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Respiratory Aspects of Neurological Disease
Published in John W. Scadding, Nicholas A. Losseff, Clinical Neurology, 2011
In idiopathic Parkinson’s disease respiratory impairment is associated with upper airflow obstruction, reduced tidal volume, respiratory muscle weakness, restrictive defect due to respiratory muscle rigidity, abnormalities of central CO2 sensitivity and impairment of voluntary control. Patients with primary and secondary dystonic syndromes occasionally develop severe episodes of generalized dystonia and rigidity (status dystonicus) which may be refractory to standard drug therapy. The most severe cases may develop bulbar and ventilatory failure necessitating intubation and ventilation.
Deep brain stimulation for childhood dystonia: current evidence and emerging practice
Published in Expert Review of Neurotherapeutics, 2018
Lior M. Elkaim, Phillippe De Vloo, Suneil K. Kalia, Andres M. Lozano, George M. Ibrahim
A detailed, revised definition of status dystonicus (SD) has recently been proposed [98]. Succinctly, SD is a life-threatening movement disorder emergency requiring early recognition, hospitalization and intervention. DBS has previously been described as an effective method of treating children and youth in SD [99]; however, overall mortality in reported cases of SD is stable at roughly 10% [98], perhaps due to underutilization or delayed use of DBS within this setting. In a recent literature review of DBS use for SD including both children and adults, resolution of SD was achieved in 94% of patients within 3 months of surgical (DBS or intrathecal baclofen pump (ITB)) intervention [100], with best outcomes reported in DYT1 patients. Similar results were seen in a review of the pediatric literature which identified 18 patients with SD treated with DBS, with resolution achieved in 16/18 patients [17]. Consistent with previous reports, increased complication rates were observed (8 of 16 reported), and included peri-operative heart failure with subsequent death in one patient [34]. SD in patients with active DBS may be very rare, except in those with PKAN.
Exploring the role of botulinum toxin in critical care
Published in Expert Review of Neurotherapeutics, 2021
Muhammad Ubaid Hafeez, Michael Moore, Komal Hafeez, Joseph Jankovic
Dystonia is a movement disorder characterized by sustained muscle contraction. Status dystonicus (SD) is a medical emergency when a preexisting generalized dystonia rapidly escalates to a life-threatening, extreme dystonic crises [7–9]. The condition can lead to complications such as respiratory arrest or rhabdomyolysis requiring close monitoring in ICU. In addition to identifying underlying triggers and supportive treatment; benzodiazepines, baclofen, anticholinergics, and vesicular monoamine transporter-2 inhibitors are often used. For refractory cases, midazolam, propofol, barbiturate, neuromuscular blockade, deep brain stimulation, or targeted neurosurgical ablations are tried in a stepwise approach [9,10].
Staged pallidotomy: MRI and clinical follow-up in status dystonicus
Published in British Journal of Neurosurgery, 2019
Angelo Franzini, Vincenzo Levi, Andrea Franzini, Ivano Dones, Giuseppe Messina
A minority of patients with dystonia may present, in the context of a worsening picture of such disease, a condition characterized by severe and generalized dystonic spasms often refractory to standard pharmacological treatment. This condition is termed status dystonicus (SD) or “dystonic storm”. This is a rare and heterogeneous condition, with a male predominance. In addition, 60% of these cases are between 5 and 16 years of age.2 The real incidence of this disease is not precisely know, because of the relative paucity of reported cases in international literature, and, as such, it could be higher than expected. we SD is considered to be a life threatening pathology because the spasms, that are extremely painful, often interfere with respiratory and neuro-vegetative functions, thus causing metabolic imbalances such as hyperpyrexia, dehydration, respiratory insufficiency, and acute renal failure secondary to rhabdomyolisis.3,4 The latter condition is particularly dangerous for the subsequent hyperkalaemia, acidosis, hypocalcaemia and other complications. SD more mostly occurs in the context of acquired (secondary) dystonias and is often precipitated by events such as fever, trauma, surgery and by drugs such as haloperidol, metoclopramide, clonazepam.5 In a rececent series of 5 patients, Deep Brain Stimulation (DBS) was shown to be an effective therapy for such patients,6 although the reported cases are too few to draw any definitive conclusion on its overall efficacy; anyway, surgical lesional techniques remain a viable option in cases where DBS is not feasible (such as when the risk of complications of DBS are high, or in the case patients or their caregivers do not accept the insertion of an implanted device). Recent reports witness the feasibility and efficacy of bilateral pallidotomy in patients with status dystonicus or with sever and disabling dystonia.7