eosinophilia is a rare finding in neuromuscular disorders ( nmds ) but has occasionally been reported in some of them [ 1 , 2 , 3 , 4 ] . excellent overviews of nmds with eosinophilia have been provided by schrder et al .   and pellissier et al . . in mitochondrial disorders ( mids ) , eosinophilia has not been reported so far but , in our own experience , can be a rare hematological manifestation of nonsyndromic mids . here , we report a patient with clinical features of a mid and permanent eosinophilia and hyper - creatine - kinase ( ck)-emia for at least 10 years . the patient is a 57-year - old caucasian male ( may 2015 ) with a height of 180 cm and a weight of 82 kg , in whom eosinophilia was first detected in august 2003 and has remained almost constantly present until today ( fig . 1 ) . eosinophilia from chronic infection , worms , fungi , drugs , toxins , or allergy was excluded . his individual history was positive for several items . in 1990 , he had to undergo surgery for spontaneous left - sided achilles tendon rupture . since at least october 1999 , hyperlipidemia has been noteworthy ( fig . 2 ) , which was treated successively with fluvastatin , bezafibrate , atorvastatin , and since march 2015 with ezetimibe ( 10 mg / day ) . at the same time ( october 1999 ) , hyperuricemia was noted . in july 2001 , mild renal insufficiency became apparent which has persisted until today . in june 2007 , l - thyroxine was prescribed to prevent the growth of diffuse and nodular goiter but was discontinued by the patient . later , he developed autoimmune hypothyroidism requiring l - thyroxine since march 2013 . since march 2006 , elevation of the -glutamyl - transpeptidase has been noted . since 2009 , he has suffered from recurrent exanthema of the buttocks . in june 2013 , he experienced pneumonia . neurologic symptoms were noticed in 2002 when he recognized double vision for 34 min upon strenuous fixation of objects , an abnormality that has been inducible until today . in july 2005 , he experienced a left - sided transient peripheral facial palsy . since then , he has complained about mild memory impairment . at that time , also hyper - ck - emia was first recognized and has remained present until today with a tendency to increase . his wife noted a seizure - like event with a vacant stare lasting for a few seconds . in january 2006 , he experienced a first tonic - clonic seizure , and carbamazepine was given . because carbamazepine caused generalized fatigue , it was replaced by lamotrigine in april 2006 . despite changing the antiepileptic regimen , he experienced a second tonic - clonic seizure in september 2006 , and lamotrigine was increased . since then , he has only experienced recurrent focal seizures , manifesting as ascending sensory disturbance , deja vus , or smacking , with a frequency of 6 per year , and a third tonic - clonic seizure in july 2009 . since april 2006 , he has recognized muscle aching after mild exercise . neurologic exam at that time only revealed sore neck muscles and mild postural tremor . neurologic exam in march 2009 electroencephalography in december 2010 was normal . in september 2013 , after cardiac surgery , he experienced transient weakness for right foot extension and transient numbness of the left digit 5 . neurologic exam in november 2013 revealed mild hypoacusis , bilateral ptosis , lid edema , mild weakness for right foot extension , reduced achilles tendon reflexes bilaterally , an exaggerated left patella tendon reflex , and postural tremor . neurologic exam in march 2015 revealed mild hypoacusis , exaggerated tendon reflexes on the right arm , bradydiadochokinesis bilaterally with right - sided predominance , wasting of the thighs , and reduced achilles tendon reflexes bilaterally . echocardiography in october 2007 revealed mitral insufficiency grade 3 . since may 2013 , he has developed resting and exertional dyspnea . heart failure and atrial fibrillation were diagnosed , and heart failure therapy and phenprocoumon were initiated . echocardiography revealed pulmonary hypertension ( 60 mm hg ) and combined mitral valve stenosis and mitral valve insufficiency grade 4 ; therefore , he underwent mitral valve replacement therapy ( st . four months after cardiac surgery , he required implantation of a pacemaker because of atrioventricular block iii ( january 2010 ) . stress testing during rehabilitation after cardiac surgery had to be interrupted 2 times because of early muscle fatigue . the family history was positive for pulmonary embolism ( father ) , hyperlipidemia ( mother , sister ) , hyperuricemia ( mother , sister ) , hypothyroidism ( mother , sister , sister of mother ) , cataract ( sister ) , stroke ( mother ) , thyroid cancer ( mother ) , osteoporosis ( mother ) , arthrosis ( mother , sister ) , liver cirrhosis ( sister of mother ) , colon cancer ( grandmother from the mother 's side ) , ovarian cancer ( grandmother from the mother 's side ) , and breast cancer ( grandmother from the mother 's side , mother , sister of mother ) . his medication in april 2015 included phenprocoumon , l - thyroxine ( 87.5 g / day ) , nebivolol ( 10 mg / day ) , lamotrigine ( 700 mg / day ) , and ezetimibe ( 10 mg / day ) . the presented patient is interesting because of a multisystem disease affecting the central nervous system ( epilepsy , mild cognitive impairment ) , the peripheral nerves ( sensory neuropathy ) , the skeletal muscles ( hyper - ck - emia , double vision , spontaneous tendon rupture ) , the endocrine system ( hypothyroidism , hyperlipidemia ) , the ears ( hypoacusis ) , the liver , the kidneys ( renal insufficiency ) , and the bone marrow ( eosinophilia ) . if all manifestations are assumed to result from a single cause , the only disorder in which such a plethora of different manifestations occurs is a mid . arguments for a mid in the presented patient are the clinical findings ( episodes of double vision , epilepsy , myopathy , sensory neuropathy , spontaneous tendon rupture , renal insufficiency , hypothyroidism , hypoacusis , hepatopathy , hyperlipidemia ) and the family history . most likely , he inherited the mitochondrial multiorgan disorder syndrome from his mother who had developed hyperlipidemia , hyperuricemia , hypothyroidism , ischemic stroke , thyroid cancer , and breast cancer . malignancy has been previously reported to be increased in mids . very likely , also his sister was affected , so that it is highly probable that a mutation in an mtdna - located gene was responsible for the phenotype . muscle biopsy has not been carried out yet because it is often negative in mildly manifesting patients and because even biochemical investigations may be negative . whether cardiac manifestations can be attributed to the underlying mid or not remains speculative . most likely , he experienced a combined mitral valve cardiac defect following initially unrecognized endocarditis . histological and bacteriological workup of the explanted valve , however , did not reveal a histological abnormality indicative of infection , and the culture was negative . a further argument against mid as the underlying cause of the valve pathology is the negative family history for cardiac disease . a rheumatologic cause of the cardiac defect was excluded since the history was negative for rheumatologic disease . he never complained about arthralgia , and there has never been inflammation with swelling , hyperthermia , or immobility of joints . a further argument against a causal relationship between suspected mid and the valve abnormality is that such a relation has not been reported before . eosinophilia has not been reported as a manifestation of a mid , but clinical experience suggests that eosinophilia can very well be a mid manifestation . eosinophilia in mids with myopathy could represent a nonspecific inflammatory reaction in response to myofiber necrosis . generally , eosinophilia in nmds may be exclusively present within the muscle ( eosinophilic myositis ) or within the muscle and the peripheral blood . nmds which have been reported in association with eosinophilic myositis include limb - girdle muscular dystrophy 2a [ 1 , 10 ] , limb - girdle muscular dystrophy 2c , becker muscular dystrophy , myotonic dystrophy type 2 , amyotrophic lateral sclerosis , and inflammatory myopathy manifesting as focal eosinophilic myositis , eosinophilic polymyositis , or eosinophilic perimyositis . eosinophilia in these conditions may be idiopathic or due to parasitic infection , connective tissue disease , hematologic or nonhematological malignancy , drugs , or toxins . eosinophils may not only induce fibrosis , but vice versa , fibroblasts may induce eosinophilia as they are a natural source of eotaxin , a chemokine able to attract eosinophils . this case shows that eosinophilia can be associated with myopathic mid most likely in reaction to myofiber necrosis . if eosinophilia is associated with progressive hyper - ck - emia and multisystem disease , a mid should be suspected . written informed consent was obtained from the patient for publication of this case report and any accompanying images . a copy of the written consent is available for review by the editor - in - chief of this journal . performed clinical investigations , participated in the design and coordination , and helped to draft the manuscript .
backgroundeosinophilia has not been reported as a manifestation of a mitochondrial disorder ( mid ) . here , 
 we report a patient with clinical features suggesting a mid and permanent eosinophilia , multisystem disease , and progressive hyper - creatine - kinase ( ck)-emia for at least 10 years.materials and methodsmethods applied included a clinical exam , blood chemical investigations , electrophysiological investigations , imaging , and invasive cardiological investigations . 
 the patient was repeatedly followed up over several years . 
 he required replacement cardiac surgery.resultsin a 57-year - old male , eosinophilia was first detected at the age of 44 years and has remained almost constantly present until today . 
 in addition to eosinophilia , he developed progressive hyper - ck - emia at the age of 47 years . 
 his history was further positive for hepatopathy , hyperlipidemia , hypothyroidism , renal insufficiency , spontaneous achilles tendon rupture , double vision , exercise intolerance , muscle aching , mild hypoacusis , sensory neuropathy , seizures , and mitral insufficiency / stenosis requiring valve replacement therapy , oral anticoagulation , and pacemaker implantation . 
 based on the multisystem nature of his abnormalities and permanent hyper - ck - emia , a mid was suspected.conclusioneosinophilia can be associated with a mid with myopathy , possibly as a reaction to myofiber necrosis . if eosinophilia is associated with progressive hyper - ck - emia and multisystem disease , a mid should be suspected .