Artykuły w czasopismach na temat „Clumsiness in literature”

Cerebellar abnormalities have been linked to a number of developmental disorders. Much evidence is based on the analysis of highresolution MRI scans. Imaging and behavioral studies have led researchers to consider functional contributions of the cerebellum beyond that associated with motor control. I review this literature, providing an analysis of different ways to consider the relation between cerebellar abnormalities and developmental disorders. Interestingly, although clumsiness is a problem of coordination, the contribution of cerebellar dysfunction to this developmental problem has received little attention. Select studies indicate that some clumsy children have difficulties on tasks requiring precise timing, similar to that observed in adult patients with cerebellar lesions. I suggest that the underlying neural bases of clumsiness are heterogeneous, with cerebellar dysfunction likely a major contributor for a subpopulation of such children.

AbstractThe association of meningioma with intra- and peritumoral brain abscess is rare. All cases reported in literature had some focus of infection in the body. We report a 56-year-old man with well-controlled diabetes mellitus found to have a space-occupying lesion upon evaluation for headache, vomiting, and clumsiness of right upper limb. Perioperatively, pus was found intra- and perilesionally around a thick, capsulated, dural-based, vascular lesion. Histopathology was suggestive of meningioma, and Escherichia coli was isolated upon culture.

Introduction. Superficial siderosis (SS) is caused by chronic subarachnoid bleeding and is characterized by free iron and hemosiderin deposition along the pial and subpial structures of central nervous system. SS leads to progressive and irreversible CNS damage. The most common causes of chronic subarachnoidal bleeding are tumors, head and spinal cord trauma, arteriovenous malformations and aneurysms. SS is characterized by clinical triad: sensorineural hearing loss, cerebellar ataxia and piramydal signs. Brain MR imaging is the investigation of choice for the diagnosis of SS. Typical findings include hypointensities seen on T2?weighted MR imaging around the brain, cerebellum, brain stem, spinal cord, VIII cranial nerve and atrophy of cerebellum and medulla. Case Outline. A 71-year?old female patient noticed hand tremor in the middle of the third decade of life, and later slowly progressive bilateral hearing loss. At the age of 64 she developed un? steady gate, hand clumsiness and dysarthria, to became incapable of independent walking and standing five years later. Clinical course and brain MRI findings were typical for SS, but additional investigation did not reveal the couse of subarahnoidal bleeding. Conclusion. SS represents a rare and under?recognized condition that must be considered in all patients with cerebellar syndrome of unknown cause. Early diagnosis of SS in some cases with identified cause of chronic bleeding allowes therapeutic interventions that may prevent further progression of the disease.

Background: Many different surgical strategies for arthrosis of the carpometacarpal joint of the thumb are described in the literature. In 2010 we changed our routine procedure from an interposition arthroplasty using the abductor pollicis longus (APL) tendon to simple trapeziectomy without suspension or interposition. The purpose of this study was to review the clinical outcome after trapeziectomy and to compare it to those we had achieved with the APL procedure. Methods: We examined 49 hands operated with simple trapeziectomy during 2011-2012. Time between operation and review was 26 (15-26) months. Subjective estimation of pain before and after surgery and satisfaction with the general results were evaluated with visual analogue scales. The ability to participate in various activities of daily living before and after the operation were noted and patients completed the Quick-DASH and the PRWHE questionnaires. The mobility of the thumb and wrist were recorded and grip and key pinch strength were measured. The distance between the base of the first metacarpal and the scaphoid was noted on radiographs. Results: There were no significant differences between the present results and those we had achieved with the APL procedure regarding subjective estimation of pain and satisfaction, activities of daily living, mobility, strength and radiographic carpometacarpal distance. For many outcome parameters there was a non-significant trend for better results among the trapeziectomy patients, but increased clumsiness was reported by 20 of them. Conclusions: We conclude that our results with simple trapeziectomy are at least as good as with the APL procedure. However, the considerable number of patients complaining of clumsiness is worrying.

The case describes a 25-year-old Caucasian female diagnosed with Alexander’s disease (AxD) as an outpatient after extensive inpatient workup. Her presenting complaints included incontinence, clumsiness, seizures, dysphagia, and dysarthria. She was also found to have pancytopenia and dysautonomia. A full neurologic and hematologic workup yielded very little results, until a thorough literature search of her presenting complaints and radiologic findings pointed to adult-onset Alexander’s Disease. Alexander’s disease is a rare genetic leukodystrophy with a broad variety of presentations. Despite its infrequency in adults and the difficulty in diagnosis, the prevalence of AxD has been increasing due to ease of genetic analysis and identification of key clinical and radiological findings. This case illustrates the necessity of vigilance and persistence in the face of unusual patient presentations; occasionally, the sound of hoofbeats is zebras.

Corticobasal degeneration (CBD) is an uncommon, sporadic, neurodegenerative disorder of mid- to late-adult life. We describe a further example of the pathologic heterogeneity of this condition. A 71-year-old woman initially presented dysarthria, clumsiness, progressive asymmetric bradykinesia, and rigidity in left arm. Rigidity gradually involved ipsilateral leg; postural instability with falls, blepharospasm, and dysphagia subsequently developed. She has been previously diagnosed as unresponsive Parkinson's Disease. At our clinical examination, she presented left upper-arm-fixed-dystonia, spasticity in left lower limb and pyramidal signs (Babinski and Hoffmann). Brain MRI showed asymmetric cortical atrophy in the right frontotemporal cortex. Neuropsychological examination showed an impairment in visuospatial functioning, frontal-executive dysfunction, and hemineglect. This case demonstrates that association of asymmetrical focal cortical and subcortical features remains the clinical hallmark of this condition. There are no absolute markers for the clinical diagnosis that is complicated by the variability of presentation involving also cognitive symptoms that are reviewed in the paper. Despite the difficulty of diagnosing CBD, somatosensory evoked potentials, motor evoked potentials, long latency reflexes, and correlations between results on electroencephalography (EEG) and electromyography (EMG) provide further support for a CBD diagnosis. These techniques are also used to identify neurophysiological correlates of the neurological signs of the disease.

