bluuebunny/crossref_metadata_2025 huggingface dataset:
Anesthesia in the Military Setting
Authors: McFarland Craig , Burch Robert H. , Maani Christopher V.
Published: October 2018

Military anesthesia provides many unique and challenging situations, particularly in the current age of unconventional warfare. The role of the anesthesia provider has been redefined throughout American warfare, and it will continue to evolve alongside the U.S. military and its global involvement. Providers must learn to adapt to their environments and improvise care according to the conveniences available while maintaining standards that ensure the best possible care to military personnel. As emerging data and technologies become available, the practice of combat anesthesia will almost certainly change in order to continue providing a high level of care to the military community.
Telemedicine, Teleanesthesia, and Telesurgery
Authors: Murray W. Bosseau , Vaduva Sorin , Berg Benjamin W.
Published: October 2018

Telemedicine overcomes barriers to the delivery of health care services including, distance, cost, gaps in distribution and availability of providers. Anesthesia and surgery applications include remote robotic anesthesia and surgery, preoperative assessment at a distance, physiologic monitoring, remote mentoring, and comprehensive critical care services. The Patient Protection and Affordable Care Act and other legislation supports expansion of telemedicine by enabling telemedicine regulation and reimbursement. Tele-Anesthesia applications are expanding and future economically viable telehealth programs will address a variety of anesthesia specific challenges and domains including pain management, postoperative assessment, just in time training for new clinical applications and anesthesia procedures. Telemedicine techniques include synchronous and asynchronous interactions, virtual presence, mobile-health, and haptics enabled robotics, which are used alone or in combination. Focus areas for development of efficient and effective new Tele-Anesthesia programs include chronic care, remote emergency care, and disaster support. Provider acceptance in tele-anesthesia requires education and training to achieve the full advantages of telemedicine.
The Scope of the EMG Examination
Authors: Katirji Bashar
Published: October 2018

Clinical electromyography (EMG) refers to the diagnostic tool in the electrophysiological evaluation of disorders of peripheral nerve and muscle. This introductory chapter defines the terms of the discipline and its scope. Clinical EMG used in the evaluation of Clinical EMG is utilized by a variety of physicians, including specialists in the field of neurology, physical medicine and rehabilitation, orthopedics, hand surgery, neurosurgery, spine, rheumatology and pain management. The scope of the EMG Examination includes nerve conduction studies and needle EMG. It also includes other specialized testing such as late responses, repetitive nerve stimulation and single fiber EMG. This chapter discusses the referral process to the EMG laboratory and guides the readers to the best practice in the EMG evaluation of patients with neuromuscular disease. Special attention to testing young children and testing patients in the intensive care unit is given. The generation, format and final layout of the EMG report is also advised.
Specialized Electrodiagnostic Studies
Authors: Katirji Bashar
Published: October 2018

In addition to nerve conduction nerve studies and needle EMG, the clinical EMG study include more specialized examinations Some of the tests, such as the F waves and H reflexes are now often used as part of the routine nerve conduction studies. Others are utilized for specific indications: Repetitive nerve stimulation and single fiber EMG are used predominantly in patients with suspected neuromuscular junction disorders such as myasthenia gravis, Lambert-Eaton myasthenic syndrome or botulism; the blink reflexes are used in patients with disorders of the facial and trigeminal nerves as well as brainstem conditions.
This chapter covers the late responses, including the F waves, H reflexes and blink reflexes and their applications in the diagnosis of peripheral nerve disorders. This is a followed by a discussion of the basic concepts of slow and rapid repetitive nerve stimulation and single fiber EMG, as well as their applications in the diagnosis of neuromuscular junction disorders.
Case 2
Authors: Katirji Bashar
Published: October 2018

Low back pain with or without lumbosacral root compression is an extremely common clinical situation presenting to primary care physicians and specialists. This case illustrates a patient with lumbosacral radiculopathy due to vertebral disc herniation, supplemented by several magnetic resonance imaging images and diagrams that enhance the pathophysiology of this disorder. A discussion of the anatomy is followed by the clinical findings of various individual lumbosacral radiculopathies. The findings on electrodiagnostic studies are detailed with emphasis on myotomal and segmental innervation of muscles in the lower extremity. The advantages and limitations of the electrodiagnostic studies in patients with suspected lumbosacral radiculopathy are thoroughly debated. A discussion of the findings in lumbar canal stenosis completes this section.
Case 3
Authors: Katirji Bashar
Published: October 2018

Sciatic nerve injury is a relatively uncommon lower extremity mononeuropathy. The various etiologies of sciatic neuropathies are highlighted in this case. The clinical manifestations and diagnosis include distinguishing foot drop due to sciatic neuropathy from peroneal (fibular) neuropathy across the fibular neck, L5 radiculopathy, and lumbosacral plexopathy. The electrodiagnostic features of sciatic nerve lesion are separated from those of foot drop due to other peripheral nerve causes. In contrast to sciatic nerve injury, the piriformis syndrome is mostly a painful syndrome with no or minimal sensory or motor deficits. The clinical manifestations of piriformis syndrome and controversies surrounding this syndrome completes the discussion in this case.
Case 5
Authors: Katirji Bashar
Published: October 2018

