Difference between revisions of "Neural System - Abnormalities"

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| valign=top|Huntington's disease (HD) is an adult onset inherited neurodegenerative disorder affecting both the striatum and cerebral cortex. A recent mouse model study of this disease{{#pmid:31353533|PMID31353533}} has demonstrated a developmental cortical hyperexcitability, suggesting that there may also be a developmental component to this disease. More? [[2011 Group Project 4|2011 Student project]]
| Huntington's disease (HD) is an adult onset inherited neurodegenerative disorder affecting both the striatum and cerebral cortex. A recent mouse model study of this disease{{#pmid:31353533|PMID31353533}} has demonstrated a developmental cortical hyperexcitability, suggesting that there may also be a developmental component to this disease.
 
 
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==Some Recent Findings==
 
==Some Recent Findings==
 
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Latest revision as of 15:56, 21 November 2019

Embryology - 13 Dec 2019    Facebook link Pinterest link Twitter link  Expand to Translate  
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 ICD-11 LA00-LA0Z Structural developmental anomalies of the nervous system
View full table

Introduction

Australian abnormalities (1981-92)
Neural groove closing to neural tube, early week 4
(Stage 10)

There are many different congenital and environmentally derived abnormalities associated with the nervous system. There are potentially 1000's of neurological abnormalities that could be listed on this page, therefore this current page is only a very brief introduction to some of these neural abnormalities. For additional information see links at the bottom of each sub-heading. The latest  ICD-11 coding now also includes a new category "LD90 Disorders of intellectual development" and also lists LA05.3 Corpus callosum agenesis as "the most common brain malformation and is characterized by total or partial absence of the main interhemispheric commissure, the corpus callosum."


As the nervous system continues to develop fetally and postnatally, environmental issues both before and after birth are relevant to neural development. Embryonic and fetal nutrition folate (required early for neural tube closure) and iodine (required later for neural growth and differentiation). The developmental environment can also impact upon neural growth; maternal drugs such as alcohol and heavy metals such as lead (mining, historically both petrol and paint). Finally, postnatally other sensory abnormalities (hearing) can also impact on achieving developmental milestones.

George Huntington.jpg Huntington's disease (HD) is an adult onset inherited neurodegenerative disorder affecting both the striatum and cerebral cortex. A recent mouse model study of this disease[1] has demonstrated a developmental cortical hyperexcitability, suggesting that there may also be a developmental component to this disease. More? 2011 Student project


Links: neural tube defect


Neural Links: ectoderm | neural | neural crest | ventricular | sensory | Stage 22 | gliogenesis | neural fetal | Medicine Lecture - Neural | Lecture - Ectoderm | Lecture - Neural Crest | Lab - Early Neural | neural abnormalities | folic acid | iodine deficiency | Fetal Alcohol Syndrome | neural postnatal | neural examination | Histology | Historic Neural | Category:Neural


Abnormality Links: abnormal development | abnormal genetic | abnormal environmental | Unknown | teratogens | ectopic pregnancy | cardiovascular abnormalities | coelom abnormalities | endocrine abnormalities | gastrointestinal abnormalities | genital abnormalities | head abnormalities | integumentary abnormalities | musculoskeletal abnormalities | limb abnormalities | neural abnormalities | neural crest abnormalities | placenta abnormalities | renal abnormalities | respiratory abnormalities | hearing abnormalities | vision abnormalities | twinning | Developmental Origins of Health and Disease |  ICD-11
Historic Embryology  
1915 Congenital Cardiac Disease | 1917 Frequency of Anomalies in Human Embryos | 1920 Hydatiform Degeneration Tubal Pregnancy | 1921 Anencephalic Embryo | 1921 Rat and Man | 1966 Congenital Malformations
System Abnormalities 
Abnormality Links: abnormal development | abnormal genetic | abnormal environmental | Unknown | teratogens | ectopic pregnancy | cardiovascular abnormalities | coelom abnormalities | endocrine abnormalities | gastrointestinal abnormalities | genital abnormalities | head abnormalities | integumentary abnormalities | musculoskeletal abnormalities | limb abnormalities | neural abnormalities | neural crest abnormalities | placenta abnormalities | renal abnormalities | respiratory abnormalities | hearing abnormalities | vision abnormalities | twinning | Developmental Origins of Health and Disease |  ICD-11
Historic Embryology  
1915 Congenital Cardiac Disease | 1917 Frequency of Anomalies in Human Embryos | 1920 Hydatiform Degeneration Tubal Pregnancy | 1921 Anencephalic Embryo | 1921 Rat and Man | 1966 Congenital Malformations

Some Recent Findings

  • Quantitative Folding Pattern Analysis of Early Primary Sulci in Human Fetuses with Brain Abnormalities[2] "Aberrant gyral folding is a key feature in the diagnosis of many cerebral malformations. However, in fetal life, it is particularly challenging to confidently diagnose aberrant folding because of the rapid spatiotemporal changes of gyral development. Currently, there is no resource to measure how an individual fetal brain compares with normal spatiotemporal variations. In this study, we assessed the potential for automatic analysis of early sulcal patterns to detect individual fetal brains with cerebral abnormalities. ... Automated analysis of interrelated patterning of early primary sulci could outperform the traditional gyrification index and has the potential to quantitatively detect individual fetuses with emerging abnormal sulcal patterns."
  • The Lateral Temporal Lobe in Early Human Life[3] "Abnormalities of lateral temporal lobe development are associated with a spectrum of genetic and environmental pathologic processes, but more normative data are needed for a better understanding of gyrification in this brain region. Here, we begin to establish guidelines for the analysis of the lateral temporal lobe in humans in early life. We present quantitative methods for measuring gyrification at autopsy using photographs of the gross brain and simple computer-based quantitative tools in a cohort of 28 brains ranging in age from 27 to 70 postconceptional weeks (end of infancy). We provide normative ranges for different indices of gyrification and identify a constellation of qualitative features that should also be considered in these analyses. The ratio of the temporal area to the whole brain area increased dramatically in the second half of gestation, but then decelerated after birth before increasing linearly around 50 postconceptional weeks. Tertiary gyrification continued beyond birth in a linear process through infancy with considerable variation in patterns. Analysis of 2 brains with gyral disorders of the lateral temporal lobe demonstrated proof-of-principle that the proposed methods are of diagnostic value. These guidelines are proposed for assessments of temporal lobe pathology in pediatric brains in early life. "
More recent papers  
Mark Hill.jpg
PubMed logo.gif

This table allows an automated computer search of the external PubMed database using the listed "Search term" text link.

  • This search now requires a manual link as the original PubMed extension has been disabled.
  • The displayed list of references do not reflect any editorial selection of material based on content or relevance.
  • References also appear on this list based upon the date of the actual page viewing.


