Cantú Salinas, Adriana Carlota

Chávez Lluévamos, E. B.

De León Flores, Laura

Duncan, M. A.

Villarreal Velázquez, Héctor Jorge

Vázquez Flores, S.

Repositorio Academico Digital UANL

Medicina Universitaria 2014;16(63):71-73www.elsevier.com.mxmedicinauniversitaria* Corresponding author: Service of Pediatric Neurology, Department of Neurology, “Dr. José Eleuterio González” University Hospital, Fa-culty of Medicine, Universidad Autónoma de Nuevo León, Monterrey, N. L., Mexico. Telephone: (044) 818 016 9944. E-mail address: kazaamba@hotmail.com (M. A. Duncan).SCiENTiFiC LETTErDyke-Davidoff-Masson syndrome: A case studyM. A. Duncan*, S. Vázquez-Flores, E. B. Chávez-Lluévanos, A. C. Cantú-Salinas, L. de León-Flores, H. J. Villarreal-VelázquezService of Pediatric Neurology, Department of Neurology, “Dr. José Eleuterio González” University Hospital, Faculty of Medicine, Universidad Autónoma de Nuevo León, Monterrey, N. L., Mexicoreceived: July 2013; Accepted: January 2014KEYWORDS Dyke-Davidoff-Masson syndrome; Cerebral hemiatrophy; Facial asymmetry; Mexico.Abstract Cerebral hemiatrophy or Dyke-Davidoff-Masson syndrome is a rare condition irst described in 1933, with characteristic clinical and radiological features such as facial asymmetry, hemiplegia, seizures, mental retardation, cerebral hemiatrophy, and skull and frontal sinus abnormalities. We describe the clinical features, brain imaging and clinical course of a 13 year-old patient with this syndrome. 1665-5796 © 2014 revista Medicina Universitaria. Facultad de Medicina UANL. Publicado por Elsevier México. Todos los derechos reservados.Introduction Cerebral hemiatrophy or Dyke-Davidoff-Masson syndrome (DDMS) is a rare condition, which may be congenital or de-velop in early childhood. in 1933, Dyke, Davidoff and Masson irst described the syndrome in a series of 9 patients with hemiplegia and cranial asymmetry in plain cranial X-rays. However, since then there have been few pediatric cases reported.1-3 The syndrome is characterized by facial asym-metry, seizures, hemiplegia or contralateral hemiparesis, and mental retardation of variable severity.1,3,4 it occurs in both genders, with a predominance in males (73.5%).5 A diagnosis is usually made during late childhood, adolescen-ce or adulthood.6 The etiology can be congenital or acquired as a result of an alteration in the cerebral perfusion during prenatal, perinatal or early childhood periods.1,4,5,7,8 The bra-in scan (CT) or magnetic resonance imaging (Mri) is utilized to perform a diagnosis and speciic indings include unilateral brain volume loss and compensatory bone alteration resulting in cerebral hemiatrophy, usually on the left hemisphere (69%). in addition, ipsilateral cranial hypertrophy, overdeve-lopment of the orbital roof and hyperpneumatisation of frontal sinuses may occur.4,5,7,9,10Case presentationA 13-year-old female, product of a fourth pregnancy, with a history of a monozygotic twin pregnancy, full term with Documento descargado de http://www.elsevier.es el 24-08-201672 M. A. Duncan et aladequate weight and no contingencies, normal psychomotor development up to the irst year of age, when a gait altera-tion with right hemibody weakness was noticed. She was admitted to the Pediatric Neurology Service at “Dr. José Eleuterio González” University Hospital after presenting simple partial seizure with motor symptoms at age 7. An electroencephalogram (EEG) was performed with normal re-sults, and the patient was controlled with 400 mg/day of oral carbamazepine. During the physical exam the patient displayed hemifacial atrophy of the right side causing asym-metry (Fig. 1), right spastic hemiparesis with atrophy, hype-rreflexia, ipsilateral extensor plantar response and a hemiplegic gait. We are able to observe in the brain Mri a reduction of ridges and grooves in the left frontoparietal region, associated with ectasia of the ipsilateral ventricle, which is compatible with cerebral hemiatrophy of the left frontoparietal lobe (Figs. 2 and 3). The patient is currently stable with proper control of seizure crisis and an adequate level of antiepileptic medication (9.0 µg/dl). She is atten-ding our integral follow-up service and rehabilitation.DiscussionCerebral hemiatrophy or DDMS is an entity characterized by seizures, hemiplegia or hemiparesis and mental retarda-tion.1,4,5,9 The condition may be congenital or acquired. in the congenital form, there are usually no evident etiological