7/16/2020
Movement Disorders Emergencies Kapil D Sethi MD FRCP MCG at Augusta University Augusta Georgia
Definition
• “…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.”
S Fahn & S Frucht, 2002
1 7/16/2020
Movement Disorders Emergencies
-Acute Parkinsonism-Toxin, Viral, Other infections Neuroleptic Malignant Syndrome -Parkinsonism-hyperpyrexia syndrome -Lethal catatonia -Psychiatric symptoms in PD -Severe l-dopa Dyskinesias -Deep brain Stimulation Related Problems
Acute Onset Parkinsonism
• Vascular/Structural • Subdural Hematoma • BG/brainstem stroke • Hydrocephalous
• Infectious • Encephalitis lethargica • Viral encephalitis (HIV, PML) • Fungal Infections • Drug induced • Neuroleptics, Chemotherapy, Amphotericin • Toxin induced • CO, Methanol, Cyanide, Organophosphate • Metabolic • CPM
2 7/16/2020
Acute Parkinsonism
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A young immunocompetent woman with 2 weeks history Courtesy Jose Luiz Pedroso
Brain MRI:
T2 weighted FLAIR T1 weighted (Post-gadolinium)
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Investigations: Cerebrospinal fluid analysis:
Lumbar puncture open pressure: 47 cmH2O 120 cells/mm³ (92% lymphocytes; 4% monocytes; 4% neutrophils) Glucose: 43 mg/dL Proteins: 70 mg/dL
• Latex agglutination to cryptococcal antigen was positive on CSF and serum • CSF culture evidenced Cryptococcus gatti
Secondary parkinsonism due to Cryptococcus gatti meningoencephalitis
PD Emergencies
129 patients; 324 incidents -Not disease related -Disease related
• Infections (21%) • Pneumonia (11%) • Falls (13%) • UTI (9%) • Fractured (4%) • Cardiovascular (20%) • Decreased mobility/dyskinesia • Angina (6%) (8%) • Heart failure (6%) • Psychiatric (8%) • Stroke/MI/Arrhythmia (2% each) • Delirium (4%) • Other • Hallucinations (3%) • Surgical (4%) • Autonomic • GI Bleed (3%) • Orthostatic Hypotension (4%) • Diarhea/vomiting/ dehydration (2%) • Constipation (2%)
From: Woodford et al Mov Disord 2005
4 7/16/2020
Video in Presentation
A 43 year old man with asymmetric young onset akinetic rigid Parkinsonismone month following the initiation of ropinirole suddenly worsened and started having “attacks” of worsening
A flurodopa PET demonstrating asymmetric reduced binding-the patient had functional attacks superimposed on PD
5 7/16/2020
Unusual or severe l-dopa dyskinesia
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6 7/16/2020
A young man with Parkinsonism on levodopa
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Brother and sister with parkinsonism and axonal neuropathy-on levodopa-POLG mutation
Neuroleptic Malignant Syndrome
• Neuroleptic- (including atypical agents) induced • Potentially fatal • Blockade of dopaminergic transmission • Rapidly progressive; peaks 72 hours • May be self-limited • Duration-variable(average 7-14 days after stopping the drug) • Mortality 5% with treatment; 20-30% without
7 7/16/2020
Neuroleptic Malignant Syndrome/Parkinsonism Hyperpyrexia Syndrome
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Neuroleptic Malignant Syndrome Core Clinical Features • Hyperthermia - peaks in 48 hours • Muscle rigidity – lead pipe, • Autonomic dysfunction • Tachypnea, dyspnea • Arrhythmia, lability of BP • Diaphoresis, pallor, urinary symptoms • Mental status change • Fluctuating alertness, agitation, lethargy, muteness, confusion, delirium, stupor
• Other motor symptoms • Dystonia (30%), tremor (50%), bradykinesia (40%), myoclonus, chorea
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Neuroleptic Malignant Syndrome Laboratory Features • Elevated CK (>90%) • Polymorphonuclear leukocytosis (75%) • Hypoferremia (95%): Acute phase reactants: • Acute phase reactants Antichymotrypsin elevated • Elevated aldolase, alk phos, AST, ALT Fibrinogen elevated • Lesser extent than CK ESR & CRP elevated • Muscle origin Interleukin-6 elevated • Hypocalcemia (50%) Albumin decreased • Hypomagnesemia (60%) Changed by day 4, normal by day 14 • Proteinuria & myoglobinuria Rosebush et al 2008
Neuroleptic Malignant Syndrome Atypical form-Premalignant? • More common with atypical antipsychotics • Insufficient evidence to support a dx of NMS • Absence or varied severity of core features: rigidity or fever • Impending NMS? • Or benign side effects – transient benign hyperthermia, hemodynamic changes from the drug • Missed diagnosis common • May represent spectrum of disease • Requires less strict diagnostic criteria.
