Case report
Acute respiratory failure and sleep-disordered breathing in Arnold–Chiari malformation

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Abstract

We report on the case of a 32-year-old man who was admitted after an episode of acute respiratory failure. Clinical and laboratory investigations revealed nocturnal hypoventilation with predominately obstructive sleep apneas accompanied by lower cranial nerve palsies, cerebellar and mild pyramidal signs. Magnetic resonance imaging disclosed Arnold–Chiari type I malformation with syringomyelia. Transcranial magnetic stimulation demonstrated the integrity of the corticodiaphragmatic pathway and it was postulated that the respiratory disorder was mainly due to the severe and irreversible lower cranial nerve palsies. Two years after decompressive craniectomy, sleep disordered-breathing persisted despite no radiological evidence of brain stem compression. Nevertheless, non-invasive positive pressure ventilation (NIPPV) during sleep proved to be quite effective in the management of the patient's refractory respiratory insufficiency. In conclusion, Arnold–Chiari type I may rarely present with acute respiratory failure and sleep apneas. An electrophysiological investigation into the mechanism of the respiratory dysfunction is presented.

Introduction

Arnold–Chiari type I malformation (ACM) is a congenital disease consisting of caudal herniation of the cerebellar tonsils through the foramen magnum into the cervical canal often associated with syringomyelia. Sleep-disordered breathing, characterized by central and/or obstructive apneas, has occasionally been associated with this disease whereas acute respiratory failure (ARF) is considered a particularly rare first manifestation [1].

The present case illustrates the diagnostic challenge of unexplained ARF in a previously healthy young person, who was finally diagnosed with ACM type I. In addition, an electrophysiological investigation of the pathophysiological mechanisms underlying the patient's respiratory disorder is presented.

Section snippets

Case report

The patient, a 32-year-old man, was referred for investigation of intractable hypercapnia after ICU hospitalization for acute hypercapnic respiratory failure. The patient was a heavy smoker and had an unremarkable medical history until 3 months earlier, when progressive fatigue and morning headaches developed. He was then prescribed the noradrenergic and specific serotonergic antidepressive agent mirtazapine.

All routine lab test results were within normal limits. Arterial blood gases analysis

Discussion

Respiratory disorders are well-recognized manifestations of ACM and accompany neurological findings, such as motor deficiency, sensory loss, lower cranial nerve palsies and a cerebellar syndrome. Acute respiratory failure may be brought on by: (1) aspiration; (2) respiratory infection; and (3) central causes, including medullary compression by ectopic cerebellar tonsils. Central causes, as discussed below, seem to be the case in our patient, in whom ARF was the first and predominant clinical

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