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## Complete Summary

#### GUIDELINE TITLE

Management of paraneoplastic neurological syndromes: report of an EFNS
Task Force.

#### BIBLIOGRAPHIC SOURCE(S)

Vedeler CA, Antoine JC, Giometto B, Graus F, Grisold W, Hart IK,
Honnorat J, Sillevis Smitt PA, Verschuuren JJ, Voltz R, Paraneoplastic
Neurological Syndrome Euronetwork. Management of paraneoplastic
neurological syndromes: report of an EFNS Task Force. Eur J Neurol 2006
Jul;13(7):682-90. \[73 references\]
[PubMed](http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=16834698)

#### GUIDELINE STATUS

This is the current release of the guideline.

### COMPLETE SUMMARY CONTENT

 SCOPE\
 METHODOLOGY - including Rating Scheme and Cost Analysis\
 RECOMMENDATIONS\
 EVIDENCE SUPPORTING THE RECOMMENDATIONS\
 BENEFITS/HARMS OF IMPLEMENTING THE GUIDELINE RECOMMENDATIONS\
 QUALIFYING STATEMENTS\
 IMPLEMENTATION OF THE GUIDELINE\
 INSTITUTE OF MEDICINE (IOM) NATIONAL HEALTHCARE QUALITY REPORT
CATEGORIES\
 IDENTIFYING INFORMATION AND AVAILABILITY\
 DISCLAIMER

### SCOPE

#### DISEASE/CONDITION(S)

Paraneoplastic neurological syndromes:

-   Limbic encephalitis

-   Subacute sensory neuronopathy

-   Cerebellar degeneration

-   Opsoclonus-myoclonus

-   Lambert--Eaton myasthenic syndrome

-   Peripheral nerve hyperexcitability

**Note**: Myasthenia gravis, paraproteinemic neuropathies,
paraneoplastic retinopathy and dermatomyositis have not been included in
this report.

#### GUIDELINE CATEGORY

Diagnosis\
Evaluation\
Management\
Treatment

#### CLINICAL SPECIALTY

Family Practice\
Internal Medicine\
Neurology\
Oncology\
Pediatrics

#### INTENDED USERS

Physicians

#### GUIDELINE OBJECTIVE(S)

To outline guidelines for the management of classical paraneoplastic
neurological syndrome (PNS)

#### TARGET POPULATION

Patients with paraneoplastic neurological syndrome (PNS)

#### INTERVENTIONS AND PRACTICES CONSIDERED

**Diagnosis/Evaluation**

1.  Assessment of onconeuronal antibodies

2.  High resolution computed tomography (CT) and Fluorodeoxyglucose
    positron-emission tomography (FDG PET)

3.  Follow-up at regular intervals to search for tumors

**Management/Treatment**

1.  Treatment of underlying tumor

2.  Specialist consultation

3.  Immune therapy (steroids, plasma exchange, or intravenous
    immunoglobulin) for children with paraneoplastic opsoclonus
    myoclonus (POM) or adults with Lambert--Eaton myasthenic syndrome
    (LEMS) or peripheral nerve hyperexcitability (PPNH)

4.  Symptomatic therapy

#### MAJOR OUTCOMES CONSIDERED

-   Sensitivity and specificity of diagnostic tests

-   Effectiveness of treatment

### METHODOLOGY

#### METHODS USED TO COLLECT/SELECT EVIDENCE

Searches of Electronic Databases

#### DESCRIPTION OF METHODS USED TO COLLECT/SELECT THE EVIDENCE

Search strategies have included English literature from the following
databases: Cochrane Library, MedLine, PubMed (last search 15 December
2004). The key words used for the search included \'limbic
encephalitis\', \'sensory neuronopathy\' \'cerebellar ataxia\',
\'opsoclonus-myoclonus\', \'Lambert--Eaton myasthenic syndrome\',
\'neuromyotonia\' in combination with \'investigation\' and \'therapy\'.

#### NUMBER OF SOURCE DOCUMENTS

Not stated

#### METHODS USED TO ASSESS THE QUALITY AND STRENGTH OF THE EVIDENCE

Weighting According to a Rating Scheme (Scheme Given)

#### RATING SCHEME FOR THE STRENGTH OF THE EVIDENCE

**Evidence Classification Scheme for a Diagnostic Measure**

**Class I**: A prospective study in a broad spectrum of persons with the
suspected condition, using a \"gold standard\" for case definition,
where the test is applied in a blinded evaluation, and enabling the
assessment of appropriate tests of diagnostic accuracy

**Class II**: A prospective study of a narrow spectrum of persons with
the suspected condition, or a well-designed retrospective study of a
broad spectrum of persons with an established condition (by \"gold
standard\") compared to a broad spectrum of controls, where test is
applied in a blinded evaluation, and enabling the assessment of
appropriate tests of diagnostic accuracy

