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| CASE REPORT |
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| Year : 2021 | Volume
: 8
| Issue : 4 | Page : 272-275 |
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Cerebral hemiatrophy: Case series of three cases
Suhail Rafiq, Musaib Ahmad Dar, Obaid Ashraf, Inayat Ellahi
Department of Radiodiagnosis and Imaging, GMC, Srinagar, Jammu and Kashmir, India
| Date of Submission | 25-May-2020 |
| Date of Acceptance | 23-Sep-2020 |
| Date of Web Publication | 27-May-2022 |
Correspondence Address:
Obaid Ashraf
Department of Radiodiagnosis and Imaging, GMC, Srinagar, Jammu and Kashmir
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/cjhr.cjhr_57_20
Pediatric cerebral hemiatrophy is a rare entity with widespread etiology. Imaging in form of computed tomography and magnetic resonance imaging is helpful in differentiating between the etiologies of hemiatrophy. We describe imaging findings in three different cases of hemiatrophy due to Sturge-Weber syndrome, Dyke-Davidoff-Masson syndrome, and Rasmussen encephalitis.
Keywords: Dyke-Davidoff-Masson syndrome, hemiatrophy, magnetic resonance imaging, Rasmussen encephalitis, Sturge-Weber syndrome
How to cite this article:
Rafiq S, Dar MA, Ashraf O, Ellahi I. Cerebral hemiatrophy: Case series of three cases. CHRISMED J Health Res 2021;8:272-5 |
| Introduction | |  |
Atrophy of one cerebral hemisphere in the presence of normal contralateral hemisphere is known as hemiatrophy. It occurs due to variety of congenital like intrauterine vascular injury and acquired conditions such as postictal hemiatrophy, Rassmussens encephalitis, Sturge-Weber syndrome (SWS), hemimegalencephaly, Dyke-Davidoff-Mason syndrome, perinatal intracranial hemorrhage, and postischemic, traumatic or hypoxic changes. Shift of midline structures toward abnormal or diseased and paucity of sulcal prominence[1] help to differentiate in utero insults from secondary cerebral hemiatrophy. We describe hemiatrophy in three pediatric patients, one each of SWS, Dyke Davidoff Masson syndrome (DDMS), and Rasmussen Encephalitis. SWS is a vascular phakomatosis characterized by cortical anoxia due to stasis or occlusion of persistent fetal vasculature.[2] Classical features of SWS such as leptomeningeal pial angioma and choroidal angioma can be demonstrated by computed tomography (CT) and magnetic resonance imaging (MRI).[3] DDMS was first described by Dyke et al. in 1933 characterized by the clinical features of hemiparesis, seizures, facial asymmetry, and mental retardation.[4],[5] Rasmussen encephalitis is a chronic inflammatory disease characterized by progressive neurological deterioration, cognitive decline, and intractable seizures.[6]
| Case Reports | | |
Case 1
A 3-year-old child presented to the causality department with a history of recurrent seizures, developmental delay weakness of the left side of the body. Clinical examination reveals port wine stain on the right side of the face and visual impairment. MRI was advised. MRI revealed evidence of right cerebral hemiatrophy, right enhancing pial angioma with enhancing and enlarged ipsilateral choroid plexus (choroidal angioma) [Figure 1] with gyriform areas of hypointensity in right temporooccipeto-parietal region on fluid attenuated inversion recovery [Figure 2], T2-weighted images with blooming on susceptibility weighted imaging (SWI) suggestive of calcification. There is evidence of smooth enhancing thickening around the posterior segment of the globe suggestive of choroidal angioma [Figure 3]. The diagnosis of SWS was made. | Figure 1: Coronal postcontrast magnetic resonance image revealing enhancing right choroidal angioma with overlying enhancing pial angioma and right hemiatrophy
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 | Figure 2: Axial fluid attenuated inversion recovery magnetic resonance revealing hypointensity along pial angioma in right temporo-occipetal region
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 | Figure 3: Axial postcontrast magnetic resonance image showing evidence of right smoothly enhancing posterior segment choroidal angioma along with enhancing right pial angioma
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Case 2
A 15-year-old child presented to the pediatric outpatient department with right hemiplegia, cognitive impairment, difficulty in studying and walking, history of seizures, and mental retardation. Basic laboratory tests are normal. Her perinatal period was uneventful. MRI is advised, but the patient is unable to cooperate and still despite proper sedation. The patient underwent computerized tomography of the head which revealed hemiatrophy of left cerebral hemisphere, dilated left lateral ventricle [Figure 4] and [Figure 5], prominent frontal subarachnoid spaces, mildly thickened left calvarial vault [Figure 6], and mild hyperpneumatization of paranasal sinuses. There was no involvement of basal ganglia. Diagnosis of DDMS was made. | Figure 4: Axial computed tomography revealing evidence of left hemiatrophy with dilated left lateral ventricle and enlarged extraaxial spaces
