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Humana Genetic Testing for Hereditary Peripheral Neuropathies Form

This procedure is not covered

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Effective Date

06/22/2023

Last Reviewed

Original Document

Reference

Description

Genetic testing may be performed to analyze an individual’s deoxyribonucleic acid (DNA) to detect gene variants to assist in confirming a diagnosis in those who exhibit disease signs and symptoms and to aid with treatment decisions for hereditary peripheral neuropathies. Peripheral neuropathy causes nerve damage to the nerves located outside of the brain and spinal cord. It is characterized by numbness or weakness. This nerve damage can be the result of several disorders and can be inherited.

Charcot-Marie-Tooth (CMT) disease is the most common type of the hereditary peripheral neuropathies. CMT is categorized into 7 subtypes and involves as many as 30 disorders though most cases are type 1 and type 2. An individual with CMT may experience weaker muscles, numbness, spasticity and difficulty walking. Onset of symptoms usually occurs in adolescence or early adulthood.

Hereditary neuropathy with liability to pressure palsies (HNPP) is an inherited disorder characterized by recurring sensory and motor neuropathy. An individual will typically experience symptoms in early adulthood.

Spastic paraplegia is a group of hereditary conditions that involves weakness and spasticity and may lead to the inability to walk. It is a rare condition with variable age of onset.

Multigene (or expanded) panels analyze a broad set of genes simultaneously (as opposed to single gene testing that searches for variants in one specific gene) and have been proposed to evaluate the DNA of individuals with a personal and/or family history of more than one hereditary condition or syndrome. Panels often include medically actionable genes but may also include those with unclear medical management. Targeted (or focused) multigene panels analyze a limited number of genes targeted to a specific condition.

For information regarding comparative genomic hybridization (CGH) to detect deletions/duplications and/or for full gene sequence analysis for single gene disorders, please refer to Comparative Genomic Hybridization/Chromosomal Microarray Analysis Medical Coverage Policy.

For information regarding genetic testing for the following, please refer to Genetic Testing Medical Coverage Policy:

DNA banking or preservation
General population screening
Individual 17 years of age or younger for adult-onset conditions
Interpretation and reporting for molecular pathology procedure
Polygenic risk score (PRS) and single nucleotide polymorphisms (SNPs)
Repeat germline or somatic genetic testing
Retrieved archival tissue

Humana recognizes that the field of genetic testing is rapidly changing and that other tests may become available.

Coverage Determination

Any state mandates for genetic testing for diagnosis of noncancer indications take precedence over this medical coverage policy.

Genetic testing may be excluded by certificate. Please consult the member’s individual certificate regarding Plan coverage.

Apply General Criteria for Genetic and Pharmacogenomics Tests when disease- or gene-specific criteria are not available on a medical coverage policy.

For information regarding general criteria for genetic tests, please refer to Genetic Testing Medical Coverage Policy.

CHARCOT-MARIE-TOOTH NEUROPATHY

Charcot-Marie-Tooth Neuropathy (PMP22) – Affected Individual

Humana members may be eligible under the Plan for Charcot-Marie-Tooth (CMT) neuropathy (PMP22 full gene sequencing [81325] and deletion/duplication analysis [81324]) when the following criteria are met:

Pre- and post-test genetic counseling; AND
Performed to confirm a diagnosis of CMT neuropathy when the individual to be tested exhibits symmetric, slowly progressive distal motor neuropathy of the arms and legs

Testing strategy: testing begins with PMP22 deletion/duplication analysis. If negative, proceed to multigene panel testing that includes core genes associated with CMT (GJB1, MFN2, MPZ, PMP22) (81448).

Charcot-Marie-Tooth Neuropathy [PMP22]) – Unaffected Individual

Humana members may be eligible under the Plan for genetic testing to determine risk for CMT neuropathy (PMP22 full gene sequencing [81325] and deletion/duplication analysis [81324]) when the following criteria are met:

Pre- and post-test genetic counseling; AND
Individual to be tested is unaffected; AND
An affected first-degree relative is unavailable for genetic testing (e.g., deceased, declines genetic testing or unable to contact); AND
Individual to be tested has a personal history of Philadelphia chromosome-negative (Ph-) acute lymphoblastic leukemia (ALL); AND
Has a first-degree relative diagnosed with neuropathy; AND
Testing performed prior to initiation of treatment with Vinca alkaloids (e.g., Vincristine)

