Association of neuromyotonia with peripheral neuropathy, myasthenia gravis and thymoma: A case report

1994 ◽  
Vol 15 (6) ◽  
pp. 307-310 ◽  
Author(s):  
M. Perini ◽  
A. Ghezzi ◽  
P. F. Basso ◽  
R. Montanini
Keyword(s):  
Case Report ◽  
Peripheral Neuropathy ◽  
Myasthenia Gravis

Peripheral Neuropathy in a Patient with Churg-Strauss Syndrome - A case report -

2007 ◽  
Vol 20 (2) ◽  
pp. 208
Author(s):  
Jun Hwa Lee ◽  
Jeong Hyun Lee ◽  
Young Kwon Go ◽  
Won Hyung Lee
Keyword(s):  
Case Report ◽  
Peripheral Neuropathy ◽  
Churg Strauss Syndrome ◽  
Strauss Syndrome ◽  
Churg Strauss

CIGUATERA AND PERIPHERAL NEUROPATHY: A CASE REPORT

2000 ◽  
Vol 5 (4) ◽  
pp. 250-251 ◽  
Author(s):  
F Derouiche ◽  
E Cohen ◽  
G Rodier ◽  
C Boulay ◽  
S. Courtois
Keyword(s):  
Case Report ◽  
Peripheral Neuropathy

scholarly journals AB0854 A CASE REPORT OF BROWN SYNDROME COMPLICATING THE MANAGEMENT OF SCLEROMYOSITIS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1451.1-1451
Author(s):  
P. Arora ◽  
L. Croot
Keyword(s):  
Case Report ◽  
Myasthenia Gravis ◽  
Muscle Weakness ◽  
Tendon Sheath ◽  
Motility Disorder ◽  
Ocular Motility ◽  
Muscle Involvement ◽  
Eye Muscle ◽  
Brown Syndrome ◽  
Superior Oblique

Background:Brown syndrome is a rare ocular motility disorder which has been reported in JRA, RA and SLE but never in a patient with scleromyositis.Objectives:To report the first case of Brown syndrome in a patient with scleromyositis and increase awareness of this condition.Methods:A case report and discussion.Results:The patient was diagnosed with scleromyositis, at the age of 34, after presenting with arthralgia, sclerodactyly, skin pigmentation, Raynaud’s phenomenon, mild muscle weakness and dyspnoea. His labs were CRP 47 mg/L, CK 868 IU/L, ANA strongly positive; anticentromere Ab negative and Anti-PM/Scl-75 and Anti- PM/Scl-100 Ab positive. HRCT chest showed extensive pulmonary fibrosis with lower lobe honeycombing. TLCO was 3.98 (33% of predicted).He was initially managed with high dose steroids and pulsed IV cyclophosphamide with azathioprine for maintenance therapy. His lung disease stabilised and myositis resolved but he continued to develop calcinosis cutis so was switched to 6 monthly IV rituximab.6 years later, he developed morning headaches with intermittent diplopia, described as double vision in vertical gaze with one image being above the other. Episodes lasting 10 minutes to 2 hours. Examination showed normal visual acuity and fundoscopy, no peripheral or eye muscle weakness.Investigations to exclude myasthenia gravis, cerebral vasculitis and atypical infection were organised (MRI, AChR antibody, lumbar puncture, MRA) and were normal.Because of intermittent nature of his episodes, his eye examination was always normal but he captured images in disconjugate gaze with right eye looking upwards and outwards when trying to look straight (Figure 1). Occasionally this was associated with orbital pain and an audible click. These features are suggestive of Brown syndrome.He continues to have recurrent episodes despite immunosuppression but prednisolone 20mg daily for 1-2 days at onset of each attack causes rapid resolution of symptoms.Figure 1.Right eye looking upwards and outwards when trying to look straightConclusion:Scleromyositis is an overlap syndrome of scleroderma and dermatomyositis. Muscle involvement is mild and clinical presentation can be variable. The PM/Scl antibodies are highly characteristic of the syndrome. (1)Brown syndrome is an ocular motility disorder, first described in 1950, characterized by the inability to fully elevate the affected eye in adduction due to pathology of the superior oblique tendon sheath. (2)It can be congenital or acquired, viz, trauma, surgery or sinusitis and also been described in RA, JIA and SLE. (3)If superior oblique tendon cannot relax or slide freely through the trochlea then the affected eye cannot depress completely, leading to diplopia on upward gaze. (4) In inflammatory disease it is thought that swelling of the posterior part of the superior oblique tendon or tenosynovitis are likely causes of the tendon sheath abnormality. (4) This is likely to be the case in this patient because his symptoms are recurrent, respond to steroids and tend to occur more towards the end of rituximab cycles.Recognition of this syndrome is important because invasive investigations can be avoided. Also, intermittent diplopia in a patient with autoimmune disease is suggestive of myasthenia gravis which maybe incorrectly diagnosed.Finally, this case demonstrates the syndrome can be easily managed with short courses of oral steroids, although patients who are already on immunosuppressant treatment may need this in addition.References:[1]Török L, Dankó K, Cserni G, Szûcs G. PM-SCL autoantibody positive scleroderma with polymyositis (mechanic’s hand: clinical aid in the diagnosis). JEADV 2004; 18: 356–359[2]Brown H W. Congenital structural muscle anomalies. In:Alien J H, ed. Strabismus ophthalmic symposium I. St Louis:CV Mosby, 1950: 205-6.[3]Cooper C, Kirwan JR, McGill NW, Dieppe PA. Brown’s syndrome: an unusual ocular complication of rheumatoid arthritis. Ann Rheum Dis 1990; 49:188-9.[4]Sandford-Smith JH. Superior oblique tendon syndrome and its relationship to stenosing tenosynovitis. Br JOphthalmol 1973; 57:859-65.Disclosure of Interests:None declared