**Class III**: Evidence provided by a retrospective study where either
persons with the established condition or controls are of a narrow
spectrum, and where test is applied in a blinded evaluation

**Class IV**: Any design where test is not applied in blinded evaluation
OR evidence provided by expert opinion alone or in descriptive case
series (without controls)

**Evidence Classification Scheme for a Therapeutic Intervention**

**Class I**: An adequately powered prospective, randomized, controlled
clinical trial with masked outcome assessment in a representative
population or an adequately powered systematic review of prospective
randomized controlled clinical trials with masked outcome assessment in
representative populations. The following are required:

a.  Randomization concealment

b.  Primary outcome(s) is/are clearly defined

c.  Exclusion/inclusion criteria are clearly defined

d.  Adequate accounting for dropouts and crossovers with numbers
    sufficiently low to have minimal potential for bias

e.  Relevant baseline characteristics are presented and substantially
    equivalent among treatment groups or there is appropriate
    statistical adjustment for differences

**Class II**: Prospective matched-group cohort study in a representative
population with masked outcome assessment that meets a--e above or a
randomized, controlled trial in a representative population that lacks
one criteria a--e

**Class III**: All other controlled trials (including well-defined
natural history controls or patients serving as own controls) in a
representative population, where outcome assessment is independent of
patient treatment

**Class IV**: Evidence from uncontrolled studies, case series, case
reports, or expert opinion

#### METHODS USED TO ANALYZE THE EVIDENCE

Systematic Review

#### DESCRIPTION OF THE METHODS USED TO ANALYZE THE EVIDENCE

Not stated

#### METHODS USED TO FORMULATE THE RECOMMENDATIONS

Expert Consensus

#### DESCRIPTION OF METHODS USED TO FORMULATE THE RECOMMENDATIONS

All evidence available was evaluated as class IV -- case reports, case
series and expert opinion. Thus no recommendations reach level A, B or
C. Good practice points were agreed by consensus.

#### RATING SCHEME FOR THE STRENGTH OF THE RECOMMENDATIONS

**Rating of Recommendations for a Diagnostic Measure**

**Level A rating** (established as useful/predictive or not
useful/predictive) requires at least one convincing class I study or at
least two consistent, convincing class II studies.

**Level B rating** (established as probably useful/predictive or not
useful/predictive) requires at least one convincing class II study or
overwhelming class III evidence.

**Level C rating** (established as possibly useful/predictive or not
useful/predictive) requires at least two convincing class III studies.

**Rating of Recommendations for a Therapeutic Intervention**

**Level A rating** (established as effective, ineffective, or harmful)
requires at least one convincing class I study or at least two
consistent, convincing class II studies.

**Level B rating** (probably effective, ineffective, or harmful)
requires at least one convincing class II study or overwhelming class
III evidence.

**Level C rating** (possibly effective, ineffective, or harmful)
requires at least two convincing class III studies.

**Good Practice Points** Where there was lack of evidence but consensus
was clear the Task Force members have stated their opinion as good
practice points.

#### COST ANALYSIS

A formal cost analysis was not performed and published cost analyses
were not reviewed.

#### METHOD OF GUIDELINE VALIDATION

Peer Review

#### DESCRIPTION OF METHOD OF GUIDELINE VALIDATION

The guidelines were validated according to the European Federation of
Neurological Societies (EFNS) criteria (see \"Availability of Companion
Documents\").

### RECOMMENDATIONS

#### MAJOR RECOMMENDATIONS

The levels of evidence (class I-IV) supporting the recommendations and
ratings of recommendations (A-C, Good Practice Points) are defined at
the end of the \"Major Recommendations\" field.

**Good Practice Points**

-   Patients with paraneoplastic neurological syndrome (PNS) most often
    present with neurological symptoms before an underlying tumour is
    detected. Onconeural antibodies should be sought in sera from
    patients with suspected PNS. The antibodies are important for
    diagnosis and tumour search.

-   Radiological investigations for tumours, such as high resolution CT
    for the detection of small cell lung cancer (SCLC), are important,
    but should be followed by fluorodeoxyglucose positron-emission
    tomography (FDG PET) if no tumour is found.

-   Patients should also be followed at regular intervals, for example
    every 6 months for up to 4 years, to search for tumour in cases
    where the initial tumour screen was negative.

-   Early detection and treatment of the tumour is the approach that
    seems to offer the greatest chance for PNS stabilization. This is
    carried out in cooperation with the oncologist, pulmonologist,
    gynaecologist or paediatrician depending on the associated tumour.

