Pediatric dystonic storm : A hospital-based study

2020 ◽  
pp. 10.1212/CPJ.0000000000000989
Author(s):  
Goswami Jyotindra Narayan ◽  
Roy Shuvendu ◽  
Patnaik Saroj Kumar

AbstractBackground:Pediatric dystonic storm is an under-recognized entity. We aimed to evaluate the profiles of children presenting with dystonic storm in a referral hospital. Management schema and treatment-responsiveness of this uncommonly reported entity were analyzed.Methods:Retrospective review of all children (up to 18 years) hospitalized with dystonic storm over 39 months in the aforementioned facility.Results:23 children aged 2year 2months to 14 year 4 months years (median:6 year 11 months)(males: 13, females:11) presented with dystonic storm. Annual incidence was 0.4 per 1000 fresh admissions with event rate of 0.9 per 1000 for all admissions. All had Dystonia Severity Action Plan (DSAP) grades 4/5 with identifiable trigger in 13 (50%). Underlying dystonic disorder pre-existed in 10(43.4%); 08 of these had Cerebral Palsy . Polypharmacotherapy with >4 drugs out of trihexyphenydyl, tetrabenazine, clonazepam, gabapentin, levodopa-carbidopa, triclophos and melatonin were needed. Supportive care and adequate sedation helped in symptom control. All children were managed with midazolam infusion over 2-10 days (median: 5 days). Mechanical ventilation was resorted to in 6 children (3-22 days). Vecuronium and propofol were used in 3/23 (13%) and 4/23(17%) children respectively. Deep brain stimulation was curative in 1 child. Hospitalization ranged from 5-31(median: 11) days. While there were no deaths, rhabdomyolysis was noted in 1 child. Post discharge,6(26%) children relapsed.Conclusions:Dystonic storm is a medical emergency mandating aggressive multimodal management. Supportive care, anti-dystonic drugs, early elective ventilation alongside adequate sedation with benzodiazepines ameliorate complications. Relapses of dystonic storm are not uncommon.

2015 ◽  
Vol 5 (1) ◽  
Author(s):  
Hemmings Wu ◽  
Hartwin Ghekiere ◽  
Dorien Beeckmans ◽  
Tim Tambuyzer ◽  
Kris van Kuyck ◽  
...  

Abstract Conventional deep brain stimulation (DBS) applies constant electrical stimulation to specific brain regions to treat neurological disorders. Closed-loop DBS with real-time feedback is gaining attention in recent years, after proved more effective than conventional DBS in terms of pathological symptom control clinically. Here we demonstrate the conceptualization and validation of a closed-loop DBS system using open-source hardware. We used hippocampal theta oscillations as system input and electrical stimulation in the mesencephalic reticular formation (mRt) as controller output. It is well documented that hippocampal theta oscillations are highly related to locomotion, while electrical stimulation in the mRt induces freezing. We used an Arduino open-source microcontroller between input and output sources. This allowed us to use hippocampal local field potentials (LFPs) to steer electrical stimulation in the mRt. Our results showed that closed-loop DBS significantly suppressed locomotion compared to no stimulation and required on average only 56% of the stimulation used in open-loop DBS to reach similar effects. The main advantages of open-source hardware include wide selection and availability, high customizability and affordability. Our open-source closed-loop DBS system is effective and warrants further research using open-source hardware for closed-loop neuromodulation.


2020 ◽  
Vol 163 (2) ◽  
pp. 356-363
Author(s):  
Chen Lin ◽  
Stephen Y. Kang ◽  
Samantha Donermeyer ◽  
Theodoros N. Teknos ◽  
Sharla M. Wells-Di Gregorio

Objective Patients with head and neck cancer (HNC) face a unique set of unmet needs. A subset of these patients experience symptom control challenges related to their disease burden and treatments. A multidisciplinary approach involving palliative medicine is underutilized but crucial to identify and address these concerns. There is limited information on palliative integration with head and neck oncology. Study Design Case series with planned data collection. Setting Academic quaternary care center. Subjects and Methods We provide descriptive analyses of patients with HNC, including psychodiagnostic assessment and validated quality-of-life screening, from patients’ first encounter at outpatient palliative medicine. Results HNC (N = 80) contributed the greatest number of palliative referrals (25%) between 2010 and 2012. This cohort was 74% male and 79% Caucasian with a mean age of 53 years (95% CI, 51.1-54.9) and with stage IV disease of the oral cavity (28%) or oropharynx (31%). Sixty-three percent of patients had no evidence of disease. Seventy-five percent had a psychological history based on DSM-IV criteria ( Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition), and 70% had a history of substance use disorder. The most distressing quality-of-life concerns were pain, housing and financial problems, and xerostomia. Conclusions Patients with HNC who were referred to palliative medicine are burdened by multiple physical, psychological, substance use, and social challenges. We recommend comprehensive cancer-specific screening, such as the James Supportive Care Screening, to triage patients to appropriate supportive care services. Palliative care is one of many services that these patients may need, and it should be utilized at any point of the disease trajectory rather than reserved for end-of-life care.


