bluuebunny/crossref_metadata_2025 huggingface dataset:
Electrodiagnostic Findings in Neuromuscular Disorders
Authors: Katirji Bashar
Published: October 2018

Neuromuscular disorders are often classified into four major categories: anterior horn cell disorders, peripheral neuropathies, neuromuscular junction disorders and myopathies. This chapter discusses the electrodiagnostic and clinical EMG findings in these various neuromuscular disorders. Peripheral neuropathies are subdivided into focal mononeuropathies, radiculopathies, plexopathies and generalized peripheral polyneuropathies. Focal peripheral nerve lesions and generalized peripheral polyneuropathies may be axonal or demyelinating, and manifest quite distinctly on nerve conduction studies. Neuromuscular junction disorders may be presynaptic, as seen with the Lambert-Eaton myasthenic syndrome, or postsynaptic, as seen with myasthenia gravis.
Case 1
Authors: Katirji Bashar
Published: October 2018

Peripheral nerve injuries are most prevalent in young adults and are sometimes overlooked by medical staff caring for trauma patients. They are classified based on damage to myelin or axon and degree of supporting structures injury. The diagnosis of these injuries is often aided by electrodiagnostic studies. The case illustrates a patient with peripheral nerve injury (more specifically, a common peroneal [fibular] nerve injury) and highlights the anatomy of peripheral nerve and classification of peripheral nerve injury. It emphasizes the role and challenges of electrodiagnostic studies, including nerve conduction studies and needle electromyography, in the diagnosis, localization, prognosis, and management of peripheral nerve injury.
Case 4
Authors: Katirji Bashar
Published: October 2018

Femoral neuropathy is the second most common peripheral nerve lesion in the lower extremity and often leads to significant disability and high risk of falls. This case presents a patient with severe femoral nerve injury resulting in weakness of hip flexion and knee extension and requiring specialized knee bracing. A discussion of the anatomy of the femoral nerve follows. Common causes of femoral neuropathy are lithotomy positioning and retroperitoneal/iliacus hematoma. This case highlights the challenging electrodiagnostic findings of the femoral nerve and the role of femoral nerve conduction studies in prognosis. It also attempts to clarify the distinguishing clinical and electrodiagnostic features between overlapping disorders, including femoral neuropathy, upper lumbar radiculopathy, and lumbar plexopathy.
Case 10
Authors: Katirji Bashar
Published: October 2018

Axillary nerve lesions are relatively uncommon peripheral nerve lesions most often encountered after trauma to the shoulder including dislocation and fracture. They are often confused and may be difficult to distinguish clinically from cervical radiculopathy and shoulder joint or tendon pathology, such as rotator cup tear and impingment syndrome. Axillary mononeuropathy may be also a component of neuralgic amyotrophy (acute brachial neuritis, Parsonage-Turner syndrome). This case discusses anatomy of the axillary nerve and highlights the clinical and electrodiagnostic manifestations of axillary mononeuropathy. It also distinguishes axillary nerve lesions from posterior cord brachial plexopathy, upper trunk brachial plexopathy, and C5 and C6 radiculopathies.
Case 12
Authors: Katirji Bashar
Published: October 2018

Radial nerve lesion is the third most common mononeuropathy of the upper extremity seen in clinical practice, often presenting with wrist drop. The radial nerve has a long course in the arm with close contact to the humerus, which renders it susceptible to compression and trauma. This case highlights the clinical and electrodiagnostic findings of radial nerve lesion. The radial nerve has well distributed motor branches along its course which renders it a well suited nerve for localization by needle electromyography. This case also distinguishes radial nerve lesions from posterior interosseous nerve lesions, posterior cord plexus lesions, and lower cervical radiculopathies.
Case 19
Authors: Katirji Bashar
Published: October 2018

Amyotrophic lateral sclerosis is a fatal neurological disorder, classically presenting with signs of upper motor neuron and lower motor neuron degeneration. Several motor neuron disease variants with purely upper or lower motor neuron degeneration exist. These includes primary lateral sclerosis, progressive muscular atrophy and progressive bulbar palsy. The diagnostic criteria, including El-Escorial criteria and its most recent Awaji revision, are not used in clinical practice and for research purposes. This case highlights the clinical features and electrodiagnostic characteristics of amyotrophic lateral sclerosis. The findings on nerve conduction studies and needle electromyography are emphasized in detail. The role of electrodiagnostic studies in the diagnosis of amyotrophic lateral sclerosis is to establish evidence of lower motor neuron degeneration, confirm its diffuse nature, and exclude treatable causes (such as multifocal motor neuropathy and mimickers of motor neuron disease such as chronic myopathies).
Case 24
Authors: Katirji Bashar
Published: October 2018

Botulism is an extremely rare neuromuscular disorder, caused by botulinum toxin which is produced by the anaerobic bacteria Clostridium botulinum. It has several forms: classic foodborne, infantile, wound, intestinal, and iatrogenic forms. The presentation is often acute and severe but may be occasionally subacute and moderate. The diagnosis may be difficult and requires a high index of suspicion. This case presents an adult with classic foodborne botulism and highlights the clinical and electrodiagnostic findings that distinguish this disorder from other neuromuscular junction disorders including myasthenia gravis and Lambert-Eaton myasthenic syndrome. Specifically, the findings on repetitive nerve stimulation are discussed and distinguished from the results seen in other neuromuscular junction disorders.
A Case of Monocular Blurred Vision
Authors: Miller Aaron E. , DeAngelis Tracy M. , Fabian Michelle , Sand Ilana Katz
Published: October 2018

