Acquired Ondine's curse: case report

Abstract

-We report and discuss the case of a 55-year old man who presented a history of stroke as well as chronic obstructive pulmonary disease. When admitted into the emergency room, he was diagnosed with a vertebro-basilar syndrome. A brain MRI showed a hyperintense area in the lower right brainstem laterally within the medulla, which corresponds to the area of the pathways descending from the autonomic breathing control center. During hospitalization, the patient had several episodes of prolonged apnea, mainly when asleep, having often to be "reminded" to breath. A tracheostomy was then performed with the patient under mechanical ventilation. Treatment with medroxyprogesterone, fluoxetine and acetazolamide was also started. He was discharged after 64 days breathing environmental air with no apparent episodes of apnea. He returned to the emergency room in the following day with a clinical picture of aspiration bronchopneumonia, followed by septic shock and death. Conclusion: the Ondine´s curse is one of the posterior stroke´s presentation characterized by loss of automatic breathing and for the unpredictability of clinical evolution and prognosis. Such a syndrome has rarely been reported in adults and the diagnostic criteria are not consensual in the reviewed literature.Thus any dignostic confirmation should be flexible.There are many therapeutic symptomatic options in such cases, ranging from pharmacologic approach, use of bilevel positive airway pressure and implantation of diaphragmatic pacemaker.KEY WORDS: Ondine´s curse, posterior stroke, loss of automatic breathing. Maldição de Ondina adquirida: relato de casoRESUMO -Relatamos e discutimos o caso de um paciente de 55 anos de idade que apresentara história de acidente vascular cerebral e doença broncopulmonar obstrutiva crônica. Quando admitido no setor de emergência, foi diagnosticado como vítima de sín-drome vértebro-basilar.A ressonância magnética mostrou hiperintensidade no tronco inferior à direita ao nível do bulbo, o que corresponde a área das vias descendentes do centro do controle respiratório automático. Durante a hospitalização o paciente teve vários episódios de apnéia prolongada, especialmente durante o sono, tendo sido freqüentemente "lembrado" a respirar pela equipe médica e enfermagem. A traqueostomia foi então realizada, com o paciente inicialmente mantido em ventilação mecânica. Tratamento com medroxiprogesterona, fluoxetina e acetazolamida foram também iniciados de forma escalonada. Foi liberado após 64 dias, respirando ao ar ambiente sem epsiódios aparentes de apnéia. Retornou à emergência no dia seguinte com quadro clíni-co compatível com broncopneumonia aspirativa, seguida por choque séptico e morte. Conclusão: a Maldição de Ondina é uma das formas de apresentação de um acidente vascular encefálico de circulação posterior, marcada por perda da respiração automáti-ca e pela imprevisibilidade da evolução clínica e prognóstico. Tal situação foi raramente descrita em adultos e os critérios diagnós-ticos não são um consenso na literatura...

Successful delivery of adjuvant external beam radiotherapy for ependymoma in a patient with Ondine׳s curse

“…Although Ondine's curse is most commonly described as a congenital syndrome, rare cases of acquired CAH caused by lesions involving the ventrolateral descending medullocervical pathways have been reported. 2,3,5,6 Chemosensitive respiratory neurons initiating inspiration and expiration have been localized to the medulla. We postulate that, in our case, compression from residual tumor and subgaleal effusion, and exacerbation by supine positioning and pressure from mask immobilization, interfered with dorsal respiratory group functions responsible for ventilatory responses to hypercapnea and hypoxemia, causing the patient to go into respiratory arrest.…”

“…1,2 The eponym, Ondine's curse, was first coined in 1962 to describe the symptoms of 3 patients whose respiratory centers failed to perform automatically after upper bilateral cervical cordotomy for pain relief. The term stems from the German legend Der Ritter von Staufenberg where "the water nymph, Ondine, having been jilted by her mortal husband, took from him all automatic functions, requiring him to remember to breathe.…”

Ondine’s Curse with Accompanying Trigeminal and Glossopharyngeal Neuralgia Secondary to Medullary Telangiectasia

“…Few cases have been reported of patients with Electrodes are seen on both views tethered to the right and left side of the pericardium. Circular device receivers are seen embedded in subcutaneous pockets on each side of the lower chest medullary tumors, acute vertebrobasilar strokes, as well as other structural lesions presenting with hypoventilation [1,2,[6][7][8]. Most, but not all cases in adults do have corresponding bulbar lesions on MRI.…”

Lateral medullary syndrome with Ondine's curse

“…Two types have been described: congenital and acquired CAH. The congenital CAH occurs at birth and is extremely rare [3] while the acquired CAH can result from medulla ischemia, hemorrhage, tumor, infection, demyelinating diseases, trauma, some mitochondrial diseases, degenerative diseases or anoxic-ischemic damage [2,4].…”