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 , 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 -Toxin, Viral, Other infections Neuroleptic Malignant Syndrome -Parkinsonism-hyperpyrexia syndrome -Lethal catatonia -Psychiatric symptoms in PD -Severe l-dopa -Deep brain Stimulation Related Problems

Acute Onset Parkinsonism

• Vascular/Structural • Subdural Hematoma • BG/brainstem • Hydrocephalous

• Infectious • lethargica • (HIV, PML) • Fungal Infections • Drug induced • Neuroleptics, Chemotherapy, Amphotericin • Toxin induced • CO, Methanol, Cyanide, Organophosphate • Metabolic • CPM

2 7/16/2020

Acute Parkinsonism

Video in Presentation

A young immunocompetent woman with 2 weeks history Courtesy Jose Luiz Pedroso

Brain MRI:

T2 weighted FLAIR T1 weighted (Post-gadolinium)

3 7/16/2020

Investigations: 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

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/ • 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

Video in Presentation

6 7/16/2020

A young man with Parkinsonism on levodopa

Video in Presentation

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

Video in Presentation

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 • (30%), (50%), bradykinesia (40%), , chorea

8 7/16/2020

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 • 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

Video in Presentation

• 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

Video in Presentation

Movement Disorders Emergencies-Hyperkinetic Movement Disorders

. Myoclonus-multifocal . Ballism/Chorea -Choreic storm . Dystonic storm . status, malignant vocal Cervical cord compression- disc prolapse complicating tics . DBA Induced Acute or Tardive Dystonia -Severe - Withdrawal Emergent Syndrome . Other Drug Induced Movement Disorders .

12 7/16/2020

Video in Presentation

A woman self medicating with Pepto Bismal for diarrhea

HEMIBALLISM/HEMICHOREA

Video in Presentation

A26 year old with acute onset movement disorder

13 7/16/2020

Video in Presentation

After clonazepam 1 mg tid

Biballism/Generalized Chorea

Video in Presentation

A 66 year old woman with no history of diabetes developed these movements over 2 days

14 7/16/2020

Blood sugar 430 mg/dl Serum Osmolality 320 mmol/kg

Video in Presentation

A 70 year old man with acute onset Movement Disorder

15 7/16/2020

Polycythemia Rubra Vera

Video in Presentation

Hb was 18 gm/l-here he is after venesection

Dystonic Storm

Video in Presentation

16 7/16/2020

Management

• Supportive care • Anticholinergics, dopamine depleters and blocking agents • Baclofen Pump • Globus pallidus deep brain stimulation

17 7/16/2020

Movement Disorders Emergencies Paroxysmal Dyskinesias

Paroxysmal Kinesigenic Dyskinesia - Idiopathic and Secondary Paroxysmal Non- Kinesigenic Dyskinesia- Idiopathic and secondary Paroxysmal Dystonia (Tonic Spasms) of TIA presenting as Psychogenic Paroxysmal Movement disorders

Video in Presentation

A 65 year old being treated for -Note eye movements- CT scan lung hilar mass-small cell CA Anti HU AB increased

18 7/16/2020

Video in Presentation

First part-tonic spasm –diagnosed MS treated with 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

Video in Presentation

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 ) • 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

20