Dystonic Storm

Ayesha Hameed (1) Tasneem Kousar (2),  Adeel Memon (3)
1 Fatima Jinnah Medical College, Pakistan
2 Foundation University Medical College, Pakistan
3 Dow Medical College, Pakistan


Outline
  • Introduction
  • Clinical features
Table 1: Signs and Symptoms
Table 2: Lab Findings
  • Differential Diagnosis
Table 3: Differential Diagnosis of Dystonic Storm
  • Triggers
Table 4: Triggers of Dystonic Storm -Medication Change
  • Clinical Course of Dystonic Storm
Figure 1: Clinical Course of Dystonic Storm Patient
  • Management
Management in first 24 hours
Management in 2-4 weeks
Supportive Treatment
Invasive Therapies
Deep Brain Stimulation
Intrathecal Baclofen
Pallidotomy
Figure 2: Management in first 24 hours
Table 5: Management in next 2-4 weeks
  • Complications
  • Bibliography

Introduction

  • Also known as Status Dystonicus
  • A rare life-threatening movement disorder emergency, characterized by marked exacerbations of dystonia that requires immediate intervention and ICU admission

Clinical Features

  • It usually occurs in patients with severe or poorly controlled dystonia.
  • In addition to the development of increasingly frequent and severe episodes of generalized dystonia, signs, and symptoms of Dystonic Storm are included in Table 1.

Signs and Symptoms of Dystonic Storm

  • Fever
  • Tachypnea
  • Sweating
  • Dysarthria
  • Respiratory failure
  • Tachycardia
  • Hypertension
  • Autonomic instability
  • Dysphagia
  • Pain

Lab Findings

Leukocytosis
High C-reactive protein
Elevated serum CK
Myoglobinemia

Differential Diagnosis

  • There are several conditions which present with fever, elevated serum CK, with or without muscle rigidity and thus resembling Dystonic Storm.
  • As compared to other conditions, dystonic storm is usually present in the pediatric population and develops quickly over hours to several days. Triggers may or may not be present.

Table 3: Differential Diagnosis of Dystonic Storm

Differential Diagnosis of Dystonic Storm
Entity
Time course
Movement disorder phenomenology
Other Neuro Signs
Altered Mental Status
Autonomic instability
Hours-Days
Dystonia +/− chorea
-
-
+
Hours-Days
Chorea
-
-
-
Acute
Dystonia
Oculogyria
-
+/-
Days-Weeks
Parkinsonism
-
+
+
Hours-Days
Myoclonus
-
+
+
Hours-Days
Parkinsonism
-
+
+
Acute
-
Psychosis
+
+/-
Acute
Myoclonus
Psychosis
+
+
Days-Weeks
Myoclonus
Psychosis
+
+
 

Triggers and Clinical Course Triggers

Triggers

  • Dystonic Storm is usually triggered, however, one-third of events are unprovoked.
  • The main triggers are infection and change in medication (Table 4)
  • Less common triggers are trauma, surgical procedures, anaesthesia, ‘metabolic disorder’ decompensation, stress, pain, gastro-oesophageal reflux disease, constipation, and puberty-related deterioration in Cerebral Palsy.
🤦‍♀️
Triggers of Dystonic Storm -Medication Change
ITB withdrawal
Reduction/withdrawal of other dystonia medications
Initiation of D-penicillamine or Zinc in Wilson Disease
Introduction of Clonazepam
Zolpidem discontinuation in Parkinson’s disease

Clinical Course of Dystonic Storm

  • Dystonic Storm usually appears after weeks or months in the patients who have already been diagnosed with Dystonia
  • In some patients, a prodrome of status dystonicus may be present where dystonia is worse than baseline but has not become as severe as in true storm (Figure 1)
  • The true storm usually lasts 2-4 weeks with slow recovery
  • The mortality is 10% even with treatment
  • Some patients are prone to relapses and patients with a history of Dystonic Storm should be monitored for recurrence

Figure 1: Typical Clinical Course of Dystonic Storm Patient

 
notion image
Source: Termsarasab, Pichet, and Steven J. Frucht. “Dystonic Storm: A Practical Clinical and Video Review.” Journal of clinical movement disorders 4 (2017): 10. Web. https://clinicalmovementdisorders.biomedcentral.com/articles/10.1186/s40734-017-0057-z

