Paroxysmal sympathetic hyperexcitation syndrome caused by ventriculoperitoneal shunt pressure-regulation in post-traumatic hydrocephalus: a case report

Li-Jun Yang; Xin-Wei Tang; Hai-Qing Li; Wang-Huan Dun; Wen-Ke Fan; Hong-Yu Xie; Nian-Hong Wang; Jun-Fa Wu; Yi Wu

doi:10.4103/2773-2398.356524

CASE REPORT

Year : 2022 | Volume : 1 | Issue : 3 | Page : 139-142

Paroxysmal sympathetic hyperexcitation syndrome caused by ventriculoperitoneal shunt pressure-regulation in post-traumatic hydrocephalus: a case report

Li-Jun Yang¹, Xin-Wei Tang², Hai-Qing Li³, Wang-Huan Dun⁴, Wen-Ke Fan², Hong-Yu Xie², Nian-Hong Wang², Jun-Fa Wu², Yi Wu²
¹ Department of Rehabilitation, Huashan Hospital, Fudan University, Shanghai; Yanzhou District People's Hospital, Jining, Shandong Province, China
² Department of Rehabilitation, Huashan Hospital, Fudan University, Shanghai, China
³ Department of Radiology, Huashan Hospital, Shanghai Medical School, Fudan University, Shanghai, China
⁴ Department of Rehabilitation, Huashan Hospital, Fudan University, Shanghai; First Affiliated Hospital of Xiʼan Jiaotong University, Xiʼan, Shaanxi Province, China

Date of Submission	06-Jun-2022
Date of Decision	05-Jul-2022
Date of Acceptance	20-Jul-2022
Date of Web Publication	29-Sep-2022

Correspondence Address:
Hong-Yu Xie
Department of Rehabilitation, Huashan Hospital, Fudan University, Shanghai, China
China

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2773-2398.356524

Abstract

Paroxysmal sympathetic hyperactivity (PSH) is a rare symptom, but is difficult to manage. Here, we report a case of post-trauma PSH in a young male patient. The main reason for the occurrence of PSH in trauma patients may be nonnoxious or noxious stimuli. In this case, the detection of positive sympathetic parameters and heart rate variability after pressure regulation provided strong evidence for the PSH attack, thus enhancing the accuracy and reliability of early diagnosis. Clinicians should be alert to the possibility of PSH caused by rapid decline of ventricular pressure. Moreover, the appropriate regulation of ventricular pressure combined with pharmacologic interventions, rehabilitation and nutritional support may reduce and control this symptom.

Keywords: hydrocephalus; paroxysmal sympathetic hyperexcitation; traumatic brain injury; ventriculoperitoneal shunt

How to cite this article:
Yang LJ, Tang XW, Li HQ, Dun WH, Fan WK, Xie HY, Wang NH, Wu JF, Wu Y. Paroxysmal sympathetic hyperexcitation syndrome caused by ventriculoperitoneal shunt pressure-regulation in post-traumatic hydrocephalus: a case report. Brain Netw Modulation 2022;1:139-42

How to cite this URL:
Yang LJ, Tang XW, Li HQ, Dun WH, Fan WK, Xie HY, Wang NH, Wu JF, Wu Y. Paroxysmal sympathetic hyperexcitation syndrome caused by ventriculoperitoneal shunt pressure-regulation in post-traumatic hydrocephalus: a case report. Brain Netw Modulation [serial online] 2022 [cited 2023 Jun 7];1:139-42. Available from: http://www.bnmjournal.com/text.asp?2022/1/3/139/356524

Introduction

Paroxysmal sympathetic hyperactivity (PSH) often occurs after a traumatic brain injury (TBI) (Choi et al., 2013). The symptoms of PSH are more severe in patients with moderate-to-severe brain injury with impaired consciousness, thus resulting in prolonged periods of hospitalization along with the aggravation of cognitive and motor dysfunction (Totikov et al., 2019; Lucca et al., 2021; Podell et al., 2021). PSH is often induced by nonnoxious or noxious stimuli and manifests with recurrent episodes of symptoms like tachycardia, hypertension, tachypnea, fever, sweating, and increased muscle tone. Autonomic dysfunction following TBI is the predominant causative etiology (Khalid et al., 2019; Podell et al., 2022). Here, we report a case of PSH after severe craniocerebral injury in a young male patient. The reduction of ventriculoperitoneal shunt pressure led to paroxysmal sympathetic over-excitation. We suggest that intraventricular pressure should be evaluated in a timely manner following the placement of a ventriculoperitoneal shunt in cases involving TBI-induced hydrocephalus. PSH syndrome caused by over-drainage of hydrocephalus should be detected and treated as early as possible.