-   Immune therapy (steroids, plasma exchange or intravenous
    immunoglobulin) usually has no or modest effect on paraneoplastic
    limbic encephalitis (PLE), subacute sensory neuropathy (SSN) or
    paraneoplastic cerebellar degeneration (PCD).

-   Children with paraneoplastic opsoclonus-myoclonus (POM) may respond
    to immune therapy, whereas no clear evidence of such therapy has
    been shown in adults with POM.

-   Patients with Lambert-Eaton myasthenic syndrome (LEMS) or
    paraneoplastic peripheral nerve hyperexcitability (PPNH) usually
    improve with immune therapy.

-   Symptomatic therapy should be offered to all patients with PNS.

**[Definitions]{.underline}**:

**Evidence Classification Scheme for a Diagnostic Measure**

**Class I**: A prospective study in a broad spectrum of persons with the
suspected condition, using a \"gold standard\" for case definition,
where the test is applied in a blinded evaluation, and enabling the
assessment of appropriate tests of diagnostic accuracy

**Class II**: A prospective study of a narrow spectrum of persons with
the suspected condition, or a well-designed retrospective study of a
broad spectrum of persons with an established condition (by \"gold
standard\") compared to a broad spectrum of controls, where test is
applied in a blinded evaluation, and enabling the assessment of
appropriate tests of diagnostic accuracy

**Class III**: Evidence provided by a retrospective study where either
persons with the established condition or controls are of a narrow
spectrum, and where test is applied in a blinded evaluation

**Class IV**: Any design where test is not applied in blinded evaluation
OR evidence provided by expert opinion alone or in descriptive case
series (without controls)

**Evidence Classification Scheme for a Therapeutic Intervention**

**Class I**: An adequately powered prospective, randomized, controlled
clinical trial with masked outcome assessment in a representative
population or an adequately powered systematic review of prospective
randomized controlled clinical trials with masked outcome assessment in
representative populations. The following are required:

a.  Randomization concealment

b.  Primary outcome(s) is/are clearly defined

c.  Exclusion/inclusion criteria are clearly defined

d.  Adequate accounting for dropouts and crossovers with numbers
    sufficiently low to have minimal potential for bias

e.  Relevant baseline characteristics are presented and substantially
    equivalent among treatment groups or there is appropriate
    statistical adjustment for differences

**Class II**: Prospective matched-group cohort study in a representative
population with masked outcome assessment that meets a--e above or a
randomized, controlled trial in a representative population that lacks
one criteria a--e

**Class III**: All other controlled trials (including well-defined
natural history controls or patients serving as own controls) in a
representative population, where outcome assessment is independent of
patient treatment

**Class IV**: Evidence from uncontrolled studies, case series, case
reports, or expert opinion

**Rating of Recommendations for a Diagnostic Measure**

**Level A rating** (established as useful/predictive or not
useful/predictive) requires at least one convincing class I study or at
least two consistent, convincing class II studies.

**Level B rating** (established as probably useful/predictive or not
useful/predictive) requires at least one convincing class II study or
overwhelming class III evidence.

**Level C rating** (established as possibly useful/predictive or not
useful/predictive) requires at least two convincing class III studies.

**Rating of Recommendations for a Therapeutic Intervention**

**Level A rating** (established as effective, ineffective, or harmful)
requires at least one convincing class I study or at least two
consistent, convincing class II studies.

**Level B rating** (probably effective, ineffective, or harmful)
requires at least one convincing class II study or overwhelming class
III evidence.

**Level C rating** (possibly effective, ineffective, or harmful)
requires at least two convincing class III studies.

**Good Practice Points** Where there was lack of evidence but consensus
was clear the Task Force members have stated their opinion as good
practice points.

#### CLINICAL ALGORITHM(S)

None provided

### EVIDENCE SUPPORTING THE RECOMMENDATIONS

#### TYPE OF EVIDENCE SUPPORTING THE RECOMMENDATIONS

The type of supporting evidence is identified and graded for selected
recommendations (see \"Major Recommendations\").

### BENEFITS/HARMS OF IMPLEMENTING THE GUIDELINE RECOMMENDATIONS

#### POTENTIAL BENEFITS

Appropriate management of paraneoplastic neurological syndromes

#### POTENTIAL HARMS

Not stated

### QUALIFYING STATEMENTS

#### QUALIFYING STATEMENTS

This guideline provides the view of an expert task force appointed by
the Scientific Committee of the European Federation of Neurological
Societies (EFNS). It represents a peer-reviewed statement of minimum
desirable standards for the guidance of practice based on the best
available evidence. It is not intended to have legally binding
implications in individual cases.

### IMPLEMENTATION OF THE GUIDELINE

#### DESCRIPTION OF IMPLEMENTATION STRATEGY

The European Federation of Neurological Societies has a mailing list and
all guideline papers go to national societies, national ministries of
health, World Health Organisation, European Union, and a number of other
destinations. Corporate support is recruited to buy large numbers of
reprints of the guideline papers and permission is given to sponsoring
companies to distribute the guideline papers from their commercial
channels, provided there is no advertising attached.

