Long-term outcome of basilar stenosis in Erdheim-Chester disease: A case report.
BACKGROUND
Erdheim-Chester disease (ECD) is a rare form of non-Langerhans cell histiocytosis. This inflammatory myeloid neoplasm is frequently complicated by neurological symptoms, but stroke is an exceptional manifestation of this disease.


METHODS
We report the case of a 59-year-old woman who presented a vertebrobasilar stroke secondary to infiltration and severe stenosis of the basilar artery, improved after interferon-alpha therapy. We performed a review of the relevant literature and reported the few other cases described.


RESULTS
With our patient, we have found only 7 observations of cerebrovascular disorder in ECD. Most of them had supravascular arteries involvement.


CONCLUSIONS
Stroke is a rare treatable and potentially reversible complication of ECD. The pathophysiological processes explaining stroke in this disease are uncertain, but periarterial stenosis of cerebral arteries may be a mechanism.
Department of Neurology, CHU of Poitiers, University of Poitiers, Poitiers Department of Internal Medicine and French reference Center for Rare Auto-immune and Systemic Diseases, Assistance Publique-Hôpitaux de Paris (AP-HP), Pitié-Salpêtrière Hospital Université Pierre et Marie Curie, UPMC University Paris 6, Paris Department of Pathology, CHU of Poitiers, University of Poitiers, Poitiers, France.
Stéphane
S
Mathis
Gaëlle
G
Godenèche
Julien
J
Haroche
Serge
S
Milin
Adrien
A
Julian
Aline
A
Berthomet
Clément
C
Baron
Paola
P
Palazzo
Jean-Philippe
JP
Neau
D031249:Erdheim-Chester Disease / Q000150:complications* / Q000188:drug therapy; D005260:Female; D006801:Humans; D016898:Interferon-alpha / Q000627:therapeutic use; D008875:Middle Aged; D020521:Stroke / Q000209:etiology*; D013997:Time Factors; D014715:Vertebrobasilar Insufficiency / Q000209:etiology*
D016898:Interferon-alpha