2019 ◽  
Vol 95 (1130) ◽  
pp. 656-659 ◽  
Author(s):  
Prudence Gregory ◽  
Najib M Rahman ◽  
Y C Gary Lee

Sir William Osler’s great work and achievements are extensively documented. Less well known is his prolonged battle with postinfluenza pneumonia, lung abscess and pleural infection that eventually led to his demise. At the age of 70, he was a victim of the global Spanish influenza epidemic, and subsequently developed pneumonia. In the era before antibiotics, he received supportive care and opium for symptom control. The infection extended to the pleura and he required repeated thoracentesis which failed to halt his deterioration. He proceeded to open surgical drainage involving rib resection. Unfortunately, he died shortly after the operation from massive pleuropulmonary haemorrhage. In this article, we review the events leading up to Osler’s death and contrast his care 100 years ago with contemporary state-of-the-art management in pleural infection.


2009 ◽  
Vol 110 (6) ◽  
pp. 1274-1277 ◽  
Author(s):  
Niels Allert ◽  
Holger Kirsch ◽  
Waldemar Weirich ◽  
Hans Karbe

Object Impulse generators (IPGs) for deep brain stimulation (DBS) need to be replaced when their internal batteries fail or when technical problems occur. New IPGs are routinely programmed with the previous stimulation parameters. In this study, the authors evaluate the stability of symptom control after such IPG replacements. Methods The authors retrospectively analyzed the outcome of 56 IPG replacements in 42 patients with various movement disorders treated using DBS. Results Stable symptom control was found in 65% of single-channel IPG replacements and 53% of dual-channel IPG replacements. Worsening of symptoms resulted primarily from changes in stimulation effects requiring reprogramming of stimulation parameters (17% of dual-channel IPG and 25% of single-channel IPG). In 14% of dualchannel IPG replacements, instability resulted from erroneous extension adjustment with change in laterality. A new short circuit of active with previously inactive contacts of the quadripolar stimulation lead resulted in a worsening of symptoms in 4% of replacements. Conclusions Replacement of the IPG requires careful follow-up of patients with DBS to ensure stable symptom control.


2002 ◽  
Vol 16 (1) ◽  
pp. 105-121 ◽  
Author(s):  
Neil M Ellison ◽  
Eric Chevlen ◽  
Christopher D Still ◽  
Srinivastan Dubagunta

2018 ◽  
Vol 36 (34_suppl) ◽  
pp. 63-63
Author(s):  
David Christopher Currow ◽  
Hiromichi Matsuoka ◽  
Samuel Allingham ◽  
Belinda Fazekas ◽  
Linda Brown ◽  
...  

63 Background: There are no agreed national nor international criteria for referral to palliative care. Key population characteristics have been defined to aid the generalizability of research findings in palliative care clinical studies. To codify differences in key demographic factors between patients with cancer participating in the Australian national Cancer Supportive Care Clinical Studies Collaborative (CSCCSC) phase III symptom control studies and the population referred to other Australian palliative care services. Methods: This study compares two contemporaneous consecutive cohorts generated through clinical trial participation and the national palliative care clinical quality improvement registry in Australia. Age, sex, cancer diagnosis, language, and socio-economic status were compared. Results: Cohorts were people with cancer: enrolled in CSCCSC phase III clinical studies (n=902; 17 sites); and registered by the Australian national Palliative Care Outcomes Collaboration (PCOC; n=75,240; 117 sites). Participants in CSCCSC studies were younger than those of PCOC (median 71 (IQR 62, 79) versus median 73 (IQR 63, 81); p=0.003 respectively). There was no significant difference in sex (p=0.483). Patients who spoke English accounted 95.0% of enrollees in the CSCCSC group and 92.2% in the PCOC group (p = 0.004). Clinical study participants had higher socioeconomic status that the PCOC group (p=0.022). Conclusions: Overall, the slightly different demographic patterns are reflective of the differences often seen between phase III trials and the populations to whom the results will be applied. Age differences particularly need to be taken into account when considering the best way to apply each study’s findings.