### INSTITUTE OF MEDICINE (IOM) NATIONAL HEALTHCARE QUALITY REPORT CATEGORIES

#### IOM CARE NEED

Getting Better

#### IOM DOMAIN

Effectiveness

### IDENTIFYING INFORMATION AND AVAILABILITY

#### BIBLIOGRAPHIC SOURCE(S)

Vedeler CA, Antoine JC, Giometto B, Graus F, Grisold W, Hart IK,
Honnorat J, Sillevis Smitt PA, Verschuuren JJ, Voltz R, Paraneoplastic
Neurological Syndrome Euronetwork. Management of paraneoplastic
neurological syndromes: report of an EFNS Task Force. Eur J Neurol 2006
Jul;13(7):682-90. \[73 references\]
[PubMed](http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=16834698)

#### ADAPTATION

Not applicable: The guideline was not adapted from another source.

#### DATE RELEASED

2006 Jul

#### GUIDELINE DEVELOPER(S)

European Federation of Neurological Societies - Medical Specialty
Society

#### SOURCE(S) OF FUNDING

European Federation of Neurological Societies

#### GUIDELINE COMMITTEE

European Federation of Neurological Societies Task Force on the
Management of Paraneoplastic Neurological Syndromes

#### COMPOSITION OF GROUP THAT AUTHORED THE GUIDELINE

*Task Force Members*: C. A. Vedeler, Department of Neurology, Haukeland
University Hospital, Bergen, Norway, Department of Clinical Medicine,
University of Bergen, Bergen, Norway; J. C. Antoine, Department of
Neurology, Hopital Bellevue, Saint Etienne, France; B. Giometto,
Department of Neurology and Psychiatry (Neurologic Clinic) University of
Padua, Padua, Italy; F. Graus, Service of Neurology, Institut
d\'Investigacio Biomedica August Pi i Sunyer (IDIBAPS), Hospital Clinic,
University of Barcelona, Barcelona, Spain; W. Grisold, Ludwig Boltzmann
Institut fur Neuroonkologie, Vienna, Linz, Austria; I. K. Hart,
Neuroimmunology Group, Department of Neurological Science, Liverpool,
UK; J. Honnorat, Ataxia Research Center, Neurology B, Hospital
Neurologique, Lyon, France; P. A. E. Sillevis Smitt, Department of
Neurology, Erasmus University Medical Center, Rotterdam, The
Netherlands; J. J. G. M. Verschuuren, Department of Neurology, Leiden
University Medical Center, Leiden, The Netherlands; R. Voltz, Department
of Palliative Medicine, University of Cologne, Cologne, Germany for the
Paraneoplastic Neurological Syndrome Euronetwork

#### FINANCIAL DISCLOSURES/CONFLICTS OF INTEREST

Not stated

#### GUIDELINE STATUS

This is the current release of the guideline.

#### GUIDELINE AVAILABILITY

Electronic copies: Available to registered users from the [European
Federation of Neurological Societies Web
site](http://www.efns.org/content.php?pid=142).

Print copies: Available from Christian A. Vedeler, Department of
Neurology, Haukeland University Hospital, Bergen, Norway; Phone: 47 55
97 5044; Fax: 47 55 97 5164; E-mail: <christian.vedeler@helse-bergen.no>

#### AVAILABILITY OF COMPANION DOCUMENTS

The following are available:

-   Brainin M, Barnes M, Baron JC, Gilhus NE, Hughes R, Selmaj K,
    Waldemar G; Guideline Standards Subcommittee of the EFNS Scientific
    Committee. Guidance for the preparation of neurological management
    guidelines by EFNS scientific task forces -- revised
    recommendations 2004. Eur J Neurol. 2004 Sep;11(9):577-81.
    Electronic copies: Available in Portable Document Format (PDF) from
    the [European Federation of Neurological Societies Web
    site](http://www.efns.org/files/guideline_23.pdf).

-   Guideline papers. European Federation of Neurological Societies.
    Electronic copies: Available from the [European Federation of
    Neurological Societies Web
    site](http://www.efns.org/content.php?pid=141).

#### PATIENT RESOURCES

None available

#### NGC STATUS

This NGC summary was completed by ECRI on April 6, 2007. The information
was verified by the guideline developer on May 15, 2007.

#### COPYRIGHT STATEMENT

This NGC summary is based on the original guideline, which is subject to
the Blackwell-Synergy copyright restrictions.

### DISCLAIMER

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© 1998-2008 National Guideline Clearinghouse

Date Modified: 11/3/2008