Multiple sclerosis (MS), an inflammatory demyelinating disease of the central nervous system, typically affects young to middle-aged adults. Women are affected nearly three times as often as men. The diagnosis requires the demonstration of dissemination in space (DIS) and time (DIT) in a patient with no better clinical explanation. Evidence for either DIS or DIT or both may now be obtained from MRI, in addition to clinical manifestations. The most widely used diagnostic criteria are known as the McDonald criteria, and have been revised several times, most recently in 2017. In the current criteria, DIS may be achieved by the demonstration of at least one lesion in at least two different locations: periventricular, cortical/juxtacortical, infratentorial, or spinal cord. DIT may be considered in an initial magnetic resonance imaging (MRI) if there are simultaneously at least one gadolinium-enhancing lesion and one non-enhancing T2 hyperintense lesion; or alternatively, by the demonstration of a new lesion on any MRI subsequent to the first. The course of MS is characterized as relapsing-remitting (RRMS), secondary progressive, or primary progressive. Many effective disease-modifying agents are available for RRMS, but progressive forms have been much less successfully treated.
Irritability in a Man with Multiple Sclerosis
Authors: Miller Aaron E. , DeAngelis Tracy M. , Fabian Michelle , Sand Ilana Katz
Published: October 2018

Natalizumab is a highly effective therapy used for the treatment of relapsing forms of MS. However, use of the drug has been complicated by the occurrence of progressive multifocal leukoencephalopathy (PML), a disease caused by the John Cunningham (JC) virus. The risk of PML in MS is almost exclusively confined to individuals who test positive for JC virus antibody by the STRATIFY assay. Additional risk factors in JC virus antibody-positive patients include the duration of therapy and the prior use of immunosuppressive medications. Although symptoms and signs of PML can mimic those of MS, personality change, cognitive dysfunction, language disturbance, and hemiparesis are common early findings in PML. As soon as the suspicion of possible PML arises, natalizumab should be discontinued. MRI of the brain should be obtained. The appearance of any new brain lesions on MRI obtained after six months of natalizumab treatment should raise the question of PML. The diagnosis is usually confirmed by the identification of JC virus in the cerebrospinal fluid (CSF) by polymerase chain reaction. However, this test should be obtained in a laboratory that is capable of determining low copy numbers of the viral DNA. Treatment also includes plasma exchange to speed the clearance of the monoclonal antibody. Many patients develop the immune reconstitution inflammatory syndrome (IRIS) when the natalizumab is discontinued. This may entail neurological deterioration, necessitating intervention with high-dose intravenous methylprednisolone.
Double Vision and Droopy Eyelids
Authors: Miller Aaron E. , DeAngelis Tracy M. , Fabian Michelle , Sand Ilana Katz
Published: October 2018

Myasthenia gravis is a disorder manifest by fluctuating neurological symptoms, most commonly occurring in younger women or older men. Ocular manifestations, including ptosis and double vision, are particularly frequent. However, bulbar symptoms are also common, and weakness of the extremities may occur. Respiratory impairment may be life-threatening. MG most often results from a loss of functional acetylcholine receptors (AChR) on the postsynaptic membrane of the neuromuscular junction caused by the presence of antibodies to the AChR. The serological demonstration of these antibodies most frequently establishes the diagnosis. Occasional patients, particularly with atypical presentations, are negative for anti-AChR antibodies but demonstrate the presence of anti-muscle specific receptor tyrosine kinase (MuSK) antibodies or lipoprotein receptor-related protein 4 (LRP4). Demonstration of a decremental response of greater than 10% to repetitive nerve stimulation (RNS) is strong evidence for the diagnosis of MG, but this finding is usually absent in patients with purely ocular myasthenia. Management of patients includes symptomatic treatment with acetylcholine esterase inhibitors and immunotherapy including corticosteroids, plasma exchange, or intravenous immunoglobulin, and immunosuppressive agents. A recent randomized, controlled trial has demonstrated the benefit of trans-sternal thymectomy in appropriate patients.
A Headache Gone Viral
Authors: Miller Aaron E. , DeAngelis Tracy M. , Fabian Michelle , Sand Ilana Katz
Published: October 2018

Giant cell (temporal) arteritis (GCA) is an important cause of new-onset headaches in patients over age 50. GCA is the most common cause of vasculitis in the elderly, and left untreated, risks permanent vision loss, strokes, even death. The hallmark of the condition is a new-onset temporal headache and elevated ESR, often accompanied by jaw claudication and systemic symptoms of fever, malaise, and anorexia. If clinically suspected, corticosteroids should be started immediately to prevent vision loss, which once present is generally severe and permanent. Recently, varicella zoster virus (VZV) has been identified in temporal artery biopsy specimens of GCA patients, suggesting a potential key role of the virus in the immunopathogenesis of this disease. VZV infection is a well-known cause of various central nervous system conditions ranging from viral meningitis to vasculitis complicated by ischemic and hemorrhagic strokes. Establishing a definitive causal relationship between VZV and GCA has been controversial, but a viral vasculopathy has been postulated. This would have important therapeutic implications for GCA such as standardized use of antiviral therapies in addition to corticosteroids and potentially shortening duration of steroid therapy.
The Unforgettable Encephalitis
Authors: Miller Aaron E. , DeAngelis Tracy M. , Fabian Michelle , Sand Ilana Katz
Published: October 2018

