Cryptogenic late-onset epileptic spasms
Synonyms: Late-onset infantile spasms
- Type
- Disease
- Group
- Disorder
- Age of onset
- Childhood
- Status
- active
Cryptogenic late-onset epileptic spasms is a rare epilepsy syndrome characterized by late-onset (after 1 year old) epileptic spasms that ocurr in clusters, associated with tonic seizures, atypical absences and cognitive deterioration. Language difficulties and behavior problems are frequently present. EEG is characterized by a temporal, or temporofrontal, slow wave or spike focus combined with synchronous spike-waves and no hypsarrhythmia or background activity.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Cryptogenic late-onset epileptic spasms | Late-onset infantile spasms |
| fr | Spasme cryptogénique à début tardif | Spasme infantile à début tardif |
| de | Epilepsie, kryptogenetische, spät beginnende | |
| es | Espasmos epilépticos criptogénicos de inicio tardío | Espasmos infantiles de inicio tardío · Espasmos infantiles tardíos |
| it | Spasmi epilettici criptogenici ad esordio tardivo | |
| nl | Cryptogene epileptische spasmen met late aanvang | Infantiele spasmen met late aanvang |
| pt | Espasmos epilépticos criptogénicos de início tardio | |
| pl | Kryptogenne drgawki padaczkowe o późnym początku | Drgawki dziecięce o późnym początku |
| cs | Kryptogenní epileptické spazmy s pozdním nástupem | Infantilní spazmy s pozdním nástupem |
| uk | Криптогенні пізні епілептичні спазми | Пізній початок дитячих спазмів |
| zh | 隐源性迟发性癫痫痉挛 | 晚发性婴儿痉挛 |
| tr | Kriptojenik geç başlangıçlı epileptik spazmlar | Geç başlangıçlı infantil spazmlar |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | G40.4 | NTBT | attributed | validated |
| ICD-11 | 8A61.2Y | NTBT | index | validated |
| MONDO | 0015585 | E | validated | |
| UMLS | C4755310 | 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:98259Childhood-onset epilepsy syndrome
rare genetic diseases; rare neurological diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | 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
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/163708
- This record as JSON: /data/d/163708.json