Hallervorden-Spatz disease (HSD) was and is known as a rare disorder primarily characterized by progressive extrapyramidal dysfunction and dementia alongside optic nerve atrophy or retinal degeneration and pyramidal signs. The rate of occurence of HSD is thus far unknown. Progress in DNA diagnostics stirred up a nomenclature and from HSD, or, perhaps better put, the Hallervorden-Spatz syndrome,...

Disease summary: Pantothenate kinase-associated neurodegeneration (PKAN) is a rare autosomal recessive disorder that was first described by the neuropathologist Julius Hallervorden and the neurologist Hugo Spatz in 1922 [1]. The active involvement of Hallervorden in euthanasia in Germany during World War II and the discovery of the defective gene (mutation in pantothenate kinase 2 gene, located...

Patients with Hallervorden-Spatz disease may be confronted by invasive procedure, like gastrostomy and thalamotomy for care of the status of extreme dystonia and rigidity. This rare disorder possesses potential perioperative risks, such as difficult airway management, aspiration pneumonia, hyperpyrexia, dehydration, acute renal failure, and postoperative pulmonary insufficiency. As patients wer...

We describe fluid attenuated inversion recovery sequence MRI and proton MR spectroscopy appearances of the brain in a 7-year old Saudi boy who presented with the clinical features of Hallervorden-Spatz disease to King Fahad National Guard Hospital, Riyadh, Kingdom of Saudi Arabia.

The case report on Hallervorden Spatz disease (HSD)(1) aroused interest, particularly because we are currently managing a child with this disease in our Pediatric Neurology Clinic. Although the authors have rightly suspected the condition, they have not presented parameters on the basis of which the diagnosis was made. Absence of criteria for Wilson's disease and presence of family history of s...

Hallervorden and Spatz first described, in a sibship of 12, five sisters with clinically increasing dysarthria and progressive dementia, whose brains showed a brown discoloration of the globus pallidus and substantia nigra. Subsequently the basis has been shown to be a neurodegeneration with brain iron accumulation or pantothenate kinase-associated neurodegeneration due to mutations in the pant...