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Lambert eaton

Isabell Cordts, Fabian Funk, Jörg B Schulz, Joachim Weis, Kristl G Claeys
Tubular aggregates are accumulations of densely packed tubules in muscle fibers, occurring in distinct hereditary and acquired disorders. We present a patient with tubular aggregates and autoimmune Lambert-Eaton myasthenic syndrome. Initially, he showed mild proximal weakness, borderline decrement on 3 Hz stimulation, and slightly elevated creatine kinase. Muscle biopsy revealed tubular aggregates in type II fibers. Due to a good response to pyridostigmine, a limb-girdle myasthenia with tubular aggregates was suspected, but genetic analyses of GFPT1, DPGAT1, and ALG2 were normal...
September 20, 2016: Neuromuscular Disorders: NMD
Amit A Kothari, A L Kakrani
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
Paulo Victor Sgobbi de Souza, Gabriel Novaes de Rezende Batistella, Valéria Cavalcante Lino, Wladimir Bocca Vieira de Rezende Pinto, Marcelo Annes, Acary Souza Bulle Oliveira
Neuromuscular junction disorders represent a wide group of neurological diseases characterized by weakness, fatigability and variable degrees of appendicular, ocular and bulbar musculature involvement. Its main group of disorders includes autoimmune conditions, such as autoimmune acquired myasthenia gravis and Lambert-Eaton syndrome. However, an important group of diseases include congenital myasthenic syndromes with a genetic and sometimes hereditary basis that resemble and mimick many of the classic myasthenia neurological manifestations, but also have different presentations, which makes them a complex clinical, therapeutic and diagnostic challenge for most clinicians...
September 2016: Arquivos de Neuro-psiquiatria
Brett M Morrison
Neuromuscular diseases are a broadly defined group of disorders that all involve injury or dysfunction of peripheral nerves or muscle. The site of injury can be in the cell bodies (i.e., amyotrophic lateral sclerosis [ALS] or sensory ganglionopathies), axons (i.e., axonal peripheral neuropathies or brachial plexopathies), Schwann cells (i.e., chronic inflammatory demyelinating polyradiculoneuropathy), neuromuscular junction (i.e., myasthenia gravis or Lambert-Eaton myasthenic syndrome), muscle (i.e., inflammatory myopathy or muscular dystrophy), or any combination of these sites...
October 2016: Seminars in Neurology
Uma Sundar, Sanjay Gulhane, D Asole
Lambert Eaton myasthenic syndrome is a presynaptic neuromuscular junction disorder, which has unique features on electrodiagnostic testing. Here we describe a middle aged lady with symmetric, progressive, areflexic weakness in lower limbs, who had the typical electrodiagnostic findings of diffuse attenuation of motor amplitudes, and increase in these amplitudes after brief exercise of the muscle sampled.
December 2015: Journal of the Association of Physicians of India
Robert S Rogers, Hiroshi Nishimune
The synapse between motor neurons and skeletal muscle is known as the neuromuscular junction (NMJ). Proper alignment of presynaptic and post-synaptic structures of motor neurons and muscle fibers, respectively, is essential for efficient motor control of skeletal muscles. The synaptic cleft between these two cells is filled with basal lamina. Laminins are heterotrimer extracellular matrix molecules that are key members of the basal lamina. Laminin α4, α5, and β2 chains specifically localize to NMJs, and these laminin isoforms play a critical role in maintenance of NMJs and organization of synaptic vesicle release sites known as active zones...
September 7, 2016: Matrix Biology: Journal of the International Society for Matrix Biology
Kwok-Ying Chan, Richard Shek-Kwan Chang, Vikki Wai-Kee Lau, Man-Lui Chan, Theresa Lai
Lambert-Eaton myasthenic syndrome (LEMS) is an uncommon autoimmune idiopathic or paraneoplastic syndrome producing antibodies against voltage presynaptic calcium channels. According to previous studies, many patients with LEMS experience remission in both the clinical symptoms of muscle weakness and the electrophysiologic abnormalities after successful treatment of lung SCC. However, some patients might not respond to conventional therapy and eventually require palliative care. Hereby, we reported a LEMS patient with advanced lung malignancy was referred for palliative care...
June 22, 2016: Annals of Palliative Medicine
Divpreet Kaur, Divisha Raheja
The presence of P/Q type voltage gated calcium channel (VGCC) antibodies has been strongly correlated with Lambert Eaton Syndrome (LES), present in 90% of non-immunocompromised patients with LES. However, there have been case reports which have shown its association between paraneoplastic syndrome affecting both central nervous system and the peripheral nervous system causing encephalomyelitis and sensory neuronopathy/neuropathy. We present a case of a young man, who presented with encephalomyelitis, and was further noted to have superimposed cervical polyradiculopathy associated with P/Q type VGCC antibodies...
