Schizencephaly can be distinguished from porencephaly by the fact that in schizencephaly the fluid-filled component, if present, is entirely lined by heterotopic grey matter while a porencephalic cyst is lined mostly by white matter. Individuals with clefts in both hemispheres, or bilateral clefts, are often developmentally delayed and have delayed speech and language skills and corticospinal dysfunction. Individuals with smaller, unilateral clefts (clefts in one hemisphere) may be weak or paralyzed on one side of the body and may have average or near-average intelligence. Patients with schizencephaly may also have varying degrees of microcephaly, Intellectual disability, hemiparesis (weakness or paralysis affecting one side of the body), or quadriparesis (weakness or paralysis affecting all four extremities), and may have reduced muscle tone (hypotonia). Most patients have seizures, and some may have hydrocephalus.

Schizencephaly () is a rare birth defect characterized by abnormal clefts lined with grey matter that form the ependyma of the cerebral ventricles to the pia mater. These clefts can occur bilaterally or unilaterally. Common clinical features of this malformation include epilepsy, motor deficits, and psychomotor retardation.

There are various symptoms of colpocephaly and patients can experience effects ranging from mild to severe. Some patients do not show most of the symptoms related to colpocephaly, such as psychomotor abnormalilities and agenesis of the corpus callosum. In some cases, signs appear later on in life and a significant number of children suffer only from minor disabilities.

The following list includes common symptoms of colpocephaly.

- partial or complete agenesis of the corpus callosum

- intellectual disability

- motor abnormalities

- visual defects such as, crossing of the eyes, missing visual fields, and optic nerve hypoplasia

- spasticity

- seizures

- cerebral palsy

Intracranial abnormalities include:

- Microcephaly

- Agenesis of the corpus callosum

- Meningomyelocele

- Lissencephaly

- Periventricular leukomalacia (PVL)

- Enlargement of the cisterna magna

- Cerebellar hypoplasia

Colpocephaly is characterized by disproportionately large occipital horns of the lateral ventricles (also frontal and temporal ventricles in some cases). MRI and CT scans of patients demonstrate abnormally thick gray matter with thin poorly myelinated white matter. This happens as a result of partial or complete absence of the corpus callosum. Corpus callosum is the band of white matter connecting the two cerebral hemispheres. The corpus callosum plays an extremely important role in interhemispheric communication, thus lack of or absence of these neural fibers results in a number of disabilities.

The lemon sign on CT scans of patients refers to the shape of the fetal skull when the frontal bones lose their normal convex contour and appear flattened or inwardly scalloped. This gives the skull a shape similar to that of a lemon. The sign is seen on transverse sonograms of the fetal cranium obtained at the level of the ventricles.

A special case is found in literature where lissencephaly, colpocephaly, and septal agenesis are all present together. The CT scans of the patient shows the ventricular system having a unique appearance of a crown of a king. This is referred to as the 'CROWN SIGN'.

Usually the cerebellum and brain stem are formed normally, although in some cases the cerebellum may also be absent. An infant with hydranencephaly may appear normal at birth or may have some distortion of the skull and upper facial features due to fluid pressure inside the skull. The infant's head size and spontaneous reflexes such as sucking, swallowing, crying, and moving the arms and legs may all seem normal, depending on the severity of the condition. However, after a few weeks the infant sometimes becomes irritable and has increased muscle tone (hypertonia). After several months of life, seizures and hydrocephalus may develop, if they did not exist at birth. Other symptoms may include visual impairment, lack of growth, deafness, blindness, spastic quadriparesis (paralysis), and intellectual deficits.

Some infants may have additional abnormalities at birth including seizures, myoclonus (involuntary sudden, rapid jerks), limited thermoregulation abilities, and respiratory problems.

Still other infants display no obvious symptoms at birth, going many months without a confirmed diagnosis of hydranencephaly. In some cases a severe hydrocephalus, or other cephalic condition, is misdiagnosed.

