[Superficial siderosis of the central nervous system in a patient with hemochromatosis and Wilson's disease]

Abstract

History and findings on admission: A 55-year-old patient with a history of Wilson's disease and biopsy proven hemochromatosis complained of hearing loss, vertigo, and gait disturbance. Clinical examination confirmed hearing loss, revealed cerebellar syndrome and bilateral pyramidal tract disturbances.

Investigations: Neurophysiology confirmed pathological findings on clinical examination. Cerebral magnetic resonance imaging (MRI) disclosed deposition of hemosiderin suggestive for superficial siderosis of the central nervous system. Cerebrospinal fluid findings were normal.

Diagnosis: The triad of hearing loss, cerebellar syndrome, and pyramidal tract disturbances associated with typical findings on MRI led to diagnosis of superficial siderosis.

Clinical course and therapy: Etiology in this case remained unclear; no source of bleeding was detected. Thus, no causal therapeutic option was available.

Conclusion: A unique case of superficial siderosis in a patient with a history of Wilson's disease and hemochromatosis is presented. Unexpected new symptoms in a successfully treated Wilson's disease patient require further diagnostic work-up not to miss potentially curable differential diagnoses. Thus, regular neurological follow-up visits of Wilson's disease patients are required.