Leucine-rich glioma-inactivated 1 (LGI1) limbic encephalitis is an autoimmune encephalitis characterized by subacute cognitive impairment, amnesia, seizures, faciobrachial dystonic seizures (FBDS), and hyponatremia. MRI of the brain typically demonstrates abnormal T2/FLAIR hyperintense signal in the mesial temporal lobe, hippocampal regions. CSF is surprisingly bland, and laboratory investigations often disclose a hyponatremia. Patients with LGI1 encephalitis generally respond to immunotherapies such as glucocorticoids, intravenous immunoglobulin (IVIg), and plasma exchange and steroid-sparing immunosuppressive agents. The etiology of LGI1 encephalitis can be either autoimmune or paraneoplastic, more commonly the former. Reported prognosis is favorable in about two-thirds of cases; however, some patients can relapse, and long-term outcome can be poor if diagnosis is delayed and residual memory impairment from hippocampal damage occurs.
The Peppered Brainstem
Authors: Miller Aaron E. , DeAngelis Tracy M. , Fabian Michelle , Sand Ilana Katz
Published: October 2018

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare inflammatory syndrome of the CNS. Patients present with subacute, progressive symptoms that usually localize to the brainstem. MRI shows a characteristic appearance of diffuse involvement that is greatest in the pons and surrounding areas and is associated with a “peppered” pattern of gadolinium enhancement. Pathologically, CLIPPERS is characterized by the perivascular and parenchymal infiltration of CD4+ T cells as well as by B cells and activated microglia. Patients with CLIPPERS generally respond to steroid treatment but are at high risk for rebound when steroids are discontinued. Long-term immunosuppression is often required in order to avoid relapse. The prognosis for CLIPPERS is variable, though many good outcomes have been reported.
Checks and Balances
Authors: Miller Aaron E. , DeAngelis Tracy M. , Fabian Michelle , Sand Ilana Katz
Published: October 2018

In recent years, immune checkpoint inhibitors have been shown to be effective tools in treating multiple types of cancer. In a healthy person, immune checkpoint molecules function to remove self-reactive T cells. However, cancer cells may also use these same pathways to suppress T cells. Checkpoint inhibition brings about non-specific T cell activation, which allows for a tumor-specific immune response. However, an infrequent, but potentially severe, complication of checkpoint inhibitor therapies is the formation of autoimmune conditions. Cases of new or worsened autoimmune conditions have been reported in many organ systems. In the nervous system, central and peripheral processes, as well as disorders of the neuro-muscular junction or muscle, have been identified. Treatment is targeted towards the acute episode. Chronic immunosuppression is usually not initiated because stopping the checkpoint inhibitor therapy will typically abort the autoimmune process. In the future, more of these drugs are likely to be used to treat cancer, and thus more must be known about the mechanisms that induce autoimmunity in this setting and the best strategies to treat them.
A Case of Cognitive Change in HIV
Authors: Miller Aaron E. , DeAngelis Tracy M. , Fabian Michelle , Sand Ilana Katz
Published: October 2018

Progressive multifocal leukoencephalopathy (PML) is caused by CNS infection with the John Cunningham (JC) virus. It occurs exclusively in patients who are immunosuppressed or under treatment with particular immunomodulatory therapies. Despite advances in antiretroviral therapy (ART), PML remains an issue in HIV patients with drug resistance or with poor access or compliance to ART. PML typically presents with the subacute onset of focal neurological symptoms and/or cognitive/personality changes, dependent upon the location of the lesion(s). MRI usually shows a poorly demarcated T2 hyperintensity, which may or may not enhance with gadolinium. Diffusion restriction is common. CSF profile is typically bland, often with mildly elevated protein. A positive CSF JC virus PCR is diagnostic, but sensitivity is suboptimal, leading to brain biopsy in patients where the diagnosis is not clear. No specific antiviral treatments have been proven effective for the treatment of PML. ART should be initiated or optimized in patients with HIV, as this has been demonstrated to improve outcomes. Some HIV patients treated with ART will develop immune reconstitution inflammatory syndrome (IRIS), which usually is associated with improved prognosis but, in and of itself, may be fatal. Steroid treatment of IRIS remains controversial.
A Case of Diabetes Insipidus and Enhancing Brain Lesions
Authors: Miller Aaron E. , DeAngelis Tracy M. , Fabian Michelle , Sand Ilana Katz
Published: October 2018