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.

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.

- Lissencephaly

- Microlissencephaly

- Schizencephaly

- Porencephaly

- Pachygyria

- Polymicrogyria

- Agyria

- Macrogyria

- Microgyria

- Micropolygyria

- Neuronal heterotopias

- Agenesis of the corpus callosum

- Agenesis of the cranial nerves

- Band heterotopias

Focal cortical dysplasia. Miller-Dieker syndrome, , Fukuyama congenital muscular dystrophy and Walker Warburg syndrome are genetic disorders associated with lissencephaly.

Hydranencephaly or hydrancephaly is a condition in which the brain's cerebral hemispheres are absent to varying degrees and the remaining cranial cavity is filled with cerebrospinal fluid.

Hydranencephaly (or hydrancephaly) is a type of cephalic disorder.

These disorders are congenital conditions that derive from either damage to, or abnormal development of, the fetal nervous system in the earliest stages of development in utero. Cephalic is the medical term for “head” or “head end of body.” These conditions do not have any definitive identifiable cause factor; instead generally attributed to a variety of hereditary or genetic conditions, but also by environmental factors such as maternal infection, pharmaceutical intake, or even exposure to high levels of radiation.

This should not be confused with hydrocephalus, which is an accumulation of excess cerebrospinal fluid in the ventricles of the brain.

In hemihydranencephaly, only half of the cranial cavity is filled with fluid.

Where known, the ICD-10 code is listed below.

- Anencephaly (Q00.0)

- Colpocephaly (ICD10 unknown)

- Holoprosencephaly (Q04.2)

- Ethmocephaly (ICD10 unknown)

- Hydranencephaly (Q04.3)

- Iniencephaly (Q00.2)

- Lissencephaly (Q04.3)

- Megalencephaly (Q04.5)

- Microcephaly (Q02)

- Porencephaly (Q04.6)

- Schizencephaly (Q04.6)

Cephalic disorders (from the Greek word "κεφάλι", meaning "head") are congenital conditions that stem from damage to, or abnormal development of, the budding nervous system. Cephalic means "head" or "head end of the body."

Cephalic disorders are not necessarily caused by a single factor, but may be influenced by hereditary or genetic conditions, nutritional deficiencies, or by environmental exposures during pregnancy, such as medication taken by the mother, maternal infection, or exposure to radiation. Some cephalic disorders occur when the cranial sutures (the fibrous joints that connect the bones of the skull) join prematurely. Most cephalic disorders are caused by a disturbance that occurs very early in the development of the fetal nervous system.

The human nervous system develops from a small, specialized plate of cells on the surface of the embryo. Early in development, this plate of cells forms the neural tube, a narrow sheath that closes between the third and fourth weeks of pregnancy to form the brain and spinal cord of the embryo. Four main processes are responsible for the development of the nervous system: cell proliferation, the process in which nerve cells divide to form new generations of cells; cell migration, the process in which nerve cells move from their place of origin to the place where they will remain for life; cell differentiation, the process during which cells acquire individual characteristics; and cell death, a natural process in which cells die.

Damage to the developing nervous system is a major cause of chronic, disabling disorders and, sometimes, death in infants, children, and even adults. The degree to which damage to the developing nervous system harms the mind and body varies enormously. Many disabilities are mild enough to allow those afflicted to eventually function independently in society. Others are not. Some infants, children, and adults die, others remain totally disabled, and an even larger population is partially disabled, functioning well below normal capacity throughout life.

The National Institute of Neurological Disorders and Stroke (NINDS) is currently "conducting and supporting research on normal and abnormal brain and nervous system development."

The optic nerve hypoplasia is generally manifested by nystagmus (involuntary eye movements, often side-to-side) and a smaller-than-usual optic disc. The degree of visual impairment is variable, and ranges from normal vision to complete blindness. When nystagmus develops, it typically appears by 1–8 months of age, and usually indicates that there will be a significant degree of visual impairment, but the severity is difficult to predict in infancy. Although there are many measures to compensate for visual impairment, there are few treatments available to induce normal optic nerve function.

