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Respiratory disturbances during sleep in syringomyelia and syringobulbia

From the Department of Clinical Neurophysiology (Drs. Nogués and Encabo, and C. Calderón) and Neurology (R. Leiguarda), Instituto de Investigaciones Neurológicas "Dr. Raúl Carrea," Buenos Aires; the Department of Pneumonology (Dr. Gené), Hospital de Clínicas "José de San Martín," Buenos Aires, Argentina; and the Department of Neurology (Dr. Benarroch), Mayo Clinic, Rochester, MN.

Address correspondence and reprint requests to Dr. Martín Nogués, Montañeses 2325, (1428) Buenos Aires, Argentina; e-mail: mnogues{at}fleni.org.ar

OBJECTIVE: To determine the frequency and types of abnormalities of respiratory control during sleep in syringomyelia and syringobulbia and to provide a basis to predict patients at risk of sudden death.

METHODS: Thirty patients (15 male and 15 female; mean age 39.0 ± 12.6 years) with communicating syringomyelia were divided into two groups: those with evidence of syringobulbia (17 patients) and those without compromise of the medulla or syringomyelia (13 patients). Patients were studied with pulmonary function studies and polysomnography. Respiratory center sensitivity to CO₂ (rebreathing technique) was measured in 9 patients.

RESULTS: Severely affected patients had mild-to-moderate restriction and individual patients had bilateral diaphragmatic or vocal cord palsy, abnormal respiratory rhythm, prolonged inspiratory time, or an abnormal respiratory response to CO₂. Very prolonged central, obstructive, and mixed sleep apneas with low O₂ saturation values and a fixed heart rate were recorded in most patients with syringobulbia. Five patients developed severe respiratory complications and died during a follow-up period of 10 years. Respiratory abnormalities failed to correlate with syrinx size.

CONCLUSIONS: Severe abnormalities in respiratory rhythm generation during sleep occur in patients with syringobulbia. The respiratory disturbances are not due to muscle weakness and they are not correlated with the size of the cavity. The combination of dysphagia and dysphonia in patients with longstanding syringomyelia and syringobulbia predicted likelihood of respiratory disturbances during sleep.

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