Erdheim-Chester disease (ECD) is a rare non-Langerhans histiocytosis that typically involves several organ systems. Approximately 50% of patients have CNS involvement. Symptoms are related to localization of lesions and may include diabetes insipidus or other endocrinopathy due to pituitary involvement, focal neurological symptoms due to parenchymal lesions, compressive symptoms related to pachymeningitis, or general worsening of gait and cognition when lesions are more widespread. Infiltration of the orbits may also occur, resulting in oculomotor abnormalities, retro-orbital pain, exophthalmos, or visual compromise. Osteosclerosis of the long bones seen on imaging is nearly universal. In addition, involvement of the cardiovascular and pulmonary systems, kidneys and retroperitoneum, and skin may occur. The diagnosis is made when pathological findings consistent with ECD are noted in a patient with clinical and imaging findings suggestive of this entity. Interferon-alfa is the most well-studied therapy for ECD; however, suboptimal efficacy and side effects have led to exploration of alternative agents. B-rapidly accelerated fibrosarcoma protein (BRAF) inhibitors seem particularly promising and are currently being studied in clinical trials.
A Case of Optic Neuritis and Leg Weakness
Authors: Miller Aaron E. , DeAngelis Tracy M. , Fabian Michelle , Sand Ilana Katz
Published: October 2018

Mononeuritis multiplex, a condition characterized by the involvement of multiple individual peripheral (and sometimes cranial) nerves, is frequently caused by vasculitides of various types. Vasculitides affect various-sized blood vessels. Among these are the ANCA-associated small vessel vasculitides, which include eosinophilic granulomatous polyangiitis (EGPA), granulomatous polyangiitis, and microscopic polyangiitis. Asthma and nasal polyposis are suggestive of the diagnosis of EGPA, and eosinophilia is a hallmark. Multiple systemic organs may be affected, and involvement of both the central and peripheral nervous should always raise the possibility of vasculitis. The cornerstone of treatment is corticosteroids, but other immunosuppressant agents, most often cyclophosphamide, are frequently needed, especially when organ or life-threatening manifestations are present.
A Man with Multiple Sclerosis and Continuing Disease Activity on Disease-Modifying Therapy
Authors: Miller Aaron E. , DeAngelis Tracy M. , Fabian Michelle , Sand Ilana Katz
Published: October 2018

The decision about whether to switch a patient with multiple sclerosis from one disease-modifying therapy (DMT) to another in the context of disease activity is often difficult. Controlled studies to guide the decision are lacking, and clinicians may differ on the criteria. In addition, the decision about which alternative DMT to choose may vary from one individual situation to another. Some guidance is available from data examining the subsequent behavior of patients taking beta interferon based on their level of disease activity at one year of treatment. Once a decision to switch therapy is made, the patient should avoid a prolonged period off therapy. Special consideration is necessary with certain specific switches.
Progressive Numbness and Tingling
Authors: Miller Aaron E. , DeAngelis Tracy M. , Fabian Michelle , Sand Ilana Katz
Published: October 2018

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a subacute progressive polyneuropathy thought to arise from an autoimmune origin. Patients most typically present with symmetrical, progressive weakness that often affects proximal and distal muscles equally. Sensory involvement is common. Confirmation of the likelihood of demyelinating polyneuropathy can be obtained by electrodiagnostic study, which should include all four limbs. Marked slowing of conduction velocity is found in the vast majority of cases. Electromyography will typically show signs of denervation and partial reinnervation, such as fibrillation potentials and giant, long-duration, polyphasic potentials. Corticosteroids, intravenous immunoglobulins, or plasmapheresis are the most typical first-line treatments for CIDP. Steroid-sparing immunosuppressants may be used if chronic therapy is necessary. Most patients will improve to some degree with treatment.
Neuroscience at the Intersection of Mind and Brain
Authors: Gorman Jack M.
Published: October 2018

This book makes complicated concepts and findings in modern neuroscience accessible to anyone with an interest in how the brain works. It explains in detail how every experience we have from the moment we are conceived changes our brains. Finally, it advances the idea that psychotherapy is a type of life experience that alters brain function and corrects aberrant brain connections. The chapters explore what makes our brains different from our nearest genetic neighbors; how life’s experiences affect the way genes in the brain are expressed and neurons connect with each other; why connections between different parts of the brain are important in both health and disease; what happens in the brains of animals and humans in the face of sudden fear, in depression, or when falling in love; and how medications and psychotherapies work. The book is based on cutting-edge research in neuroscience, psychiatry, and psychology and includes references to the scientific literature. Written by an author who studied human behavior and brain function for three decades, it is presented in a highly accessible manner, full of personal anecdotes and observations, and it touches on many of the controversies in contemporary mental health practice.
Introduction
Authors: Gorman Jack M.
Published: October 2018

After World War II, mental health turned toward psychopharmacology, the use of medications to treat psychiatric illnesses, as its mainstay. The success of medications led some to insist that all mental illness is due to the inheritance of abnormal genes and that life’s experiences play a diminished role. This alienated many who believe that psychotherapy is also an effective way of treating these disorders and led to a mistrust of neuroscience research. Some insisted that neuroscience ignores the human “mind.” In fact, neuroscience research in the past 50 years has clearly shown that adverse life experiences have profound effects on brain function and are involved in every psychiatric illness. By accepting this view of neuroscience, we can also accept the idea that the “mind” is in reality the work of the physical brain.
Sadness and Depression
Authors: Gorman Jack M.
Published: October 2018

