Movement Disorder Emergencies Research Articles

Curriculum Innovations: A Podcast-Based Neurologic Emergency Flipped Classroom Curriculum for Neurology Residents.

Education on the management of neurologic emergencies is vital for neurology residents, and effective educational methods are needed. This study aims to implement and evaluate the impact of a podcast-based flipped classroom curriculum in neurologic emergencies. "Flipped classroom" instructional methods have been used in GME and informed by experiential learning theory. The objectives of this curriculum were to (1) compare the clinical phenomena discussed in the neurologic emergencies podcast(s) with your own clinical experience; (2) discuss the pitfalls in the management of the neurologic emergencies discussed in the podcast(s); and (3) develop a management plan for the neurologic emergencies discussed in the podcast(s). At 10 neurology residency programs, we implemented a 3-session flipped classroom curriculum covering topics in acute stroke, movement disorder emergencies, and status epilepticus. Each session consisted of a Neurology® podcast followed by content discussion with a clinical expert. Assessment of the curriculum included presession and postsession surveys focused on learners' confidence and attitudes toward podcast-based education. Our data sample consisted of survey responses from residents, with response volumes ranging from 29-111 across all surveys. Podcasts are already highly used by neurology residents in their self-directed education. Confidence increased among learners in the management of movement disorder emergencies (18% confident before vs 79% confident after, p < 0.001) and status epilepticus (72% confident before vs 91% confident after, p = 0.014) among those who completed the curriculum. A change in confidence in acute stroke management was not found (p = 0.15). Podcasts were consistently preferred over lectures and reading-based instructional methods while less preferred compared with simulation-based learning and case-based discussion with faculty. The podcast-based curriculum studied here showed high levels of enjoyment and perceived utility. We present a 3-part curriculum to help build learners' familiarity and confidence in 3 neurologic emergency categories. The educational impact is established in Level 1 of the Kirkpatrick paradigm. Future studies can explore a higher-level impact of this curriculum. Evolution in neurology education is shifting increasingly toward immediately accessible information via digital media. This curriculum can be useful to neurology educators who need to be increasingly agile and facile with multiple educational techniques to meet learners' needs.

High Dose Clonidine as a Novel Therapy for Symptomatic Status Dystonicus in a Pediatric Patient (P5-9.009)

<h3>Objective:</h3> To highlight the role of high dose clonidine in the treatment of refractory status dystonicus in children. <h3>Background:</h3> Status dystonicus (SD) is a movement disorder emergency characterized by involuntary sustained dystonia that can result from infections, traumatic brain injuries, hypoxia, or toxins. If under-treated, it can result in respiratory failure and end organ dysfunction. Therefore, it is crucial for SD to be identified and treated early with both supportive care and pharmacologic agents. Common pharmacologic agents include dopamine receptor blockers, baclofen, anticholinergics, and benzodiazepines. However, some patients remain refractory to standard treatments. <h3>Design/Methods:</h3> Case report and literature review. <h3>Results:</h3> A 16-month-old female presented to an outside facility in febrile status epilepticus of &gt;90 minutes duration. She suffered a brief cardiorespiratory arrest, was quickly resuscitated, and then subsequently suffered a second cardiorespiratory arrest and was transferred to our facility. Due to concern for status dystonicus, she was treated with a combination of Baclofen, Diazepam, Clonazepam, Amantadine and Trihexyphenidyl with minimal improvement. MRI showed acute bilateral globus pallidus injury. Extensive infectious, autoimmune, and genetic testing was overall unremarkable. After several days of continued refractory SD with maximized medication therapy, high-dose enteral clonidine was started, increasing dosing over the course of the week from 6 mcg/kg/day to 33 mcg/kg/day, which correlated with significant improvement in symptoms after reaching maximal dose. As she continued to improve with high dose clonidine, she was able to be weaned off of several medications and discharged to acute inpatient rehab. <h3>Conclusions:</h3> Status dystonicus can be a difficult condition to treat, as there are limited pharmaceutical treatment options in pediatric patients. Studies in adult populations have shown effectiveness of high dose clonidine, yet this has not been well described in children. This case suggests that high dose oral clonidine may be a safe and effective treatment option for refractory SD in children. <b>Disclosure:</b> Mr. Woods has nothing to disclose. Dr. Chandler has nothing to disclose. Dr. Barton has nothing to disclose.

