Hallervorden‐spatz syndrome: Clinical and magnetic resonance imaging correlations

  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},
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.



MR Imaging of Hallervorden‐Spatz Disease

MR imaging of two patients with HSD provided good quality imaging of these patients' atrophie brain stems and cerebella, with signal aberrations in the lentiform nuclei and surrounding white matter consistent with iron storage or disordered myelination.

Hallervorden-Spatz syndrome.

• Two siblings with Hallervorden-Spatz syndrome showed striking homotypism and homochronism. Neuropathologic examination and electron microscopic studies were done; neutron activation analysis showed

Computed tomography in Hallervorden-Spatz disease.

We report computed tomography (CT) findings of an autopsy-proven case of late-infantile Hallervorden-Spatz disease (HSD). The patient's symptoms started in preschool age with dystonic posture,

Computed tomography in Hallervorden‐Spatz disease

Computed tomography in a 34-year-old woman with Hallervorden-Spatz disease showed severe diffuse cerebral atrophy and ventricular enlargement, andRatios between the intercaudate distance and the width of the frontal horns exceeded those found in patients with Huntington disease.

Mineralization of the basal ganglia detected by CT in Hallervorden‐Spatz syndrome

We studied a patient with autopsy-proven Hallervorden Spatz syndrome (HSS) and the previously unreported finding of high-density lesions in the basal ganglia on CT. The diagnosis of HSS should be

Clinical NMR imaging of the brain in children: normal and neurologic disease.

The results of initial clinical nuclear magnetic resonance imaging of the brain in eight normal and 52 children with a wide variety of neurologic diseases were reviewed. The high level of gray-white

A study of iron metabolism in neuropsychiatric patients. Hallervorden-Spatz disease.

A study of the iron metabolism with the help of γ-ray 59 Fe isotope in patients with Hallervorden-Spatz disease and in some other neuropsychiatric patients in order to make a comparison between the two groups.

Hallervorden‐Spatz disease: Cysteine accumulation and cysteine dioxygenase deficiency in the globus pallidus

It is suggested that cysteine accumulates locally in the globus pallidus in Hallervorden‐Spatz disease as a result of an enzymatic block in the metabolic pathway from Cysteine to taurine.

Magnetic resonance imaging of transfusional hemosiderosis complicating thalassemia major.

In patients with transfusional hemosiderosis and thalassemia major the liver and bone marrow demonstrated abnormally low spin-echo intensities and the kidneys and muscles had abnormally high intensities, which correlate with in vitro MR observations of ferric (Fe+3) solutions.

NMR imaging of the liver in two cases of iron overload.

Two cases of iron overload in the liver are presented--one of hemochromatosis with associated hepatoma and one of hemosiderosis with probable androgen dependent multicentric hepatic adenomas. Nuclear