Dysfunction in central control of breathing in some amyotrophic lateral sclerosis (ALS) patients is not adequately detected with standard evaluation for respiratory dysfunction. Nocturnal oximetry reveals periodic desaturations despite normal respiratory muscle movements. Continuous diaphragmatic electromyography has provided in vivo data consistent with impaired central control of diaphragm motor units. Current understanding of central control of breathing identifies the pre-Botzinger complex as the inspiratory rhythm generator. Animal models of pre-Botzinger complex neurodegeneration demonstrate rapid eye movement-related central sleep apneas progressing to loss of rapid eye movement sleep, also apparent in some ALS patients. Evidence supports the hypothesis that dysfunction in central control of breathing in some ALS patients may be related to pre-Botzinger complex degeneration. As the impact dysfunction of central control of breathing has on ALS becomes better defined the current standard of evaluating respiratory dysfunction in ALS patients may need updating. Muscle Nerve 56: 197-201, 2017.

A brainstem syndrome is recognizable in patients presenting with a combination of visual disturbances, incoordination, gait problems, speech and swallowing difficulties, and new-onset sleep symptomatology. Brainstem disorders of subacute onset (onset and progression with accumulation of disabling deficits in 6–12 weeks) are generally of autoimmune, infectious, inflammatory, or infiltrative neoplastic cause. An autoimmune or infectious brainstem disorder may be referred to as brainstem encephalitis or rhombencephalitis. We describe a patient with paraneoplastic autoimmune rhombencephalitis, in whom diagnostic clues included the following: diverse visual and sleep symptoms, trismus, and choking in the history; see-saw nystagmus, opsoclonus, dysarthria, jaw dystonia, and episodic laryngospasm on examination; subtle but longitudinal and nonenhancing T2 MRI abnormalities in the brainstem and upper cervical cord; and oligoclonal bands in the CSF. His movement disorder–specific neural IgG profile revealed ANNA-2 (anti-Ri) and KLHL-11-IgG. Both are biomarkers of paraneoplastic brainstem encephalitis. KLCHL-11-IgG has been reported to accompany germ cell tumors, which was found in a solitary metastasis to the left inguinal lymph node in our patient, along with an atrophic left testis. Multidisciplinary treatment (autoimmune neurology, sleep medicine, ophthalmology, and physiatry) led to significant clinical improvements. This case provides a framework for the evaluation of patients with subacute-onset brainstem syndromes and the investigation and management of those with paraneoplastic and other autoimmune diseases.

Background Extracorporeal membrane oxygenation (ECMO) in critically ill patients serves as a management option for end-stage cardiorespiratory failure in medical and surgical conditions. Patients on ECMO are at a high risk of neurologic adverse events including intracranial hemorrhage (ICH), acute ischemic stroke (AIS), seizures, diffuse cerebral edema, and hypoxic brain injury. Standard approaches to neurological monitoring for patients receiving ECMO support can be challenging for multiple reasons, including the severity of critical illness, deep sedation, and/or paralysis. This narrative literature review provides an overview of the current landscape for neurological monitoring in this population. Methods A literature search using PubMed was used to aid the understanding of the landscape of published literature in the area of neurological monitoring in ECMO patients. Results Review articles, cohort studies, case series, and individual reports were identified. A total of 73 varied manuscripts were summarized and included in this review which presents the challenges and strategies for performing neurological monitoring in this population. Conclusion Neurological monitoring in ECMO is an area of interest to many clinicians, however, the literature is limited, heterogenous, and lacks consensus on the best monitoring practices. The evidence for optimal neurological monitoring that could impact clinical decisions and functional outcomes is lacking. Additional studies are needed to identify effective measures of neurological monitoring while on ECMO. Supplementary Information The online version contains supplementary material available at 10.1007/s13760-023-02193-2.