2012 ◽  
Vol 9 (1) ◽  
pp. 99-102 ◽  
Author(s):  
Brian P. Walcott ◽  
Brian V. Nahed ◽  
Kristopher T. Kahle ◽  
Ann-Christine Duhaime ◽  
Nutan Sharma ◽  
...  

Generalized dystonic syndromes may escalate into persistent episodes of generalized dystonia known as status dystonicus that can be life-threatening due to dystonia-induced rhabdomyolysis and/or respiratory compromise. Treatment of these conditions usually entails parenteral infusion of antispasmodic agents and sedatives and occasionally necessitates a medically induced coma for symptom control. The authors report a series of 3 children who presented with medically intractable, life-threatening status dystonicus and were successfully treated with bilateral pallidal deep brain stimulation. Bilateral globus pallidus internus stimulation appears to be effective in the urgent treatment of medically refractory and life-threatening movement disorders.


2006 ◽  
Vol 12 (1) ◽  
pp. 58-62
Author(s):  
Gary E. MCILVAIN ◽  
Ronda STURGILL ◽  
Daniel R. MARTIN

LANGUAGE NOTE | Document text in English; abstract also in Chinese.Leisure activities, recreation, exercise, sport and sporting events are a part of life in society today. In a public school setting, it is the responsibility of the school/facility administrator to ensure safety for the participants and spectators or audience. Safe facilities, playing surfaces and equipment are a foundation for providing safety. Also, the administrator should be proactive in having a written emergency action plan for foreseeable situations, specifically, a medical emergency. Having a predetermined plan of action is imperative for the hosting school/facility. Some important aspects for an Emergency Action Plan (EAP) should include all personnel that will be part of the plan, scheduled mock practice sessions, availability of equipment, current certifications, etc. After implementing these aspects it is imperative that all individuals involved understand and know the details of the EAP in order for it to be effective and successful.隨著生活文化的轉變,康體活動已經成為了人們生活的重要部份。可是,當進行這些活動時一旦出現了意外事故,應該如何去處理較為恰當呢?本文試從緊急應變醫療角度分享經驗,給予有系統及效能的建議,以供參考。


2020 ◽  
Vol 91 (8) ◽  
pp. e6.1-e6
Author(s):  
Peter Brown

Professor Peter Brown is Professor of Experimental Neurology and Director of the Medical Research Council Brain Network Dynamics Unit at the University of Oxford. Prior to 2010 he was a Professor of Neurology at University College London.For decades we have had cardiac pacemakers that adjust their pacing according to demand and yet therapeutic adaptive stimulation approaches for the central nervous system are still not clinically available. Instead, to treat patients with advanced Parkinson’s disease we stimulate the basal ganglia with fixed regimes, unvarying in frequency or intensity. Although effective, this comes with side-effects and in terms of sophistication this treatment approach could be compared to having central heating system on all the time, regardless of temperature. This talk will describe recent steps being taken to define the underlying circuit dysfunction in Parkinson’s and to improve deep brain stimulation by controlling its delivery according to the state of pathological activity.Evidence is growing that motor symptoms in Parkinson’s disease are due, at least in part, to excessive synchronisation between oscillating neurons. Recordings confirm bursts of oscillatory synchronisation in the basal ganglia centred around 20 Hz. The bursts of 20 Hz activity are prolonged in patients withdrawn from their usual medication and the dominance of these long duration bursts negatively correlates with motor impairment. Longer bursts attain higher amplitudes, indicative of more pervasive oscillatory synchronisation within the neural circuit. In contrast, in heathy primates and in treated Parkinson’s disease bursts tend to be short. Accordingly, it might be best to use closed-loop controlled deep brain stimulation to selectively terminate longer, bigger, pathological beta bursts to both save power and to spare the ability of underlying neural circuits to engage in more physiological processing between long bursts. It is now possible to record and characterise bursts on-line during stimulation of the same site and trial adaptive stimulation. Thus far, this has demonstrated improvements in efficiency and side-effects over conventional continuous stimulation, with at least as good symptom control in Parkinsonian patients.


CNS Spectrums ◽  
2016 ◽  
Vol 21 (1) ◽  
pp. 77-121 ◽  

Congratulations to the scientific poster winners of the 2015 NEI Psychopharmacology Congress!1stPLACE: Tardive Dyskinesia in the Era of Second Generation Antipsychotics: A Case Report and Literature Review (page 22)2ndPLACE: Comparing Deep Brain Stimulation (DBS) and ECT for Treatment Resistant Depression in Elderly Population (page 37)3rdPLACE: The Complex Multimodal Management of First-Psychotic Episode in Patients with Morphometric Alteration in Hippocampal Formation (page 30)


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