The dangers of neuroleptic use in Parkinsonian-spectrum illnesses - management of emergent hypodopaminergic syndromes and catatonia

IntroductionIn Parkinson disease (PD), the incidence of psychotic symptoms has an estimated rate of 25-30%, a cumulative long term prevalence rate of 60%, with visual hallucinations being the most common type of sensory hallucination. The use of high and low potency, antidopaminergic, antipsychotic medications (AP) have the potential to exacerbate motor symptoms of PD and precipitate neuroleptic malignant syndrome (NMS), neuroleptic malignant-like syndrome in PD known as Parkinson hyperpyrexia syndrome (PHS), and catatonia in at risk population. MethodsHere, we present a clinical case and a review of the current treatment modalities available for movement disorder emergencies in parkinsonian-spectrum illnesses (PSI). Patient was assessed by a multidisciplinary team, including neurology and a geriatric consult-liaison psychiatry service. ResultsThis is a case report of a 79-year-old man with no previous psychiatric history, an initial diagnosis of PD due to early emergence of tremors and motor symptoms responsive to levodopa-carbidopa (LC). This was followed by progressive cognitive impairments, delusional jealousy, and formed visual hallucinations, which prompted concern for cortical lewy body involvement. Pimavanserin was initiated for presumed diagnosis of Parkinson disease psychosis and ameliorated the frequency of visual hallucinations. Patient presented to the hospital for agitation and was positive for COVID-19 on admission; prior to this, he had missed doses of LC and was febrile per family. He received intramuscular haloperidol and ziprasidone for behavioral disturbances and subsequently developed delirium, severe VH's, elevated serum CK, diffuse rigidity, and hyperactive non-purposeful activity, raising concern for NMS.Emergent treatment included bromocriptine, lorazepam, and supportive measures; home dose of LC and pimavanserin were resumed after significant reductions in CK levels. Unfortunately, rigidity and delirium persisted leading to a prolonged hospitalization and requirement of a nasogastric tube for malnutrition. Neurology was concerned for PHS due to clinical overlap with NMS. Psychiatry consult-liaison service suspected protracted delirium and catatonia secondary to recent COVID-19 infection and neuroleptic use.Initial Bush Francis Catatonia Rating Score (BFCRS) was 22. Treatment trials of benzodiazepines, amantadine and memantine were unsuccessful due to adverse effects of excessive sedation, myoclonus, and increase aggression, respectively. Ultimately, bilateral, brief wave, electroconvulsive therapy (ECT) was initiated. Patient had improvements in increased alertness, executive speech, verbal comprehension, appetite, with BFCRS 6 by ninth ECT session, and was more attuned to his surroundings. After twelve ECT sessions and successful treatment of catatonia, despite some residual delirium, family felt comfortable caring for patient at their own home with supportive services. ConclusionThis case demonstrates the nebulous presentation of overlapping syndromes of delirium and catatonia - with NMS and PHS also in the differential - in the setting of acute COVID-19 infection and iatrogenic neuroleptic use in a patient with PSI. NMS and catatonia can be precipitated by dopaminergic blockade, while PHS is most encountered during dopaminergic withdrawal and intercurrent infections. Theorized pathophysiology is due to central dopamine hypofunction, leading to clinical features of altered consciousness, fevers, elevated serum CK, and parkinsonian exacerbation – all of which can be indistinguishable from the above-mentioned diagnoses. These syndromic illnesses appear to occur on an NMS-like spectrum, are a neurological emergency and necessitate prompt treatment with supportive measures, intensive care level monitoring, and restoration of the dopaminergic balance with LC, bromocriptine, and dantrolene. ECT has shown to improve both motor and psychotic symptoms in PD, should be first line in NMS, malignant and benzodiazepine-refractory catatonia, and has even demonstrated effectiveness in a case report of PHS. ECT could be the ideal next step in exacerbated movement disorder emergencies following medication trials and supportive measures. However, the evidence is limited and the logistical nuances of conducting a randomized controlled trial in the geriatric population would be ethically challenging.

Clinico-Etiological Spectrum and Functional Outcomes of Children with Pre-Status Dystonicus and Status Dystonicus (SD): A Descriptive Study.

