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    Myasthenia Gravis: Association of British Neurologists' Management Guidelines

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    afm26
    These guidelines provide recommendations for managing myasthenia gravis based on available evidence and clinical experience. They suggest diagnostic tests, initial treatment plans involving pyridostigmine and prednisolone, protocols for dose escalation and withdrawal of medications, consideration of immunosuppression and thymectomy, and inpatient management principles. The guidelines are intended to offer general guidance that clinicians can apply flexibly based on individual patient presentations and symptoms.

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    Myasthenia Gravis: Association of British Neurologists' Management Guidelines

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    afm26
    34 views14 pages
    These guidelines provide recommendations for managing myasthenia gravis based on available evidence and clinical experience. They suggest diagnostic tests, initial treatment plans involving pyridostigmine and prednisolone, protocols for dose escalation and withdrawal of medications, consideration of immunosuppression and thymectomy, and inpatient management principles. The guidelines are intended to offer general guidance that clinicians can apply flexibly based on individual patient presentations and symptoms.

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    These guidelines provide recommendations for managing myasthenia gravis based on available evidence and clinical experience. They suggest diagnostic tests, initial treatment plans involving pyridostigmine and prednisolone, protocols for dose escalation and withdrawal of medications, consideration of immunosuppression and thymectomy, and inpatient management principles. The guidelines are intended to offer general guidance that clinicians can apply flexibly based on individual patient presentations and symptoms.

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    © All Rights Reserved
    Download as PPTX, PDF, TXT or read online from Scribd
    Download as pptx, pdf, or txt
    34 views14 pages

    Myasthenia Gravis: Association of British Neurologists' Management Guidelines

    Uploaded by

    afm26
    These guidelines provide recommendations for managing myasthenia gravis based on available evidence and clinical experience. They suggest diagnostic tests, initial treatment plans involving pyridostigmine and prednisolone, protocols for dose escalation and withdrawal of medications, consideration of immunosuppression and thymectomy, and inpatient management principles. The guidelines are intended to offer general guidance that clinicians can apply flexibly based on individual patient presentations and symptoms.

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    MYASTHENIA GRAVIS: ASSOCIATION OF

