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PubMed This is a summary of 19 peer-reviewed journal articles Updated
Pediatrics

Recognizing the Signs: Symptoms of Schizencephaly

At a Glance

Schizencephaly symptoms vary widely based on the size and location of the brain cleft. Common signs include motor weakness on one side of the body, muscle stiffness, developmental delays, and seizures. Children with unilateral, closed-lip clefts often experience milder symptoms.

Return to Overview

Because schizencephaly affects the brain’s physical structure, it can impact how a child moves, learns, and interacts with the world. However, there is no “one-size-fits-all” list of symptoms. Some children show clear signs shortly after birth, while others may not show any symptoms until they are adults [1][2]. The symptoms your child experiences will depend heavily on whether the cleft is on one side (unilateral) or both sides (bilateral), and whether it is open-lip or closed-lip [3][4].

Motor Symptoms and Physical Movement

Motor deficits are often the first sign parents notice. These occur because the cleft passes through areas of the brain that send signals to the muscles [5].

  • Hemiparesis and Hemiplegia: You will likely see these two terms used by your child’s doctors. Hemiparesis is weakness on one side of the body, while hemiplegia refers to more severe paralysis on one side. They are common in unilateral cases; for example, a cleft on the right side of the brain may cause weakness on the left side of the body [6][7].
  • Quadriparesis: This is weakness affecting all four limbs, which is more common in bilateral cases [4].
  • Spasticity: This refers to stiff or tight muscles that make movement difficult. You may notice your child has trouble straightening their arms or legs, or they may seem “stiff” when you try to change their clothes [5].

Epilepsy and Seizures

Seizures are one of the most common features of schizencephaly, but they do not affect every child [8][9].

  • Bilateral Risk: Children with clefts on both sides of the brain have a much higher risk of developing epilepsy (recurrent seizures) [10].
  • Timing: Seizures often start in childhood, but they can also appear for the first time in adulthood, even if the person had no symptoms as a child [11][12].
  • Types: Seizures can range from subtle “staring spells” to more obvious rhythmic shaking. In some cases, these seizures can be refractory, meaning they are difficult to control with standard medications [13][14].

Developmental and Cognitive Delays

Developmental progress often follows the “severity rule”—the larger and more numerous the clefts, the more likely there will be delays [3].

  • Milestones: You may notice your child takes longer to reach milestones like sitting up, crawling, or speaking [6][15].
  • Cognitive Impact: Children with bilateral clefts are more likely to experience cognitive or learning challenges [4]. However, many children with unilateral, closed-lip clefts have normal intelligence and live typical lives [16][2].
  • Microcephaly: Some children are born with a head size that is smaller than average (microcephaly), which can sometimes be an indicator of more significant cognitive delays [4].

A Spectrum of Experience

It is helpful to remember that “structural” does not always mean “symptomatic.”

  • Mild Cases: A child with a single, small, closed-lip cleft may reach every milestone on time and only discover the condition later in life, perhaps after a single seizure or as an incidental finding on an MRI for an unrelated reason [1][16].
  • Severe Cases: A child with large, bilateral, open-lip clefts may show signs of hydrocephalus (fluid pressure in the brain) or significant motor delays in the first few months of life [6][17].

Your child’s medical team will use these symptoms, alongside imaging, to create a personalized care plan that focuses on their specific strengths and challenges [18][19].

Common questions in this guide

What are the first signs of schizencephaly in a baby?
Motor deficits are often the first sign parents notice. You may observe weakness on one side of the body, unusual muscle stiffness, or your child favoring one side when reaching, rolling, or crawling.
Will a child with schizencephaly develop seizures?
Seizures are common but do not affect every child. The risk of developing epilepsy is significantly higher in children with bilateral clefts. These seizures can start during childhood or appear for the first time in adulthood.
Can a child with schizencephaly have normal intelligence?
Yes, many children with unilateral, closed-lip clefts have normal intelligence and achieve standard developmental milestones. Cognitive challenges and learning delays are more frequently seen in children with bilateral or large, open-lip clefts.
Are motor delays caused by the brain cleft or by hydrocephalus?
Motor delays can be caused directly by the cleft interrupting nerve signals, or by increased pressure in the brain known as hydrocephalus. Your child's medical team will use MRI imaging to determine the exact cause of the delays.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Given my child's specific MRI, what motor symptoms (like hemiparesis or spasticity) are they most likely to experience?
  2. 2.If my child hasn't had a seizure yet, what is the statistical likelihood they will develop epilepsy later in life?
  3. 3.What specific developmental milestones should I be tracking most closely for a child with this type of cleft?
  4. 4.Are my child's current motor delays caused by the cleft itself or by high intracranial pressure (hydrocephalus)?
  5. 5.Does my child have microcephaly, and how does that relate to their long-term cognitive outlook?

Questions For You

Tap a prompt to share your answer — we'll use it plus this page's context to start a tailored conversation.

References

References (19)
  1. 1

    Unilateral right closed-lip schizencephaly.

    Alkareem M, Ahmed H, Ahmed G

    BMJ case reports 2020; (13(9)) doi:10.1136/bcr-2020-235004.

    PMID: 32928828
  2. 2

    A rare case of schizencephaly in an adult with late presentation.

    Kamble V, Lahoti AM, Dhok A, et al.

    Journal of family medicine and primary care 2017; (6(2)):450-452 doi:10.4103/jfmpc.jfmpc_43_17.

