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Background. Well-written and transparent case reports (1) reveal early signals of potential benefits, harms, and information on the use of resources; (2) provide information for clinical research and clinical practice guidelines, and (3) inform medical education. High-quality case reports are more likely when authors follow reporting guidelines. During 2011–2012, a group of clinicians, researchers, and journal editors developed recommendations for the accurate reporting of information in case reports that resulted in the CARE (CAse REport) Statement and Checklist. They were presented at the 2013 International Congress on Peer Review and Biomedical Publication, have been endorsed by multiple medical journals, and translated into nine languages.Objectives. This explanation and elaboration document has the objective to increase the use and dissemination of the CARE Checklist in writing and publishing case reports.Article design and setting. Each item from the CARE Checklist is explained and accompanied by published examples. The explanations and examples in this document are designed to support the writing of high-quality case reports by authors and their critical appraisal by editors, peer reviewers, and readers.Results and conclusion. This article and the 2013 CARE Statement and Checklist, available from the CARE website [www.care-statement.org] and the EQUATOR Network [www.equator-network.org], are resources for improving the completeness and transparency of case reports.Source. This article is a translation of the original paper «CARE guidelines for case reports: explanation and elaboration document» in the Journal of Clinical Epidemiology (doi: 10.1016/j.jclinepi.2017.04.026), prepared under the permission of the copyright holder (Elsevier Inc.), with supervision from the Scientific Editor by Professor E.G. Starostina, MD, PhD (translator) (Moscow, Russia). Present translation was first published in Digital Diagnostics. doi: 10.17816/DD105291. It is published with minor changes related to the literary editing of the translation itself. Keywords: case report; case study; EQUATOR network; health research reporting guidelines; CARE guideline; timelines; N-of-1 For citation: Riley David S., Barber Melissa S., Kienle Gunver S., Aronson Jeffrey K., von Schoen-Angerer Tido, Tugwell Peter, Kiene Helmut, Helfand Mark, Altman Douglas G., Sox Harold, Werthmann Paul G., Moher David, Rison Richard A., Shamseer Larissa, Koch Christian A., Sun Gordon H., Hanaway Patrick, Sudak Nancy L., Kaszkin-Bettag Marietta, Carpenter James E., Gagnier Joel J. CARE Guidelines for Case Reports: Explanation and Elaboration Document. Translation into Russian. Voprosy sovremennoi pediatrii — Current Pediatrics. 2023;22(2):88–108. (In Russ). doi: https://doi.org/10.15690/vsp.v22i2.2540

Since the establishment of the Journal of Medical Case Reports in 2006, the number of journals that publish case reports has increased rapidly, and most of these journals are open access. Open access publishing usually requires authors to pay publication fees while offering the articles online, free of charge, and free of most copyright and licensing restrictions. The movement for open access has gained support in the research community, with the publishers BioMed Central and PLOS ONE becoming leaders in scientific publishing in their number of articles and citations. As the number of open access publishers has exploded, so too has the number of publishers that act in bad faith to profit from the open access model. Simple guidelines have been developed and resources are available to help authors choose a suitable journal for publication of their case reports.

Since the establishment of the Journal of Medical Case Reports in 2006, the number of journals that publish case reports has increased rapidly, and most of these journals are open access. Open access publishing usually requires authors to pay publication fees while offering the articles online, free of charge, and free of most copyright and licensing restrictions. The movement for open access has gained support in the research community, with the publishers BioMed Central and PLOS ONE becoming leaders in scientific publishing in their number of articles and citations. As the number of open access publishers has exploded, so too has the number of publishers that act in bad faith to profit from the open access model. Simple guidelines have been developed and resources are available to help authors choose a suitable journal for publication of their case reports.