9 7/16/2020
Neuroleptic Malignant Syndrome Treatment
• Early recognition-stop offending agent • Supportive Therapy • Correct metabolic abnormalities • Traditionally bromocriptine up to 60mg/day • Other agonists and levodopa may work • Dantrolene in severe cases • Benzodiazepines • No anticholinergics • ECT-if overlap with catatonia and diagnosis unclear
Parkinsonism Hyperpyrexia Syndrome Dyskinesia Hyperpyrexia Syndrome
• Abrupt discontinuation of Dyskinesia Hyperpyrexia syndrome Dopaminergic Medications • Drug holidays Severe levodopa dyskinesias • Patient non-compliance with fever- • Abrupt reduction or discontinuation • Neuroleptic use treated by reducing dopaminergic drugs • Motor fluctuations • Severe “Off” times • Perimenstrual- very rare • Metabolic disturbances • Dehydration, hyponatremia
10 7/16/2020 Courtesy Victor Fung Courtesy
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• 24 yo man, past h/o IVDU, Hep C positive, UL tremor. On methadone, mirtazapine 30mg/day 18/12, fluoxetine 20mg/day 6/12. 2 days of increasing confusion, anxiety, “jittery”. CK 45
11 7/16/2020
Catatonia
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Movement Disorders Emergencies-Hyperkinetic Movement Disorders
. Myoclonus-multifocal . Ballism/Chorea -Choreic storm . Dystonic storm . Tic status, malignant vocal tics Cervical cord compression- disc prolapse complicating tics . DBA Induced Acute or Tardive Dystonia -Severe tardive dyskinesia - Withdrawal Emergent Syndrome . Other Drug Induced Movement Disorders . Serotonin syndrome
12 7/16/2020
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A woman self medicating with Pepto Bismal for diarrhea
HEMIBALLISM/HEMICHOREA
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A26 year old with acute onset movement disorder
13 7/16/2020
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After clonazepam 1 mg tid
Biballism/Generalized Chorea
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A 66 year old woman with no history of diabetes developed these movements over 2 days
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Blood sugar 430 mg/dl Serum Osmolality 320 mmol/kg
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A 70 year old man with acute onset Movement Disorder
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Polycythemia Rubra Vera
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Hb was 18 gm/l-here he is after venesection
Dystonic Storm
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16 7/16/2020
Management
• Supportive care • Anticholinergics, dopamine depleters and blocking agents • Baclofen Pump • Globus pallidus deep brain stimulation
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Movement Disorders Emergencies Paroxysmal Dyskinesias
Paroxysmal Kinesigenic Dyskinesia - Idiopathic and Secondary Paroxysmal Non- Kinesigenic Dyskinesia- Idiopathic and secondary Paroxysmal Dystonia (Tonic Spasms) of Demyelinating Disease TIA presenting as Paroxysmal dyskinesia Psychogenic Paroxysmal Movement disorders
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A 65 year old being treated for status epilepticus-Note eye movements- CT scan lung hilar mass-small cell CA Anti HU AB increased
18 7/16/2020
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First part-tonic spasm –diagnosed MS treated with carbamazepine resolved Second part psychogenic
Stereotypy Catatonia and Other Movement Disorders • NMDA Receptor Mediated Encephalitis • Drug Induced Movement Disorders • Severe Augmentation of RLS
19 7/16/2020
Drug Induced Acute Dystonia
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A 28 year old man after two tablets of prochlorperazine
Conclusions
• Movement disorders emergencies are not very common (as compared to other sub-specialities such as stroke and epilepsy) • However, these can be severe and in some cases life threatening • Prompt recognition and therapy • Careful history (especially focusing on medications and concomitant illnesses) • Involve physicians from other specialties
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