Session Time: 1:45pm-3:15pm
Location: Les Muses Terrace, Level 3
Objective: To evaluate the efficacy of calcium disodium edetate (CaNa2EDTA) chelation as a treatment for acquired hepatocerebral degeneration.
Background: Acquired hepatocerebral degeneration is a rare complication of liver cirrhosis occurring predominantly through manganese accumulation in basal ganglia, manifesting most commonly as dyskinesia, parkinsonism, cognitive decline, neuropsychiatric symptoms and ataxia. Treatment options lack a strong evidence base and are limited anecdotally to liver transplant or reversal of portosystemic shunt (1), chelation with trientine (2) or branched chain amino acid therapy (3). CaNa2EDTA chelation therapy has been used with good effect in cases of hepatocerebral degeneration secondary to environmental exposure to manganese (4), but has rarely been trialled in acquired hepatocerebral degeneration cases.
Method: Case report and review of the literature.
Results: We report a case of a 71 year old lady who presented with an 18 month history of orobuccal dyskinesias, cognitive decline, disinhibition and gait disturbance. MRI brain revealed typical bilateral symmetrical basal ganglia T1 hyperintensity, alongside significantly elevated serum manganese (765nmol/L, normal range 72.8-218.5nmol/L). There was no exposure history to suggest environmental manganism. The presence of cirrhosis secondary to non-alcoholic fatty liver disease led to a diagnosis of acquired hepatocerebral degeneration. Liver transplantation and reversal of portosystemic shunting were not options in our patient’s case, and oral trientine was not available. We therefore elected to administer CaNa2EDTA chelation (75mg/kg intravenously daily for 5 days). There was a marked increase in manganese clearance (urinary manganese 363 to 2967nmol/24hour collection; serum manganese 765 to 652 nmol/L) accompanied by a concomitant rapid, marked and objective improvement in the patient’s gait, cognition and involuntary movements, which was sustained at review at five months. This is the second report (5) of calcium disodium edetate (CaNa2EDTA) chelation bringing symptomatic benefit in acquired hepatocerebral degeneration.
Conclusion: This case suggests that CaNa2EDTA chelation may represent an efficacious treatment for acquired hepatocerebral degeneration as well as environmental manganism. Larger studies are needed to evaluate this more robustly.
References: 1. Shin H, Park HK. Recent updates on Acquired Hepatocerebral Degeneration. Tremor Other Hyperkinet Mov 2017;7 2. Park HK, Kim SM, Choi CG, Lee MC, Chung SJ.Effect of trientine on manganese intoxication in a patient with acquired hepatocerebral degeneration. Mov Disord 2008;23:768-770 3. Ueki Y, Isozaki E, Miyazaki Y, Koide R, Shimizu T, Yaki K et al. Clinical and neuroradiological improvement in chronic acquired hepatocerebral degeneration after branched-chain amino acid therapy. Acta Neurol Scand 2002; 106: 113-116 4.Herrero Hernandez E, Discalzi G, Valentini C, et al. Follow up of patients affected by manganese-induced Parkinsonism after treatment with CaNa2EDTA. Neurotoxicology 2006 May; 27(3):333-9 5. Akyildiz A, Parlaz A, Kiylioglu N, Tataroglu C, Akyol A. Chronic acquired hepatocerebral degeneration: the effect of CaNa2EDTA chelation. J Neurol Sci [Turk] 2013 30: (3) 37; 579-582
To cite this abstract in AMA style:Z. Voysey, L. Azzopardi, B. Fiddes, S. Bradberry. Acquired hepatocerebral degeneration treated with calcium disodium edetate chelation [abstract]. Mov Disord. 2019; 34 (suppl 2). https://www.mdsabstracts.org/abstract/acquired-hepatocerebral-degeneration-treated-with-calcium-disodium-edetate-chelation/. Accessed December 5, 2023.
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MDS Abstracts - https://www.mdsabstracts.org/abstract/acquired-hepatocerebral-degeneration-treated-with-calcium-disodium-edetate-chelation/