Anaesthetic Management of Parturient with Motor Neuron Disease for Caesarean Delivery: Case Report

Motor neuron disease (MND) is a progressive neurodegenerative disease of unknown aetiology, which results in weakness of muscles of phonation, ambulation, deglutition and respiration. It has low prevalence but high disability and fatality. Death often follows respiratory failure. There is no known cure. It is extremely rare in pregnancy, but when it occurs, the respiratory compromise worsens and anaesthesia becomes challenging. There is no consensus yet on the choice of anaesthesia. We present a 29-year-old, 32-week parturient with MND who presented with severe dyspnea, orthopnea and was diagnosed with severe respiratory distress. She was admitted into ICU and given anticoagulant and steroid therapy. Sequential mini-dose combined spinal-epidural anaesthesia (CSE) was given and surgery performed in the semi-sitting position successfully. There was no deterioration of symptoms post anaesthesia. We conclude that CSE is adequate to manage parturient with MND who has severe orthopnea following respiratory muscle paresis.

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042 Respiratory function and cognitive profile in motor neuron disease

Journal of Neurology Neurosurgery & Psychiatry ◽

10.1136/jnnp-2019-anzan.38 ◽

2019 ◽

Vol 90 (e7) ◽

pp. A14.2-A14

Author(s):

William Huynh ◽

Lara E Sharplin ◽

Jashelle Caga ◽

Elizabeth Highton-Williamson ◽

Matthew C Kiernan

Keyword(s):

Cognitive Impairment ◽

Cognitive Function ◽

Motor Neuron ◽

Motor Neuron Disease ◽

Respiratory Function ◽

Normal Function ◽

Cognitive Testing ◽

Respiratory Compromise ◽

Respiratory Dysfunction ◽

Non Invasive Ventilation

IntroductionMotor neuron disease (MND) is increasingly recognised as a multisystems disorder with 30–50% of patients having mild to moderate cognitive impairment. Mechanisms of cognitive dysfunction in MND are multifactorial but chronic hypoxia secondary to respiratory dysfunction may contribute to cognitive decline in patients.ObjectivesThe current study aimed to identify the relationship between respiratory function in MND patients and the presence and degree of cognitive impairment.MethodsMND patients were prospectively recruited from a multidisciplinary MND clinic. Patients meeting the criteria for frontotemporal dementia were excluded. Baseline clinical assessments including respiratory function as assessed by spirometry were recorded with FVC ≤ 75% considered to have reduced respiratory function. Cognitive testing was performed utilising the Addenbrooke’s Cognitive Examination (ACE).ResultsFrom a cohort of 100 MND patients 48% were categorised as having impaired respiratory function whilst 52% had normal function. Compared to the group with normal respiratory function (ACE: 86.83±1.5), patients with respiratory dysfunction had significantly reduced cognitive function (ACE: 90.68±0.89, P=0.025). Subscores demonstrated significant differences between the groups with respect to domains in memory, attention with a trend observed in fluency. There was a significant correlation between FVC and ACE scores as well as between FVC and memory and fluency subscores (P<0.01).ConclusionMND patients with respiratory compromise were more likely to develop reduced cognitive function. In addition to improving physical function, it remains plausible that non-invasive ventilation may alter the progression of cognitive impairment in MND patients, thereby potentially improving their overall quality of life and carer burden.

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P027 The clinical role and outcomes of non-invasive ventilation in motor neuron disease: An Australian tertiary hospital experience

SLEEP Advances ◽

10.1093/sleepadvances/zpab014.075 ◽

2021 ◽

Vol 2 (Supplement_1) ◽

pp. A30-A30

Author(s):

A Cruickshank ◽

D Curtin

Keyword(s):

Motor Neuron ◽

Motor Neuron Disease ◽

Muscle Weakness ◽

Respiratory Muscle ◽

Tertiary Hospital ◽

Invasive Ventilation ◽

Respiratory Muscle Weakness ◽

Non Invasive ◽

Non Invasive Ventilation ◽

Average Survival

Abstract Introduction In motor neuron disease (MND), non-invasive ventilation (NIV) in patients who develop respiratory muscle weakness improves both quality of life and survival. This study aimed to evaluate the current practice and outcomes of NIV use in MND patients in an Australian tertiary hospital. Methods The medical records of all MND patients who attended a specialist multidisciplinary clinic requiring NIV treatment between January 2015 and January 2020 were retrospectively analysed. Progress to date: Forty-five patients have been analysed with a mean age at time of NIV commencement of 61±10(SD) years, 67% were male, 33% were current or past smokers and 7% had OSA with previous CPAP use. MND onset was limb in 58%, bulbar in 36% and respiratory muscle in 7%. Riluzole was prescribed in 47% and PEG/RIG insertion performed in 47%. At time of NIV commencement, 82% were symptomatic and 47% hypercapnic. No patient was commenced based on functional testing alone. NIV adherence (usage ≥4hours/night) was observed in 80%. NIV non-adherence was associated with bulbar subtype (p=0.02) and empirical NIV initiation (p<0.01) on univariate analysis. Average survival from NIV commencement was 17±22(SD)months. Average survival on NIV in adherent patients was 19±24(SD)months and non-adherent patients was 2±2(SD)months, although this did not reach statistical significance (p=0.1). Intended outcome & impact Overall clinical practice and outcomes of NIV use in this study is comparable to literature. The factors influencing NIV tolerance and adherence require further study to optimise outcomes in MND patients with respiratory muscle weakness.

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The Benefit of Non-invasive Ventilation in Motor Neuron Disease

The Open Respiratory Medicine Journal ◽

10.2174/1874306402014010053 ◽

2020 ◽

Vol 14 (1) ◽

pp. 53-61

Author(s):

Laura J. Walsh ◽

Desmond M. Murphy

Keyword(s):

Quality Of Life ◽

Motor Neuron ◽

Motor Neuron Disease ◽

Respiratory Muscle ◽

Survival Advantage ◽

Inspiratory Pressure ◽

Invasive Ventilation ◽

Non Invasive ◽

Non Invasive Ventilation

Background: Motor Neuron Disease (MND) is a progressive neurodegenerative disorder leading to respiratory muscle weakness with dyspnoea, morning headaches, orthopnoea, poor concentration, unrefreshing sleep, fatigue and daytime somnolence. Respiratory failure is the primary cause of death in those with MND. Methods: Although guidelines suggest the use of non-invasive ventilation (NIV) in MND, there lacks clear guidance as to when is the optimal time to initiate NIV and which markers of respiratory muscle decline are the best predictors of prognosis. There have been a number of studies that have found a significant survival advantage to the use of NIV in MND. Similarly, in quality-of-life questionnaires, those treated with NIV tend to perform better and maintain a better quality of life for longer. Furthermore, studies also suggest that improved compliance and greater tolerance of NIV confer a survival advantage. Results and Discussion: Forced Vital Capacity (FVC) has traditionally been the main pulmonary function test to determine the respiratory function in those with MND; however, FVC may not be entirely reflective of early respiratory muscle dysfunction. Evidence suggests that sniff nasal inspiratory pressure and maximum mouth inspiratory pressure may be better indicators of early respiratory muscle decline. These measures have been shown to be easier to perform later in the disease, in patients with bulbar onset disease, and may indeed be better prognostic indicators. Conclusion: Despite ongoing research, there remains a paucity of randomised controlled data in this area. This review aims to summarise the evidence to date on these topics.

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