Sex Hormone-Specific Neuroanatomy of Takotsubo Syndrome: Is the Insular Cortex a Moderator?

Takotsubo syndrome (TTS), a transient form of dysfunction in the heart’s left ventricle, occurs predominantly in postmenopausal women who have emotional stress. Earlier studies support the concept that the human circulatory system is modulated by a cortical network (consisting of the anterior cingulate gyrus, amygdala, and insular cortex (Ic)) that plays a pivotal role in the central autonomic nervous system in relation to emotional stressors. The Ic plays a crucial role in the sympathovagal balance, and decreased levels of female sex hormones have been speculated to change functional cerebral asymmetry, with a possible link to autonomic instability. In this review, we focus on the Ic as an important moderator of the human brain–heart axis in association with sex hormones. We also summarize the current knowledge regarding the sex-specific neuroanatomy in TTS.

Symptom Frequencies and Correlations with Temperature Variations in Suspected Cases of Neuroleptic Malignant Syndrome (NMS)

Background: The incidence of NMS is rare. Relative frequencies of symptoms that are most valuable in making a diagnosis of NMS can be assessed statistically only if a large sample of suspected NMS cases is available. Similarly, the relationship of such NMS symptoms to temperature (a cardinal symptom of NMS) can be meaningfully evaluated only by studying large samples. Method: De-identified archival data on 212 suspected NMS cases were obtained from professionals across the USA and Canada or were extracted from studies published in medical journals. We recorded the symptoms frequencies. The patients’ temperature ranged from 37.20C to 43.00C, with the mean at 39.50 (SD=1.3). All cases were caused by older first-generation antipsychotics (FGAs). We evaluated the frequencies of symptoms reported in these cases. These included symptoms in the realm of mental status, rigidity, and autonomic symptoms. We calculated the frequency of abnormal blood pressure, respiration and heart rate, symptoms such as Dysarthria, Dysphagia, Rigidity, Focal Dystonia, Waxy Flexibility, Myoclonus, Masked Facies, Bradykinesia, Akinesia, Cogwheeling, Stupor, Coma, Obtundation, Mutism, Decrease in Consciousness, Disorientation, Diaphoresis, Sialorrhea, and Seizures. We also calculated their correlations with temperature elevations, the most spectacular symptom of this dangerous syndrome. Results: The highest symptom frequencies (those > 10%) were found for Rigidity (91.0% of patients), Autonomic Instability (66.5%), Diaphoresis (45.8%), Mutism (34.4%), Tremor (31.6%), Stupor (20.3%), Confusion (15.6%), Incontinence (15.6%), Sialorrhea (14.6%), Coma (13.2%), and Dysphagia (11.3%). Other symptoms were too rare within the sample of the 212 suspected NMS cases to calculate the statistical significances of their relationships to temperature. The only significant correlations found of temperature were to increased heart rate and to the severe cases of coma. Discussion and Conclusion: Besides the elevated temperature, the most frequently reported symptoms in this sample of suspected NMS caused by FGAs were Rigidity, Autonomic Instability, Diaphoresis, Mutism, and Tremor. Higher temperature was associated with tachycardia as well as profound impairment of consciousness or coma. Reviews and database studies of second generation antipsychotics (SGAs), also referred to as atypical antipsychotics, suggest a lower incidence of NMS and milder severity of symptoms such as hyperthermia and rigidity. It would be of clinical interest to generate similar de-identified files of archival data for suspected cases of NMS in patients treated with SGAs. Furthermore, a similar profile derived from archival data on milder or prodromal NMS cases could enhance our understanding of this syndrome from a spectrum perspective.

Autonomic instability in severe tetanus: a case report

Tetanus is an infectious disease of the nervous system caused by Clostridium tetani, and is characterized by tonic muscle contractions, painful spasms, and autonomic dysfunction. Severe autonomic dysfunction associated with tetanus can be life-threatening. We present a 62-year-old female who experienced lockjaw after an ankle fracture. The patient was diagnosed with tetanus and received tetanus immunoglobulin and a vaccination. The patient subsequently experienced labile hypertension. This case highlights the challenge and importance of managing cardiovascular instability.

