Author: Bernadette Basilico; Reviewer: Alda Rocca
The risk of complicated COVID-19 infection is increased in certain groups, including those over 70 years, with chronic diseases or a weakened immune system. Here, it is reported a guidance on the risks from a variety of neurological conditions and treatments, with particular focus on immunosuppressed patients and people with dementia. The importance of the spread of telemedicine is also discussed.
The primary action to constrain the spread of the virus is social isolation. Given this public health principle, and the shortage of personal protective equipment during the global pandemic, it is strongly encouraged the use of remote patient health care, thus avoiding non-essential hospital visits during the COVID-19 pandemic.
At the moment, there is a wide spectrum of telehealth services available, including real time interactive video communications, telephone communication, email, text messages, remote monitoring of patient data (i.e. images or physiological parameters).
When telemedicine is not applicable, steps to reduce hospital visits like home delivery of medications, delaying follow-up MRI scans in stable patients and reducing the frequency of routine laboratory monitoring should also be considered.
Dementia care during COVID-19
People living with dementia have limited access to accurate information and facts about the COVID-19 pandemic. They might have difficulties in remembering safeguard procedures, such as wearing masks, or in understanding the public health information issued to them. Ignoring the warnings and lacking sufficient self-quarantine measures could expose them to higher chance of infection.
In addition to physical protection from virus infection, psychosocial support should be delivered. For instance, service teams could support behavioural management through telephone hotlines; psychological counsellors could provide online consultation for carers at home and in nursing homes.
General advice related to immune therapies
Immunosuppressive treatments could lead to alterations in lymphocyte number, trafficking, proliferation and function, with an increased risk of infections, including viral infections and respiratory infections. Thus, it is reasonable to hypothesize that these therapies may predispose to a greater risk of SARS-CoV-2 infection, and potentially more severe infection.
Overall, the patients should follow the following recommendation:
- Do not stop or alter medication without prior discussion with the neurologist
- Patients on immunosuppressive medications should practice extra-vigilant social distancing, including avoiding public gatherings/crowds, avoiding crowded public transport and where possible use alternatives to face-to-face consultations (i.e. telemedicine)
- Therapies with immune depleting properties or primary immune suppressive agents (especially Ocrelizumab, Rituximab, Cladribine, Alemtuzumab, Mitoxantrone) could increase the risk of infections. In older patients and patients with comorbidity (cardiovascular, pulmonary), treatment initiation should be delayed (if disease activity allows)
- Any combination of immunosuppressive drugs with a daily prednisolone dose of 20 mg or above is considered high risk, thus self-isolation is recommended
- Infliximab/Rituximab/Ocrelizumab. These infusions moderately increase the risk of viral infections, so individuals may be more prone to SARS-CoV-2 and its complications. Treatment benefits should be evaluated, when possible, delay the treatment or consider alternative option
- The indiscriminate use of intravenous (IV) immunoglobulin should be avoided. Plasma exchange and IV immunoglobulin should be reserved for patients with acute exacerbations or for maintenance therapies, with extra precautions
Estimation of the risk associated with COVID-19 for patients affected by neurological diseases
Patients with pathological conditions that do not affect their swallowing, breathing muscles or the immune system are not considered at increased risk for COVID-19. The risk has been classified into low, moderate and high. Obviously, the risk to develop a more severe COVID-19 infection increase when the patient presents more than one risk factor (e.g. multi-system disorders, immunodeficiency resulting from therapies).
Overall, the patients should follow the following recommendation:
- Social distancing for all people with any neurological condition, their carers and family
- Frequent hand-washing with soap and water or an alcohol-based hand rub
- Self-isolation only for people in the high risk category
People presenting a neurological condition with low or moderate risk might be considered high risk if they have additional risk associated with other conditions affecting the lungs, heart, kidneys, etc.
Risk factors for specific neurological diseases
In general, in case of active disease do not stop medication as the risk of a flare exceeds the risk of the medication itself.