Two cellular processes are affected by stressful life events: dendritic remodeling and neurogenesis. Dendritic remodeling occurs when dendrites—projections from neurons that receive signals from the axons of other neurons—either grow or retract. Stress causes retraction of dendrites and disrupts brain connections. This may occur when people are depressed and is responsible for the inability of the logical brain to communicate with the emotional brain. Psychotherapy and antidepressant medications may both cause the dendritic tree to grow and restore connections. Neurogenesis is the creation of new neurons in the adult brain, which occurs in only a few regions. Neurogenesis is decreased by adverse events and increased by enriched environments and by antidepressant treatments. Thus, cellular brain processes that respond to our environment may underlie depression and other changes in mood.
Making Connections
Authors: Gorman Jack M.
Published: October 2018

Although some functions, like speech and vision, can be linked to single, specific locations in the brain, complex emotions and behaviors usually involve complex interactions among brain regions. As our brains mature, these connections are shaped by our lived experiences. Scientists in basic neuroscience laboratories have traced the pathways and networks necessary for the acquisition, expression, and extinction of one emotion: fear. Brain imaging studies have shown that these same connected brain regions are activated by fear and anxiety in humans. The “fear network” includes the amygdala, hippocampus, and prefrontal cortex. Abnormalities in activity and strength of connections in the fear network are present in children and adults with anxiety disorders and depression. Brain networks that are necessary for other emotions and behaviors have been identified, so that today we look to how our brains are connected to understand our actions and emotions.
Conclusion
Authors: Gorman Jack M.
Published: October 2018

This chapter concludes the book’s discussion of how experience changes the brain. It summarizes the positions that have been taken in the preceding chapters: that the human brain is unique even in comparison to our nearest genetic neighbors in the great ape family, that the mind is the organ of the brain and all of its functions can be explained by molecular activity within the brain, and that psychotherapy is an experience that changes the brain. It provides a discussion on how neuroscience and psychology are fundamentally intertwined, linking the mind and brain, and it emphasizes the rapidly evolving, changing nature of the field of neuroscience and argues the need for more research linking psychotherapy with neurobiology.
Assessment
Authors: Mee Sarah , Clift Zoe
Published: October 2018

Meticulous assessment—both subjective and objective—is an essential part of managing hand conditions. Objective measures include range of motion, power grip, pinch grip, oedema, and dexterity testing. Subjective measures include a clinical history, sensibility, and sensation testing, pain scales, and Patient Related Outcome Measures (PROMS), global (e.g. EQ5D, VAS pain), region specific (e.g. Michigan Hand Questionnaire, Disabilities of the Hand, Arm, Shoulder, QuickDASH, Patient Related Wrist and Hand Evaluation), and condition specific (e.g. Boston Carpal Tunnel Questionnaire).
Hand Therapy
Authors: Mee Sarah , Clift Zoe
Published: October 2018

Rehabilitation is a multidisciplinary, patient-centred, evidence-based process to promote healing, restore function, and promote independence. The physical and psychological and social consequences of the hand condition or injury have to be considered. Mobilization can be active or passive, supplemented by accessory movements and proprioceptive rehabilitation. Splinting may be static, serial static, static progressive, dynamic. Many materials are available. Oedema may be acute or chronic; it is treated with elevation, active movement, retrograde massage, compression, kinesiotaping, cold therapy, and contrast bathing. Scars may be mature or immature; keloid or hypertrophic. Management is generally empiric: massage, silicone, pressure therapy, steroid injections, and surgery all have roles. Hypersensitivity (allodynia, causalgia, dysaesthesia, hyperpathia, etc.) is treated with desensitization, graded textures, percussion, and mirror visual feedback. Stiffness is managed especially by prevention; movement, splinting, and surgery have a role. Pain is treated with medication, oedema control, acupuncture, TENS, education, psychological measures. Complex Regional Pain Syndrome has sensory, vasomotor, sudomotor, and trophic elements. Treatment includes medication, hand therapy, and occasionally surgery.
Infection
Authors: Krishnan Raja Shanmuga , Sabapathay S. Raja , Dunn Roderick
Published: October 2018

Hand infections are common and range from minor nail fold or pulp infections to severe necrotizing sepsis (often in the presence of other significant co-morbidity). We discuss general principles of the surgical management of soft tissue infection, the microbiology of hand infection and antibiotic policy. Cellulitis, abscess, and specific hand infections are covered, including flexor tendon sheath infection, intra-articular sepsis, and bites (human and animal). The chapter concludes with osteomyelitis and chronic infections, including leprosy, and other conditions which can mimic infection.
Rheumatology
Authors: Edwards Chris , Warwick David
Published: October 2018

A number of rheumatological diseases are manifest in the hand and wrist. Many are associated with considerable systemic inflammation. This produces chronic ill-health, poor wound healing, and osteoporosis. Attempts to eliminate inflammation at the earliest opportunity are vital. Multiple involved joints with high acute phase markers (CRP and erythrocyte sedimentation rate raised) and early morning stiffness are key features. Multi-disciplinary management, involving the rheumatologist and hand therapist is essential. With improving medical management, surgical problems are becoming far less common. Typical conditions include metacarpophalangeal joint malalignment (ulnar drift, sagittal band subluxation), spontaneous arthrodesis, tenosynovitis, tendon rupture, and proximal interphalangeal joint imbalance (swan neck and boutonnière deformity).
Ulnar corner
Authors: Warwick David
Published: October 2018