November 2016: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
Miguel Ángel Merino-Ramírez, Charles F Bolton
Lambert-Eaton syndrome (LES) is a rare immune-mediated disorder characterized by proximal leg weakness, autonomic symptoms and hypoactive tendon reflexes. The paraneoplastic form is associated with small-cell lung cancer in 50-60% of cases, whereas the remaining cases are found in younger adults with a higher likelihood of coexisting autoimmune disease. The early recognition of LES is crucial for improving clinical outcomes but remains a major challenge. In this review, we analyze the clinical characteristics and diagnostic considerations in treating LES through a series of three case studies, one of which showed definitive response to pyridostigmine and corticosteroid combination therapy, followed by spontaneous remission...
September 2016: Canadian Journal of Neurological Sciences. le Journal Canadien des Sciences Neurologiques
K A Eaton, H A Lloyd, M Wheeler, J Sullivan, C Klass, Y Allen, S Lambert-Humble
Objective This pilot study aimed to produce and evaluate training resources and training in oral health care, including oral hygiene, for carers in care homes in Surrey and Medway.Methods During two training days, for carers from these homes, short, interactive presentations were given on a range of topics relevant to oral health care and oral hygiene of older people, followed by practical training. Prior to any training all attendees completed a 39 question questionnaire to establish their baseline knowledge of oral health and hygiene...
July 8, 2016: British Dental Journal
Fumio Asano, Keisuke Watanabe, Masaharu Shinkai, Yoshitaka Tei, Kei Mishina, Mikiko Tanabe, Hiroshi Ishii, Masahiro Shinoda, Tadasuke Shimokawaji, Makoto Kudo, Takeshi Kaneko
Lambert-Eaton myasthenic syndrome (LEMS) is a paraneoplastic syndrome and only 3% of small cell lung carcinoma (SCLC) patients have LEMS. Moreover, the recurrence of SCLC after a disease-free survival (DFS) of more than 10 years is rare. We report a patient who had a recurrence of both SCLC and LEMS after a 13-year DFS period. A 69-year-old man was diagnosed with LEMS and SCLC (cT0N2M0, stage IIIA) 13 years ago. Chemoradiotherapy was performed and a complete response was achieved. With anticancer treatment, the LEMS symptoms was alleviated...
2016: Chinese Journal of Cancer
N G Fiona, Diana C Lee, Leah A Schrumpf, Mary E Mazurek, Victoria Lee Lo, Sharleen K Gill, Ricardo A Maselli
INTRODUCTION: We investigated the effects of 3,4 diaminopyridine (3,4-DAP) and its acetylated metabolite, N-(4-Amino-pyridin-3-yl) acetamide (3-Ac), at the mammalian neuromuscular junction. METHODS: Quantal release of acetylcholine was studied in diaphragm muscles of mice, using in vitro intracellular microelectrode recordings. RESULTS: Under conditions of low probability of release, 3,4-DAP produced a 1000% increase in quantal release, but 3-Ac had no effect...
June 2, 2016: Muscle & Nerve
Jayasri G Iyer, Kaushik Parvathaneni, Shailender Bhatia, Erica S Tarabadkar, Astrid Blom, Ryan Doumani, Jill McKenzie, Maryam M Asgari, Paul Nghiem
BACKGROUND: Paraneoplastic syndromes (PNS) are commonly associated with neuroendocrine cancers, such as small cell lung cancer. OBJECTIVES: We examined the association of PNS in Merkel cell carcinoma (MCC), a rare neuroendocrine skin cancer. METHODS: We identified PNS associated with MCC based on chart review of a Seattle-based repository and examined the incidence of MCC-associated hyponatremia in an independent cohort within Kaiser Permanente Northern California...
September 2016: Journal of the American Academy of Dermatology
Jan Verschuuren, Ellen Strijbos, Angela Vincent
Diseases of the neuromuscular junction comprise a wide range of disorders. Antibodies, genetic mutations, specific drugs or toxins interfere with the number or function of one of the essential proteins that control signaling between the presynaptic nerve ending and the postsynaptic muscle membrane. Acquired autoimmune disorders of the neuromuscular junction are the most common and are described here. In myasthenia gravis, antibodies to acetylcholine receptors or to proteins involved in receptor clustering, particularly muscle-specific kinase, cause direct loss of acetylcholine receptors or interfere with the agrin-induced acetylcholine receptor clustering necessary for efficient neurotransmission...
