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Duraisamy J, P.R R, K.S T, Rajkumar A, Denishya S. Paroxysmal Sympathetic Hyperactivity in Acquired Brain Injury: A Case Series. Iran J Neurosurg 2022; 8 (1) : 19
URL: http://irjns.org/article-1-309-en.html
1- MBBS, Consultant Neurosurgeon, Department of Neurosurgery, PSG Institute of Medical Sciences and Research, Coimbatore, India , jaidurai2011@gmail.com
2- MBBS, Consultant Neurosurgeon, Department of Neurosurgery, PSG Institute of Medical Sciences and Research, Coimbatore, India
3- MBBS, Senior Consultant Neurosurgeon, Department of Neurosurgery, PSG Institute of Medical Sciences and Research, Coimbatore, India
4- Raja Muthiah Medical College, Annamalai University, Chidambaram, India
5- MBBS, Junior Resident, Department of Neurosurgery, PSG Institute of Medical Sciences and Research, Coimbatore, India
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1. Background and Importance
In 1954, Penfield and Jasper first reported a series of cases with episodic sympathetic hyperactivity which led to hyperthermia, hypertension, tachycardia, tachypnea, diaphoresis, and dystonia following non-noxious stimuli in the survivors of severe traumatic brain injury [123]. Subsequently, this condition gained more attention because of the increased mortality and morbidity, need for prolonged intensive care, and interference with rehabilitation [4]. Different sets of diagnostic criteria were proposed and more than 25 nomenclatures were given, including autonomic dysfunction syndrome, sympathetic storming, hypothalamic midbrain deregulation syndrome, acute midbrain syndrome, and diencephalic epilepsy [4]. In 2014, Baguley et al. proposed the term paroxysmal sympathetic hyperactivity (PSH) to identify the condition characterized by simultaneous, paroxysmal, and transient increases in sympathetic activities [1,  5]. It is an under-recognized condition with a diagnostic pitfall, especially in ICU because of the high prevalence of concomitant diseases that mimic PSH.
Potential mechanisms of paroxysmal sympathetic hyperactivity 
The simple disconnection theory and the excitatory-inhibitory ratio model can explain the potential mechanisms for the development of PSH [6, 7].
1) Simple Disconnection Theory: Diffuse or focal brain injury leads to disconnection of cortical inhibitory centers (insula and cingulate gyrus) from the hypothalamus, thalamus, and brain stem which is responsible for the loss of supraspinal control of sympathetic tone leading to autonomic hyperactivity.
2) Excitatory-Inhibitory Ratio Model: Excitatory-inhibitory ratio model explains the development of PSH in 2 stages. Various cortical centers, hypothalamus, and thalamus modulate the activity of the periaqueductal gray matter located within the brain stem. In turn, the periaqueductal gray matter provides the inhibitory drive to spinal reflex arcs, thereby maintaining the balance between excitatory and inhibitory interneurons. If there is a breach in these excitatory-inhibitory circuits, the disconnection of descending inhibition produces maladaptive dendritic arborization and the spinal circuit excitation triggers the motor response and sympathetic output following non-noxious stimuli.
Diagnosis
Since there is no radiological investigation available to detect PSH, the diagnosis is attained by proper clinical examination and grading of the cardinal features [2, 8]. The paroxysmal sympathetic hyperactivity-assessment measure (PSH-AM) is the scale adopted by many centers to confirm PSH [2, 9]. It is calculated by using two constructs, namely the clinical severity scale which measures the intensity of the cardinal features of PSH, and the diagnosis likelihood tool which is based on the presence of specific features estimating the possibility of PSH [3, 9]. This scoring system (Table 1) helps to diagnose the disease and to grade its severity [8, 10].


