Dyke-Davidoff-Masson Syndrome in a Tanzanian patient: A case report

Dyke-Davidoff-Masson Syndrome in a Tanzanian patient: A case report | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Case Report Dyke-Davidoff-Masson Syndrome in a Tanzanian patient: A case report Baraka Alphonce, Ng’weina Magitta, Francisca Komanya, Mbelwa Bitesigilwe, and 2 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-4095531/v1 This work is licensed under a CC BY 4.0 License Status: Posted Version 1 posted You are reading this latest preprint version Abstract Background Dyke-Davidoff-Masson Syndrome (DDMS) is a rare neurological disorder resulting from an insult to the developing brain in utero or during early life which is characterized by refractory seizures and a wide variety of deficits. Due to its rarity, the condition is often overlooked although a detailed history and imaging can help distinguish it from other similar conditions and provide early diagnosis. Case presentation: A thirty-year-old female presented with a history of recurrent seizures and intellectual disability. Her delivery was through the vaginal route with unremarkable post-natal history. Her developmental milestones were normal until the age of three years when she was hospitalized for two weeks due to a febrile illness and generalized seizures, with full recovery. She earned a score of 14/60 on the Ravens Standard Progressive Matrices (RSPM) indicating an intellectual disability. The neurological examination revealed constructional apraxia, facial nerve upper motor neuron lesion (UMNL). A slow, sinuous writhing movement involving all fingers on the left hand was observed, and left spastic paresis. She underwent a computed tomography (CT) of the head which confirmed DDMS due to presence of right cerebral hemisphere volume loss with gross dilatation of the right lateral ventricle with ex-vacuo dilatation of the right lateral ventricle, right calvarial thickening and hyperpneumatization of the right frontal sinus. She was managed conservatively with anticonvulsants and had her seizure well controlled. Conclusion In the setting of recurrent seizures, intellectual disability, hemiparesis, or facial asymmetry, a clinician should have a high index of suspicion for DDMS. Late diagnosis is often related to intractable seizures to anticonvulsants, necessitating hemispherectomy, which is not a readily available option in limited resource settings. Dyke-Davidoff-Masson Syndrome Recurrent Seizures Cerebral Hemiatrophy Paranasal sinus Hyperpneumatization Figures Figure 1 Figure 2 Introduction Dyke-Davidoff-Masson Syndrome (DDMS), also known as cerebral hemiatrophy, was initially defined by Dyke, Davidoff, and Masson in 1933 [ 1 ]. It is an uncommon condition that results after a developing brain injury leading to hypoplasia in one cerebral hemisphere [ 2 ]. Indeed, by the year 2022, there were only five cases that were reported in African population [ 3 ]. In four cases, the diagnosis was made in the second decade of life [ 3 – 5 ], with the exception of one case in which the diagnosis was made at the age of three [ 6 ]. The disease is characterized by diverse neurological features and deficits. In the majority of patients, epileptic seizures, contralateral hemiparesis or hemiplegia, intellectual disability, facial asymmetry, language and speech disorders, learning problems, contralateral choreic movements, sensory disorders, and unstable gait are among the hallmark’s features of the disease [ 1 , 2 , 7 ]. The pathological insults in DDMS can either be congenital or acquired [ 7 ]. The congenital or primary type is marked by cerebral injury during the intrauterine or neonatal period, most often due to vascular etiologies, resulting in symptoms at birth or shortly afterwards. The acquired or secondary type develops during infancy or later in life and can be caused by trauma, infections, cerebral vascular abnormalities, cerebral bleeding or ischemia, immunological disorders, neoplasms, or an indeterminate cause[ 1 , 7 ]. The age of onset is determined by when the neurologic insult transpired, and distinct changes may not appear until puberty. For reasons which remains elusive, DDMS displays a male gender and left hemispheric predominance [ 8 ]. However, it is speculated that left hemisphere is more susceptible to cerebral vascular accidents in early life because its reduced blood flow compared to the right hemisphere [ 8 ]. Computed tomography (CT) scan, or magnetic resonance imaging (MRI) of the brain are the gold standard for diagnosing DDMS. The etiology influences the radiological findings: Congenital DDMS is characterized by cerebral volume loss, ipsilateral ventricular enlargement, gliosis, falx cerebri shift, hypoplasia of the cerebral peduncle, thalamus, or internal capsule, and contralateral cerebellar atrophy, in contrast acquired DDMS is distinguished by dilated sulci, encephalomalacia, calvarial thickening, and hyperpneumatization of the paranasal sinuses [ 9 ]. In this report, we present a 30-year-old Tanzanian female whose clinical and radiological findings are consistent with DDMS Case