Clinical presentation and management strategies of cardiovascular autonomic dysfunction following a COVID-19 infection – A systematic review

Background

Cardiovascular autonomic dysfunction may reportedly occur after a coronavirus-disease-2019 (COVID-19) infection, but the available evidence is scattered. Here we sought to understand the acute and mid-term effects of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection on cardiovascular autonomic function.

Methods

We performed a systematic PubMed, Embase, Web of Science, medRxiv, and bioRxiv search for cases of cardiovascular autonomic dysfunction during an acute SARS-CoV-2 infection or post-COVID-19 condition. The clinical-demographic characteristics of individuals in the acute versus post-COVID-19 phase were compared.

Results

We screened 6470 titles and abstracts. Fifty-four full-length articles were included in the data synthesis. One-hundred and thirty-four cases were identified: 81 during the acute SARS-CoV-2 infection (24 thereof diagnosed by history) and 53 in the post-COVID-19 phase. Post-COVID-19 cases were younger than those with cardiovascular autonomic disturbances in the acute SARS-CoV-2 phase (42 vs. 51 years old, p = 0.002) and were more frequently women (68% vs. 49%, p = 0.034). Reflex syncope was the most common cardiovascular autonomic disorder in the acute phase (p = 0.008) and postural orthostatic tachycardia syndrome (POTS) the most frequent diagnosis in individuals with post-COVID-19 orthostatic complaints (p < 0.001). Full recovery was more frequent in individuals with acute versus post-COVID-19 onset of cardiovascular autonomic disturbances (43% vs. 15%, p = 0.002).

Conclusions

There is evidence from the scientific literature about different types of cardiovascular autonomic dysfunction developing during and after COVID-19. More data about the prevalence of autonomic disorders associated with a SARS-CoV-2 infection are needed to quantify its impact on human health.     

Phrenic nerve palsy associated with birth trauma – Case reports and a literature review

Phrenic nerve palsy is a peripheral nerve disorder caused by excessive cervical extension due to birth trauma or cardiac surgery. We describe two new patients with phrenic nerve palsy associated with birth trauma. Both patients exhibited profound dyspnea and general hypotonia immediately after birth. A chest roentgenogram and fluoroscopy revealed elevation of the diaphragm, leading to a diagnosis of phrenic nerve palsy associated with birth trauma. Since they had intermittently exhibited dyspnea and recurrent infection, we performed video-assisted thoracoscopic surgery (VATS) plication in both cases, at an early and a late stage, respectively. Both patients subsequently exhibited a dramatic improvement in dyspnea and recurrent respiratory infection. Interestingly, the late stage operated infant exhibited spontaneous recovery at 7 months with cessation of mechanical ventilation once. However, this recovery was transient and subsequently led to an increased ventilation volume demand, finally resulting in surgical treatment at 15 months. Histological examination of the diaphragm at this time showed grouped muscle atrophy caused by phrenic nerve degeneration. To our knowledge, this is the first pathologically proven report of grouped muscle atrophy of the diaphragm due to phrenic nerve degeneration, suggesting that partial impairment of phrenic nerves resulted in respiratory dysfunction with incomplete recovery. We conclude that recently developed VATS plication is a safe and effective treatment for infants with phrenic nerve palsy, and should be considered as a surgical treatment at an early period.     

Langerhans cell histiocytosis of the parietal bone with epidural and extracranial expansion – case report and a review of the literature

Langerhans cell histiocytosis is a rare neoplasm that belongs to the histiocytic and dendritic cell neoplasm group according to the 2008 WHO classification. It has been defined as neoplastic proliferation of Langerhans cells that express CD1a and S-100 proteins and have Birbeck granules on the ultrastructural examination. Clinical presentation and behaviour are heterogeneous and can range from a solitary lytic bone lesion with a favourable course to a fatal disseminated leukaemia-like form, with a wide spectrum of intermediate clinical presentations between these two extremes. Here, we present a case report of a solitary calvarial lesion in an adolescent boy along with a review of the literature. Presenting features, initial diagnostic evaluation and treatment protocol of a unifocal monosystemic calvarial location of LCH are presented.     

