Neurologic complications of Churg-Strauss syndrome--a prospective monocentric study.
BACKGROUND
Churg-Strauss syndrome (CSS) is a rare systemic vasculitis. Case series with a focus on neurologic involvement are not common. With this study, we intended to evaluate the frequency and types of neurologic manifestations and complications at time of diagnosis and during follow-up of patients with CSS.


METHODS
In this monocentric study, consecutive patients of our hospital with first diagnosis of CSS based on the criteria of the American College of Rheumatology were included between 2001 and 2007. Each patient underwent a periodic follow-up with clinical and electrophysiologic examination. Data were obtained prospectively.


RESULTS
Fourteen patients were included. All patients had a hypereosinophilia and a history of asthma. Twelve of 14 patients had a neurologic involvement, mainly as an acute or subacute multiplex mononeuropathy (eight patients) or an axonal polyneuropathy (three patients). Three patients suffered from a neuropathy of cranial nerves, and two patients had a cerebral infarct. Mean follow-up period was 31 months. With immunosuppressive therapy, 13 patients had no additional neurologic complications, one patient suffered from a cerebral infarct. Initial neurologic symptoms as a result of peripheral neuropathy improved, but sequelae of axonal damage were persistently detectable.


CONCLUSIONS
Even at time of diagnosis of a CSS, neurologic manifestations are common, especially as a multiplex mononeuropathy. With a consequent immunosuppressive therapy, new neurologic complications can be avoided for the most part.
Wolf
J
J
Department of Neurology, Klinikum der Stadt Ludwigshafen, Bremserstrasse 79, Ludwigshafen, Germany. Wolfj@klilu.de
Bergner
R
R
Mutallib
S
S
Buggle
F
F
Grau
A J
AJ
D000208:Acute Disease; D000328:Adult; D000368:Aged; D020520:Brain Infarction / Q000150:complications* / Q000188:drug therapy; D015267:Churg-Strauss Syndrome / Q000150:complications* / Q000188:drug therapy; D003389:Cranial Nerve Diseases / Q000150:complications* / Q000188:drug therapy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D020422:Mononeuropathies / Q000150:complications / Q000188:drug therapy; D010523:Peripheral Nervous System Diseases / Q000150:complications* / Q000188:drug therapy; D011115:Polyneuropathies / Q000150:complications / Q000188:drug therapy; D011446:Prospective Studies
D007166:Immunosuppressive Agents