The ulnar corner is complex with many anatomical structures and many potential pathologies. This may render diagnosis and treatment a challenge. Meticulous history taking and examination are required, supplemented, if necessary, by tests such as X-ray, MRI, and arthroscopy. Condition include ECU and FCU tendinopathy; hook of hamate fracture; carpal instability; osteoarthritis of the DRUJ, pisotriquetral joint and lunatehamate joint; degenerative and traumatic lesions of the TFCC; ulnar neuropathy, and hypothenar hammer syndrome. Successful treatment depends on accurate diagnosis; non-operative measures usually suffice but surgical options include excision arthroplasty (e.g. pisiform arthritis), joint replacement (e.g. ulnar head), neurolysis (e.g. ulnar nerve neuropathy), surgical debridement (e.g. TFCC central perforations and lunate–hamate arthritis), and ulnar shortening (ulnocarpal impaction).
Wrist
Authors: Miranda Sanjay , Warwick David
Published: October 2018

The carpus is characterized by complex anatomy and biomechanics. Twenty-four tendons traverse the eight bones and several named extrinsic and intrinsic ligaments. The joint are controlled by integrated co-ordination of the tendons and interosseous ligaments such that that movement yet stability in any direction can be achieved. Pathologies include ligamentous instability, ganglia, osteoarthritis, Kienbock’s, dorsal rim impingement, infection, rheumatoid. Treatment options depend on the diagnosis but include hand therapy, steroid injections, and surgery (arthroscopy, replacement, partial fusion, total fusion, excision arthoplasty).
Congenital abnormalities of the gastrointestinal tract
Authors: Stanton Mike
Published: October 2018

This chapter covers the common congenital abnormalities. This includes detailed discussion of the gut development. Conditions covered include gastroschisis, exomphalos, malrotation, duodenal atresia, small bowel obstruction, distal intestinal obstruction, meconium ileus, Hirschsprung disease, and anorectal malformation.
Iron deficiency
Authors: Puntis John
Published: October 2018

Iron deficiency is the most common nutritional deficiency in the world, affecting around 5 billion people mostly in developing countries. Risk factors in infants include low birthweight, high cow milk consumption, low intake of iron containing complementary foods, low socioeconomic status, and immigrant status. Developmental delay and poor educational achievement are among the long-term complications. Preventative strategies include promotion of breastfeeding, use of iron-fortified formula if breast milk not available, encouraging intake of iron-rich foods, vitamin C-rich drinks with meals to promote iron absorption, and avoiding whole cow’s milk in the first year of life. Poor response to oral iron treatment is most likely due to poor compliance (iron ingestion may cause abdominal pain diarrhoea or constipation) but should also raise the possibility of underlying disease causing inflammation, malabsorption, or blood loss.
Intestinal transplantation
Authors: Hind Jonathan
Published: October 2018

The chapter on intestinal transplantation discusses the indications and contraindications of this procedure. It covers the relevant nomenclature and describes the various operation techniques used. In addition it presents postoperative management including immunosuppression and nutrition, as well as surgical and medical complications and outcomes of intestinal transplantation.
Difficult eating behaviour in the young child
Authors: Puntis John
Published: October 2018

Food refusal is common in early life for a variety of reasons that include parental anxiety and forced feeding. From about 6 weeks of age, infants regulate their energy intake according to energy needs. Newborns prefer sweet-tasting feed and at 3–5 months readily accept new tastes. At 12–18 months fear of new foods (neophobia) develops. Self-feeding by the end of the first year should be encouraged and the child allowed to be messy and enjoy meal times. Repeated exposure to a food is the best way of it becoming accepted. Faddy/picky eating is most likely to occur around 18 months of age. There are some simple rules for modifying eating behaviour, as well as increasing energy intake when appropriate.
Nutritional problems in the child with neurodisability
Authors: Puntis John
Published: October 2018

Feeding problems occur in around 50% of children with neurological disability. Needing help with feeds, choking, prolonged meal times, and poor weight gain are common. Gastro-oesophageal reflux and constipation may further complicate management, and obesity is a risk particularly in those with low energy requirements. Assessment should include a detailed feeding history and anthropometry. Oromotor skills and safety of swallow can be gauged by a speech and language therapist, and appropriate seating and eating tools by an occupational therapist. The dietician advises on nutritional requirements and how these can be met. Tube feeding is indicated for an unsafe swallow, recurrent aspiration pneumonia, and when an adequate energy intake cannot be achieved despite optimizing oral intake. On rare occasions, parenteral nutrition may be considered if gastrointestinal dysmotility progresses to the point that enteral feeding is no longer possible. This raises difficult ethical issues and early involvement of a palliative care team is highly recommended.
Vomiting
Authors: Batra Akshay , Beattie Mark
Published: October 2018

This chapter covers the physiology of vomiting in detail. There are also sections on the assessment of vomiting, differential diagnosis, and practical management including indications for consideration of antiemetic therapy.
Achalasia
Authors: Stanton Mike
Published: October 2018

This chapter covers achalasia including discussion of the incidence, presenting features, differential diagnosis, investigation, and management.
Pyloric stenosis
Authors: Stanton Mike
Published: October 2018