2016: Handbook of Clinical Neurology
Marilyn McKasson, Stacey L Clardy, Susan A Clawson, Kenneth E Hill, Blair Wood, Noel Carlson, Mark Bromberg, John E Greenlee
OBJECTIVES: To describe response to treatment in a patient with autoantibodies against voltage-gated calcium channels (VGCCs) who presented with autoimmune cerebellar degeneration and subsequently developed Lambert-Eaton myasthenic syndrome (LEMS), and to study the effect of the patient's autoantibodies on Purkinje cells in rat cerebellar slice cultures. METHODS: Case report and study of rat cerebellar slice cultures incubated with patient VGCC autoantibodies. RESULTS: A 53-year-old man developed progressive incoordination with ataxic speech...
June 2016: Neurology® Neuroimmunology & Neuroinflammation
Francesc Miralles
Myasthenia gravis (MG) is an autoimmune postsynaptic disorder of neuromuscular transmission caused, in most patients, by antibodies against postsynaptic acetylcholine receptors. Lambert-Eaton myasthenic syndrome (LEMS) is a presynaptic autoimmune disease in which there is a reduction in Ca(2+) entry with each impulse due to the action of antibodies against Ca(2+) channels. These diseases have a distinct pattern of response to low-frequency repetitive nerve stimulation which allows its recognition in a particular subject...
March 25, 2016: Medical & Biological Engineering & Computing
Jae-Won Hyun, Su-Hyun Kim, In Hye Jeong, Heung Tae Kim, Ho Jin Kim
Lambert-Eaton myasthenic syndrome (LEMS) is an autoimmune disorder of the neuromuscular junction that is characterized by muscle weakness. LEMS is usually associated with cancer, most commonly small cell lung cancer (SCLC). The potassium-channel blocker 3,4-diaminopyridine (3,4-DAP), has been previously used for the symptomatic treatment of LEMS. 3,4-DAP increases the release of acetylcholine and prolongs the duration of nerve action potentials at the presynaptic membrane of the neuromuscular junction. The present study describes the case of a patient with LEMS and SCLC, for which the symptoms did not improve with anticancer therapy, but did so markedly following treatment with 3,4-DAP...
March 2016: Oncology Letters
Shin J Oh, Natalya Shcherbakova, Anna Kostera-Pruszczyk, Mohammad Alsharabati, Mazen Dimachkie, Jose Munoz Blanco, Thomas Brannagan, Dragana Lavrnić, Perry B Shieh, Christophe Vial, Andreas Meisel, Samuel Komoly, Benedikt Schoser, Kumaraswamy Sivakumar, Yuen So
OBJECTIVE: We evaluated the efficacy and safety of amifampridine phosphate (Firdapse(®)) for symptomatic treatment in Lambert-Eaton myasthenic syndrome (LEMS). METHODS: Phase 3, randomized, double-blind, study. Patients were treated initially with amifampridine phosphate for 7-91 days, followed by randomization to continue amifampridine phosphate for 14 days or placebo (7-day taper, 7-day placebo). The primary efficacy endpoints were changes from baseline at day 14 in Quantitative Myasthenia Gravis and Subject Global Impression scores...
May 2016: Muscle & Nerve
Lixin Zhou, Hongzhi Guan, Hongsheng Liu, Yicheng Zhu, Bin Peng, Shanqing Li, Liying Cui
OBJECTIVE: The aim of this study is to explore the clinical characteristics, treatment and prognosis of pareneoplastic neurological syndrome (PNS) associated with small cell lung cancer (SCLC) in a single-center cohort. METHODS: We retrospectively and consecutively reviewed 34 patients (22 male and 12 female, mean age 55.4 years old) diagnosed as PNS associated with SCLC in our hospital from 2001 to 2014 and investigated the clinical features, related antibodies, treatment and prognosis in this cohort...
October 6, 2015: Zhonghua Yi Xue za Zhi [Chinese medical journal]
Nicholas L Zalewski, Vanda A Lennon, Daniel H Lachance, Christopher J Klein, Sean J Pittock, Andrew Mckeon
INTRODUCTION: Voltage-gated calcium-channel autoimmunity (VGCC-P/Q and VGCC-N types) occurs beyond Lambert-Eaton syndrome and lung cancer. METHODS: We reviewed records for 236 Mayo Clinic patients with VGCC antibodies found in evaluation for paraneoplastic neurological autoimmunity (generally without myasthenic syndromes). RESULTS: VGCC autoantibodies were detected in 3.4% of neurological patients, 1.7% of healthy controls, and 4% of neurologically asymptomatic lung cancer controls...
August 2016: Muscle & Nerve
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