References listed on the rest of the content page and the associated discussion page (listed under the publication year sub-headings) do include some editorial selection based upon both relevance and availability.

More? References | Discussion Page | Journal Searches | 2019 References

Search term: Abnormal Neural Development

Older papers  
These papers originally appeared in the Some Recent Findings table, but as that list grew in length have now been shuffled down to this collapsible table.

See also the Discussion Page for other references listed by year and References on this current page.

  • Identification of PCSK9 as a novel serum biomarker for the prenatal diagnosis of neural tube defects using iTRAQ quantitative proteomics[4] "To identify candidate serum molecule biomarkers for the non-invasive early prenatal diagnosis of neural tube defects (NTDs), we employed an iTRAQ-based quantitative proteomic approach to analyze the proteomic changes in serum samples from embryonic day (E) 11 and E13 pregnant rats with spina bifida aperta (SBA) induced by all-trans retinoic acid. ...We observed the space-time expression changes of proprotein convertase subtilisin/kexin type 9 (PCSK9) at different stages of fetal development, including a marked decrease in the sera of NTD pregnancies and gradual increase in the sera of normal pregnancies with embryonic development."
  • Mutations in genes encoding the glycine cleavage system predispose to neural tube defects in mice and humans[4] "Function of folate one-carbon metabolism (FOCM) has been implicated as a key determinant of susceptibility to NTDs. The glycine cleavage system (GCS) is a multi-enzyme component of mitochondrial folate metabolism, and GCS-encoding genes therefore represent candidates for involvement in NTDs. To investigate this possibility, we sequenced the coding regions of the GCS genes: AMT, GCSH and GLDC in NTD patients and controls. ... Overall, our findings suggest that loss-of-function mutations in GCS genes predispose to NTDs in mice and humans. These data highlight the importance of adequate function of mitochondrial folate metabolism in neural tube closure." Prenatal Diagnosis
  • A randomized trial of prenatal versus postnatal repair of myelomeningoce[5] "Prenatal repair of myelomeningocele, the most common form of spina bifida, may result in better neurologic function than repair deferred until after delivery. We compared outcomes of in utero repair with standard postnatal repair. ....Prenatal surgery for myelomeningocele reduced the need for shunting and improved motor outcomes at 30 months but was associated with maternal and fetal risks. (Funded by the National Institutes of Health; ClinicalTrials.gov number, NCT00060606.)." (see also paper comments)
  • Australia 1998–2005[6] "944 births over this period affected by NTD. Of these births, 523 were live births and 421 were fetal deaths (still births and terminations after 20 weeks gestation). This equates to a prevalence of neural tube defects (NTD) among births of 4.6 per 10,000."

Neural Tube Closure

Mouse mutant showing neural tube closure defect

Dysraphism is the term often used to describe the defective fusion of the neural folds. The position and degree of failure of fusion will result in either embryonic death or a range of different neural defects. The way (mode) in which the human neural tube fuses has been a source of contention. In humans, fusion appears to initiate at multiple sites along the neural groove[7][8], this mode of closure may differ from that found in many animal species used in developmental studies.

Human Embryonic Death:

  • 5 weeks with total failure of fusion.
  • 6.5 weeks with opening over the rhombencephalon.
  • survive beyond 7 weeks with a defect at the frontal and parietal regions.

Mouse posterior neuropore Axd mutant.jpg

Mouse posterior neuropore Axd mutant[9]


Links: Folic Acid and Neural Tube Defects

Spina Bifida

Spina Bifida and Anencephaly (dorsal view)
 ICD-11 LA02 Spina bifida
  • LA02.0 Spina bifida cystica - failure of the neural tube to correctly develop during the antenatal period. This condition is characterized by nerve damage and the presence of meningoceles on the back. This condition may present with physical or mental impairment.
  • LA02.00 Myelomeningocele with hydrocephalus - failure of the neural tube to correctly develop during the antenatal period. This condition is characterized by nerve damage and hydrocephalus. This condition may also present with syringomyelia, hip dislocation, headache, nausea, vomiting, blurry vision, balance problems, bladder control problems, meningitis, or mental impairment.
  • LA02.01 Myelomeningocele without hydrocephalus - failure of the neural tube to close completely during fetal development. This condition is characterized by nerve damage. This condition may also present with syringomyelia, hip dislocation, headache, nausea, vomiting, blurry vision, balance problems, bladder control problems, meningitis, or mental impairment.
  • LA02.02 Myelocystocele - failure of the neural tube to close completely during fetal development. The condition is characterized by skin covered lumbosacral masses, an arachnoid lined meningocele that is directly continuous with the spinal subarachnoid space, and a low lying hydromyelic spinal cord that traverses the meningocele and expands into a large terminal cyst. This condition can present with neural damage and consequent impairment of function below the site of the myelocystocele.
  • LA02.1 Spina bifida aperta - failure of the neural tube to correctly develop during the antenatal period. This condition is characterized by nerve damage originating from a known location in the spine, signified by the presence of a meningocele or myelomeningocele. This condition may present with physical or mental impairment.
spina bifida | neural abnormalities |  ICD-11


(Meningomyelocele)

Double meningomyelocele.jpg Double meningomyelocele[10]
Neural tube defect meningomyelocele.jpg Spina Bifida Rates (USA Data)
Meningomyelocele Spina Bifida Rates (USA Data)

There are potentially many different causes of spina bifida and neural tube defects. The basis of the abnormality is a failure of the neural tube to close caudally. At least one known cause is a low maternal diet of folic acid (folate) containing foods. (see Neural Tube Defects and Low Folic Acid or Folic Acid below)

Neural tube defects from failure to close can be screened by amniocentesis or ultrasound.

Alpha-fetoproetin normally present in the CSF, leaks and can be detected in amniotic fluid.


Neural Tube Defects - Potential risk factors
Maternal nutrition Alcohol use, Caffeine use, low folic acid, Low dietary quality, Elevated glycaemic load or index, Low methionine intake, Low serum choline level, Low serum vitamin B12 level, Low vitamin C level, Low zinc intake
Other maternal factors smoking, maternal hyperthermia, Low socio-economic status, Maternal infections and illnesses (TORCH, viral infection, bacterial infection, fungal infection), maternal diabetes, Pregestational obesity, Psychosocial stress, Valproic acid use
Environmental factors Ambient air pollution, Disinfectant by-products in drinking water, Indoor air pollution, Nitrate-related compounds, Organic solvents, Pesticides, Polycyclic aromatic hydrocarbons
Table data from review[11] (see original review for references)


Links: neural tube defect

Cephalic Disorders

Cephalic (Greek, kephale = head) are a large group of abnormalities that relate to both skeletal (skull) and neural (brain) associated defects including: anencephaly, hydrocephalus, encephalocele, colpocephaly (occipital horn enlargement), lissencephaly (smooth brain), porencephaly (cyst or cavity in cerebral hemisphere), acephaly (absence of head), exencephaly (brain outside skull), macrocephaly (large head), micrencephaly (small brain), otocephaly (absence of lower jaw), brachycephaly (premature fusion of coronal suture), oxycephaly (premature fusion of coronal suture + other), plagiocephaly (premature unilateral fusion of coronal or lambdoid sutures), scaphocephaly (premature fusion of sagittal suture), trigonocephaly.

