Pediatric Physical Medicine and Rehabilitation Physician CHP Pittsburgh, Pennsylvania, United States
Case Diagnosis: Status dystonicus (SD)is a life-threatening movement disorder described by increasingly frequent andsevere episodes of generalized dystonia. Populations at risk include patients with primary or secondary chronic dystonia, cerebral palsy, neurodegenerative disorders, neurometabolic disorders, vascular events, hypoxia, trauma, and acute drug withdrawal. With SD, it is important to identify the underlying etiology and possible triggers.The mortality rate for SD is thought to be around 12.5%. This high mortality rate suggests a need for earlier identification of SD and investigation of new treatment plans.
Case Description: This patient wasa 2 year old male with medical history of cardiac arrest due to drowning resulting in a hypoxic ischemic brain injury. He had recently tested positive for adenovirus and was brought to the hospital in SD. He was managed in the ICU,intubated and sedated andtrialed on several treatments including oral baclofen,clonidine, benzodiazepines, dilaudidboluses, propofol and others, which he was refractory. The patient was not a candidate for a baclofen pump due to body habitus. One treatment option that had not been considered was intranasalbaclofen. Hewas started on 100 mcg of intranasal baclofen which was increased by 100mcg every four hours. At 800 mcg, there were four to five hours where the patient did not require any as needed medications and had improvement in tone. Unfortunately, he continued in dystonic crisis and life saving care was withdrawn.
Discussions: The patient did have some clinical improvement with intranasal baclofen. Intranasal baclofen may have a role in other acute medical conditions such as baclofen withdrawal or baclofen pump failure. It could be utilized as a home rescue medication, similarly, to rescue medications for seizures.
Conclusions: The role of intranasal baclofen in the treatment of SD requires further investigation, however, could offer patients, families and clinicians an additional option.
