Movement Disorders Emergencies: a Review Emergências Em Distúrbios Do Movimento: Uma Revisão Renato P
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VIEWS AND REVIEWS Movement disorders emergencies: a review Emergências em distúrbios do movimento: uma revisão Renato P. Munhoz1,2, Mariana Moscovich1, Patrícia Dare Araujo1, Hélio A. G. Teive2 ABSTRACT Movement disorders (MD) encompass acute and chronic diseases characterized by involuntary movements and/or loss of control or ef- ficiency in voluntary movements. In this review, we covered situations in which the main manifestations are MDs that pose significant risks for acute morbidity and mortality. The authors examine literature data on the most relevant MD emergencies, including those related to Parkinson`s disease, acute drug reactions (acute dystonia, neuroleptic malignant syndrome, serotonergic syndrome and malignant hyper- thermia), acute exacerbation of chronic MD (status dystonicus), hemiballism and stiff-person syndrome, highlighting clinical presentation, demographics, diagnosis and management. Key words: movements disorders, dystonia, neuroleptic malignant syndrome, serotonergic syndrome, malignant hyperthermia, status dystonicus, dyskinesias, stiff person syndrome. RESUMO Os distúrbios do movimento (DM) englobam doenças agudas e crônicas caracterizadas por movimentos involuntários e/ou perda do controle ou eficiência em movimentos voluntários. Nesta revisão, incluímos situações nas quais as principais manifestações são DM que represen- tam risco devido à alta morbidade e mortalidade. Os autores revisaram aspectos relacionados às principais emergências em DM, incluindo aquelas relacionadas a doença de Parkinson; reações causadas por drogas (distonia aguda, síndrome neuroléptica maligna, síndrome se- rotoninérgica, hipertermia maligna); exacerbação aguda de DM crônicos (status distonicus); hemibalismo e síndrome da pessoa rígida. São destacados a apresentação clínica, os dados demográficos, o diagnóstico e o tratamento. Palavras-Chave: distúrbios de movimentos, distonia, síndrome maligna neuroléptica, síndrome serotoninérgica, hipetermia maligna, status distonicus, discinesias, síndrome da pessoa rígida. Movement disorders (MD) encompass disorders charac- II. Acute drug reactions terized by involuntary movements and/or loss of control or a. Acute dystonia efficiency in voluntary movement1. Patients with MD usu- b. Neuroleptic malignant syndrome ally have disabling symptoms; however, most cases are fol- c. Serotonergic syndrome lowed and treated in ambulatory care settings. A MD emer- d. Malignant hyperthermia gency is defined as a neurological disorder evolving acutely III. Acute exacerbation of chronic MDs or subacutely, in which the clinical presentation is dominated a. Status dystonicus by MD and in which failure to accurately diagnose and man- b. Neurological and orthopedic complications of dysto- age the patient may result in significant morbidity or even nia and pseudodystonia mortality1. In this review, we used these criteria to divide MD c. Laryngeal dystonia in multiple system atrophy and emergencies into six main topics. other conditions I. Emergencies in Parkinson’s disease (PD) d. Tic status and neurological complications of tics a. Severe motor fluctuations and the super OFF e. Wilson’s disease emergencies phenomenon f. Others conditions b. Parkinsonism-hyperpyrexia and dyskinesia-hyperpy- IV. Acute chorea and hemiballism-hemichorea rexia syndromes V. Stiff-person syndrome c. Acute parkinsonism VI. Lethal catatonia d. Acute psychosis in Parkinson’s disease 1Department of Neurology, Pontifical Catholic University of Paraná (PUCPR), Curitiba PR, Brazil; 2Movement Disorders Unit, Neurology Service, Internal Medicine Department, Federal University of Paraná (UFPR), Curitiba PR, Brazil. Correspondence: Renato P. Munhoz; Avenida Sete de Setembro 4698/1507; 80240-000 Curitiba PR - Brasil; E-mail: [email protected] Conflict of interest: There is no conflict of interest to declare. Received 14 January 2012; Received in final form 27 January 2012; Accepted 06 February 2012 453 I. EMERGENCIES IN PARKINSOn’s diseaSE (PD) low levels of levodopa in the rising or declining phase of the plasma concentration curve3. Gershanik et al.4 reported a re- a. Severe motor fluctuations and the super OFF duction in the severity of super OFF phenomenon in patients phenomenon treated with clozapine, while Dziewczapolski et al.3 found Motor fluctuations (MF) develop in 70% of Parkinson’s the same beneficial effects of clozapine in an animal mod- disease patients treated with levodopa for nine years or lon- el of parkinsonism. These authors speculate on the possibil- ger and in nearly all patients with early onset PD after less ity that clozapine could inhibit the inhibitory action of low than ten years of treatment2. The definition of MF is