Paediatric anti-GABAB receptor encephalitis associated with SARS-CoV-2 (COVID-19) infection

Though we are approaching the end of second wave of COVID pandemic, we are still unrevealing the various presentation this viral infection can result in. There are few cases reported to have anti NMDA autoimmune encephalitis associated with COVID-19 infection or MIS-C. Here we are reporting one of its variants that was anti-GABAB receptor encephalitis associated with COVID-19 infection. We are reporting a 9 years old female child who was a known case of seizure disorder and on regular medications presented on 4th day of RT-PCR positive status for COVID-19 with complains of convulsions which was managed with antiepileptics. On day 6 of hospitalization she had autonomic instability in the form of tachyarrhythmia, repitation of speech, sudden outburst of laughter and on day 7 child landed in status epilepticus. Autoimmune encephalitis suspected secondary to COVID-19 infection and the child was started on IVIG but not much of improvement seen with this. CSF analysis showed weakly positive anti GABAB antibodies and child had persistently elevated inflammatory markers hence started on high dose corticosteroid. MIS-C ruled out. Child showed drastic improvement both clinical and biochemical after high dose corticosteroids. Prompt treatment with IVIG/corticosteroids have shown a drastic improvement in our child just like in any other autoimmune encephalitis. Though further detailed study is required to prove its exact mechanism in COVID-19 infection, it should be thought of when appropriate and prompt early initiation of therapy will help us reduce morbidity and mortality associated with it.

A narrative review of pharmacologic approaches to symptom management of pediatric patients diagnosed with anti-NMDA receptor encephalitis

Anti-N-Methyl-D-Aspartate Receptor Encephalitis (ANMDARE) is one of the most common autoimmune encephalitis in the pediatric population. Patients with ANMDARE initially present with a prodrome of neuropsychiatric symptoms followed by progressively worsening seizures, agitation, and movement disorders. Complications can include problems such as aggression, insomnia, catatonia, and autonomic instability. Due to the complexity of this disease process, symptom management can be complex and may lead to significant polypharmacy. The goal of this review is to educate clinicians about the challenges of managing this disorder and providing guidance in symptom management.

Intracranial Hypotensive Crisis From an Insidious Spinal Cerebrospinal Fluid-Venous Fistula: A Case Report

BACKGROUND AND IMPORTANCE Progressive episodic spells of altered levels of consciousness, often advancing to include paroxysmal autonomic instability, may be indicative of a diencephalic dysfunction underlying spontaneous intracranial hypotension (SIH). A rare, and often indolent, etiology may be spinal cerebrospinal fluid (CSF) leak—an elusive diagnosis, especially in cases of CSF-venous fistula (CVF) that are often missed on routine computed tomography (CT) myelography and magnetic resonance imaging (MRI). CLINICAL PRESENTATION We report an unusual case of a 50-yr-old woman who presented with rapidly progressive cyclical, self-resolving episodes of altered mentation and decreased arousal later in the day. Scrutiny of serial brain MRIs led to a diagnosis of SIH, with severe downward diencephalic and brain stem displacement—resulting in cerebral aqueduct occlusion with obstructive hydrocephalus. Initial clinical improvement occurred with CSF diversion, but the patient quickly deteriorated—developing diencephalic spells, including extensor posturing and severely depressed levels of consciousness. Clinical improvement was seen with stopping CSF diversion and Trendelenburg-positioning. After intensive spinal imaging, dynamic CT myelography identified a left T10 nerve root diverticula and CSF-venous fistula. Surgical obliteration resulted in rapid, profound neurological improvement, and ultimately full neurological recovery by 1 yr. CONCLUSION In our patient, worsening episodes of confusion, postural headaches, and autonomic instability developed due to SIH, which induced profound downward displacement and compression of the diencephalon and brain stem, and accompanied by subsequent obstructive hydrocephalus. Diagnostic persistence identified the CVF, which had caused the complex multifold pathophysiology and clinical presentation. If suspicion remains high for CVF, persistent spinal imaging, particularly with dynamic myelography, may be crucial.