Lumbosacral plexopathy is a relatively rare entity with a variety of etiology and pathophysiology. The diagnosis of lumbosacral plexopathy requires high index of suspicion and knowledge of the detailed anatomy of the lumbosacral plexus is a prerequisite to the accurate conclusion. This case describes a young woman with injury to the lumbosacral trunk (cord), a main component of the lumbosacral plexus, occurring during the delivery of her newborn and outlines its mechanism of action, pathophysiology and risks. The case highlights also the differential diagnosis of lumbosacral plexopathy and discusses the most common causes including retroperitoneal hemorrhage, lumbosacral plexitis, and intrapartum maternal lumbosacral plexopathy.
Case 6
Authors: Katirji Bashar
Published: October 2018

Tarsal tunnel syndrome is relatively rare entrapment syndrome, and should be considered in patients with foot pain or numbness. It is the result of compression or entrapment of the tibial nerve or any of its three terminal branches under the flexor retinaculum. This case discusses a patient with typical manifestations of tarsal tunnel syndrome and highlights its causes, differential diagnosis and electrodiagnostic findings. It also covers the challenges in making an accurate diagnosis in view of the technical difficulties encountered with foot and sole nerve conduction studies. Tarsal tunnel syndrome is often overdiagnosed, since it may be confused with other more common causes of foot pain and numbness including plantar fasciitis, peripheral polyneuropathy, and S1 radiculopathy.
Case 7
Authors: Katirji Bashar
Published: October 2018

Diabetic amyotrophy is a relatively uncommon neurological complication of diabetes mellitus. However, the disorder is often not recognized by internists and neurologists and misdiagnosed as myopathy, radiculopathy, or peripheral polyneuropathy. The discussion starts by outlining the classification of the diabetic neuropathies. This case highlights the classical clinical presentation of diabetic amyotrophy, also referred to as diabetic polyradiculoplexopathy or subacute diabetic neuropathy, in a man with pain in the anterior thigh and knee followed by thigh and hip weakness. It also emphasizes the electrodiagnostic findings including the subacute needle electromyography changes and stresses the frequent coexistence of diabetic amyotrophy with the more common distal peripheral polyneuropathy in the majority of patients.
Case 9
Authors: Katirji Bashar
Published: October 2018

Ulnar nerve lesions are the second most common mononeuropathies encountered in clinical practice. Although the majority of the lesions are due to entrapment/compression of the ulnar nerve around the elbow (at the cubital tunnel or ulnar groove), it is important to consider and exclude distal ulnar nerve lesions at the wrist or palm. This case highlights the clinical and electrodiagnostic findings of ulnar neuropathies at the wrist, including Guyon canal and pisohamate hiatus. It also discusses the electrodiagnostic challenges in distinguishing distal from proximal ulnar nerve lesions. Emphasis is placed on the use of additional studies, such as the dorsal ulnar sensory nerve action potential, first dorsal interosseous recording, inching studies, and palm stimulation, in the accurate diagnosis of ulnar nerve lesions at the wrist.
Case 11
Authors: Katirji Bashar
Published: October 2018

Cervical radiculopathy and neck pain are among the most common neurological presentations seen in clinical practice. Cervical radiculopathy results in radicular pain, sensory manifestations, motor weakness and reflex changes, that are dependent on the specific compressed cervical root. The accurate diagnosis of cervical radiculopathy depends on a detailed neurological examination supplemented by electrodiagnostic studies and imaging of the cervical spine. This case highlights the anatomy, pathophysiology, and findings of the various cervical radiculopathies and distinguishes them from brachial plexopathies and other upper limb mononeuropathies. The benefits, pitfalls, and challenges of electrodiagnostic studies, including nerve conduction studies and needle electromyography, are also discussed.
Case 13
Authors: Katirji Bashar
Published: October 2018

Thoracic outlet syndrome remains a controversial syndrome despite being described more than a century ago. This syndrome has neurogenic, vascular, and disputed types. True neurogenic thoracic outlet syndrome is relatively rare syndrome often associated with a cervical rib or cervical band. Symptoms include pain, hand and forearm numbness, and hand weakness and atrophy. The true neurogenic disorder has classical electrodiagnostic presentations. This case highlights the anatomy of the brachial plexus and distinguishes true neurogenic thoracic outlet syndrome from carpal tunnel syndrome, cubital tunnel syndrome, C8 radiculopathy, T1 radiculopathy, and post-median sternotomy brachial plexopathy, with emphasis on the electrodiagnostic findings.
Case 15
Authors: Katirji Bashar
Published: October 2018

Ulnar nerve lesions are the second most common mononeuropathies encountered in clinical practice. The majority of ulnar neuropathies are across the elbow, more specifically due to entrapment or compression of the ulnar nerve at the cubital tunnel or ulnar groove. This case highlights the clinical and electrodiagnostic findings of ulnar neuropathies across the elbow and discusses the challenges in making an accurate diagnosis. Focal slowing of conduction velocities and/or conduction block are the main findings that pinpoint the site of the lesion, while the needle electromyography is poor in accurate localization, mostly due to the limited number of ulnar innervated muscles in the forearm. Important additional testing that often is recommended to aid in the accurate diagnosis of ulnar nerve lesions across the elbow includes the dorsal ulnar sensory nerve action potential, ulnar motor conduction study recording the first dorsal interosseous, and inching studies across the elbow.
Case 16
Authors: Katirji Bashar
Published: October 2018

