Journal of Neurosciences in Rural Practice
 


 
  Table of Contents 
IMAGES IN NEUROSCIENCES
Year : 2018  |  Volume : 9  |  Issue : 3  |  Page : 404-405  

A patient with schizencephaly and agenesis of corpus callosum with no neurological deficits


1 Department of Medicine, St. George Hospital University Medical Center, University of Balamand, Lebanon
2 Department of Radiology and Imaging, St. George Hospital University Medical Center, University of Balamand, Lebanon
3 Department of Medicine - Division of Neurology, St. George Hospital University Medical Center, University of Balamand, Lebanon

Date of Web Publication6-Jul-2018

Correspondence Address:
Rosette Jabbour
St. George Hospital University Medical Center, Faculty of Medicine, University of Balamand, Achrafieh, Beirut
Lebanon
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jnrp.jnrp_564_17

Rights and Permissions

How to cite this article:
Ghosn Y, Kamareddine MH, Adem C, Jabbour R. A patient with schizencephaly and agenesis of corpus callosum with no neurological deficits. J Neurosci Rural Pract 2018;9:404-5

How to cite this URL:
Ghosn Y, Kamareddine MH, Adem C, Jabbour R. A patient with schizencephaly and agenesis of corpus callosum with no neurological deficits. J Neurosci Rural Pract [serial online] 2018 [cited 2018 Jul 23];9:404-5. Available from: http://www.ruralneuropractice.com/text.asp?2018/9/3/404/236036



A 23-year-old male presented to the emergency room (ER) for polytrauma after involvement in a motor vehicle accident with head trauma but no loss of consciousness. He was previously healthy with no history of seizures or other neurological problems and had not been taking any medications. He is a senior mass communication student who lives with his parents and plays football weekly. The investigations in the ER revealed a right femur fracture and right frontal skin laceration necessitating suturing. A computed tomography scan of the brain revealed no acute intracranial bleed; however, partial agenesis of the corpus callosum was noted with possible accompanying schizencephaly of the left frontal lobe. These findings were further evaluated by a brain magnetic resonance imaging [Figure 1] which confirmed the presence of the partial agenesis of the anterior part of the corpus callosum, but the splenium visualized and noted to be thin. The anterior aspect of the left cingulate gyrus was abnormal with evidence of focal communication between the anterior horn of the lateral ventricle and the left aspect of the interhemispheric fissure. All findings were compatible with chronic left open lip schizencephaly (Type II). The patient underwent femur surgery and received standard postoperative care. Postsurgery, a neurological examination was also conducted that investigated patient's mental status, cranial nerves, motor function, muscle strength and tone, sensation, reflexes, and coordination which were all negative. Gait was not assessed because of femur fracture. The patient was subsequently discharged.
Figure 1: (a) An axial T1-weighted image demonstrates left frontal lobe open-lip schizencephaly. (b) A sagittal T1-weighted image (to the left of the midline) demonstrates a cerebrospinal fluid-filled cleft. Agenesis of the anterior part of the corpus callosum is also noted

Click here to view


Schizencephaly is a rare disorder of cerebral cortical development with an incidence of 1.54/100,000.[1] It is caused by defective neuronal migration resulting in the formation of clefts that extend from the cerebral wall to the underlying ventricular surface. Types of schizencephaly include closed-lip clefts (Type I) and open-lip clefts (Type II). In addition, schizencephaly may occur as a unilateral or bilateral lesion.[1],[2] Studies demonstrate multiple possible etiologies for schizencephaly ranging from intrauterine insults to possible genetic etiology.[1],[2] This patient's prenatal history was negative for complications, maternal drug use or infection.

With regard to clinical presentation, patients could present with a variety of neurological and developmental defects. The correlation between imaging features and clinical outcome has been analyzed showing a correlation between the size of the cleft and motor deficits and mental retardation, but not seizures.[1],[2] Although half of the patients present with epilepsy and especially those with unilateral lesions,[1],[2] this patient had neither seizure activity nor a history of epilepsy.

A number of similar cases have been previously reported,[3],[4] but all exhibit certain degrees of central nervous system manifestations such as cerebral palsy and epilepsy which are absent in this patient. This case demonstrates that it is possible for a patient to have schizencephaly along with partial agenesis of the corpus callosum and be without neurological deficits.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Halabuda A, Klasa L, Kwiatkowski S, Wyrobek L, Milczarek O, Gergont, A. Schizencephaly-diagnostics and clinical dilemmas. Childs Nerv Syst 2015;31:551-6.  Back to cited text no. 1
    
2.
Granata T, Freri E, Caccia C, Setola V, Taroni F, Battaglia G, et al. Schizencephaly: Clinical spectrum, epilepsy, and pathogenesis. J Child Neurol 2005;20:313-8.  Back to cited text no. 2
    
3.
Han X, Cheng J, Surapaneni K, Altschuler EL. Schizencephaly: Dramatic images in a normally functioning adult. PM R 2015;7:901-2.  Back to cited text no. 3
    
4.
Spreer J, Dietz M, Raab P, Arnold S, Klisch J, Lanfermann H, et al. Functional MRI of language-related activation in left frontal schizencephaly. J Comput Assist Tomogr 2000;24:732-4.  Back to cited text no. 4
    


    Figures

  [Figure 1]



 

Top
 
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this article
    References
    Article Figures

 Article Access Statistics
    Viewed55    
    Printed1    
    Emailed0    
    PDF Downloaded0    
    Comments [Add]    

Recommend this journal