Amifampridines are the Most Effective Drugs for Treating Lambert-Eaton Myasthenic Syndrome With a Focus on Pediatric Lambert-Eaton Myasthenic Syndrome

In 1983, the first successful trial of 3,4-diaminopyridine (3,4-DAP) in Lambert-Eaton myasthenic syndrome (LEMS) was reported. Efficacy of amifampridine (3,4-DAP and 3,4-diaminopyridine phosphate [3,4-DAPP]) for symptomatic treatment in LEMS was proven by seven randomized studies in 3,4-DAP and two randomized studies in 3,4-DAPP. US Food Drug Administration approved 3,4-DAPP usage for adult LEMS in 2018 and for pediatric LEMS in 2022. Nineteen pediatric LEMS cases were identified in the literature. Compared with adult LEMS, the rate of malignancy is low as expected and the rate of dysautonomia is also low in pediatric LEMS. Unexpected finding is two cases of pediatric LEMS following antecedent infection. Amifampridine can be safely used as long the daily dose is less than 80 mg a day for adult LEMS patients and less than 30 mg a day for pediatric LEMS patients. Amifampridines can be supplemented with a liberal amount of pyridostigmine for long term usage. Amifampridine was used as symptomatic treatment in eight (42%) of 19 pediatric LEMS patients: 3,4-DAP in six and 3,4-DAPP in two patients. The most common practice of 3,4-DAP was a combination with pyridostigmine in four patients. With 3,4-DAP, normal activity was reported in 3 cases and mild to moderate-improvement in other 3 cases. In two patients with 3,4-DAPP, significant improvement in one and no improvement in one. Amifampridines are proven to be effective and safe drugs for the symptomatic treatment without serious side reaction in adults as well as in children as long as the dosage is properly adhered.

Sensory Chronic Inflammatory Demyelinating Polyradiculoneuropathy: Neglected ImmunotherapyResponsive Sensory Neuropathy

Background@#and Purpose To report an improvement with immunotherapy in 34 (85%)/40 patients who required an immunotherapy among 56 patients with sensory chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). @*Methods@#Sensory CIDP was diagnosed when two inclusion criteria are met: 1) acquired, chronic progressive or relapsing symmetrical or asymmetrical sensory polyneuropathy that had progressed for >2 months; and 2) definite electrophysiological and/or biopsy evidence of demyelinating neuropathy. @*Results@#Fifty-six patients with sensory CIDP were identified. Evidence of demyelination was obtained from by the routine motor nerve conduction study (NCS) in 39 (70%) patients, from a nerve biopsy in 10, and from a near-nerve needle sensory NCS in 7 patients. The most prominent laboratory abnormality was a high protein level in the cerebrospinal fluid in 21 (49%) of 43 tested patients. Immunotherapy was required in 41 (79%) of the 52 followed-up patients. An improvement with immunotherapy was observed in 36 (88%)/41 patients. In three patients, motor weakness developed in 5–8 years’ follow-up period and so, their diagnosis was changed to CIDP. @*Conclusions@#Sensory CIDP is responded to an immunotherapy in 88% of the treated patients.Sensory CIDP was diagnosed by the routine motor NCS in 70% of patients and by a sural nerve biopsy in 18% of patients. Thus, sensory CIDP should be recognized as a treatable CIDP variant among the different types of “idiopathic sensory neuropathy.”

Effect of dietary metabolic energy on growth performance, blood homeostasis in ducks under heat stress-related climate change