Neuralgic amyotrophy is a relatively uncommon disorder but important to recognize since it may be confused with brachial plexopathy, cervical radiculopathy and entrapment/compressive mononeuropathies of the upper extremity. Neuralgic amyotrophy is also known as acute brachial neuritis, acute brachial plexitis, and Parsonage-Turner syndrome. This case highlights the variable clinical and electrodiagnostic findings encountered in patients with neuralgic amyotrophy, with special attention to the most common mononeuropathies affected in this disorder. This include the long thoracic nerve, axillary nerve, phrenic nerve and anterior interosseous nerve. The peculiar needle electromyography findings associated with neuralgic amyotrophy, including selective motor branch involvement, are also discussed.
Case 18
Authors: Katirji Bashar
Published: October 2018

Immune-mediated polyneuropathies are important to recognize since the majority of them are treatable. Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is the prototype of the acquired chronic demyelinating polyneuropathies. It should be distinguished from other acquired demyelinating polyneuropathy such as those associated with monoclonal gammopathy, myeloproliferative disorder, and myelin-associated glycoprotein. This case presents a patient with a chronic demyelinating polyneuropathy associated with monoclonal gammopathy of unknown significance. The discussion includes the clinical and electrodiagnostic criteria for CIDP and distinguishing features from axonal polyneuropathies, other acquired demyelinating polyneuropathies, and inherited demyelinating polyneuropathies such as Charcot-Marie-Tooth disease type I. The case also highlights the diagnostic criteria for conduction block and temporal dispersion based on analysis of compound muscle action potential amplitude, area, and duration.
Case 20
Authors: Katirji Bashar
Published: October 2018

Inflammatory myopathies are a group of disorders characterized by necrotizing myopathies with inflammatory infiltrates. Dermatomyositis, polymyositis, and inclusion body myositis are the classical types although other overlapping disorders are now more commonly diagnosed, including necrotizing autoimmune myopathy and the anti-synthetase syndrome. This case presents a typical patient with polymyositis and outlines the clinical and pathological findings of the various inflammatory myopathies. It highlights the differential diagnosis as well as the differences and similarities among the autoimmune inflammatory myopathies. A detailed discussion of the needle electromyography findings in the various myopathies is included: Myopathies are classified into those with or without fibrillations, with or without myotonic discharges, and with or without changes in the morphology of the motor unit action potentials.
Case 21
Authors: Katirji Bashar
Published: October 2018

Lambert-Eaton myasthenic syndrome is a rare yet very important neuromuscular disorder that may be difficult to confirm if not considered in the differential diagnosis. It is often misdiagnosed as myasthenic gravis or other nonspecific neuromuscular disorder. The electrodiagnostic findings in Lambert-Eaton myasthenic syndrome continue to be the cornerstone of the diagnosis. This case outlines the clinical and electrodiagnostic features of a patient with this syndrome and highlights the findings on repetitive nerve stimulation. It also discusses the practical approach in the search for occult malignancy. The distinguishing features among the various neuromuscular junction disorders are emphasized.
Case 22
Authors: Katirji Bashar
Published: October 2018

Charcot-Marie-Tooth disease is the most common inherited neuropathy encountered in clinical practice. The disorder encompasses a large number of subtypes; most share common neurological manifestations. Charcot-Marie-Tooth disease is often subdivided into many subtypes based on pathophysiological or inheritance patterns. There are demyelinating and axonal types, as well as dominant, recessive, or X-linked forms. This case presents a typical patient with Charcot-Marie-Tooth disease and highlights the neurological findings. This is followed by emphasis on the findings seen on nerve conduction studies showing the differences between the demyelinating, axonal, and intermediate types. A list of the known genetic defects described so far is presented. A working and practical differential diagnosis and algorithm is also suggested.
Case 23
Authors: Katirji Bashar
Published: October 2018

Guillain-Barré syndrome is the prototype of acute immune-mediated neuropathies. Guillain-Barré syndrome has several subtypes including acute inflammatory demyelinating polyneuropathy, acute motor axonal neuropathy, and acute motor sensory axonal neuropathy. Guillain-Barré syndrome has also several variants including Miller Fisher syndrome, ataxic form, and pharyngeal–cervical–brachial form. This case highlights the clinical findings in Guillain-Barré syndrome and discusses in details the diagnostic criteria that are essential in confirming the diagnosis and excluding mimickers of the disorder. This is followed by a detailed discussion on the electrodiagnostic findings in Guillain-Barré syndrome during the acute presentation and recovery phase. The diagnostic sensitivity and specificity of the various findings seen on nerve conduction studies are included.
Case 26
Authors: Katirji Bashar
Published: October 2018

Peripheral polyneuropathy is a common clinical presentation, and most cases seen in clinical practice are axonal, dying-back, sensorimotor polyneuropathies. Axonal polyneuropathies should be distinguished from the acquired demyelinating peripheral polyneuropathies which are often immune-mediated and amenable to treatment. This case presents a typical patient with a dying-back sensorimotor axonal peripheral polyneuropathy and presents a practical approach to the etiologic diagnoses of peripheral neuropathy. It then highlights its distinguishing features on nerve conduction studies. A comparison between axonal and demyelinating polyneuropathies is revealed. An algorithmic approach to the workup of a patient presenting with a peripheral neuropathy is recommended.
Case 27
Authors: Katirji Bashar
Published: October 2018