    PMID: 29302567
  3. 3

    Schizencephaly in children: A single medical center retrospective study.

    Hung PC, Wang HS, Chou ML, et al.

    Pediatrics and neonatology 2018; (59(6)):573-580 doi:10.1016/j.pedneo.2018.01.009.

    PMID: 29371079
  4. 4

    Schizencephaly: A Review of 734 Patients.

    Braga VL, da Costa MDS, Riera R, et al.

    Pediatric neurology 2018; (87()):23-29 doi:10.1016/j.pediatrneurol.2018.08.001.

    PMID: 30501885
  5. 5

    Motor Organization in Schizencephaly: Outcomes of Transcranial Magnetic Stimulation and Diffusion Tensor Imaging of Motor Tract Projections Correlate with the Different Domains of Hand Function.

    Yoon JY, Kim DS, Kim GW, et al.

    BioMed research international 2021; (2021()):9956609 doi:10.1155/2021/9956609.

    PMID: 34527746
  6. 6

    A Rare Case of an Infant with Left Hemiparesis: A Case Report of Bilateral Open-lip Schizencephaly.

    Siti BC, Zulkifli MM, Mohd Yusoff SS, et al.

    Malaysian family physician : the official journal of the Academy of Family Physicians of Malaysia 2020; (15(3)):90-94.

    PMID: 33329869
  7. 7

    Unilateral Open-lip Schizencephaly with Tonsillar Herniation in a Preterm Infant.

    Gonzalez JC, Singhapakdi K, Martino AM, et al.

    Journal of pediatric neurosciences 2019; (14(4)):225-227 doi:10.4103/jpn.JPN_75_19.

    PMID: 31908665
  8. 8

    Undiagnosed Schizencephaly Presenting as Breakthrough Seizures.

    Coacci J, Viccellio P

    Clinical practice and cases in emergency medicine 2024; (8(4)):377-378 doi:10.5811/cpcem.20922.

    PMID: 39704574
  9. 9

    Early-Onset 15q11.2 Microdeletion Syndrome in a Six-Year-Old Child: A Case Report of Refractory Epilepsy, Autism, and Multisystem Manifestations.

    Ortiz Hernández IY, Noboa Rodríguez J, Bueno Fernandez LA, Rijo Florimon K

    Cureus 2025; (17(8)):e90530 doi:10.7759/cureus.90530.

    PMID: 40843057
  10. 10

    Epilepsy in paediatric patients with schizencephaly.

    Kopyta I, Skrzypek M, Raczkiewicz D, et al.

    Annals of agricultural and environmental medicine : AAEM 2020; (27(2)):279-283 doi:10.26444/aaem/122796.

    PMID: 32588606
  11. 11

    Complex neurological interplay: adult-onset migraine-triggered seizures in schizencephaly-a case report.

    Wang Y, Zhou F, Liu F, et al.

    Frontiers in human neuroscience 2025; (19()):1606004 doi:10.3389/fnhum.2025.1606004.

    PMID: 41127412
  12. 12

    Schizencephaly Associated With Bipolar Affective Disorder.

    Rama Rao R, Reddy ABS, P D, Koul A

    Cureus 2024; (16(2)):e54534 doi:10.7759/cureus.54534.

    PMID: 38516433
  13. 13

    Stereo-EEG for Epileptogenic Focus Localization in Schizencephaly: A Single-center Experience in Four Patients.

    Liu PC, Chen HH, Chou CC, et al.

    World neurosurgery 2023; (172()):e319-e325 doi:10.1016/j.wneu.2023.01.019.

    PMID: 36632895
  14. 14

    A rare case of super-refractory epileptic status in pregnant woman: Schizencephaly.

    Jbili N, Mounir K, Kechna H, et al.

    Anaesthesia, critical care & pain medicine 2019; (38(2)):195-198 doi:10.1016/j.accpm.2018.06.002.

    PMID: 30012509
  15. 15

    Persistent falcine sinus with temporo-occipital schizencephaly: case report with a review of literature in relation to the undeveloped vein of Galen and/or straight sinus.

    Sunilkumar D, Nagarajan K, Kiran M, et al.

    Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery 2020; (36(2)):417-421 doi:10.1007/s00381-019-04234-9.

    PMID: 31154486
  16. 16

    Schizencephaly as an Unusual Cause of Adult-Onset Epilepsy: A Case Report.

    Battah A, DaCosta TR, Shanker E, et al.

    Cureus 2022; (14(6)):e25848 doi:10.7759/cureus.25848.

    PMID: 35836438
  17. 17

    Bilateral open lip schizencephaly.

    Hakimi T, Mohammad Qasem K

    Annals of medicine and surgery (2012) 2022; (73()):103204 doi:10.1016/j.amsu.2021.103204.

    PMID: 35079358
  18. 18

    A case of closedlip schizencephaly with absent septum pellucidum in an adult presenting with seizure disorder.

    Khadka C, Gupta U, Bhandari P, et al.

    Clinical case reports 2023; (11(6)):e7536 doi:10.1002/ccr3.7536.

    PMID: 37323273
  19. 19

    Open lip schizencephaly: An unusual cause of hemiparesis: A case report.

    Mequanint MB, Mengesha CA, Alemu HT, Molla YD

    Radiology case reports 2024; (19(11)):5354-5358 doi:10.1016/j.radcr.2024.07.192.

    PMID: 39280747

This page is for informational purposes only and does not replace professional medical advice. Always consult your pediatric neurologist about your child's specific symptoms and developmental progress.

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