This chapter covers pyloric stenosis. This includes sections on the aetiology, pathophysiology of the electrolyte disturbance, assessment, diagnosis, and management.
Gastrointestinal endoscopy
Authors: Bremner Ronald
Published: October 2018

This chapter covers gastrointestinal endoscopy—gastroscopy and colonoscopy. This includes sections on environment and equipment, indications, contraindications, bowel preparation for colonoscopy, and complications.
Chronic diarrhoea
Authors: Wiskin Anthony , Beattie Mark
Published: October 2018

This chapter discusses the investigation and management of chronic diarrhoea including common and rarer causes. The aetiology, assessment, and management are discussed in detail.
Coeliac disease
Authors: Batra Akshay
Published: October 2018

The prevalence of coeliac disease is over 1% in the general population and higher in high-risk groups. The chapter discusses the clinical features, associated conditions, different investigations available for diagnosis, diagnostic criteria, and the long-term monitoring of children with coeliac disease.
Inflammatory bowel disease: introduction
Authors: Batra Akshay , Beattie Mark
Published: October 2018

The incidence of inflammatory bowel disease is 5.2/100,000 children less than 16 years in the UK. Crohn’s disease is the commonest form of inflammatory bowel disease followed by ulcerative colitis and indeterminate colitis. The precise aetiology of inflammatory bowel disease is unknown and reflects a complex interaction between genetic predisposition, immune dysfunction, and environmental triggers.
Crohn’s disease
Authors: Batra Akshay , Beattie Mark
Published: October 2018

The chapter describes the presentation, diagnosis, and clinical course of paediatric Crohn’s disease. Different treatment options, both medical and surgical, are covered in detail.
Ulcerative colitis
Authors: Batra Akshay , Beattie Mark
Published: October 2018

This chapter discusses the presentation and management of ulcerative colitis with a special emphasis on management of acute severe colitis. Risks and benefits associated with different therapies are described.
Eosinophilic disorders
Authors: Beattie Mark , Lajeunesse Mich
Published: October 2018

This chapter discusses the wide spectrum of eosinophilic (allergic) disorders of the gut. This includes specifically eosinophilic proctocolitis of infancy, eosinophilic enterocolitis of infancy, eosinophilic enterocolitis in the older child, and eosinophilic oesophagitis.
Biliary atresia
Authors: Hadzic Dino
Published: October 2018

The chapter on biliary atresia describes the pathophysiology and the various types of the condition, as well as the diagnosis, management, and common complications.
Autoimmune liver disease
Authors: Vergani Giorgina , Samyn Marianne
Published: October 2018

The chapter on autoimmune liver disease describes the features of the various types of autoimmune liver disease as well as the clinical features, diagnosis, treatment, co-morbidities, and complications of this clinical entity.
Metabolic liver disease
Authors: Hegarty Rob , Grammatikopoulos Tassos
Published: October 2018

The chapter on metabolic liver disease covers the metabolic disorders that affect the liver. It covers the important features in the history that may raise the suspicion of such a condition, as well as the clinical features that may be found. A detailed differential is discussed depending on the clinical presentation, for example, acute liver failure, infantile cholestasis, neonatal ascites, or acidosis and ketosis. Suggested investigations and the general management of these conditions are also included.
Hepatitis B
Authors: Bansal Sanjay
Published: October 2018

The chapter on hepatitis B discusses the epidemiology and transmission of this infection. It covers the clinical features, complications, and prevention of the disease, but also the interpretation of the serology as well the management of the chronic hepatitis B infection.
Paediatric liver transplantation
Authors: Jain Van , Mouat Stephen
Published: October 2018

The chapter on paediatric liver transplantation presents the indications and contraindications of this potentially life-saving procedure. It explains the pre-transplant assessment process and the survival rates that can be expected. It also covers the types of different transplants as well as the initial post-liver transplant management and commonly associated complications.
Stress and anxiety
Authors: Harding Duncan
Published: October 2018

This chapter will consider the value of anxiety, and how it might be reframed into something that is helpful in our interview. Anxiety is a precious commodity in the interview, and rather than trying to stifle or dampen it down to a more comfortable state of relaxed internal calm, anxiety should be examined, nurtured, and reframed into something that will overtly help. The chapter will revisit the idea of being mindfully anxious, and will consider how we can understand and control stress. This chapter describes practical techniques, including using a mood diary, to help us to control and utilize our anxiety in order to give our best in the interview.
From internal to external
Authors: Harding Duncan
Published: October 2018

This chapter looks at how a key to interview success is the ability to step outside of one’s internal world and into the external space around us; to break free of the shackles of rumination and contemplation inside, and to exist wholly in the interview space outside. Much of this book is about moving from the internal to the external, and an external objective standpoint will put the candidate in the best possible position for interview success. This chapter starts that process, and considers communication from a psychological perspective as well as the power of non-verbal communication. It then considers body language and the dissemination of thought processes out into the external world.
Situational awareness
Authors: Harding Duncan
Published: October 2018