Status dystonicus (SD) is a life-threatening movement disorder emergency characterized by increasingly frequent and severe episodes of generalized dystonia, requiring urgent hospital admission. The diverse clinico-etiological spectrum, high risk of recurrence, and residual disabilities complicate functional outcomes. We aim to describe the clinico-etiological spectrum, radiology, therapeutic options, and follow-up of patients with pre-status dystonicus (pre-SD) and SD. A cross-sectional retrospective study was carried out in a tertiary care referral center. The clinical, laboratory, and radiology data of all patients aged less than 18 years with pre-SD and SD from January 2010 to December 2020 were collected. The Dystonia Severity Assessment Plan (DSAP) scale for grading severity and the modified Rankin Scale (mRS) for assessing outcome were used at the last follow-up visit. Twenty-eight patients (male:female: 2.1:1) experiencing 33 episodes of acute dystonia exacerbation were identified. The median age at the onset of dystonia and SD presentation was 8.71 (range: 0.25-15.75) and 9.12 (range: 1-16.75) years, respectively. Four patients experienced more than one episode of SD. The etiological spectrum of SD includes metabolic (Wilson's disease-13, L-aromatic amino acid decarboxylase deficiency-one, and Gaucher's disease-one), genetic (neurodegeneration with brain iron accumulation-three and KMT2B and THAP 1 gene-related-one each), structural-three, post-encephalitic sequelae (PES)-four, and immune-mediated (anti-NMDA receptor encephalitis-one). Five patients had pre-SD (DSAP grade 3), and 23 patients had established SD (DSAP grade 4-17 and DSAP grade 5-six). The Rapid escalation of chelation therapy precipitated SD in 11 patients with Wilson's disease. Febrile illness or pneumonia precipitated SD in nine patients. Twenty-three episodes of SD required midazolam infusion in addition to anti-dystonic medications. The median duration of hospital stay was 10 days (range: 3-29). Twenty-three patients had resolution of SD but residual dystonia persisted, while two patients had no residual dystonia at follow-up. Three patients succumbed owing to refractory SD and its complications. Early identification of triggers, etiology, and appropriate management are essential to calm the dystonic storm.

Viewpoint on Milestones for Fellowship Training in Movement Disorders.

Viewpoint on Milestones for Fellowship Training in Movement Disorders.

Status Dystonicus in Children: A Cross-Sectional Study and Review of Literature.

Background: Status dystonicus is a life-threatening, underrecognized movement disorder emergency. We aimed to ascertain the etiology, clinical presentation, complications, and outcomes of status dystonicus in children and reviewed the literature for similar studies. Methods: Records of all children aged <14 years admitted to a single center with status dystonicus between 2014 and 2018 were reviewed. Results: Twenty-four children (75% male) were identified with status dystonicus. The annual incidence rate was 0.05 per 1000 new admissions <12 years of age. The mean age at presentation was 6.3 ± 3.6 years. Median duration of hospital stay was 10.5 days (interquartile range 5-21.7). The severity of dystonia at presentation was grade 3 (n = 9; 37.5%) and 4 (n = 9; 37.5%). The most common triggering factor was intercurrent illness/infection (n = 18; 75%). The most common underlying etiologies were cerebral palsy (n = 8; 33.3%), complicated tubercular meningitis (n = 3; 12.5%), and mitochondrial disorders (n = 3; 12.5%). Basal ganglia involvement was seen in 15 cases (62.5%). Respiratory and/or bulbar compromise (n = 20; 83.3%) and rhabdomyolysis (n = 15; 62.5%) were most commonly seen. Oral trihexyphenidyl (96%) followed by oral or intravenous diazepam (71%), oral baclofen (67%), and midazolam infusion (54%) were the most common drugs used. Clonidine was used in 33% cases, without any significant side effects. Three children died owing to refractory status dystonicus and its complications; the mortality rate was 12.5%. Conclusion Status dystonicus is a neurologic emergency in children with severe dystonia, with significant complications and a high mortality rate. Static and acquired disorders are more common than heredo-familial causes. Identification and treatment of infection in children is important as the majority of cases are triggered by an intercurrent infection.

Spectrum of Movement Disorder Emergencies in a Tertiary Care Center in India: A Prospective Observational Study.

Movement disorders can present in emergency services in an acute severe form which can be life threatening if not recognized. The relative frequency and spectrum of movement disorder emergencies have not been studied extensively. We studied the frequency, spectrum, and outcome of patients presenting with movement disorders emergencies. This was a prospective, descriptive single center study. Patients presenting with acute movement disorders to the neurology emergency services of the institute during the study period from April 2019 to June 2021 were analyzed. A total of 71 patients presented with acute movement disorders during the study period. Out of them, 65 patients had hyperkinetic and 6 patients had hypokinetic movement disorders emergencies. Fifteen patients were below the age of 18 years. Chorea (59.1%) was the most common movement disorder emergencies followed by dystonia and myoclonus in adults. Dystonia (33.3%) was the common movement disorder emergencies in children. Hyperglycemia followed by stroke was the most common etiology of acute movement disorders. This study brings out some novel findings on the movement disorders emergencies in Indian scenario. Chorea was the most common movement disorder emergencies presenting to the neurology emergency services. Early recognition and management of movement disorders emergencies help in reducing morbidity.

