Polyneuropathy associated with IgM monoclonal gammopathy
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult, Elderly
- Status
- active
A rare acquired peripheral neuropathy characterized by symmetric, slowly progressive, predominantly sensory neuropathy, mostly limited to the legs with numbness and paresthesia of the distal leg and mild imbalance. Some patients may experience pain, weakness of foot dorsiflexion, mild proximal leg weakness, and/or upper limb involvement. Systemic illnesses are absent in most cases.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Polyneuropathy associated with IgM monoclonal gammopathy | |
| fr | Polyneuropathie associée à une gammopathie monoclonale IgM | |
| de | Polyneuropathie mit assoziierter monoklonaler IgM-Gammopathie | |
| es | Polineuropatía asociada con gammopatía monoclonal IgM | |
| it | Polineuropatia assonale associata a gammopatia monoclonale IgG/IgM/IgA | |
| nl | Polyneuropathie geassocieerd met monoklonale IgM-gammopathie | |
| pt | Polineuropatia associada a gamopatia monoclonal IgM | |
| pl | Polineuropatia aksonalna związana z gammapatią monoklonalną IgG/IgA/IgM | |
| cs | Axonální polyneuropatie asociovaná s IgG/IgM/IgA monoklonální gamapatií | |
| uk | Аксональна полінейропатія, асоційована з моноклональною гамма-патією IgG/IgM/IgA | |
| zh | 与IgG/IgM/IgA单克隆丙种球蛋白病相关的轴索性多发性神经病 | |
| tr | IgG/IgM/IgA monoklonal gammopati -ilişkili aksonal polinöropati |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | G61.8 | NTBT | attributed | validated |
| MONDO | 0016176 | E | validated | |
| UMLS | C5680822 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:209010Peripheral neuropathy associated with monoclonal gammopathy
rare neurological diseases
No children.
Codes that Orphanet moved or referred to this one:
- ORPHA:639Polyneuropathy associated with IgM monoclonal gammopathy with anti-MAG moved to · inactive
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | ORPHANET |
| Annual incidence | Unknown | — | Worldwide | Class only | Validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
19 annotations · validated 2018-09-19| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0011402 | Demyelinating sensory neuropathy | |
| HP:0002166 | Impaired vibration sensation in the lower limbs | |
| HP:0007133 | Progressive peripheral neuropathy | |
| Frequent (79-30%) | ||
| HP:0007267 | Chronic axonal neuropathy | |
| HP:0007220 | Demyelinating motor neuropathy | |
| HP:0003693 | Distal amyotrophy | |
| HP:0002936 | Distal sensory impairment | |
| HP:0100287 | EMG: slow motor conduction | |
| HP:0007340 | Lower limb muscle weakness | |
| HP:0007002 | Motor axonal neuropathy | |
| Occasional (29-5%) | ||
| HP:0031006 | Acroparesthesia | |
| HP:0012514 | Lower limb pain | |
| HP:0005508 | Monoclonal immunoglobulin M proteinemia | |
| HP:0007240 | Progressive gait ataxia | |
| HP:0003390 | Sensory axonal neuropathy | |
| Very rare (<4-1%) | ||
| HP:0100778 | Cryoglobulinemia | |
| HP:0025346 | Increased circulating beta-2-microglobulin level | |
| HP:0002665 | Lymphoma | |
| HP:0006775 | Multiple myeloma | |
Run the differential on the 3 obligate / very frequent terms
Annotation source: 14641513[PMID]_10540371[PMID]_8380902[PMID]_7953603[PMID]_1706212[PMID]_19720975[PMID]_8673490[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/209004
- This record as JSON: /data/d/209004.json