INTRODUCTION Chronic inflammatory demyelinating polyneuropathy (CIDP) is a progressive demyelinating neuropathy, which typically presents with proximal and distal neuropathic symptoms and is typically responsive to immunomodulatory therapies. Many variants have been subsequently described in the literature and have similarly shown to be responsive to immunotherapy. CASE PRESENTATION We present a case of a 43-year-old Middle Eastern/Arabic man presenting with symptoms of mixed sensorimotor neuropathy most evident at entrapment sites mimicking hereditary neuropathy with liability to pressure palsies. His electrodiagnostic study revealed features of acquired demyelinating neuropathy and a negative genetic workup. Alternative diagnosis of CIDP was considered in the context of symptomatic disease progression, negative genetic workup, and electrodiagnosis leading to initiation of immunotherapy with intravenous immunoglobulins. His neuropathy responded confirming our diagnosis of an inflammatory demyelinating polyneuropathy. CONCLUSIONS We describe a previously unknown variant of CIDP with phenotypic characteristics of hereditary neuropathy with liability to pressure palsies and its potential for successful treatment with intravenous immunoglobulins. This case illustrates an unusual presentation of CIDP mimicking hereditary neuropathy with liability to pressure palsies.

© 2017 Elsevier Inc. Background Well-written and transparent case reports (1) reveal early signals of potential benefits, harms, and information on the use of resources; (2) provide information for clinical research and clinical practice guidelines, and (3) inform medical education. High-quality case reports are more likely when authors follow reporting guidelines. During 2011–2012, a group of clinicians, researchers, and journal editors developed recommendations for the accurate reporting of information in case reports that resulted in the CARE (CAse REport) Statement and Checklist. They were presented at the 2013 International Congress on Peer Review and Biomedical Publication, have been endorsed by multiple medical journals, and translated into nine languages. Objectives This explanation and elaboration document has the objective to increase the use and dissemination of the CARE Checklist in writing and publishing case reports. Article Design and Setting Each item from the CARE Checklist is explained and accompanied by published examples. The explanations and examples in this document are designed to support the writing of high-quality case reports by authors and their critical appraisal by editors, peer reviewers, and readers. Results and Conclusion This article and the 2013 CARE Statement and Checklist, available from the CARE website [www.care-statement.org] and the EQUATOR Network [www.equator-network.org], are resources for improving the completeness and transparency of case reports.

Introduction: Exploding head syndrome (EHS) is a rare parasomnia in which affected individuals awaken from sleep with the sensation of a loud bang. The etiology is unknown, but other conditions including primary and secondary headache disorders and nocturnal seizures need to be excluded. Case Presentation: A 57-year-old Indian male presented with four separate episodes of awakening from sleep at night after hearing a flashing sound on the right side of his head over the last 2 years. These events were described ‘as if there are explosions in my head’. A neurologic examination, imaging studies, and a polysomnogram ensued, and the results led to the diagnosis of EHS. Conclusion: EHS is a benign, uncommon, predominately nocturnal disorder that is self-limited. No treatment is generally required. Reassurance to the patient is often all that is needed.

Neurology case reports have a long history of transmitting important medical information across many generations for the improvement of patient care. Case reports contribute much to the physician’s knowledge base from which treatment hypotheses and ideas form. Elements of a modern case report, as presented in the CARE (CAse REport) guidelines, include the abstract, introduction, case presentation, discussion, conclusion, patient’s perspective, and consent statement. The sections are described here, as well as the application of CARE guidelines to a published neuromuscular case report. Writing case reports offer an ideal opportunity for neurologists to publish interesting case findings and carry on the tradition of neurologic case reporting.

Background Neuropathy is a rare adverse side effect of disulfiram therapy and is under-recognized. There have been few case reports documenting this side effect. Case presentation Two cases of disulfiram peripheral neuropathy are discussed. The first case is that of a 25-year-old Caucasian woman who was exposed to disulfiram therapy for a total of 8 months and developed pain and stiffness that prevented her from walking. The second case is that of a 46-year-old Caucasian woman who developed sudden-onset numbness in her lower extremities with progression to pain. Her symptoms improved over the course of 2 months after cessation of disulfiram therapy. In both cases, symptoms improved after cessation of disulfiram therapy. Conclusions Disulfiram neuropathy occurs in persons with a history of chronic alcohol use. It is under-recognized and often attributed to alcoholic neuropathy given its comorbidity with alcoholic neuropathy. A greater understanding of this side effect may reduce neurologic complications related to disulfiram neuropathy and aid in early withdrawal of this offending agent.

