Emel Koyuncu KUTUK, Hasan KAYA, NESLİHAN AKKİŞİ KUMSAR, Ozlem Bolat KAYA, Nesrin DİLBAZ

Psychiatric Aspects of Adult-Onset Hallervorden- Spatz Syndrome: A Report of Two Cases

Hallervorden Spatz Sendromu, nadir görülen, ailesel, ilerleyici ve genellikle ölümcül seyredebilen bir hastalıktır. Extrapiramidal rijidite, dizartri ve demans bulguları ile karakterize, globus pallidus ve substantia nigrada patolojik demir birikimi ile giden bir seyri vardır. Genellikle tanı MRI incelemede bilateral globus pallidusta lokalize olan hiperintens alanların çevrelediği hipointens alanların görülmesi ile konur. Bu görünüme kaplan gözü denilmektedir ve hastalığa spesifiktir. Bu sendromda kognitif yıkımdan kişilik değişikliklerine, dürtüsellik ve şiddet davranışından depresyon ve emosyonel değişikliklere kadar çeşitli psikiyatrik belirtiler görülebilmektedir. Bu sunumda psikiyatrik belirtilerle giden iki Hallervorden Spatz Sendromu olgusunun tartışılması hedeflenmiştir

Erişkin başlangıçlı hallervorden-spatz sendromunun psikiyatrik yönleri: İki olgu sunumu

Hallervorden-Spatz Syndrome (HSS) is a rare, familial, progressive, and ultimately fatal disorder characterized by extrapyramidal rigidity, dysarthria, pyramidal tract involvement, and dementia, with pathological evidence of iron deposition in the globus pallidus and substantia nigra. The diagnosis is usually made using magnetic resonance imaging (MRI), where bilateral high signal intensity in the globus pallidus surrounded by low signal intensity areas is found. This is called an "eye-of-the-tiger" appearance and is considered specific to this disorder. Psychiatric aspects of this syndrome are common and include cognitive decline, personality changes with impulsivity and violent outbursts, depression and emotional lability. Here we report two cases of Hallervorden-Spatz Syndrome with psychiatric presentation

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