Cerebellar degeneration and hearing loss in a patient with idiopathic myenteric ganglionitis

Guido Basilisco, Carlotta Gebbia, Maddalena Peracchi, Pietro Velio, Dario Conte, Nereo Bresolin, Eduardo Nobile-Orazio

Research output: Contribution to journalArticlepeer-review

Abstract

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.

Original languageEnglish
Pages (from-to)449-452
Number of pages4
JournalEuropean Journal of Gastroenterology and Hepatology
Volume17
Issue number4
DOIs
Publication statusPublished - Apr 2005

Keywords

  • Anti-Hu antibody
  • Intestinal pseudo-obstruction
  • Megacolon

ASJC Scopus subject areas

  • Gastroenterology

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