[Hallervorden-Spatz syndrome. Differential diagnosis of early onset dementia]

We report the history of a 38 year old patient who began to develop mental deterioration at the age of 26. After a time of 7 years neurological signs like writing dystonia occurred. Hallervorden-Spatz-Disease (HSD) was diagnosed at the age of 36 in vivo with the clinical presentation of severe dystonia, rigidity, dementia, and typical signal loss in the globus-pallidus the reticular part of the substantia nigra, and the nucleus ruber in the T-2 weighted MRI. The "eye-of-the-tiger"-sign, a bilateral hyperintensity in the rostral globus pallidus, was not observed in follow-up examinations. HSD is a rare autosomal-recessive or sporadic disease of unknown etiology. In one third of the patients a dementing process is the first clinical sign of the disorder, and is a rare differential diagnosis of early onset dementia.