The brain effects are also variable. Seizures sometimes occur. Prediction of intellectual outcome in infancy is difficult. Various types of early intervention or equivalent programs can help a child reach full developmental potential.

Hydranencephaly is a condition in which the cerebral hemispheres are missing and instead filled with sacs of cerebrospinal fluid.

Encephaloceles are characterized by protrusions of the brain through the skull that are sac-like and covered with membrane. They can be a groove down the middle of the upper part of the skull, between the forehead and nose, or the back of the skull. Encephaloceles are often obvious and diagnosed immediately. Sometimes small encephaloceles in the nasal and forehead are undetected.

Developmental regression is when a child loses an acquired function or fails to progress beyond a prolonged plateau after a period of relatively normal development. Developmental regression could be due to metabolic disorders, progressive hydrocephalus, worsening of seizures, increased spasticity, worsening of movement disorders or parental misconception of acquired milestones. The timing of onset of developmental regression can be established by repeated medical evaluations, prior photographs and home movies. Whether the neurologic decline is predominantly affecting the gray matter or the white matter of the brain needs to be ascertained. Seizures or EEG changes, movement disorders, blindness with retinal changes, personality changes and dementia are features suggestive of grey matter involvement.

The Landau–Kleffner syndrome is characterized by the sudden or gradual development of aphasia (the inability to understand or express language) and an abnormal electroencephalogram (EEG). LKS affects the parts of the brain that control comprehension and speech (Broca's area and Wernicke's area). The disorder usually occurs in children between the ages of 3 and 7 years. There appears to be a male dominance in the diagnosis of the syndrome (ratio of 1.7:1, men to women).

Typically, children with LKS develop normally, but then lose their language skills. While many affected individuals have clinical seizures, some only have electrographic seizures, including electrographic status epilepticus of sleep (ESES). The first indication of the language problem is usually auditory verbal agnosia. This is demonstrated in patients in multiple ways including the inability to recognize familiar noises and the impairment of the ability to lateralize or localize sound. In addition, receptive language is often critically impaired, however in some patients, impairment in expressive language is the most profound. In a study of 77 cases of Landau–Kleffner syndrome, 6 were found to have this type of aphasia. Because this syndrome appears during such a critical period of language acquisition in a child's life, speech production may be affected just as severely as language comprehension. The onset of LKS is typically between 18 months and 13 years, the most predominant time of emergence being between 3 and 7 years.

Generally, earlier manifestation of the disease correlates with poorer language recovery, and with the appearance of night seizures that last for longer than 36 months. LKS has a wide range of symptom differences and lacks a uniformity in diagnostic criteria between cases, and many studies don't include follow-ups on the patients, so no other relationships between symptoms and recovery have been made known.

Language deterioration in patients typically occurs over a period of weeks or months. However, acute onset of the condition has also been reported as well as episodic aphasia.

Seizures, especially during the night, are a heavily weighted indicator of LKS. The prevalence of clinical seizures in acquired epileptic aphasia (LKS) is 70-85%. In one third of patients, only a single episode of a seizure was recorded. The seizures typically appear between the ages of 4 and 10 and disappear before adulthood (around the age of 15).

Often, behavioral and neuropsychologic disturbances accompany the progression of LKS. Behavioral issues are seen in as many as 78% of all cases. Hyperactivity and a decreased attention span are observed in as many as 80% of patients as well as rage, aggression, and anxiety. These behavior patterns are considered secondary to the language impairment in LKS. Impaired short-term memory is a feature recorded in long-standing cases of acquired epileptic aphasia.

Landau–Kleffner syndrome (LKS)—also called infantile acquired aphasia, acquired epileptic aphasia or aphasia with convulsive disorder—is a rare childhood neurological syndrome.

It is named after William Landau and Frank Kleffner, who characterized it in 1957 with a diagnosis of six children.