3-Methylglutaconic aciduria type I (MGCA1) is an inborn error of the leucine degradation pathway caused by pathogenic variants in the AUH gene, which encodes 3-methylglutaconyl-coenzyme A hydratase (MGH). To date, MGCA1 has been diagnosed in 19 subjects and has been associated with a variable clinical picture, ranging from no symptoms to severe encephalopathy with basal ganglia involvement. We report the case of a 31-month-old female child referred to our center after the detection of increased 3-hydroxyisovalerylcarnitine levels at newborn screening, which were associated with increased urinary excretion of 3-methylglutaconic acid, 3-hydroxyisovaleric acid, and 3-methylglutaric acid. A next-generation sequencing (NGS) panel for 3-methylglutaconic aciduria failed to establish a definitive diagnosis. To further investigate the strong biochemical indication, we measured MGH activity, which was markedly decreased. Finally, single nucleotide polymorphism array analysis disclosed the presence of two microdeletions in compound heterozygosity encompassing the AUH gene, which confirmed the diagnosis. The patient was then supplemented with levocarnitine and protein intake was slowly decreased. At the last examination, the patient showed mild clumsiness and an expressive language disorder. This case exemplifies the importance of the biochemical phenotype in the differential diagnosis of metabolic diseases and the importance of collaboration between clinicians, biochemists, and geneticists for an accurate diagnosis.

The authors report a case of cervical myelopathy caused by invagination of the bilaterally separated lamina of the axis. They also present a literature review. The patient was a previously healthy 68-year-old man with a 1-year history of slowly progressive gait disturbance, right-hand clumsiness, and right dominant sensory disturbance in his trunk and extremities. Both MRI and CT showed that the spinal cord was markedly compressed at the C2–3 level, on the right side, by a deeply invaginated anomalous lamina of the axis. A bilaterally separated lamina was also visible. The patient underwent removal of the anomalous invaginated fragment of the separated lamina and the spinous process of the axis. One year after surgery, his myelopathic symptoms had almost completely resolved. Here, the authors present the case of a patient with an extremely rare anomaly of the lamina of the axis. The underlying pathogenesis of this anomaly could be the failure of the 2 chondrification centers on either side to fuse into a single ossification center. Surgical removal of the anomalous invaginated lamina produced a satisfactory outcome.

Background and Aim: The classic definition of acute encephalitis consists of altered consciousness associated with fever, seizures or focal neurological alterations on neuroimaging or electroencephalography. However, there are particularities that may provide a glimpse of the probably autoimmune versus infectious etiology of the same neurological picture. Case presentation: A 19-month-old male starts with motor clumsiness and refusal to ambulation and leg claudication in the context of febrile pharyngotonsillitis. Simultaneously, first episode of forced gaze’s lateralization and cephalic deviation with right tonic movements and sucking. On examination: intense irritability with no contact, denial-type stereotypies, hemiparetic gait, pronation and adduction of the right arm and foot with frequent stumbling. Results: Neuroimaging tests, electroencephalogram, laboratory tests and antistreptolysin-O were normal, no pathological clinical exome and detection of antibodies in cerebrospinal fluid paired with serum. Such as diagnosis: acute autoimmune encephalitis due to anti-NMDA-R (N-methyl-D-aspartate Receptor) antibodies and movement disorder (choreoathetosis, hemidystonia). We initiate treatment with intramuscular penicillin and oral clobazam with erratic response. Fortunately, high doses of intravenous (iv) corticosteroids and immunoglobulins, oral corticotherapy and iv rituximab on our patient were used. Months later, clear improvement with autonomous ambulation without assistance, adequate manual opening and entire disappearance of dystonic-myoclonic movements. Conclusions: Choreoathetoid movements accompanied by irritability in an infectious context should lead us to think of Sydenham's chorea. However, new developments in the analysis of biological samples and a high index of suspicion may lead us to autoimmune pathology and the consequent early use of immunotherapy with optimal results.

Introduction:Infantile-onset saccade initiation delay (ISID), also known as congenital ocular motor apraxia, is characterized by the inability to initiate volitional horizontal saccades. Other abnormalities including developmental delay and ataxia have been reported. The frequency of these abnormalities is unknown. We performed a detailed review of the medical literature to quantify features of ISID.Methods:We searched the English medical literature for articles related to ISID from 1952 to 2010. Whenever possible, patients were excluded if they had acquired SID, Joubert syndrome or neurodegenerative conditions. The minimum prevalence was calculated for each abnormality.Results:Sixty-six articles with information on 288 patients were included in the analysis. Head thrusts were reported in 84.7%. Blinks without head thrusts were used to initiate saccades in 41%. The fast phases of the optokinetic response and vestibulo-ocular reflex were impaired in 69.8% and 34.4% respectively. Smooth ocular pursuit was abnormal in 33%. Global developmental delay occurred in 41.3%, speech or language delay in 36.5%, cognitive delay in 17%, hypotonia in 35.8%, motor delay in 48.6%, and ataxia/clumsiness in 49.3% of patients. Neuroimaging was performed on 197 patients and was normal in 39.1%. Abnormalities involved the cerebellum (24.9%), cerebrum (15.7%), other infratentorial structures (11.7%), and corpus callosum (6.1%).Conclusions:Infantile-onset saccade initiation delay is frequently associated with deficits in reflexive saccades and less frequently with impaired smooth ocular pursuit. Developmental delay, hypotonia, and ataxia occur frequently in ISID, suggesting more global brain impairment and not just a saccadic disorder.

Copy number variations (CNVs) involving the JAG1 gene are rare and infrequently reported in the scientific literature. Recently, a generally healthy young patient presenting with a history of behavioural concerns was referred to us. Herein, we discuss the patient, a 7-year-old female possessing a 0.797 Mb microduplication within the short arm of chromosome 20 at band 12.2. The patient generates considerable curiosity due to the rarity of her case, which includes a de novo partial duplication involving the JAG1 gene. The patient exhibits a wide range of symptoms including facial dysmorphism (dolichocephaly, round face, tented philtrum, anteverted nares, and micrognathia), clinodactyly, and an inborn congenital heart defect. She presented with behavioural concerns including ADHD-I, SPD, motor clumsiness, and poor self-regulation. Deletions in JAG1 are often linked to Alagille Syndrome; however, complete duplications have not been specifically identified as disease-causing. JAG1 mutations are reported alongside various clinical features including facial dysmorphology, heart defects, vertebral abnormalities, and ocular dysmorphic features (strabismus, epicanthal folds, and slanted palpebral fissures). This particular microduplication is rare, and thus, limited data exist regarding its significance. To our knowledge, most reported duplications are larger than 0.797 Mb. This may define a critical region causing phenotypical changes in some patient cases.