P222 Heart rate variability is a predictor to disease exacerbation in paediatric Inflammatory Bowel Disease

Background Previous studies have shown a lower level of parasympathetic activity in patients with inflammatory bowel disease (IBD) compared to healthy subjects. Heart rate variability (HRV) oscillations are used to detect autonomic instability and was found to be lower in adults with IBD compared to controls. Nevertheless, the data on the relation between the autonomic function and IBD outcome are scarce. The aim of the study was to evaluate HRV as a predictor to paediatric IBD outcome. Methods Children (<18 years of age) with IBD were prospectively recruited. For each patient, a 10-minute measurement of HRV was conducted by Pulse Oximeter (BM1000A/Shanghai Berry Electronic Tech Co., Ltd). The square root of the mean squared differences of successive RR intervals (RMSSD) was calculated. Clinical data, including demographic variables, disease activity and course, medications and laboratory results were collected during a follow-up of 12 months. The relation between RMSSD and clinical measures was evaluated. Results Overall, 34 IBD children with an average age of 15.32±2.18 years were included: 21 (61.8%) children with Crohn’s disease and 13 (38.2%) with ulcerative colitis. Children in clinical remission had a significantly higher RMSSD compared to patients with active disease (67.72±27.81 and 45.76±22.04, respectively, p=0.022). In addition, patients with normal serum C-reactive protein (<5 mg/L), patients with normal serum hemoglobin and patients with long duration of IBD (more than a year from diagnosis) had a significantly higher RMSSD (Figure 1). Children that experienced disease exacerbation during the follow-up had a significantly lower RMSSD compared to children that kept clinical remission (Figure 2). In a multivariate analysis, higher RMSSD was a significant and independent predictor of lower risk of IBD exacerbation [OR (odd ratio) = 0.941, 95% CI (confidence interval) 0.887–0.998, p=0.044)]. Conclusion HRV may not only reflect IBD activity, but also serve as a predictor to disease exacerbation in paediatric IBD.

Case Report: Ziprasidone induced neuroleptic malignant syndrome

Neuroleptic malignant syndrome (NMS) is a well-recognized neurologic emergency. It presents with classic features including hyperthermia, autonomic instability, muscle hypertonia, and mental status changes. The syndrome is potentially fatal and is associated with significant morbidity due to complications such as rhabdomyolysis, acute kidney injury, and ventricular arrhythmias due to the trans-cellular electrolyte shift. NMS is conventionally associated with the first-generation antipsychotic agents, however, has been described with the use of atypical and novel antipsychotics including Ziprasidone. A case of NMS with Ziprasidone use at the therapeutic dose is reported here.

Diagnosis of Severe Tetanus Assisted by Next-Generation Sequencing Analysis of Etiology: A Case Report

We shared our successful treatment experience of a severe tetanus patient in China. A 50 year old male patient was admitted to our hospital 10 days after the right arm injury due to pain and masticatory weakness. The pathogen of wound secretion was confirmed to be clostridium tetanus by next-generation sequencing (NGS).The patient's condition rapidly progressed to a severe state with autonomic instability. After debridement and comprehensive treatment in ICU, including deep analgesia and sedation with dexmedetomidine, ventilator support and anti-infection treatment, the patient finally recovered and discharged. This case suggested that early diagnosis and reasonable intervention of severe tetanus could reduce mortality.

Neuroleptic malignant syndrome with abnormally elevated cardiac troponin I: a case report

Neuroleptic malignant syndrome (NMS) is a life-threatening neurological emergency that is primarily characterized by altered consciousness, hyperpyrexia, muscular rigidity, and autonomic instability. Here, we describe a unique case of NMS. A 54-year-old woman with major depressive disorder (MDD) was admitted to our hospital to relieve painful emotions; her laboratory tests and physical examinations were unremarkable. Her medication regime was as follows: day 1, quetiapine (200 mg), clonazepam (2 mg), and zopiclone (7.5 mg); day 2, olanzapine (5 mg) and sertraline (100 mg); day 3, olanzapine (15 mg), sertraline (100 mg), zopiclone (7.5 mg), and clonazepam (2 mg); day 4, olanzapine (15 mg) and haloperidol (5 mg); and day 5, sertraline (50 mg) and olanzapine (5 mg). The patient then developed NMS, and a series of tests showed further abnormalities. Unusually, her cardiac troponin I (TNI) was abnormally elevated as her NMS symptoms worsened, but gradually decreased after she was transferred to the cardiology department for treatment. The increased TNI was suspected to be related to the NMS. Here, we provide several potential explanations for the relationship between TNI and NMS. Based on the present case, it may be important to measure and monitor TNI concentrations in NMS patients.