Microcephaly

Skull microcephaly
Skull - microcephaly

Template:ICD11 LA05.0 Microcephaly - A condition caused by failure of the head to correctly develop during the antenatal period. This condition is characterized by a head size that is significantly smaller than normal for their age and sex. This condition may also present with developmental delays, difficulties with balance and coordination, short stature, hyperactivity, mental retardation, seizures, or other neurological abnormalities.

A recent Spanish study[12] has shown a concordance between a head circumference growth function and intellectual disability in relation with the cause of microcephaly.

"3,269 head circumference (HC) charts of patients from a tertiary neuropediatric unit were reviewed and 136 microcephalic participants selected. Using the Z-scores of registered HC measurements we defined the variables: HC Minimum, HC Drop and HC Catch-up. We classified patients according to the presence or absence of intellectual disability (IQ below 71) and according to the cause of microcephaly (idiopathic, familial, syndromic, symptomatic and mixed). Using Discriminant Analysis a C-function was defined as C=HC Minimum + HC Drop with a cut-off level of C=-4.32 Z-score. In our sample 95% of patients scoring below this level, severe microcephaly, were classified in the disabled group while the overall concordance was 66%. In the symptomatic-mixed group the concordance between HC function and outcome reached 82% in contrast to only 54% in the idiopathic-syndromic group (P-value=0.0002)."

Microlissencephaly MRI-01.jpg

Microlissencephaly (MRI)[13] - Zika virus associated lishencephaly (smooth brain) in combination with severe congenital microcephaly (small head).

Anencephaly

Anencephaly Rates (USA Data)[14]

A neural tube defect, anencephaly is a failure of the neural tube to close cranially. Also called exencephaly or craniorachischisis. (More? anencephaly)


 ICD-11 LA00.0 Anencephaly 
a neural tube defect, characterized by the total or partial absence of the cranial vault and the covering skin, the brain being missing or reduced to a small mass. Most cases are stillborn, although some infants have been reported to survive for a few hours. In most cases autopsy findings reveal absence of adrenal glands. Anencephaly is likely to be multifactorial, the result of gene-environment interactions. Familial cases with a seemingly autosomal recessive mode of inheritance have been described but most cases are sporadic. Folic acid and zinc deficiencies, as well as maternal obesity, have been shown to be risk factors.
  • LA00.1 Iniencephaly - a rare form of neural tube defect in which a malformation of the cervico-occipital junction is associated with a malformation of the central nervous system. The cardinal features are occipital bone defect, partial or total absence of cervicothoracic vertebrae, fetal retroflexion of the head and characteristic absence of the neck. It is associated with malformations of the central nervous (spina bifida and/or anencephaly), gastrointestinal (omphalocele) and cardiovascular systems.
  • LA00.2 Acephaly
  • LA00.3 Amyelencephaly - Amyelencephaly is the absence of both the brain and spinal cord.

LA01 Cephalocele - failure of the skull to correctly close during the antenatal period. This condition is characterized by herniation of the meninges. This condition may present with herniation of brain, or developmental delay. Confirmation is through observation of herniated meninges by imaging.

anencephaly | neural abnormalities |  ICD-11
Fetal Anencephaly
Spina bifida front.JPG ZAnencephaly.jpg
ventral view lateral view

Anencephaly ultrasound.jpg

Anencephaly in a fetus (GA week 18) from a diabetic mother. Ultrasound images (coronal) show a complete absence of the cranial vault and brain and enlarged orbits.[15]


Links: anencephaly | maternal diabetes

Cerebral Anomalies

 ICD-11 LA05 Cerebral structural developmental anomalies
  • LA05.0 Microcephaly - failure of the head to correctly develop during the antenatal period. This condition is characterized by a head size that is significantly smaller than normal for their age and sex. This condition may also present with developmental delays, difficulties with balance and coordination, short stature, hyperactivity, mental retardation, seizures, or other neurological abnormalities.
  • LA05.1 Megalencephaly - failure of the brain to correctly develop during the antenatal period. This condition is characterized by increased size or weight of an otherwise correctly formed brain. This condition may also present with seizures, motor deficits, mental retardation and mild cognitive impairment.
  • LA05.2 Holoprosencephaly - brain malformation resulting from incomplete cleavage of the prosencephalon, occurring between the 18th and the 28th day of gestation and affecting both the forebrain and the face. In most of the cases, facial anomalies are observed: cyclopia, proboscis and median or bilateral cleft lip/palate in severe forms, and ocular hypotelorism or solitary median maxillary central incisor in minor forms. These latter midline defects can occur without the cerebral malformations (microforms). Children with HPE have many medical problems: developmental delay and feeding difficulties, epilepsy, and instability of temperature, heart rate and respiration. Endocrine disorders like diabetes insipidus, adrenal hypoplasia, hypogonadism, thyroid hypoplasia and growth hormone deficiency are frequent.
  • LA05.3 Corpus callosum agenesis - the most common brain malformation and is characterized by total or partial absence of the main interhemispheric commissure, the corpus callosum.
  • LA05.4 Arhinencephaly - failure of the olfactory organs to correctly develop during the antenatal period. This condition is characterized by absence of the olfactory bulbs and tracts.
  • LA05.5 Abnormal neuronal migration - any condition caused by abnormal migration of neuronal cells during the antenatal period. These conditions may present with poor muscle tone and motor function, seizures, developmental delays, mental retardation, failure to grow and thrive, difficulties with feeding, swelling in the extremities or microcephaly.
  • LA05.6 Encephaloclastic disorders
  • LA05.7 Brain cystic malformations - A disease caused by expansion of the roof plate of the brain vesicle, or by extraaxial structures such as an arachnoid membrane or migrating ependymal cells. This disease is characterized by the presence of fluid filled cysts in the brain. This disease may present with asymmetry of the skull, brain compression, raised intracranial pressure, hydrocephalus, bleeding or seizures. This disease may also be asymptomatic. Confirmation is through observation of intracerebral cysts by imaging.
cerebral anomalies | neural abnormalities |  ICD-11

Holoprosencephaly

 ICD-11 LA05 Cerebral structural developmental anomalies - LA05.2 Holoprosencephaly

Below are shown images of fetal holoprosencephaly and associated cyclopia.[16]

Human holoprosencephaly cyclopia dissection.jpg Proboscis histology.jpg
Human holoprosencephaly cyclopean dissection Proboscis histology

Hydrocephalus

Hydrocephalus (historic image from Hess, 1922)

This is a defect of cerebrospinal fliud (CSF) flow, excess fluid production or impaired fluid absorption and can be congenital or acquired. Estimated incidence of 1 in 1000 live births the condition leads to enlarged ventricles and head, separated skull cranial sutures and fontanelles. Obstruction of CSF flow can occur at any time (prenatally or postnatally) and leads to accumulation of within the ventricles. The time of onset will have different effects and should be compared to the equilivant neurological events that are occuring.

