title: Neurological disorders in patients with bullous pemphigoid.
abstract: BACKGROUND
  Unexpected cases of bullous pemphigoid (BP) have been reported in adult patients with various neurological disorders suggesting a possible relationship between these diseases.
  
  
  OBJECTIVE
  (1) To determine the prevalence and types of neurological disorders in patients with BP, (2) to assess patients' functional impairment, and (3) to compare the clinical and biological findings as well as prognosis of BP patients presenting with or without neurological disorders.
  
  
  METHODS
  BP patients with neurological disorders were selected in a series of 341 consecutive BP patients treated in 20 French Dermatology Departments. Functional impairment was prospectively assessed using the Karnofsky score which is a measure of patients' general condition.
  
  
  RESULTS
  At least one neurological disorder was present in 123 of the 341 BP patients (36%). They primarily consisted of dementia (n = 68; 20%; 95% CI: 16-25%), cerebral stroke (n = 52; 15%; 95% CI: 4-19%), and/or Parkinson's disease or parkinsonism (n = 32; 9%; 95% CI: 7-13%). BP patients with neurological disease were older than patients without neurological disease (83.8 +/- 7.5 years vs. 79.3 +/- 10.3 years, p < 10(-4)). They also had a lower Karnofsky score (47 +/- 19% vs. 74 +/- 20%, p < 10(-4)). One-year overall survival rates of the two groups were 50.8% (95% CI: 41.8-59.7) and 78.7% (95% CI: 73.0-84.2), respectively (p < 10(-4)). In contrast, the number of bullae and main biological features at baseline were not different between the two groups of patients.
  
  
  CONCLUSIONS
  This study demonstrated a high frequency of neurological disorders, particularly dementia, in BP patients. Most of these patients had a severe functional impairment and a poor prognosis.
authors.affiliation: Department of Dermatology, Pointe-à-Pitre University Hospital, Pointe-à-Pitre, Guadeloupe, Pointe-à-Pitre Cedex, France. nadege.cordel@chu-guadeloupe.fr
authors.forename: Nadège
authors.initials: N
authors.lastname: Cordel
authors.forename: Olivier
authors.initials: O
authors.lastname: Chosidow
authors.forename: Marie-France
authors.initials: MF
authors.lastname: Hellot
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Delaporte
authors.forename: Catherine
authors.initials: C
authors.lastname: Lok
authors.forename: Loïc
authors.initials: L
authors.lastname: Vaillant
authors.forename: Philippe
authors.initials: P
authors.lastname: Bernard
authors.forename: Michel
authors.initials: M
authors.lastname: D'Incan
authors.forename: Jean Claude
authors.initials: JC
authors.lastname: Roujeau
authors.forename: Pascal
authors.initials: P
authors.lastname: Joly
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006801:Humans; D017567:Karnofsky Performance Status; D008297:Male; D009422:Nervous System Diseases / Q000453:epidemiology / Q000209:etiology*; D010391:Pemphigoid, Bullous / Q000150:complications*; D015995:Prevalence; D011379:Prognosis; D011446:Prospective Studies