Mononeuropathy multiplex is a disorder which distinguishes itself from peripheral polyneuropathy by involvement of specific individual peripheral nerves. Mononeuropathy multiplex is often asymmetrical and is classified, based on electrophysiologic and pathologic findings, into axonal and demyelinating types. Mononeuritis multiplex is a term that is used exclusively for cases of axonal multiple mononeuropathies due to peripheral nerve vasculitis. This may be associated with connective tissue disease or hepatitis B and C infection. This case outlines the clinical and electrophysiological findings in mononeuritis multiplex and discusses the differential diagnosis of systemic and nonsystemic vasculitic neuropathy. The causes and pathological findings that characterize vasculitic neuropathy are highlighted.
Neuroimmunology
Authors: Miller Aaron E. , DeAngelis Tracy , Fabian Michelle , Katz Sand Ilana
Published: October 2018

Part of the What Do I Do Now?” series, Neuroimmunology uses a case-based approach to cover common and important topics in the examination, investigation, and management of central and peripheral demyelinating diseases, vasculitis, and other immune system related neurological disorders. Each chapter provides a discussion of the diagnosis, key points to remember, and selected references for further reading. For this edition, all cases and references have been updated and new cases have been added, including POEMS, CASPR2 Antibody Syndrome, Isaac’s Syndrome, Histiocytosis, and Churg-Strauss. Neuroimmunology is an engaging collection of thought-provoking cases which clinicians can utilize when they encounter difficult patients. The volume is also a self-assessment tool that tests the reader’s ability to answer the question, “What do I do now?”
A Case of Multifocal Neurological Symptoms
Authors: Miller Aaron E. , DeAngelis Tracy M. , Fabian Michelle , Sand Ilana Katz
Published: October 2018

Acute disseminated encephalomyelitis (ADEM) is an immune-mediated demyelinating disorder characterized by multifocal involvement of the CNS and encephalopathy. Diagnostic criteria also require MRI lesions characteristic for demyelination and that new symptoms/findings not occur more than three months after initial onset. ADEM preferentially affects young children but also occurs in adolescents and adults. An antecedent infection or vaccine may be reported, but a clear causal relationship has not been established, and more often no clear provoking factor is identified. In addition to encephalopathy, patients present with symptoms reflecting multifocal CNS involvement such as optic neuritis, ataxia, hemiparesis, spinal cord syndrome, and seizures. Respiratory failure may occur. ADEM is typically treated with high-dose corticosteroids. Plasma exchange or intravenous immunoglobulin may also be used. The course is most often monophasic with good recovery. However, a subset of patients will go on to have recurrent disease, reflecting a diagnosis of multiple sclerosis, neuromyelitis optica spectrum disorder, or myelin oligodendrocyte glycoprotein related disease.
Recurrent Ulcers and a Brainstem Lesion
Authors: Miller Aaron E. , DeAngelis Tracy M. , Fabian Michelle , Sand Ilana Katz
Published: October 2018

Behçet syndrome (BS) is a multisystem, relapsing, inflammatory vasculopathy of unclear etiology. It occurs frequently, though not exclusively, in patients who are positive for HLA B51. Patients most often present with recurrent orogenital ulcers. Other common systemic manifestations include other skin lesions, uveitis, gastrointestinal symptoms including ulceration, thromboembolic phenomena, and arthralgias. Neurological involvement (neuro-Behçet syndrome, NBS) occurs in approximately 10% of patients and may manifest as cerebral venous sinus thrombosis (CVST, “extra-axial”) or with parenchymal involvement (“intra-axial”). In order to diagnose a neurological presentation as NBS, the patient must already have systemic evidence of BS. CVST presents with increased intracranial pressure: symptoms include headache, sixth-nerve palsy or other cranial neuropathy, encephalopathy, and/or seizures. Intra-axial NBS typically presents as a subacute brainstem syndrome with dysarthria, ophthalmoparesis and other cranial neuropathies, and corticospinal tract symptoms/signs. Brain MRI demonstrates this characteristic brainstem involvement. Other, less common, manifestations of NBS include cognitive changes, myelopathy, and very rarely, optic neuritis. CSF profile during active disease is typically inflammatory, and neutrophils may be present, though lymphocytes may also predominate. Protein is often elevated. Oligoclonal bands are positive only rarely. Intra-axial disease is treated acutely with high-dose corticosteroids and chronically with various immunomodulatory therapies.
Ataxia and Orthostasis
Authors: Miller Aaron E. , DeAngelis Tracy M. , Fabian Michelle , Sand Ilana Katz
Published: October 2018

Neurological complications occur in approximately 20% of patients with Sjögren’s syndrome. Most commonly, patients have peripheral manifestations, including sensory ataxic ganglionopathy, painful sensory neuropathy, cranial neuropathies, and radiculoneuropathies. Central nervous system manifestations, including multiple sclerosis–like conditions, may also occur. Sensory ataxic ganglionopathy in Sjögren’s typically begins with distal paresthesias, which then may spread proximally. This is followed by gait ataxia and, in severe cases, significant proprioceptive abnormalities in the hands as well. The characteristic antibodies, anti-RO (SSA) and anti-LA (SSB), are present in approximately 60% of patients, but their presence is not specific for Sjögren’s syndrome. If a Sjögren’s patient develops myelitis, NMO antibodies should be checked because of the common co-occurrence of these conditions.
Blurry Vision, Hearing Loss, and Constant Headache
Authors: Miller Aaron E. , DeAngelis Tracy M. , Fabian Michelle , Sand Ilana Katz
Published: October 2018