Effects of Hesel-coil deep transcranial magnetic stimulation for depression – a systematic review

Background: One third of the depressed patients are not improved by antidepressant drugs and psychological treatments, and there is a need for additional treatments. Repetitive transcranial magnetic stimulation (rTMS) is being developed towards an alternative in treatment-resistant depression. Deep transcranial stimulation (dTMS) with the Hesel-coil (H-coil) is a further development of rTMS aiming to enhance the effect by getting the magnetic pulses to penetrate deeper into the brain.

Aims: This report aims to assess the evidence-base for dTMS for depression. The report also includes an assessment of the ethical and economic aspects involved.

Methods: A systematic review of the effects of H-coil dTMS on depression was conducted and the scientific support was evaluated using GRADE (Grading of Recommendations Assessment, Development and Evaluation).

Results: Only one controlled study was identified. In the sham-controlled randomized study, 212 participants with major depression that had not responded to antidepressant medication were enrolled. A two-point superiority in Hamilton Depression Rating Scale was observed in the dTMS arm vs the sham-arm at 4 weeks, but the difference was not statistically significant. No serious adverse events were reported apart from rare cases of epileptic seizures.

Conclusions: The existing scientific support for H-coil dTMS therapy for depression is insufficient. The clinical implication is that the use of dTMS in depression should be restricted to the framework of clinical trials pending further studies. Fortunately, additional studies are underway and the evidence base should presumably improve over the next several years.     

Clinical–neurophysiological correlations in a series of patients with IgM-related neuropathy

ObjectiveWe aim to draw clinical–neurophysiological correlations in our cohort of patients affected by IgM-related neuropathy to investigate whether neurophysiological parameters may help differentiate the classical phenotype from atypical forms.MethodsWe retrospectively evaluated patients with IgM-related neuropathy referred to our Institute from 1990 to 2011. All patients underwent extensive laboratory, clinical and neurophysiological evaluation.ResultsA classic sensory-ataxic form was observed in 20 of 34 patients, while an atypical phenotype (multiple mononeuropathy, polyneuropathy with predominant motor impairment, painful small-fibre neuropathy) was identified in the remaining 14 cases. Nerve conduction studies revealed in almost all cases a pattern typical of demyelination.A reduced terminal latency index and a prolonged distal motor latency of median nerve, as well as a prolonged distal motor latency and a reduced motor conduction velocity of peroneal nerve when recorded from extensor digitorum brevis, were significantly associated with classic sensory-ataxic phenotype. Conversely, a compound muscle action potential amplitude reduction of peroneal nerve from the tibialis anterior, was mostly associated with atypical forms.ConclusionsNo clear electrophysiological differences between classical forms and atypical cases can be identified in IgM-related neuropathy. Still, we demonstrated that demyelinating abnormalities are more often associated with classical phenotypes, while axonal impairment occurs more often in atypical clinical patterns.SignificancePerforming correlations between clinical and neurophysiological findings in IgM-related neuropathy may help to better understand different disease mechanisms in this heterogeneous form of inflammatory neuropathy.     

Tremor associated with Klinefelter syndrome – A case series and review of the literature

BackgroundPrevious case series suggested a link between Klinefelter syndrome (KS) and essential tremor (ET) or an ET-like syndrome.MethodsWe investigated three KS-patients with tremor including tremor-analyzes and discuss our data in context to findings from a literature review. The clinical outcome after deep brain stimulation (DBS) is also reviewed.ResultsTremor in KS is predominantly a postural and kinetic tremor that resembles ET. Our patients were further characterized by absent family history for tremor in first degree relatives, lack of subjective alcohol responsiveness inquired by history, and tremor onset in childhood. One of our patients and two cases from literature improved after DBS of the ventral intermediate nucleus (VIM) of the thalamus.ConclusionsTremor in KS shares several features with ET. If other characteristics such as family history, alcohol responsiveness, and age at tremor onset may serve as discriminating factors from ET, needs to be further investigated. First observations suggest that VIM-DBS may be efficacious.     

Clinical manifestation and neurosurgical intervention of encephalocraniocutaneous lipomatosis—a case report and review of the literature

Introduction

Currently, there are only a few reported cases of symptomatic or asymptomatic subpial (intramedullary) spinal lipoma, and therefore no guidelines are available to indicate surgery. These lesions are infrequently associated with spina bifida.

