Movement Disorder Emergencies. Anne E. A. Constantino, MD Attending Neurologist Holy Cross Hospital Silver Spring, MD June 28, 2014

Transcription

1 Movement Disorder Emergencies Anne E. A. Constantino, MD Attending Neurologist Holy Cross Hospital Silver Spring, MD June 28, 2014

2 Objectives Provide a working definition of movement disorder emergencies Classify the movement disorder emergency according to phenomenology Clinical presentation and treatment of diseases presenting with: a. Rigidity b. Dystonia c. Hyperkinetic movements Tremor, Chorea and Ballism

3 What are Movement Disorder Emergencies? A movement disorder emergency (MDE) has been defined by Fahn and Frucht1 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.

4 Infectious Tetanus (Rigidity remains between spasms) Rabies Drug-induced Neuroleptic malignant syndrome Parkinsonism hyperpyrexia syndrome Serotonin syndrome Toxic Strychnine (Muscle tone normal between spasms) Metabolic Hypocalcemia Inherited Hyperekplexia Malignant hyperthermia Autoimmune Stiff man syndrome Psychiatric Lethal catatonia

5 Neuroleptic Malignant Syndrome Clinical Presentation: Triad: Generalized rigidity accompanied by akinesia which ma lead to rhabomyolysis and renal failure, swallowing disturbances and pneumonia, Dysautonomia with fever, sweating, tachypnea, tachycardia, labile blood pressure Alteration in mental status. Cause: Neuroleptics such as Haldol (less with atypical neuroleptics), Metoclopramide, prochlorprazine (Compazinez), Lithium, Treatment: Cessation of Neuroleptics, Bromocriptine, Dantrolene

6 Serotonin Syndrome Clinical Presentation syndrome of severe rigidity, dysautonomia, alteration in mental status. May also present with myoclonus, hyperreflexia Treatment Stop serotonin agent Benzodiazepines Hydration and cooling Antihistaminics within hours More rapid onset (hours to days) Complications: rhabdomyolysis, myoglobinuria, renal failure, severe metabolic acidosis, disseminated intravascular coagulation, and adult respiratory distress syndrome.

7 Inhibitors of serotonin reuptake SSRI, tricyclic antidepressants, dextromethorphan, dexamphetamine, cocaine, meperidine, opiates (except morphine) Inhibitors of serotonin metabolism MAO-B inhibitors (selegiline), MAO inhibitor antidepressants Agents increasing serotonin synthesis L- tryptophan Enhancers of serotonin release MDMA (ecstasy), amphetamines, cocaine, fenfluramine Serotonin agonists Sumatriptan, ergotamines, buspirone Nonspecific enhancers of serotonin activity Lithium, ECT

8 Malignant Hyperthermia CLINICAL MANIFESTATIONS Rapid onset of fever, fluctuations in blood pressure, hyperkalemia, metabolic acidosis, rapid onset of fever, fluctuations in blood pressure, CAUSE/TRIGGERS: Anesthetics including inhalational agents and depolarizing muscle relaxants CLINICAL SYNDROME: Results from uncontrolled calcium flux across skeletal muscle membrane. In over 50% of families, GENETIC LINKAGE: Autosomal dominant trait to a gene encoding the skeletal muscle ryanodine receptor. TREATMENT: Muscle relaxant dantrolene is highly effective and needs to be combined with discontinuationof the triggering agents and correction of acidosisand electrolyte abnormalities. Mortality is around 10%.29

9 HYPOCALCEMIA PATHOPHYSIOLOGY: Tetanic muscle spasms can develop acutely secondary to hypocalcemia, the severity of which varies with the magnitude and rapidity of the fall in serum calcium. CAUSES: thyroid or parathyroid surgery, rhabdomyolysis, hypomagnesemia, malignancy,chronic renal failure, pancreatitis, and septic shock. There are also reports of hypocalcemia and tetany developing after plasma exchange. DIAGNOSIS: Prolongation of the QT interval with attendant risk of arrhythmia, laryngospasm. TREATMENT: Intravenous Calcium

10 Movement Disorder Emergencies Causing Parkinsonism Vascular and structural: Lesions in the basal ganglia, midbrain, Hydrocephalus Infectious : Encephalitis lethargica, viral (Japanese B encephalitis), Mycoplasma Drug induced Parkinsonism hyperpyrexia syndrome Caused by dopaminergic drug withdrawal Amphotericin B Toxic : Carbon Monoxide, Methanol, Cyanide, Organophosphate poisoning, MPTP Metabolic : Central pontine myelinosis usually associated with encephalopathy Inherited: Rapid-onset dystonia parkinsonism Psychiatric: Neuroleptic-induced

11 Parkinsonism Hyperpyrexia Syndrome CAUSE: Lowering the dose or withdrawing dopaminergic drugs in patients with Parkinson s disease Withdrawal of nondopaminergic drugs to include amantadine, tolcapone TREATMENT Reinstituting dopaminergic therapy Supportive care (takes weeks) Drugs given through NGT Parenteral apomorphine and anti nausea medications

12 DISORDERS PRESENTING WITH DYSTONIA Acute Dystonia Most Common Cause: Drug induced METOCLOPRAMIDE Occurs as early as 24 hours after taking the drug Presents with akathisia as well and some patients, especially older individuals develop tardive dyskinesia Laryngeal Spasm in Multiple System Atrophy Acute Baclofen Withdrawal

13 DISORDERS PRESENTING WITH HYPERKINETIC DISORDERS ACUTE HEMICHOREA AND BALLISM Causes: Vascular Post infectious streptococcus Auto immune diseases Anti Phospholipid Antibody Syndrome Treatment: Consider Reserpine, Benzodiazepines, ACUTE DYSKINESIA Main cause is high doses of levodopa Treatment: Lower dose of levodopa If lowering dose of levodopa is not possible, consider Amantadine

14 MYOCLONUS Movement disorder emergencies causing myoclonus Metabolic: Negative myoclonus/asterixis Infective: Focal encephalitis Drug induced: Serotoninergic drugs Opiate Induced May respond to naloxone or benzodiazepines Opiate withdrawal Responds to benzodiazepines and not naloxone Lithium Cortical action myoclonus Tricyclic antidepressants Especially serotonin syndrome Imipenem, cefuroxime Epilepsia partialis continua: Subdural haemorrhage, cortical sinus thrombosis, Anti-Hu paraneoplastic encephali

15 Acute Akathisia Occurs in susceptible patients exposed to dopamine receptor blockers or dopamine depletors Feeling of restlessness Usually self limiting if the drug implicated is withdrawn Other drug treatments: Anticholinergics Vitamin B6 low dose Remeron

16 Hallucinations in Patients with Parkinson s Disease Risks: Dementia Long standing disease High doses of levodopa Use of anticholinergics with levodopa Use of dopamine agonists Treatment Decrease dose of levodopa Selegiline and anticholinergics can be stopped immediately Consider Clozapine or Seroquel as an alternative Zyprexa and Risperidol worsens Parkinsonism

17 Thank you!