Migraine-like headache caused by rheumatic heart disease: The second case of ‘Mahler’s migraine’?

We read the interesting report by Haan and Ferrari (1) regarding the headache from which the famous composer Gustav Mahler suffered. In their paper the authors argued that it could not be ruled out that this migrainous headache was associated with the endocarditis that would later be the cause of death of the artist, hypothesizing a so-called ‘Mahler’s migraine’ (1). To the best of our knowledge, other associations between endocarditis and the development of migraine-like headache have not been reported.

We describe the case of a 44-year-old Russian woman who came to our attention for acute visual disturbances associated with headache. She had a long history of headache episodes characterized by bilateral, non-throbbing, occipital pain, occurring more than 10 times per month, lasting many hours, preceded by visual symptoms (scintillating scotoma) and accompanied by nausea, vomiting, photophobia and phonophobia. In childhood, the patient suffered from frequent sore throats; in the last 10 years she had persistent knee pain that limited her in working and, in the last two years, she also complained of wheezing while climbing the stairs. At the time of our observation, an ophthalmological evaluation revealed left upper quadrantanopsia. She had no fever, the antistreptolysin titre was >200 IU/ml and the erythrocyte sedimentation rate was 43 mm/h. She denied smoking or taking oral contraceptives. Brain magnetic resonance imaging showed an acute right ischemic occipital lesion (Figure 1).

Figure 1.

Magnetic resonance imaging of the brain in our patient. (a), (b) Diffusion weighted imaging (DWI) shows an area of restricted diffusion. (c), (d) Apparent diffusion coefficient (ADC) maps show a hypointense signal. These findings are consistent with an area of acute ischemia in the right occipital lobe. (e), (f) T1-weighted enhanced imaging showing contrast enhancement within the ischemic area. (g), (h) Fluid attenuation inversion recovery (FLAIR) sequences show a hyperintense signal in the same right occipital areas.

A subsequent trans-esophageal echocardiogram showed severe mitral valve stenosis, medium aortic regurgitation and severe tricuspid regurgitation, with a pulmonary pressure of 45 mmHg. The valve disease was consistent with rheumatic heart disease (2). On day 6 after our first examination, the patient underwent cardiac-surgical intervention with the implantation of mechanical aortic and mitral valve prostheses, and tricuspid plastic. In the left atrium, a massive blood clot that filled the entire auricle was found and removed. The patient started anticoagulant therapy. At the follow-up examination, performed six months after surgery, her visual field impairment was unmodified and she reported having had only two migraine episodes.

At a further follow-up, performed 18 months after surgery, the brain MRI was unchanged, and the patient did not report any other headache attack.

Our patient presented a long-lasting history of migraine with aura-like headache attacks which completely remitted after the insertion of valvular prosthetics and the removal of a blood clot that filled the auricle. Moreover, the patient presented with a posterior circulation stroke. This observation allows us to hypothesize that the pathogenic mechanism of her migraine attacks was cardiogenic microembolism. Previous studies suggested that microembolism plays a role in the pathophysiology of migraine, especially migraine with aura (3).

The case previously described as ‘Mahler’s migraine’ shares the possible pathogenic mechanism underlying migraine. Migraine can be associated with stroke. One of the mechanisms hypothesized is microembolization, which is more likely associated with migraine with aura, and with involvement of the posterior circulation (3). In conclusion, these observations suggest that a systematic search for embolic sources should be performed in patients with migraine associated with clinically manifest, or even silent neuroimaging evidence of, brain ischemia.