We report a 50-year-old man who suffered from Parkinson's disease (PD) and mild hemophilia A. Initial motor symptoms of PD appeared at 40 years of age. He was treated with ropinirole and levodopa. At the age of 44, motor fluctuations emerged and worsened after ropinirole discontinuation due to ongoing impulse control disorder (hypersexuality and excessive purchase of expensive breeding birds). Deep brain stimulation (DBS) was first considered at another movement disorders (MDS) center at the age of 47, but it was ultimately contraindicated due to hemophilia. At his first consultation at our MDS center, the main complaints were motor fluctuations and left–sided biphasic dyskinesias, particularly in the leg (Video 1: Pre LCIG). His levodopa equivalent daily dose (LEDD) was 1300 mg. We reconsidered DBS, but due to a cognitive deficit and psychiatric complications—progressive multidomain PD-MCI (executive-memory-visual–spatial) (MMSE 26/30) and pre/psychotic phenomena (instead of socks, he sees bugs, he has to check manually, a rat ran across the room, etc. ) —we decided it was contraindicated as well. Quetiapine treatment (50 mg daily) was initialized, and psychiatric complications disappeared. Continuous subcutaneous apomorphine infusion (CSAI) was also at risk of the above symptoms. Levodopa/carbidopa intestinal gel (LCIG) treatment was recommended and approved by the MDS center team. The primary procedural risk of LCIG treatment was PEG placement. After consultation with the attending hematologist, the patient received 4000 IU of recombinant factor VIII (Advate®) just before the PEG procedure. The gastrostomy was performed without complications, and LCIG treatment was initiated (LEDD 1240 mg: morning dose, 14 mL, continuous dose, 3 mL/h, extra dose, 1. 5 mL). During the first hours after LCIG initiation, bleeding from the surgical wound occurred. We administered 2000 IU (Advate®) 8 hours after the first dose, one stitch was placed in the PEG wound, and bleeding stopped within several hours. Factor VIII supplementation was extended to 5 days. The jejunal tube was initially twisted and misplaced in the stomach, and it was necessary to place it in the jejunum under endoscopic control. Following correct jejunal placement, the patient experienced a significant reduction in fluctuations and biphasic dyskinesias. The patient has currently been on LCIG therapy for 18 months. The PEG insertion site healed without further bleeding (Picture 1). PD-MCI remains stable under donepezil therapy (MMSE 25/30), and the patient does not exhibit biphasic dyskinesias or psychotic complications (Video 1: Post LCIG). We introduced the first documented intestinal pump treatment for PD in a patient with hereditary hemophilia A. Our patient demonstrates that LCIG treatment is safe; however, close cooperation with a hematologist is necessary. Essential care includes the preoperative administration of recombinant factor VIII and vigilant postoperative monitoring, with prompt supplementation extension if bleeding occurs. Device-aided PD therapies are feasible in patients with hemophilia; however, clinical experience is limited. Only two cases of patients with PD and hemophilia treated with DBS have been reported. When specific precautions are implemented, DBS implantation appears safe. 1, 2 In addition, a few reports have documented the safety of subcutaneous injections or infusions in hemophilic patients, supporting the consideration of subcutaneous PD pump system therapies. 3, 4 (1) Research project: A. Conception, B. Organization, C. Execution; (2) Manuscript Preparation: A. Writing of the first draft, B. Review and Critique. P. H.: 1A, B, C, 2A J. M.: 1B, C, 2B I. V.: 1A, C, 2B M. V.: 1A, 2B B. K.: 1C, 2B L. K.: 1B, C, 2A P. Š.: 1B, C, 2A A. R.: 1C, 2B J. V.: 1A, 2B R. J.: 1A, 2B Ethical Compliance Statement: The authors confirm that this work was approved by an institutional review board/ local ethical committee (Eticka komise VFN). Informed consent from the patient was obtained. We confirm that we have read the Journal's position on issues involved in ethical publication and affirm that this work is consistent with those guidelines. Funding Sources and Conflict of Interest: This work was supported by the Cooperatio Program in Neurosciences, Charles University, by the project MH CZ–DRO–VFN64165, and by the project National Institute for Neurological Research (Programme EXCELES, ID Project No. LX22NPO5107) —Funded by the European Union—Next Generation EU. The authors declare no conflicts of interest relevant to this work. Financial Disclosures for the Previous 12 Months: The authors declare that PH received honoraria from AbbVie and Medis Pharma, JM, IV, MV, PŠ, LK, BK, JV and RJ had no additional disclosures to report. Author disclosures are available in the Supporting Information. The data that support the findings of this study are available from the corresponding author upon reasonable request. Data S1. COIdisclosure. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.
Havránková et al. (Thu,) studied this question.