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 | Figure 5: Coronal computed tomography revealing evidence of left hemiatrophy with dilated left lateral ventricle and enlarged extraaxial spaces
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 | Figure 6: Coronal computed tomography showing mildly thickened left calvarial bony vault
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Case 3
A 6-year-old female child presents to the pediatric outpatient department with left-sided hemiplegia, speech disturbances, and intractable tonic–clonic seizures from 3 years. Seizures are resistant to pharmacotherapy. Her developmental milestones are normal. There is no history of birth trauma. She has exaggerated upper and lower limb tendon reflexes. MRI is advised. MRI revealed evidence of prominent right extraaxial spaces, especially around the insular region and dilated right ventricle [Figure 7]. There was atrophy of right caudate nucleus [Figure 8] and hippocampus [Figure 9] along with diffuse loss of right cerebral white matter. Left cerebral hemisphere is normal. There was no evidence of any calvarial thickening or hyperpneumatization of paranasal sinuses. In view of right hemiatrophy, the involvement of basal ganglia and loss of white matter diagnosis of Rasmussen's encephalitis is made. | Figure 7: Coronal fluid-attenuated inversion recovery magnetic resonance showing evidence of right lateral ventricle dilatation with prominent extra-axial spaces and white matter loss
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 | Figure 8: Axial fluid attenuated inversion recovery magnetic resonance revealing evidence atrophied right caudate nucleus with prominent periinsular extra-axial spaces with white matter loss
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 | Figure 9: Coronal fluid attenuated inversion recovery magnetic resonance showing evidence of right lateral ventricle dilatation, atrophied right hippocampus, and caudate nucleus with white matter loss
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| Discussion | | |
The prevalence of SWS is 1 in 50,000 live births.[7] It is a sporadic neurocutaneous syndrome with predominant vascular manifestations. Contrast-enhanced MRI is the investigation of choice in SWS for depicting the presence and extent of leptomeningeal angiomatosis which is the hallmark of SWS. Enhancement of the leptomeninges occurs due to leptomeningeal angiomatosis and homogeneous enhancement of an enlarged ipsilateral choroid plexus are due to choroid plexus angioma.[8] Ischemia and hypoxia due to abnormal cerebrovenous drainage lead to progressive neurologic deterioration. MR perfusion imaging and single-photon emission CT scanning have revealed decreased perfusion in the areas of leptomeningeal enhancement[9] which then progresses to hemiatrophy. Hemiatrophy in SWS is usually unilateral and confined to the parieto-occipital area, but can involve entire hemisphere or there can be bilateral involvement.[10],[11] Gyriform hypointensities on T1- and T2-weighted MRI images seen as the areas of blooming on SWI occur due to venous collateralization and cortical calcification. These present as tramline calcifications on CT with underlying leptomeningeal angiomatosis.[11]
DDMS is a childhood hemiatrophy with compensatory ipsilateral calvarial hypertrophy.[12],[13] DDMS occurs secondary to vascular, congenital or acquired ischaemic disease, traumatic or inflammatory insult occurring in utero or during the childhood period.[12] Calvarial changes occur only if brain damage occurs before 3 years of age.[13] Failure of brain growth results in compensatory changes like hyperpneumatized sinuses due to inward redirection of nearby calvarial growth. CT is preferred over conventional radiography in DDMS.[14]
Rasmussen encephalitis was discovered in 1958 by Theodore Rasmussen.[15],[16] Rasmussen encephalitis is usually unicerebral and generally occurs in children under the age of 15 years with median age of 6 years.[15],[16] It is a diagnosis of exclusion and typically insidious in onset.[15] It is characterized by unilateral hemispheric atrophy, abrupt appearance of focal, persistent motor seizures, followed by hemiplegia and progressive cognitive deterioration.[17] Atrophy of the head of the caudate, volume loss most accentuated in the insular and peri-insular regions, atrophy of hippocampus along with hemiatrophy as in our case have been seen in Rasmussen encephalitis.[15],[16]
Differential diagnosis of hemiatrophy include basal cell germinoma, Fishman syndrome, Silver-Russell Syndrome, Post-Ictal cerebral hemiatrophy, Hemiconvulsion-Hemiplegia-Epilepsy Syndrome, Linear Scleroderma, Linear Sebaccious Nervus Syndrome, Parry Romberg Syndrome, and Haberland Syndrome (Fishman Syndrome).[18]
| Conclusion | | |
Cross-sectional imaging in form of MRI and CT plays a vital role in differentiating the etiology of cerebral hemiatrophy as well as correctly describing the spectrum of the disease.
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.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9]
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