Movement disorders in emergencies

Movement disorder emergencies refer to clinical scenarios, evolving acutely or subacutely in which the clinical presentation is dominated by a primary movement disorder, and failures to accurately diagnose and manage the patient may result in significant morbidity or even mortality. Whilst this entity may seem to be relatively uncommon compared to other medical emergencies, the last two decades have seen increasing recognition of various movement disorder emergencies as recognised in the literature to include such diverse entities from acute forms of parkinsonism, chorea, dystonia, myoclonus, and tics to acute abnormal movements manifestations in those with underlying disorders like Wilson's disease, NMDA-receptor antibody encephalitis, and several acquired disorders. In this session, important not-to-miss emergencies will be divided into hypokinetic, hyperkinetic, and movement disorder conditions with neuropsychiatric emergencies. The presentation of each example will be supported by video illustrations with the objectives to highlight clinical features, precipitating factors, prodromal symptoms or signs that are associated with specific forms of movement disorder emergencies as well as management strategies. During this pandemic era, examples of movement disorder emergencies related to COVID-19 infection will also be presented and discussed.

Treatment of Movement Disorder Emergencies in Autoimmune Encephalitis in the Neurosciences ICU.

Immune response against neuronal and glial cell surface and cytosolic antigens is an important cause of encephalitis. It may be triggered by activation of the immune system in response to an infection (para-infectious), cancer (paraneoplastic), or due to a patient's tendency toward autoimmunity. Antibodies directed toward neuronal cell surface antigens are directly pathogenic, whereas antibodies with intracellular targets may become pathogenic if the antigen is transiently exposed to the cell surface or via activation of cytotoxic T cells. Immune-mediated encephalitis is well recognized and may require intensive care due to status epilepticus, need for invasive ventilation, or dysautonomia. Patients with immune-mediated encephalitis may become critically ill and display clinically complex and challenging to treat movement disorders in over 80% of the cases (Zhang et al. in Neurocrit Care 29(2):264-272, 2018). Treatment options include immunotherapy and symptomatic agents affecting dopamine or acetylcholine neurotransmission. There has been no prior published guidance for management of these movement disorders for the intensivist. Herein, we discuss the immune-mediated encephalitis most likely to cause critical illness, clinical features and mechanisms of movement disorders and propose a management algorithm.

Urgencias en trastornos del movimiento

Movement disorders emergencies are those related with abnormal movements characterized by their rapid onset, and appearing both in usual clinical practice and emergency department. Different entities, phenomenological similar, are included (parkinsonism, dystonia, chorea, tremor and myoclonus). Detailed clinical assessment is the only differential diagnosis test, becoming their diagnosis a challenge for the inexperienced physician, and even for general neurologist. Most of them are drug-induced movement disorders. Today this is a key feature, due to the fact that the aging population is growing and it is a population highly polymedicated. In order to avoid iatrogenic harm, high suspicious index has to be maintained. A proper management usually ensures a good prognosis. Main movement disorders and their clinical, diagnostic and therapeutic features, both in hospitals and in emergency departments are described in current review.

Movement Disorders Emergencies.

Many acute and potentially life-threatening medical conditions have hyperkinetic or hypokinetic movement disorders as their hallmark. Here we review the clinical phenomenology, and diagnostic principles of neuroleptic malignant syndrome, malignant catatonia, serotonin syndrome, Parkinsonism hyperpyrexia, acute parkinsonism, acute chorea-ballism, drug-induced dystonia, and status dystonicus. In the absence of definitive lab tests and imaging, only a high index of clinical suspicion, awareness of at-risk populations, and variations in clinical presentation can help with diagnosis. We also discuss the principles of management and rationale behind treatment modalities in the light of more recent evidence.

Status dystonicus: management and prevention in children at high risk.