Case report

A 19-year-old male patient was admitted to the Department of Rehabilitation, Huashan Hospital, Fudan University owing to “unconsciousness for 6 months following a severe head injury caused by a car accident.” The unconsciousness was aggravated by an unsuccessful ventriculoperitoneal shunt inserted at a previous local hospital. Head computed tomography (CT) scans indicated increased hydrocephalus and increased ventricular pressure on February 26, 2021 [Figure 1]A. A ventriculoperitoneal shunt was re-inserted on February 27, 2021 in the Neurosurgical Department of Huashan Hospital, and the ventriculoperitoneal shunt pressure was adjusted to 1.42-1.62 kPa (equivalent to 145-165 mmH₂O) after surgery. Subsequently, on March 3, 2021, the patient suddenly began sweating without obvious precipitating causes; he also developed fever (37.7-38.8°C), dyspnea (30-40 beats/min), increased blood pressure (160/100-180/100 mmHg), increased heart rate (120-170 beats/min) and flexion of both upper extremities and hyperextension of both lower extremities with significantly increased muscle tone (Modified Ashworth grade 4) (Meseguer-Henarejos et al., 2018). These symptoms recurred frequently. Head CT suggested a decrease in hydrocephalus and ventricular pressure, and subdural hematoma formation [Figure 1]B. The PSH assessment measure score (the combined total score of the Clinical Feature Scale and the Diagnosis Likelihood Tool) (Baguley et al., 2014) was 29 (Additional Table 1 [Additional file 1]). Thus, the patient was clinically diagnosed with PSH. A series of treatments were promptly administered. First, we regulated the ventriculoperitoneal shunt pressure to 1.96-2.16 kPa (equivalent to 200-220 mmH₂O) and administered abortive and preventive medications (Tu et al., 2021) including morphine, propranolol, gabapentin, bromocriptine, baclofen, clonazepam, and clonidine. Meanwhile, the patient also received comprehensive rehabilitation treatment including 40 minutes physiotherapy (passive stretching of the limbs), 20 minutes acupuncture treatment (using the following acupoints: DU20, EX-HN1, EX-HN3, GV25, LI11, TE5, LI4 and ST36), 20 minutes neuromuscular electrical stimulation (of bilateral tibialis anterior, wrist and finger extensors), and 20 minutes electric stand-up assisted standing training for 5 days a week. Furthermore, considering the increased resting energy expenditure during paroxysms (Caldwell et al., 2014), he was applied with intensified enteral nutrition support of enteral nutritional suspension 3000 kcal (12,557.55 kJ) per day. After 19 days of intensive care and treatment, the patient’s symptoms gradually improved. Body temperature, respiratory rate, blood pressure, and heart rate returned to the normal range. Arm and limb muscle tone had reduced (Modified Ashworth grade: 1) and the symptoms were completely relieved. A subsequent CT scan of the head revealed that the hydrocephalus had somewhat increased, along with ventricular pressure, but the subdural hematoma had been absorbed [Figure 1]C.

Figure 1: Head computed tomographyimages of the patient.
Note: (A)Before the ventriculoperitoneal shunt was re-inserted.(B)A decrease in hydrocephalus and ventricular pressure, and subdural hematoma formation.(C)After downregulation of the ventriculoperitoneal shunt pressure.The green circles indicate the regions of interest where the computed tomography values relating to the lateral ventricular angle exudation were measured.

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Table 1: Blood indicators during pressure regulation

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The clinical research protocol involving clinical data acquisition was performed in compliance with the Helsinki Declaration and approved by the Institutional Review Board of Huashan Hospital. Written informed consent was obtained from his mother.

Discussion

Severe and excessive autonomic overactivity has been detected in a number of patients who have survived after TBI. Most of these patients showed paroxysmal sympathetic and motor overactivity (Thomas and Greenwald, 2019; van Eijck et al., 2019). PSH is a serious condition, because this symptom can increase long-term disability or even mortality. Despite its clinical impact, the existing literature pertaining to PSH is very inconsistent and pathophysiological and management strategies remain largely unknown. Furthermore, there is no consensus on etiological information or even nomenclature. In 2014, PSH consensus working groups met in Sydney, Australia, and discussed and reached an agreement on the concept, definition, and diagnostic criteria of PSH. This led to the publication of the Sydney Consensus, specifically titled, “Paroxysmal sympathetic hyperactivity after acquired brain injury: consensus on the conceptual definition, nomenclature, and diagnostic criteria” (Baguley et al., 2014).

