Objective: To present a patient diagnosed with secondary hypermanganism due to cirrhosis whose extrapyramidal findings had recovered significantly after chelation therapy.

Background: Manganese is an essential trace element that causes neurotoxicity with induction of permanent damage of globus pallidus resulting in Parkinsonian and dystonic symptoms. Although the benefit of chelation therapy in a subgroup of inherited or toxic hypermanganism has been relatively well documented, the efficiency of chelation in hypermanganism due to cirrhosis remains to be clarified as only a few reports had been available.

Methods: We report a patient presenting with Parkinsonian symptoms who after further investigations had the diagnosis of secondary parkinsonism due to hypermanganism in the background of cirrhosis. We will demonstrate recovery of his symptoms on video recordings of pre and post-chelation therapy of one month duration (2 grs weekly, intravenous CaNaEDTA).

Results: 48-year-old male, with a history of idiopathic cirrhosis for 5 years, admitted to our center due to gait disturbance, ataxia and lisping which had started 5 years ago and progressed. On neurological examination, he was bilateral moderate rigidity and bradykinesia with Parkinsonian gait that was also evaluated to a degree as dystonic. Previously symptomatic levodopa treatments had failed. Laboratory examinations revealed mild disturbances in liver function, other test results were in normal ranges (Hg: 14 g/dL, serological markers for Wilson disease). Cranial MRI showed bilateral T1 hyperintensity in bilateral globus pallidus and blood manganese levels was at the high range [17.8 microgram/L, normal range: 0-18]. We started chelation treatment of intravenous CaNaEDTA with the diagnosis of hypermanganasemia secondary to cirrhosis and after 4 week of therapy period, a significant improvement was achieved. A lower dose CaNaEDTA regimen (0,5-1 gr/weekly) was continued in the follow up; however, not any further improvement was achieved on the last evaluation at 6th month afterwards.

Conclusions: For our knowledge, this case illustrates the most dramatic and permanent response to chronic chelation treatment that has ever been documented in a patient with secondary hypermanganasemia due to cirrhosis. Further case studies are needed to clarify the indications addressing chelation treatment in symptomatic patients of cirrhosis with brain manganese accumulation.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/parkinsonism-due-to-hypermanganism-in-the-background-of-cirrhosis-and-recovery-by-canaedta/