Background: Status dystonicus (SD) is a movement disorder emergency associated with significant morbidity and life-threatening events that requires immediate and effective treatment. Nevertheless, SD is currently an under-recognized and undertreated condition, partly due to the lack of a standard definition and because it can be the acute complicated course of both primary and secondary dystonias. In subjects with SD, due to the delay of identification and lacking prevention of trigger and precipitant factors, intensive care management is consistently required. Objectives: We performed a critical review of this topic, outlining clinical features and linked genetic disorders to recognize subject at higher risk of SD, describing precipitant and trigger factors and proposing potential pharmacological treatment strategies in order to prevent hospitalization. Results: Genetic predisposition included: primary dystonias particularly in the case of TOR1A mutation; epileptic encephalopathy such as ARX and GNAO1 genetic variants and neurodegenerative disorders as PANK2. Early recognition of SD should be oriented by the following sign and symptoms: fever, tachycardia, respiratory change, hypertension, sweating and autonomic instability, elevated serum CK. Pain, fever and dehydration are main trigger factors that have to be prevented or quickly controlled. Achieving sleep could be the first therapeutic option in those with high risk of developing SD. Recently, enteral or transdermal clonidine as safety and efficacy therapeutic alternative was proposed. Conclusion: Recognizing high risk children for Status dystonicus from the onset of subtle signs and avoiding trigger factors could drive towards better management avoiding intensive treatments. (www.actabiomedica.it)

When time is of the essence: Managing care in emergency situations in Parkinson's disease

A movement disorder emergency is an acute or sub-acutely evolving neurological illness predominated by a primary movement disorder. Although Parkinson's disease (PD) is a chronic, progressive disorder, patients may present with a variety of acute symptoms. Timely diagnosis and management is crucial to reduce mortality and morbidity. The underlying causes of an emergency in PD may be attributable to either disease related emergencies which occur as a direct consequence of the disease pathophysiology or secondary to anti-parkinsonian medications, such as Parkinsonism-hyperpyrexia syndrome, acute psychosis, etc. Indirect disease related emergencies, are those which not directly associated with the disease but occur secondary to deficits produced by the disease, e.g. falls and pneumonia. Emergencies in patients with PD may also be related to deep brain stimulation or systemic illnesses which are unrelated to PD. Complications may also occur during the surgical management of patients with PD owing to the effects of the disease on cardiovascular and respiratory functions or interactions of PD medication with anesthetic agents. Additionally, although motor complications are expected to form a majority of ER admissions in PD, several studies have reported indirect or non-PD related complications as the predominant primary reason for emergency admissions. The present article aims to review emergencies encountered in patients with PD with focus on the identification and management of these emergencies.

Challenges in PD Patient Management After DBS: A Pragmatic Review.

Deep brain stimulation (DBS) of the subthalamic nucleus (STN) or internal globus pallidus (GPi) represents an effective and universally applied therapy for Parkinson's disease (PD) motor complications. However, certain procedure-related problems and unrealistic patient expectations may detract specialists from indicating DBS more widely despite significant clinical effects. This review provides a pragmatic educational summary of the most conflicting postoperative management issues in patients undergoing DBS for PD. DBS in PD has been associated with certain complications and post-procedural management issues, which can complicate surgical outcome interpretation. Many PD patients consider DBS outcomes negative due to unfulfilled expectations, even when significant motor symptom improvement is achieved. Speech, gait, postural stability, and cognition may worsen after DBS and body weight may increase. Although DBS may induce impulse control disorders in some cases, in others, it may actually improve them when dopamine agonist dosage is reduced after surgery. However, apathy may also arise, especially when dopaminergic medication tapering is rapid. Gradual loss of response with time suggests disease progression, rather than the wearing off of DBS effects. Furthermore, implantable pulse generator expiration is considered a movement disorder emergency, as it may worsen parkinsonian symptoms or cause life-threatening akinetic crises due to malignant DBS withdrawal syndrome. Major unsolved issues occurring after DBS therapy preclude complete patient satisfaction. Multidisciplinary management at experienced centers, as well as careful and comprehensive delivery of information to patients, should contribute to make DBS outcome expectations more realistic and allow post procedural complications to be better accepted.

Rethinking status dystonicus.

