The idiopathic hypereosinophilic syndrome. Clinical, electrophysiological and histological study of a case.
A case of idiopathic hypereosinophilic syndrome (HES) is reported. The disease started at the age of 31, with polyneuropathic-like symptoms and disorders of the gastrointestinal tract. Hypereosinophilia and leukocytosis were observed. Instrumental investigation of the digestive tract showed esophagitis, a peptic ulceration, signs of chronic rectocolitis. Charcot-Leyden crystals were present in feces. Peripheral nervous system changes were seen with EMG and nerve conduction studies. Muscle biopsy findings were in agreement with the electrophysiological data and showed inflammatory abnormalities. Qualitative histology and teased nerve fiber studies of sural nerve indicated axonal degeneration. Protein substances derived from eosinophils degranulation may account for the disturbances observed in various systems and organs.
Marolda
M
M
Istituto di Neurologia, II Facoltà di Medicina, Università di Napoli.
Orefice
G
G
Barbieri
F
F
Striano
S
S
Camporeale
F S
FS
Santagelo
R
R
Vecchione
V
V
Catalano
A
A
Crisci
C
C
Ragno
M
M
D000328:Adult; D004802:Eosinophilia / Q000473:pathology* / Q000503:physiopathology; D005260:Female; D005767:Gastrointestinal Diseases / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D009220:Myositis / Q000473:pathology / Q000503:physiopathology; D010523:Peripheral Nervous System Diseases / Q000473:pathology / Q000503:physiopathology; D013577:Syndrome