Ventricular obstruction usually occurs at the level of the cerebral aqueduct (narrowest site), but can occur elsewhere, and can be caused by viral infection or zoonotic disease.


Links: Congenital Hydrocephalus

Dandy Walker malformation

Dandy Walker malformation (MRI)[17] is another form of ventricular abnormality that affects cerebellar development.

The vermis of the cerebellum can be small or absent, the fourth ventricle enlarges due to cyst formation.

An ultrasound study[18] of fetuses with Dandy-Walker malformation 13 to 16 weeks (GA 15-18 weeks) identified the fourth ventricle widely open posteriorly, even in the standard transcerebellar view, and the brainstem-vermis (BV) angle was > 45°, significantly increased compared to that in normal fetuses (P < 0.0001). Note that at this age, an open fourth ventricle can also found in about 10% of normal fetuses.

Named after Walter Dandy (1886 – 1946) and Arthur Earl Walker (1907 – 1995), two USA neurosurgeons.

US Dandy-Walker 01.jpg
 ‎‎Dandy-Walker
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Dandy Walker malformation MRI 01.jpg
Links: Congenital Hydrocephalus

Encephalocele

This defect is generally mesodermal in origin, leading to protrusion of brain and meninges outside the crainal cavity. The severity of the disorder can vary dependent upon the degree of mesodermal abnormality.

Encephalitis

Normally a postnatal clinical syndrome of the central nervous system resulting in inflammation of the brain parenchyma and caused by a range of pathogens (viral, bacterial and protozoal infections). This infectious disease is due mainly to viral pathogens: Herpes simplex encephalitis 10%–20% of cases, Murray Valley encephalitis virus, Japanese encephalitis virus, Australian bat lyssavirus, West Nile virus, Hendra virus and Nipah virus. The pathogen is generally included in the specific encephalitis naming; Varicella encephalitis and Toxoplasma meningoencephalitis.

Links: neural abnormalities | TORCH | PMC2700670)

Fragile X Syndrome

Fragile X Syndrome (FXS) is the most common form of inherited mental retardation and autism. The condition is caused by a loss of the functional fragile X mental retardation protein (FMRP) an RNA-binding protein that can regulate the translation of specific mRNAs. There are several suggested additional roles for this protein including synaptic development and function[19] and in adult neurogenesis.[20]


Links: Fragile X Syndrome

Autism

Autism (autism spectrum disorder, ASD) is a behaviourally defined brain disorder in children. Features include: impoverished verbal and non-verbal communication skills, reduced social interactions (bias their attention towards objects rather than the surrounding social situation), behavioural impairments in attention engagement/disengagement, poor emotional discrimination and facial recognition, and fail to response to their own names. There exist many different and unproven claims as to the origins of autism.

Developmentally associated with neural maturation changes in cortical thickness and organization, and particularly affecting pyramidal neurons. A rat model shows structural and behavioural features of autism as a result of altering the trajectory of early postnatal cortical development.[21]

Links: Neural Exam Movies

Rett Syndrome

(RTT) A severe neurodevelopment disorder, with intellectual disability and abnormalities of movement, mainly caused by mutations in the X-linked (Xq28) Methyl-CpG-binding protein 2 (MECP2) gene and therefore almost exclusively in females. The congenital variant of Rett syndrome is caused by heterozygous mutation in the FOXG1 gene on chromosome 14q13.

Links: OMIM - Congenital Rett syndrome | OMIM - Rett syndrome

Cerebral Palsy

 ICD-11 Cerebral palsy
8D20 Spastic cerebral palsy - characterized by increased muscle tone associated with hyperactive muscle stretch reflexes (deep tendon reflexes) and an increase in resistance to rapid muscle stretch. Extensor plantar responses are commonly present.
  • 8D20.0 Spastic unilateral cerebral palsy - a form of cerebral palsy in which the spasticity is confined to one side; it is often accompanied by cortical sensory impairment and varying degrees of hemineglect, demonstrable by testing stereognosis and graphesthesia. Early hand preference is often the first sign of this disorder, and may be apparent in the first months of life.
  • 8D20.1 Spastic bilateral cerebral palsy
  • 8D21 Dyskinetic cerebral palsy - (extrapyramidal cerebral palsy) is characterized by impairment of voluntary movement because of the presence of interfering involuntary movements, and inappropriate co-contraction of agonist and antagonist muscles (dystonia). This group of disorders includes choreoathetotic cerebral palsy and dystonic cerebral palsy. The former is characterized by large amplitude, involuntary movements of mainly distal limbs(athetosis) with or without small amplitude, fleeting, asymmetric contractions of individual muscle groups (chorea). Dystonic cerebral palsy predominantly affects proximal trunk and limb muscles, which may show slow, persistent movements, leading to the adoption of unusual postures, such as torticollis.
  • 8D22 Ataxic cerebral palsy - dominated by signs of cerebellar dysfunction, including hypotonia, ataxia, dysdiadochokinesis, dysmetria, dysarthria and nystagmus. Reflexes may be pendular, although there are often also signs of spasticity.
  • 8D23 Worster-Drought syndrome - a form of cerebral palsy characterized by congenital pseudobulbar (suprabulbar) paresis manifesting as selective weakness of the lips, tongue and soft palate, dysphagia, dysphonia, drooling and jaw jerking.
International Classification of Diseases - Cerebral palsy 
 ICD-11 Cerebral palsy
8D20 Spastic cerebral palsy - characterized by increased muscle tone associated with hyperactive muscle stretch reflexes (deep tendon reflexes) and an increase in resistance to rapid muscle stretch. Extensor plantar responses are commonly present.
  • 8D20.0 Spastic unilateral cerebral palsy - a form of cerebral palsy in which the spasticity is confined to one side; it is often accompanied by cortical sensory impairment and varying degrees of hemineglect, demonstrable by testing stereognosis and graphesthesia. Early hand preference is often the first sign of this disorder, and may be apparent in the first months of life.
  • 8D20.1 Spastic bilateral cerebral palsy
  • 8D21 Dyskinetic cerebral palsy - (extrapyramidal cerebral palsy) is characterized by impairment of voluntary movement because of the presence of interfering involuntary movements, and inappropriate co-contraction of agonist and antagonist muscles (dystonia). This group of disorders includes choreoathetotic cerebral palsy and dystonic cerebral palsy. The former is characterized by large amplitude, involuntary movements of mainly distal limbs(athetosis) with or without small amplitude, fleeting, asymmetric contractions of individual muscle groups (chorea). Dystonic cerebral palsy predominantly affects proximal trunk and limb muscles, which may show slow, persistent movements, leading to the adoption of unusual postures, such as torticollis.
  • 8D22 Ataxic cerebral palsy - dominated by signs of cerebellar dysfunction, including hypotonia, ataxia, dysdiadochokinesis, dysmetria, dysarthria and nystagmus. Reflexes may be pendular, although there are often also signs of spasticity.
  • 8D23 Worster-Drought syndrome - a form of cerebral palsy characterized by congenital pseudobulbar (suprabulbar) paresis manifesting as selective weakness of the lips, tongue and soft palate, dysphagia, dysphonia, drooling and jaw jerking.

Cerebral palsy is a group of disorders in motor impairment that limits activity, and is attributed to non-progressive disturbances during brain development in fetuses or infants (3-4 / 1,000).[22]

Represented by one or more of these features:

  • impaired cognition
  • impaired communication
  • impaired sensory perception
  • behavioural abnormalities
  • seizure disorders


Links: Neural Exam Movies | Medline Plus | NINDS - Info | CDC Screening and Diagnosis | Cerebral Palsy Alliance

Newborn Neural Exam

Neural - The collapsed tables below link to a number of short videos that demonstrate simple assessments of the postnatal developing nervous system.

3 Months Normal Neural Exam Movies

Normal 3 Month Neural Exam Movies
Normal 3 Month Neural Exam Movies

The neuromuscular system can be initially assessed by 6 quick tests (posture, square window, arm recoil, popliteal angle, scarf sign and heel to ear). The following short videos show clinical examination of these assessments and a number of reflexes.

Later developmental assessment includes behaviour, reflexes (primitive and postural), muscular tone, and motor (gross, fine, co-ordination).


3 Months Neural Exam Movies: normal behaviour | cranial nerves | Tone - head and trunk control | upper extremity‎ | hand movements | lower extremity Positions - supine | prone | ventral suspension | vertical suspension | Reflexes - Deep tendon reflexes | Plantar reflex‎ | Root | Moro | Galant | Grasp | Asymmetric tonic neck‎ | Neural Exam Movies | Neonatal Diagnosis

3 Month Behaviour

03mo 04.jpg
 ‎‎Behaviour
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03mo 02.jpg
 ‎‎Cranial Nerves
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normal behaviour cranial nerves

3 Month Tone

03mo 03.jpg
 ‎‎Control
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03mo 03.jpg
 ‎‎Tone Upper Limb
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03mo 04.jpg
 ‎‎Hand Movements
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03mo 05.jpg
 ‎‎Lower Limb
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3 Month Positions

03mo 04.jpg
 ‎‎Supine Position
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03mo 04.jpg
 ‎‎Prone Position
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03mo 04.jpg
 ‎‎Ventral Suspension
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03mo 04.jpg
 ‎‎Vertical Suspension
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3 Month Reflexes

03mo 04.jpg
 ‎‎Deep Tendon
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03mo 04.jpg
 ‎‎Plantar Reflex
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03mo 04.jpg
 ‎‎Suck-Root
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03mo 04.jpg
 ‎‎Moro Reflex
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03mo 04.jpg
 ‎‎Galant Reflex
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03mo 04.jpg
 ‎‎Grasp Reflex
Page | Play
03mo 04.jpg
 ‎‎Neck Tone
Page | Play
3 Months Neural Exam Movies: normal behaviour | cranial nerves | Tone - head and trunk control | upper extremity‎ | hand movements | lower extremity Positions - supine | prone | ventral suspension | vertical suspension | Reflexes - Deep tendon reflexes | Plantar reflex‎ | Root | Moro | Galant | Grasp | Asymmetric tonic neck‎ | Neural Exam Movies | Neonatal Diagnosis
Normal 12 Month Neural Exam Movies  
Normal 12 Month Neural Exam Movies

The following short videos show clinical examination of neural development assessments and a number of reflexes.


12 Months Neural Exam Movies: Shy | Social and Language | Cranial Nerves | Tone - Tone | Reflexes - deep tendon reflexes | plantar reflex‎ | Parachute | Pincer Grasp | Beads in the Cup | Play Ball | Transition in and out of Sitting | Creeping | Stoop and Recover | Motor/Gait - Stand, Walks with Support | Toddler’s Gait | Head Circumference | Neural Exam Movies
Normal 18 Month Neural Exam Movies
Normal 18 Month Neural Exam Movies

The following short videos show clinical examination of neural development assessments and a number of reflexes.


Neural Exam Movies: Wants | Understanding | Points to Pictures | Points to Body Parts | Cranial Nerves Motor/Coordination - Blocks in Cup | Beads in Cup | Stacking Blocks | Pincer Grasp and Handedness | Drawing/Scribbling | Tone | Deep Tendon Reflex | Throwing Ball | Walking
Normal 30 Month Neural Exam Movies  
Normal 30 Month Neural Exam Movies

The following short videos show clinical examination of neural development assessments and a number of reflexes.


Neural Exam Movies: Establishing Relationship | Follows Commands | Points to Pictures | Names Pictures | Response to Questions | Pointing to and Naming Body Parts | Motor/Coordination - Using Puppets | Using Measuring Tape | Block Tower | Drawing | Tone | Deep Tendon Reflex | Kicking and Throwing a Ball | Walking, Running


Links: Neural Exam Movies | Neural Exam Movies -Newborn Abnormal

International Classification of Disease

International Classification of Diseases  ICD-11 Neural developmental anomalies 
 ICD-11 LA00-LA0Z Structural developmental anomalies of the nervous system
  • LA00.0 Anencephaly - a neural tube defect, characterized by the total or partial absence of the cranial vault and the covering skin, the brain being missing or reduced to a small mass. Most cases are stillborn, although some infants have been reported to survive for a few hours. In most cases autopsy findings reveal absence of adrenal glands. Anencephaly is likely to be multifactorial, the result of gene-environment interactions. Familial cases with a seemingly autosomal recessive mode of inheritance have been described but most cases are sporadic. Folic acid and zinc deficiencies, as well as maternal obesity, have been shown to be risk factors.
  • LA00.1 Iniencephaly - a rare form of neural tube defect in which a malformation of the cervico-occipital junction is associated with a malformation of the central nervous system. The cardinal features are occipital bone defect, partial or total absence of cervicothoracic vertebrae, fetal retroflexion of the head and characteristic absence of the neck. It is associated with malformations of the central nervous (spina bifida and/or anencephaly), gastrointestinal (omphalocele) and cardiovascular systems.
  • LA00.2 Acephaly
  • LA00.3 Amyelencephaly - Amyelencephaly is the absence of both the brain and spinal cord.
  • LA01 Cephalocele - failure of the skull to correctly close during the antenatal period. This condition is characterized by herniation of the meninges. This condition may present with herniation of brain, or developmental delay. Confirmation is through observation of herniated meninges by imaging.
  • LA02 Spina bifida -
    • LA02.0 Spina bifida cystica - failure of the neural tube to correctly develop during the antenatal period. This condition is characterized by nerve damage and the presence of meningoceles on the back. This condition may present with physical or mental impairment.
    • LA02.00 Myelomeningocele with hydrocephalus - failure of the neural tube to correctly develop during the antenatal period. This condition is characterized by nerve damage and hydrocephalus. This condition may also present with syringomyelia, hip dislocation, headache, nausea, vomiting, blurry vision, balance problems, bladder control problems, meningitis, or mental impairment.
    • LA02.01 Myelomeningocele without hydrocephalus - failure of the neural tube to close completely during fetal development. This condition is characterized by nerve damage. This condition may also present with syringomyelia, hip dislocation, headache, nausea, vomiting, blurry vision, balance problems, bladder control problems, meningitis, or mental impairment.
    • LA02.02 Myelocystocele - failure of the neural tube to close completely during fetal development. The condition is characterized by skin covered lumbosacral masses, an arachnoid lined meningocele that is directly continuous with the spinal subarachnoid space, and a low lying hydromyelic spinal cord that traverses the meningocele and expands into a large terminal cyst. This condition can present with neural damage and consequent impairment of function below the site of the myelocystocele.
    • LA02.1 Spina bifida aperta - failure of the neural tube to correctly develop during the antenatal period. This condition is characterized by nerve damage originating from a known location in the spine, signified by the presence of a meningocele or myelomeningocele. This condition may present with physical or mental impairment.
  • LA03 Arnold-Chiari malformation type II - failure of the brain and spinal cord to correctly develop during the antenatal period. This condition is characterized by extension of both cerebellar and brain stem tissue into the foramen magnum. This condition may present with partial or complete absence of the cerebellar vermis, myelomeningocele, neck pain, balance problems, muscle weakness, limb numbness, dizziness, vision problems, difficulty swallowing, ringing in the ears, hearing loss, vomiting, insomnia, depression, or impairment of motor skills.
  • LA04 Congenital hydrocephalus - failure of the brain to correctly develop during the antenatal period. This condition is characterized by a rapid increase in head circumference or an unusually large head size due to excessive accumulation of cerebrospinal fluid in the brain. This condition may also present with vomiting, sleepiness, irritability, downward deviation of the eyes, or seizures. Confirmation is through observation of cerebrospinal fluid within cerebral ventricles by imaging.
  • LA05 Cerebral structural developmental anomalies
    • LA05.0 Microcephaly
    • LA05.1 Megalencephaly
    • LA05.2 Holoprosencephaly
    • LA05.3 Corpus callosum agenesis
    • LA05.4 Arhinencephaly
    • LA05.5 Abnormal neuronal migration
    • LA05.6 Encephaloclastic disorders
    • LA05.7 Brain cystic malformations
  • LA06 Cerebellar structural developmental anomalies
    • LA06.0 Dandy-Walker malformation
    • LA06.1 Hypoplasia or agenesis of cerebellar hemispheres
    • LA06.2 Focal cerebellar dysplasia
  • LA07 Structural developmental anomalies of the neurenteric canal, spinal cord or vertebral column
International Classification of Diseases - Spina bifida 
 ICD-11 LA02 Spina bifida
  • LA02.0 Spina bifida cystica - failure of the neural tube to correctly develop during the antenatal period. This condition is characterized by nerve damage and the presence of meningoceles on the back. This condition may present with physical or mental impairment.
  • LA02.00 Myelomeningocele with hydrocephalus - failure of the neural tube to correctly develop during the antenatal period. This condition is characterized by nerve damage and hydrocephalus. This condition may also present with syringomyelia, hip dislocation, headache, nausea, vomiting, blurry vision, balance problems, bladder control problems, meningitis, or mental impairment.
  • LA02.01 Myelomeningocele without hydrocephalus - failure of the neural tube to close completely during fetal development. This condition is characterized by nerve damage. This condition may also present with syringomyelia, hip dislocation, headache, nausea, vomiting, blurry vision, balance problems, bladder control problems, meningitis, or mental impairment.
  • LA02.02 Myelocystocele - failure of the neural tube to close completely during fetal development. The condition is characterized by skin covered lumbosacral masses, an arachnoid lined meningocele that is directly continuous with the spinal subarachnoid space, and a low lying hydromyelic spinal cord that traverses the meningocele and expands into a large terminal cyst. This condition can present with neural damage and consequent impairment of function below the site of the myelocystocele.
  • LA02.1 Spina bifida aperta - failure of the neural tube to correctly develop during the antenatal period. This condition is characterized by nerve damage originating from a known location in the spine, signified by the presence of a meningocele or myelomeningocele. This condition may present with physical or mental impairment.
spina bifida | neural abnormalities |  ICD-11
International Classification of Diseases - Anencephaly 
 ICD-11 LA00.0 Anencephaly 
a neural tube defect, characterized by the total or partial absence of the cranial vault and the covering skin, the brain being missing or reduced to a small mass. Most cases are stillborn, although some infants have been reported to survive for a few hours. In most cases autopsy findings reveal absence of adrenal glands. Anencephaly is likely to be multifactorial, the result of gene-environment interactions. Familial cases with a seemingly autosomal recessive mode of inheritance have been described but most cases are sporadic. Folic acid and zinc deficiencies, as well as maternal obesity, have been shown to be risk factors.
  • LA00.1 Iniencephaly - a rare form of neural tube defect in which a malformation of the cervico-occipital junction is associated with a malformation of the central nervous system. The cardinal features are occipital bone defect, partial or total absence of cervicothoracic vertebrae, fetal retroflexion of the head and characteristic absence of the neck. It is associated with malformations of the central nervous (spina bifida and/or anencephaly), gastrointestinal (omphalocele) and cardiovascular systems.
  • LA00.2 Acephaly
  • LA00.3 Amyelencephaly - Amyelencephaly is the absence of both the brain and spinal cord.