Sarcoidosis is a multisystem inflammatory disorder of unknown etiology, characterized pathologically by the presence of noncaseating granulomas and multinucleated giant cells. For unknown reasons, the disorder tends to be more common among African Americans and Scandinavians. Among the most common systemic manifestations are pulmonary involvement, lymphadenopathy, and skin lesions. Nervous system involvement occurs in 5–10% of cases. Despite the rarity of neurological involvement, 50–70% of the time, the neurological symptoms are the initial symptoms. In most patients with neurosarcoid, there is evidence for involvement in other systems, either at presentation or during the course of the condition. The presentation in neurosarcoidosis is varied. Steroids are the first-line treatment, but some patients may also require steroid-sparing agents to control the condition.
The Snowball Effect
Authors: Miller Aaron E. , DeAngelis Tracy M. , Fabian Michelle , Sand Ilana Katz
Published: October 2018

Susac syndrome is a rare disease involving a triad of subacute encephalopathy, vision loss involving branch retinal artery occlusions (BRAOs), and sensorineural hearing loss. Clinical presentation is variable and generally does not involve the entire triad. The suspected pathophysiology is an immune-mediated endotheliopathy affecting the precapillary arterioles of the brain, retina, and inner ear. MRI of the brain reveals abnormal white matter lesions similar to demyelinating disease, but with distinct characteristic central callosal lesions, as well as deep gray matter and leptomeningeal involvement. There is no standardized therapy for Susac’s, but suspicion of an immune-mediated endotheliopathy has informed empirical treatment with corticosteroids and various immunosuppressant regimens, which appear to improve the prognosis.
A Case of Cognitive Impairment and Ataxia
Authors: Miller Aaron E. , DeAngelis Tracy M. , Fabian Michelle , Sand Ilana Katz
Published: October 2018

Celiac disease is an autoimmune enteropathy characterized by the presence of antigliadin antibodies and small intestinal mucosal injury, mediated by the adaptive immune system in response to gluten. Gastrointestinal symptoms typically include diarrhea, malabsorption/weight loss, and bloating/abdominal pain. Extra-intestinal symptoms often occur. The most commonly reported neurological syndromes are peripheral neuropathy, headache, and “gluten ataxia.” Gluten ataxia may occur in the setting of celiac disease or with non-celiac gluten sensitivity. Consistent with this being a cerebellar phenomenon, autopsy studies demonstrate inflammatory infiltration and Purkinje cell loss. Cerebellar atrophy as well as white matter abnormalities may be evident on MRI. Treatment is institution of a gluten-free diet. Intravenous immune globulin may also be helpful in select patients.
A Case of Fever and a Wrist Drop
Authors: Miller Aaron E. , DeAngelis Tracy M. , Fabian Michelle , Sand Ilana Katz
Published: October 2018

Polyarteritis nodosa (PAN) is a systemic illness that most often involves the skin, kidneys, and gastrointestinal tract, in addition to the peripheral nervous system. It most often affects middle-aged individuals, men more than women. The particular type of peripheral neuropathy is often mononeuropathy or mononeuritis multiplex—a condition in which there is discrete involvement of multiple individual nerves. Definitive diagnosis of the vascular nature of the neuropathy requires peripheral nerve biopsy, which will demonstrate inflammation throughout the walls of small and medium-sized arteries. The CNS is also frequently involved in PAN. Constitutional symptoms, including fever, fatigue, and diffuse muscle and joint pain, are common and raise the suspicion of a systemic illness. Treatment generally involves the use of corticosteroids and immunosuppressive agents such as cyclophosphamide or azathioprine.
A Case of Personality Change and Seizure
Authors: Miller Aaron E. , DeAngelis Tracy M. , Fabian Michelle , Sand Ilana Katz
Published: October 2018

The limbic encephalitides comprise a group of immune-mediated conditions that preferentially affect the limbic system, resulting in clinical manifestations such as memory deficits, seizures, and psychiatric disturbances. Limbic encephalitis (LE) may occur as a paraneoplastic syndrome related to underlying tumor, or as a primary autoimmune phenomenon. MRI often shows T2/FLAIR hyperintensities in the temporal lobe. CSF may be normal or may demonstrate mild lymphocytic pleocytosis and/or elevated protein. As the number of identifiable autoantibodies has rapidly increased in recent years, frequently one of these will be noted in the serum or spinal fluid. Antibodies to “classic paraneoplastic” intracellular antigens may only be markers of disease, given pathology studies demonstrating T cell infiltration, whereas antibodies to extracellular/cell membrane-associated antigens seem to be pathogenic and thus these tend to exhibit superior responses to immunotherapies. Treatment involves removal of the tumor if one is present, as well as various immunomodulatory therapies.
Drowsiness and Double Vision
Authors: Miller Aaron E. , DeAngelis Tracy M. , Fabian Michelle , Sand Ilana Katz
Published: October 2018

