AESD
MCID: ACT191
MIFTS: 19

Acute Encephalopathy with Biphasic Seizures and Late Reduced Diffusion (AESD)

Categories: Neuronal diseases, Rare diseases

Aliases & Classifications for Acute Encephalopathy with Biphasic Seizures and Late Reduced...

MalaCards integrated aliases for Acute Encephalopathy with Biphasic Seizures and Late Reduced Diffusion:

Name: Acute Encephalopathy with Biphasic Seizures and Late Reduced Diffusion 58 36
Acute Infantile Encephalopathy Predominantly Affecting the Frontal Lobes 58
Aesd 58
Aief 58

Characteristics:

Orphanet epidemiological data:

58

Classifications:

Orphanet: 58  
Rare neurological diseases


External Ids:

KEGG 36 H01841
ICD10 via Orphanet 33 G40.4
Orphanet 58 ORPHA363549

Summaries for Acute Encephalopathy with Biphasic Seizures and Late Reduced...

KEGG : 36 Acute encephalopathy with biphasic seizures and reduced diffusion (AESD) is a syndrome of encephalopathy characterized by biphasic seizures. The etiology of AESD has been attributed to viral infection like influenza A and human herpes virus 6. AESD is the most common subtype of infectious pediatric encephalopathy in Japan, and is often observed in children with neurological problems, such as intellectual disability and motor delay. AESD is clinically characterized by a prolonged febrile seizure on day 1, followed by late seizures associated with deterioration of the consciousness level on days 4 to 6, and is radiologically characterized by delayed reduced diffusion in the frontal or frontoparietal subcortical white matter, the so-called bright tree appearance, on days 3 to 9. The exact pathogenesis of AESD is uncertain, however, excitotoxic injury with delayed (or apoptotic) neuronal death is hypothesized to be a possible mechanism.

MalaCards based summary : Acute Encephalopathy with Biphasic Seizures and Late Reduced Diffusion, also known as acute infantile encephalopathy predominantly affecting the frontal lobes, is related to encephalopathy and seizure disorder. An important gene associated with Acute Encephalopathy with Biphasic Seizures and Late Reduced Diffusion is ADORA2A (Adenosine A2a Receptor). Affiliated tissues include heart.

Related Diseases for Acute Encephalopathy with Biphasic Seizures and Late Reduced...

Diseases related to Acute Encephalopathy with Biphasic Seizures and Late Reduced Diffusion via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 43)
# Related Disease Score Top Affiliating Genes
1 encephalopathy 11.2
2 seizure disorder 11.2
3 status epilepticus 10.9
4 febrile seizures 10.8
5 ocular motor apraxia 10.6
6 epilepsy 10.6
7 west syndrome 10.5
8 exanthem 10.5
9 bacterial infectious disease 10.5
10 hyperglycemia 10.5
11 head injury 10.5
12 atrial standstill 1 10.3
13 tuberous sclerosis 1 10.3
14 dravet syndrome 10.3
15 kawasaki disease 10.3
16 alacrima, achalasia, and mental retardation syndrome 10.3
17 carbonic anhydrase va deficiency, hyperammonemia due to 10.3
18 striatal degeneration, autosomal dominant 2 10.3
19 aphasia 10.3
20 middle east respiratory syndrome 10.3
21 mumps 10.3
22 parotitis 10.3
23 meningoencephalitis 10.3
24 hemiplegia 10.3
25 spinal muscular atrophy 10.3
26 quadriplegia 10.3
27 tuberous sclerosis 10.3
28 toxic shock syndrome 10.3
29 acute necrotizing encephalitis 10.3
30 dystonia 10.3
31 pneumonia 10.3
32 muscular atrophy 10.3
33 bap1 tumor predisposition syndrome 10.3
34 broken heart syndrome 10.3
35 cytokine deficiency 10.3
36 staphylococcal toxic shock syndrome 10.3
37 cerebral atrophy 10.3
38 ohtahara syndrome 10.3
39 staphylococcal scarlet fever 10.3
40 apraxia 9.9
41 hepatitis a 9.9
42 hepatitis 9.9
43 disease of mental health 9.5 VSNL1 ADORA2A

Graphical network of the top 20 diseases related to Acute Encephalopathy with Biphasic Seizures and Late Reduced Diffusion:



Diseases related to Acute Encephalopathy with Biphasic Seizures and Late Reduced Diffusion

Symptoms & Phenotypes for Acute Encephalopathy with Biphasic Seizures and Late Reduced...

