Frontotemporal dementia with motor neuron disease

General Information (adopted from Orphanet):

Synonyms, Signs: FTD-MND
FTD-ALS
Frontotemporal dementia with amyotrophic lateral sclerosis
Number of Symptoms 23
OrphanetNr: 275872
OMIM Id: 105550
608030
612069
ICD-10:
UMLs:
MeSH:
MedDRA:
Snomed:

Prevalence, inheritance and age of onset:

Prevalence: No data available.
Inheritance: Autosomal dominant
[Orphanet]
Age of onset: Adult
[Orphanet]

Disease classification (adopted from Orphanet):

Parent Diseases: Frontotemporal degeneration with dementia
 -Rare neurologic disease
Frontotemporal neurodegeneration with movement disorder
 -Rare genetic disease
 -Rare neurologic disease
Genetic frontotemporal degeneration with dementia
 -Rare genetic disease

Symptom Information: Sort by abundance 

1
(HPO:0000605) Supranuclear gaze palsy Rare [HPO:probinson] 16 / 7739
2
(HPO:0001260) Dysarthria 329 / 7739
3
(HPO:0002273) Tetraparesis 15 / 7739
4
(HPO:0000738) Hallucinations 60 / 7739
5
(HPO:0007308) Extrapyramidal dyskinesia 5 / 7739
6
(HPO:0002385) Paraparesis 12 / 7739
7
(HPO:0001300) Parkinsonism 75 / 7739
8
(HPO:0002366) Abnormal lower motor neuron morphology 12 / 7739
9
(HPO:0002145) Frontotemporal dementia 14 / 7739
10
(HPO:0002186) Apraxia 22 / 7739
11
(HPO:0007354) Amyotrophic lateral sclerosis 25 / 7739
12
(HPO:0000746) Delusions 21 / 7739
13
(HPO:0002442) Dyscalculia 5 / 7739
14
(HPO:0000741) Apathy 42 / 7739
15
(HPO:0000716) Depression 99 / 7739
16
(HPO:0001324) Muscle weakness 859 / 7739
17
(HPO:0003202) Skeletal muscle atrophy 281 / 7739
18
(HPO:0003581) Adult onset 117 / 7739
19
(HPO:0003678) Rapidly progressive 33 / 7739
20
(HPO:0002529) Neuronal loss in central nervous system 37 / 7739
21
(HPO:0000006) Autosomal dominant inheritance 2518 / 7739
22
(HPO:0002059) Cerebral atrophy 171 / 7739
23
(HPO:0002171) Gliosis 48 / 7739

Associated genes:

ClinVar (via SNiPA)

Gene symbol Variation Clinical significance Reference

Additional Information: