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Botulinum Toxin-A Injection in the Treatment of Spasticity in a Infantile-Onset Neurodegeneration With Brain Iron Accumulation: A Case Report

Do HK, Jo GY, Kwon JK, Kim WJ

Pantothenate kinase-associated neurodegeneration (PKAN) is a neurodegenerative disorder characterized by iron accumulation in the globus pallidus (GP) of the brain (neurodegeneration with brain iron accumulation [NBIA]), which is characterized by...
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A Case of Retinal Pigmentary Degeneration in PKAN

Jo SH, Cheon CK, Kim YU, Jung JH

PURPOSE: Pantothenate kinase-associated neurodegeneration (PKAN), also known as neurodegeneration with brain iron accumulation is an extremely rare degenerative disease. The present study reports a case of retinal pigmentary changes in...
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Clinical Heterogeneity of Atypical Pantothenate Kinase-Associated Neurodegeneration in Koreans

Lee JH, Park J, Ryu HS, Park H, Kim YE, Hong JY, Nam SO, Sung YH, Lee SH, Lee JY, Lee MJ, Kim TH, Lyoo CH, Chung SJ, Koh SB, Lee PH, Cho JW, Park MY, Kim YJ, Sohn YH, Jeon BS, Lee MS

OBJECTIVE: Neurodegeneration with brain iron accumulation (NBIA) represents a group of inherited movement disorders characterized by iron accumulation in the basal ganglia. Recent advances have included the identification of new...
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Hallervorden-Spatz Disease: 2 Cases of Siblings

Kim WS, Kim IO, Yeon KM, Song JG

  • KMID: 2470395
  • J Korean Radiol Soc.
  • 1994 Apr;30(4):779-781.
We report two patients with Hallervorden-Spatz disease, who were diagnosed by same MR findings of marked low signal intensity in the globus and substantia nigra. They presented with ataxic and spastic gait,...
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A Case of Suspected Hallervorden-Spatz Disease

Song CW, Lee SH, Lee SI, Kim DS, Park KH, Kim SW, Kim SH

  • KMID: 1957414
  • J Korean Neurol Assoc.
  • 1992 Sep;10(3):407-412.
Hallervorden-Spatz disease is a rare, autosomal recessive disorder of mainly early childhood which is characterized by pigmentary degeneration of the globus pallidus, substantia nigra, and red nucleus. Clinically it manifests...
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A Case of Fly Catcher's Tongue Phenomenon in Hallervorden-Spatz Disease

Ha CK, Kang SY, Rha JH, Lee IK

  • KMID: 2186061
  • J Korean Neurol Assoc.
  • 2000 Mar;18(2):255-257.
Hallervorden-Spatz disease (HSD) is a rare, progressive, autosomal recessive hereditary disorder characterized by pyramidal and extrapyramidal signs, speech disturbances, mental deterioration and retinal degeneration during childhood or adolescence. In late-onset...
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Hallervorden-Spatz syndrome in two siblings diagnosed by the clinical features and magnetic resonance imaging (MRI)

Kim DW, Choi YI, Kim KJ, Ko TS, Hwang YS, Kim IW

Hallervorden-Spatz syndrome (HSS) is a heredodegenerative disorder characterized by both progressive pyramidal and extrapyramidal signs, dysarthric speech, and mental deterioration. No diagnostic biochemical test is yet available, and diagnosis of...
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Psychiatric Disorder in Two Siblings with Hallervorden-Spatz Disease

Sunwoo YK, Lee JS, Kim WH, Shin YB, Lee MJ, Cho IH, Ock SM

  • KMID: 2315842
  • Psychiatry Investig.
  • 2009 Sep;6(3):226-229.
Hallervorden-Spatz disease (HSD) is a rare autosomal-recessive hereditary disorder characterized by the early onset of progressive movement alterations, including dystonia, rigidity, choreoathetosis, and mental deterioration. HSD is also associated with...
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Dental Treatment of a Patient with Hallervorden-Spatz Disease under General Anesthesia: A Case Report

Lee YK, Seo KS, Kim HJ, Yum KW, Ahn BD

Hallervorden-Spatz disease (HSD) is a rare autosomal recessive disorder associated with excessive iron deposition in the basal ganglia. In general, HSD is characterized by onset in first two decade of...
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Proton Nuclear MR Spectroscopy (H-MRS) in Adult Onset Hallervorden-Spatz Disease

Ha JH, Koh JY, Im JH, Lee MC, Choi CK

  • KMID: 2342575
  • J Korean Neurol Assoc.
  • 1996 Sep;14(3):855-860.
Background & Significance : Hallervorden-Spatz disease (HSD) is a rare neurologic disorder characterized by progressive dystonia, retinal degeneration, pyramidal sign, and mental deterioration. The neuropathological findings include preferential deposition of...
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