Management

  • Management must be initiated immediately
  • Patients should be admitted to ICU
  • The initial stabilization measures include intubation, mechanical ventilation,fluid resuscitation, antibiotics, nasogastric or parenteral nutrition and antipyretics

Management in first 24 hours

  • Goal during the first 24 hours
    • Identify and treat triggers such as infection or medications
    • Evaluate if the patient requires Globus Pallidus Interna (GPi) Deep Brain Stimulation or ITB therapy (Figure 2)

Management in 2-4 weeks

  • The usual list of medical treatment for the next 2-4 weeks is listed in table 5
  • The anti-dystonic drugs are used in combination
    • Most successful combination include an anticholinergic (trihexyphenidyl), a dopamine blocker (haloperidol or pimozide), and tetrabenazine

Table 5: Management in next 2-4 weeks

Management in next 2-4 weeks
Dystonic Specific treatment
Supportive Treatment
Ist line: IV midazolam
2nd Line: Propofol
3rd Line: Non-depolarizing neuromuscular blockers or Barbiturates
Source: Termsarasab, Pichet, and Steven J. Frucht. “Dystonic Storm: A Practical Clinical and Video Review.” Journal of clinical movement disorders 4 (2017): 10. Web. https://clinicalmovementdisorders.biomedcentral.com/articles/10.1186/s40734-017-0057-z

Supportive Treatment

  • Includes airway protection, sedation and pain control
  • Anesthetic agents such as IV midazolam is generally chosen as a first choice i.e. because of its muscle relaxant effect, rapid onset of action, and short half-life
  • For refractory symptoms, propofol is considered as a second line
  • Third line agents are pancuronium and barbiturates which are non-depolarizing paralytic agents.
  • To prevent dependence or tolerance, attempts to reduce sedative and anesthetics drugs should be made

Invasive therapies

Intrathecal baclofen

  • ITB has been used in some patients with refractory status dystonicus
  • The risks of using ITB are over-dosage, withdrawal syndrome and migration or breakage of catheter

Deep brain stimulation

  • In majority of patients DBS has been an effective treatment
  • The anatomical site of choice is globus pallidus interna (bilaterally)
  • The effects are usually evident within days or weeks
  • It is however challenging to operate on patients with status dystonicus due to higher rate of hardware and other complications present in these patients
 

Pallidotomy and thalamotomy

  • Pallidotomy is rarely used unless DBS is not available

Figure 2: Management in first 24 hours

notion image
Source: Termsarasab, Pichet, and Steven J. Frucht. “Dystonic Storm: A Practical Clinical and Video Review.” Journal of clinical movement disorders 4 (2017): 10. Web. https://clinicalmovementdisorders.biomedcentral.com/articles/10.1186/s40734-017-0057-z

Complications

  • Respiratory compromise/failure, rhabdomyolysis and acute renal failure due to severe generalized muscle spasms
  • Secondary complications include dysphagia, thrombosis, injuries, fractures and sepsis

Further Reading

Termsarasab, P., & Frucht, S. J. (2017). Dystonic storm: a practical clinical and video review. Journal of Clinical Movement Disorders, 4, 10. https://doi.org/10.1186/s40734-017-0057-z

References

  • Termsarasab, P., & Frucht, S. J. (2017). Dystonic storm: A practical clinical and video review. Journal of Clinical Movement Disorders, 4(1). https://doi.org/10.1186/s40734-017-0057-z
  • Allen, N. M., Lin, J.-P., Lynch, T., & King, M. D. (2013). Status dystonicus: A practice guide. Developmental Medicine & Child Neurology, 56(2), 105–112. https://doi.org/10.1111/dmcn.12339
  • R, H. (2021). Intermittent cerebral ischemia as a cause of dystonic storms in hypermobile Ehlers-Danlos syndrome with upper cervical instability, and Prolotherapy as successful treatment: 4 case series. Clinical and Medical Case Reports, 7(07).
  • Mayo Foundation for Medical Education and Research. (2020, April 25). Dystonia. Mayo Clinic.https://www.mayoclinic.org/diseases-conditions/dystonia/symptoms-causes/syc-20350480.

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