Cerebellar degeneration and hearing loss in a patient with idiopathic myenteric ganglionitis.
A 35-year-old male with an 11-year history of intestinal pseudo-obstruction associated with an idiopathic inflammatory insult of the myenteric plexus and the presence of circulating anti-Hu antibodies developed a neurological syndrome characterized by bilateral hearing loss, deteriorating balance, an unsteady gait and difficulty in estimating distances. A similar neurological syndrome has previously been described in older patients among the paraneoplasic syndromes associated with small-cell lung carcinoma and the presence of circulating anti-Hu antibodies, but never in the rare cancer-free patients with anti-Hu-associated chronic idiopathic intestinal pseudo-obstruction. The patient underwent a steroid treatment. No further episodes of functional intestinal obstruction were observed and, after an initial improvement, the neurological symptoms stabilized, leaving a permanent reduction in hearing function and an unsteady gait. The case shows that an idiopathic inflammatory insult of the myenteric plexus may precede (and perhaps lead to) central nervous system impairment in patients with anti-Hu-associated chronic idiopathic intestinal pseudo-obstruction.
Basilisco
Guido
G
Gastroenterology Unit, Department of Medical Sciences, University of Milan, IRCCS-Ospedale Maggiore, Milan, Italy. basilisc@policlinico.mi.it
Gebbia
Carlotta
C
Peracchi
Maddalena
M
Velio
Pietro
P
Conte
Dario
D
Bresolin
Nereo
N
Nobile-Orazio
Eduardo
E
D000328:Adult; D001323:Autoantibodies / Q000276:immunology; D051959:ELAV Proteins; D005684:Gait; D034381:Hearing Loss / Q000175:diagnosis / Q000209:etiology* / Q000276:immunology; D006801:Humans; D007415:Intestinal Obstruction / Q000175:diagnosis / Q000209:etiology / Q000276:immunology; D008279:Magnetic Resonance Imaging; D008297:Male; D009197:Myenteric Plexus; D009419:Nerve Tissue Proteins / Q000276:immunology; D016601:RNA-Binding Proteins / Q000276:immunology; D011843:Radiculopathy / Q000150:complications* / Q000276:immunology; D013132:Spinocerebellar Degenerations / Q000175:diagnosis / Q000209:etiology* / Q000276:immunology
D001323:Autoantibodies; D051959:ELAV Proteins; D009419:Nerve Tissue Proteins; D016601:RNA-Binding Proteins