Status dystonicus: study of five cases

Abstract

-Status Dystonicus (SD) is characterized by generalized muscle contractions in dystonic patients. We report 5 cases of SD, two of which in patients with dystonic cerebral palsy, one in a patient with primary segmental dystonia, one in a patient with Hallervorden-Spatz syndrome and one in a patient with Wilson's disease (WD). Three patients were admitted to an intensive care unit and treated with propofol and midazolam, and two were submitted to neurosurgical procedures (bilateral pallidotomy and bilateral pallidal deep brain stimulation). Triggering factors were identified in three patients as follows: infection, stress-induced and zinc therapy for WD. On follow-up, two patients presented with significant improvement of dystonia, whereas the other three cases the clinical picture ultimately returned to baseline pre-SD condition.

Indwelling intrathecal catheter with subcutaneous abdominal reservoir: a viable baclofen delivery system in severely cachectic patients

“…15 Precipitating causes include infection, 29,30 medication change, 29,43 and surgery, 33,47 so careful thought should be given to minimizing these factors in patients with dystonia. As with dyskinetic CP, response to oral drugs is generally poor, with one series reporting that just 10.1% of patients responded to treatment.…”

Movement disorders emergencies: a review

“…SD was first recognized by Jankovic and Penn 39 in 1982 and is defined as "(…) increasingly frequent and severe episodes of generalized dystonia, which necessitate urgent hospital admission". These episodes are usually refractory to traditional pharmacological therapy and may be triggered by trauma, surgery, infection, fever and abrupt introduction, withdrawal or change in medical treatment, including lithium, tetrabenazine and clonazepam [40][41][42] . The muscle contractures are painful, interfering with respiratory function and causing metabolic complications as hyperpyrexia, dehydration, respiratory insufficiency, rhabdomyolysis and acute renal failure.…”

“…The muscle contractures are painful, interfering with respiratory function and causing metabolic complications as hyperpyrexia, dehydration, respiratory insufficiency, rhabdomyolysis and acute renal failure. SD is rare and no definitive consensus exists regarding the ideal treatment strategy that most often include a combination of several drugs and even surgical interventions [40][41][42] . Most reported cases required prompt therapeutic intervention with adequate fluid balance, analgesia, antipyretics, ventilatory support and hemodynamic monitoring, including admission in intensive care units to avoid metabolic, renal and ventilatory complications.…”

“…Agents used in previous case series and reports include tetrabenazine, baclofen, chlormethiazole, anticholinergics (benzhexol and biperiden), dopamine receptor blockers (haloperidol and pimozide), anticonvulsants (carbamazepine, primidone and valproic acid), levodopa, acetazolamide, benzodiazepines (midazolan, clonazepam and diazepam), deep sedation with propofol, barbiturate anesthesia and muscle blockers [39][40][41][42] . These drugs were used in anecdotal reports as combinations with variable efficacy, often requiring additional interventions, for example, continuous infusion of intrathecal baclofen and stereotactic surgery (thalamotomy, pallidotomy or DBS electrodes placement).…”

“…These drugs were used in anecdotal reports as combinations with variable efficacy, often requiring additional interventions, for example, continuous infusion of intrathecal baclofen and stereotactic surgery (thalamotomy, pallidotomy or DBS electrodes placement). As already outlined, SD is a potentially life threatening complication and its course and outcome is highly variable, including cases with complete remission, deterioration of clinical condition and death [40][41][42] .…”