Neurostorm: Modern understanding and nomenclature. Mitch Stanek RN, CBIS Charge Nurse/Infection Preventionist On With Life

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1 Neurostorm: Modern understanding and nomenclature Mitch Stanek RN, CBIS Charge Nurse/Infection Preventionist On With Life

2 Despite its significant clinical impact, the scientific literature on this syndrome is confusing; there is no consensus on nomenclature, etiological information for diagnoses preceding the condition is poorly understood, and the evidence base underpinning our knowledge of the pathophysiology and management strategies is largely anecdotal. -Annals of Neurology, August 2010 This is a common syndrome and failure to recognize it is associated with increased morbidity and mortality, higher health costs, longer hospitalizations and poorer outcomes. -European Journal of Case Reports in Internal Medicine, January 2017 The literature indicates that the understanding of PSH following TBI is in its infancy. The majority of information is based on small case series. Conclusions: Nurses play a critical role in the identification of at-risk patients,, symptom complexes, and in the education of family. Surgical Neurology International (Nov. 2014)

3 A Rose by any Other Name Term Initial use Total No. citations autonomic storms/storming Epileptogenic tonic decerebrate seizures autonomic seizures with decerebrate seizures diencephalic (autonomic) seizures/epilepsy/fits autonomic sympathetic seizures Structural brain stem attack hypothalamic-midbrain dysregulation syndrome acute midbrain syndrome acute hypothalamic instability hypothalamic storm Clinically Descriptive paroxysmal hypertension associated with diaphoresis (central) autonomic dysfunction/syndrome hyperthermic syndrome neuroleptic malignant-like syndrome autonomic hyperactivity dysautonomia (central) autonomic instability hyperpyrexia with sustained muscle contraction autonomic dysregulation paroxysmal sympathetic storms sympathetic storms/storming autonomic disorder (and spasticity) neuro storm paroxysmal autonomic instability with dystonia hyperadrenergic state paroxysmal autonomic instability dysautonomic crises arc de cercle and dysautonomia storming: transient autonomic dysfunction

4 Adding to confusion Only 27 of 81 papers had any diagnostic criteria Of the 27 there were 9 unique versions No consensus on: Nomenclature Treatment common systems The lack of clarity is believed to have hindered research efforts and may have caused those affected to suffer.

5 In 2014 a steering committee produced: Conceptual Definition Common Name Diagnostic Criteria Diagnostic tool

6 Paroxysmal Sympathetic Hyperactivity A syndrome, recognized in a subgroup of survivors of severe acquired brain injury, of simultaneous, paroxysmal transient increases in sympathetic [elevated heart rate, blood pressure, respiratory rate, temperature, sweating] and motor [posturing] activity.

7 Causes of PSH Overview Found in 7-33% of ICU patients post brain injury Common Causes Traumatic Brain injury (79.4%) Hypoxic brain injury (9.7%) Stroke(5.4%) Risk factors Severity of the initial brain injury Younger age Male gender Traumatic Brain Injury Hypoxic Injury Stroke Hydrocephalus Other

8 Impact Difficult Recovery Longer length of stay 25% body weight loss Secondary conditions heterotopic ossification Ischemia Immunosuppression Dehydration Kidney injury Cardiac damage Cerebral edema Intracranial bleeding

9 Episodes occur after stimulus Suctioning Turning Bathing Physical exam Etc. Signs Increased Heart Rate, Increased Respiratory Rate, Increased Systolic Blood Pressure, Sweating Posturing Signs

10 Pathophysiology Limited understanding Caused by damaged connections within the brain. Unneeded and overstated activation of the sympathetic nervous system Does not appear to have connected to damage to a specific area of the brain Variable symptoms and severity

11 Diagnostic Criteria 1. That the clinical features seem to happen all at once 2. The clinical features are episodic in nature 3. The episodes occur after being subjected to non-painful stimulus 4. No parasympathetic clinical features during an episode, including: 1. Slow heart rate (bradycardia) 2. Constriction of bronchial tubes in the lungs 3. constriction of pupils 4. relaxation of muscles 5. features persist for 3 or more days 6. features persist two weeks after initial injury 7. features persist despite treatment of alternative differential diagnoses 8. medication administered to decrease sympathetic features 9. 2 or more episodes daily 10. lack of alternative explanations 11. occurs after acquired brain injury

12 Diagnostic Tool Diagnosis must be one of exclusion Two separate diagnostic aides Diagnosis Likelihood Tool Clinical Feature Scale To be used at least daily at standardized time. Value of both tools added to gauge probability of PSH

13 Diagnosis Likelihood Tool Few patients will exhibit all features Diagnostic items are non exclusionary

14 Clinical feature scale

15 Therapeutic Management Non Pharmaceutical interventions Focus on decreasing pain and neural stimulus. Monitor for untreated conditions Decrease external Stimuli Touching Turning Smells Limit visitation Minimize exams Cluster cares Increase fluids and caloric intake when appropriate

16 Pharmacological Treatment Optimize the outcome while minimizing side effects. Ideal approach Short acting medications Appropriate regimen Avoid non effective medications Management using combination of medicines, focusing on the immediate control of break through episodes and prevention of symptoms.

17 Nonselective Beta blockers Treats Hypertension Tachycardia Diaphoresis Dystonic Posturing Propanolol-first line Decreases hyperthermia response Improves mortality rates when compared to other Betablockers (3% VS 15%) Labatolol 2 nd line Additional effect on alpha receptors Metoprol-Selective betablocker Ineffective

18 A2 agonist Clonidine - 2 nd line Manages Hypertension Tachycardia Ineffective as monotherapy Dexmedetomidine manages hypertension Tachycardia Seditive Used in ICU May Shorten hospital stay

19 Opioid receptor agonist Morphine Manages Pain Controls possible episodic triggers Tachycardia Hypertension Converted to oxycodone for maintenance therapy

20 Gaba Receptor Agonist Manages Spasms Improves mobility Associated pain Clonus Baclofen Intrathecal is the most effective Intraventricular catheter as effective High doses of oral baclofen has been found ineffective for treating spasticity in TBI Gabapentin Similar to baclofen Effective for breakthrough episodes

21 Gaba Receptor Agonist Benzodiazepines Effective as other Gaba agonists Long term use linked with worsening neurological function Needs tapering Short term preferred for break through episodes

22 Dopamine Receptor antagonists: WARNING Haloperidol- leads to exacerbation of cognitive deficit psychosis Neuroleptic mslignant syndrome Masks symptoms of PSH