The bilateral form of FCMS ("also known as facio-labio-pharyngo-glosso-laryngo-brachial paralysis)" is consistent with the classic presentation of bilateral corticobulbar involvement. It is characterized by well-preserved automatic and reflex movements. It is caused by lesions in the cortical or subcortical region of the anterior opercular area surrounding the insula forming the gyri of the frontal, temporal, and parietal lobes.

The unilateral operculum syndrome is a very rare form of FCMS caused by the formation of unilateral lesions. In this form of FCMS, the unaffected hemisphere of the brain compensates for the unilateral lesion. Usually, this occurs when the unaffected region is the individual's dominant hemisphere.

There are a variety of medical conditions affecting cognitive ability. This is a broad concept encompassing various intellectual or cognitive deficits, including intellectual disability, deficits too mild to properly qualify as intellectual disability, various specific conditions (such as specific learning disability), and problems acquired later in life through acquired brain injuries or neurodegenerative diseases like dementia. These disabilities may appear at any age.

AOS and expressive aphasia (also known as Broca's aphasia) are commonly mistaken as the same disorder mainly because they often occur together in patients. Although both disorders present with symptoms such as a difficulty producing sounds due to damage in the language parts of the brain, they are not the same. The main difference between these disorders lies in the ability to comprehend spoken language; patients with apraxia are able to fully comprehend speech, while patients with aphasia are not always fully able to comprehend others' speech.

Conduction aphasia is another speech disorder that is similar to, but not the same as, apraxia of speech. Although patients who suffer from conduction aphasia have full comprehension of speech, as do AOS sufferers, there are differences between the two disorders. Patients with conduction aphasia are typically able to speak fluently, but they do not have the ability to repeat what they hear.

Similarly, dysarthria, another motor speech disorder, is characterized by difficulty articulating sounds. The difficulty in articulation does not occur due in planning the motor movement, as happens with AOS. Instead, dysarthria is caused by inability in or weakness of the muscles in the mouth, face, and respiratory system.

The most common symptoms of acquired and transient cortical blindness include:

- A complete loss of visual sensation and of vision

- Preservation/sparing of the abilities to perceive light and/or moving, but not static objects (Riddoch syndrome)

- A lack of visual fixation and tracking

- Denial of visual loss (Anton–Babinski syndrome)

- Visual hallucinations

- Macular sparing, in which vision in the fovea is spared from the blindness.

Apraxia of speech can be caused by impairment to parts of the brain that control muscle movement and speech. However, identifying a particular region of the brain in which AOS always occurs has been controversial. Various patients with damage to left subcortical structures, regions of the insula, and Broca’s area have been diagnosed with AOS. Most commonly it is triggered by vascular lesions, but AOS can also arise due to tumors and trauma.

More than 70% of children with ONH experience developmental delay, ranging from isolated focal defects to delay in all areas of development (global delay). Motor delay is most common (75%) and communication delay is least common (44%). Predictors of significantly delayed development include hypoplasia or agenesis of the corpus callosum and hypothyroidism. The absence of the septum pellucidum does not predict developmental delay. Delays may occur in unilateral (39%) as well as bilateral (78%) cases.

An acquired brain injury (ABI) is brain damage caused by events after birth, rather than as part of a genetic or congenital disorder. It usually affects cognitive, physical, emotional, social or independent functioning and can result from either traumatic brain injury or nontraumatic injury such as stroke, infection or substance abuse. Most definitions of ABI exclude neurodegenerative disorders.

People with a brain injury may have difficulty controlling, coordinating and communicating their thoughts and actions. They may or may not retain their intellectual abilities, depending on the type and extent of the injury. However, the intellectual abilities of a person with a brain injury are likely to be interfered with by the resulting thought coordination and communication difficulties, which can make it difficult for them to express themselves in a manner intelligible to others. This may give the impression of a damaged intelligence even in people with normal intellectual capacity.