Case report

Herein, we provide our experience in the neurosurgical intervention of compressive myeloradiculopathy for encephalocraniocutaneous lipomatosis (ECCL). The patient initially presented with bilateral upper hand paralysis, then regained muscle power after surgery and during 1 year of follow-up. We discuss the neurosurgical indications and intervention, imaging studies, other associated symptoms, and the pathogenesis of ECCL in an infant.     

Clinical characteristics and outcomes of patients with Guillain–Barré and acquired CNS demyelinating overlap syndrome: a cohort study based on a literature review

Abstract

Background:

Some patients with Guillain–Barré syndrome (GBS) also have acquired demyelination of the central nervous system (CNS) (i.e. acquired demyelinating syndrome, ADS). Often, the overlap of GBS and ADS is overlooked. Therefore, we evaluated case reports of GBS/ADS overlap syndrome.

Methods:

We mainly performed website-based research based on articles in cases presented with GBS/ADS overlap syndrome. A total of 66 cases were included. Clinical and prognosis data were analyzed.

Results:

A total of 85% of patients with simultaneous or consecutive occurrence of GBS and ADS were identified within 4 weeks of the initial diagnosis. Transverse myelitis (TM) (32%) was the most common ADS found in GBS/ADS. Patients with Miller Fisher syndrome (MFS)/ADS overlap syndrome had greater female predominance, mean age, frequency of onset at the same time period, or within a short period, and percentage of sole involvement of the subtentorial region. The outcome was favorable based on the functional status in 74% of patients. The sensory level (OR = 0·182, 95% CI = 0·055–0·598; P = 0·005) was the best predictor of a poor outcome, while visual deficit (OR = 4·667, 95% CI = 1·187–18·352; P = 0·027) predicted a favorable outcome.

Conclusion:

The ADS in GBS are diverse, CNS demyelinating may occur at any time, but early in the GBS course (and vice versa). MFS/ADS overlap syndromes is more common. The prognosis is generally good, but patients with sensory level deficit are likely to have a poor prognosis. The features of MFS/other CIS may better reflect involvement of the brainstem in MFS itself, rather than ADS in autoimmune peripheral neuropathies.     

Art materials and processes – a place of meeting art psychotherapy with a four-year-old boy

Abstract

This article explores the complex role of art materials and processes in art psychotherapy with a four-year-old boy with developmental delay. Individual art psychotherapy sessions were conducted over an eighteen-month period. By focusing in detail on work done in the first seven months of his therapy, the author is able to highlight the multifaceted role played by art materials and processes in supporting therapeutic change. The pivotal role played by engagement with the art materials paint, water, sand, whiteboard and clay is described in detail. Initial negotiations that focused on the boys use of and relationship with the materials laid the foundations of a relationship with his therapist in which he was able to develop an internal space that was separate from outside experience and so begin to develop an independent identity. Countertransference was used as a tool for understanding the boys interactions with the materials, for finding meaning in apparent mindlessness and for providing containment in order to lay the foundations of a sense of self. This assisted the development of his language and ability to play in the sessions as well as supporting his social development in his family and elsewhere. The authors arguments are contextualised in current art therapy theory as well as with reference to psychoanalytic concepts, child psychotherapy and art education.     

Botulinum toxin treatment of children with cerebral palsy — a short review of different injection techniques

The intramuscular application of botulinum toxin type A (BoNT/A) has emerged to be an established treatment option to reduce muscular hyperactivity due to spasticity in children with cerebral palsy. Accurate injection is a prerequisite for efficient and safe treatment with BoNT/A. So far, treatment procedures have not been standardized. This paper is a short review of different injection techniques, i.e., manual needle placement as well as guidance by electromyography, electrical stimulation, and ultrasound. Advantages and disadvantages of the different injection techniques are discussed with a focus on needle positioning within the targeted muscle, injection close to the neuromuscular junction and diffusion of BoNT/A within the target muscles and through fascia. The additional information gained by each injection technique is weighed in terms of the clinical impact for children with cerebral palsy.     