LA01 Cephalocele - failure of the skull to correctly close during the antenatal period. This condition is characterized by herniation of the meninges. This condition may present with herniation of brain, or developmental delay. Confirmation is through observation of herniated meninges by imaging.

anencephaly | neural abnormalities |  ICD-11
International Classification of Diseases - Cerebral structural developmental anomalies 
 ICD-11 LA05 Cerebral structural developmental anomalies
  • LA05.0 Microcephaly - failure of the head to correctly develop during the antenatal period. This condition is characterized by a head size that is significantly smaller than normal for their age and sex. This condition may also present with developmental delays, difficulties with balance and coordination, short stature, hyperactivity, mental retardation, seizures, or other neurological abnormalities.
  • LA05.1 Megalencephaly - failure of the brain to correctly develop during the antenatal period. This condition is characterized by increased size or weight of an otherwise correctly formed brain. This condition may also present with seizures, motor deficits, mental retardation and mild cognitive impairment.
  • LA05.2 Holoprosencephaly - brain malformation resulting from incomplete cleavage of the prosencephalon, occurring between the 18th and the 28th day of gestation and affecting both the forebrain and the face. In most of the cases, facial anomalies are observed: cyclopia, proboscis and median or bilateral cleft lip/palate in severe forms, and ocular hypotelorism or solitary median maxillary central incisor in minor forms. These latter midline defects can occur without the cerebral malformations (microforms). Children with HPE have many medical problems: developmental delay and feeding difficulties, epilepsy, and instability of temperature, heart rate and respiration. Endocrine disorders like diabetes insipidus, adrenal hypoplasia, hypogonadism, thyroid hypoplasia and growth hormone deficiency are frequent.
  • LA05.3 Corpus callosum agenesis - the most common brain malformation and is characterized by total or partial absence of the main interhemispheric commissure, the corpus callosum.
  • LA05.4 Arhinencephaly - failure of the olfactory organs to correctly develop during the antenatal period. This condition is characterized by absence of the olfactory bulbs and tracts.
  • LA05.5 Abnormal neuronal migration - any condition caused by abnormal migration of neuronal cells during the antenatal period. These conditions may present with poor muscle tone and motor function, seizures, developmental delays, mental retardation, failure to grow and thrive, difficulties with feeding, swelling in the extremities or microcephaly.
  • LA05.6 Encephaloclastic disorders
  • LA05.7 Brain cystic malformations - A disease caused by expansion of the roof plate of the brain vesicle, or by extraaxial structures such as an arachnoid membrane or migrating ependymal cells. This disease is characterized by the presence of fluid filled cysts in the brain. This disease may present with asymmetry of the skull, brain compression, raised intracranial pressure, hydrocephalus, bleeding or seizures. This disease may also be asymptomatic. Confirmation is through observation of intracerebral cysts by imaging.
cerebral anomalies | neural abnormalities |  ICD-11
International Classification of Diseases - Cerebral palsy 
 ICD-11 Cerebral palsy
8D20 Spastic cerebral palsy - characterized by increased muscle tone associated with hyperactive muscle stretch reflexes (deep tendon reflexes) and an increase in resistance to rapid muscle stretch. Extensor plantar responses are commonly present.
  • 8D20.0 Spastic unilateral cerebral palsy - a form of cerebral palsy in which the spasticity is confined to one side; it is often accompanied by cortical sensory impairment and varying degrees of hemineglect, demonstrable by testing stereognosis and graphesthesia. Early hand preference is often the first sign of this disorder, and may be apparent in the first months of life.
  • 8D20.1 Spastic bilateral cerebral palsy
  • 8D21 Dyskinetic cerebral palsy - (extrapyramidal cerebral palsy) is characterized by impairment of voluntary movement because of the presence of interfering involuntary movements, and inappropriate co-contraction of agonist and antagonist muscles (dystonia). This group of disorders includes choreoathetotic cerebral palsy and dystonic cerebral palsy. The former is characterized by large amplitude, involuntary movements of mainly distal limbs(athetosis) with or without small amplitude, fleeting, asymmetric contractions of individual muscle groups (chorea). Dystonic cerebral palsy predominantly affects proximal trunk and limb muscles, which may show slow, persistent movements, leading to the adoption of unusual postures, such as torticollis.
  • 8D22 Ataxic cerebral palsy - dominated by signs of cerebellar dysfunction, including hypotonia, ataxia, dysdiadochokinesis, dysmetria, dysarthria and nystagmus. Reflexes may be pendular, although there are often also signs of spasticity.
  • 8D23 Worster-Drought syndrome - a form of cerebral palsy characterized by congenital pseudobulbar (suprabulbar) paresis manifesting as selective weakness of the lips, tongue and soft palate, dysphagia, dysphonia, drooling and jaw jerking.
ICD-11 Conditions with disorders of intellectual development as a relevant clinical feature  
LD90 Conditions with disorders of intellectual development as a relevant clinical feature  
  • LD90.0 Angelman syndrome  
  • LD90.1 Early-onset parkinsonism - intellectual deficit  
  • LD90.2 Pelizaeus-Merzbacher-like disease  
  • LD90.3 Prader-Willi syndrome  
  • LD90.4 Rett syndrome  
  • 5C55.01 Lesch-Nyhan syndrome  
  • LA04.0 Hydrocephalus with stenosis of the aqueduct of Sylvius  
  • 8A44.0 Pelizaeus-Merzbacher disease  
  • 8C21.2 Hereditary sensory or autonomic neuropathy type IV  
  • LD20.00 Joubert syndrome  
  • 5C50.0 Phenylketonuria  
  • 5C50.12 Tyrosinaemia type 2  
  • 5C50.A1 Carbamoylphosphate synthetase deficiency  
  • 5C50.F1 Carnosinaemia  
  • 5C50.F2 Homocarnosinosis  
  • LD20.1 Syndromes with lissencephaly as a major feature  
  • 5C52.03 Sjögren-Larsson syndrome  
  • LA05.50 Polymicrogyria  
  • LA05.60 Porencephaly  
  • 5C53.02 Pyruvate dehydrogenase complex deficiency  
  • CB04.5 Brain-lung-thyroid syndrome  
  • 5C56.02 Metachromatic leukodystrophy  
  • 5C56.1 Neuronal ceroid lipofuscinosis  
  • 5C56.31 Mucopolysaccharidosis type 2  
  • 5C56.33 Mucopolysaccharidosis type 6  
  • 5C60.0 Oculocerebrorenal syndrome  
  • LD44.N0 CATCH 22 phenotype  
  • LD24.80 Langer-Giedion syndrome  
  • 5C58.00 Crigler-Najjar syndrome  
  • LD55 Fragile X chromosome  
  • LD27.00 Incontinentia pigmenti  
  • LD2D.2 Tuberous sclerosis  
  • LD2F.15 Noonan syndrome  
  • KA62.8 Congenital rubella syndrome  
  • KA62.3 Congenital cytomegalovirus infection  
  • LD40.0 Complete trisomy 21  
  • LD50.31 Klinefelter syndrome, male with more than two X chromosomes  
LD90.Y Other specified conditions with disorders of intellectual development as a relevant clinical feature  
LD90.Z Conditions with disorders of intellectual development as a relevant clinical feature, unspecified  
LD9Y Other specified developmental anomalies

LD9Z Developmental anomalies, unspecified

neural abnormalities
ICD-11 Links:  ICD-11 | Anomalies one body system | Multiple anomalies or syndromes | Chromosomal anomalies | Intellectual development | Abnormal Development | Prenatal Diagnosis | Birth | Neonatal Diagnosis | ICD-10 | Reports | | Category:ICD-11
International Classification of Diseases  ICD-11 20 Developmental anomalies (beta draft)  
ICD-11 Beta Draft - NOT FINAL, updated on a daily basis, It is not approved by WHO, NOT TO BE USED for CODING except for agreed FIELD TRIALS.

Chapter 20 Developmental anomalies, only a few examples of the draft ICD-11 Beta coding and tree structure for "structural developmental anomalies" within this section are shown in the table below.

Mortality and Morbidity Statistics - 20 Developmental Anomalies  
Structural Developmental Anomalies  
  • Structural developmental anomalies of the nervous system  
    • LA00 Anencephaly or similar anomalies
    • LA01 Cephalocele
    • LA02 Spina bifida
    • LA03 Arnold-Chiari malformation type II
    • LA04 Congenital hydrocephalus
    • LA05 Cerebral structural developmental anomalies
    • LA06 Cerebellar structural developmental anomalies
    • LA07 Structural developmental anomalies of the neurenteric canal, spinal cord or vertebral column
    • LA0Y Other specified structural developmental anomalies of the nervous system
    • LA0Z Structural developmental anomalies of the nervous system, unspecified
  • Structural developmental anomalies of the eye, eyelid or lacrimal apparatus
    • LA10 Structural developmental anomalies of ocular globes
    • LA11 Structural developmental anomalies of the anterior segment of eye
    • LA30 Structural developmental anomalies of lens or zonula
    • LA31 Structural developmental anomalies of the posterior segment of eye
    • LA32 Structural developmental anomalies of eyelid, lacrimal apparatus or orbit
    • LA3Y Other specified structural developmental anomalies of the eye, eyelid or lacrimal apparatus
    • LA3Z Structural developmental anomalies of the eye, eyelid or lacrimal apparatus, unspecified
  • Structural developmental anomalies of the ear  
    • LA40 Structural anomaly of eustachian apparatus
    • LA41 Minor anomalies of pinnae
    • LA42 Structural developmental anomalies of ear causing hearing impairment
    • LA43 Otocephaly
    • LA44 Accessory auricle
    • LA4Y Other specified structural developmental anomalies of the ear
    • LA4Z Structural developmental anomalies of the ear, unspecified
  • Structural developmental anomalies of the face, mouth or teeth
    • LA50 Structural developmental anomalies of teeth and periodontal tissues
    • LA51 Structural developmental anomalies of mouth or tongue
    • Clefts of lip, alveolus or palate
    • LA70 Congenital velopharyngeal incompetence
    • LA71 Facial clefts
    • LA72 Facial asymmetry
    • LA73 Macrocheilia
    • LA74 Microcheilia
    • LA75 Compression facies
    • LA76 Pierre Robin syndrome
    • LC20 Dermoid cyst
    • LA7Y Other specified structural developmental anomalies of the face, mouth or teeth
    • LA7Z Structural developmental anomalies of the face, mouth or teeth, unspecified
  • Structural developmental anomalies of the neck  
  • Structural developmental anomalies of the respiratory system  
  • Structural developmental anomalies of the circulatory system  
    • Structural developmental anomaly of heart and great vessels
      • LB00 Congenital heart or great vessel related acquired abnormality
      • LB01 Congenital anomaly of atrioventricular or ventriculo-arterial connections
      • LB02 Congenital anomaly of the mediastinal veins Congenital anomaly of atria or atrial septum
      • LB20 Congenital anomaly of atrioventricular valves or septum
      • LB21 Congenital anomaly of ventricles and ventricular septum
      • LB22 Functionally univentricular heart
      • LB23 Congenital anomaly of ventriculo-arterial valves and adjacent regions
      • LB24 Congenital anomaly of great arteries including arterial duct
      • LB25 Anomalous position-orientation of heart
      • LB26 Total mirror imagery
      • LB27 Left isomerism
      • LB28 Congenital anomaly of coronary arteries
      • LB29 Structural developmental anomalies of the pericardium
      • LB2Y Other specified structural developmental anomaly of heart and great vessels
      • LB2Z Structural developmental anomaly of heart and great vessels, unspecified
    • LB30 Structural developmental anomalies of the peripheral vascular system
      • LB30.1 Capillary malformations
      • LB30.2 Lymphatic malformations
        • LB30.21 Macrocystic lymphatic malformation
        • LB30.22 Microcystic lymphatic malformation
        • LB30.23 Cystic hygroma in fetus
        • BD23.1 Primary lymphoedema
            • EK91 Yellow nail syndrome
            • LC5F.26 Noonan syndrome
        • LB30.2Y Other specified lymphatic malformations
        • LB30.2Z Lymphatic malformations, unspecified
      • LB30.3 Peripheral venous malformations
      • LB30.4 Peripheral arteriovenous malformations
      • LB30.5 Peripheral arterial malformations
      • LB30.6 Pulmonary arteriovenous fistula
      • LB30.Y Other specified structural developmental anomalies of the peripheral vascular system
      • LB30.Z Structural developmental anomalies of the peripheral vascular system, unspecified
    • LB3Y Other specified structural developmental anomalies of the circulatory system
    • LB3Z Structural developmental anomalies of the circulatory system, unspecified
  • Structural developmental anomalies of the diaphragm, abdominal wall or umbilical cord  
  • Structural developmental anomalies of the digestive tract  
  • Structural developmental anomalies of the liver, biliary tract, pancreas or spleen  
  • Structural developmental anomalies of the urinary system  
  • Structural developmental anomalies of the female genital system  
  • Structural developmental anomalies of the male genital system  
  • Structural developmental anomalies of the breast  
  • Structural developmental anomalies of the skeleton  
  • Structural developmental anomalies of the skin  
  • Structural developmental anomalies of the adrenal glands  
Multiple developmental anomalies or syndromes
Chromosomal anomalies, excluding gene mutations
Conditions with disorders of intellectual development as a relevant clinical feature
LD6Y Other specified developmental anomalies

LD6Z Developmental anomalies, unspecified

CD-11 Beta Draft - NOT FINAL, updated on a daily basis, It is not approved by WHO, NOT TO BE USED for CODING except for agreed FIELD TRIALS.


See also International Classification of Diseases
ICD-10

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Journals

Journal of Pediatric Neurosciences - is official publication of the Indian Society for Pediatric Neurosurgery.

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Cite this page: Hill, M.A. (2019, December 13) Embryology Neural System - Abnormalities. Retrieved from https://embryology.med.unsw.edu.au/embryology/index.php/Neural_System_-_Abnormalities

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