P-PN018. The co-occurrence of myasthenia gravis and Guillain-Barré Syndrome: A case report

2021 ◽  
Vol 132 (8) ◽  
pp. e111
Author(s):  
R. Freshimona ◽  
A. Wijaya ◽  
Aryatama ◽  
S. Djojoatmodjo ◽  
J. Liman ◽  
...  
Keyword(s):  
Case Report ◽  
Myasthenia Gravis ◽  
Guillain Barre Syndrome ◽  
Guillain Barré Syndrome ◽  
Guillain Barré

Emergence of myasthenia gravis in dengue infection—first case report

2021 ◽  
Vol 27 (1) ◽  
pp. 183-185
Author(s):  
Yue Hui Lau ◽  
Suganthi Chinnasami
Keyword(s):  
Case Report ◽  
Myasthenia Gravis ◽  
Dengue Infection ◽  
First Case

Coeliac Disease Associated with Peripheral Neuropathy in a Child: A Case Report

1998 ◽  
Vol 29 (3) ◽  
pp. 155-158 ◽  
Author(s):  
A. Simonati ◽  
P. Battistella ◽  
C. Guariso ◽  
M. Clementi ◽  
N. Rizzuto
Keyword(s):  
Case Report ◽  
Peripheral Neuropathy ◽  
Coeliac Disease

scholarly journals Rare association of thymoma, myasthenia gravis and sarcoidosis : a case report

2008 ◽  
Vol 2 (1) ◽  
Author(s):  
Mohankumar Kurukumbi ◽  
Roger L Weir ◽  
Janaki Kalyanam ◽  
Mansoor Nasim ◽  
Annapurni Jayam-Trouth
Keyword(s):  
Case Report ◽  
Myasthenia Gravis ◽  
Rare Association

Post-thymectomy myasthenia gravis: a case report and systematic review of literature

2021 ◽  
Vol 14 (12) ◽  
pp. e246005
Author(s):  
Louise Gurowich ◽  
Gabriel Yiin ◽  
Adam Maxwell ◽  
Alexandra Rice
Keyword(s):  
Systematic Review ◽  
Case Report ◽  
Myasthenia Gravis ◽  
Early Onset ◽  
Late Onset ◽  
High Morbidity ◽  
Review Of Literature ◽  
Prompt Treatment ◽  
Autoimmune Condition ◽  
Anti Acetylcholine

Myasthenia gravis (MG) is an autoimmune condition affecting the neuromuscular junction characterised by weakness and fatiguability, carrying a high morbidity if treatment is delayed. A clear association with thymoma has led to management with thymectomy as a common practice, but MG presenting post-thymectomy has rarely been reported. We present a case of an 82- year-old woman developing fatigue, ptosis and dysarthria 3 months after thymectomy. After a clinical diagnosis of MG was made, she responded well to prompt treatment with prednisolone and pyridostigmine. Her anti-acetylcholine receptor antibody (anti-AChR) subsequently came back positive. Our systematic review reveals that post-thymectomy MG can be categorised as early-onset or late-onset form with differing aetiology, and demonstrated correlation between preoperative anti-AChR titres and post-thymectomy MG. The postulated mechanisms for post-thymectomy MG centre around long-lasting peripheral autoantibodies. Clinicians should actively look for MG symptoms in thymoma patients and measure anti-AChR preoperatively to aid prognostication.

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