    BRITISH NEUROLOGISTS MANAGEMENT


    GUIDELINES
    Jon Sussman,1 Maria E Farrugia,2 Paul
    Maddison,3 Marguerite Hill,4
    M Isabel Leite,5 David Hilton-Jones5
    These guidelines are designed to guide physicians
    and general neurologists in the management of
    myasthenia gravis
    They are based on evidence where available, and
    on the experience of experts where well-
    established treatments lack evidence
    These guidelines can only offer suggestions on
    how to treat common clinical scenarios. This
    document is intended to offer guidance: it could
    be followed to the letter or used flexibly.
    Individuals with myasthenia vary, so it is assumed
    that clinicians will select therapy accordingly.
    INVESTIGATIONS, INITIAL ASSESSMENT AND
    DIAGNOSIS
    Diagnostic tests
    Serum
    Thyroid
    Neurophysiology
    Thymus
    MRanti-muscle-specific
    Referral
    Edrophonium
    anti-acetylcholine
    scan
    function
    scanning
    of
    to brain
    a myasthenia
    receptor
    specialist
    (
    INITIAL TREATMENT PLAN
    Choose inpatient care: For significant bulbar
    symptoms early on, low vital capacity,
    respiratory symptoms or progressive
    deterioration.
    Choose outpatient care: For ocular
    symptoms, mild-tomoderate limb weakness
    and mild bulbar symptoms.
    Ocular myasthenia gravis: See section
    Treatment of ocular myasthenia gravis.
    Generalised myasthenia gravis: See section
    Treatment of generalised myasthenia gravis
    TREATMENT OF OCULAR MYASTHENIA
    GRAVIS
    Starting treatment for ocular myasthenia gravis
    1. Start pyridostigmine following protocol. See section
    Pyridostigmine dose escalation protocol.
    2. If the serum ACh-R antibody is positive and the patient is
    aged under 45 years: consider thymectomy at
    presentation.
    3. If symptomatic despite pyridostigmine, start prednisolone
    (generally given on alternate days) See section Protocol
    for starting prednisolone for outpatients with ocular
    myasthenia gravis.
    4. If symptoms relapse on prednisolone withdrawal at a dose
    of 7.510 mg/day (or 1520 mg alternate days) or greater,
    consider immunosuppression. See sections Patients who do
    not achieve remission with prednisolone and
    Immunosuppression.
    PYRIDOSTIGMINE DOSE
    ESCALATION PROTOCOL
    Titrate up to find the lowest effective dose:
    Initially 30 mg ( tablet) four times daily for
    24 days.
    Then 60 mg (1 tablet) four times daily for 5
    days and experiment with timing.
    Then increase to 90 mg four times daily over
    1 week if required. The duration of action
    varies, and some patients require five
    divided daily doses. If pyridostigmine fails to
    control symptoms satisfactorily within a few
    weeks, start prednisolone.
    TREATMENT OF GENERALISED
    MYASTHENIA GRAVIS
    Start pyridostigmine following protocol
    ACh-R antibody seropositive and aged under
    45 years:
    consider thymectomy
    If symptomatic despite pyridostigmine, start
    prednisolone(generally given on alternate
    days)
    If relapse occurs on prednisolone withdrawal
    at a dose of7.510 mg/day (or 1520 mg
    alternate days) or greater,introduce
    immunosuppression.
    PROTOCOL FOR STARTING PREDNISOLONE
    FOR OUTPATIENTS WITH
    GENERALISED MYASTHENIA GRAVIS
    Start 10 mg on alternate days for three doses
    and increase by 10 mg every three doses
    until symptoms improve. Maximum dose is
    100 mg alternate days or 1.5 mg/kg for
    generalised myasthenia gravis. It may take
    many months to achieve full remission.
    PROTOCOL FOR WITHDRAWAL OF
    PREDNISOLONE FOR GENERALISED
    MYASTHENIA GRAVIS
    Prednisolone should be titrated down seeking
    the lowest effective dose only after
    achieving remission (absence of symptoms
    and signs having withdrawn pyridostigmine)
    for at least 23 months.
    IMMUNOSUPPRESSION
    We recommend that an immunosuppressive
    agent is introduced only if a patient does not
    achieve remission on corticosteroid
    monotherapy.
    Immunosuppression should be considered
    early on in patients with diabetes mellitus,
    osteoporosis, or ischaemic heart disease or
    significant bulbar or respiratory weakness
    that does not respond rapidly to
    corticosteroids.
    IMMUNOSUPPRESSION WITH
    AZATHIOPRINE
    Azathioprine is the first-line agent.
    Azathioprine should be increased over 1
    month to a maintenance dose of 2.5 mg/kg.
    Blood tests should be monitored weekly (full
    blood count, urea and electrolytes, liver
    function tests) as the dose is titrated
    upwards.
    Other immunosuppressive agents :
    Mycophenolate mofetil, methotrexate,
    ciclosporin, rituximab
    INPATIENT MANAGEMENT OF MYASTHENIA
    GRAVIS
    General principles
    A patient should be managed in hospital for
    significant bulbar symptoms, low vital
    capacity, respiratory symptoms or
    progressive deterioration.
    Assessment by speech and language
    therapist to advise on swallowing is
    mandatory.
    Regular assessment of vital capacity is
    mandatory
    MANAGEMENT OF MYASTHENIA GRAVIS IN
    INTENSIVE CARE UNITS
    patients may require intensive care or highdependency care
    to monitor respiratory function
    For ventilated patients:
    Start prednisolone at 100 mg alternate days.

    Start intravenous immunoglobulin immediately.

    Avoid inappropriate use of intravenous magnesium.xlix

    Consider avoiding pyridostigmine/neostigmine as these

    may increase secretions.

    For non-ventilated patients:


    Start prednisolone following the standard protocol for

    generalised myasthenia gravis.

    Start intravenous immunoglobulin immediately.

    Avoid using pyridostigmine/neostigmine.


    Thank you

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