Charcot-Marie-Tooth Neuropathy – Known Familial Pathogenic or Likely Pathogenic Variant

Humana members may be eligible under the Plan for CMT known familial variant (KFV) genetic testing (81326) when the following criteria are met:

Pre- and post-test genetic counseling; AND
Individual to be tested has an affected first-, second- or third-degree relative with a pathogenic or likely pathogenic variant (genetic testing should be limited to the KFV)

HEREDITARY NEUROPATHY WITH LIABILITY TO PRESSURE PALSY

Hereditary Neuropathy with Liability to Pressure Palsy (PMP22) – Affected Individual

Humana members may be eligible under the Plan for hereditary neuropathy with liability to pressure palsy (HNPP) (PMP22 full gene sequencing [81325] and deletion/duplication analysis [81324]) when the following criteria are met:

Pre- and post-test genetic counseling; AND

Refer to Medical and Pharmacy Coverage Policies to verify that this is the current version before utilizing.

Performed to confirm a diagnosis of HNPP when the individual to be tested exhibits recurrent acute sensory and motor neuropathy in a single or multiple nerves

Hereditary Neuropathy with Liability to Pressure Palsy (PMP22) – Known Familial Pathogenic or Likely Pathogenic Variant

Humana members may be eligible under the Plan for HNPP KFV genetic testing (81326) when the following criteria are met:

Pre- and post-test genetic counseling; AND
Individual to be tested has an affected first-, second- or third-degree relative with a pathogenic or likely pathogenic variant of the PMP22 gene (genetic testing should be limited to the KFV)

HEREDITARY SPASTIC PARAPLEGIA

Hereditary Spastic Paraplegia – Affected Individual

Humana members may be eligible under the Plan for multigene panel testing (81448) for hereditary spastic paraplegia when the following criteria are met:

Pre- and post-test genetic counseling; AND
Performed to confirm a diagnosis of hereditary spastic paraplegia in an individual who exhibits bilateral lower-extremity spasticity and weakness

Hereditary Spastic Paraplegia – Known Familial Pathogenic or Likely Pathogenic Variant

Humana members may be eligible under the Plan for hereditary spastic paraplegia KFV genetic testing when the following criteria are met:

Pre- and post-test genetic counseling; AND
Individual to be tested has an affected first-, second- or third-degree relative with a pathogenic or likely pathogenic variant (genetic testing should be limited to the KFV)

Genetic Testing for Hereditary Peripheral Neuropathies

Effective Date: 06/22/2023
Revision Date: N/A
Review Date: 06/22/2023
Policy Number: HUM-0611-000
Page: 6 of 10

Humana's documents are updated regularly online. When printed, the version of this document becomes uncontrolled. Do not rely on printed copies for the most up-to-date version. Refer to Medical and Pharmacy Coverage Policies to verify that this is the current version before utilizing.

Coverage Limitations

Humana members may NOT be eligible under the Plan for genetic testing for noncancer indications for the following:

Deletion/duplication information is obtained as part of the sequencing procedure but submitted as an independent analysis
Individual to be tested has an affected first-, second- or third-degree relative with a negative genetic testing result for the associated condition
Individual to be tested is unaffected and an affected first-, second- or third- degree relative is available for genetic testing
KFV analysis using a multigene panel that includes the KFV
Sequencing, deletion/duplication analysis and large genomic rearrangement analysis of a single gene, multigene panel or sequentially for the detection of a KFV without the KFV results of a relative

These are considered not medically necessary as defined in the member’s individual certificate. Please refer to the member’s individual certificate for the specific definition.

Additional information about hereditary peripheral neuropathies may be found from the following websites:

Background

National Library of Medicine

Medical Alternatives

Physician consultation is advised to make an informed decision based on an individual’s health needs.

Humana may offer a disease management program for this condition. The member may call the number on his/her identification card to ask about our programs to help manage his/her care.

Genetic Testing for Hereditary Peripheral Neuropathies

Effective Date: 06/22/2023
Revision Date: N/A
Review Date: 06/22/2023
Policy Number: HUM-0611-000
Page: 7 of 10

Humana's documents are updated regularly online. When printed, the version of this document becomes uncontrolled. Do not rely on printed copies for the most up-to-date version.