MCID: NRN016
MIFTS: 31

Neuronal Migration Disorders malady

Neuronal diseases category
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Summaries for Neuronal Migration Disorders

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NINDS:43 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 based summary: Neuronal Migration Disorders is related to zellweger syndrome and mental retardation. 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:65 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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Neuronal Migration Disorders, Aliases & Descriptions:

Name: Neuronal Migration Disorders 43 62


Classifications:

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


Related Diseases for Neuronal Migration Disorders

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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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Drug clinical trials:

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Genetic Tests for Neuronal Migration Disorders

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Anatomical Context for Neuronal Migration Disorders

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MalaCards organs/tissues related to Neuronal Migration Disorders:

32
Brain, Cerebellum, Cortex, Skin, Temporal lobe

Animal Models for Neuronal Migration Disorders or affiliated genes

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MGI Mouse Phenotypes related to Neuronal Migration Disorders:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053898.9EMX2, DCX, ARX
2MP:00053798.7PEX2, ARX, EMX2
3MP:00107688.3DCX, ARX, PEX2, EMX2
4MP:00053867.6ARX, DCX, EMX2, GPR56, PEX2
5MP:00036317.5ARX, DCX, EMX2, GPR56, PEX2

Publications for Neuronal Migration Disorders

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Articles related to Neuronal Migration Disorders:

(show top 50)    (show all 79)
idTitleAuthorsYear
1
Rufinamide for the treatment of refractory epilepsy secondary to neuronal migration disorders. (24548548)
2014
2
The Relevance of Human Fetal Subplate Zone for Developmental Neuropathology of Neuronal Migration Disorders and Cortical Dysplasia. (25312583)
2014
3
Cytoskeleton in action: lissencephaly, a neuronal migration disorder. (23495356)
2013
4
Embryonic disruption of the candidate dyslexia susceptibility gene homolog Kiaa0319-like results in neuronal migration disorders. (23831424)
2013
5
Neuronal migration disorders in microcephalic osteodysplastic primordial dwarfism type I/III. (20857301)
2011
6
Neuronal migration disorders. (19245832)
2010
7
The beta-tubulin gene TUBB2B is involved in a large spectrum of neuronal migration disorders. (20101787)
2010
8
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
9
Neuronal migration disorders: clinical, neuroradiologic and genetics aspects. (19120042)
2009
10
Sudden death in a patient with mosaic ring X Turner syndrome and a neuronal migration disorder. (18049086)
2008
11
Neuronal migration disorders, agenesis of corpus callosum, preauricular skin tag and bilateral morning glory syndrome in a term newborn infant. (18388784)
2008
12
N-cofilin is associated with neuronal migration disorders and cell cycle control in the cerebral cortex. (17875668)
2007
13
Reading impairment in the neuronal migration disorder of periventricular nodular heterotopia. (16434687)
2006
14
Neuronal migration disorders, genetics, and epileptogenesis. (15921228)
2005
15
Neurophysiologic findings of neuronal migration disorders: intrinsic epileptogenicity of focal cortical dysplasia on electroencephalography, electrocorticography, and magnetoencephalography. (15921239)
2005
16
Are altered excitatory synapses found in neuronal migration disorders? (16175215)
2005
17
Reading impairment in the neuronal migration disorder of periventricular nodular heterotopia. (15753412)
2005
18
Radial and tangential neuronal migration disorder in ibotenate-induced cortical lesions in hamsters: immunohistochemical study of reelin, vimentin, and calretinin. (15072103)
2004
19
Evidence of neuronal migration disorders in Knobloch syndrome: clinical and molecular analysis of two novel families. (12707952)
2003
20
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
21
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
22
Mutations in the O-mannosyltransferase gene POMT1 give rise to the severe neuronal migration disorder Walker-Warburg syndrome. (12369018)
2002
23
Pathogenic significance of neuronal migration disorders in temporal lobe epilepsy. (11431720)
2001
24
Neuronal migration disorders. (11553932)
2001
25
Development and developmental disorders of the human brain. III. Neuronal migration disorders of the cerebrum]. (11268908)
2001
26
Immunohistochemical expression of doublecortin in the human cerebrum: comparison of normal development and neuronal migration disorders. (10773210)
2000
27
Single cause, polymorphic neuronal migration disorders: an animal model. (11085292)
2000
28
Neuronal migration disorders: from genetic diseases to developmental mechanisms. (10906798)
2000
29
Electrophysiological responses in vivo of hippocampal CA1 pyramidal neurons in an animal model of neuronal migration disorders. (10575261)
1999
30
The right neuron at the wrong place: biology of heterotopic neurons in cortical neuronal migration disorders, with special reference to associated pathologies. (10487203)
1999
31
Neuronal migration disorders in humans and in mouse models--an overview. (10515161)
1999
32
Characterization of neuronal migration disorders in neocortical structures: extracellular in vitro recordings. (9786203)
1998
33
Familial neuronal migration disorder: subcortical laminar heterotopia in a mother and pachygyria in the son. (9489790)
1998
34
Characterization of neuronal migration disorders in neocortical structures: quantitative receptor autoradiography of ionotropic glutamate, GABA(A) and GABA(B) receptors. (9786204)
1998
35
Neuronal migration disorders. Part I: Terminology, classification, pathophysiology, EEG and epilepsy. (10028855)
1998
36
Evidence of enhanced kindling and hippocampal neuronal injury in immature rats with neuronal migration disorders. (9860059)
1998
37
Neuronal migration disorders. Part II: Magnetic resonance imaging. (10028856)
1998
38
Congenital muscular dystrophy, white-matter abnormalities, and neuronal migration disorders: the expanding concept. (9796753)
1998
39
Transplacentally induced neuronal migration disorders: an animal model for the study of the epilepsies. (9514201)
1998
40
Positive epileptiform discharges in children with neuronal migration disorders. (9044397)
1997
41
Increased seizure susceptibility in adult rats with neuronal migration disorders. (9449431)
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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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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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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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet