A Quandary of Cuprum - Wilson’s Disease Disguising as Progressive Myoclonic Epilepsy

Abstract

Although metals are indispensable for the production of articles in our daily usage, the deposition of these metals in human tissue is known to cause disease. However, it is not always the ingestion of abnormal amounts of lead, iron, or copper that makes our tissues morbid; our hereditary and metabolic issues are to be blamed as well. Wilson's disease is one such hereditary disease that creates chaos in tissues, usually the brain and liver, via deposition of abnormal amounts of copper in them.While Wilson's disease almost seems to bring a picture of a young patient with dystonia and other extrapyramidal symptoms in our imagination, seizures are very uncommon in this disorder. Non-stimulus-sensitive myoclonic jerks along with cognitive decline as the initial presentation of this disease have never been reported until now. In fact, such a presentation would make the neurologist believe that the patient has some type of progressive myoclonic epilepsy (PME), thus, creating a dilemma. We report two such dilemmatic cases of Wilson's disease that disguised as PME.

Coexistence of seizure with Wilson's disease: a systematic review

“…Firstly, multiple seizure types were noted in some patients, for example in the Berger et al case report the patient developed recurrent generalised tonic‐clonic seizures followed by complete partial SE. Secondly, six studies did not report the seizure type while eight studies did not report EEG findings. Lastly, available data are mostly of case reports, case series and low sample size studies with consequent selection bias.…”