and failures by drug tolerance or hardware complications.
There is a tendency to indicate ITB in patients with dystonia associated with significant
spasticity (4,7,9,11, 12). The first target for stereotactic ablative neurosurgery was
thalamus which was abandoned because the disruption reached solely thalamocortical
connections with worse results than pallidotomy which also include pallidal blocks and
brainstem fibers running to the pedunculopontine nucleus (14). Currently, pallidotomy
has been substituted by deep brain stimulation and it has restricted use in the DBS
unavailability (7). By clinical severity, it’s usually performed bilaterally with fewer
complications than expected (14). There are good results reported in literature about
pallidotomy(15). A neuromodulative technique became DBS globus pallidus interna
(GPI) the main surgical treatment for dystonic storm (14). The effects of DBS emerge
faster than habitual dystonia besides achieving excellent results, until above the
baseline with programming (7,16). The risk of DBS infection is considerable in the
circumstances of intensive care to dystonic storm (7,17). Recently, there have been
reports that show the targeting of subthalami nucleus for DBS to treat dystonic storm
with effectiveness, based on news therapeutics experiences in dystonia (17,18).

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References:

1. Sanger TD, Chen D, Fehlings DL, et al. Definition and classification of hyperkinetic
movements in childhood. Mov Disord 2010; 25: 1538–49. 

2. Manji H, Howard RS, Miller DH, Hirsch NP, Carr L, Bhatia K, Quinn N, Marsden
CD. Status dystonicus: the syndrome and its management. Brain. 1998; 121(Pt
2):243–52.

3. Munhoz RP, Scorr LM, Factor SA. Movement disorders emergencies. Curr Opin
Neurol. 2015;28(4):406–12. A very detailed update on the clinical features and
natural history of all movement disorders emergencies.
4. Ruiz-Lopez M, Fasano A. Rethinking status dystonicus. Mov Disord
2017;32(12):1667-1676.
5. Teive HA, Munhoz RP, Souza MM, Antoniuk SA, Santos ML, Teixeira MJ, et al.
Status Dystonicus: study of five cases. Arq Neuropsiquiatr 2005; 63: 26 -29.
6. Cossu, G., Colosimo, C. Hyperkinetic Movement Disorder Emergencies. Curr
Neurol Neurosci Rep (2017) 17: 6. https://doi.org/10.1007/s11910-017-0712-
7
7. Allen, N. M., Lin, J. , Lynch, T. and King, M. D. (2014), Status dystonicus: a practice
guide. Dev Med Child Neurol, 56: 105-112. doi:10.1111/dmcn.12339
8. Combe, Laura et al. Status dystonicus in children: Early recognition and
treatment prevent serious complications European Journal of Paediatric
Neurology , Volume 20 , Issue 6 , 966 - 970
9. Lumsden DE, King MD, Allen NM. Status dystonicus in childhood. Curr Opin
Pediatr. 2017;29:674–682. doi: 10.1097/MOP.0000000000000556. [PubMed]
10. Fasano A, Ricciardi L, Bentivoglio AR, et al. Status dystonicus: predictors of
outcome and progression patterns of underlying disease. Mov Disord 2012; 27:
783–8. 


11. Kyriagis M, Grattan-Smith P, Scheinberg A, Teo C, Nak- aji N, Waugh M. Status

dystonicus and Hallervorden- Spatz disease: treatment with intrathecal baclofen
and pal- lidotomy. J Paediatr Child Health 2004; 40: 322–5.
12. Muirhead W, Jalloh I, Vloeberghs M. Status dystonicus resembling the
intrathecal baclofen withdrawal syndrome: a case report and review of the
literature. J Med Case Rep. 2010;4:294.
13. Termsarasab P, Frucht SJ. Dystonic storm: a practical clinical and video & review.
J Clin Mov Disord 2017; 4:10.
 A detailed review of ‘dystonic storm’, which
includes both a proposed pathophysiological explantion for how episodes of
severe dystonia may develop, as well as an alternative treatment algorithm for
status dystonicus.
14. Sobstyl MR, Sławek JW, Zaa ̨bek M. The neurosurgical treatment of patients in
dystonic state—overview of the literature. Neurol Neurochir Pol 2014;48:63-70.
15. Marras CE, Rizzi M, Cantonetti L, Rebessi E, De Benedictis A, Portaluri F, Randi F,
Savioli A, Castelli E, Vigevano F. Pallidotomy for medically refractory status
dystonicus in childhood. Dev Med Child Neurol. 2014; 56:649–56.
16. Nerran E, et al. Deep Brain Stimulation Treated Dystonia-Trajectory Via Status
Dystonicus. Mov Disord. 2018 May 22. doi: 10.1002/mds.27357
17. Lobato-Polo J, Ospina-Delgado D, Orrego-González E, Gómez-Castro JF, Orozco
JL, Enriquez-Marulanda A, Deep Brain Stimulation Surgery for Status
Dystonicus;
 A Single Center Experience and Literature Review, World
Neurosurgery (2018), doi: 10.1016/ j.wneu.2018.03.129.
18. Breno José Alencar Pires Barbosa, Rafael Bernhardt Carra, Kleber Paiva Duarte,
Fabio Godinho, Daniel Ciampi de Andrade, Manoel Jacobsen Teixeira, Egberto
Reis Barbosa, Rubens Gisbert Cury. Bilateral subthalamic nucleus stimulation in
refractory status dystonicus. Jns(2018), doi:10.1016/ j.jns.2018.03.007