Introduction: Transient global amnesia is a syndrome of temporary and reversible disruption of short-term memory accompanied by repetitive questioning. Although the etiology is unknown, the prognosis usually benign, and no particular treatment is required, it is important for all involved clinicians to recognize the diagnosis and possess knowledge about the evaluation of these affected patients. Case Presentation: A middle-aged Caucasian woman presented for neurologic evaluation for acute forgetfulness. Neurologic examination disclosed repetitive questioning with preserved orientation and no focal motor, speech, sensory, coordination, or cranial nerve deficits. Neurologic investigations did not reveal any pathologic findings. Her memory improved and reverted to normal baseline over the course of a 24-hour hospital stay. Conclusion: Transient global amnesia is an interesting syndrome of reversible anterograde amnesia associated with repetitive questioning that occurs with an unclear etiology in middle-aged and elderly individuals. Due clinical diligence is required in the investigation of these patients. Treatment is generally not required, and the condition usually does not recur. Clinicians, including neurologists, internists, family practice physicians, and psychiatrists, need awareness of this condition.

Introduction Bilateral phrenic neuropathy is an uncommon complication of diabetes mellitus. Case presentation We present a patient with diabetes mellitus who developed dyspnea from diaphragmatic paresis secondary to bilateral phrenic neuropathies on the background of subclinical generalized polyneuropathy documented by electrodiagnostic studies. The patient was treated with intravenous immunoglobulin with improved diaphragmatic function. Conclusion Phrenic neuropathy is a rare but important complication of diabetes mellitus. We report the first case of the beneficial use of intravenous immunoglobulin in diabetic phrenic neuropathy.

INTRODUCTION Delayed progressive cervicobulbar palsy with secondary lingual, facial, and neck myokymia after radiation therapy to the head and neck is a known but rare event with few reported case descriptions. CASE PRESENTATION We report a case with a video description of a young Asian woman who developed progressive dysarthria and dysphagia after approximately 1 year after radiation treatment for nasopharyngeal carcinoma. Her symptoms, clinical, and electromyographic findings are discussed and were felt to be secondary to a cervicobulbar neuronopathy secondary to the previous radiation she received. A brief literature review was performed and pathophysiological mechanisms and treatment options are discussed. CONCLUSION Delayed progressive cervicobulbar neuronopathy with myokymia is an uncommon complication of radiotherapy for nasopharyngeal carcinoma. Clinicians should be aware of this entity to prevent misdiagnosis with other neuromuscular and nonneuromuscular diseases.

Propofol is a widely used rapidly acting sedating or hypnotic agent in the intensive care setting. It is generally considered safe in both pediatric and adult patients and has been used frequently in cases of refractory status epilepticus. The formulation of propofol is highly lipophilic to facilitate central nervous system penetration and has a high fat content, and prolonged infusions have been known to cause both extrahepatic complications and hepatomegaly secondary to fatty liver. Whereas extrahepatic manifestations of prolonged propofol infusions have been well reported in non-neurologic intensive care patients, cases of pathologically confirmed fatty liver in patients with status epilepticus are relatively few. Furthermore, these cases of hepatomegaly and fatty liver have been also in the context of concomitant extrahepatic side effects. We report on a pediatric patient with refractory status epilepticus treated with a prolonged propofol infusion who developed isolated pathologically confirmed fatty liver without the usually reported extrahepatic manifestations.

Multifocal motor neuropathy (MMN) is a disorder of peripheral nerve often associated with a high monosialoganglioside (GM1) antibody and multifocal conduction block. It has a chronic, indolent course with involvement of predominantly peripheral motor nerves, usually in an asymmetric fashion. There have been few reported cases of progression to frank quadriplegia. Secondary amyloidosis refers to the deposition of amyloid in various tissues due to an underlying chronic inflammatory state. We report the first case, to our knowledge, of a patient with MMN associated with high titer of GM1 antibody who developed acute paraplegia with both cranial nerve and worsening sensory involvement associated with multiorgan compromise due to a secondary amyloidosis involving the myocardium.