Treatment
Management strategies include medical treatment or surgical intervention for primary disease along with a combination of drugs to overcome sympathetic hyperactivity. Opioids, beta-blockers, gabapentin, bromocriptine, benzodiazepines, and central α-agonists are the widely used medications to alleviate sympathetic responses [11, 12]. Hypertension and tachycardia respond well to beta-blockers, propofol, and benzodiazepines. Opioids are used to control tachypnea. Diaphoresis responds well to beta blockers. Meanwhile, bromocriptine and acetaminophen reduce hyperthermic episodes. Dystonic postures can be treated with baclofen and gabapentin [11, 12, 13]. Supportive measures, including early tracheostomy, gentle suctioning and sponging, increasing the calorie intake, maintaining hydration, and early implementation of enteral feeding ensure early recovery [13, 14].
2. Case Presentation
This was a retrospective analysis study on the data of patients admitted to the neurosurgery ICU at PSG Institute of Medical Sciences and Research, Coimbatore, India, from October 2020 to February 2022. The patients admitted with acquired brain injury (trauma, stroke, and meningoencephalitis) who stayed in the ICU for more than 7 days with a poor Glasgow Coma Scale (GCS<10) were considered a standard and reliable factor to be included in the study. PSH-AM scale was the scoring system adopted in the study to diagnose PSH. The patients admitted with metabolic encephalopathy and acquired brain injury who had less than one week of staying in the ICU and a GCS score of more than 10 were excluded from the study. 
3. Discussion
Of the 412 patients admitted to the neurosurgery ICU with acquired brain injury, 11 patients (2.6%) were diagnosed with PSH. Findings of all 11 patients are provided in Table 2, Table 3, and Table 4.






In this study, trauma (72%) was the leading cause of the development of PSH. A minority of the patients with stroke (18%) and meningoencephalitis (9%) also developed PSH. Individuals with ages less than 40 years (63%) were more vulnerable to developing PSH. All patients (100%) developed PSH after 1 or 2 weeks of acquired brain injury. About 72% of patients had features of PSH for more than 2 weeks and 80% of patients developed episodes of PSH as paroxysms. As discussed in the potential mechanisms of PSH, most of the patients in the study had pathology in any of the following regions, including the epithalamus, thalamus, subthalamus, hypothalamus, brainstem, or corpus callosum. Patients with comorbidities, such as diabetes, hypertension, and coronary artery disease had poor outcomes. Patients with associated injuries in the chest, abdomen, spine, or long bone had a high chance to develop PSH with poor outcomes. All patients (100%) had developed at least two PSH episodes per day even with mild stimuli, such as positioning, suctioning, and sponging that persisted for at least 3 consecutive days. Tachycardia and tachypnoea were the most common symptoms noted in all patients. Dystonic posturing was the least persistent feature noted in less than 45% of patients and about 80% to 90% of patients presented with hypertension, hyperthermia, and diaphoresis. About 45% of patients developed 2 or more symptoms simultaneously which responded well to the medications used to reduce sympathetic activity. Decompressive craniectomy or ventriculoperitoneal shunt (depending upon the primary pathology) was required for 45% of the patients who were diagnosed with PSH, and 91% of the patients needed a tracheostomy to wean off from the mechanical ventilation. The hospitalization period increased by at least 14 days in all PSH patients compared with other brain injury cases without PSH. The functional outcome of PSH patients was assessed by the Glasgow Outcome Score (GOS) which was less than 3 in 72% of PSH patients at the time of discharge. It was on the poorer side compared with non-PSH patients.
4. Conclusion
PSH is a condition characterized by hyperthermia, hypertension, tachycardia, tachypnea, diaphoresis, and dystonia following non-noxious stimuli in acquired brain injury. The PSH-AM is a method adopted by many centers to confirm PSH. Management strategies, including the treatment of primary disease with a combination of medications to overcome sympathetic hyperactivity and early tracheostomy in ventilator-dependent patients, helped in achieving better recovery for the affected patients. It is the responsibility of the treating doctor to make the bystander understand the severity of PSH and the morbidity associated with it.

Ethical Considerations
Compliance with ethical guidelines

Written informed consent was obtained from all patients. The Institutional Ethics Committee approval was obtained and this study complies with ethical guidelines.

Funding
This research did not receive any grant from funding agencies in the public, commercial, or non-profit sectors.

Authors' contributions
Conception, design, data collection, data Analysis and Interpretation: Jayaprakash Duraisamy; Drafting the manuscript, critically revising, reviewing the final version of the manuscript and final version approval: All authors.

