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Year : 2020  |  Volume : 8  |  Issue : 1  |  Page : 163-164

Hemihydrencephaly in adult


Department of Radiodiagnosis, GMC, Srinagar, Jammu and Kashmir, India

Date of Submission14-Apr-2019
Date of Decision21-Apr-2019
Date of Acceptance29-Apr-2019
Date of Web Publication20-Jun-2020

Correspondence Address:
Dr. Musaib Ahmad Dar
Department of Radiodiagnosis, GMC, Srinagar, Jammu and Kashmir
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/amhs.amhs_49_20

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How to cite this article:
Rafiq S, Dar MA. Hemihydrencephaly in adult. Arch Med Health Sci 2020;8:163-4

How to cite this URL:
Rafiq S, Dar MA. Hemihydrencephaly in adult. Arch Med Health Sci [serial online] 2020 [cited 2020 Nov 29];8:163-4. Available from: https://www.amhsjournal.org/text.asp?2020/8/1/163/287365



Hemihydrancephaly (HHE) is a very rare anomaly characterized by complete/near complete absence of one cerebral hemisphere. HHE is much rarer than hydranencephaly, which has been reported with a prevalence of 0.2% at autopsy.[1] The affected hemisphere is transformed into cerebrospinal fluid (CSF) containing membranous sac containing minimal glioneuronal tissue along with some blood vessels.[2] It occurs due to vascular occlusion of unilateral internal cerebral artery [3] prior to third trimester of pregnancy occurring after neural migration and before synaptogenesis.[4] It tends to favor left side more than the right.

A 27-year-old male presents to emergency with seizures. He has history of right sided hemiplegia, right lower limb hyperflexia and gaze abnormalities from childhood. He suffers from mild mental retardation. He has right homonymous hemianopia on visual field examination. He was able to walk with assistance. Language was appropriate. He was stabilized in emergency with intravenous diazepam and was referred to radiology department for computerized tomography (CT) of Brain. Routine laboratory investigations were normal. Noncontrast CT of brain revealed evidence of whole of left cerebral hemisphere replaced by fluid filled sac having attenuation similar to CSF with preservation of thin mantle of cortex in occipital region [Figure 1] and [Figure 2]. Although HHE usually spares basal ganglia but in our case basal ganglia were also affected. Falx was present and right cerebral hemisphere had normal morphology and attenuation. There was evidence of mild ventriculomegaly. Posterior fossa and brain stem were normal.
Figure 1: Axial non contrast computerized tomography revealing replacement of left cerebral hemisphere by cerebrospinal fluid attenuation fluid with preservation of minimal cortical mantle in occipital region. There is evidence of slight displacement of falx towards right side with dilated right lateral ventricle

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Figure 2: Axial non contrast computerized tomography at level of basal ganglia revealing involvement of basal ganglia along with left cerebral hemisphere in a case of hemihydrancephaly

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Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Dähnert W. Brain Disorders: Radiology Review Manual. New York: Lippincott Williams & Wilkins; 2003. p. 298-99.  Back to cited text no. 1
    
2.
Mori K: Anomalies of the central nervous system. Neuroradiology and neurosurgery. New York: Thieme-Stratton; 1985. p. 39-42.  Back to cited text no. 2
    
3.
Myers RE. Cerebral ischemia in the developing primate fetus. Biomed Biochim Acta 1989;48:S137-42.  Back to cited text no. 3
    
4.
Eyre JA, Miller S, Clowry GJ, Conway EA, Watts C. Functional corticospinal projections are established prenatally in the human foetus permitting involvement in the development of spinal motor centres. Brain 2000;123(Pt 1):51-64.  Back to cited text no. 4
    


    Figures

  [Figure 1], [Figure 2]



 

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