Our patient met the diagnostic criteria proposed by the Sydney Consensus, as the episodes of PSH occurred after the placement of a hydrocephalus ventriculoperitoneal shunt in our patient post TBI. Based on the data available for this patient, we considered that there was a higher probability of PSH attack because the PSH-AM score was high. The positive sympathetic parameters [Table 1] and heart rate variability detection [Table 2] after pressure regulation provided a stronger objective determination for PSH attack, thus enhancing the accuracy and reliability of early diagnosis. At present, the established evidence for PSH therapy is limited to small cases series (Meyfroidt et al., 2017). Therefore, we used the clinical routine treatment principle for managing such patients, namely “The four-combination comprehensive treatment approach for PSH.”This approach is based on the clinical severity of patients’ characteristics and includes minimizing stimulation inducement, combination drug therapy, rehabilitation, and nutritional support.

Table 2: The detection of heart rate variability

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In this case, we used the Evans index score [Table 4] to evaluate changes in the volume of the hydrocephalus (Evans, 1942) and discovered a significant reduction in the pressure within the hydrocephalus after regulation compared to before regulation. We noted a slight decrease of pressure following treatment and a slight increase in pressure compared with post-regulation, thus suggesting excessive drainage of the hydrocephalus after pressure regulation. The CT values relating to the lateral ventricular angle exudation were more accurate and representative of the changes in ventricular pressure [Table 4]; these values revealed a significant reduction in ventricular pressure after regulation compared to that before regulation, and a slight increase after treatment to that before regulation (although these values were very similar), thus suggesting that excessive drainage of the hydrocephalus after pressure regulation could lead to a rapid reduction in ventricular pressure.

Table 3: Hydrocephalus score

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Table 4: Computed tomography value in anterior angle of the lateral ventricle

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As is known, PSH can be classified into two categories: PSH characterized by relatively pure sympathetic overactivity and another group of disorders with mixed parasympathetic/sympathetic features. Our patient exhibited pure sympathetic overactivity, as there were no parasympathetic symptoms. Previously, the diagnosis of PSH mainly relied on scales and clinical signs (Meyfroidt et al., 2017; Zheng et al., 2020); however, in this case, we combined sympathetic-related markers in the blood with tests of heart rate variability to make a more objective and quantitative judgment on PSH (Moreira et al., 2017; Lee et al., 2021). Comparison of autonomic function, including sympathetic indicators in the blood [Table 2] and heart rate variability [Table 3] showed that the sympathetic activity after pressure regulation was significantly higher than that before pressure regulation but decreased after treatment. These results suggested that the rapid and excessive drainage of a hydrocephalus could cause changes within the sympathetic system. Therefore, these findings indicated that changes in ventricular pressure correlated with the occurrence of PSH. The excessive drainage of a hydrocephalus can cause formation of a subdural hematoma; furthermore, PSH could occur following pressure regulation. This suggests that a reduction in ventricular pressure in patients with hydrocephalus may result in a PSH attack. Monitoring the dynamics of hydrocephalus drainage following pressure regulation and the prevention of PSH attacks is vital.

Author contributions:

HYX conceived the study, was involved in data interpretation and revised the final manuscript. LJY, HQL and WHD collected data, performed the literature search, prepared [Figure 1] and [Table 1]. LJY, XWT and WKF performed data analysis and interpretation, prepared [Tables 2] and [Table 3]. NHW, JFW and YW provided the scientific inputs and clinical support. XWT and HYX conducted data interpretation and supervised the study. LJY and HYX wrote the first draft. All authors approved the final version of the manuscript.

Conflicts of interest:

All authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Editor note: HYX is an Editorial Board member of Brain Network and Modulation. He was blinded from reviewing or making decisions on the manuscript. The article was subject to the journal’s standard procedures, with peer review handled independently of this Editorial Board member and his research group.

Availability of data and materials

All data generated or analyzed during this study are included in this published article and its supplementary information files.

Open access statement:

This is an open access journal, and articles are distributed under the terms of the Creative Commons AttributionNonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.

Additional file

Additional Table 1: PSH assessment measure score.

