scholarly journals Case of myasthenia gravis and Lambert-Eaton myasthenic syndrome overlap syndrome accompanied by autoimmune encephalitis and cerebellar ataxia with multiple neuronal antibodies

2017 ◽  
Vol 5 (5) ◽  
pp. 152-154
Author(s):  
Yumiko Nakano ◽  
Emi Nomura ◽  
Toru Yamashita ◽  
Kota Sato ◽  
Mami Takemoto ◽  
...  
2018 ◽  
Vol 89 (6) ◽  
pp. A39.1-A39
Author(s):  
Sameer Saleem ◽  
Ronak Patel ◽  
Yash Gawarikar

IntroductionMyasthenia gravis (MG) is an antibody-mediated autoimmune disease of neuromuscular transmission. 6 to 12 percent of MG are negative for acetyl choline receptor (AChR) and MuSK antibodies and are defined as seronegative MG. Lambert-Eaton myasthenic syndrome (LEMS) is an autoimmune condition with antibodies to presynaptic voltage-dependent calcium channelCaseA 39 year old male presented with blurred vision and right-sided ptosis. Initial examination showed a pupil-sparing left complete third nerve palsy. Demyelination and intracranial aneurysms were ruled out with gadolinium-enhanced MRI/MRA. Outpatient follow-up 2 weeks later showed new onset proximal muscle weakness of the upper limbs with fatigability and a complex ophthalmoplegia with almost complete paralysis of gaze. A repeat MRI with gadolinium and CSF analysis were normal. His AChR and MuSK antibodies were negative; however, voltage-dependent calcium channel antibodies, ANA, dsDNA, and SSA were positive. Initial nerve conduction tests were normal, but repeat NCS on two separate occasions showed decrement on repetitive stimulation in the right trapezius with no evidence of facilitation post exercise. CT chest, abdomen and pelvis was normal. He improved with pulsed steroids and was discharged on a tapering dose of oral steroids, pyridostigmine and regular IVIG infusions as a steroid-sparing agent.ConclusionLambert-Eaton myasthenic syndrome shares the same pathologic site and similar pathophysiology with MG but has a markedly different clinical and electro-physiological picture. There are reports of MG and LEMS overlap syndrome, however, they exhibit phenotypic characteristics of both LEMS and MG. Voltage-dependent calcium channel antibodies have not been described in patients with seronegative MG. Ours is potentially the first reported case of seronegative myasthenia with voltage-dependent calcium channel antibodies and only clinical and neurophysiological features of MG.


Author(s):  
Alexandra Duplaine ◽  
Camille Prot ◽  
Gwendal Le-Masson ◽  
Antoine Soulages ◽  
Fanny Duval ◽  
...  

2021 ◽  
Vol 11 (8) ◽  
pp. 1035
Author(s):  
Maria Pia Giannoccaro ◽  
Patrizia Avoni ◽  
Rocco Liguori

The neuromuscular junction (NMJ) is the target of a variety of immune-mediated disorders, usually classified as presynaptic and postsynaptic, according to the site of the antigenic target and consequently of the neuromuscular transmission alteration. Although less common than the classical autoimmune postsynaptic myasthenia gravis, presynaptic disorders are important to recognize due to the frequent association with cancer. Lambert Eaton myasthenic syndrome is due to a presynaptic failure to release acetylcholine, caused by antibodies to the presynaptic voltage-gated calcium channels. Acquired neuromyotonia is a condition characterized by nerve hyperexcitability often due to the presence of antibodies against proteins associated with voltage-gated potassium channels. This review will focus on the recent developments in the autoimmune presynaptic disorders of the NMJ.


2021 ◽  
Vol 46 ◽  
pp. 51-55 ◽  
Author(s):  
Demis N. Lipe ◽  
Elkin Galvis-Carvajal ◽  
Eva Rajha ◽  
Adriana H. Wechsler ◽  
Susan Gaeta

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