closely plasma levodopa levels, leading to an apparent antiparkin- related to the definition of ON and OFF periods: an ON pe- sonian effect. Another study5 explored the pathophysiology riod is the time of levodopa responsiveness, with adequate of super OFF episodes by examining the effects of subthresh- control of motor symptoms, while an OFF period is the time old, threshold and suprathreshold infusions of apomorphine when parkinsonian symptoms are significantly present. Both in a randomized, double-blind, placebo controlled, crossover usually correlate directly or indirectly with levodopa plasma study. They concluded that these episodes may be present levels, however the mechanisms that lead to MF are com- only in certain subgroups of PD patients. They also suggested plex and involve the reduction of striatal synthesis and stor- that the main mechanism involved is not likely to be a pre- age of dopamine from exogenous levodopa and subsensitiza- synaptic or autoreceptor effect of dopaminergic agents, thus tion of postsynaptic dopaminergic receptors2. The “wearing leaving the possibility of a post synaptic cause5. off ” phenomenon describes the presence of end dose OFF periods, mainly due to the loss of stratial dopamine stor- b. Parkinsonism-hyperpyrexia and dyskinesia- age capacity and short levodopa half-life2. The ON-OFF phe- hyperpyrexia syndromes nomenon and unpredictable OFFs describe the occurrence Parkinsonism-hyperpyrexia syndrome (PHS), also known of sudden and disabling OFF periods with no clear relation- as neuroleptic malignant-like syndrome, is a rare complica- ship with timing of levodopa intake. The “delayed ON” (in- tion of PD6, characterized by hyperthermia, autonomic dys- creased time latencies from dose intake to the start-up of an function, altered consciousness, severe rigidity and elevat- ON period) and “no-ON” phenomenon (complete failure of a ed serum creatine kinase (CK) levels. PHS may be triggered levodopa dose to exert an ON response) are typically related by infections, reduction in dopaminergic drug dosage, hot to impaired absorption of oral levodopa2. weather or dehydration. Potentially life-threatening compli- In rare instances, MF can represent a MD emergency, es- cations include deep venous thrombosis and pulmonary em- pecially in the case of frequent and distressful ON-OFF pe- bolism, aspiration pneumonia and renal failure. Treatment is riods. Typically, management involves various combined based on intravenous fluids infusion, antithermics and use approaches, such as administration of small multiple daily of levodopa and dopamine agonists. Occasionally, dantrolene doses of levodopa, controlled release, dispersible and solu- may be necessary. ble levodopa formulations, subcutaneous dopamine ago- Another MD emergency related to PD, recently desig- nists, MAO-B and COMT inhibitors, and surgical approach- nated dyskinesia-hyperpyrexia syndrome7, consists of se- es. Administration of crushed levodopa tablets may also be vere dyskinesias (dyskinetic status), leading to muscle ex- helpful in certain circumstances2. haustion, rhabdomyolysis, hyperthermia and confusion. The “super OFF” phenomenon is a rare complication This complication shares some of the clinical characteris- found in patients with PD defined as an increase in motor tics of PHS, but differs in those dyskinesias – instead of ri- disability scores in comparison to basal (OFF period) mo- gidity, dominates the clinical picture. Additionally, the dys- tor scores described in those experiencing MF3. As the de- kinesia-hyperpyrexia syndrome, contrarily to PHS, should nomination implies, it means that patients affected by this be treated by reducing the dosage of dopaminergic drugs, phenomenon have the sensation of being more symptom- particularly dopamine agonists. atic from most parkinsonian motor standpoints than their usual OFF levodopa dose status. The super OFF state can be c. Acute parkinsonism divided into a beginning-of-dose and an end-of-dose inhibi- Acute severe de novo parkinsonism is an uncommon form tory effect, depending on whether it occurs before or after of parkinsonism that occurs over hours or days. In general, levodopa has reached a therapeutic plasma concentration4. the most frequent cause is exposure to dopamine-blocking The phenomenon is often very distressing and disabling, agents, such as neuroleptics and anti-emetics, however other lasting from minutes to hours. The pathophysiology of su- rare causes have been described, including hypoxic-ischemic per OFF episodes is not entirely clear, but is probably linked encephalopathy, intoxications (organophosphate pesticide, either to presynaptic inhibition of firing and release of dop- carbon monoxide and drugs used for cancer therapy, as cyto- aminergic neurons, or an effect mediated by the preferential sine arabinoside, cyclophosphamide