References

1.	Baguley IJ, Perkes IE, Fernandez-Ortega JF, Rabinstein AA, Dolce G, Hendricks HT (2014) Paroxysmal sympathetic hyperactivity after acquired brain injury: consensus on conceptual definition, nomenclature, and diagnostic criteria. J Neurotrauma 31:1515-1520.
2.	Caldwell SB, Smith D, Wilson FC (2014) Impact of paroxysmal sympathetic hyperactivity on nutrition management after brain injury: a case series. Brain Inj 28:370-373.
3.	Choi HA, Jeon SB, Samuel S, Allison T, Lee K (2013) Paroxysmal sympathetic hyperactivity after acute brain injury. Curr Neurol Neurosci Rep 13:370.
4.	Evans WA, Jr. (1942) An encephalographic ratio for estimating ventricular enlargement and cerebral atrophy. Arch Neurol Psychiatry 47:931-937.
5.	Khalid F, Yang GL, McGuire JL, Robson MJ, Foreman B, Ngwenya LB, Lorenz JN (2019) Autonomic dysfunction following traumatic brain injury: translational insights. Neurosurg Focus 47:E8.
6.	Lee Y, Walsh RJ, Fong MWM, Sykora M, Doering MM, Wong AWK (2021) Heart rate variability as a biomarker of functional outcomes in persons with acquired brain injury: systematic review and meta-analysis. Neurosci Biobehav Rev 131:737-754.
7.	Lucca LF, De Tanti A, Cava F, Romoli A, Formisano R, Scarponi F, Estraneo A, Frattini D, Tonin P, Bertolino C, Salucci P, Hakiki B, D'Ippolito M, Zampolini M, Masotta O, Premoselli S, Interlenghi M, Salvatore C, Polidori A, Cerasa A (2021) Predicting outcome of acquired brain injury by the evolution of paroxysmal sympathetic hyperactivity signs. J Neurotrauma 38:1988-1994.
8.	Meseguer-Henarejos AB, Sánchez-Meca J, López-Pina JA, Carles-Hernández R (2018) Inter- and intra-rater reliability of the Modified Ashworth Scale: a systematic review and meta-analysis. Eur J Phys Rehabil Med 54:576-590.
9.	Meyfroidt G, Baguley IJ, Menon DK (2017) Paroxysmal sympathetic hyperactivity: the storm after acute brain injury. Lancet Neurol 16:721-729.
10.	Moreira HG, Lage RL, Martinez DG, Ferreira-Santos L, Rondon M, Negrão CE, Nicolau JC (2017) Sympathetic nervous activity in patients with acute coronary syndrome: a comparative study of inflammatory biomarkers. Clin Sci (Lond) 131:883-895.
11.	Podell J, Pergakis M, Yang S, Felix R, Parikh G, Chen H, Chen L, Miller C, Hu P, Badjatia N (2022) Leveraging continuous vital sign measurements for real-time assessment of autonomic nervous system dysfunction after brain injury: a narrative review of current and future applications. Neurocrit Care doi: 10.1007/s12028-022-01491-6.
12.	Podell JE, Miller SS, Jaffa MN, Pajoumand M, Armahizer M, Chen H, Tripathi H, Schwartzbauer GT, Chang WW, Parikh GY, Hu P, Badjatia N (2021) Admission features associated with paroxysmal sympathetic hyperactivity after traumatic brain injury: a case-control study. Crit Care Med 49:e989-e1000.
13.	Thomas A, Greenwald BD (2019) Paroxysmal sympathetic hyperactivity and clinical considerations for patients with acquired brain Injuries: a narrative review. Am J Phys Med Rehabil 98:65-72.
14.	Totikov A, Boltzmann M, Schmidt SB, Rollnik JD (2019) Influence of paroxysmal sympathetic hyperactivity (PSH) on the functional outcome of neurological early rehabilitation patients: a case control study. BMC Neurol 19:162.
15.	Tu JSY, Reeve J, Deane AM, Plummer MP (2021) Pharmacological management of paroxysmal sympathetic hyperactivity: a scoping review. J Neurotrauma 38:2221-2237.
16.	van Eijck MM, Sprengers MOP, Oldenbeuving AW, de Vries J, Schoonman GG, Roks G (2019) The use of the PSH-AM in patients with diffuse axonal injury and autonomic dysregulation: a cohort study and review. J Crit Care 49:110-117.
17.	Zheng RZ, Lei ZQ, Yang RZ, Huang GH, Zhang GM (2020) Identification and management of paroxysmal sympathetic hyperactivity after traumatic brain injury. Front Neurol 11:81.