Drugs & Therapeutics for Acute Encephalopathy with Biphasic Seizures and Late Reduced...

Search Clinical Trials , NIH Clinical Center for Acute Encephalopathy with Biphasic Seizures and Late Reduced Diffusion

Genetic Tests for Acute Encephalopathy with Biphasic Seizures and Late Reduced...

Anatomical Context for Acute Encephalopathy with Biphasic Seizures and Late Reduced...

MalaCards organs/tissues related to Acute Encephalopathy with Biphasic Seizures and Late Reduced Diffusion:

40
Heart

Publications for Acute Encephalopathy with Biphasic Seizures and Late Reduced...

Articles related to Acute Encephalopathy with Biphasic Seizures and Late Reduced Diffusion:

(show top 50) (show all 113)
# Title Authors PMID Year
1
Prediction of AESD and neurological sequelae in febrile status epilepticus. 61
33563484 2021
2
Early non-convulsive seizures are associated with the development of acute encephalopathy with biphasic seizures and late reduced diffusion. 61
33342582 2021
3
Acute encephalopathy with biphasic seizures and late reduced diffusion; posterior frontal hyperperfusion before late seizures revealed by arterial spin labeling: A case report. 61
33663993 2021
4
Spanish Case of Acute Encephalopathy With Biphasic Seizures and Late Reduced Diffusion: Are We Only Seeing the Tip of the Iceberg? 61
33093430 2021
5
Guidelines for the diagnosis and treatment of acute encephalopathy in childhood. 61
32829972 2021
6
Acute encephalopathy with biphasic seizures and late reduced diffusion: A case report. 61
33120854 2020
7
DWI scoring system for prognosis of acute encephalopathy with biphasic seizures and late reduced diffusion. 61
32385727 2020
8
Epidemiological changes of acute encephalopathy in Japan based on national surveillance for 2014-2017. 61
32360071 2020
9
Efficacy of hypothermia therapy in patients with acute encephalopathy with biphasic seizures and late reduced diffusion. 61
32279899 2020
10
Case-control association study of rare nonsynonymous variants of SCN1A and KCNQ2 in acute encephalopathy with biphasic seizures and late reduced diffusion. 61
32276107 2020
11
Acute Encephalopathy with Biphasic Seizures and Late Reduced Diffusion Associated with Dengue Infection in a Child. 61
32594149 2020
12
Vitamin B6 in acute encephalopathy with biphasic seizures and late reduced diffusion. 61
32107100 2020
13
Detailed characteristics of acute encephalopathy with biphasic seizures and late reduced diffusion: 18-year data of a single-center consecutive cohort. 61
32001378 2020
14
A case of respiratory syncytial virus-associated encephalopathy in which the virus was detected in cerebrospinal fluid and intratracheal aspiration despite negative rapid test results. 61
31879187 2020
15
Acute encephalopathy with biphasic seizures and late reduced diffusion in a Spanish girl. 61
31992519 2020
16
J-type aggregation and thermochromic behavior of a schiff base in solution: Role of keto-enol tautomerization. 61
31806474 2020
17
Human Herpesvirus 7-Related Acute Encephalopathy with Biphasic Seizures and Late Reduced Diffusion. 61
31929436 2020
18
Serum and cerebrospinal fluid cytokines in children with acute encephalopathy. 61
31787380 2020
19
Kawasaki disease presented with status epilepticus and diffusion MRI abnormalities in the subcortical white matter. 61
32419426 2020
20
Perfusion abnormality on three-dimensional arterial spin labeling in patients with acute encephalopathy with biphasic seizures and late reduced diffusion. 61
31715327 2020
21
Prognostic value of MR spectroscopy in patients with acute excitotoxic encephalopathy. 61
31879119 2020
22
Thermolabile polymorphism of carnitine palmitoyltransferase 2: A genetic risk factor of overall acute encephalopathy. 61
31351739 2019
23
Acute encephalopathy with biphasic seizures and late reduced diffusion (central sparing type)-MRI and MR spectroscopy findings. 61
31949346 2019
24
[Acute encephalopathy with biphasic seizures and late reduced diffusion in a child]. 61
31530361 2019
25
Early administration of vitamins B1 and B6 and l-carnitine prevents a second attack of acute encephalopathy with biphasic seizures and late reduced diffusion: A case control study. 61
