UNUSUAL DECREMENTAL RESPONSE IN LAMBERT EATON MYASTHENIC SYNDROME

2015 ◽  
Vol 86 (11) ◽  
pp. e4.50-e4
Author(s):  
Girija Sadalage ◽  
Sajjad Ali ◽  
Paul Mocroft ◽  
Adrian Williams ◽  
Saiju Jacob

Lambert Eaton Myasthenic Syndrome (LEMS) is an autoimmune disorder affecting the neuromuscular junction caused by antibodies against voltage gated calcium channel antibodies. The neurophysiological hallmark of LEMS is incremental response to repetitive nerve stimulation, especially at high frequency stimulation.A 54-year-old gentleman presented to the Neurology clinic with a 9-month history of progressive walking difficulty. He had difficulty standing up from a sitting down position and the symptoms were worse towards the end of the day. He had a transient history of dry mouth when the symptoms started, but denied any postural dizziness, sweating or bowel/bladder symptoms. He denied having any double vision, droopy eyelids, speech or swallowing symptoms. He had lost 1.5 stones in weight over the preceding 7–8 months. Examination showed proximal muscle weakness with reduced reflexes, which could not be potentiated. A clinical suspicion of LEMS was made, but the neurophysiological examination (repeated on three occasions) showed consistently decremental response at low and high frequency stimulations.The VGCC antibody was positive (293, normal 0–45), confirming the clinical suspicion of LEMS. The AChR and MuSK antibodies were negative.This is an unusual neurophysiological finding of a decremental response in a patient with clinical and immunological evidence of LEMS.

2013 ◽  
Vol 29 (1) ◽  
pp. 64-69
Author(s):  
Narayan Chandra Kundu ◽  
Moushumi Sen ◽  
KM Nazmul Islam Joy ◽  
Feroj Ahmed Quraish

Juvenile myasthenia gravis (JMG) is a rare autoimmune disorder of childhood. Pediatric presentation of MG is more common in Oriental than in Caucasian populations. JMG need to be differentiated from congenital myasthenia gravis which do not have haan autoimmune basis. An 11 years old girl presented with drooping of eye lids which was more marked at the later part of day and was gradually progressive . She had complained of double vision. She had no family history of myasthenia gravis. Ice pack test, repetitive nerve stimulation test, and anti acetylcholine receptor antibody test support the diagnosis. She was treated with pyridostigmine and was started as 30mg four times daily and increased to 60 mg/qds. Subsequently her symptoms improved gradually and she became stable. Bangladesh Journal of Neuroscience 2013; Vol. 29 (1) : 64-69


2021 ◽  
Vol 39 (3) ◽  
pp. 222-224
Author(s):  
Seungwon Song ◽  
Jinhyuk Cho ◽  
Seong Ho Jeong

A 79-year-old man visited neurology clinic due to gait ataxia and vertigo for 10 months. Neurologic examination revealed saccadic pursuit, mild dysmetria, impaired tandem gait, and areflexia that recovers after exercise. The amplitude of compound muscle action potentials recorded on the abductor digiti minimi increased up to 6,639.4% during repetitive nerve stimulation at 50 Hz stimulation. This case demonstrates that clinicians should consider Lambert-Eaton myasthenic syndrome as a differential diagnosis when a patient complains of gait ataxia and vertigo.


2020 ◽  
Vol 44 (3) ◽  
pp. 241-249
Author(s):  
Yoshiaki Omura

While a visiting Professor at the University of Paris, VI (formerly Sorvonne) more than 40 years ago, the Author became very good friends with Dr. Paul Nogier who periodically gave seminars and workshops in Paris. After the author diagnosed his cervical problem & offered him simple help, Dr. Nogier asked the Author to present lectures and demonstrations on the effects of ear stimulation, namely the effects of acupuncture & electrical stimulation of the ear lobules. It is only now, in 2019 that we have discovered 2–5 minute high frequency stimulation of the ear lobule inhibits cancer activity for 1– 4 hours post stimulation. Although the procedure is extremely simple. First take optimal dose of Vitamin D3, which has the most essential 10 unique beneficial factors required for every human cell activity. Next, apply high frequency stimulation to ear lobule while the worst ear lobule is held by all fingers with vibrator directly touching the surface of the worst ear lobule, preferably after patient repeatedly takes optimal dose of Vitamin D3. When the worst ear lobule is held between thumb & index fingers and applying mechanical stimulation of 250 ~ 500 mechanical vibration/second for 2 ~ 5 minutes using an electrical vibrator, there is rapid disappearance of cancer activity in both the brain and rest of the body for short time duration 1 ~ 4 hours. The effect often increases by additional pressure by holding fingers. As of May 2019, the Author found that many people from various regions of the world developed early stages of multiple cancers. For evaluation of this study, U. S. patented Bi-Digital O-Ring Test (BDORT) was used which was developed by the Author while doing his Graduate experimental physics research at Colombia University. BDORT was found to be most essential for determining the beneficial effects as well as harmful effects of any substance or treatment. Using BDORT, Author was the first to recognize severe increasing mid-backache was an early sign of pancreatic cancer of President of New York State Board of Medicine after top pain specialists failed to detect the cause after 3 years of effort, while the BDORT showed early stages of cancer whereas conventional X-Ray of the pancreas did not show any cancer image until 2 months after Author detected with BDORT. For example, the optimal dose of the banana is usually about 2.0 - 2.5 millimeters cross section of the banana. A whole banana is more than 50 ~ 100 times the optimal dose. Any substance eaten in more than 25 times of its optimal dose becomes highly toxic and creates DNA mutations which can cause multiple malignancies in the presence of strong electro-magnetic field. With standard medication given by doctor, patients often become sick and they are unable to reduce body weight, unless medication is reduced or completely stopped. When the amount of zinc is very high, DNA often becomes unstable and multiple cancers can grow rapidly in the presence of strong electromagnetic field. Large amount of Vitamin C from regular orange or orange juice inhibit the most important Vitamin D3 effects. At least 3 kinds of low Vitamin C oranges will not inhibit Vitamin D3. Since B12 particularly methyl cobalamin which is a red small tablet is known to improve brain circulation very significantly we examined its effect within 20 seconds of oral intake we found the following very significant changes. Acetylcholine in both sides of the brain often increases over 4,500 ng. Longevity gene Sirtuin 1 level increases significantly for short time of few hours. Thymosin α1 and Thymosinβ4 both increase to over 1500 ng from 20 ng or less.


2021 ◽  
Vol 11 (2) ◽  
pp. 216-218
Author(s):  
Marta Brandão Calçada ◽  
Luís Fernandes ◽  
Rita Soares Costa ◽  
Sara Montezinho ◽  
Filipa Martins Duarte ◽  
...  

Sodium-glucose cotransporter 2 inhibitors (SGLT2i) are the most recently approved drug class for the treatment of type 2 diabetes mellitus (T2D). Although they are largely well-tolerated, their intake has been associated with euglycemic diabetic ketoacidosis (DKA) in some rare cases. We report the case of a 70-year-old male with type 2 diabetes and no history of DKA, who started therapy with empagliflozin one day before presenting with acute pancreatitis and laboratory findings consistent with euglycemic DKA. SGLT2i can induce euglycemic DKA from the first dose. Given the atypical presentation, a high degree of clinical suspicion is required to recognize this complication.


2021 ◽  
pp. 1-3
Author(s):  
Setareh Alabaf ◽  
Karen O'Connell ◽  
Sithara Ramdas ◽  
David Beeson ◽  
Jacqueline Palace

Congenital Myasthenic Syndrome (CMS) are a rare group of genetic disorders of neuromuscular transmission. Some subtypes of CMS can be associated with respiratory and bulbar weakness and these patients may therefore be at high risk of developing a severe disease from COVID-19. We screened 73 patients with genetically confirmed CMS who were attending the UK national referral centre for evidence of previous Severe Acute Respiratory Syndrome Corona Virus 2 infection and their clinical outcome. Of 73 patients, seven had history of confirmed COVID-19. None of the infected patients developed a severe disease, and there were no signals that CMS alone carries a high risk of severe disease from COVID-19.


Sign in / Sign up

Export Citation Format

Share Document