Neurology

Multiple Sclerosis 

Risk from COVID-19:

High (H), Moderate

(M) or Low (L)

Additional comments

Multiple Sclerosis

M/H

Risk conferred by immunosuppression Occasional patients may have bulbar or respiratory weakness which confers risk.

  Muscle Diseases

Risk from coronavirus: High

(H), Moderate (M) or low (L)

Additional comments

Myositis, polymyositis

H/M

If disease active – increased risk due to respiratory muscle weakness as well as co-existing interstitial lung disease which is common in these patients and other overlap connective tissue disorders

May be on immunosuppression, do not stop steroids

X-linked Muscular dystrophies (Duchenne/Becker)

H

High risk: FVC<60%, non-invasive ventilation, weak cough or cardiomyopathy. Ensure Duchenne patients have sufficient supply of steroids and cardiac medications. Do not stop steroids, ACE Inhibitors or beta blockers. If moderately unwell (more than a simple cold) double the steroid dose for 3 days and then taper back to original dose over 5 days. For severe illness give systemic steroids.

Limb girdle muscular dystrophies

H/M

High risk: FVC<60%, NIV, weak cough, cardiomyopathy Ensure patients have supply of cardiac medications, do not stop ACE inhibitors or beta blockers.

Myotonic dystrophy

M

Poor cough, risk of chest infection, risk of choking with coughing. 

Congenital muscular dystrophy

H

High risk: patients with FVC<60%, those NIV and with weak cough. Patients with cardiomyopathy. Ensure patients have supply of cardiac medications, do not

stop ACE inhibitors or beta blockers.

Spinal Muscular Atrophy

H/M

Type 2 SMA high risk

Type 3 high risk if FVC<60% or using BiPAP (usually nonambulant)

Congenital Myopathy

H/M

High risk: patients with FVC<60%, those NIV and with weak cough.

Fatty acid oxidation disorder

H

Risk of acute rhabdomyolysis with fever, infection, fasting. Ensure emergency regimen is in place see BIMDG website for details. May need IV dextrose

Mitochondrial disease

H/M

Risk of decompensation during infection, risk of cardiomyopathy

Patients with diabetes at high risk

Patients immunosuppressed for organ transplantation at high risk

Aggressively treat seizure activity during episodes of decompensation or stroke like episodes

Glycogen Storage Diseases

H/M/L

Patients with Pompe disease with FVC<60% or on BiPAP at risk of respiratory decompensation

Patients with glycolytic disorders (GSDV, VII, XIII) should take care to avoid disease related rhabdomyolysis

Patients with secondary diabetes and ischaemic heart disease at high risk

Nerve  Diseases

Risk from coronavirus: High

(H), Moderate (M) or low (L)

Additional comments

Guillain-Barre Syndrome

M

Likely only to be at risk if acutely ventilated and whilst there is neuromuscular respiratory weakness. No additional risk of causation or recurrence known. No usual immunosuppressant.

Vasculitis (any)

H/M

Risk conferred by immunosuppression

Lung/renal involvement further increases risk

Chronic Inflammatory Demyelinating Polyneuropathy

M/L

Except in exceptional cases with

respiratory/diaphragmatic involvement, no additional risk from COVID-19 infection. Risk conferred by immunosuppression agents

POEMS syndrome

H/M

Acute disease patients with reduced transfer factors

secondary to alveolar fluid.

Risk conferred by immunosuppression from lenalidomide/dexamethasone or autografting.

Some patients with neuromuscular weakness from diaphragm weakness.

Paraproteinaemic neuropathies

H/L

No additional risk from COVID-19 if untreated

Risk conferred by immunosuppression, such as rituximab

Multifocal motor neuropathy

H/L

Intravenous immunoglobulin probably does not increase risk. Cyclophosphamide high risk.

Idiopathic neuropathies

None

Inherited neuropathies

None/H

No risk to uncomplicated Charcot-Marie-Tooth.

Patients with kyphoscoliosis and/or neuromuscular chest/diaphragm weakness may by at high risk. 

