Central Neurogenic Respiratory Failure: A Challenging Diagnosis

1990

We present two patients without primary cardiopulmonary disease. wbo developed adult respiratory distress syndrome in response to lesions of the central nervous system. Clinical and laboratory investigations are described in detail and pertinent literature is reviewed.

QJM, 1996

Twenty-nine patients with a neuronopathic or neuropathic disorder were referred for assessment of respiratory insufficiency between 1978 and 1994. Diagnoses included spinal muscular atrophy (6), chronic idiopathic demyelinating neuropathy (4), Vialetto-van Laere syndrome (3), hereditary motor and sensory neuropathy (3) and a miscellaneous group (5). We also describe seven patients with Guillain-Barre syndrome (GBS) who required longterm ventilatory support for over 6 months to 7 years after the initial illness. Respiratory insufficiency occurred as a consequence of respiratory muscle weakness, impaired bulbar function and restrictive lung defects. In some groups presentation was with progressive nocturnal hypoventilation culminating in acute respiratory failure. Five patients with GBS or chronic idiopathic demyelinating neuropathy were weaned from ventilatory support up to 18 months after the initial illness. The remaining 24 patients required continuous or nocturnal ventilatory support using intermittent positive-pressure ventilation (13), negative pressure ventilation (4), nasal-mask-delivered intermittent positive-pressure ventilation (4), nasal-maskdelivered continuous positive-pressure ventilation (3), mouthpiece-assisted ventilation by day (2) and rocking bed (1). None have been weaned from support after a period of ventilation ranging from one month to 10 years. Eight patients have subsequently died.

Neurology, 2021

The authors report no targeted funding Disclosures: B.Parayil Sankaran reports receipt of the clinical research fellowship from Mito Foundation Australia. S.B Wortmann reports no disclosures relevant to the manuscript.M.A Willemsen reports no disclosures relevant to the manuscript. S.Balalsubramaniam reports no disclosures relevant to the manuscript.

Neurology, 2021

The authors report no targeted funding Disclosures: B.Parayil Sankaran reports receipt of the clinical research fellowship from Mito Foundation Australia. S.B Wortmann reports no disclosures relevant to the manuscript.M.A Willemsen reports no disclosures relevant to the manuscript. S.Balalsubramaniam reports no disclosures relevant to the manuscript.

International Journal of General Medicine, 2012

Patients with certain neurological diseases are at increased risk of developing chest infections as well as respiratory failure due to muscular weakness. In particular, patients with certain neuromuscular disorders are at higher risk. These conditions are often associated with sleep disordered breathing. It is important to identify patients at risk of respiratory complications early in the course of their disease, although patients with neuromuscular disorders often present in the acute setting with respiratory involvement. This review of the respiratory complications of neurological disorders, with a particular focus on neuromuscular disorders, explores why this happens and looks at how to recognize, investigate, and manage these patients effectively.

BMC Pulmonary Medicine, 2012

Background: Right-to-left shunt (RLS) may be the cause of marked hypoxemia, a respiratory insufficiency which is usually difficult to diagnose by respiratory physicians as it develops in the absence of an intrinsic lung disease. Case presentation: We report a case of RLS in a patient with a hepatopulmonary syndrome caused by chronic autoimmune cholangitis. RLS was suspected clinically by physical examination and by standard CT imaging and MIP reconstruction of the pulmonary vascular bed. Repeated previous transthoracic echocardiography (TTE) studies did not reveal shunts or any cardiac defect. The final diagnosis was made by means of a minimally invasive transcranial Doppler examination with the use of saline agitated with 0.5 ml of patient's blood as contrast solution. Conclusions: Transcranial Colour-Coded Duplex Sonography (TCCS) with saline contrast medium injection is described to have a higher sensitivity than TTE and comparable to transesophageal echocardiography (TEE) in RLS diagnosis. The collaboration of neurologists in diagnosing respiratory insufficiency is very important as the examination is simple, well tolerated in comparison with the discomfort associated with transesophageal echocardiography, and minimally invasive in comparison with angiography, which is the last diagnostic procedure in this clinical scenario. In order to confirm RLS, TCCS with blood-saline contrast medium injection should be performed for the diagnosis of chronic hypoxemia for which causes are not detected with routine clinical examinations.

Archives of Neurology, 2010

Objective: To identify the spectrum of causes, analyze the usefulness of diagnostic tests, and recognize prognostic factors in patients with acute neuromuscular respiratory failure.

Open Medicine, 2014

We report the case of a 73 year old woman who presented for progressive dyspnea. Her medical history included thyroidectomy 15 years ago, myocardial infarction, recurrent paroxysmal atrial fibrillation and femoral fracture two weeks previously, conservatively treated. Physical examination revealed absent breath sounds in the left hemithorax, up to the apex, and crackles in the right hemithorax. The acid-base balance showed acute hypoxemic respiratory failure. The chest X-Ray revealed left diaphragmatic paralysis. Thoracic CT-scan was performed, which excluded the pulmonary embolism and revealed left diaphragmatic relaxation, ascension of the splenic angle of the colon, stomach and spleen up to the projection of left lung hilum, and right postero-basal alveolar condensation process. Diaphragm dysfunction can be caused by various disorders, including phrenic paralysis. This pathology should be considered in the differential diagnosis of acute respiratory failure.

Journal of Intensive Care Medicine, 2000