Background: Dyke-Davidoff-Masson syndrome is a rare condition of unknown frequency resulting from brain injury due to a multitude of causes; especially in early life. Characteristics include cerebral hemiatrophy/hypoplasia, contralateral hemiparesis, seizures, and compensatory osseous hypertrophy. Case presentation: We present a case of a 13-year-old girl who initially presented with headaches, followed by episodic complex-partial seizures; which was controlled via medication. She also had right sided hemiparesis. Computed tomography (CT) showed evidence of left parieto-temporal infarct with cerebral atrophy. Complementary magnetic resonance imaging (MRI) did not reveal additional information. Workup for young stroke was negative. Upon further evaluation by Neuroradiology, features suggesting Dyke-Davidoff-Masson syndrome were confirmed. Patient has been under Neurology follow up since. Conclusions: Due to its rarity, Dyke-Davidoff-Masson syndrome may easily be missed by the majority of treating clinicians. Knowledge of its features on imaging enables timely and accurate diagnosis – allowing appropriate management. Keywords: Dyke-Davidoff-Masson syndrome, Computed tomography (CT), Magnetic resonance imaging (MRI) Background A head CT was done, which revealed left parieto-temporal Dyke-Davidoff-Masson syndrome (DDMS) is a rare neuro- infarct, with cerebral atrophy. Complementary MRI was of logical condition of unknown frequency, with available no significant additional value. Workup for young stroke, literature mostly from case reports/series [1–3]. Most af- which included protein S, protein C, anti-thrombin III, fected patients are among the pediatric population. Due to anti-phospholipid antibody, anti-cardiolipin antibody, and its rarity, it may be misdiagnosed or under-reported by the lupus anticoagulant were negative; she was subsequently majority of clinicians. We describe a patient who was ini- put on routine follow up. One year after the initial presen- tially thought to have young stroke of unknown etiology; tation, her mother noticed new onset of brief moments of eventually diagnosed having DDMS via imaging findings. the child mumbling followed by blank stare and drowsi- ness, prior regaining consciousness. She was diagnosed as having complex-partial seizures, and initiated on syrup so- Case presentation dium valproate 200 mg bd. Upon further evaluation of the A 13-year-old girl presented to us for further manage- previous imaging studies, in addition to the left sided in- ment of episodic complex-partial seizures and right farct, we noticed that the left cerebral hemisphere was uni- hemiparesis. She initially had episodes of headaches versally atrophic with ventricular enlargement. There was starting at the age of 7. Mother noticed that she had also evidence of calvarial thickening on the ipsilateral side, accompanying right sided weakness; which prompted and hyperpneumatization of the left frontal sinus (Fig. 1). A medical attention. Initial evaluation revealed power of final diagnosis of DDMS was made. Patient has been on 4/5 on the right side, with reduced sensation and regular Neurology follow up since. brisk reflexes. Minimal facial asymmetry was evident. * Correspondence: firstname.lastname@example.org Discussion and conclusions Department of Imaging, Faculty of Medicine and Health Sciences, Universiti In 1933, Dyke, Davidoff, and Masson described 9 pa- Putra Malaysia, Serdang, Malaysia Full list of author information is available at the end of the article tients with clinical characteristics of hemiparesis, facial © The Author(s). 2018 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. Abdul Rashid and Md Noh BMC Neurology (2018) 18:76 Page 2 of 3 Fig. 1 CT (a, b)and MRI(c) images, in axial section showing. a CT image in bone window showing hyperpneumatization of the left frontal sinus (blue arrow) with compensatory calvarial thickening (yellow arrow). b CT image (non-contrasted) showing the left parieto-temporal infarct. c MRI T2 FLAIR image showing left cerebral hemiatrophy, with dilated left lateral ventricle. Focal encephalomalacia and gliotic changes are also noted asymmetry, seizures, and mental retardation noted to have pneumatoencephalographic changes on skull radiograph . CT and MRI features of this entity in- clude cerebral hemiatrophy, ipsilateral ventriculomegaly, hyperpneumatization of the sinuses on the affected side, and compensatory calvarial thickening [1, 3, 5]. Affected patients are largely from the paediatric population; how- ever, occurrence in adult patients have been reported . Common causes include congenital anomalies, perinatal hypoxia, intracranial