Comparison of IPX066 with carbidopa–levodopa plus entacapone in advanced PD patients

BackgroundIPX066, an investigational extended-release carbidopa–levodopa (CD-LD) preparation, has demonstrated a rapid attainment and prolonged maintenance of therapeutic LD plasma concentrations in advanced Parkinson's disease (PD). This phase-3 crossover study assessed its efficacy and safety vs. CD-LD plus entacapone (CL + E).MethodsAt baseline, all patients had motor fluctuations despite a stable regimen of CL + E or CD-LD-entacapone combination tablets (CLE). The study included a 6-week conversion from CL + E or CLE to IPX066, followed by two 2-week, double-blind crossover treatment periods in randomized order, one on IPX066 (and placebo CL + E), the other on CL + E (and placebo IPX066), separated by 1-week open-label IPX066 treatment. The primary efficacy measure was mean percent daily “off” time during waking hours (from patient diaries).ResultsOf 91 randomized patients, 84 completed the study. Their median daily LD dosage was 1495 mg from IPX066 and 600 mg from CL + E, corresponding, after correction for bioavailability, to an approximately 22% higher LD exposure on IPX066. Compared with CL + E, IPX066 demonstrated a lower percent “off” time (24.0% vs. 32.5%; p < 0.0001), lower “off” time (3.8 vs. 5.2 h/day; p < 0.0001), and higher “on” time without troublesome dyskinesia (11.4 vs. 10.0 h/day; p < 0.0001). Other endpoints, including patient-reported treatment preference, also favored IPX066 (p < 0.05). During double-blind treatment, 20.2% and 13.6% of patients reported adverse events on IPX066 and CL + E, respectively. The most common were dyskinesia (4 patients), insomnia (3), and confusional state (3) for IPX066, and fall (2) for CL + E.ConclusionsIn advanced PD, IPX066 showed improved efficacy, compared with CL + E, and appeared to be well tolerated.     

Diastematomyelia associated with ectopic dysplastic renal tissue – report of a rare case

BACKGROUND: Presence of heterotopic dysplastic renal tissue in the lumbosacral region is an extremely uncommon condition. CASE REPORT: We report the first case of diastematomyelia associated with ectopic renal tissue. A 10-month-old male child presented with lipomeningomyelocele associated with spina bifida in the lumbosacral region, and the lipoma was excised. Imaging of the spine at 5 years of age showed spina bifida, bony diastematomyelia, lipomeningomyelocele and a small intraspinal cystic lesion. The boy was then operated upon at the age of 5 years, and histopathological examination of the cystic lesion revealed ectopic dysplastic renal tissue.     

Spinal tumors in neurofibromatosis-2: Management considerations – a review

Neurofibromatosis Type 2 (NF-2) is a distinct clinical entity, characterized by multiple intracranial and spinal tumors. While bilateral vestibular schwannomas are the pathological hallmark of the disease, significant morbidity in NF-2 is attributable to the presence of both intramedullary and extramedullary spinal tumors. With the advent of MRI as a screening modality, multiple, extensive spinal tumors in the NF-2 population are often seen, which may be clinically quiescent at the time of initial diagnosis. All NF-2 patients should have routine screening with full spinal MRI at the time of diagnosis, regardless of symptoms. Early surgical intervention is indicated in cases where a neurological deficit is attributable to a focal expanding spinal lesion. In asymptomatic patients, the decision to operate is tailored to the individual patient, with the ultimate goal of preserving function. In these cases, surgery should be considered where there is evidence of progressive tumor growth, with attendant risk to the patient of functional deterioration.     

Isolated thrombosis of cortical veins – Clinical and radiological correlation

Isolated cortical venous thrombosis (ICVT) is a relatively rare clinical entity with non-specific clinical presentations. Anatomical variations in cortical veins and the lack of a gold standard imaging feature make the diagnosis of ICVT challenging. Headache and seizures were the most common presentations. The Vein of Trolard followed by superficial middle cerebral vein (SMCV) were the most commonly involved. Susceptibility Weighted Imaging (SWI) cord sign was observed in 100% of the cases. CT cord sign and filling defects on contrast enhanced CT were evident in 46.7% and 10% of the cases, respectively. Notably, in the absence of filling defect visualized on contrast CT, MRI, replacement of flow void was the surrogate marker for the ICVT. A high index of clinical suspicion, a thorough understanding of neurovascular anatomy, multiparametric, multiplanar MRI protocol is required to diagnose this rare entity. A serpiginous blooming structure within the subarachnoid space identifiable in less than two contiguous sections on SWI in the vicinity of haemorrhagic infarction should alert the clinician to the imaging possibility of ICVT.