Acute respiratory failure as the first sign of Arnold-Chiari malformation associated with syringomyelia

Abstract

We report a rare case of acute respiratory failure in a previously asymptomatic patient showing clinical signs of inferior cranial nerve palsy together with weakness and muscular atrophy of the upper limbs. Magnetic resonance imaging revealed Arnold-Chiari malformation associated with platybasia, basilar impression, syringomyelia and Klippel-Feil syndrome. Episodes of apnoea required tracheostomy and recurred upon tentative closure of the tracheostome, but remitted upon decompression of the posterior fossa. This case involved both obstructive mechanisms and dysfunction of the respiratory centre. Patients with respiratory failure not explained by pulmonary pathology should be checked for underlying neurological disease.

Protracted respiratory failure in a case of global spinal syringomyelia and Chiari malformation following administration of diazepam: illustrative case

“…24,25 There have been numerous reported cases of patients with syringomyelia and ACM1 developing acute RF, with a number of these patients reported to continue experiencing hypercapnic RF weeks or even years after their posterior fossa decompression. 6,14,26,27 Despite this, this case is still an extremely rare phenomenon in that there has been only one other reported case in which a patient with a CM developed hypercapnic RF that was incidentally induced through administered medications, although the patient in that case had a subarachnoid block of morphine and experienced RF that gradually resolved over the course of 36 hours. 28 To our knowledge, this is the only incidental case of sudden, chronic protracted hypercapnic RF in a patient with syringomyelia and ACM1 with no known history of respiratory distress after administration of an anxiolytic.…”

Coronavirus disease 2019–associated persistent cough and Chiari malformation type I resulting in acute respiratory failure: illustrative case

“…36 Although a number of conditions have been associated with acute exacerbations of symptomatology in CM-I, acute respiratory failure has been described in the literature fewer than five times and never in association with COVID-19. 19,23,24 The patient reported herein was otherwise asymptomatic until she contracted COVID-19 with constant, severe coughing fits. Several weeks later, she began waking up one to two times per night confused and gasping for breath, which worsened over the following months and progressed with additional symptoms of gait instability, blurry vision, dysphagia, and vertigo.…”

“…12,16,[20][21][22] Although SDB and other stressors have been associated with respiratory distress in CM-I, respiratory failure as the initial presentation of CM-I has seldom been described, with only 4 known cases reported in the literature. 19,23,24 To date, coronavirus disease 2019 (COVID-19) infection has numbered over 768 million cases worldwide and 6.9 million deaths primarily due to severe hypoxemia and acute respiratory distress syndrome. 25,26 Infection with COVID-19 as the inciting event in a patient with CM-I, leading to SDB and loss of the CO 2 drive to breathe during sleep, causing acute respiratory failure, has not been reported in the literature.…”