If we look to the Protagoras for philosophical lessons, it may seem an irritating patchwork of niggling argument, irrelevant digressions, false starts and downright fallacy. Read as a play in which the most outstanding and individual minds of a brilliant period meet and engage in a battle of wits, it will give a different impression. That is how it should be read. A serious discussion of the nature of virtue, and how it is acquired, must be left, as Protagoras said, for another occasion - and, we may add, for different company: it is not to be achieved in the competitive atmosphere of a public gathering of Sophists.The Protagoras is palpably interested in character, vividly reviving a lost intellectual generation. But it also seems to argue for a conclusion, albeit a very provisional one. How should we relate the dialogue's array of luminaries to its argument? A reader may be tempted, as in the quotation above, to use the dialogue's literary lustre merely to excuse its arguments from charges of philosophical clumsiness. But by the end of the work we have been shown apparently successful arguments for the unity of virtue, with Protagoras' attempt at a counter-example refuted (360d8–e5).

The second most frequent central nervous system involvement pattern in Langerhans cell histiocytosis (LCH) is a rare condition documented in a number of reports called “neurodegenerative LCH” (ND-LCH). Magnetic resonance images confirming the presence of the disease usually demonstrate striking symmetric bilateral hyperintensities predominantly in the cerebellum, basal ganglia, pons, and/or cerebral white matter. The authors here describe for the first time in the literature a patient with ND-LCH and concomitant hydrocephalus initially treated using endoscopic third ventriculostomy (ETV). This 9-year-old boy, who had undergone chemotherapy for skin and lung LCH without central nervous system involvement at the age of 10 months, presented with acute ataxia, headaches, and paraparesis and a 1-year history of gradually increasing clumsiness. Magnetic resonance images showed obstructive hydrocephalus at the level of the aqueduct of Sylvius and signs of ND-LCH. After registering high intracranial pressure (ICP) spikes with an intraparenchymal pressure monitor, an ETV was performed. A second ETV was required months later because of ostomy occlusion, and finally a ventriculoperitoneal shunt was placed because of ostomy reocclusion. Endoscopic third ventriculostomy was initially considered the treatment of choice to divert cerebrospinal fluid without leaving a ventriculoperitoneal shunt and to obtain biopsy specimens from the periinfundibular recess area. The third ventriculostomy occluded twice, and an endoscopic aqueduct fenestration was unsuccessful. The authors hypothesized that an inflammatory process related to late ND disease was responsible for the occlusions. Biopsy specimens from the infundibular recess and fornix column did not show histopathogical abnormalities. Increased ICP symptoms resolved with cerebrospinal fluid diversion. This case is the first instance of ND-LCH with hydrocephalus reported in the literature to date. Shunt placement rather than ETV seems to be the favorable choice in relieving elevated ICP.

A dominant theme in the ninth of the Heroides, Deianira's letter to Hercules, is Deianira's indignation that Hercules has been defeated by a woman: first by Iole (especially in the first part of the letter: for example, lines 2, 5f., 1 If., 25f.); then by Omphale (especially in the section 103–18). The theme is exploited so insistently that Vessey, who regards the epistle as spurious, sees in this insistence a sign of the forger's clumsiness. consider the exploitation of the motive of‘victor victus’ in Heroides 9, on the contrary, as a strong sign of Ovidian authorship. From the very beginning of the letter, the reader is reminded that if a woman, Iole, has metaphorically destroyed Hercules, another woman is on the point of destroying Hercules in a much more real and literal way, and this woman is none other than Deianira herself. When Deianira writes the letter, she has just sent to Hercules the garment soaked in Nessus' poison that will provoke Hercules' horrible death (see 143–68): thus Deianira, rather than Iole or Omphale, is the woman who vanquishes the unvanquished hero. But this is not only a matter of dramatic irony based on the general lines of the story. Heroides 9 is an elegiac rewriting of Sophocles' Trachiniae (it is no coincidence that the letter opens with an allusion to Propertius 3.11), and at the same time is inserted in the time and the ‘body’ of the tragedy. Ironic prefiguration in Heroides is normally realized through intertextual anticipation: thefuture events that are prefigured are present in the texts of the model epic or tragedy. Deianira blames Hercules for bis defeat:quern numquam Iuno seriesque immensa laborumfregerit, huic Iolen imposuisse iugum (Her. 9.5f.)quem non mille ferae, quern non Stheneleius hostis,non potuit Iuno vincere, vincit amor. (Her. 9.25f.)

e17019 Background: Chronic Lymphocytic Leukemia is a lymphoproliferative disorder which can have variable course from being indolent to aggressive. Reported annual incidence of Chronic Lymphocytic Leukemia is 3:100000. The most common manifestation is Lymphadenopathy and Leptomeningeal involvement is very rare with only a few reported cases in literature. Methods: This is an observational case report. Results: We report a case of 57 year old White male who presented with CNS disease as his first manifestation of CLL. He reported a 6 week history of cognitive decline with inability to perform complex tasks, difficulty with calculations, word finding trouble, progressive clumsiness of right arm and leg and a weight loss. MRi revealed Left sided Leptomeningeal enhancement. As part of his work up; patient received LP, PET scans, Infectious and autoimmune studies and eventually brain biopsy which initially showed Lymphocytic predominance.On further review by NIH immunohistochemical stains for CD5, CD20 and CD23 were positive on the leptomeningeal and parenchymal lymphocytes. His bone marrow showed CLL involvement both morphologically and immunophenotypically by means of flow cytometric analysis. He was started on treatment with High dose methotrexate and Rituximab and showed remarkable improvement in his cognitive function by the completion of his second cycle. His bone marrow repeat biopsy showed complete remission and no involvement of lymphoma. Conclusions: CLL is an indolent disease and direct CNS invasion is extremely rare. Our case becomes unique in terms of its presentation. It highlights the importance of timely diagnosis and management of leptomeningeal disease in CLL can lead to resolution of neurologic symptoms.