factors and symptoms present at birth or shortly after. Cere-bral damage is likely due to an intrauterine vascular occlu-sion during the prenatal period. in the acquired form, sym- ptoms are related to a damage of the central nervous system occurring around or after the perinatal period. Some of the etiological factors include trauma, infection, vascular anomalies, hemorrhagic and ischemic conditions, amniotic bands, and an intraventricular and subependymal germinal matrix.5,8,10 The mechanism of cerebral atrophy remains unknown.1 Our patient displayed symptoms during an early stage of her development; moreover, because of the histo- ry of being a product of a twin pregnancy, a high risk state during pregnancy and the lack of evidence of injury during postpartum, most likely the damage occurred during the prenatal stage. Seizures do not always appear during early childhood and often begin months or years after the he- miparesis onset.3,4 Sometimes patients display refractory epilepsy, and the treatment must focus on seizure control with anticolvulsant medication being either mono or po-lytherapy.5 Children with refractory epilepsy and hemiplegia are potential candidates for hemispherectomy, with a suc-cess rate of 85%. Vagal stimulation is another alternative. There is evidence that 30%-50% of patients with partial epi-lepsy may experience a decrease in convulsions in over 50% of the cases.3 The prognosis is better if the hemiparesis ap-pears after the irst 2 years of age and in absence of recu-rrent and prolonged epilepsy. This syndrome is a rare condition, with few cases described in medical literatu- re and limited experience in pediatric patients. it is impor-tant to keep it in mind when dealing with a patient with facial asymmetry associated with neurologic hemiplegia data, seizures, and mental retardation, in order to perform a correct diagnosis and proper care. Even though an esta-blished protocol for management is lacking, there is the in-dication for therapy including anticonvulsants and surgery in speciic cases. In addition, physical therapy, occupational therapy and language therapy play signiicant roles in the long term management of the patients.5 Figure 1 A 13-year-old female in whom facial asymmetry is observed.Figure 2 Axial magnetic resonance imaging (Mri), T2 FLAir sequence which shows left frontoparietal atrophy with ipsilate-ral ventriculomegaly.Documento descargado de http://www.elsevier.es el 24-08-2016Dyke-Davidoff-Masson syndrome: A case study 73Conlicts of interestThe authors have no conlicts of interest to declare.FundingNo inancial support was provided.References 1. Paudel K, Venugopal A. Dyke-Davidoff-Masson Syndrome. JNMA J Nepal Med Assoc 2013;52:272-274.2. Sharma S, Goyal D, Negi A, et al. Dyke-Davidoff Masson Syndro-me. ind J radiol imag 2006;16:165-166.3. Erdem A, Acik V, Leventoğlu A, et al. Effect of vagal nerve stimu-lation in Dyke-Davidoff-Masson syndrome with refractory genera-lized seizures - case report. Turk Neurosurg 2009;19:197-199.4. Aguiar PH, Liu CW, Leitão H, et al. Mr and CT imaging in the Dyke-Davidoff-Masson syndrome. report of three cases and contribution to pathogenesis and differential diagnosis. Arq Neuropsiquiatr 1998;56:803-807.5. Behera Mr, Patnaik S, Mohanty AK. Dyke-Davidoff-Masson syn-drome. J Neurosci rural Pract 2012;3:411-413.6. Piro E, Piccione M, Marrone G, et al. Dyke-Davidoff-Masson syndro-me: case report of fetal unilateral ventriculomegaly and hypoplas-tic left middle cerebral artery. ital J Pediat. 2013;14:39-32.7. Zúñiga-González EA, Molina-Carrión LE, Diego-Silva rC. Síndro-me de Dyke-Davidoff-Masson y hemiatroia cerebral del adulto. informe de casos. rev Med inst Mex Seguro Soc 2009;47:215-218.8. Lee JH, Lee Zi, Kim HK, et al. A case of Dyke-Davidoff-Masson syndrome in Korea. Korean J Pediatr 2006;49:208-211.9. Narain NP, Kumar r, Narain B. Dyke Davidoff- Syndrome. indian Pediatr 2008;45:927-928.10. Shrestha B. Acquired cerebral hemiatrophy: Dyke-Davidoff-Masson Syndrome – A case report. Turk Neurosurg 2013;23:117-121.Figure 3 Coronal magnetic resonance imaging (Mri), T2 FLAir sequence which shows interhemispheric asymmetry with atro-phy and left ventricular ectasia.Documento descargado de http://www.elsevier.es el 24-08-2016

Dyke-Davidoff-Masson syndrome: A case study

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Dyke-Davidoff-Masson syndrome: A case study

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