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Anesthesia for patients with pantothenate-kinase-associated neurodegeneration (Hallervorden-Spatz disease) – a literature review

Published online by Cambridge University Press:  24 June 2014

Jochen Hinkelbein ,
Armin Kalenka  and
Markus Alb
Jochen Hinkelbein*
Affiliation:
University Clinic for Anesthesiology and Intensive Care Medicine, Faculty for Clinical Medicine Mannheim, Ruprecht-Karls-University Heidelberg, University Hospital Mannheim, Mannheim, Germany
Armin Kalenka
Affiliation:
University Clinic for Anesthesiology and Intensive Care Medicine, Faculty for Clinical Medicine Mannheim, Ruprecht-Karls-University Heidelberg, University Hospital Mannheim, Mannheim, Germany
Markus Alb
Affiliation:
University Clinic for Anesthesiology and Intensive Care Medicine, Faculty for Clinical Medicine Mannheim, Ruprecht-Karls-University Heidelberg, University Hospital Mannheim, Mannheim, Germany
*
Jochen Hinkelbein, University Clinic for Anesthesiology and Operative Intensive Care Medicine, Faculty for Clinical Medicine Mannheim, Ruprecht-Karls-University Heidelberg, University Hospital Mannheim, Theodor-Kutzer-Ufer 1-3, 68167 Mannheim, Germany. Tel: +49.621.383.2415; Fax: +49.621.383.732740; E-mail: jochen.hinkelbein@physiologie.uni-heidelberg.de
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Abstract

Background:

Hallervorden-Spatz disease (HSD) is a rare, progressive neurodegenerative disorder; the new and preferred name for HSD is ‘pantothenate-kinase-associated neurodegeneration’ (PKAN). Other suggested names are ‘neurodegeneration with brain iron accumulation type 1’ or ‘infantile neuroaxonal dystrophy’. Patients with PKAN have many complications, which lead to numerous anesthetic management challenges. Reports concerning the anesthetic management of patients with PKAN are very limited.

Objective:

To determine the anesthetic management and techniques as well as relevant complications for patients with PKAN.

Methods:

In this study, we review previously published literature regarding the anesthesia-relevant clinical symptoms, the anesthetic management and techniques, and possible complications for this disorder.

Results:

Only four studies describing the anesthetic management and anesthetic techniques in patients with PKAN were found. Anesthesia-relevant symptoms influence the preanesthetic management (eg difficulties in articulation, dementia), the induction of anesthesia (eg oromandibular rigidity, seizures, dysphagia, aspiration) and the postoperative care (eg respiratory disability).

Conclusion:

Reports concerning the anesthetic management of patients with PKAN are very limited, possibly as a result of the rareness of the disorder. Like many other patients with neurodegenerative diseases, patients with PKAN have many anesthesia-relevant symptoms, leading to numerous anesthetic management challenges. In general, the anesthetic complications associated with PKAN are usually no different from those associated with other neurodegenerative diseases, and the management of these are usually concordant.

Keywords

anesthetic managementHallervorden-Spatz diseasepantothenate-kinase-associated disorderneurodegeneration
Type
Brief Report
Information
Acta Neuropsychiatrica , Volume 18 , Issue 3-4 , June 2006 , pp. 168 - 172
Copyright
Copyright © 2006 Blackwell Munksgaard

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