Multifocal motor neuropathy (MMN) is a rare, treatable neuropathy, but good long-term outcomes are dependent on early treatment. Appropriate diagnostic criteria exist for MMN, but it has a heterogeneous clinical presentation, and, when confined to a small group of nerves, its clinical presentation can overlap with entrapment neuropathies. Electrodiagnosis is a useful diagnostic technique but occasionally MMN can be misdiagnosed as entrapment neuropathies. Misdiagnosis should rarely occur since the nerve involvement in MMN is usually not at sites of common nerve entrapment, and the impact of misdiagnosis can be substantial. The treatments of these conditions differ significantly: intravenous immunoglobulin is the standard therapy for MMN, while treatment options for entrapment neuropathies include conservative treatment, such as splinting, corticosteroids, or surgery. Such treatment in MMN may worsen symptoms, potentially leading to progressive motor symptoms, including muscle weakness, atrophy, and significant motor disability. Two exemplary cases are presented, which highlight the importance of differential diagnosis of these conditions. Emerging technologies, such as high-resolution sonography and magnetic resonance neurography, will aid in defining future diagnostic criteria of MMN.

From antiquity to present day, the act of recording and publishing our observations with patients remains essential to the art of medicine and the care of patients. Neurology is rich with case reports over the centuries. They contribute to our understanding and knowledge of disease entities, and are a cornerstone of our professional development as physicians and the care of our patients. This editorial seeks to enthuse and invigorate house staff and practicing physicians everywhere to continue the long and time-honored tradition of neurology case reporting.

Introduction Giant cell arteritis is an emergency requiring prompt diagnosis and treatment. Superficial temporal artery biopsy is the gold diagnostic standard. Complications are few and infrequent; however, facial nerve injury has been reported, leaving an untoward cosmetic outcome. This case report is to the best of our knowledge only the fourth one presented in the available literature so far regarding facial nerve injury from superficial temporal artery biopsy. Case presentation A 73-year-old Caucasian woman presented for neurological evaluation regarding eyebrow and facial asymmetry after a superficial temporal artery biopsy for presumptive giant cell arteritis-induced cephalalgia. Conclusion Damage to branches of the facial nerve may occur after superficial temporal artery biopsy, resulting in eyebrow droop. Although an uncommon and sparsely reported complication, all clinicians of various specialties involved in the care of these patients should be aware of this given the gravity of giant cell arteritis and the widespread use of temporal artery biopsy.

The effects of amygdala, hippocampus, and periaqueductal gray (PAG) lesions on contextual fear conditioning in rats were examined. Freezing behavior served as the measure of conditioning. Unlesioned control animals showed reliable conditional freezing in the testing chamber when observed both immediately and 24 hr after footshocks. In contrast, rats with amygdala or ventral PAG lesions exhibited a significant attenuation in freezing both immediately and 24 hr after the shocks. Dorsal PAG lesions had no effect on freezing at either time. Animals with hippocampal lesions displayed robust freezing behavior immediately following the shock, even though they showed a marked deficit in freezing 24 hr after the shock. These results indicate that there are anatomically dissociable short- and long-term conditional fear states.

A 39-year-old woman with nasopharyngeal carcinoma underwent partial resection along with head and neck radiation therapy. Approximately 10 months later she developed …

Background Acute inflammatory polyradiculoneuropathy secondary to chemotherapy for leukemia has been described in the pediatric literature. However, the reports are rare and have been mainly from intrathecal methotrexate in pediatric acute lymphoblastic leukemia patients who developed demyelinating polyradiculoneuropathy. Case presentation A case report is presented of an unfortunate 53 year old Hispanic woman with acute myelogenous leukemia who developed profound weakness with cranial nerve palsies following both intravenous and intrathecal chemotherapy. Conclusion This is an interesting and unusual case of predominantly axonal ascending sensory motor polyradiculoneuropathy with cranial nerve involvement in an adult patient with acute myelogenous leukemia following intravenous Cytosine arabinoside and intrathecal methotrexate.

Moyamoya phenomenon is a term used to describe extensive collateralization of the circle of Willis arteries associated with severe unilateral or bilateral internal carotid artery stenosis or occlusion in the presence of certain conditions. Down syndrome is among these conditions. A case is reported of a young girl with Down syndrome who presented with fluctuating right-sided weakness and facial droop found to have cerebral ischemia. Subsequent investigations disclosed characteristic "puff of smoke" patterns on angiographic studies consistent with moyamoya phenomenon. The patient was initially treated with aspirin and eventually underwent an encephalomyosynangiosis. This young patient with Down syndrome and moyamoya phenomenon serves as a reminder of the association between these two conditions.

