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Indian Pediatr 2010;47: 883-885 |
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Status Dystonicus: A Rare Complication of
Dystonia |
Devendra Mishra, Swati Singhal and
Monica Juneja
From the Department of Pediatrics, Maulana Azad
Medical College (University of Delhi), Delhi, India.
Correspondence to: Dr Devendra Mishra, Department of
Pediatrics, Maulana Azad Medical College,
2, BSZ Marg, Delhi 110002, India.
Received: May 22, 2009;
Initial review: July 7, 2009;
Accepted: July 14, 2009.
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A severe episode of dystonia refractory to standard drug therapy has
been labeled as status dystonicus or dystonic storm. We report the
development of this complication in a 10-year old boy with idiopathic
torsion dystonia, the probable precipitating factor being either an
infection or introduction of clonazepam.
Key words: Child, Dystonia, India, Management, Status
dystonicus.
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Patients with primary and secondary
dystonic conditions occasionally develop severe episodes of generalized
dystonia and rigidity, which may be refractory to standard drug
therapy(1). This condition has variously been labeled as ‘status
dystonicus (SD)’ or ‘dystonic storm’(2), or the patients labeled as
‘desperate dystonics’(3). The condition is quite rare with less than 40
episodes reported in the literature at the last count, and none from
India(4). We herein describe the clinical presentation and management of
an episode of SD in a pediatric patient with idiopathic torsion dystonia.
Case Report
A 10-year-old boy, a known patient of idiopathic
torsion dystonia, presented with a one-month history of progressive
worsening of dystonia. The child was a product of a non-consanguineous
marriage, born of a normal pregnancy and delivery, and had been developing
normally till 5 years of age. There was no family history of dystonia, and
hepatic or neurological disease. At the age of five years, the parents
noted the child to have an abnormal gait. Subsequently, there was gradual
onset of dystonia of his legs (left followed by right) apparent over a
6-month period. The dystonia gradually spread to involve all four limbs.
There was no diurnal variation. He received treatment at multiple centers
with various diagnoses including, seizure disorder, cerebral palsy,
conversion reaction, Wilson disease, etc. The various medi-cations
received at different times, till one month prior to presentation
(mid-march 2008), included multivitamin combinations, Vitamin E, calcium,
Vitamin D, zinc, alprazolam, trihexyphenidyl, tizanidine, tetrabenazine,
penicillamine and sodium valproate, in addition to many medications from
the various traditional Indian systems of medicine (Unani, Ayurveda,
Homeopathy, etc.). He had been investigated for Wilson disease with
negative results.
Prior to this episode of dystonia (early march), the
child could not walk, could sit without support, could feed himself by
hand (with spillage), but had no other abnormal movements and had normal
speech. In mid-march 2008 (one month prior to presentation), the child
changed physicians and two new drugs were added to the previous treatment
viz., clonazepam and syndopa (levodpoa with carbidopa). Over the
next 2 weeks, the child had increased dystonia, including the axial
musculature. In addition, he had choreiform movements, slurring of speech,
drooling of saliva and difficulty in swallowing. This was associated with
excessive exhaustion and pain, and the child could not sleep. He had no
metabolic derangements, no impairment of respiratory function and no
hypoxemia. Ten days prior to admission, the child had developed a gluteal
abscess, which was managed with incision and drainage, and oral co-amoxyclav.
At our center, initial medical treatment consisted of
increasing the dose of trihexyphenidyl (4.5mg/d) and diazepam (30mg/d),
and continuing with clonazepam (1.5 mg/d) and L-dopa (100mg/d). Transfer
to ICU could not be arranged. Over the next few days, there was no
improvement in the dystonia, and sleep disorder. After a detailed review
of the drug history, the possibility of choreiform movements being
drug-induced was also entertained, possibly due to trihexyphenidyl or
L-dopa. Further, the status dystonicus could also have been precipitated
by the drugs. On this premise, trihexyphenidyl and L-dopa were gradually
tapered (to avoid the possibility of malignant hyperthermia) and, diazepam
and clonazepam increased. Over the next three weeks, the dystonia
gradually decreased, and, pain and exhaustion subsided.