Aim: This study was carried out to investigate the effect of dietary metabolic energy on growth performance, homeostasis of blood biochemical parameters, immunoglobulin G (IgG), and corticosterone in meat duck exposed to heat stress.Methodology: A total of 320 meat ducks (Cherry valley, Anas platyrhynchos) were randomly allotted into five groups. In all experimental treatment groups, dietary crude protein content was fixed at 18%. metabolic energy level for the control group (thermononeutral temperature, 26°C) was set at 3000 kcal kg-1, while metabolic energy levels for heat stress groups were set at 2900, 3000, 3100 or 3200 kcal kg-1. Heat stress conditions were artificially induced at 36°C with relative humidity of 70% once daily (from 11:00 to 16:00 hr) during finisher periods (day 22 to 42). Results: Compared to control group, heat stress groups with different metabolic energy showed significant decrease in body weight gain, feed intake, red blood cell, and platelets. Heat stress groups with different metabolic energies showed significant increase in feed conversion ratio, blood lipid profiles, aspartate aminotransferase, alanine transferase, electrolytes, pH, gas concentration, immunoglobulin G, and corticosterone compared to the control group.Among heat stress groups, red blood cells and platelets were significantly higher in the heat stress group with metabolic energy of 3000 compared to those in other groups. Blood IgG and corticosterone levels were significantly lower in 2900 and 3000 groups than those in heat stress groups with metabolic energy of 3100 and 3200. Interpretation: The results of this study suggest that dietary metabolic energy 3000 kcal kg-1 level can improve the growth performance of meat duck exposed to heat stress by modulating homeostasis of blood biochemical parameters.

Effect of topical glaucoma medication on tear lipid layer thickness in patients with unilateral glaucoma

Purpose: To compare the lipid layer thickness (LLT) using the LipiView® ocular surface interferometer (TearScience® Inc, Morrisville, NC) between the eye treated with glaucoma medication and untreated normal eye in the unilateral glaucoma patients, and evaluate the effect of topical glaucoma medication on the LLT parameters in glaucoma eyes. Methods: The participants in this cross-sectional comparative study were unilateral glaucoma patients treated with topical glaucoma medications for more than 12 months. Three LLT parameters (average, minimum, and maximum) obtained by the LipiView® were compared between the glaucomatous eye and normal eye. The factors associated with LLT parameters in the eyes treated with glaucoma medication were investigated with multiple regression analysis. Results: Thirty patients with unilateral normal tension glaucoma were enrolled in the present study. Lipid layer average, minimum, and maximum were 64.83 ± 16.50, 51.63 ± 16.73, and 82.53 ± 20.62 in glaucomatous eyes, 77.26 ± 17.81, 62.83 ± 20.99, and 86.13 ± 15.42 in normal eyes. Lipid layer average and minimum were significantly thinner than those in normal eyes (P < 0.001, P < 0.001, respectively). Longer duration of glaucoma eye drops and a greater number of glaucoma medications were associated with the lower LLT average (? = ?0.456, P < 0.001, ? = ?8.517, P = 0.003, respectively), and increasing glaucoma medications have a significant correlation with lower LLT minimum in glaucoma eyes (? = ?8.814, P = 0.026). Conclusion: The present study highlights that patients with long-term glaucoma medications need to be assessed for LLT parameters objectively evaluate their ocular surface health.

Reduction in Omega-6 to Omega 3 ratio and improvement in blood lipid profiles in flaxseed fed Hanwoo steer and human

Aim: To determine the effect of feeding flaxseed on Omega-6 to Omega-3 ratio (n-6/n-3) in Korean native steers (Hanwoo) and effect of flaxseed-fed beef consumption on reducing blood lipid profile and glucose in normal human. Methodology: A total of 60 Hanwoo steers (750 kg b.wt.) were assigned three treatments (20 per treatment). Each treatment group was divided into C (control, feeding basal diets without flaxseed for 40 days before slaughter), FS5 (feeding 5% flaxseed for 60 days before slaughter), and FS7.4 (feeding 7.4% flaxseed for 40 days before slaughter). Fatty acid composition from Hanwoow jugular vein and beef loin were analyzed. Clinical trials were carried out to investigate the effect of consumption of flaxseed-fed beef loin on blood lipid profile and glucose in twenty human subjects. Results: n-6/n-3 ratio in the blood and beef loin of Hanwoo steers were lowered to 2.26-2.27 and 3.67-3.71 in the FS group, respectively, compared with the other groups. Oleic acid level in the blood and beef loin of Hanwoo steers increased to 40.12-42.01 and 52.27-52.79%, respectively, compared with other groups. Blood triacylglycerol, total cholesterol, and low-density lipoprotein-cholesterol levels in normal human fed with FS beef loin reduced by 25.35, 5.22, and 17.59%, compared to those before intake of beef loin. Blood high density lipoprotein-cholesterol (HDL-C) level in normal human fed with FS beef loin was increased by 6.07%. In human subjects fed with FS and C beef loin, blood glucose level was decreased by 6.42 and 11.82%, respectively. Interpretation: The results demonstrated that feeding 5 and 7.4% flaxseed to Hanwoo steers for 40 to 60 days before slaughter could lower n-6 to n-3 ratio and inhance oleic acid in the blood and beef loin. Further, consumption of flaxseed-fed beef loin by human subjects could improve blood lipid profile.

Seropositive Myasthenia Gravis Associated with Small-Cell Lung Carcinoma

BACKGROUND: Lambert-Eaton myasthenic syndrome is well known to be a classical paraneoplastic syndrome of small cell lung carcinoma (SCLC). Three cases of seronegative myasthenia gravis (MG) and SCLC were previously reported. CASE REPORT: A 65-year-old man developed a severe progressive respiratory failure with clinical features of MG. Tests showed a decremental response in the repetitive nerve stimulation test, abnormal single-fiber electromyography, and positive acetylcholine receptor antibody. SCLC was confirmed by the lung biopsy. CONCLUSIONS: This case represents the first case of seropositive MG and SCLC.

True neurogenic thoracic outlet syndrome in a competitive swimmer: a case report of this rare association

True neurogenic thoracic outlet syndrome (TOS) is an uncommon disorder despite of be a frequent reason for referral to the EMG laboratories. We describe the second case in the literature of true TOS in a competitive swimmer with progressive weakness and severe atrophy of the left thenar eminence. EMG showed lower trunk plexopathy. X-ray and MRI of the cervical spine and brachial plexus were normal. Surgical exploration evidenced the lower trunk retracted and pulled by a fibrous band. It was excised and neurolysis of the plexus was done with gradual improvement of function. We discuss the possible pathophysiology of this association.

A síndrome do desfiladeiro torácico verdadeiro é condição incomum, apesar de ser uma razão freqüente de encaminhamento aos laboratórios de eletroneuromiografia. Nós descrevemos o segundo caso na literatura desta doença rara em um nadador competitivo com fraqueza e atrofia severa da eminência tenar esquerda. A ENMG mostrou plexopatia do tronco inferior. O RX e as ressonâncias magnéticas da coluna cervical e do plexo braquial foram normais. Exploração cirúrgica mostrou o tronco inferior tracionado por uma banda fibrosa. Ela foi ressecada e procedeu-se a neurólise do plexo com melhora gradual da função. Nós discutimos a possível fisiopatologia desta associação.

Abnormal dip phenomenon: a characteristic electrophysiological marker in interdigital neuropathy of the foot

OBJECTIVE: The nerve conduction findings in interdigital neuropathy of the foot (IDN; Morton's neuroma) have rarely been reported. We analyzed the nerve conduction data in 23 patients with suspected IDN studied between 1982 and 2002. METHOD: Diagnosis of IDN was made on the basis of clinical features. All patients underwent routine nerve conduction studies and a near-nerve needle sensory nerve conduction study of the interdigital nerves by Oh's method in the symptomatic foot. RESULTS:Of the 23 patients, the diagnosis of definite IDN was made in 13 cases and of possible NDN in the others cases. Nineteen were females. Twenty two patients had only one nerve affected. One patient had two nerves affected. The III-IV interdigital nerve was affected in 17 cases and the II-III interdigital nerve in 7 cases. The near-nerve needle nerve conduction showed abnormality in the affected interdigital nerves in all definite IDN cases and confirmed the diagnosis of IDN in 10 cases by the abnormal dip phenomenon (a selective decrease of 50 percent or more in the sensory CNAP amplitude of the affected nerve compared with that of the preceding interdigital nerve). In 11 possible IDN cases, IDN was identified by the abnormal dip phenomenon. CONCLUSION: The near-nerve needle sensory nerve conduction of the interdigital nerves is a highly sensitive diagnostic test, and abnormal dip phenomenon is the most characteristic electrophysiological marker for the diagnosis of IDN.

OBJETIVO: Os achados da condução nervosa na neuropatia interdigital do pé (NIP) têm sido raramente descritos. Nós analisamos os dados da condução nervosa de 23 pacientes com suspeita de NIP entre 1982 e 2002. MÉTODO: O diagnóstico de NIP foi clínico. Todos os pacientes foram submetidos a estudos de condução nervosa de rotina e ao estudo de condução sensitiva dos nervos interdigitais com agulha justa-neural pelo método de Oh. RESULTADOS: Dos 23 pacientes, o diagnóstico de NIP foi definitivo em 13 casos é possível nos demais. Dezenove pacientes eram mulheres e 22 tinham somente um nervo afetado. Apenas um paciente teve dois nervos comprometidos. O nervo interdigital III-IV foi afetado em 17 casos e o nervo interdigital II-III em 7 casos. A condução nervosa justa-neural foi anormal nos nervos interdigitais acometidos em todos os casos definitivos e confirmou o diagnóstico de neuropatia interdigital em 10 casos pelo fenômeno da diminuição de amplitude anormal (uma diminuição seletiva de 50 por cento ou mais da amplitude do PANS do nervo afetado quando comparado com o nervo anterior. Em 11 casos possíveis, a neuropatia interdigital foi identificada pelo fenômeno da diminuição de amplitude anormal. CONCLUSÃO: A condução nervosa sensitiva justa-neural dos nervos interdigitais do pé é um teste diagnóstico altamente sensível e o fenômeno da diminuição da amplitude anormal é o marcador eletrofisiológico mais característico para o diagnóstico de neuroma de Morton.

Control motor y nocicepción: parte II: hemiespasmo facial y toxina botulínica / Motor control and nociception: parte II: hemifacial spasm and botulinum toxin

Quince sujetos, 9 con hemiespasmo facial y 6 controlados, neurológica y neurofisiológicamente normales, fueron investigados en el laboratorio. Los 9 pacientes tenían una edad promedio de 57.4 años y una duración promedio de la enfermedad de 22.2 meses. En todos los pacientes se practicó el reflejo orbicular de los ojos de acuerdo con metodologías validadas en estudios previos, a nivel internacional. En 5 de los 9 pacientes y en los controles se pudo registrar el ROO de manera simultánea, tanto en el músculo orbicularis oculi (MOO) como en el músculo orbicularis oris (MOOr) bilateralmente. El ROO se practicó en los pacientes antes y después de la aplicación de toxina botulínica. Las latencias y las amplitudes de las respuestas obtenidas se midieron pre y post toxina botulínica en el MOO y en el MOOr. El detectar respuestas anormales en pacientes con HeFa, en músculos distantes al sitio de estimulación como aquellos observados a nivel contralateral del NeSo estimulado, sugiere que en esta patología estas respuestas aparecen no sólo por las sinkinesias neurales que se desarrollan en el curso de la enfermedad sino que además existe una hiperexcitabilidad neuronal alrededor de las interneuronas del tallo cerebral que median esta clase de respuestas. De otro lado, la modificación de la actividad anormal que produce la aplicación de la toxina botulínica se debe no sólo a la acción de la misma a nivel local en la unión neuromuscular, sino que existe además una modificación de la información aferente a nivel supraespinal con subsecuente reorganización del programa central del control motor en los humanos

Molecular mechanisms of angiogenesis: experimental models define cellular trafficking of FGF-1

Numerous studies have established that stimulation of cell growth by members of the fibroblast growth factor (FGF) family of polypeptides is dependent upon an extracellular pathway. Acidic FGF (FGF-1), however, lacks a classical signal sequence for secretion, thereby making it difficult to evaluate regulation of biological activity by this growth factor. Efforts in this laboratory have utilized molecular techniques of retrovirology and transgenic modeling to introduce cDNA sequences encoding either an intracellular or extracellular form of FGF-1 into primary diploid cells to examine trafficking and compartmentalization of FGF-1. Several lines of evidence obtained from these models provide a compelling argument that the stimulation of FGF-1-associated cellular transformation is restricted to an extracellular, receptor-mediated pathway, involving protein tyrosine phosphorylation and nuclear localization. In addition, an unconventional secretion pathway for intracellular FGF-1 has been identified that involves mechanisms associated with oxidative stress