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MR Imaging of N Euronal Migration Anomaly

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MR Imaging of N Euronal Migration Anomaly 대 한 방 사 선 의 학 회 지 1991; 27(3) : 323~328 Journal of Korean Radiological Society. May. 1991 MR Imaging of N euronal Migration Anomaly Hyun Sook Hong, M.D., Eun Wan Choi, M.D., Dae Ho Kim, M.D., Moo Chan Chung, M.D., Kuy Hyang Kwon, M.D., Ki Jung Kim, M.D. Department o[ RadíoJogy. Col1ege o[ Medícine. Soonchunhyang University patients ranged in age from 5 months to 42 years with Introduction a mean of 16 years. The mean age was skewed by 2 patients with schizencephaly who were 35 and 42 Abnormalities of neuronal migration Sl re years old. characterized by anectopic location of neurons in the MR was performed with a 0:2T permanent type cerebral cortex (1-9). This broad group of anomalies (Hidachi PRP 20). Slice thickness was 5mm with a includes agyria. pachygyria. schizencephaly. 2.5mm interslice gap or 7.5mm thickness. Spin echo unilateral megalencephaly. and gray matter axial images were obtained. including Tl weighted hcterotopia. Patients with this anomaly present images (TIWI) with a repetition time (TR) of clinically with a variety of symptoms which are pro­ 400-500ms and echo time (TE) of 25-40ms. in­ portional to the extent of the brain involved. These termediate images of TR/TE 2000/38. and T2 abnormalities have been characterized pathologically weighted images (T2Wl) with a TR/TE of 2000/110. in vivo by sonography and CT scan (2. 3. 10-14. Occasionally. sagittal and coronal images were ob­ 15-21). tained. Gd-DTPA enhanced Tl WI were 려 so obtain­ MR appears to be an imaging technique of choice ed in 6 patients. Migration anomalies were diagnosed in evaluating these anomalies because it is capable on the basis of characteristic morphology of the af­ of exceptionally good differentiation between gray fected brain on MR or CT. and white matter. high contrast resolution. m비 tiplan와 display of the anatomy. and lack of Results overlying bone artifact (1. 22-24). The purpose of this paper is to describe the MR fin­ The clinical and radiologic findings of eleven pa­ dings of neuronal migration anomaly. tients are presented in Table 1. The neuronal migra­ tion anomalies in 11 patients included one with Subjects and Methods lissencephaly. 9 with schizencephaly (2 with open lip type and 7 with closed lip typeJ, and one with isolated Eleven patients with neuronal migration heterotopia. In one patient with lissencephaly. MR anomalies were examined with MR (10 patients) and demonstrated a figure of 8 appearance with a shallow CT (one patient) from May 1989 to September 1990 sylvian fissure caused by lack of opercularization (Fig. because of seizure. mental retardation. developmen­ 1). The cortex was thick and white matter was tal delay. enlarged head. and motor weakness. The decreased in volume. The gray-white matter interface Index Words: Brain. abnormalities. 13. 14. was smooth due to the lack of white matter inter­ digitation. This patient also had persistent wide Brain‘ MR imagings. 13 ‘ 1214 이 논문은 1 990 년 11 월 1 4 일 접수하여 1991 년 3 월 30 일 에 채택되었 음 Received November 14. accepted March 30. 1991 - 323- Journal of Korean Radiological Society 1991; 27(3) 323-328 Table L Summary of Patients Data Case Age (year) Presenting Symptoms MR diagnosis No. I Sex 1. 5/l2/F seizure. large head agyria/pachygyria complex. colpocephaly 2 11M developmental delay. abnormal face & hair schizencephaly. closed lip type. right 3 l lF left motor weakness schizencephaly. closed lip type. right 4. 41F seizure. development & m ental retardation schizencephaly. closed lip type. bilateral 5 101F seizure. m ental retardation. right schizencephaly‘ closed lip type. left motor weakness heterotopia 6 121F seizure. left hand paresis schizencephaly. closed lip type. right 7. 23/F facial palsy due to chronic mastoiditis isolated heterotopia. nodular form incidental finding for migration disorder 8. 241F seizure. right hemiparesis schizencephaly. open lip type. left 9. 241M selzure schizence ph려 y . closed lip type. right heterotopia 10 351M selzure schizencephaly. closed lip type. bilateral 11. 42/M skull fracture by fall down. seizure. left schizencephaly. open lip type. right motor weakness. mental retardation lateral ventricles (colpocephaly). Nine patients with The process ofneuroblast formation begins at ap­ schizencephaly had unilateral (7/9) (Fig. 2 .. Fig. 3b.) proximately 6 gestational weeks (3. 4. 9. 29). First or bilateral clefts (2/9) (Fig. 3a.) that were lined by wave of neuroblast formed in the germinal matrix gray matter. The clefts were commonly seen in the migrates through the white matter along ependymo­ parietal (parasylvian region) and temporal areas glial process that stretches from the ventricular wall Associated anomalies included absent septum through the white matter to the forming cortex. Such pellucidum (3/9) (Fig. 2.) and heterotopias (2/9). peripheral migration along the glial guide is Hydrocephalus was commonly seen associated designated radial migration and is most active dur­ with an open form (Fig. 2.) ing the 3rd to 5th gestational months but continues Heterotopias were seen in 3 patients. one with until approximately 5 months postnatally (1-4. 