Amyloid neuropathy

   H/M

Moderate risk with Autonomic neuropathy 

High risk with cardiac involvement 

Motor Neurone Diseases

Risk from coronavirus: High

(H), Moderate (M) or Low (L)

Additional comments

Motor neurone disease

H

Patients on NIV are at higher risk from COVID-19 infection than those with no ventilator support. 

 Neuromuscular Junction Diseases

Risk from coronavirus: High

(H), Moderate (M) or Low (L)

Additional comments

Myasthenia Gravis (AChR and MuSK antibody positive and negative

H/M

Well controlled MG (M); Myasthenia gravis on immunosuppression and/or with respiratory involvement is at a greater risk from COVID-19 infection (H)

Lambert Eaton myasthenic syndrome

H/M

Fast channel congenital myasthenic syndrome or Congenital myasthenic syndrome with respiratory crises that needing hospital admission within the last 10 years

H/M

Congenital myasthenia with previous respiratory involvement or needing nocturnal ventilation 

H

Ocular myasthenia or well controlled adult congenital myasthenia without respiratory involvement in last 10 years and normal sleep studies

L

 Inflammatory or autoimmune diseases of the central nervous system

Risk from coronavirus: High

(H), Moderate (M) or low (L)

Additional comments

Neuromyelitis optica spectrum disorder

H/M

Risk conferred by immunosuppression

Occasional patients may have bulbar or respiratory weakness that confers additional risk.

Autoimmune encephalitis

H/M

Risk conferred by immunosuppression

Cerebral vasculitis

H/M

Risk conferred by immunosuppression and also by any co-morbidities [e.g. renal or lung disease]

Occasional patients may have bulbar or respiratory weakness that confers additional risk.

Anti-MOG disease

H/M

Risk conferred by immunosuppression

Neurosarcoidosis

H/M

Risk conferred by immunosuppression and also by any co-morbidities [e.g. lung disease]

Non-inflammatory diseases of the central nervous system

Risk from coronavirus: High   (H), Moderate (M) or Low (L)

Additional comments

Any disorder affecting swallowing or respiratory function

H/M/L

See specific disorders below

Stroke

H/M/L

Depending on disability and comorbidities, including cardiac disease and diabetes

Risk factors for stroke also might influence infection risk

(diabetes, hypertension, other cardiovascular disease).

Genetic Degenerative/Ataxic Syndromes

M

If associated with bulbar weakness

Cognitive Disorders 

H/M/L

More advanced forms of dementia at higher risk, especially with reduced mobility/frailty.

Increased risk particularly with bulbar difficulties (e.g.

FTD/MND)

Hereditary spastic paraparesis

M/L

Cerebral Palsy

L

Depending on disability

Complex Epilepsy

L

Risk associated with:

Significant bulbar or respiratory muscle weakness Those with respiratory compromise associated with kyphoscoliosis or impaired mobility.

Fever-sensitive epilepsies (e.g. Dravet Syndrome)

Rasmussen’s encephalitis on immunosuppressive medication.

Idiopathic Intracranial Hypertension

L

BMI>40 places patients in ‘increased risk of severe illness from coronavirus’ category. This applies to many IIH patients

Traumatic Brain Injury

M/L

Patients with significantly impaired bulbar function

Movement Disorders (for example, Parkinson’s disease, so-called atypical parkinsonism, dystonia)

H/M/L

Patients in care homes

Patients with significantly impaired bulbar or respiratory function

Patients with additional cognitive impairment which may limit their ability to understand and follow healthcare advice 

Immunosuppressant or indicative medications

The risks for a patient are often more defined by their immunotherapy than the underlying individual disease. 

Many patients are on more than one drug, thus increasing their overall risk.

All of the drugs listed below would put an individual at an increased risk. The presence of additional risk factors would put them at a moderate or high risk. 