Bickerstaff brainstem encephalitis (BBE) is a syndrome defined by encephalopathy, ophthalmoplegia, and ataxia. It is associated with GQ1b antibodies, the same antibodies that are thought to be pathogenic in Miller-Fisher Syndrome (MFS). Thus, one may consider these syndromes as a continuum; the central nervous system is affected in BBE, while the peripheral nervous system is affected in MFS. BBE is an idiopathic autoimmune disorder that typically presents with acute to progressive cranial nerve dysfunction, usually ophthalmoplegia, associated with cerebellar ataxia, reflex abnormalities, and altered mental status, potentially progressing to coma. Bickerstaff first described this disorder in 1957, reporting eight patients with “brainstem encephalitis,” who presented with symptoms of drowsiness, acute ophthalmoplegia, ataxia, extensor plantar responses, and hemisensory loss. Prognosis for BBE is generally good, though patients may have a complicated course. The treatment options for BBE include intravenous immunoglobulin or plasmapheresis.
On the Right Tract
Authors: Miller Aaron E. , DeAngelis Tracy M. , Fabian Michelle , Sand Ilana Katz
Published: October 2018

Paraneoplastic myelopathies are uncommon, but maintenance of a high index of clinical suspicion is critical, as the neurological syndrome often predates discovery of an underlying malignancy and can facilitate early detection and potentially improve prognosis. Most paraneoplastic myelopathies are associated with additional neurological symptomatology, such as encephalitis, peripheral neuropathy, or cerebellar ataxia, due to the specific, respective auto-antibodies involved. Anti-Hu antibody, identified in our patient, appears to be the most common antibody, followed by amphiphysin, and collapsing response mediator protein 5, among others. The therapeutic approach to patients with paraneoplastic myelopathies focuses primarily on treatment of the underlying cancer and, thereafter, subsequent trials of immunotherapies, which have demonstrated variable response.
Extinguishing a “Burning Fire”
Authors: Miller Aaron E. , DeAngelis Tracy M. , Fabian Michelle , Sand Ilana Katz
Published: October 2018

Isaacs syndrome is a rThTare acquired neuromuscular disorder characterized by peripheral nerve hyperexcitability. Symptoms involve progressive muscle pain, stiffness, and notable continuous diffuse myokymic muscle-twitching clinically consistent with neuromyotonia. There can be associated weakness, hyporeflexia, numbness, dysesthesias, and hyperhidrosis. Isaacs syndrome generally presents between ages 25–60, more commonly in men. Etiologies include autoimmune, often paraneoplastic, syndromes, typically associated with malignant thymic carcinomas. Therapeutic management involves treating the underlining malignancy, as well as using immune-mediated therapies including corticosteroids, intravenous immunoglobulin, plasma exchange, and rituximab. The long-term prognosis for patients with Isaacs syndrome varies, generally dependent on the underlying cause. While Isaacs syndrome is a chronic condition without a cure, it is generally treatable and not fatal.
Confusion, Muscle Cramps, and Weight Loss
Authors: Miller Aaron E. , DeAngelis Tracy M. , Fabian Michelle , Sand Ilana Katz
Published: October 2018

Morvan’s syndrome is a rare autoimmune antibody syndrome causing central, peripheral, and autonomic manifestations. It has been most consistently associated with Caspr2 antibodies, although LGI1 antibodies have also been found in some patients. The Caspr2 protein is located throughout the central and peripheral nervous system. This may account for the diversity of presentations that have been reported in patients with Caspr2 antibodies. The seven core symptoms of Caspr2 antibodies include cognitive dysfunction/seizures, cerebellar symptoms, peripheral nerve hyperexcitability, autonomic dysfunction, insomnia, neuropathic pain, and weight loss. Treatment of Morvan’s syndrome and other Caspr-associated antibody syndromes is largely empirical. Typically, patients are given steroids, intravenous immunoglobulin, and if necessary, other immunosuppressive agents. Prognosis for remission is very good, but relapses may occur.
A Case of a Fall After a Trip
Authors: Miller Aaron E. , DeAngelis Tracy M. , Fabian Michelle , Sand Ilana Katz
Published: October 2018

Guillain-Barré syndrome (GBS) is an acute inflammatory polyradiculoneuropathy that is hypothesized to be most commonly caused through the autoimmune mechanism of molecular mimicry. Typical GBS symptoms include back pain, paresthesiae in the limbs, and ascending, symmetrical muscle weakness. Examination findings in GBS include muscle weakness, areflexia, and a usually mild sensory loss. The majority of GBS patients report an infection one to four weeks before symptom onset. Zika virus (ZIKV) has now been associated with GBS in multiple case-control studies. GBS patients must be monitored for signs of impending respiratory failure and autonomic dysfunction. The treatment for ZIKV-associated GBS is the same as with other GBS cases; intravenous immunoglobulin and plasmapheresis are both thought to be equally effective treatments in ameliorating the condition. The prognosis for recovery from ZIKV-associated GBS is very good, based on the previously reported case series.
A Case of Intractable Vomiting
Authors: Miller Aaron E. , DeAngelis Tracy M. , Fabian Michelle , Sand Ilana Katz
Published: October 2018

Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory disorder affecting the central nervous system, with clinical, imaging, and laboratory characteristics that are distinct from multiple sclerosis. It presents most commonly with optic neuritis, myelitis, or an area postrema syndrome consisting of intractable nausea, vomiting, or hiccups. Most patients are positive for serum antibodies to aquaporin 4. Prompt treatment with corticosteroids and/or plasma exchange is critical for recovery, as is the initiation of disease-modifying therapy with an immunomodulatory agent to prevent future attacks. First-line disease-modifying therapies for NMOSD include rituximab, mycophenolate mofetil, and azathioprine. Several additional agents are used less commonly, and others are currently in clinical trials.
A Case of Progressive Myelopathy
Authors: Miller Aaron E. , DeAngelis Tracy M. , Fabian Michelle , Sand Ilana Katz
Published: October 2018

Primary progressive multiple sclerosis (PPMS) is an inflammatory and neurodegenerative disease affecting the central nervous system. It most commonly presents as a progressive myelopathy, although it may also present as a progressive cerebellar, brainstem, or cognitive syndrome. The diagnosis is supported by brain and spinal cord imaging demonstrating lesions in a pattern consistent with MS, as well as by the presence of unique oligoclonal bands and elevated IgG index in the CSF. MS treatments can be divided into those that are disease-modifying and those that manage symptoms. Ocrelizumab is the first agent to be approved as a disease-modifying therapy for PPMS, based on a large clinical trial demonstrating modest ability to slow disease progression. It is a monoclonal antibody against CD20, resulting in rapid B cell depletion, with a relatively favorable safety profile based on clinical trial data, though long-term data in a large patient population are not yet available. PPMS patients should also be offered both non-pharmacological and pharmacological methods to treat bothersome symptoms such as gait difficulty; spasticity; pain; bowel, bladder, and sexual dysfunction; and cognitive impairment.
A Case of Cranial Neuropathies and Leptomeningeal Enhancement
Authors: Miller Aaron E. , DeAngelis Tracy M. , Fabian Michelle , Sand Ilana Katz
Published: October 2018

IgG4-related disease is an immune-mediated fibroinflammatory syndrome that can affect nearly any organ system. Neurological involvement is uncommon. However, hypertrophic pachymeningitis, hypophysitis, and peripheral neuropathies have been described. Hypertrophic pachymeningitis may present with diffuse symptoms such as headache, cognitive changes, or seizure, as well as with focal symptoms dependent upon the involved anatomical location. Headache and multiple cranial neuropathies are the most common presenting features. Many patients have evidence of peripheral systemic disease at the time of presentation, and serum IgG4 levels are often elevated. A diagnosis of IgG4-related hypertrophic pachymeningitis is best made by exclusion of alternative diagnoses along with the presence of characteristic clinical, radiographic, and histological features. Biopsy is highly recommended. Immediate treatment is with high-dose corticosteroids to be tapered down over time. Steroid-sparing agents are often needed. Rituximab seems promising, but further research is needed.
A Case of Syncope and Impaired Micturition
Authors: Miller Aaron E. , DeAngelis Tracy M. , Fabian Michelle , Sand Ilana Katz
Published: October 2018

Panautonomic failure is manifested by a variety of symptoms indicating dysfunction of the sympathetic, parasympathetic, and enteric autonomic systems. An acute onset of this syndrome, in the absence of other systemic illness or neurological symptoms and signs, indicates autoimmune autonomic ganglionopathy (AAG), in which symptoms are causally related to the presence of antibodies to ganglionic nicotinic acetyl choline receptors. Differential diagnosis consists principally of toxic/metabolic disorders and other autoimmune illnesses, most notably Sjögren’s syndrome. Antibodies to ganglionic nicotinic acetyl choline receptors are present in 50–60% of cases, and, unlike in some other antibody-mediated disorders, symptom severity is related to the antibody level. Paroxysmal coughing spells are a feature of the illness in Japanese, but not Western, patients. Corticosteroids are generally ineffective when used alone, but may provide better response when used in combination with IVIg or plasma exchange. Other immunosuppressive medications are often required.
A Case of Longitudinally Extensive Transverse Myelitis
Authors: Miller Aaron E. , DeAngelis Tracy M. , Fabian Michelle , Sand Ilana Katz
Published: October 2018

Primary CNS angiitis (PACNS) is distinguished from other vasculitides by its exclusive involvement of the CNS, generally in a multifocal manner. Lesions are typically, but not invariably, ischemic or hemorrhagic. Symptoms most frequently include headache, but also commonly involve cognitive impairment, focal neurological symptoms, and sometimes seizures. The disorder may involve medium or small-sized vessels. MRI of brain and spinal cord is usually abnormal, frequently showing cortical and subcortical infarcts. However, in the case of smaller vessel involvement, ischemic demyelination may be seen. Angiography frequently suggests the diagnosis by demonstration of “beading,” but biopsy is often necessary to firmly establish the diagnosis. Treatment is usually with high-dose pulse methylprednisolone followed by a prolonged course of oral prednisone, but the addition of other immunosuppressant medication is often necessary.
A Perplexing Pattern of Weakness
Authors: Miller Aaron E. , DeAngelis Tracy M. , Fabian Michelle , Sand Ilana Katz
Published: October 2018

