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J R Soc Med 2006;99:259-260
doi:10.1258/jrsm.99.5.259
© 2006 Royal Society of Medicine

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J R Soc Med 2006;99:259-260
© 2006 The Royal Society of Medicine

Grand round

Simultaneous presentation of myasthenia gravis and mesothelioma

D J Thomson A Soni M Ward   H W Jones

Department of General Medicine, John Radcliffe Hospital, Oxford, UK

Correspondence to: Dr HW Jones E-mail: hywel.jones{at}orh.nhs.uk

Myasthenia gravis may be a paraneoplastic phenomenon, caused by underlying cancer.1 This case describes the first co-presentation of myasthenia gravis and pleural mesothelioma. If this is a paraneoplastic occurrence, then with the expected increase in incidence of mesothelioma,2 further cases should arise.

CASE HISTORY

A man aged 71 years, and previously in good health, presented to the acute medical take with difficulty chewing and swallowing. He gave a 6-week history of variable left upper eyelid drooping, slurred and nasal speech and difficulty with chewing and swallowing. Symptoms were most prominent in the evenings and it took him over an hour to eat his evening meal. There was no double vision or shortness of breath. Upper and lower limbs were unaffected. He had lost 3.5 kg in weight over the preceding month. His past medical history was unremarkable, and his only medication was a statin for hypercholesterolaemia. He was a life-long non-smoker and a retired brick-layer and car factory worker.


Figure 1
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Figure 1. Computerized tomograph of thorax, pleural mass indicated

 


Figure 2
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Figure 2. Thorax computerized tomograph showing pleural mass

 
On examination, he was well. He was not cachectic or clubbed. Respiratory and cardiovascular examinations were normal. He had a pronounced nasal dysathria that fatigued. There was a subtle left ptosis that was not clearly fatiguable on upward gaze. Eye movements were normal and there was no diplopia. There was bilateral facial weakness with incomplete eyelid closure and a very poor lip seal whereby he could not whistle or blow his cheeks out; the forehead was spared. Palatal elevation was mildly affected. The tongue was normal. The limbs were of normal tone and power, both proximally and distally: there was no fatiguablility. All reflexes were present and showed no post-tetanic augmentation. His vital capacity was 2.5 L. A chest X-ray demonstrated multiple right-sided pleural masses and a small pleural effusion.

Clinically, the diagnosis was myasthenia gravis with a predominant bulbar presentation. This was confirmed by the presence of anti-acetylcholine receptor antibodies, and supportive neurophysiological findings. There were no anti-voltage gated calcium channel antibodies or anti-MuSK antibodies detected. He responded well to anti-cholinesterase and steroid treatment.

Computerized tomography thorax confirmed the presence of pleural masses (Figures 1 and 2). There were no thymic tissues seen within the anterior mediastinum. One of the masses was biopsied and a histological diagnosis of sarcomatoid mesothelioma was made. There was no evidence of malignancy elsewhere.

COMMENT

Both mesothelioma and myasthenia gravis are rare diseases. In the UK, the prevalence of mesothelioma is 8/100 000 in men and 1/100 000 in women2 and the prevalence of myasthenia gravis 15/100 000.3 Their dual diagnosis is unlikely, and this is the first reported case.

Paraneoplastic syndromes result from non-local effects of cancer and in 80% of cases they are diagnosed before the cancer.1 Myasthenia gravis is the most common paraneoplastic neurological disease and 10% of cases are paraneoplastic.4 The classical association is that of thymoma and myasthenia gravis, where 30% of thymomas cause myasthenia gravis.4

There is one reported case of paraneoplastic cerebellar degeneration in a patient with known mesothelioma.1 However, this is the first case report of the co-presentation of myasthenia gravis and pleural mesothelioma. If the association is more than chance, then with the expected increase in incidence of mesothelioma2 further cases should be described.

REFERENCES

  1. Candler PM, Hart PE, Barnett M, Weil R, Rees JH. A follow up study of patients with paraneoplastic neurological disease in the United Kingdom. J Neurol Neurosurg Psychiatry2004; 75:1411 -15[Abstract/Free Full Text]

  2. Treasure T, Sedrakyan A. Pleural mesothelioma: little evidence, still time to do trials. Lancet2004; 364:1183 -5[CrossRef][Medline]

  3. Robertson NP, Deans J, Compston DAS. Myasthenia gravis: a population based epidemiological study in Cambridgeshire, England. J Neurol Neuorosurg Psychiat1998; 65:492 -6

  4. Posner JB. Immunology of paraneoplastic syndromes. Ann. N. Y. Sci. 2003;998:178 -86


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