Mitochondrial myopathies are heterogeneous disorders with diverse presentations including laboratory findings of lactic acidosis. Often times they are diagnosed in childhood or the early teenage years following an infectious illness. Minocycline is a common antibiotic used for the treatment of acne. It has been reported to alter mitochondrial respiratory chain complexes. We report an interesting case of a teenager in which mitochondrial myopathy with severe lactic acidosis presented following a bout of infectious mononucleosis and minocycline use. It is hypothesized that oxidative stress from the infectious illness plus the minocycline use triggered the patient's presentation. The clinical manifestations and genetics of mitochondrial myopathies and treatment are discussed along with the management of lactic acidosis.

Introduction May-Thurner syndrome is an anatomic abnormality that predisposes patients to increased risk of paradoxical embolism and stroke. It consists of chronic compression of the left common iliac vein by the overlying right common iliac artery which may predispose to local thrombus formation, which in turn may be the nidus of a paradoxical embolus leading to cerebral ischemia in patients with a right-to-left shunt secondary to an atrial septal defect or patent foramen ovale. Case presentation We report the case of an embolic cerebral ischemic event in a 53-year-old Caucasian woman whose investigations revealed findings suggestive of possible May-Thurner syndrome coupled with an atrial septal defect. Her atrial septal defect was closed, she was placed on aspirin therapy, and has not had any recurrent events. Conclusion May-Thurner syndrome is an important consideration in patients with paradoxical embolic cerebral ischemia and atrial septal defects.

Introduction Patients with polycythemia vera are at high risk for vaso-occlusive events including cerebral ischemia. Although unusual, acute ischemic stroke may be an initial presentation of polycythemia vera. It had been previously assumed that cerebral ischemic events were due to increased blood viscosity and platelet activation within the central nervous system arterial vessels. However, there are now a few isolated case reports of probable micro-embolic events originating from outside of the brain. This suggests unique management issues for these patients. Case presentation We present the case of a 57-year-old right-handed Caucasian male in excellent health who presented to the Emergency Department with acute right-handed clumsiness. Hematologic investigations revealed a hyperviscous state and magnetic resonance imaging was consistent with cerebral emboli. Symptoms rapidly improved with phlebotomy and hydration. Conclusion The etiology of stroke in polycythemic patients is likely to be multifactorial. While hemodilution has been generally discredited for general stroke management, it is potentially beneficial for patients with polycythemia vera and euvolemic hemodilution should be considered for the polycythemic patient with acute cerebral ischemia.

A 50-year-old man with a history of recurrent optic neuritis and past thoracic transverse myelitis presented with rapidly progressive quadriparesis, urinary incontinence, dyspnea, dysphagia, dysarthria, intractable hiccoughs, nausea, vomiting, and blurry vision. MRI revealed prominent demyelinating findings at the upper cervical cord and pontomedullary junction …

Bulbar weakness and fatigue resulting in dysphagia and dysarthria is common in myasthenia gravis. In chronic MG it is often assumed that these symptoms herald an exacerbation of the patient's disease and doses of cholinergic agents and immunomodulatory therapies may be increased, along with initiation of plasma exchange. A case is presented in which dysphagia was refractory to standard MG therapy, leading to the subsequent discovery of cricopharyngeal sphincter achalasia as the primary cause of the patient's symptoms rather than an assumed myasthenia gravis exacerbation. The patient's dysphagia resolved after esophageal dilatation. Cricopharyngeal sphincter achalasia is a common disorder producing dysphagia in the elderly and needs to be considered in the evaluation of a myasthenic patient with worsening dysphagia when standard myasthenia gravis therapy fails. Discussion of myasthenia gravis, cholinergic therapy and cricopharyngeal sphincter achalasia is undertaken. Clinicians are encouraged to consider non-neurologic causes of worsening dysphagia in the myasthenic patient.