Conflict of interest
The authors declare that they have no conflict of interest to disclose.

Acknowledgements
The authors would like to thank Kavipriya Dev Sabareeshwara Kumar and Ruveka Velusamy, for their help in finalizing the project. The authors thank the faculty and management of PSGIMSR for their support.


​​​​​​References
  1. Bower RS, Sunnarborg R, Rabinstein AA, Wijdicks EFM. Paroxysmal sympathetic hyperactivity after traumatic brain injury. Neurocrit Care. 2010; 13:233-4. [DOI:10.1007/s12028-010-9381-y] [PMID]
  2. Baguley IJ, Perkes IE, Fernandez-Ortega JF, Rabinstein AA, Dolce G, Hendricks HT, et al. Paroxysmal sympathetic hyperactivity after acquired brain injury: Consensus on conceptual definition, nomenclature, and diagnostic criteria. Journal of Neurotrauma. 2014; 31(17):1515-20. [DOI:10.1089/neu.2013.3301] [PMID]
  3. Wang VY, Manley G. Recognition of paroxysmal autonomic instability with dystonia (PAID) in a patient with traumatic brain injury. The Journal of Trauma. 2008; 64(2):500-2.  [DOI:10.1097/TA.0b013e31804a5738] [PMID]
  4. Penfield W, Jasper H. Autonomic seizures. In: Penfield W, Jasper H, editors. Epilepsy and the functional anatomy of the human brain. London: Churchill; 1954. [link]
  5. Hinson HE, Sheth KN. Manifestations of the hyperadrenergic state after acute brain injury. Current Opinion in Critical Care. 2012; 18(2):139-45. [DOI:10.1097/MCC.0b013e3283513290] [PMID]
  6. Shields RW Jr. Functional anatomy of the autonomic nervous system. Journal of Clinical Neurophysiology. 1993; 10(1):2-13. [DOI:10.1097/00004691-199301000-00002] [PMID]
  7. Meyer KS. Understanding paroxysmal sympathetic hyperactivity after traumatic brain injury. Surgical Neurology International. 2014; 5(Suppl 13):S490-2. [DOI:10.4103/2152-7806.144632] [PMID] [PMCID]
  8. Perkes IE, Menon DK, Nott MT, Baguley IJ. Paroxysmal sympathetic hyperactivity after acquired brain injury: A review of diagnostic criteria. Brain Injury. 2011; 25:925-32. [DOI:10.3109/02699052.2011.589797] [PMID]
  9. Hughes JD, Rabinstein AA. Early diagnosis of paroxysmal sympathetic hyperactivity in the ICU. Neurocritical Care. 2014; 20(3):454-9. [DOI:10.1007/s12028-013-9877-3] [PMID]
  10. Hughes EG, Peng X, Gleichman AJ, Lai M, Zhou L, Tsou R, et al. Cellular and synaptic mechanisms of anti-NMDA receptor encephalitis. Journal of Neuroscience. 2010; 30(17):5866-75. [DOI:10.1523/JNEUROSCI.0167-10.2010] [PMID] [PMCID]
  11. Rabinstein AA, Benarroch EE. Treatment of paroxysmal sympathetic hyperactivity. Current Treatment Options in Neurology. 2008; 10(2):151-7. [DOI:10.1007/s11940-008-0016-y] [PMID]
  12. Samuel S, Allison TA, Lee K, Choi HA. Pharmacologic management of paroxysmal sympathetic hyperactivity after brain injury. Journal of Neuroscience Nursing. 2016; 48(2):82-9.[DOI:10.1097/JNN.0000000000000207] [PMID]
  13. Feng Y, Zheng X, Fang Z. Treatment progress of paroxysmal sympathetic hyperactivity after acquired brain injury. Pediatric Neurosurgery. 2014; 15:301-9. [DOI:10.1159/000439282] [PMID]
  14. Caldwell SB, Smith D, Wilson FC. Impact of paroxysmal sympathetic hyperactivity on nutrition management after brain injury: A case series. Brain Injury. 2014; 28(3):370-3. [DOI:10.3109/02699052.2013.865265] [PMID]
Type of Study: Case Series | Subject: Neuroscience

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