|Neurological disease||Risk from COVID-19||Risk factors||Advice|
|Amyloid neuropathy||H/M||Autonomic neuropathy, cardiac involvement|
|Cerebral vasculitis||H/M||Immunosuppression, co-morbidities, bulbar or respiratory weakness|
|Chronic Inflammatory Demyelinating Polyneuropathy||M/L||Respiratory/diaphragmatic involvement, immunosuppression|
|Cognitive Disorders||H/M/L||Bulbar difficulties|
|Complex Epilepsy||L||Bulbar or respiratory muscle weakness, fever-sensitive epilepsies, Rasmussen’s encephalitis on immunosuppressive medication|
|Congenital muscular dystrophy||H||FVC<60%, NIV, weak cough, cardiomyopathy||-Supply cardiac medications -Do not stop ACE inhibitors or beta blockers|
|Congenital myasthenia with previous respiratory involvement or needing nocturnal ventilation||H|
|Congenital Myopathy||H/M||FVC<60%, NIV and weak cough|
|Fast channel congenital myasthenic syndrome or Congenital myasthenic syndrome with respiratory crises that needing hospital admission within the last 10 years||H/M|
|Fatty acid oxidation disorder||H||Risk of acute rhabdomyolysis with fever, infection, fasting||-Ensure emergency regimen is in place -May need IV dextrose|
|Genetic Degenerative/Ataxic Syndromes||M||Bulbar weakness|
|Glycogen Storage Diseases||H/M/L||FVC<60%, glycolytic disorders (GSDV, VII, XIII), diabetes and ischaemic heart disease, BiPAP ventilation|
|Guillain-Barre Syndrome||M||Ventilation, neuromuscular respiratory weakness|
|Hereditary spastic paraparesis||M/L|
|Idiopathic Intracranial Hypertension||L||BMI>40|
|Inherited neuropathies||None/H||Kyphoscoliosis and/or neuromuscular chest/diaphragm weakness|
|Lambert Eaton myasthenic syndrome||H/M|
|Limb girdle muscular dystrophies||H/M||FVC<60%, NIV, weak cough, cardiomyopathy||-Supply cardiac medications -Do not stop ACE inhibitors or beta blockers|
|Mitochondrial disease||H/M||Risk of decompensation during infection, risk of cardiomyopathy. Diabetes, immunosuppression||Aggressively treat seizure activity during episodes of decompensation or stroke-like episodes|
|Motor neuron disease||H||Ventilator support|
|Movement Disorders (e.g. Parkinson)||H/M/L||Co-morbidities, bulbar or respiratory difficulties|
|Multifocal motor neuropathy||H/L||Cyclophosphamide treatment|
|Multiple sclerosis||M/H||Immunosuppression or swallowing/breathing difficulties||– Avoid autologous haematopoietic stem cell transplantation, alemtuzumab or cladribine treatments (higher risk of viral infections in the 3-6 months after treatment) -Drugs associated with moderate risk of infection: ocrelizumab, fingolimod -Safe therapies: interferon beta 1a, interferon beta 1b, glatiramer, teriflunomide, dimethyl fumarate -Consider to stop treatments in patients hospitalised with severe or complicated COVID-19 infection. Treatment can be restarted after 4 weeks, or when symptoms are fully resolved|
|Myasthenia Gravis||H/M||Immunosuppression, respiratory muscle weakness||Higher dose of steroids may be necessary. Do not increase too rapidly because of risk of increasing muscle weakness|
|Myositis, polymyositis||H/M||If active disease, increased risk due to respiratory muscle weakness, co- existing interstitial lung disease and other overlap connective tissue disorders||-May be on immunosuppression -Do not stop steroids|
|Myotonic dystrophy||M||Poor cough, risk of chest infection, risk of choking with coughing|
|Neuromyelitis optica spectrum disorder (NMOSD)||H/M||Immunosuppression, bulbar or respiratory weakness||-Relapses may be devastating. Patients should be encouraged to continue therapies for attack-prevention including corticosteroids, azathioprine, mycophenolate mofetil, rituximab, tocilizumab and eculizumab -If there is a clinical need to stop or delay treatment, moderate dose corticosteroids (e.g. prednisolone 20mg) can be used to prevent relapses in the short to medium term|
|Ocular myasthenia or well controlled adult congenital myasthenia without respiratory involvement in last 10 years and normal sleep studies||L|
|POEMS syndrome||H/M||Immunosuppression, neuromuscular weakness|
|Spinal Muscular Atrophy||H/M||Type 2 SMA is high risk Type 3 is high risk if FVC<60% or using BiPAP (usually nonambulant)|
|Stroke||H/M/L||Co-morbidities||Aerosol-generating medical procedures (i.e. nebulization of medications, CPAP, BiPAP, and nasal High Flow therapy) should be avoided whenever possible|
|Traumatic Brain Injury||M/L||Bulbar difficulties|
|Vasculitis (any)||H/M||Immunosuppression, lung/renal involvement further increases risk|
|X-linked Muscular dystrophies (Duchenne/Becker)||H||FVC<60%, non-invasive ventilation, weak cough or cardiomyopathy||-Do not stop steroids, cardiac medications, ACE Inhibitors or beta blockers -If moderately sick (more than a simple cold) double the steroid dose for 3 days, then back to original dose over 5 days -For severe illness, give systemic steroids|
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