Ruiz-Sandoval J L, García-Ramos G, Vega-Boada F, Estañol-Vidal B
Instituto Nacional de Neurología y Neurocirugía, Manuel Velasco Suárez, México, D.F.
Rev Invest Clin. 1998 May-Jun;50(3):249-53.
Hallervorden-Spatz disease (HSD) is an uncommon disorder, progressive and degenerative of the basal ganglia. It begins in the first or second decade of life and is characterized by a dominant extrapiramidal signs, dystonia and progressive dementia. It is autosomic recessive, although sporadic cases are seen in 15%. There is no biological marker for the disease. The post-mortem findings include iron deposits in the globus pallidum and pars reticulata of the substantia nigra. Magnetic resonance imaging (MRI) in T2 shows symmetric hypointense lesions in both globus pallidum with a hyperintense center: giving the "tiger's eye" sign. This is the first case reported in Mexico of sporadic HSD with typical clinical and MRI findings.
