Hallervorden‐spatz syndrome: Clinical and magnetic resonance imaging correlations

@article{Sethi1988HallervordenspatzSC,
  title={Hallervorden‐spatz syndrome: Clinical and magnetic resonance imaging correlations},
  author={Kapil Sethi and Rebecc A J. Adams and David W. Loring and Taher El Gammal},
  journal={Annals of Neurology},
  year={1988},
  volume={24}
}
Two women with a presumptive diagnosis of Hallervor‐den‐Spatz syndrome had a combination of dystonia and parkinsonism. One had retinitis pigmentosa. Neuropsychological testing revealed Decemberreased verbal fluency and visuoconstructional and motor deficits. Magnetic resonance imaging performed with a high‐field‐strength unit (1.5 Tesla) showed striking abnormalities in the globus pallidus bilaterally (“eye‐of‐the‐tiger” sign). Magnetic resonance imaging may prove useful in the diagnosis of… 

[Hallervorden Spatz syndrome: magnetic resonance findings. Case report].

Hallervorden-Spatz syndrome is a neurodegenerative disease, autosomic recessive with two clinical features: early and late onset. Psychiatric, pyramidal and extrapyramidal signs are present in the

Generalized freezing in Hallervorden‐Spatz syndrome: case report

A female patient who was exhibiting slowly progressive, severe, generalized freezing of voluntary movement and disequilibrium is believed to be a case of Hallervorden‐Spatz syndrome, although without neuropathological examination the authors were unable to confirm the diagnosis with certainty.

Atypical Hallervorden–Spatz disease with preserved cognition and obtrusive obsessions and compulsions

This case demonstrates that HSD patients who survive into middle age should not be assumed to have a progressive dementia, in such cases, atypical behavioral problems such as persistent obsessions and compulsions may be present instead.

Hallervorden spatz disease: MR and pathological findings of a rare case.

We describe a child with pathologically proven Hallervorden Spatz disease. He presented with extrapyramidal symptoms and characteristic "eye-of-the-tiger" sign on magnetic resonance imaging. He was

[Hallervorden-Spatz disease: findings in the nigrostriatal system].

This constellation of findings shows that the degeneration lies primarily outside the nigrostriatal system, supporting arguments for the nosologic distinction of HSD from other extrapyramidal illnesses.

Radiologic features of Hallervorden Spatz Disease.

Sometimes the pallidal hypointense signals surround hyperintense signals, this is known as “tigereye-sign” and is postulated to be specific for Hallervorden Spatz Disease and is reported on two brothers with such MRI findings.

Hallervorden Spatz Disease

Gene mapping has located the defect to be in the coding sequence of a gene called PANK– 2, indicative of iron deposition, suggesting Hallervorden Spatz Disease.

Hallervorden-Spatz Disease: 2 Cases of Siblings

T2-weighted images showed marked low signal intensity in the globus pallidus and substantia nigra indicating an increased iron-deposition, and it mightsuggest Hallervorden-Spatzdisease.

Hallervorden-Spatz syndrome: a rare cause of extrapyramidal manifestations

An unusual case of atypical Hallervorden-Spatz syndrome with onset during adolescence and rapid progression in a young female patient who showed the characteristic “eye of the tiger” appearance on magnetic resonance imaging [MRI] of brain.

Pure akinesia: An unusual phenotype of Hallervorden‐Spatz syndrome

This case report highlights the phenotypic diversity of Hallervorden Spatz syndrome and the need for further investigation of adult‐onset pure akinesia syndromes.
...

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