|Year : 2022 | Volume
| 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|
Department of Rehabilitation, Huashan Hospital, Fudan University, Shanghai, China
Source of Support: None, Conflict of Interest: None
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 2022 Dec 8];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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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.
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 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.
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 journalís 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 Table 1: PSH assessment measure score.
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[Table 1], [Table 2], [Table 3], [Table 4]