A 38-year-old, otherwise healthy man had a 6- month history of shortness of breath that had been evaluated as an outpatient, including pulmonary function testing. He recently complained of numbness in both upper extremities, difficulty walking, dysphagia, daytime hypersomnolence, snoring, and weight gain. These symptoms had recently been investigated, including a magnetic resonance imaging of the cervical spine.

Examination

On arrival in the emergency department, he was in florid respiratory distress with tachypnoea and oxygen saturation of 88%. Pulmonary auscultation revealed decreased airflow bilaterally. He had a decreased gag bilaterally, mild motor weakness in his upper extremities, decreased sensation to pinprick from C2–T4, ataxia, diffuse hyperreflexia, and ankle clonus on neurological examination. Imaging Pulmonary function testing two weeks before admission showed a forced vital capacity of 2.75 litres (52% of predicted). Magnetic resonance imaging of the brain and spinal cord was performed. Chest X-ray on admission showed a slight decrease in lung volumes. As he was at the point of requiring mechanical ventilation, he was taken to the operating room on an emergent basis on the day of admission. A suboccipital craniectomy and C1, 2, 3 laminectomies were performed. The tonsil on the right side was protruding beneath arachnoidal bands. The bands were dissected with obvious relief of pressure. A large duraplasty was then performed.

 

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