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Perry Shieh, Khema Sharma, Bruce Kohrman, Shin J Oh
OBJECTIVE: To assess tolerability and efficacy of amifampridine phosphate versus placebo for symptomatic treatment of Lambert-Eaton Myasthenic Syndrome (LEMS). METHODS: This phase 3 randomized, double-blind, placebo-controlled withdrawal trial in 26 adults with LEMS compared efficacy of amifampridine phosphate versus placebo over a 4-day period. The primary endpoints were quantitative myasthenia gravis score (QMG) and subject global impression, and the secondary endpoint was Clinical Global Impression-Improvement...
March 2019: Journal of Clinical Neuromuscular Disease
Sherene Loi, Anita Giobbie-Hurder, Andrea Gombos, Thomas Bachelot, Rina Hui, Giuseppe Curigliano, Mario Campone, Laura Biganzoli, Hervé Bonnefoi, Guy Jerusalem, Rupert Bartsch, Manuela Rabaglio-Poretti, Roswitha Kammler, Rudolf Maibach, Mark J Smyth, Angelo Di Leo, Marco Colleoni, Giuseppe Viale, Meredith M Regan, Fabrice André
BACKGROUND: HER2-positive breast cancers usually contain large amounts of T-cell infiltrate. We hypothesised that trastuzumab resistance in HER2-positive breast cancer could be mediated by immune mechanisms. We assessed the safety and anti-tumour activity of pembrolizumab, a programmed cell death protein 1 (PD-1) inhibitor, added to trastuzumab in trastuzumab-resistant, advanced HER2-positive breast cancer. METHODS: We did this single-arm, multicentre, phase 1b-2 trial in 11 centres based in five countries...
February 11, 2019: Lancet Oncology
Hiroko Kitanosono, Masakatsu Motomura, Hiroki Tomita, Hiroshi Iwanaga, Naoki Iwanaga, Takashi Irioka, Hirokazu Shiraishi, Akira Tsujino
A 63-year-old female who developed dizziness, diplopia and subsequent gait disturbance from September X-1 year was analyzed. The first neurological findings in May X year revealed cerebellar ataxia, weakness in the proximal limbs, decreased tendon reflexes, and autonomic symptoms (ADL:mRS 3). Furthermore, an incremental phenomenon was observed in the repetitive nerve stimulation test, and she was diagnosed with Lambert-Eaton myasthenic syndrome (LEMS) based on the serum P/Q-type calcium channel (VGCC) antibody positivity...
February 2019: Brain and Nerve, Shinkei Kenkyū No Shinpo
Sasha A Mansukhani, Erick Bothun, Nancy N Diehl, Brian G Mohney
PURPOSE: To report the incidence, demographics, and ocular findings of children with myasthenia DESIGN: Retrospective cohort study METHODS: The medical records of all children (< 19 years) examined at Mayo Clinic with any form of myasthenia from January 1 1966, through December 31, 2015, were retrospectively reviewed. RESULTS: A total of 364 children were evaluated during the study period, of which 6 children were residents of the Olmsted County at the time of their diagnosis, yielding an annual age- and sex-adjusted incidence of 0...
January 14, 2019: American Journal of Ophthalmology
Yuki Nakatani, Natsuki Tanaka, Tomomi Enami, Seigo Minami, Tomoko Okazaki, Kiyoshi Komuta
Lambert-Eaton myasthenic syndrome (LEMS) is a representative paraneoplastic neurological syndrome. Recently, nivolumab, an anti-programmed cell death 1 inhibitor, has been approved for advanced non-small-cell lung cancer. Careful attention should be paid to immune-related adverse events (irAEs), including neurotoxicity. We herein report a 73-year-old woman with LEMS that occurred during nivolumab treatment for pulmonary squamous cell carcinoma. After the 20th week of nivolumab, she experienced various neurological symptoms such as ptosis, lower limb weakness, and photophobia...
September 2018: Case Reports in Neurology
Adriano M Priola, Dario Gned, Andrea Veltri, Sandro M Priola
History A 29-year-old woman presented with a 6-month history of progressive general fatigue, fluctuating limb weakness, and difficulty climbing stairs. She initially experienced occasional episodes of transient diplopia that developed while reading in the evening. She subsequently started to experience dry eyes and mouth, difficulty chewing, and mild dysphagia that worsened throughout the day. Her medical history included hypothyroidism from Hashimoto thyroiditis and pneumonia with left pleural effusion. She had no smoking history, and her body mass index was normal (23...
