Myasthenia gravis associated with a pelvic follicular lymphoma

2022 ◽  
Vol 15 (1) ◽  
pp. e248011
Author(s):  
Charlotte Willis ◽  
Max Williamson ◽  
Nicholas Constantinou ◽  
Emily Johns
Keyword(s):  
Myasthenia Gravis ◽  
Follicular Lymphoma ◽  
Calcium Channel ◽  
Acetylcholine Receptor ◽  
Antibody Level ◽  
Pelvic Mass ◽  
Successful Outcome ◽  
Voltage Gated ◽  
Achr Antibody ◽  
Myasthenic Syndrome

An 81-year-old woman presented with neck weakness, dysarthria, dysphasia and left-sided ptosis. Myasthenia gravis (MG) was strongly suspected. Voltage gated calcium channel (VGCC) antibodies, associated with Lambert-Eaton myasthenic syndrome (LEMS), were negative. Acetylcholine receptor (AChR) antibody level was 536 nmol/L and diagnosis of MG was confirmed. Imaging revealed a pelvic mass and subsequent biopsy confirmed a pelvic follicular lymphoma. Our searches revealed this to be the first documented case of MG associated with a pelvic follicular lymphoma. She underwent radiotherapy to treat the lymphoma and received both pyridostigmine and immunosuppression to treat the MG. Her AChR antibody level decreased to 38 nmol/L and her MG symptoms resolved aside from head drop which is continuing to improve. Her lymphoma is now in remission. We have presented a case with a successful outcome, which highlights the importance of screening for lymphoma and thymoma in new presentations of MG.

scholarly journals Clinical features of neuromuscular disorders in patients with N-type voltage-gated calcium channel antibodies

2016 ◽  
Vol 26 (4) ◽  
Author(s):  
Andreas Totzeck ◽  
Petra Mummel ◽  
Oliver Kastrup ◽  
Tim Hagenacker
Keyword(s):  
Myasthenia Gravis ◽  
Calcium Channel ◽  
Muscle Weakness ◽  
Neuromuscular Disorders ◽  
Intravenous Immunoglobulins ◽  
Voltage Gated ◽  
Dropped Head Syndrome ◽  
Seronegative Myasthenia Gravis ◽  
Myasthenic Syndrome ◽  
Voltage Gated Calcium Channel

Neuromuscular junction disorders affect the pre- or postsynaptic nerve to muscle transmission due to autoimmune antibodies. Members of the group like myasthenia gravis and Lambert-Eaton syndrome have pathophysiologically distinct characteristics. However, in practice, distinction may be difficult. We present a series of three patients with a myasthenic syndrome, dropped-head syndrome, bulbar and respiratory muscle weakness and positive testing for anti-N-type voltage-gated calcium channel antibodies. In two cases anti-acetylcholin receptor antibodies were elevated, anti-P/Q-type voltage-gated calcium channel antibodies were negative. All patients initially responded to pyridostigmine with a non-response in the course of the disease. While one patient recovered well after treatment with intravenous immunoglobulins, 3,4-diaminopyridine, steroids and later on immunosuppression with mycophenolate mofetil, a second died after restriction of treatment due to unfavorable cancer diagnosis, the third patient declined treatment. Although new antibodies causing neuromuscular disorders were discovered, clinical distinction has not yet been made. Our patients showed features of pre- and postsynaptic myasthenic syndrome as well as severe dropped-head syndrome and bulbar and axial muscle weakness, but only anti-N-type voltage-gated calcium channel antibodies were positive. When administered, one patient benefited from 3,4-diaminopyridine. We suggest that this overlap-syndrome should be considered especially in patients with assumed seronegative myasthenia gravis and lack of improvement under standard therapy.

Isolated dysphagia due to paraneoplastic myasthenic syndrome with anti-P/Q-type voltage-gated calcium-channel and anti-acetylcholine receptor antibodies

2011 ◽  
Vol 21 (2) ◽  
pp. 126-128 ◽  
Author(s):  
Roberto Fernandez-Torron ◽  
Juan Arcocha ◽  
Jose M. López-Picazo ◽  
Javier Pardo ◽  
Maria Akiko Tamura ◽  
...  
Keyword(s):  
Calcium Channel ◽  
Acetylcholine Receptor ◽  
Voltage Gated ◽  
Myasthenic Syndrome ◽  
Voltage Gated Calcium Channel ◽  
Receptor Antibodies ◽  
Anti Acetylcholine

scholarly journals Presynaptic Paraneoplastic Disorders of the Neuromuscular Junction: An Update

2021 ◽  
Vol 11 (8) ◽  
pp. 1035
Author(s):  
Maria Pia Giannoccaro ◽  
Patrizia Avoni ◽  
Rocco Liguori
Keyword(s):  
Potassium Channels ◽  
Neuromuscular Junction ◽  
Calcium Channels ◽  
Myasthenia Gravis ◽  
Neuromuscular Transmission ◽  
Voltage Gated ◽  
Voltage Gated Calcium Channels ◽  
Immune Mediated ◽  
Recent Developments ◽  
Myasthenic Syndrome

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.

Does change in acetylcholine receptor antibody level correlate with clinical change in myasthenia gravis?

2014 ◽  
Vol 49 (4) ◽  
pp. 483-486 ◽  
Author(s):  
Donald B. Sanders ◽  
Ted M. Burns ◽  
Gary R. Cutter ◽  
Janice M. Massey ◽  
Vern C. Juel ◽  
...  
Keyword(s):  
Myasthenia Gravis ◽  
Acetylcholine Receptor ◽  
Antibody Level ◽  
Clinical Change ◽  
Receptor Antibody ◽  
Acetylcholine Receptor Antibody

098 A case of myasthenia gravis lambert eaton overlap syndrome (MLOS) in seronegative myasthenia gravis

2018 ◽  
Vol 89 (6) ◽  
pp. A39.1-A39
Author(s):  
Sameer Saleem ◽  
Ronak Patel ◽  
Yash Gawarikar
Keyword(s):  
Myasthenia Gravis ◽  
Calcium Channel ◽  
Overlap Syndrome ◽  
Initial Examination ◽  
Blurred Vision ◽  
Voltage Dependent Calcium Channel ◽  
Seronegative Myasthenia Gravis ◽  
Voltage Dependent ◽  
Myasthenic Syndrome ◽  
Seronegative Mg

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.

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