These risk factors include: 

•       high doses of immunotherapy

•       use of multiple immunotherapies (not necessarily currently)

•       active disease

•       swallowing or respiratory muscle weakness

•       most importantly, the presence of other co-morbidities, such as interstitial lung disease/pulmonary fibrosis, pulmonary hypertension/pulmonary arterial hypertension, glomerulonephritis/renal impairment (any cause), neutropaenia, lymphopenia, liver disease, diabetes mellitus, ischaemic heart disease, other underlying lung disease (such as asthma, chronic obstructive pulmonary disease; COPD), pregnancy and older age.  

Some patients with very active disease, e.g. newly diagnosed and on IV cyclophosphamide, or who have received antibody depleting therapies, particularly those causing hypogammaglobulinemias (rituximab/ocrelizumab) or alemtuzemab (Campath) may be at high risk.

The following examples illustrate this:

^•      female aged 35, myasthenia gravis, no co-morbidity on azathioprine – low risk 

^•      female aged 35, myasthenia gravis, no co-morbidity, rituximab <12 months ago – moderate risk

^•      female aged 35, myasthenia gravis, no co-morbidity, rituximab <12 months ago and hypogammaglobulinemia –high risk

^•      female aged 75, myasthenia gravis, COPD and renal impairment on steroids –high risk.

Patients must not suddenly stop prednisolone and may actually require higher doses during infection.

Patients can continue hydroxychloroquine and sulfasalazine if they are infected with COVID-19. 

If a patient is infected with COVID-19, they should temporarily stop their conventional DMARD and biological therapy, unless they have myasthenia gravis or neuromyelitis optica (NMO) spectrum disorders.  Any cessation should be reported to non-neurological health care professionals. Any questions about cessation in myasthenia or NMO should be discussed with the neurology team first.

Intravenous immunoglobulin probably does not increase risk.

Prednisolone up to 10mg and also on other immunosuppressants (M), 10–19mg/day monotherapy (L); if in combination with other immunosuppressants (H), 20mg or more per day (H).

Methotrexate (M)

Leflunomide (M)

Azathioprine (H) and 6-mercaptopurine (M)

Mycophenolate mofetil (M/H)

Myfortic (M/H)

Cyclophosphamide IV or oral (H)

Ciclosporin (M)

Tacrolimus (M)

NOTE: the following biological therapies may or may not be on a primary care record/database as they are prescribed in secondary care but can be searched for on HES if given in a day-case unit, e.g. X92.1 includes rituximab, tocilizumab and infliximab. Subcutaneous drugs, e.g. adalimumab and etanercept, are supplied by homecare companies.

The receipt of most biologics probably puts the patient in the moderate or high risk categories (there are some exceptions in the multiple sclerosis category).

Rituximab (Mabthera, Truxima, Rixathon), especially if given in last 12 months and/or with low CD19 and CD27 counts

All anti-TNF drugs: etanercept (e.g. Enbrel, Elrezi, Benepali), adalimumab (e.g. Humira, Amgevita), infliximab (e.g. Remicade, Inflectra), golimumab, certolizumab

Tocilizumab – unable to mount a CRP response, IV or s/c 

Abatacept IV or SC

JAK inhibitors (e.g. baricitinib oral, tofacitinib oral)

Belimumab IV

Anakinra SCc

Secukinumab

Ixekizumab

Apremilast (L)

Sarilumab

Ustekinumab

Multiple sclerosis drugs- see link for details. Outline summarised below:

https://cdn.ymaws.com/www.theabn.org/resource/collection/6750BAE6–4CBC–4DDB–A684–

116E03BFE634/ABN_Guidance_on_DMTs_for_MS_and_COVID19_APPROVED_11_March.pdf

Beta interferons ( Avonex, Betaferon, Extavia, Rebif, Plegridy) no increased risk

Glatiramer acetate (Copaxone, Brabio)  no increased risk

Teriflunomide (Aubagio), Dimethylfumurate (Tecfedera) [L]

Fingolimod (Gilenya) [M]

Natalizumab (Tysabri) no increased risk

Ocrelizumab (Ocrevus) [M]

Cladribine (mavenclad) [M/H] VH for 3 months following treatment

Alemtuzumab (Lemtrada) [M/H] VH for 3 months following treatment 

Other treatments not listed elsewhere with an increased risk:

Human stem cell transplant

Apheresis