Background: Kabuki syndrome is a rare genetic condition characterised by pathological changes within all the systems of the body, but with variable gene expression. All the patients described in the literature so far have specific facial features resembling the masks of actors from the Japanese Kabuki Theatre and mild to moderate mental impairment. Diagnosis is made based by genetic testing for mutations of the KMT2D and KDM6A genes. Therapy is mainly based on symptomatic alleviation of the effects of mutation, rehabilitation and improvement of the quality of patients’ life. Then prognosis of patients with Kabuki syndrome is closely related to the severity of symptoms, which is very variable. Aim of the study: The purpose of the study is to present the nursing problems based on the case report and to present complications caused by the disease entity. Material and methods: The criterion for including the patient in the study was the legal guardian’s (parent’s) consent for the child to participate in the study. Qualitative research was conducted using analysis of medical records, interview with the child and the child’s legal guardian, direct and indirect observation of the child for psycho-social changes related to the disease and interpretation of the data in the context of the theoretical knowledge and our own observations. Case report: The report is based on the case of a 16-year-old girl, diagnosed (at the age of fourteen) with rare genetic disorder – Kabuki syndrome. The patient experiences some characteristic symptoms – big, red lips, lowset and sticky-out ears, drooping eyelids and short fingers and toes. One of the first symptoms suggesting Kabuki syndrome were: spitting up during breastfeeding, problems with swallowing, motor clumsiness and epilepsy. The role of the nursing team during hospitalization was to take care because of habitual dislocation of patella, and to provide psychological suport. At present time the girl uses a wheelchair or she moves on her knees Results: The manifestations of chronic disease contribute to the feeling of excessive stress, regardless of the patient’s age. An adequate diet enriched with proteins contributes to the prevention of bedsores among individuals with Kabuki syndrome, immobilised to various degrees. Regular consultations with specialists, such as cardiologist, neurologist, gastroenterologist, orthopaedist, ophthalmologist, psychologist, orthodontist, speech therapist, immunologist, endocrinologist and dietician reduce the risk of complications associated with the disease in the patient with Kabuki syndrome. Early implementation of rehabilitation, focused to increase muscle tension, contributes to maintaining autonomy and self-care in patients with Kabuki syndrome.

The article aims to identify some of the peculiarities of the XIXth century outlaw novel in relation to the conventions and the stereotypes of the adventure novel, focusing on George Baronzi’s novel, Mina the Outlaw. The Girl of the Forests, an agreeable novel in spite of several narrative clumsiness. The novelty comes from the fact that the novel proposes a female character in the role of the hero, somehow accumulating the conventions and some narrative invariants specific to the outlaw novel, but also to the sentimental novel, the plot having as a starting point a love story. The novel is, therefore, chracterized by a certain naivity specific to the subgenre and to the literary period to which it belongs, but the author cannot be blamed for the lack of a certain sense of suspense and proliferation of adventures, by moving from one conflict to another, by changing the setting of the action thus activating its narrative potential.

We report on the rare case of a male toddler presenting with myoclonic epilepsy characterized by daily episodes of upward movements of the eyebrows, and myoclonic jerks of both head and upper limbs. In addition, the child showed speech delay, tremors, and lack of motor coordination. Next Generation Sequencing analysis (NGS) performed in trio revealed in the proband the c.889C>T de novo missense variant in the KCNA2 gene in heterozygous state. This is the first case of myoclonic epilepsy in a toddler due to a c.889C>T KCNA2 missense variant. The patient was treated with valproic acid and ethosuximide with a good clinical response. At 6 years old, follow-up revealed that the proband was seizure-free with tremors and clumsiness in movements. According to the literature, this case supports the correlation between myoclonic epilepsy and KCNA2 alterations. This evidence suggests that performing genomic testing including the KCNA2 gene in preschool patients affected by myoclonic epilepsy, especially when associated with delayed neurodevelopment. Our goal is to expand the phenotypical spectrum of this rare condition and adding clinical features following a genotype-first approach.

ObjectiveTo identify the research hotspots on cognitive function in developmental coordination disorder (DCD) in recent years, predict the research frontier and development trend, and provide more perspectives for the study of the DCD population.MethodsUsing CiteSpace and VOSviewer software to draw charts, 1,082 pieces of literature about DCD and cognitive function in the Web of Science core collection database from 2010 to 2022 were visually analyzed.Results and conclusionInterest in the cognitive function of DCD has been on the rise in the past 10 years. Over 40 countries and regions, 117 institutions and 200 researchers have participated in the corresponding research, mainly in the United States, and their institutions have published more highly influential results. The hot keywords are DCD, children, attention, working memory, performance, and attention-deficit/hyperactivity disorder (ADHD), and the main research hot topics include functional performance, population, cognitive psychology. The research directions include “DCD,” “Asperger syndrome,” “memory,” “infant,” “clumsiness,” “neurodevelopmental disorder,” “occupational therapy,” “preschool children,” “motor competence,” “model,” and “online control.” Future research should focus on motor imagery and intrinsic models and use more neurophysiological techniques to reveal the cognitive characteristics of children with DCD and develop intervention programs.

Abstract Background Hereditary ataxia (HA) represents a group of genetically heterogeneous neurodegenerative diseases caused by dysfunction of the cerebellum or disruption of the connection between the cerebellum and other areas of the central nervous system. Phenotypic manifestation of HA includes unsteadiness of stance and gait, dysarthria, nystagmus, dysmetria and complaints of clumsiness. There are no specific treatments for HA. Management strategies provide supportive treatment to reduce symptoms. Objectives This systematic review aimed to identify, evaluate and summarise the published literature on the therapeutic roles of natural remedies in the treatment of HA to provide evidence for clinical practice. Methods A systematic literature search was conducted using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). Web of Science, PubMed and Science Direct Scopus were thoroughly searched for relevant published articles from June 2007 to July 2020. Results Ten pre-clinical and two clinical studies were eligible for inclusion in this systematic review. We identified the therapeutic roles of medicinal plants Brassica napus, Gardenia jasminoides, Gastrodia elata, Ginkgo biloba, Glycyrrhiza inflata, Paeonia lactiflora, Pueraria lobata and Rehmannia glutinosa; herbal formulations Shaoyao Gancao Tang and Zhengan Xifeng Tang; and medicinal mushroom Hericium erinaceus in the treatment of HA. In this review, we evaluated the mode of actions contributing to their therapeutic effects, including activation of the ubiquitin–proteasome system, activation of antioxidant pathways, maintenance of intracellular calcium homeostasis and regulation of chaperones. We also briefly highlighted the integral cellular signalling pathways responsible for orchestrating the mode of actions. Conclusion We reviewed the therapeutic roles of natural remedies in improving or halting the progression of HA, which warrant further study for applications into clinical practice.

