MCID: NRN016
MIFTS: 31

Neuronal Migration Disorders malady

Neuronal diseases category

Summaries for Neuronal Migration Disorders

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45NINDS, 66Wikipedia, 34MalaCards
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NINDS:45 Neuronal migration disorders (NMDs) are a group of birth defects caused by the abnormal migration of neurons in the developing brain and nervous system. In the developing brain, neurons must migrate from the areas where they are born to the areas where they will settle into their proper neural circuits. Neuronal migration, which occurs as early as the second month of gestation, is controlled by a complex assortment of chemical guides and signals. When these signals are absent or incorrect, neurons do not end up where they belong. This can result in structurally abnormal or missing areas of the brain in the cerebral hemispheres, cerebellum, brainstem, or hippocampus. The structural abnormalities found in NMDs include schizencephaly, porencephaly, lissencephaly, agyria, macrogyria, polymicrogyria, pachygyria, microgyria, micropolygyria, neuronal heterotopias (including band heterotopia), agenesis of the corpus callosum, and agenesis of the cranial nerves. Symptoms vary according to the abnormality, but often feature poor muscle tone and motor function, seizures, developmental delays, mental retardation, failure to grow and thrive, difficulties with feeding, swelling in the extremities, and a smaller than normal head. Most infants with an NMD appear normal, but some disorders have characteristic facial or skull features that can be recognized by a neurologist. Several genetic abnormalities in children with NMDs have been identified. Defects in genes that are involved in neuronal migration have been associated with NMDs, but the role they play in the development of these disorders is not yet well-understood. More than 25 syndromes resulting from abnormal neuronal migration have been described. Among them are syndromes with several different patterns of inheritance; genetic counseling thus differs greatly between syndromes.

MalaCards: Neuronal Migration Disorders is related to zellweger syndrome and periventricular nodular heterotopia. An important gene associated with Neuronal Migration Disorders is ARX (aristaless related homeobox). Affiliated tissues include brain, cerebellum and cortex, and related mouse phenotypes are reproductive system and endocrine/exocrine gland.

Wikipedia:66 Nervous system disease refers to a general class of medical conditions affecting the nervous system. more...

Aliases & Classifications for Neuronal Migration Disorders

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45NINDS, 63UMLS
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Anatomical: Neuronal diseases


Aliases & Descriptions:

neuronal migration disorders 45 63


Related Diseases for Neuronal Migration Disorders

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18GeneCards, 19GeneDecks
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Graphical network of the top 20 diseases related to Neuronal Migration Disorders:



Diseases related to neuronal migration disorders

Symptoms for Neuronal Migration Disorders

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Drugs & Therapeutics for Neuronal Migration Disorders

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6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials
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Genetic Tests for Neuronal Migration Disorders

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Anatomical Context for Neuronal Migration Disorders

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34MalaCards
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MalaCards organs/tissues related to Neuronal Migration Disorders:

34
Brain, Cerebellum, Cortex, Skin, Temporal lobe

Animal Models for Neuronal Migration Disorders or affiliated genes

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38MGI
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MGI Mouse Phenotypes related to Neuronal Migration Disorders:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053898.9ARX, DCX, EMX2
2MP:00053798.7ARX, EMX2, PEX2
3MP:00107688.3PEX2, EMX2, DCX, ARX
4MP:00053867.6ARX, DCX, EMX2, GPR56, PEX2
5MP:00036317.5PEX2, GPR56, EMX2, DCX, ARX

Publications for Neuronal Migration Disorders

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53PubMed
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Articles related to Neuronal Migration Disorders:

(show top 50)    (show all 77)
idTitleAuthorsYear
1
Cytoskeleton in action: lissencephaly, a neuronal migration disorder. (23495356)
2013
2
Embryonic disruption of the candidate dyslexia susceptibility gene homolog Kiaa0319-like results in neuronal migration disorders. (23831424)
2013
3
Neuronal migration disorders in microcephalic osteodysplastic primordial dwarfism type I/III. (20857301)
2011
4
Neuronal migration disorders. (19245832)
2010
5
The beta-tubulin gene TUBB2B is involved in a large spectrum of neuronal migration disorders. (20101787)
2010
6
Experimental neuronal migration disorders following the administration of ibotenate in hamsters: the role of the subventricular zone in the development of cortical dysplasia. (19258286)
2009
7
Neuronal migration disorders: clinical, neuroradiologic and genetics aspects. (19120042)
2009
8
Sudden death in a patient with mosaic ring X Turner syndrome and a neuronal migration disorder. (18049086)
2008
9
Neuronal migration disorders, agenesis of corpus callosum, preauricular skin tag and bilateral morning glory syndrome in a term newborn infant. (18388784)
2008
10
N-cofilin is associated with neuronal migration disorders and cell cycle control in the cerebral cortex. (17875668)
2007
11
Reading impairment in the neuronal migration disorder of periventricular nodular heterotopia. (16434687)
2006
12
Neuronal migration disorders, genetics, and epileptogenesis. (15921228)
2005
13
Neurophysiologic findings of neuronal migration disorders: intrinsic epileptogenicity of focal cortical dysplasia on electroencephalography, electrocorticography, and magnetoencephalography. (15921239)
2005
14
Are altered excitatory synapses found in neuronal migration disorders? (16175215)
2005
15
Reading impairment in the neuronal migration disorder of periventricular nodular heterotopia. (15753412)
2005
16
Radial and tangential neuronal migration disorder in ibotenate-induced cortical lesions in hamsters: immunohistochemical study of reelin, vimentin, and calretinin. (15072103)
2004
17
High molecular weight microtubule-associated protein 2 over-expression in neuronal migration disorders caused by N-methyl-D-aspartate receptor activation in hamsters. (15008898)
2004
18
Evidence of neuronal migration disorders in Knobloch syndrome: clinical and molecular analysis of two novel families. (12707952)
2003
19
MRI and EEG findings in surgically treated patients with partial seizures due to neuronal migration disorders, their relations to each other and to surgery outcome. (14616299)
2003
20
Various findings in surgically treated epilepsy patients with dysembryoplastic neuroepithelial tumors in comparison with those of patients with other low-grade brain tumors and other neuronal migration disorders. (12423388)
2002
21
Mutations in the O-mannosyltransferase gene POMT1 give rise to the severe neuronal migration disorder Walker-Warburg syndrome. (12369018)
2002
22
Pathogenic significance of neuronal migration disorders in temporal lobe epilepsy. (11431720)
2001
23
Neuronal migration disorders. (11553932)
2001
24
Development and developmental disorders of the human brain. III. Neuronal migration disorders of the cerebrum]. (11268908)
2001
25
Immunohistochemical expression of doublecortin in the human cerebrum: comparison of normal development and neuronal migration disorders. (10773210)
2000
26
Single cause, polymorphic neuronal migration disorders: an animal model. (11085292)
2000
27
Neuronal migration disorders: from genetic diseases to developmental mechanisms. (10906798)
2000
28
Electrophysiological responses in vivo of hippocampal CA1 pyramidal neurons in an animal model of neuronal migration disorders. (10575261)
1999
29
The right neuron at the wrong place: biology of heterotopic neurons in cortical neuronal migration disorders, with special reference to associated pathologies. (10487203)
1999
30
Neuronal migration disorders in humans and in mouse models--an overview. (10515161)
1999
31
Characterization of neuronal migration disorders in neocortical structures: extracellular in vitro recordings. (9786203)
1998
32
Familial neuronal migration disorder: subcortical laminar heterotopia in a mother and pachygyria in the son. (9489790)
1998
33
Characterization of neuronal migration disorders in neocortical structures: quantitative receptor autoradiography of ionotropic glutamate, GABA(A) and GABA(B) receptors. (9786204)
1998
34
Neuronal migration disorders. Part I: Terminology, classification, pathophysiology, EEG and epilepsy. (10028855)
1998
35
Evidence of enhanced kindling and hippocampal neuronal injury in immature rats with neuronal migration disorders. (9860059)
1998
36
Neuronal migration disorders. Part II: Magnetic resonance imaging. (10028856)
1998
37
Congenital muscular dystrophy, white-matter abnormalities, and neuronal migration disorders: the expanding concept. (9796753)
1998
38
Transplacentally induced neuronal migration disorders: an animal model for the study of the epilepsies. (9514201)
1998
39
Positive epileptiform discharges in children with neuronal migration disorders. (9044397)
1997
40
Increased seizure susceptibility in adult rats with neuronal migration disorders. (9449431)
1997
41
Ubiquitin-immunoreactive granular inclusions in neuronal migration disorders. (9144592)
1997
42
Neuronal migration disorders presenting with mild clinical symptoms. (8703229)
1996
43
Characterization of neuronal migration disorders in neocortical structures: I. Expression of epileptiform activity in an animal model. (8985688)
1996
44
Neuronal migration disorders and epilepsy: a morphological analysis of three surgically treated patients. (8985686)
1996
45
Neuronal migration disorders. (7501186)
1995
46
Posterior agyria-pachygyria with polymicrogyria: evidence for an inherited neuronal migration disorder. (7824106)
1995
47
Electrophysiology of CA1 pyramidal neurons in an animal model of neuronal migration disorders: prenatal methylazoxymethanol treatment. (8777901)
1995
48
Prenatal events and genetic factors in epileptic patients with neuronal migration disorders. (7925168)
1994
49
Neuronal migration disorders: positron emission tomography correlations. (8122881)
1994
50
Stages and patterns of centrifugal arrest of diffuse neuronal migration disorders. (8335148)
1993

Variations for Neuronal Migration Disorders

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Expression for genes affiliated with Neuronal Migration Disorders

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2BioGPS, 16Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Neuronal Migration Disorders

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Pathways for genes affiliated with Neuronal Migration Disorders

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Compounds for genes affiliated with Neuronal Migration Disorders

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GO Terms for genes affiliated with Neuronal Migration Disorders

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17Gene Ontology
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Biological processes related to Neuronal Migration Disorders according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1cell proliferation in forebrainGO:0218469.4ARX, EMX2
2axon guidanceGO:0074119.4ARX, DCX
3neuron migrationGO:0017648.9ARX, DCX, PEX2
4brain developmentGO:0074208.7DCX, GPR56

Products for genes affiliated with Neuronal Migration Disorders

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Sources for Neuronal Migration Disorders

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4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet