The detection of low density lipoprotein-4 (LRP4) antibodies in double seronegative (dSN) myasthenia gravis (MG) patients has provided new insights in the diagnosis and treatment of MG. However, there are limited data regarding the clinical presentation and treatment response in dSN MG patients with LRP4-antibodies. We present a case series of three Caucasian dSN MG patients with positive LRP4-antibodies sharing a common ethnic background that presented with isolated ocular symptoms (MGFA I). The demographic and clinical characteristics, the diagnostic work-up as well as the treatment response during a follow-up period of 12-24 months are described in detail. All patients were treated successfully with acetylcholinesterase inhibitors (AcheI) and prednisone with two exhibiting full remission of their symptoms, while the remaining exhibited mild residual diplopia. Notably, we documented no signs of generalized disease progression, while no patient required immunosuppressive treatment. In conclusion, the distinct clinical phenotype of our patients highlights the clinical relevance of screening for LRP4-antibodies in patients presenting with isolated ocular MG independent of age and gender, since it may lead to the timely diagnosis of MG and prompt initiation of effective therapy with ACheI and corticosteroids.

• Klíčová slova
• Antibodies, Diagnosis, LRP4, Myasthenia, Ocular, Treatment,
• MeSH
• blefaroptóza farmakoterapie   imunologie   MeSH
• cholinesterasové inhibitory terapeutické užití   MeSH
• diplopie farmakoterapie   imunologie   MeSH
• dospělí MeSH
• glukokortikoidy terapeutické užití   MeSH
• kombinovaná farmakoterapie MeSH
• lidé středního věku MeSH
• lidé MeSH
• myasthenia gravis farmakoterapie   imunologie   MeSH
• prednison terapeutické užití   MeSH
• proteiny související s LDL-receptory imunologie   MeSH
• pyridostigmin-bromid terapeutické užití   MeSH
• senioři nad 80 let MeSH
• výsledek terapie MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři nad 80 let MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH
• práce podpořená grantem MeSH
• Názvy látek
• cholinesterasové inhibitory MeSH
• glukokortikoidy MeSH
• LRP4 protein, human MeSH Prohlížeč
• prednison MeSH
• proteiny související s LDL-receptory MeSH
• pyridostigmin-bromid MeSH

AIM: To inform about the possibility of negative influence of repeated application of the botulotoxine A derivate on the eye lid position with the changes of their tissue structure. At the same moment, the authors report on literature analysis of positive and negative influence of this neuro toxine from the medical point of view. MATERIALS AND METHODS: At the Department of Ophthalmology in the Faculty Hospital Královské Vinohrady, Prague, Czech Republic, E.U., there were surgically treated three patients after repeated applications of botulotoxine A derivates. The first case was bilateral ptosis of upper eyelids; the other two were indicated because of entropium with trichiasis of lower eyelids. In the medical history, there was recorded ten years lasting above-mentioned drug treatment for blepharospasm diagnosis in a 65 years old man introducing bilateral symmetrical ptosis. Because of the following frontal muscle involvement, and partial decreasing levator palpebrae muscles function, the positioning of the upper eyelids was treated by the aponeurosis plasty with good functional result. In two female patients, aged 72 and 90 years, the indication for successful surgical treatment was chronic entropium of lower eyelids; initially ineffectively treated by means of repeated intradermal injections of botulotoxin A derivates. RESULTS: Histological examinations of the excided skin and subcutis samples taken during ectropium plasties showed unfavorable and irreversible changes including especially scarring of the eyelid and atrophy of the striated muscles. CONCLUSION: The indication of botulotoxine A derivates application should be carefully weighted and the proper indication should be chosen. From the ophthalmologic point of view, blefarospasmus only is the appropriate diagnosis, and the relative indication is the temporary induced ptosis of the upper eyelid closing the interpalpebral fissure to prevent corneal changes in lagophthalmos of various etiologies as an alternative to the tarsoraphy. Always we have to consider the frequency of applications, because repeatedly used derivate of this neurotoxine causes irreversible changes in cutaneous and subcutaneous tissue.

• MeSH
• blefaroptóza farmakoterapie   patologie   MeSH
• botulotoxiny typ A aplikace a dávkování   škodlivé účinky   MeSH
• entropion farmakoterapie   patologie   MeSH
• lidé MeSH
• oční víčka účinky léků   patologie   MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• Check Tag
• lidé MeSH
• mužské pohlaví MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH
• kazuistiky MeSH
• Názvy látek
• abobotulinumtoxinA MeSH Prohlížeč
• botulotoxiny typ A MeSH