Abstract Background GNAO1 encephalopathy is a rare neurodevelopmental disorder characterized by distinct movement presentations and early onset epileptic encephalopathy. Here, we report the in-depth phenotyping of genetically confirmed patients with GNAO1 encephalopathy, focusing on movement presentations. Results Six patients who participated in Korean Undiagnosed Disease Program were diagnosed to have pathogenic or likely pathogenic variants in GNAO1 using whole exome sequencing. All medical records and personal video clips were analyzed with a literature review. Three of the 6 patients were male. Median follow-up duration was 41 months (range 7–78 months) and age at last examination was 7.4 years (range 3.3–16.9 years). Initial complaints were hypotonia or developmental delay in 5 and right-hand clumsiness in 1 patient, which were noticed at median age of 3 months (range 0–75 months). All patients showed global developmental delay and 4 had severely retarded development. Five patients (5/6, 83.3%) had many different movement symptoms with various onset and progression. The symptoms included stereotyped hands movement, non-epileptic myoclonus, dyskinesia, dystonia and choreoathetosis. Whole exome sequencing identified 6 different variants in GNAO1. Three were novel de novo variants and atypical presentation was noted in a patient. One variant turned out to be inherited from patient’s mother who had mosaic variant. Distinct and characteristics movement phenotypes in patients with variant p.Glu246Lys and p.Arg209His were elucidated by in-depth phenotyping and literature review. Conclusions We reported 6 patients with GNAO1 encephalopathy showing an extremely diverse clinical spectrum on video. Some characteristic movement features identified by careful inspection may also provide important diagnostic insight and practice guidelines.

Background: Glucose-transporter-1 deficiency syndrome (GLUT1-DS), due to SLC2A1 gene mutation, is characterized by early-onset seizures, which are often drug-resistant, developmental delay, and hypotonia. Hemiplegic migraine (HM) is a rare form of migraine, defined by headache associated with transient hemiplegia, and can be caused by mutations in either CACNA1A, ATP1A2, or SCN1A. Paroxysmal movements, other transient neurological disorders, or hemiplegic events can occur in GLUT1-DS patients with a mild phenotype.Case: We report on a girl with GLUT1-DS, due to SLC2A1 mutation, with a mild phenotype. In early childhood, she developed epilepsy and mild cognitive impairment, balance disorders, and clumsiness. At the age of 9, the patient reported a first hemiplegic episode, which regressed spontaneously. Over the next 3 years, two similar episodes occurred, accompanied by headache. Therefore, in the hypothesis of HM, genetic testing was performed and CACNA1A mutation was identified. The treatment with Lamotrigine avoided the recurrence of HM episodes.Discussion: To our knowledge, among the several cases of GLUT1-DS with HM symptoms described in the literature, genetic testing was only performed in two of them, which eventually proved to be negative. In all other cases, no other genes except for SLC2A1 were examined. Consequently, our patient would be the first description of GLUT1-DS with HM due to CACNA1A mutation. We would emphasize the importance of performing specific genetic testing in patients with GLUT1-DS with symptoms evocative of HM, which may allow clinicians to use specific pharmacotherapy.

Введение. Обращение к исследованию детства в романе В. Набокова аргументировано рядом факторов: историко-культурными изменениями в начале ХХ века, вниманием к феномену детства в гуманитарном знании, значимостью детства в эстетике модернизма. Несмотря на то, что тема детства многократно становилась предметом набоковедческого анализа, оно не рассматривалось как часть социокультурного пространства в романе «Дар». Данная работа посвящена анализу социокультурной семантики этой темы и ее воплощения на уровне художественного пространства. Цель – исследовать социальные и культурные маркеры, характеризующие пространство становления личности персонажей, определить особенности детства как части социокультурного пространства романа В. Набокова «Дар». Материал и методы. Исследование проводится на материале романа «Дар». Теоретико-методологическую базу составляют социологические работы П. Сорокина, труды по семиотике художественного пространства Ю. М. Лотмана, Е. Фарино, работы по поэтике модернистской прозы Дж. Олни, Н. В. Морженковой, набоковедческие статьи Ю. Левина. Результаты и обсуждение. Субъектом осмысления детства как особого социокультурного пространства в романе является центральный герой Фёдор Годунов-Чердынцев. Он воссоздает ряд примет (конкретная локация особняка Годуновых в Санкт-Петербурге, описание интерьера усадьбы в Лешино, характеристика игрушек, социальных связей семьи и пр.), позволяющих составить представление об «удавшемся детстве». Образ своего детства вписывается начинающим поэтом в усадебный и петербургский текст русской литературы, а через ассоциацию своей няни с Ариной Родионовной устанавливается духовное родство рода Годуновых с Пушкиным. Своеобразие социокультурного пространства детства Фёдора – в объемности, полноте существования, обеспеченной включенности рода Годуновых в природный универсум (знание русской природы, «поимка» бабочек), науку (экспедиции и исследования отца, локус домашнего музея), элитарную культуру (локус библиотеки, творчество Пушкина). По контрасту с собственным детством Фёдор описывает детство «великого шестидесятника» Н. Г. Чернышевского (лучшие игрушки и одежда Годуновых контрастны «нанковым брючкам» Николя, внимательность, зоркость, физическая ловкость Фёдора противопоставлены «близорукости» и неуклюжести Чернышевского и т. п.). Внешние физические и социальные маркеры служат для характеристики внутреннего мира Николая Гавриловича. Социокультурное пространство Годуновых наполнено созиданием, открытием нового, осуществлением, семейно-родовым единением, способностью испытывать счастье самореализации, а Чернышевского – антонимичными характеристиками (разъединения, личностной ограниченности и несвободы, неспособности к реализации своих идей). Заключение. Проведенный анализ позволяет утверждать, что социокультурный код является значимым средством раскрытия идейно-тематического своеобразия романа «Дар». Социокультурное пространство детства показано как определяющее всю последующую жизнь человека. Социокультурная семантика детства представителей двух типов интеллигентов (дворянской и разночинной) помогает Набокову оформить свою историософскую концепцию недальновидности выбора пути России, пошедшей за «спасителем новым» – Чернышевским. Набоков оспаривает представление об аристократах как об оторванных от реальной жизни «барчуках». Он противопоставляет «близорукость» материалиста-разночинца Чернышевского, не знающего ни жизни, ни искусства, ни родной природы, всесторонней просвещенности и приспособленности к жизни в самых разных условиях аристократов Годуновых-Чердынцевых (деда Кирилла Ильича и Фёдора в эмиграции, отца – в экспедициях).Introduction. The appeal to the study of childhood in V. Nabokov’s novel is argued by a number of factors: historical and cultural changes at the beginning of the 20th century, attention to the phenomenon of childhood in humanitarian knowledge, and the significance of childhood in the aesthetics of modernism. Despite the fact that the topic of childhood has repeatedly become the subject of Nabokov’s analysis, it was not considered as part of the sociocultural space in the novel “Gift” (“Dar”). The purpose of the work is to explore the social and cultural features that describe the space of the formation of the personality of the characters; to determine the features of childhood as part of the socio-cultural space of V. Nabokov’s novel “Gift” (“Dar”). Material and methods. The study is based on the material of the novel “Gift” (“Dar”). The theoretical and methodological base of the research is composed of the sociological works of P. Sorokin, his works on the semiotics of the creative space of Y.M. Lotman, E. Farino, Nabokov’s studies of Yu. Levin. Results and discussion. The central character Fyodor Godunov-Cherdyntsev is the subject of understanding childhood as a special socio-cultural space in the novel. He recreates a number of signs (a description of the toys, a specific location of the Godunov mansion in St. Petersburg, a description of the interior of the estate in Leshino, a characteristic of the social relationship of the family, etc.), which make it possible to get an idea of a “successful childhood”. The image of his childhood is fitted by the novice poet into the manor and St. Petersburg’s style of Russian literature and through the association of his nanny with Arina Rodionovna, the spiritual relationship of the Godunov clan with Pushkin is established. The uniqueness of the socio-cultural space of Fyodor’s childhood is in the stereoscopic volume, the fullness of existence, ensured by the inclusion of the Godunov’s family in the natural universe, science, and elite culture. The social well-being of the Godunov-Cherdyntsev’s family is interpreted by Nabokov not like someone’s giving this privilege, but as genuine (internal) aristocracy, which is ensured by internal strength of mind, the ability to fulfill oneself in various fields: social and political (Fyodor’s Grandfather), scientific (Father Konstantin Kirillovich), creative (Fyodor). In contrast to his own childhood, Fyodor describes the childhood of the “great sixties” N. G. Chernyshevsky (Godunov’s best toys and clothes are contrasted with Nicolya’s “trousers”/“nankov’s trousers; Fyodor’s attentiveness and vigilance are contrasted with Nicolas’s shortsightedness; Godunov-Cherdyntsev’s physical dexterity is opposed to Chernyshevsky’s clumsiness, etc.). Finally, the continuity of the views and values of the father by the son in the noble family and the refusal to follow in the footsteps of the father in the family history of the son of the priest, the revolutionary fellow of Chernyshevsky are emphasized. Conclusion. Contrary to the prevailing opinion about Nabokov as a writer who is alien to social issue, the analysis allows us to state that the socio-cultural code is a significant method of revealing the ideological and thematic originality of the novel “Gift” (“Dar”). The socio-cultural space of childhood turns out to be the determining factor for all subsequent life. The socio-cultural space in Nabokov’s novels can be considered in a number of ways: intra-family and clan relations; social and personal status (the question of the correspondence of the inner world of a person to the assumed social role); the relationship of social conditions of existence with education, upbringing, cultural and personal self-determination of a person. Nabokov disputes the idea of aristocrats as people who are afraid of hard physical work and divorced from real life. On the contrary, he contrasts the “short-sightedness” of materialist-raider Chernyshevsky, who knows neither life, nor art, nor native nature, with comprehensive enlightenment and adaptability to life in the most diverse conditions of the Godunov-Cherdyntsevs (grandfather Kirill Ilyich and Fedor in exile, father - in expeditions). Social well-being is interpreted by Nabokov not as a self-worthy good, but as a basis for the freedom of personal and cultural selfdetermination.

