Develop. Med. Child Neurol. 1982, 24, 173-182 Case Reports Aphasia Following Right Striato-insular Infarction in a Left-handed Child: a Clinico-radiological Study Jose' M . Ferro Isabel Pav6o Martins Francisco Pinto A . Castro-Caldas damage in 50 per cent of cases (Woods and Teuber 1978), in contrast to only 20 to 30 per cent of left-handed aphasic adults (Brown and Htcaen 1976). This report describes a left-handed child with acquired aphasia following a right subcortical infarct, and stresses the importance of the localisation of the lesion in understanding the pattern of aphasia and the recovery process. Introduction Acquired aphasia during childhood offers an excellent method of studying inter- and intra-hemispheric specialization for language. However, it is not a common disorder, and the majority of reports have included only a few cases observed over several years, sometimes with different methodologies (e.g. Htcaen 1976, Woods and Teuber 1978). Two other potential sources of confusion are the varied aetiologies (trauma, infection, tumour, cerebrovascular accident) and the paucity of clinico-pathological studies. The introduction of the C T scan into clinical practice has not yet greatly modified this picture: lesions were localised by CT scan in only one of the cases reported by Woods and Teuber (1978), and in the child reported by Pohl (1979). Gastaut et al. (1979) used CT scan in their study of acute hemiplegia in children, but unfortunately they make no mention of speech disturbance. Aphasia among left-handed children has been associated with right-hemispheric Case report C.S., a six-year-old girl, was admitted because of sudden onset of left-sided weakness and speech disturbance. After a few hours of mutism, her spontaneous speech was scanty and lacked fluency, but she could follow two-step oral commands. .The general physical examination was unremarkable and there were no signs of meningeal irritation. Ocular fundi were normal. A defect in the left visual field was observed, together with left central facial weakness, left hemiparesis and left hypoaesthesia to touch, pain and postural sensation. Tendon reflexes were weakeron the left, and a Babinski sign was elicited on that side. Her history was unremarkable. She had been born at term after a normal labour and there had been no postnatal problems. Psychomotor development had been within normal limits. She was left-handed when assessed by the Edinburgh Handedness Inventory (Oldfield 1971), but because of pressure at school she wrote mainly with her right hand, although her hand-writing ' ~ Correspondence lo Jose M. Ferro, M.D., Language Research Laboratory, Centro de Estudos Egas Moniz, Hospital de Santa Maria, Lisbon, Portugal. 173 DEVELOPMENTAL M E D I C I N E A N D C H I L D NEIJROLOGY.1982, 24 was far better with her left hand. N o other members of the family were left-handed. She had begun primary school five months before the present illness, and already could read and write most simple words, and could d o simple calculations. Normal results were obtained from routine blood and platelet counts, ESR, serum chemistry examinations, tests for blood coagulation. ECG and in an isotopic renogram. CSF examination was normal and cultures were negative. An EEG showed global slowing on the right leads, more marked in the frontal, parietal and temporal areas. A CT scan was performed on the sixth day of illness and demonstrated a right-periventricular area of diminished density, involving the internal capsule, lenticular nucleus and the insula. This lesion produced no mass effect, showed no contrast enhancement, and was considered to be a cerebral infarction (Fig. I). A right-carotid angiogram showed corkscrew irregularities in the supraclinoid segment of the internal carotid artery, and in the proximal part of the middle and anterior cerebral arteries, suggestive of fibromuscular dysplasia. A cerebral TcYvmscan performed on the 34th day was negative. Detailed neurophysiological examinations were carried out on the third, 18th and 46th days of the Fig. 1. CT-scan cuts 3Omm lupper/eff),40mm (upper right). 50mm (/Oii'erlqft)and60mm(/olivrri~ht)above and parallel with the orbito-meatal line, demonstrating hypodense area in right hemisphere involving internal capsule, lenticular nucleus and insula. 