presentation Demographic and medical history A thirty-year-old female presented with a history of recurrent seizures and intellectual disability. Her delivery was through the vaginal route with unremarkable post-natal history. Her developmental milestones were normal until the age of three years when she was hospitalized for two weeks due to a febrile illness and generalized seizures, with full recovery. However, her school age was notable for intellectual impairment lagging behind her peers in class, which prevented her from continuing with secondary education. There was no history of speech or language regression that was obtained. During adolescence, she noticed an insidious onset of inability to lift objects and maintain grip on the left upper limb, and the left lower limb was increasingly becoming weak as well. She had recurrent seizures four years prior to presentation; they started with the right side of her body shortly afterward spread to involve the rest of her body. Seizures were tonic-clonic, with brief episodes of loss of consciousness and confusion afterwards. Following seizures, either tongue biting or urine and fecal incontinence were noticed. Since then, two seizure episodes occurred every two years. There was no history of trauma, surgery, or similar presentation within the family or close relatives. Clinical findings A general examination revealed a young asthenic female who appeared small for her age. There was no port-wine stain on her face. Her body weight was 46 kg, and her height was 1.63m (BMI = 17.3 kg/m2). She earned a score of 14/60 on the Ravens Standard Progressive Matrices (RSPM) [ 10 ] indicating an intellectual disability. The neurological examination revealed constructional apraxia, facial asymmetry consistent with a facial nerve upper motor neuron lesion (UMNL), a slow, sinuous writhing movement involving all fingers, and left spastic paresis; power was 4 out of 5 in both upper and lower limbs. Other examination findings were essentially normal. Investigations Computed Tomography A brain CT scan revealed a decrease in right cerebral hemisphere volume with gross dilatation of the right lateral ventricle with ex-vacuo dilatation of the right lateral ventricle (Fig. 1 A). Furthermore, the right side had calvarial thickening (Fig. 1 B) and hyperpneumatization of the right frontal sinus compared to the left side (Fig. 1 C). Electroencephalogram (EEG) Her EEG (Fig. 2 ) revealed a low-amplitude background over the right hemisphere with an occasional burst of widespread sharp wave and spike consistent with structural-metabolic causes [ 10 ]. Differential diagnosis The clinical features of the case study were consistent with DDMS due to a history of recurrent seizures, intellectual disability, constructional apraxia, a facial nerve UMNL, left spastic hemiparesis, and choreic movement involving the left hand's fingers. A CT scan of the head backed up DDMS by revealing diffuse right cerebral hemisphere volume loss, ex-vacuo dilatation of the right lateral ventricle, hyperpneumatization of the right frontal sinus, and thickening of the right skull vault [ 9 ]. Rasmussen encephalitis, basal ganglia germinoma, Sturge-Weber syndrome, Silver-Russel syndrome, linear nevus syndrome, and Fisherman syndrome were also considered as differential diagnoses due to their similar presentation to DDMS [ 10 ]. The clinical characteristics of Sturge-Weber syndrome include midline shift, intracranial tram track calcifications, and port-wine facial nevus, all of which were absent in this case except for seizures, mental retardation, and hemiparesis [ 11 ]. Uncontrolled focal seizures, cognitive decline, and unilateral hemiatrophy are all associated with Rasmussen encephalitis. However, the CT scan finding towards calvarial thickening ruled out Rasmussen encephalitis in this case [ 12 ]. Similarly, given the history of recurrent seizures, intellectual disability, and unilateral ventricular dilatation, the diagnosis of linear nevus syndrome was most likely drawn in this instance. Nevertheless, it was excluded due to the lack of facial nerve palsy and the presence of radiological signs indicative of DDMS [ 11 ]. On the other hand, the absence of distinguishing facial phenotypes, such as a triangle face, broad forehead, short, pointed chin, and downturned corner of the mouth ruled out Silver-Russel syndrome. Conversely, the presence of hemiatrophy and seizures in this instance prompted an inquiry of Fishman syndrome, an uncommon neurocutaneous condition. It is less likely, though, because there were no cutaneous symptoms, and brain imaging showed neither calcified cortex nor unilateral cranial lipoma [ 13 ]. Therapeutic intervention The patient was initially prescribed 200 mg of phenobarbital once daily. One year later, the dosage was reduced to 120 mg per day. Sodium valporate was started and phenobarbital was gradually tapered after additional tonic-clonic seizure episodes. She remained seizure free for the next two years while on sodium valproate 500mg twice daily, as a monotherapy. Outcome and follow up She is currently taking 500mg of