title: Anesthesia for patients with pantothenate-kinase-associated neurodegeneration (Hallervorden-Spatz disease) - a literature review.
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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: 1University Clinic for Anesthesiology and Intensive Care Medicine, Faculty for Clinical Medicine Mannheim, Ruprecht-Karls-University Heidelberg, University Hospital Mannheim, Mannheim, Germany.
authors.forename: Jochen
authors.initials: J
authors.lastname: Hinkelbein
authors.affiliation: 1University Clinic for Anesthesiology and Intensive Care Medicine, Faculty for Clinical Medicine Mannheim, Ruprecht-Karls-University Heidelberg, University Hospital Mannheim, Mannheim, Germany.
authors.forename: Armin
authors.initials: A
authors.lastname: Kalenka
authors.affiliation: 1University Clinic for Anesthesiology and Intensive Care Medicine, Faculty for Clinical Medicine Mannheim, Ruprecht-Karls-University Heidelberg, University Hospital Mannheim, Mannheim, Germany.
authors.forename: Markus
authors.initials: M
authors.lastname: Alb