Roshan Xavier Rebello
Advanced Trainee in Internal Medicine, Waikato, NZ





BIOGRAPHY

I am an advanced trainee in Internal medicine from Waikato. I have also completed respiratory training in England prior to moving to NZ.


ABSTRACT 

Hickum’s Dictum: Two Atypical Presentations of Myasthenia Gravis in Graves’ Disease

Rebello R 1, Sehgal S 1, Bukhari W 2, and Wolmarans L 1 
1Department of Endocrinology Waikato Hospital,Hamilton New Zealand
2Department of Neurology Waikato Hospital, Hamilton, New Zealand

Aims: We present two patients with Graves’ disease, bilateral ptosis, fatigability and skeletal muscle weakness. Our case evaluation reveals an association between the two common autoimmune diseases, and demonstrates the potential adverse effects of delayed diagnosis.

Methods/Case Description: Patient 1 is a 28 year old Maori man in ICU with undiagnosed Graves’ disease presenting following his second respiratory arrest, preceded by several months of dysphagia & fatigability. Exophthalmos, ptosis and muscle weakness were present on examination. Thyroid function tests revealed the following FT4 52.0( 12-20) pmol/l, (FT3 ) 14.6(3.1-6.8) pmol/l, TSH<0.02 mU/l.

Patient 2 is a 66 year old Cantonese woman with established Graves’ disease on medical therapy since September 2015. Examination revealed bilateral ptosis and right lateral rectus palsy. Thyroid function tests revealed a FT4 67(12-20) pmol/l, (FT3 ) 20.6(3.1-6.8) pmol/l, TSH<0.02.

Results: Patient 1 had Nicotinic Acetyl Choline Receptor antibodies of >20.0 and Patient 2’s titre was   5.35 nmol/l (<0.44nmol/l). EMG findings confirmed Myasthenia Gravis in patient 1 and was not performed in patient 2, an ice pack test for ptosis was negative. Both patients were commenced on Carbimazole therapy. Patient 2 developed neutropenia on Carbimazole requiring a total thyroidectomy for Graves’ disease management before confirming the diagnosis of Myasthenia Gravis. Both patients were commenced on steroids and anticholinesterase therapy when the diagnosis of Myasthenia gravis was confirmed.

Conclusions: Autoimmune thyroid disease occurs in 8.2% of patients with Myasthenia Gravis (1). The prevalence of Myasthenia Gravis in Graves’ disease is 0.14% (2).

Both disorders share common autoimmune mechanisms, and both are associated with an increased risk of Graves opthalmopathy (3). Ocular signs in both diseases closely resemble each other with the presence of ptosis suggesting co-existing Myasthenia Gravis (4,5). Both disorders complicate the presentation, evaluation, successful medical and surgical management of each other (6).

References:

1. Chen YL, Yeh JH, Chiu HC. Clinical features of myasthenia gravis patients with autoimmune thyroid disease in Taiwan. Acta Neurologica Scandinavica. 2013 Mar 1;127(3):170-4.
2. Ohno M, Hamada N, Yamakawa J, Noh J, MORII H, ITO K. Myasthenia gravis associaied with Graves' disease in Japan. Japanese journal of medicine. 1987;26(1):2-6.
3. Marinò M, Barbesino G, Pinchera A, Manetti L, Ricciardi R, Rossi B, Muratorio A, Braverman LE, Mariotti S, Chiovato L. Increased frequency of euthyroid ophthalmopathy in patients with Graves' disease associated with myasthenia gravis. Thyroid. 2000 Sep;10(9):799-802.
4. Mangaraj S, Choudhury AK, Mohanty BK, Baliarsinha AK. Neurological manifestations of Graves’ disease: A case report and review of the literature. Journal of neurosciences in rural practice. 2016 Jan;7(1):153.
5. Biswas K, Sharma M, Dutta D, Barman N. Ocular myasthenia with thyroid associated ophthalmopathy in subclinical Graves' disease: Diagnostic challenge and treatment outcomes. Thyroid Research and Practice. 2014 May 1;11(2):73.
6. Lakhal K, Blel Y, Fysekidis M, Mohammedi K, Bouadma L. Concurrent Graves disease thyrotoxicosis and myasthenia gravis: the treatment of the former may dangerously reveal the latter. Anaesthesia. 2008 Aug 1;63(8):876-9