Discussion
Status dystonicus (SD), first recognized by Jankovic
and Penn in 1982, has been defined as "increasingly frequent and severe
episodes of generalized dystonia, which necessitate urgent hospital
admission. Patients have one or more of the following life threatening
complications: bulbar weakness compromising upper airway patency with the
risk of pulmonary aspiration; progressive impairment of respiratory
function leading to the development of respiratory failure, exhaustion and
pain; and metabolic derangements"(1). Although a life-threatening
disorder, the condition is not common, with two large series of total 17
patients; 12 over a 10-year period from various centers in UK(1), and 5
patients from four neurology centers from Brazil(5). Rest of the
literature consists of multiple case-reports in both adults and
children(2,3,6).
Several drugs and surgical procedures in different
combinations have been tried in SD, but no definite data seems to be
available on the best therapeutic stategy(4). Although orally active drugs
may occasionally arrest SD(3,5), the current literature favors using
intravenous agents for deep sedation(2,4). As the patients have intense
muscle activity, they are liable to develop metabolic complications such
as rhabdomyolysis, that may lead to acute renal failure(1). In addition,
bulbar and respiratory complications may ensue, thereby requiring tracheal
intubation; in addition to hyper-pyrexia, muscle exhaustion, pain and
dehydration. In view of these, SD patients should be routinely managed in
intensive care settings(4). This also facilitates the currently accepted
management strategy for severe SD, which consists of deep sedation under
muscle paralysis and assisted ventilation(4). The sedation may be done
with increasing doses of intravenous infusion of midazolam (30-100
µg/kg/hour), to which propofol (0.5-2.0 mg/kg/hr) may be added(4). The
duration of sedation is determined empirically by intermittently reducing
the sedation and evaluating the child. Intrathecal baclofen may also be
used, although the literature does not provide clear cut evidence for
efficacy(6,7). Second-line strategies, especially in those with
progressive disorders, involve electrical deep brain stimulation of globus
pallidus internus(8) or bilateral pallidotomy(5,9).
The most important differential diagnoses are
neuroleptic malignant syndrome and malignant hyperthermia. This is more
important because drugs used in the treatment of dystonia, such as
tetrabenazine (being used in this child also) and lithium, as well as
levodopa withdrawal, have all been implicated as causing such a malignant
synd-rome(1,5,10). Of the 30 patients (37 episodes) reported in
literature, 25 (86.7%) were males and 17 (56.7%) younger than 14 years,
and 3 each worsened and died, respectively(4). Majority of them required
treatment in ICUs with intravenous sedation or muscle paralysis with
ventilation, but a few have responded to oral therapy also(3,5). Addition
of clonazepam and infection/stress (both present in this child) have been
implicated as precipitating factors in quite a few patients(4). Although
clonazepam addition seems to be incriminated in this child also, but the
child improved with continuation of clonazepam; which disfavors this
possibility. Coincidental onset of SD with clonazepam has previously also
been reported, with no improvement on stopping the drug(1). Clonazepam has
also been used for SD management(1), as in our patient. Trauma, surgery
and infection are frequently reported trigger factors(5), and two of these
were also present in this child.
We have reported a pediatric patient with status
dystonicus, a rare, life-threatening complication of dystonia. Both
pediatricians and pediatric neurologists should be aware of the condition,
so that it is recognized early and management can be initiated. The
management and outcome are highly variable but a general consensus on the
use of IV sedation and ICU management is currently evident from
literature.
Contributors: DM did the literature search and
manuscript preparation. SS helped in the literature search and preparation
of the manuscript. MJ supervised the manuscript preparation and revised it
for important intellectual content. All the authors were involved in
patient management, decision to publish and approving the final manuscript
for publication. DM will be the guarantor.
Funding: None.
Competing interests: None stated.
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