January 2019: Radiology
Nataly I Montes-Chinea, Zhuo Guan, Marcella Coutts, Cecilia Vidal, Steve Courel, Adriana P Rebelo, Lisa Abreu, Stephan Zuchner, J Troy Littleton, Mario A Saporta
Objective: To report a new SYT2 missense mutation causing distal hereditary motor neuropathy and presynaptic neuromuscular junction (NMJ) transmission dysfunction. Methods: We report a multigenerational family with a new missense mutation, c. 1112T>A (p. Ile371Lys), in the C2B domain of SYT2 , describe the clinical and electrophysiologic phenotype associated with this variant, and validate its pathogenicity in a Drosophila model. Results: Both proband and her mother present a similar clinical phenotype characterized by a slowly progressive, predominantly motor neuropathy and clear evidence of presynaptic NMJ dysfunction on nerve conduction studies...
December 2018: Neurology. Genetics
Shin J Oh, Dushin Jeong, Ikjae Lee, Mohammad Alsharabati
INTRODUCTION: We are reporting our experience with the repetitive nerve stimulation (RNS) test in myasthenia gravis (MG) crisis. METHODS: The various parameters of the RNS tests in 26 patients with MG crisis were analyzed. RESULTS: In 18 (69%) patients, MG crisis is the first manifestation of MG. The RNS tests were abnormal in 24 (92%) patients by decrement at low-rate stimulation in any of four tested muscles. Three patterns of abnormality were found: MG pattern (decrement at low-rate stimulation) in 23 patients, Lambert-Eaton myasthenic syndrome pattern in one patient, and cholinergic crisis pattern in one patient...
November 29, 2018: Muscle & Nerve
Paul Maddison, Maarten J Titulaer, Jan J Verschuuren, Paul Gozzard, Bethan Lang, Sarosh R Irani, Lidia Sabater, Francesc Graus, Andrea Murray, Caroline J Chapman
Antibodies to SOXB1 proteins in patients with paraneoplastic disorders are associated with small-cell lung cancer (SCLC), particularly in Lambert-Eaton myasthenic syndrome (LEMS). We aimed to establish if SOX2 antibodies could be used to identify SCLC and other tumours found in a range of paraneoplastic disorders and controls. SOX2 antibodies were detectable in 61% of patients with LEMS-SCLC, and in other paraneoplastic disorders, such as opsoclonus-myoclonus and paraneoplastic cerebellar degeneration, only when there was an underlying SCLC...
November 8, 2018: Journal of Neuroimmunology
David Lacomis, Araya Puwanant
This update begins with myasthenia gravis and the roles of anti-agrin and cortactin antibodies. Regarding diagnosis, a report on repeated ice pack testing is highlighted as are several reports on the close correlation of electrodiagnostic testing with clinical features and the response to treatment. The incidence of head drop and associated clinical and ventilatory features are gleaned from a retrospective study. We also discuss a study that assessed the predominantly symmetric and conjugate ocular findings in MuSK-myasthenia gravis...
December 2018: Journal of Clinical Neuromuscular Disease
B Mark Hoffman, Nuhad Abou Zeid, Umar Alam, James B Caress
BACKGROUND: Alemtuzumab administration is known to cause secondary autoimmune disease but has not been associated with the development of neurologic autoimmune conditions. Lambert-Eaton myasthenic syndrome (LEMS) is caused by autoantibodies directed against calcium channels on the neuromuscular junction. CASE REPORT: We report a case of a patient with relapsing-remitting multiple sclerosis (RRMS) treated with alemtuzumab who develop generalized weakness initially attributed to progression of MS but eventually determined to be due to LEMS...
October 22, 2018: Multiple Sclerosis and related Disorders
Jessica Frey, Ann Murray
BACKGROUND: Voltage gated calcium channels (VGCC) are well-known targets for antibody-associated disease. Of the 5 VGCC subtypes, the most well-known is the P/Q subtype associated with Lambert-Eaton Myasthenic Syndrome (LEMS). However, this case focuses on the much less understood N-type calcium channel antibody. The objective of this case is to review the literature regarding the clinical significance of the N-type calcium channel antibody and its relationship to MS. METHODS: A 37-year old male presented with vertigo, paranoia, and tremor and had MRI changes suggestive of demyelinating disease...
November 2018: Multiple Sclerosis and related Disorders
Vincenzo Todisco, Giovanni Cirillo, Rocco Capuano, Alessandro d'Ambrosio, Gioacchino Tedeschi, Antonio Gallo
Objective: To report the clinical features and the neurophysiological approach of a patient with Lambert-Eaton myasthenic syndrome (LEMS), highlighting the diagnostic role of the stimulated single fiber electromyography (sSFEMG). Case report: A 60-year-old woman presenting with the LEMS triad (proximal and axial weakness, autonomic dysfunction and areflexia) was evaluated by neurophysiological tests (electroneuromyography, repetitive stimulation test (TSR), voluntary and stimulated SFEMG)...