By most accounts, “resilience” is a pretty resilient concept. Or policy instrument. Or heuristic tool. It’s this last that really concerns us here: resilience not as a politics, but rather as a descriptive device for attempts in the humanities—particularly in rhetoric and cultural studies—to adequately describe a discursive event. Or rather, to adequately describe a class of discursive events: those that involve rhetorical resistance by victimised subjects. I’ve argued elsewhere (Munro, Descriptive; Reading) that Peircean semiosis, inflected by a rhetorical postulate of genre, equips us well to closely describe a discursive event. Here, I want briefly to suggest that resilience—“discursive” resilience, to coin a term—might usefully supplement these hypotheses, at least from time to time. To support this suggestion, I’ll signal some uses of resilience before turning briefly to a case study: a sensational Argentine homicide case, which occurred in October 2002, and came to be known as the caso Belsunce. At the time, Argentina was wracked by economic crises and political instability. The imposition of severe restrictions on cash withdrawals from bank deposits had provoked major civil unrest. Between 21 December 2001 and 2 January 2002, Argentines witnessed a succession of five presidents. “Resilient” is a term that readily comes to mind to describe many of those who endured this catastrophic period. To describe the caso Belsunce, however—to describe its constitution and import as a discursive event—we might appeal to some more disciplinary-specific understandings of resilience. Glossing Peircean semiosis as a teleological process, Short notes that “one and the same thing […] may be many different signs at once” (106). Any given sign, in other words, admits of multiple interpretants or uptakes. And so it is with resilience, which is both a keyword in academic disciplines ranging from psychology to ecology and political science, and a buzzword in several corporate domains and spheres of governmental activity. It’s particularly prevalent in the discourses of highly networked post-9/11 Anglophone societies. So what, pray tell, is resilience? To the American Psychological Association, resilience comprises “the process of adapting well in the face of adversity.” To the Resilience Solutions Group at Arizona State University, resilience is “the capacity to recover fully from acute stressors, to carry on in the face of chronic difficulties: to regain one’s balance after losing it.” To the Stockholm Resilience Centre, resilience amounts to the “capacity of a system to continually change and adapt yet remain within critical thresholds,” while to the Resilience Alliance, resilience is similarly “the capacity of a system to absorb disturbance and still retain its basic function and structure” (Walker and Salt xiii). The adjective “resilient” is thus predicated of those entities, individuals or collectivities, which exhibit “resilience”. A “resilient Australia,” for example, is one “where all Australians are better able to adapt to change, where we have reduced exposure to risks, and where we are all better able to bounce back from disaster” (Australian Government). It’s tempting here to synthesise these statements with a sense of “ordinary language” usage to derive a definitional distillate: “resilience” is a capacity attributed to an entity which recovers intact from major injury. This capacity is evidenced in a reaction or uptake: a “resilient” entity is one which suffers some insult or disturbance, but whose integrity is held to have been maintained, or even enhanced, by its resistive or adaptive response. A conjecturally “resilient” entity is thus one which would presumably evince resilience if faced with an unrealised aversive event. However, such abstractions ignore how definitional claims do rhetorical work. On any given occasion, how “resilience” and its cognates are construed and what they connote are a function, at least in part, of the purposes of rhetorical agents and the protocols and objects of the disciplines or genres in which these agents put these terms to work. In disciplines operating within the same form of life or sphere of activity—disciplines sharing general conventions and broad objects of inquiry, such as the capacious ecological sciences or the contiguous fields of study within the ambit of applied psychology—resilience acts, at least at times, as a something of a “boundary object” (Star and Griesemer). Correlatively, across more diverse and distant fields of inquiry, resilience can work in more seemingly exclusive or contradictory ways (see Handmer and Dovers). Rhetorical aims and disciplinary objects similarly determine the originary tales we are inclined to tell. In the social sciences, the advent of resilience is often attributed to applied psychology, indebted, in turn, to epidemiology (see Seery, Holman and Cohen Silver). In environmental science, by contrast, resilience is typically taken to be a theory born in ecology (indebted to engineering and to the physical sciences, in particular to complex systems theory [see Janssen, Schoon, Ke and Börner]). Having no foundational claim to stake and, moreover, having different purposes and taking different objects, some more recent uptakes of resilience, in, for instance, securitisation studies, allow for its multidisciplinary roots (see Bourbeau; Kaufmann). But if resilience is many things to many people, a couple of commonalities in its range of translations should be drawn out. First, irrespective of its discipline or sphere of activity, talk of resilience typically entails construing an object of inquiry qua system, be that system an individual, a community of circumstance, a state, a socio-ecological unit or some differently delimited entity. This bounded system suffers some insult with no resulting loss of structural, relational, functional or other integrity. Second, resilience is usually marshalled to promote a politics. Resilience talk often consorts with discourses of meliorative action and of readily quantifiable practical effects. When the environmental sciences take the “Earth system” and the dynamics of global change as their objects of inquiry, a postulate of resilience is key to the elaboration and implementation of natural resource management policy. Proponents of socio-ecological resilience see the resilience hypothesis as enabling a demonstrably more enlightened stewardship of the biosphere (see Folke et al.; Holling; Walker and Salt). When applied psychology takes the anomalous situation of disadvantaged, at-risk individuals triumphing over trauma as its declared object of inquiry, a postulate of resilience is key to the positing and identification of personal and environmental resources or protective factors which would enable the overcoming of adversity. Proponents of psychosocial resilience see this concept as enabling the elaboration and implementation of interventions to foster individual and collective wellbeing (see Goldstein and Brooks; Ungar). Similarly, when policy think-tanks and government departments and agencies take the apprehension of particular threats to the social fabric as their object of inquiry, a postulate of resilience—or of a lack thereof—is critical to the elaboration and implementation of urban infrastructure, emergency planning and disaster management policies (see Drury et al.; Handmer and Dovers). However, despite its often positive connotations, resilience is well understood as a “normatively open” (Bourbeau 11) concept. This openness is apparent in some theories and practices of resilience. In limnological modelling, for example, eutrophication can result in a lake’s being in an undesirable, albeit resilient, turbid-water state (see Carpenter et al.; Walker and Meyers). But perhaps the negative connotations or indeed perverse effects of resilience are most apparent in some of its political uptakes. Certainly, governmental operationalisations of resilience are coming under increased scrutiny. Chief among the criticisms levelled at the “muddled politics” (Grove 147) of and around resilience is that its mobilisation works to constitute a particular neoliberal subjectivity (see Joseph; Neocleous). By enabling a conservative focus on individual responsibility, preparedness and adaptability, the topos of resilience contributes critically to the development of neoliberal governmentality (Joseph). In a practical sense, this deployment of resilience silences resistance: “building resilient subjects,” observe Evans and Reid (85), “involves the deliberate disabling of political habits. […] Resilient subjects are subjects that have accepted the imperative not to resist or secure themselves from the difficulties they are faced with but instead adapt to their enabling conditions.” It’s this prospect of practical acquiescence that sees resistance at times opposed to resilience (Neocleous). “Good intentions not withstanding,” notes Grove (146), “the effect of resilience initiatives is often to defend and strengthen the political economic status quo.” There’s much to commend in these analyses of how neoliberal uses of resilience constitute citizens as highly accommodating of capital and the state. But such critiques pertain to the governmental mobilisation of resilience in the contemporary “advanced liberal” settings of “various Anglo-Saxon countries” (Joseph 47). There are, of course, other instances—other events in other times and places—in which resilience indisputably sorts with resistance. Such an event is the caso Belsunce, in which a rhetorically resilient journalistic community pushed back, resisting some of the excesses of a corrupt neoliberal Argentine regime. I’ll turn briefly to this infamous case to suggest that a notion of “discursive resilience” might afford us