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Background Hepatolenticular degeneration (HLD), also known as Wilson disease (WD), is a rare autosomal-recessive hereditary disease, which is often missed and misdiagnosed because of its various clinical manifestations. And WD is even more rare with giant subarachnoid cysts. In this report, we will provide a case of WD with an intracranial arachnoid cyst (IAC). Case description A 27-year-old woman was hospitalized in a traditional Chinese medicine hospital in Guangzhou with the first manifestation of a “slight involuntary tremor of her left upper limb”. There was no improvement after acupuncture treatment, and then she was transferred to another large general hospital in Guangzhou. MRI examination of the head showed “left frontal, parietal and temporal giant subarachnoid cyst” and the patient underwent “left frontotemporal arachnoid cyst celiac shunt operation.” After the operation, the patient’s left limb shaking remained unchanged. Subsequently, the patient was referred to another big hospital in Guangzhou, considered “Parkinson’s disease,” and given “Medopa, Antan” and other treatments. However, the patient’s limb shaking continued to increase and gradually developed to the extremities. At last, the patient was referred to our hospital, combined with the medical history, neurological signs, and auxiliary examination results, improve the examination of corneal K-F ring, blood ceruloplasmin, gene screening, and other tests; the diagnosis was confirmed as hepatolenticular degeneration. Conclusion After expelling copper and symptomatic treatment, the condition is improved.

A rare giant intracranial arachnoid cyst confused the diagnosis and treatment of Wilson disease