2018: Clinical Neurophysiology Practice
Guoqian Chen, Zhanli Fu, Xueqi Chen, Qian Li
A 46-year-old woman suffering from lower extremity weakness, dysarthria, dysphagia, dyspnea, and dry mouth was suspected of having Lambert-Eaton myasthenic syndrome based on the characteristic electromyographic abnormalities of right ulnar nerve, which demonstrated reduced resting compound muscle action potential amplitude and greater than 100% incremental change in high-frequency repetitive nerve stimulation. An F-FDG PET/CT was performed to detect the underlying neoplasm and revealed a retroperitoneal soft tissue mass with high F-FDG avidity in the left iliac fossa...
September 2018: Clinical Nuclear Medicine
Hakan Cetin, Angela Vincent
Autoimmune myasthenic syndromes are antibody-mediated disorders of the neuromuscular junction. Common antigenic targets are the acetylcholine receptor or muscle specific kinase (MuSK) in myasthenia gravis (MG) and the voltage-gated calcium channel in Lambert-Eaton myasthenic syndrome. There is evidence that antibodies directed against other antigens such as low-density lipoprotein receptor-related protein 4 (LRP4) are also involved in MG. The mechanisms by which various antibodies exert their pathogenic effect depend on the IgG subclass and also the epitope location on the antigens...
June 2018: Seminars in Neurology
Rodica Di Lorenzo, Karin Mente, Jianbo Li, Luay Shayya, Alexander Rae-Grant, Yuebing Li, Adham Jammoul
As testing for neuronal antibodies become more readily available, the spectrum of conditions potentially associated with these autoantibodies has been widening. Voltage-gated calcium channel antibodies (VGCC-Ab) are no exception to this trend. The significance of an elevated VGCC-Ab titer beyond its original clinicopathological correlate, Lambert-Eaton myasthenic syndrome (LEMS) remains undetermined. We sought to determine the diagnostic significance of an elevated serum VGCC-Ab titer in a large single-center cohort of 100 patients...
July 9, 2018: Journal of Neurology
A Bussat, S Langner-Lemercier, A Salmon, F Mouriaux
Paraneoplastic syndromes involving the visual system are a heterogeneous group of disorders occurring in the setting of systemic malignancy. Although these syndromes are rare, the clinical manifestations can herald an unsuspected, underlying malignancy. The associated antibodies and histopathology of each syndrome are presented to help in the understanding of these autoimmune phenomena. Outlined in this review article are diagnostic features useful in differentiating cancer-associated retinopathy, melanoma-associated retinopathy, paraneoplastic polymorphous vitelliform maculopathy, bilateral diffuse uveal melanocytic proliferation and paraneoplastic neurologic syndromes such as optic neuropathy, opsoclonus-myoclonus, Lambert-Eaton myasthenia and paraneoplastic cerebellar degeneration...
June 2018: Journal Français D'ophtalmologie
Rui Jia, Jinbo Chen, Ruli Ge, Qi Zheng, Fang Chen, Zhonghua Zhao
RATIONALE: Myasthenia gravis (MG) and Lambert-Eaton myasthenic syndrome (LEMS) are both neuromuscular junction diseases, and some controversy exists whether the 2 diseases occur at the same time. PATIENT CONCERNS: We report a case that a patient with presentation of acetylcholine receptor (AChR) antibody positive MG and LEMS associated with small cell lung cancer (SCLC). DIAGNOSES: The patient firstly suffered from fluctuant symptoms, including slurred speech, double eyelid ptosis, and weakness of limbs...
June 2018: Medicine (Baltimore)
A Bussat, S Langner-Lemercier, A Salmon, F Mouriaux
Paraneoplastic syndromes involving the visual system are a heterogeneous group of disorders occurring in the setting of systemic malignancy. They are unrelated to local tumor invasion or metastasis and unexplained by nutritional, metabolic, infectious or iatrogenic causes. The presence of antibodies may aid in the diagnosis of a paraneoplastic syndrome, although this is not an absolute requirement. Chorioretinal involvement is seen in CAR (cancer-associated retinopathy) syndrome, MAR (melanoma-associated retinopathy) syndrome, paraneoplastic vitelliform maculopathy, and bilateral diffuse uveal melanocytic proliferation...
May 2018: Journal Français D'ophtalmologie
Hongfang Li, Aimei Zhang, Yanlei Hao, Hongzhi Guan, Zhanyun Lv
RATIONALE: Autoimmune encephalitis and Lambert-Eaton myasthenic syndrome are classic paraneoplastic neurological conditions common in patients with small cell lung cancer. PATIENT CONCERNS: The patient complained of tiredness, fluctuating recent memory loss, and inability to find his home. His family members reported a change in character, irritability, and paranoia. One month later, the patient had 1 grand mal seizure lasting 5 minutes. DIAGNOSIS: The patient was diagnosed with limbic encephalitis combined with Lambert-Eaton myasthenic syndrome...
May 2018: Medicine (Baltimore)
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