CAMPTOCORMIA AND MYOTONIC DYSTROPHY TYPE 2

Prikazujemo rijedak slučaj sekundarne kamptokormije u pacijentice s miotoničnom distrofi jom tip 2. Dijagnoza kamptokormije temelji se na kliničkom zapažanju abnormalne antefl eksije trupa (>45°) u stojećem položaju, koja se pogoršava pri hodanju, a smanjuje u ležećem položaju. Dijagnoza miotonične distrofi je tip 2 temelji se na kliničkoj slici proksimalne miopatije, tipičnom elektromiografskom nalazu i neuroradiološkoj obradi kralježnice (MSCT/MR), a potvrđuje se DNA analizom. Postoje brojni uzroci sekundarne kamptokormije, a miotonična distrofi ja tip 2 je jedan od rjeđih. Hipotrofi ja/atrofi ja paravevertebralne muskulature pri čemu je mišićno tkivo nadomješteno masnim tkivom, verifi cirana neuroslikovnim metodama, upućuje na potrebu za dodatnom obradom s ciljem isključenja različitih mišićnih bolesti i poremećaja. Ovaj slučaj zorno prikazuje da u pozadini kroničnih, bolnih, vertebrogenih sindroma ponekad egzistiraju puno kompleksnije i rjeđe bolesti.We present a rare case of secondary camptocormia in a patient with myotonic dystrophy type 2. The diagnosis of camptocormia is based on clinical observation of abnormal torso antefl exion (>45°) in standing position, which worsens with walking and decreases in supine position. The diagnosis of myotonic dystrophy type 2 is based on the clinical picture of proximal myopathy, typical electromyographic findings, and neuroradiological examination of the spine (MSCT/MR), and is confirmed by DNA analysis. There are a number of causes of secondary camptocormia, and myotonic dystrophy type 2 is one of the rarer ones. Hypotrophy/atrophy of the paravertebral musculature, where the muscle tissue is replaced with fat tissue, verifi ed by neuroimaging methods, indicates the need for additional processing to exclude various muscle diseases and disorders. This case clearly shows that in the background of chronic, painful, vertebrogenic syndromes, much more complex and rare diseases sometimes exist