This chapter works towards improving and refining our situational awareness in the interview space, by considering the interview from a psychological perspective. It discusses theory of mind, motive, and motivations, and how to fill the required space in the interview appropriately. The chapter considers the empowering psychological state of curiosity and how this can help us retain a sense of control in a stressful interview situation. Finally, it discusses our understanding of the interviewer themselves. This chapter includes an exercise to find an anchor in the interview space, i.e. a method to ground ourselves mindfully in the moment of the interview.
Final thoughts
Authors: Harding Duncan
Published: October 2018

This is the final chapter of this book, and it brings together some of the thoughts that have been discussed previously. It explores the importance of moving on in the process, every experience good and bad contributes to an improved performance for the future. It then looks at the value of embracing the positive, having dealt with anxiety in previous chapters, and the importance of balancing the two. It considers upgrading skills, each interview itself being an upgrade, and makes suggestions for effecting change. The chapter finally reflects on our new set of psychological skills available for us in this charged and challenging process.
The EACVI Textbook of Cardiovascular Magnetic Resonance
Authors: Ferrari Victor
Published: October 2018

Cardiovascular magnetic resonance imaging (CMR) has become one of the great pillars of cardiac imaging. Modern CMR, as we now practise it, is the result of an enormous method and application development effort that has occurred over the past 25 years and has taken CMR from its humble beginnings of anatomical T1- and T2-weighted imaging to the extremely versatile, accurate, and robust technique it is now. The main developments over this time, building on the anatomical imaging, were the establishment of cine imaging for assessment of cardiac function, first-pass perfusion imaging for measurement of perfusion reserve, as well as myocardial blood flow (in millilitres per minute and gram), late gadolinium enhancement for imaging of scar and patchy fibrosis, and two-dimensional flow velocity imaging for assessment of valve and shunt lesions. This textbook intends to explore and evaluate all areas of this fascinating subject.
Basic pulse sequences
Authors: Kozerke Sebastian , Boubertakh Redha , Miquel Marc
Published: October 2018

Pulse sequences control the timing of radiofrequency pulses and time-varying gradients that are necessary to create an image. Sequences are divided into different types: gradient echo, spin echo, and hybrid echo sequences. In cardiac imaging, ‘black blood’ spin echo images are used for anatomical imaging, while ‘bright blood’ imaging is used to study function and is based on gradient echo or hybrid echo sequences. Some key applications of those sequences, e.g. water–fat imaging or T2*-mapping to detect iron loading, are discussed. Preparation pulses can be used to modify image contrast to, for example, improve black blood images or for quantitative applications, including T1- and T2-mapping. To help the reader navigate through the sea of sequence acronyms, the chapter ends with a quick guide covering the acronyms used by the main scanner manufacturers.
CMR applications
Authors: Kozerke Sebastian , Boubertakh Redha , Miquel Marc
Published: October 2018

Assessment of cardiac morphology, function, and pathology requires the use of different pulse sequences (spin echo, gradient echo, hybrid echo), preparation pulses to manipulate image contrast, and data acquisition strategies such as parallel imaging, single-shot versus segmented acquisition, or contrast versus non-contrast imaging techniques. This chapter presents some of the main cardiac applications: morphology and function, stress perfusion imaging, viability assessment using late gadolinium enhancement, flow quantification, angiography, and fat suppression techniques. For each application, a description of its use within the clinical context is provided, along with the main sequence parameters and their effect on image contrast, acquisition time, and image quality.
Image quality and artefacts
Authors: Kozerke Sebastian , Boubertakh Redha , Miquel Marc
Published: October 2018

Good and reproducible image quality is essential for clinical diagnosis. Although image quality can be defined in numerous ways, signal-to-noise and contrast-to-noise ratios are often used as indicators. Despite our best efforts, image artefacts are part and parcel of CMR imaging and are sometimes unavoidable. Image artefacts can be caused by patients (e.g. motion, chemical shift artefacts), image acquisition and reconstruction (e.g. foldover artefacts), or equipment/external interferences (e.g. radiofrequency leakage). The most common image artefacts encountered in cardiac imaging and strategies to avoid or reduce them are discussed in this chapter.
MRI contrast agents
Authors: Nguyen Kim-Lien , Finn J Paul
Published: October 2018

Pharmacologic agents used in the context of a cardiovascular magnetic resonance imaging examination can be divided into three main groups: contrast agents, stress-testing agents (dobutamine, adenosine, regadenoson, dipyridamole), and agents used for treatment of hypersensitivity reactions. The safety of stress agents will be elaborated in the chapter on ischaemic heart disease. This chapter will focus on contrast agents and briefly touch upon medications used in the setting of hypersensitivity reactions. Contrast agents are used to further augment tissue contrast and have become an integral component of cardiovascular imaging. These agents typically exert strong T1 shortening (gadolinium or manganese chelates, positive contrast) or T2 shortening (iron oxide particles, negative contrast). In some cases, very small iron particles may produce enhancement on either T1- or T2-weighted pulse sequences. The common factor lies in the paramagnetic properties of the material when exposed to an external magnetic field. The largest group of compounds consists of gadolinium-based contrast agents (GBCAs). Since the first description of nephrogenic systemic fibrosis in patients with renal insufficiency in 2000, and subsequent alerts by the US Food and Drug Administration and the European Medicines Association, interest in the safety of GBCAs has increased, along with concerted efforts to seek alternative contrast agents. Overall however, GBCAs are well tolerated.