some purchase when it comes to describing discursive events. To be clear: we’re considering resilience here not as an anticipatory politics, but rather as an analytic device to supplement the descriptive tools of Peircean semiosis and a rhetorical postulate of genre. As such, it’s more an instrument than an answer: a program, perhaps, for ongoing work. Although drawing on different disciplinary construals of the term, this use of resilience would be particularly indebted to the resilience thinking developed in ecology (see Carpenter el al.; Folke et al.; Holling; Walker et al.; Walker and Salt). Things would, of course, be lost in translation (see Adger; Gallopín): in taking a discursive event, rather than the dynamics of a socio-ecological system, as our object of inquiry, we’d retain some topological analogies while dispensing with, for example, Holling’s four-phase adaptive cycle (see Carpenter et al.; Folke; Gunderson; Gunderson and Holling; Walker et al.). For our purposes, it’s unlikely that descriptions of ecosystem succession need to be carried across. However, the general postulates of ecological resilience thinking—that a system is a complex series of dynamic relations and functions located at any given time within a basin of attraction (or stability domain or system regime) delimited by thresholds; that it is subject to multiple attractors and follows trajectories describable over varying scales of time and space; that these trajectories are inflected by exogenous and endogenous perturbations to which the system is subject; that the system either proves itself resilient to these perturbations in its adaptive or resistive response, or transforms, flipping from one domain (or basin) to another may well prove useful to some descriptive projects in the humanities. Resilience is fundamentally a question of uptake or response. Hence, when examining resilience in socio-ecological systems, Gallopín notes that it’s useful to consider “not only the resilience of the system (maintenance within a basin) but also coping with impacts produced and taking advantage of opportunities” (300). Argentine society in the early-to-mid 2000s was one such socio-political system, and the caso Belsunce was both one such impact and one such opportunity. Well-connected in the world of finance, 57-year-old former stockbroker Carlos Alberto Carrascosa lived with his 50-year-old sociologist turned charity worker wife, María Marta García Belsunce, close to their relatives in the exclusive gated community of Carmel Country Club, Pilar, Provincia de Buenos Aires, Argentina. At 7:07 pm on Sunday 27 October 2002, Carrascosa called ambulance emergencies, claiming that his wife had slipped and knocked her head while drawing a bath alone that rainy Sunday afternoon. At the time of his call, it transpired, Carrascosa was at home in the presence of intimates. Blood was pooled on the bathroom floor and smeared and spattered on its walls and adjoining areas. María Marta lay lifeless, brain matter oozing from several holes in her left parietal and temporal lobes. This was the moment when Carrascosa, calm and coherent, called emergency services, but didn’t advert the police. Someone, he told the operator, had slipped in the bath and bumped her head. Carrascosa described María Marta as breathing, with a faint pulse, but somehow failed to mention the holes in her head. “A knock with a tap,” a police source told journalist Horacio Cecchi, “really doesn’t compare with the five shots to the head, the spillage of brain matter and the loss of about half a litre of blood suffered by the victim” (Cecchi and Kollmann). Rather than a bathroom tap, María Marta’s head had met with five bullets discharged from a .32-calibre revolver. In effect, reported Cecchi, María Marta had died twice. “While perhaps a common conceit in fiction,” notes Cecchi, “in reality, dying twice is, by definition, impossible. María Marta’s two obscure endings seem to unsettle this certainty.” Her cadaver was eventually subjected to an autopsy, and what had been a tale of clumsiness and happenstance was rewritten, reinscribed under the Argentine Penal Code. The autopsy was conducted 36 days after the burial of María Marta; nine days later, she was mentioned for the second time in the mainstream Argentine press. Her reappearance, however, was marked by a shift in rubrics: from a short death notice in La Nación, María Marta was translated to the crime section of Argentina’s dailies. Until his wife’s mediatic reapparition, Carroscosa and other relatives had persisted with their “accident” hypothesis. Indeed, they’d taken a range of measures to preclude the sorts of uptakes that might ordinarily be expected to flow, under functioning liberal democratic regimes, from the discovery of a corpse with five projectiles lodged in its head. Subsequently recited as part of Carrascosa’s indictment, these measures were extensively reiterated in media coverage of the case. One of the more notorious actions involved the disposal of the sixth bullet, which was found lying under María Marta. In the course of moving the body of his half-sister, John Hurtig retrieved a small metallic object. This discovery was discussed by a number of family members, including Carrascosa, who had received ballistics training during his four years of naval instruction at the Escuela Nacional de Náutica de la Armada. They determined that the object was a lug or connector rod (“pituto”) used in library shelving: nothing, in any case, to indicate a homicide. With this determination made, the “pituto” was duly wrapped in lavatory paper and flushed down the toilet. This episode occasioned a range of outraged articles in Argentine dailies examining the topoi of privilege, power, corruption and impunity. “Distinguished persons,” notes Viau pointedly, “are so disposed […] that in the midst of all that chaos, they can locate a small, hard, steely object, wrap it in lavatory paper and flush it down the toilet, for that must be how they usually dispose of […] all that rubbish that no longer fits under the carpet.” Most often, though, critical comment was conducted by translating the reporting of the case to the genres of crime fiction. In an article entitled Someone Call Agatha Christie, Quick!, H.A.T. writes that “[s]omething smells rotten in the Carmel Country; a whole pile of rubbish seems to have been swept under its plush carpets.” An exemplary intervention in this vein was the work of journalist and novelist Vicente Battista, for whom the case (María Marta) “synthesizes the best of both traditions of crime fiction: the murder mystery and the hard-boiled novels.” “The crime,” Battista (¿Hubo Otra Mujer?) has Rodolfo observe in the first of his speculative dialogues on the case, “seems to be lifted from an Agatha Christie novel, but the criminal turns out to be a copy of the savage killers that Jim Thompson usually depicts.” Later, in an interview in which he correctly predicted the verdict, Battista expanded on these remarks: This familiar plot brings together the English murder mystery and the American hard-boiled novels. The murder mystery because it has all the elements: the crime takes place in a sealed room. In this instance, sealed not only because it occurred in a house, but also in a country, a sealed place of privilege. The victim was a society lady. Burglary is not the motive. In classic murder mystery novels, it was a bit unseemly that one should kill in order to rob. One killed either for a juicy sum of money, or for revenge, or out of passion. In those novels there were neither corrupt judges nor fugitive lawyers. Once Sherlock Holmes […] or Hercule Poirot […] said ‘this is the murderer’, that was that. That’s to say, once fingered in the climactic living room scene, with everyone gathered around the hearth, the perpetrator wouldn’t resist at all. And everyone would be happy because the judges were thought to be upright persons, at least in fiction. […] The violence of the crime of María Marta is part of the hard-boiled novel, and the sealed location in which it takes place, part of the murder mystery (Alarcón). I’ve argued elsewhere (Munro, Belsunce) that the translation of the case to the genres of crime fiction and their metaanalysis was a means by which a victimised Argentine public, represented by a disempowered and marginalised fourth estate, sought some rhetorical recompense. The postulate of resilience, however, might help further to describe and contextualise this notorious discursive event. A disaffected Argentine press finds itself in a stability domain with multiple attractors: on the one hand, an acquiescence to ever-increasing politico-juridical corruption, malfeasance and elitist impunity; on the other, an attractor of increasing contestation, democratisation, accountability and transparency. A discursive event like the caso Belsunce further perturbs Argentine society, threatening to displace it from its democratising trajectory. Unable to enforce due process, Argentina’s fourth estate adapts, doing what, in the circumstances, amounts to the next best thing: it denounces the proceedings by translating the case to the genres of crime fiction. In so doing, it engages a venerable reception history in which the co-constitution of true crime fiction and investigative journalism is exemplified by the figure of Rodolfo Walsh, whose denunciatory works mark a “politicisation of crime” (see Amar Sánchez Juegos; El sueño). Put otherwise, a section of Argentina’s fourth estate bounced back: by making poetics do rhetorical work, it resisted the pull towards what ecology calls an undesirable basin of attraction. Through a show of discursive resilience, these journalists worked to keep Argentine society on a democratising track. 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