A Hispanic man presented with a 3-month history of progressive weakness of the arms and legs. Exam was remarkable for bilateral papilloedema (fig 1), hypertrichosis and thickened skin of the arms and hands (fig 2), diminished deep tendon reflexes, splenomegaly and anasarca. Investigations revealed a homogenous band in the …

Introduction Clinical presentation of syringomyelia can mimic a variety of neuromuscular disorders. A misdiagnosis can result in progressive pressure on the spinal cord, causing the development of severe irreversible neurologic deficits. Case presentation We report the very unusual case of a 50-year-old Latino man who developed severe distal muscle atrophy and bulbar dysfunction as a result of Chiari malformation type I with chronic cervicothoracic syringomyelia. Conclusion Syringomyelia is a potentially serious neurologic condition with symptoms that can mimic other neuromuscular disorders. Severe untreated cases can result in irreversible spinal cord injury. Prompt diagnosis with magnetic resonance imaging is important in both establishing diagnosis and directing further surgical management.

Introduction Approximately ten to fifteen percent of patients with myasthenia gravis are found to have a thymoma, and twenty to twenty-five percent of patients with thymoma have myasthenia gravis. Thymomatous myasthenia gravis tends to have a difficult clinical course and poor prognosis. Case presentation We report two cases (one patient of Asian ethnicity and the other of Caucasian ethnicity) of atypical presentations of myasthenia gravis associated with invasive malignant thymoma. Both patients were diagnosed at a young age, in their 20s. They presented with a turbulent course of myasthenia gravis and recurrent thymoma, but obtained good outcome after aggressive treatment involving multiple different specialists. Conclusions Although thymomatous myasthenia gravis tends to have a difficult clinical course and poor prognosis, early and aggressive treatment along with multidisciplinary management may improve the outcome of these patients.

Case report publication has seen a resurgence in recent years as awareness of the value of case reports in clinical medicine has grown. Not all areas of medical research are amenable to large clinical trials. Many topics are better addressed by more detailed descriptions of multi-factorial components that contribute to outcomes, and these are areas where case reports shine. Determining the suitability of a case for publication requires background research and discussion. Writing a case or series reinforces many aspects of the medical training process, and house staff are encouraged to research, write, and submit reports. The medical community benefits in many ways from case reports, from improving individual patient care to guiding future research directions.

Introduction Sporadic inclusion body myositis is the most common adult myopathy in persons aged 50 years and older. The clinical presentation includes a chronic, slowly progressive course with a predilection for weakness of the forearm flexors and quadriceps muscles. Its indolent course makes it a disease frequently missed or misdiagnosed as other neuromuscular conditions by health care professionals. The degenerative processes with amyloid accumulation distinguish sporadic inclusion body myositis from other inflammatory myopathies. Currently, no effective therapy exists. This clinical report highlights the difficulties in diagnosing the disease, examples of misdiagnosis, and inappropriate therapies that can result from misdiagnosis. Case presentation We present our clinical experience with 20 patients over a 10-year period and describe in depth two cases, both men, one of Indian ethnicity and the other of Hispanic ethnicity, who were referred to our neuromuscular division for second opinions and diagnosed with sporadic inclusion body myositis years after symptom onset. Conclusions Although sporadic inclusion body myositis is rare and without effective therapy, accurate diagnosis is crucial to providing adequate counseling and information about the prognosis and disease course, and to avoiding inappropriate therapy.

Background Polymyositis is an immune-mediated myopathy with clinical features of proximal muscle weakness. Dysphagia and neck flexor weakness can develop along with respiratory muscle weakness as the disease progresses. Kennedy disease or X-linked spinobulbar muscular atrophy is a rare X-linked recessive disorder with clinical features of slowly progressive atrophy and weakness of limb and bulbar muscles. These two disorders may have overlapping clinical manifestations. Case presentation We present the case of a 52-year-old Filipino man with chronic weakness involving his proximal muscle groups who carried the diagnosis of polymyositis and was refractory to multiple immunomodulatory therapies. Further neurologic examination and history taking along with selective serologic and electrodiagnostic studies instead confirmed the diagnosis of Kennedy disease. Conclusions Distinction between polymyositis and Kennedy disease may be difficult given the potential overlapping clinical manifestations. However, with careful neurological history taking, examination, and selective serologic plus electrodiagnostic investigations the correct diagnosis may be made, thus sparing the patient ineffective therapy. One must always be sure of the diagnosis of polymyositis before it’s classified as refractory.