Status dystonicus is a movement disorder emergency that has been a source of controversy in terms of terminology, phenomenology, and management since it was first described in 1982. Here we argue that the current use of the term status dystonicus falls well short of the precision needed for either clinical or academic use. We performed a critical review on this topic, describing possible pathophysiological mechanisms and areas of uncertainties. This review also addresses the problems derived by the extreme clinical heterogeneity of this condition, as the lack of an objective criterion useful for the definition, or the fact that status dystonicus may present not only in the context of a known dystonic syndrome. We propose a new possible definition that includes not only dystonia but also other hyperkinetic movements in the wide range of movement disorders that can be seen during an episode. The new definition keeps the term status dystonicus and highlights the fact that this is a medical emergency based on the impairment of bulbar and/or respiratory function requiring hospital admission as the principal feature. Furthermore, the new definition should not consider as necessary unspecific features as patient's condition at baseline, the distribution of dystonia, occurrence of systemic symptoms such as fever or laboratory findings. We hope that this proposal will stimulate the debate on this subject among our peers, further developing a clinical and pathophysiological understanding of status dystonicus. © 2017 International Parkinson and Movement Disorder Society.

Hyperkinetic Movement Disorder Emergencies.

A movement disorder emergency has been defined by Fahn and Frucht as "any neurological disorder evolving acutely or subacutely, in which the clinical presentation is dominated by a primary movement disorder, and in which failure to accurately diagnose and manage the patient may result in significant morbidity or even mortality." In this review, we discuss the most common situations in which hyperkinetic movement disorders, including chorea, ballism, dystonia, myoclonus, tics, as well as psychogenic disorders, can present as emergencies. Some acute hyperkinetic issues that can complicate Parkinson's disease and parkinsonism, such as the rare dyskinesia-hyperpyrexia syndrome, will be also covered. The phenomenology and natural history of medication-induced dystonic reaction, a common form of acute secondary dystonia frequently leading to emergency department consultations, are discussed in detail. Despite the acute nature, most of these conditions can result in a good outcome. Rare but serious disorders such as status dystonicus or malignant Gilles de la Tourette syndrome may lead to substantial morbidity and mortality. Thus, we emphasize the need to develop means for more accurate and prompt recognition and treatment of these syndromes.

Movement disorders emergencies

Although movement disorders are traditionally viewed as chronic diseases that are followed electively, a growing number of these patients present with acute, severe syndromes or complications of their underlying neurological problem. Identifying and managing movement disorders emergencies is challenging, even for the specialist. This review summarizes evidence outlining the clinical presentation of acute, life-threatening movement disorders. We review the most significant aspects in the most common movement disorders emergencies, including acute complications related to Parkinson's disease and parkinsonism, serotonergic, and neuroleptic malignant syndromes, chorea, ballismus, dystonia, myoclonus, and tics. The increasing amount of information delineating the descriptions of movement disorders emergencies provides means for more effective prevention, identification, and management for the nonspecialist. Although the commonest of these syndromes eventually have a good outcome, serious conditions such as neuroleptic malignant syndrome and status dystonicus may induce substantial rates of morbidity and mortality. This review re-emphasizes the need for their prompt identification and management.

Treatment of movement disorder emergencies.

Movement disorder emergencies are defined as clinical scenarios where a movement disorder develops over hours to days, and in which morbidity and even mortality can result from failure to appropriately diagnose and manage the patient. The last decade has seen increasing recognition of various movement disorder emergencies, including acute parkinsonism, neuroleptic malignant syndrome, respiratory compromise in multiple system atrophy, dystonic storm, oculogyric crisis, and hemiballism, among others. This article will review the major movement disorder emergencies encountered in the hospital and office, emphasizing practical management and treatment.

Status dystonicus: a practice guide

Status dystonicus is a rare, but life-threatening movement disorder emergency. Urgent assessment is required and management is tailored to patient characteristics and complications. The use of dystonia action plans and early recognition of worsening dystonia may potentially facilitate intervention or prevent progression to status dystonicus. However, for established status dystonicus, rapidly deployed temporizing measures and different depths of sedation in an intensive care unit or high dependency unit are the most immediate and effective modalities for abating life-threatening spasms, while dystonia-specific treatment takes effect. If refractory status dystonicus persists despite orally active anti-dystonia drugs and unsuccessful weaning from sedative or anaesthetic agents, early consideration of intrathecal baclofen or deep brain stimulation is required. During status dystonicus, precise documentation of dystonia sites and severity as well as the baseline clinical state, using rating scales and videos is recommended. Further published descriptions of the clinical nature, timing of evolution, resolution, and epidemiology of status dystonicus are essential for a better collective understanding of this poorly understood heterogeneous emergency. In this review, we provide an overview of the clinical presentation and suggest a management approach for status dystonicus.

Severe exacerbation of undiagnosed restless legs syndrome presenting as a movement disorder emergency

Severe exacerbation of undiagnosed restless legs syndrome presenting as a movement disorder emergency