30862409 2019
26
Subcortical axonal loss with glial reactions following partial status epilepticus with neuroradiological findings of reduced subcortical diffusion. 61
30443827 2019
27
A nationwide survey of norovirus-associated encephalitis/encephalopathy in Japan. 61
30798941 2019
28
Acute encephalopathy with biphasic seizures and late reduced diffusion accompanied by Takotsubo cardiomyopathy. 61
30327157 2019
29
Acute Encephalopathy With Biphasic Seizures and Late Reduced Diffusion Associated With Adenoviral Pneumonia. 61
30783608 2019
30
Corrigendum to "Serum Procalcitonin Levels in Acute Encephalopathy with Biphasic Seizures and Late Reduced Diffusion". 61
31911824 2019
31
Vascular pathomechanism in acute encephalopathy with biphasic seizures and late reduced diffusion. 61
30317181 2018
32
Acute focal bacterial nephritis characterized by acute encephalopathy with biphasic seizures and late reduced diffusion. 61
29752196 2018
33
Loss of myelinated axons and astrocytosis in an autopsy case of acute encephalopathy with biphasic seizures and late reduced diffusion. 61
29929751 2018
34
Differential diagnosis of nonepileptic twilight state with convulsive manifestations after febrile seizures. 61
29866486 2018
35
An episode of acute encephalopathy with biphasic seizures and late reduced diffusion followed by hemiplegia and intractable epilepsy observed in a patient with a novel frameshift mutation in HNRNPU. 61
29858110 2018
36
Predictive indicators for the development of epilepsy after acute encephalopathy with biphasic seizures and late reduced diffusion. 61
29669310 2018
37
Infantile traumatic brain injury with a biphasic clinical course and late reduced diffusion. 61
29801909 2018
38
Acute encephalopathy with biphasic seizures and late reduced diffusion associated with Streptococcus sanguinis sepsis. 61
29721246 2018
39
Use of high b value diffusion-weighted magnetic resonance imaging in acute encephalopathy/encephalitis during childhood. 61
28838686 2018
40
Serum Procalcitonin Levels in Acute Encephalopathy with Biphasic Seizures and Late Reduced Diffusion. 61
29725488 2018
41
Acute encephalopathy with biphasic seizures and late reduced diffusion in Kawasaki disease. 61
29265712 2017
42
A case of mumps-related acute encephalopathy with biphasic seizures and late reduced diffusion. 61
28501474 2017
43
A new infectious encephalopathy syndrome, clinically mild encephalopathy associated with excitotoxicity (MEEX). 61
28870583 2017
44
Early hypoperfusion on arterial spin labeling may be a diagnostic marker for acute encephalopathy with biphasic seizures and late reduced diffusion. 61
28477981 2017
45
Reply to: Early hypoperfusion on arterial spin labelling may be a diagnostic marker for acute encephalopathy with biphasic seizures and late reduced diffusion. 61
28450095 2017
46
Transient dysautonomia in an acute phase of encephalopathy with biphasic seizures and late reduced diffusion. 61
28413125 2017
47
Total corpus callosotomy for epileptic spasms after acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) in a case with tuberous sclerosis complex. 61
27923529 2017
48
"Symptomatic" infection-associated acute encephalopathy in children with underlying neurological disorders. 61
27780632 2017
49
Clinically mild infantile encephalopathy associated with excitotoxicity. 61
28131171 2017
50
A case of acute encephalopathy with biphasic seizures and late reduced diffusion: Utility of arterial spin labeling sequence. 61
27459916 2017

Variations for Acute Encephalopathy with Biphasic Seizures and Late Reduced...

Expression for Acute Encephalopathy with Biphasic Seizures and Late Reduced...

Search GEO for disease gene expression data for Acute Encephalopathy with Biphasic Seizures and Late Reduced Diffusion.

Pathways for Acute Encephalopathy with Biphasic Seizures and Late Reduced...

GO Terms for Acute Encephalopathy with Biphasic Seizures and Late Reduced...

Sources for Acute Encephalopathy with Biphasic Seizures and Late Reduced...

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