Combined dimercaptosuccinic acid and zinc treatment in neurological Wilson’s disease patients with penicillamine-induced allergy or early neurological deterioration

Abstract The clinical data of safety and efficacy of a combined treatment with dimercaptosuccinic acid (DMSA) and Zinc with 2 years’ follow-up in 60 neurological Wilson’s disease (WD) patients was retrospectively analyzed. All the patients included in the present study were newly diagnosed and initialized with D-penicillamine (DPA) treatment but were found to have either neurological deterioration or allergy, and their treatment was switched to a combined treatment of DMSA and Zinc. Fifty-one patients (85%) had the neurological symptoms improved 1 and 2 years after treatment, 7 (11.67%) experienced a stable neurological condition, and 2 (3.33%) suffered deterioration of neurological symptoms. No early neurological deterioration was observed in all patients. Twenty-five percent patients experienced mild adverse reactions which did not require a discontinuation of the DMSA and Zinc treatment. Our study confirmed the safety and efficacy of the combined DMSA and Zinc therapy as an initial and probably long-term treatment in neurological WD patients.

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Combined Sodium Dimercaptopropanesulfonate and Zinc versus D-penicillamine as First-line Therapy for Neurological Wilson’s disease

10.21203/rs.2.346/v2 ◽

2020 ◽

Author(s):

Jing Zhang ◽

Lulu Xiao ◽

Wenming Yang

Keyword(s):

Wilson’S Disease ◽

Wilson's Disease ◽

Neurological Symptoms ◽

Neurological Deterioration ◽

First Line ◽

First Line Therapy ◽

Cell Counts ◽

Line Therapy ◽

Group 2 ◽

Group 1

Abstract Background: Even though recent research has achieved significant advancement in the development of therapeutic approaches for Wilson’s diseases (WD), the current treatment options available for the neurological symptoms of WD are significantly limited and yet to be standardized. Objective: The aim of this study was to compare the course of treatment for WD patients with neurological symptoms receiving either combined sodium 2, 3-dimercapto-1-propane sulfonate (DMPS) and zinc treatment or D-penicillamine (DPA) monotherapy as first-line therapy. Methods: The case records of 158 patients diagnosed with neurological WD were retrospectively analyzed. These patients were administrated with either intravenous DMPS + Zinc (group 1) or DPA (group 2) for 8 weeks. During the period of treatment, neurological symptoms were assessed using Global Assessment Scale (GAS), the key hematological and biochemical parameters (such as aminotransferase, serum ceruloplasmin, 24-h urine copper excretion), as well as adverse effects were recorded and analyzed. Results: 93 patients in Group 1, displayed decreased GAS scores consistently in comparison to the baseline (P < 0.01). Among them, 64 patients (68.1%) displayed a significant improvement in their neurological status after 8 weeks, while 10 patients (10.8%) experienced neurological deterioration. Among the 65 patients in Group 2, 30 patients (46.2%) displayed neurological improvements, while 21 patients (32.3%) displayed neurological deterioration. 6 patients discontinued their treatment midway due to their exacerbating neurological symptoms. A comprehensive comparison of the effectiveness of the two courses of treatment revealed that patients in Group 1 demonstrated a higher improvement ratio (P < 0.01) and lower worsening ratio of the neurological symptoms of the patients (P < 0.01) in comparison to the patients in group 2. Meanwhile, renal function, liver enzyme and the blood cell counts remained stabilized in group1. Conclusions: This study suggests that the combined therapeutic approach of treatment with DPMS and zinc should be the preferred first-line therapy in treating the neurological symptoms of WD, in comparison to the treatment with DPA as the experimental data demonstrates that it is significantly more efficient. Keywords: Wilson’s disease (WD), Sodium 2, 3-dimercapto-1-propane sulfonate (DMPS), D-penicillamine (DPA), Zinc, Global Assess

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