hemorrhage, and infections . Clinically, patients may have seizures, mental retard- ation, contralateral hemiparesis, and facial asymmetry. Our patient initially had headaches, followed by episodic complex-partial seizures. Birth history did not reveal any hypoxic-ischemic events. There was also no history to suggest intra-uterine/perinatal infection. Only after head CT, an infarct was diagnosed; and patient was treated symptomatically. The MRI that followed did not add any additional information. To the unaccustomed, subtle findings on CT/MRI may easily be missed. We were able to accurately diagnose the imaging features after con- sulting with Neuroradiology. Understandably, the rarity of this condition makes accurate diagnosis a challenge – as evident in our experience. Imaging via CT and MRI proves to be of significant value; enabling correct diagnosis and institution of ap- propriate management. These two imaging modalities are valuable in that they provide cross sectional images, with thin slices, and post processing capabilities. Pertinent imaging features for DDMS include cerebral hemiatrophy/ hypoplasia, hyperpneumatization of the paranasal sinuses, and compensatory osseous hypertrophy. These radiological features will become more evident with time, as the patient gets older. There have been reports suggesting calvarial in- volvement, as in our experience, points to cerebral damage occurring during the intrauterine period or before the age of 3 [6–8]. Our patient, interestingly, did not have the clinical history or features to suggest an early life event which may lead to cerebral damage. In essence, due to the rarity of this syndrome, it may be easily misdiagnosed by the untrained eye. CT and MRI are powerful imaging modalities to diagnose the pertinent im- aging features associated with this syndrome. Knowledge of the clinical presentation, risk factors, and imaging features is therefore indispensable for appropriate patient management. Abdul Rashid and Md Noh BMC Neurology (2018) 18:76 Page 3 of 3 Abbreviations CT: Computed tomography; DDMS: Dyke-Davidoff-Masson syndrome; MRI: Magnetic resonance imaging Availability of data and materials All are available in the manuscript. Authors’ contributions MSF drafted the manuscript and was responsible for overall content, images, and literature review. AM examined the patient and obtained the clinical history. Both authors read and approved the manuscript for publication. Ethics approval and consent to participate Both authors’ institution does not require ethical approval for publication of a single case report. Written informed consent was obtained from the patient’s next of kin (parents). Consent for publication Written informed consent for publication of clinical details and images was obtained from the patient’s next of kin (parents). Competing interests The authors declare that they have no competing interests. Publisher’sNote Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. Author details Department of Medicine, Faculty of Medicine and Health Sciences, Universiti Putra Malaysia, Serdang, Malaysia. Department of Imaging, Faculty of Medicine and Health Sciences, Universiti Putra Malaysia, Serdang, Malaysia. Received: 6 October 2017 Accepted: 22 May 2018 References 1. Verma R, Sahu R. Dyke-Davidoff-Masson syndrome. BMJ Case Reports. 2012. https://doi.org/10.1136/bcr-2012-006729. 2. Piro E, Piccione M, Marrone G, Giuffre M, Corsello G. Dyke-Davidoff-Masson syndrome: case report of fetal unilateral ventriculomegaly and hypoplastic left middle cerebral artery. Ital J Pediatr. 2013;39:32. 3. Roy U, Panwar A, Mukherjee A, Biswas D. Adult presentation of dyke- Davidoff-Masson syndrome: a case report. Case Rep Neurol. 2016;8:20–6. 4. Dyke CG, Davidoff LM, Masson CB. Cerebral hemiatrophy and homolateral hypertrophy of the skull and sinuses. Surg Gynecol Obstet. 1933;57:588–600. 5. Gokce E, Beyhan M, Sade R. Radiological imaging findings of dyke-Davidoff- Masson syndrome. Acta Neurol Belg. 2017;117(4):885–93. 6. Arora R, Rani JY. Dyke-Davidoff-Masson syndrome: imaging features with illustration of two cases. Quant Imaging Med Surg. 2015;5(3):469–71. 7. Atalar MH, Icagasioglu D, Tas F. Cerebral hemiatrophy (dyke-Davidoff- Masson syndrome) in childhood: clinicoradiological analysis of 19 cases. Pediatr Int. 2007;49(1):70–5. 8. Uduma FU, Emejulu JK, Motah M, Okere PC, Ongolo PC, Muna W. Differential diagnoses of cerebral hemiatrophy in childhood: a review of literature with illustrative report of two cases. Glob J Health Sci. 2013;5(3):195–207.
BMC Neurology – Springer Journals
Published: May 29, 2018
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