Inclusion body myositis (IBM), generally considered one of the immune-mediated inflammatory myopathies, should be readily recognizable by its characteristic clinical features. The disorder typically affects middle-aged to older individuals and has a mean age of onset in the early 60s. Onset before the age of 40 is rare. The disease is characteristically asymmetrical and most often has the relatively unique pattern of involvement of the finger flexors (typically with relative sparing of the metacarpophalangeal flexors compared to the more distal finger flexors) and the quadriceps muscles. In the latter, disproportionate wasting of the vastus medialis and lateralis often occur, with relative sparing of the rectus femoris, so that knee extension is often only mildly weak compared to the observed atrophy. Another striking historical feature is the relatively insidious onset of symptoms in comparison to other inflammatory myopathies.
Is the Human Brain Unique?
Authors: Gorman Jack M.
Published: October 2018

Some scientists now argue that humans are really not superior to other species, including our nearest genetic neighbors, chimpanzees and bonobos. Indeed, those animals seem capable of many things previously thought to be uniquely human, including a sense of the future, empathy, depression, and theory of mind. However, it is clear that humans alone produce speech, dominate the globe, and have several brain diseases like schizophrenia. There are three possible sources within the brain for these differences in brain function: in the structure of the brain, in genes coding for proteins in the brain, and in the level of expression of genes in the brain. There is evidence that all three are the case, giving us a place to look for the intersection of the human mind and brain: the expression of genes within neurons of the prefrontal cortex.
Brain Imaging
Authors: Gorman Jack M.
Published: October 2018

The blood–brain barrier vigorously limits what can get into and out of the brain, making our ability to understand brain function much more difficult than with any other organ in the body. The modern era of brain imaging began about a half-century ago with the introduction of computed axial tomography (CAT) and magnetic resonance imaging (MRI). Although CAT scanning shows brain structure in great detail and revolutionized the precision of medical diagnosis, including of brain disorders, it has had relatively little impact on psychiatry because most psychiatric illnesses do not involve visible abnormalities of the size, shape, or volume of brain structures. Similarly, although we have gained some insights from structural MRI, it primarily shows us the anatomy of the brain. Three other variants of MRI, however, have been extremely useful in studying psychiatric issues: functional magnetic resonance imaging, diffusion tensor imaging, and magnetic resonance spectroscopy.
Love, Reward, and Social Connections
Authors: Gorman Jack M.
Published: October 2018

Just as there are pathways for negative emotions and behaviors like fear and despair, the brain has networks that accompany positive ones, such as parental behavior, love, and social connectedness. One such system involves the brain hormones oxytocin and vasopressin, which are known to play a critical role in monogamous pair bonding and in both maternal and paternal nurturing behavior. Another is the so-called reward pathway that originates in the brain stem and terminates in the nucleus acumbens. This pathway allows us to anticipate reward and identify risk, and it reinforces pleasurable experiences. Both systems can also be part of aberrant behaviors like prejudice and drug addiction.
Is There a Science of Psychotherapy?
Authors: Gorman Jack M.
Published: October 2018

Traditionally, psychotherapists have been reluctant to embrace neuroscience, incorrectly believing that it is solely devoted to finding more drugs for psychiatric illnesses. By thinking of psychotherapy as a type of life experience, however, we see that many aspects of neurobiology are relevant to psychotherapy and strengthen our understanding of how psychotherapy works. One example is studies showing that the same brain pathways involved in the acquisition and extinction of conditioned fear in laboratory animals and in anxiety disorders in humans are also affected by cognitive behavioral psychotherapy. Another example is the similarity of the ability to permanently abolish fear memory by blocking its reconsolidation and the reframing of a previously unconscious memory during psychoanalytic psychotherapy. A neuroscience of psychotherapy is certainly conceivable.
Anaesthesia
Authors: Phillips Alistair , Akerman Harry
Published: October 2018

Pain-free surgery can be imposed on the hand and wrist without resort to general anaesthetic. Options include local anaesthetic infiltration which can, in higher volumes mixed with adrenaline, allow surgery without a tourniquet. This technique (wide awake local anaesthetic without tourniquet or WALANT) permits the patient to move the fingers without the muscle paralysis induced by the regional anaesthetic and tourniquet, adding invaluable information, e.g. in tendon transfers. The efficacy of specific peripheral nerve blockade and brachial plexus block can be enhanced by ultrasound or nerve stimulation. Intravenous blockade (Bier’s) is effective. Tourniquets (finger, forearm, above elbow) are essential in hand surgery to provide a view unimpeded by blood (although WALANT can achieve this at the expense of a more oedematous field for procedures in a small field).
Bone and joint injuries of the wrist and forearm
Authors: Naqui Zaf , Warwick David
Published: October 2018

The forearm is a complex quadrilateral structure linked by the proximal and distal radioulnar joints, ligaments, which include the interosseous membrane and triangular cartilage, and several obliquely orientated muscles. A displaced fracture or ligament rupture within this forearm is likely to involve other structures. Treatment requires anatomic recovery of stable function. The ulnar corner can sustain fractures or ligament ruptures which affect stable, pain-free, congruous forearm rotation. The distal radius may fracture after high- or low-energy trauma; anatomic reduction may not be essential in all; inaccuracy may lead to loss of rotation and ulnocarpal abutment but long-term arthritis is unusual. Children’s fractures are managed with consideration of remodeling potential. The scaphoid is vulnerable to non-union; plaster immobilization, early percutaneous fixation, and later bone-grafting all have roles. Salvage for osteoarthritic non-union may reduce pain but compromises function. Rupture of the carpal ligaments may cause substantial disruption and require complex reconstruction.