174 CASE REPORTS illness (Table I). At the first examination, C.S. had moderate, non-fluent aphasia a n d typical righthemisphere signs such as visual neglect, constructional apraxia a n d spatial dysgraphia (Fig. 2), a n d left-channel extinction of dichotic listening. The aphasia improved rapidly, a n d apart from some articulation errors in spontaneous speech a n d repetition, the language disturbance had disappeared within about two weeks, a s had non-verbal disturbance a n d left-channel extinction. of her aphasia, the lack of fluency and the articulatory distGrbance. A peculiar feature of this case is the subcortical location of the lesion producing aphasia. T o our knowledge there have been no reports of such lesions producing aphasia during childhood. In adults, striatal and insular lesions have been associated with mutism or with nonfluent aphasia with intact repetition, resembling transcortical aphasia (Benson 1979), and insular lesions are known to cause anarthria (Marie 1926). However, this child had a short period of rnutism and non-fluent speech but, in contrast to adult cases, repetition was also disturbed. The subcortical location of the infarct explains the relatively good comprehension Discussion This patient is similar to other children with acquired aphasia in her initial period of mutism, lack of fluency, good preservation of comprehension and rapid recovery. She also shares some features with adult sinistral aphasia (Brown and Htcaen 1976), namely the relative mildness TABLE 1 Summary of neuropsychological examinations Verbal,functions Severity of aphasia' Spontaneous speech Praying a n d singing Serial speech' Visual naming' Visual identification' Oral comprehension', Token test (22 item?) Sentence repetitjon Digit repetition Word repetition' Reading Writing (right hand) Non-verhal,func,tion.c Visual neglect Visual Retention Test Form C (Copying) Dichoiic li.\ tiwin,? I X pairs of common words 19 pairs of digits (right-left ratio) WlSC Verbal Performance Total 3rd clay 18th day 46th daja 4 5 5 non-fluent articulatory errors literal paraphasia articulatory errors literal paraphasia articulatory errors +++ disturbed normal 12/16 16/16 8/8 I0 2 2 17/30 many literal errors vowels normal spatial dysgraphia + normal 3 3 21/30 literal errors 16/16 16/16 XI8 17.5 4 3 18/30 literal errors poor handwriting poor handwriting +++ 0 0 I correct 7 I I4/0 17/16 IX/15 18/17 18/17 94 9x 96 I05 I IX I12 lelt 18/27 14/16 16/16 8/8 18 'Goodglass and Kaplan (1972): 'Castro-Caldas (1979): 'Benton ( 1969). 175 DEVELOPMENTAL MEDICINE A N D CHILD NEUROLOGY. 1982, 24 Ay & R4/ua, %-aAxL ilJ vJL Fig. 2. C.S. attempting to copy with her right hand. Note left-side visual neglect and spatial dysgraphia. demonstrated by this child, as the lesion spared the associative auditory areas of the right temporal lobe. The rapid recovery is also consistent with similar adult patients, who also had greater and faster improvement than those with lesions in the classic speech areas (Benson 1979). To explain the rapid improvement in the present case, a 'localisation' factor can be added to the lateralisation and plasticity factors which usually determine the superior recovery among left-handers and children (Brown and Htcaen 1976). This child's right hemisphere was dominant not only for speech production, as judged by her clinical and dichotic listening patterns, but also for non-verbal functions, as is often the case with lefthanded people* She a right-hemispheric syndrome, consisting of dysgraphia, apraxia and visual neglect. Spatial dysgraphia has also been reported in two other aphasic. children with right-hemispheric lesions (HCcaen 1976). Htcaen and Sauget (1971) also found that these right-hemispheric signs were significantly more frequent among left-handed adults when the lesion was'in the right rather than in the left hemisphere. Dichotic listening may be helpful in assessing improvement of aphasia. When the aphasic disturbance cleared there was improved performance of the left auditory channel, suggesting that language recovery was dependent on right-hemispheric structures. Acknowled'yernenrs: We thank Maria Gabriela Mariano, Speech Therapist, for assistance with the evaluation of this patient, and Maria de Fatima Pereira for typing the manuscript. AUTHORS' APPOINTMENTS .Jose M. Ferro, M.D., Assistant in Neurology, Lisbon Faculty of Medicine, Hospital de Santa Maria; Research Fellow, Language Research Laboratory, Centro de Estudos Egas Moniz (INIC). Isabel PavHo Martins, M.D., Research Fellow, Language Research Laboratory, Centro de Estudos @as M o n k (INIC). Francisco Pinto, M.D.. Neurologist, Department of Neurology, ~ ~de santa ~ ~ ~ ~i A. Castro-Caldas, M.D., Associate Professor of Neurology, Lisbon Faculty of Medicine; Chief, Language Research Laboratory, Centro de Estudos Egas Moniz ( I N I C ) . I76 i ~ ~ CASE REPORTS SUMMARY The case of a six-year-old girl with non-fluent aphasia following a subcortical righthemispheric cerebrovascular accident is reported. Examination by computerised tomographic scan revealed a right paraventricular area of diminished density involving the internal capsule, lenticular nucleus and the insula. The speech disturbance was followed by typical right-hemispheric signs and by left-channel extinction in dichotic listening tests. The aphasia cleared in two weeks, paralleled by an improvement in left-channel performance, suggesting that language recovery was dependent on right-hemispheric structures. RESUME