9. 29) isolated hete rotopia (Fig . 4.) and others with At this point. they differentiate further grow into ax­ associated schizencephaly (Fig. 2 .. 3.). ons and dendrites. and develop synaptic contacts with other neurons. By the 6th month of gestation. Discussion a 6 layered n eocortex is formed (4. 26). Fig. L Lissencephaly. 2000/40 ax­ ial proton density image Large areas of agyria are observed in the both parietooccipital lobes with partial gyral formation in anterior frontal lobes suggesting pachygyria. which shows hourglass configuration of brain and shallow smooth surface. There are also loss of cortical gray-white matter inter­ digitation and colpocephaly. Fig. 4. Isolated gray matter heterotopia. Nodular gray matter indents the left 1 4 frontal hom oflateral ventricle and diverticulum like ventricular pr이 ec­ tion is also seen. Adj~cent insular cortex is somewhat thickened. - 324- Hyun Sook Hong , et al : MR Imaging of Neuronal Migration Anomaly Fig. 2. Open lipped schizencephaly a . Axial proton density MR image (2000/3 8) demonstrates CSF c1 efts extending from the venticle to the sulci, lined with gray matter bands in left precentral region, and mild­ Iy dilated ventricular system . b. Coronal T1 WI image (500/38) well delineates the CSF c1eft, com­ municating cortical sulci with the lateral ventricle, and agenesis of septum pellucidum a b Fig. 3. Two cases of closed lip schizencephaly a. Axial MR image (20001112) shows a band of thick gray matter extending from the ventricle to the cortical surface in postcentral region bilaterally. b. Axial MR image (2000/40) shows unila teral c10sed lip schizencepha­ ly with gray matter heterotopia in right periventricular region. a b Smaller waves of cell migration continue up to 25 for holohemispheric agyria and at other times as a weeks. Any insu1t to the brain during this period more general term encompassing the results in a migration anomalies (2 , 7 , 25-27, 30-32). agyria/pachygyria complex (2 , 15). The common underlying feature of migration Lissencephaly can be divided into 3 types. Type anoma1 ies is an abnormal location of neurons both 1 is characterized by microcephaly and dysmorphic within and outside the cortex. facies usually associated with heritable syndromes ln general, the cortex is thickened by a large such as Miller-Dieker syndrome, Norm an-Roberts disorganized layer of neurons whose migration has syndrome, and the Neu-Laxova syndrome. Type II been prematurely halted. Pathologically, both agyric usually lacks characteris tic facies but exhibits and pachygyric regions ofthe brain have a 41ayered macrocephaly, retina1 dysplasia, congenita1 muscular cortex composed of a molecular layer, outer cellular dystrophy, and/or posterior fossa abnormalities. The layer, a sparse celllayer, and an inner cellular layer. Walker-Wanburg and the cerebro-ocular-muscular The subcorticallayer ofwhite matter is thin because syndrome are associated with type II. Type III is organization of the neurons, which subsequently heritable isolated lissencephaly a nd cerebro­ stimulates axonal growth, has not occurred. cerebellar lissencepha1 y. Type III patients have the The term agyria refers to an absence of cortical best prognosis a nd longest suπival (2 , 3 , 7). Our case gyri, usually focal and holohemispheric. ln fact. most had no associated syndromes and abnormal face , so agyric brains have at least small area of gyral forma­ probably type III. tion (1, 2 , 13, 14, 19, 20). The area of broad f1 at The clinical features are seizure, developmental shallo\V gyri are referred to area of pachygyria. The delay, or mental retardation with the degree of severi­ term lissencephaly is sometimes used as a synonym ty related to the amount of abnormal cortex (25, 27 , - 325- Journal of Korean Radiological Society 1991; 27(3) 323-328 31). MR exquisitely demonstrates the abnormal a r­ of th e ventric1 es. Polymicrogyria or a bsence of sep chitecture. MR shows the cortical convolutions and tum pellucidum is frequently observed. It is impor­ gray-white m atter interface are smoother than nor tant to understa nd that narrow c1efts can be identified m a l a nd the s ubcortical white matter is thinned . The in only one imaging plane and be missed in anothe r insulae are exposed and the middle cerebral a rteries planes. In patients with horizontal c1 efts. the abnor­ course superficially a long a shallow sylvian groove m a li ty would have been missed or misdiagnosed if No sylvian triangle is present (7. 8). A small bra in the coronal images were not obtained. Similarly. ver­ stem is ofte n observed and may ref1 ect lack of tically oriented narrow clefts can be missed if only development of the corticospinal tracts and subcor­ coronaJ images are obtained.
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