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Psychosis as presenting symptom in adult-onset Hallervorden-Spatz syndrome

Published online by Cambridge University Press:  24 June 2014

Marios Panas ,
Konstantinos Spengos ,
Georgios Koutsis ,
Georgios Tsivgoulis ,
Konstantinos Sfagos ,
Nikolaos Kalfakis  and
Dimitris Vassilopoulos
Marios Panas*
Affiliation:
Department of Neurology, Neurogenetics Unit, Eginition Hospital, University of Athens, Athens, Greece
Konstantinos Spengos
Affiliation:
Department of Neurology, Neurogenetics Unit, Eginition Hospital, University of Athens, Athens, Greece
Georgios Koutsis
Affiliation:
Department of Neurology, Neurogenetics Unit, Eginition Hospital, University of Athens, Athens, Greece
Georgios Tsivgoulis
Affiliation:
Department of Neurology, Neurogenetics Unit, Eginition Hospital, University of Athens, Athens, Greece
Konstantinos Sfagos
Affiliation:
Department of Neurology, Neurogenetics Unit, Eginition Hospital, University of Athens, Athens, Greece
Nikolaos Kalfakis
Affiliation:
Department of Neurology, Neurogenetics Unit, Eginition Hospital, University of Athens, Athens, Greece
Dimitris Vassilopoulos
Affiliation:
Department of Neurology, Neurogenetics Unit, Eginition Hospital, University of Athens, Athens, Greece
*
Dr M. Panas, Department of Neurology, Neurogenetics Unit, Eginition Hospital, University of Athens, 74 Vas. Sophias Avenue, Athens 11528, Greece. Tel: +30 210 7289404; Fax: +30 210 7216474; E-mail: mpanas@med.uoa.gr
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Abstract

Background:

Hallervorden-Spatz syndrome is characterized by pyramidal and extrapyramidal signs, and dysarthria and dementia. Psychiatric symptomatology can emerge in the course of the disorder. Mutations in the pantothenate kinase 2 gene have been found in many cases. We report a case with psychosis as sole presenting symptom.

Case:

A 41-year-old man presented with change in behavior and paranoid delusional ideation. Six months later, spasticity, extrapyramidal rigidity and dysarthria were added to the picture. Eventually, the patient became mute and wheel-chair bound. The brain magnetic resonance imaging (MRI) was consistent with iron depositions in the globus pallidus and substantia nigra.

Conclusions:

In this case, the combination of clinical and MRI findings was consistent with Hallervorden-Spatz syndrome. The combination of psychiatric and MRI findings should lead to further neurological investigation.

Keywords

Hallervorden-Spatzpantothenate kinase 2 genepsychosis
Type
Case report
Information
Acta Neuropsychiatrica , Volume 19 , Issue 2 , April 2007 , pp. 122 - 124
Copyright
Copyright © 2007 Blackwell Munksgaard

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