Aphasie a la suite d u n infarcissement striato-insulaire droit chez un enfant gaucher :Etude clinique et radiologique L’article rapporte le cas d’une fillette de six ans ayant prtsentt une anarthrie B la suite d’un accident vasculoctrtbral sous-cortical de l’htmisphtre droit. L’examen au scanner rCvCla une rtgion paraventriculaire droite de densitt diminute impliquant la capsule interne, le noyau lenticulaire et l’insula. La perturbation du langage fut suivie de signes typiques htmisphtriques droits et par une extinction du canal gauche au cours des Cpreuves d’kcoute dichotique. L’aphasie rtgressa en deux semaines, avec une amtlioration paralltle des performances d’tcoute gauche, suggtrant que la rtcuptration du langage dkpendait des structures htmisphtriques droites. ZUSAMMENFASSUNG Aphasie nach einem rechtsseitigen striato-insularen Infarkt bei einem linkshandigen Kind: eine klinisch-radiologische Untersuchung Es wird ein sechsjahriges Madchen vorgestellt, das nach einem rechtsseitigen cerebrovaskularen Zwischenfall eine Aphasie bot. Das Computertomogramm zeigte rechts paraventrikular ein Areal verminderter Dichte, das die interne Kapsel, den N. lenticularis und die Insel einbezog. Nach der Sprachstorung folgten typische rechtsseitige Befunde, sowie linksseitiger Horverlust .beim doppelseitigen Hortest. Die Aphasie bildete sich innerhalb von zwei Wochen zuriick zugleich mit einer Besserung der linksseitigen Horleistung. Man nimmt daher an, dap die Spracherholung auf Strukturen aer rechten Hemisphere beruht. RESUMEN Afasia consecutiva a un infarto estriato - insular en un nirlo zurdo: estudio clinico-radioldgico Se presenta el caso de una niila de seis ailos de edad con una afasia no fluente consecutiva a un accidente cerebro-vascular subcortical del hemisferio derecho. El examen con TAC revel6 una Area paraventricular derecha de densidad disminuida que interesaba la chpsula interna, el ndcleo lenticular y la insula. La alteracidn del lenguaje fue seguida de signos tipicos del hemisferio derecho y de una extincti6n en el canal izquierdo en 10s tests de escucha dic6ticos. La afasia desapareci6 en dos semans, con una mejoria paralela de la realizaci6n en el canal izquierdo, lo que sugiere que la recuperacidn del lenguaje dependid de las estructuras del hemisferio derecho. 177 DEVELOPMENTAL M E D I C I N E A N D CHILD NEUROLOGY. 1982. 24 REFERENCES Benson, D. F. (1979) ‘Subcortical aphasic syndromes’. In: Aphasia. Alexia and Agraphia. New Y o r k : Churchill Livingstone. Benton, A. L. 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Tome I?’. Paris; Masson. Oldfield, R. C. (1971) ‘The assessment and analysis of handedness: the Edinburgh inventory.’ Neuropsychologia. 9, 9 7- I 13. Pohl, P. (1979) ‘Dichotic listening in a child recovering from acquired aphasia.’ Brain and Lan,yua,yc. 8. 372-379. Woods, B. T., Teuber, H. L. (1978) ‘Changing patterns of childhood aphasia.’ Annul., q/”eu,olc~,y~..3. 273-280. Rhombencephalosynapsis: a Viennese Malformation? W. Schachenmayr R. L. Friede aqueduct and midline fusion of the cerebellar hemispheres. A seventh case is described in the present paper. Introduction ‘Rhombencephalosynapsis’ is a rare malformation, described in detail by Gross and colleagues (1959, 1978). It is remarkable that the first observation of this malformation (Obersteiner 19 16) and the four subsequent cases were all reported i n Vienna. The essential features are absence of the incisuria cerebelli posterior; fusion of the cerebellar hemispheres; absence of the velum medullare anterius and nuclei fastigii; and fusion of the dentate nuclei, which are shifted towards the midline. To o u r knowledge, only one additional case of this syndrome has been reported outside Europe. Kepes et af. (1969) described a case with congenital fusion of the thalami, associated with atresia of the Case Report The child was the male non-identical twin of a 23-year-old primiparous, primigravida mother from a village east of Ulm. Germany. The pregnancy was uneventful until the 38th week, when a caesarean section was performed because of hydrocephalus in one of the twins. The affected boy weighed 3510g, body length was 56.4cm and head circumference was 42.5cm. There was an ‘egg-sized’, soft, fluctuant, parietal protrusion in the region of the sagittal suture; the latter was dehiscent over an area of 5 x IOcm. Radiological examination of the skull showed craniolacunia and a bifid spine at CI to C4. Pneumoencephalography done on the third day showed communication between a left paramedian encephalocele and the left lateral ventricle, which was enormously dilated. (CT scans were not available in 1977.) Movements and tonus were reduced, but no other malformations were found. Serology for rubella, cytomegaly, lues and Correspondence to Prof. Dr. W . Schachenmayr, Department of Pathology, University of Ulm, Steinhovelstrasse 9, D-7900 Ulm, West Germany. 178