parkinsonism is used to describe a syndrome manifested by any combination of six cardinal features: tremor at rest, rigidity, bradykinesia, loss of postural reflexes, flexed posture and the freezing. decreased dopaminergic neurotransmission in the basal ganglia is the core biochemical pathology in parkinsonism. hereditary parkinsonism includes various heredodegenerative diseases such as hallerv...

Pantothenate kinase-associated neurodegeneration is a form of neurodegeneration with brain iron accumulation, characterized by a progressive movement disorder and prominent iron deposition in the globus pallidus. Formerly referred to as Hallervorden-Spatz syndrome, the disorder was renamed pantothenate kinase-associated neurodegeneration after discovery of the causative gene, PANK2. Although th...

Two siblings, from consanguineous parents, developed in their twenties a Parkinsonian syndrome. In the elder, the disease evolved for 13 years and the necropsic study was diagnostic of Hallervorden-Spatz disease. The younger sibling is severely affected after 12 years of the disorder. Several CT and one MR studies done in this patient during the last 4 years have been normal. Ultrastructural st...

Neurological dysfunction in children associated with bilateral hypodense lesions of the basal ganglia is seen in disorders like Huntington's chorea, Wilson's disease, Hallervorden Spatz syndrome, Leigh's disease, infantile striatal necrosis, hypoxia, ischemia and various intoxications(l). Goutieres and Aicardi described in 1982 a new neuroradiological syndrome characterized by a rapid clinical ...

PURPOSE To investigate spatial distribution of iron accumulation in the globus pallidus (GP) in patients with Hallevorden-Spatz syndrome (HSS) using phase imaging. We compared sensitivity of a phase imaging technique to relaxation rate measurement methods (R1,R2,R2*) for iron quantification. MATERIALS AND METHODS R1, R2, and R2* were measured in GP structure of the brain of eight pantothenate...

Background—Isolated cerebral thromboangiitis obliterans (Spatz-Lindenberg disease) is not well recognized as a cause of vascular dementia. Case Description—A 58-year-old woman presented with dementia and pyramidal signs. Neuroimaging showed multiple areas of white matter change. Brain biopsy showed intimal thickening of the walls of leptomeningeal and intraparenchymal arteries, almost to comple...