sodium valporate twice daily, in addition to cognitive and physical rehabilitation. She visits the neurology clinic on a monthly basis where blood counts, hepatic and renal function are checked. Furthermore, concerns about medication use, seizure control, and potential side effects are addressed. Discussion DDMS is a rare neurological condition named after the three physicians who first jointly described it in 1933. It occurs after brain injury and causes hypoplasia in one cerebral hemisphere. DDMS can be either congenital or acquired. Congenital DDMS usually has no obvious cause. Symptoms typically appear at birth or soon after. The acquired type is caused by a central nervous system injury arising during or after pregnancy. Trauma, ischemic and hemorrhagic conditions, infection, and vascular abnormalities have all been reported as etiological factors [ 7 ]. Even though DDMS is primarily thought of as a pediatrics illness, adult cases have been documented in the past; a recent review found only 21 cases in this group [ 14 ]. In the adult population, the mean age of seizure onset was 6.7 ± 8.2 (range: 0.52-29) years, and the mean age of diagnosis was 39 ± 18 (range: 18–83) years. The right hemisphere was primarily affected, while 57.7% of cases were female, in contrast to a study that revealed male sex dominance in a predominantly pediatrics population [ 14 ]. Our patient had her first seizure in the second decade that was preceded by an acute illness characterized by febrile illness at age of 3 years. She was 30 years old at the time of diagnosis, and the right hemisphere was primarily affected. Clinically, DDMS can present with a range of symptoms that vary in severity with regard to the extent of injury. Some of the more noticeable features include seizures (both focal and generalized), facial asymmetry, contralateral hemiparesis, language disorders, and intellectual disability, as well as delayed milestones. Contralateral choreic movements, sensory disorders, and unstable gait have been reported as well [ 9 ]. The index case presented with typical symptoms of DDMS except for well controlled epilepsy on monotherapy. Establishing the diagnosis is largely dependent on the radiological results of CT or MRI scans, patients will exhibit different findings, but commonly, there is atrophy of the cerebral hemisphere adjacent to the lesion, lateral ventricular dilatation, and prominent sulci. Hyperpneumatization of air sinuses, such as the frontal sinus, and compensatory hypertrophy of the skull bones are two other notable abnormalities [ 15 ]. It has been suggested that acquired DDMS mostly present with dilated sulci, calvarial thickening, and hyperpneumatization of the paranasal sinuses and/or mastoid cells. In the present case, the brain imaging findings were hypoplasia of right cerebral hemisphere, skull thickening on right side and frontal sinus enlargement keeping with compensatory changes in acquired type of DDMS. The goal of DDMS management is to optimize anticonvulsant to attain seizure control through dosage adjustments. In cases of debilitating seizures, hemispherectomy—which is successful in 85% of cases—should be the last alternative [ 16 ]. Occupational therapy, speech therapy, and physical rehabilitation are some of the intervention employed depending on patient needs [ 17 ]. The prognosis is favorable when hemiparesis occurs beyond the age of two years and there is an absence of protracted or recurring seizures [ 18 ]. Conclusion In the setting of recurrent seizures, intellectual disability, hemiparesis, or facial asymmetry, a clinician should have a high index of suspicion for Dyke-Davidoff Masson Syndrome (DDMS), because late diagnosis is often related to intractable seizures to anticonvulsants, necessitating hemispherectomy, which is not a readily available option in limited resource settings. Declarations Consent for publication Written informed consent was obtained from the patient to publish this report in accordance with the journal’s patient consent policy. Availability of data and materials This case report's complete data is available on a justifiable request. Competing interests The authors declare there is no conflict of interest Funding There is no funding attained for this work Author contribution Baraka Alphonce: Writing – original draft; writing – review and editing. Francisca Komanya: Writing – original draft; writing – review and editing. Ng'weina Magitta: Writing – original draft; writing – review and editing. Mbelwa Bitesigilwe: Writing – original draft; writing – review and editing. John R Meda: Supervision; writing – review and editing. Azan Nyundo: Supervision; writing – review and editing. Acknowledgements We thank the patient in question for her consent to publish this case report. We are grateful to the Departments of Internal Medicine, Psychiatry, and Radiology & Imaging at Benjamin Mkapa Hospital for their valuable support. References Angel UEB, Chico JAR, Rosales HAM, García JV, Flores PS, Parra AL, et al. Dyke Davidoff Masson: reporte de cuatro casos y revisión de la literatura. Revista Chilena de Neurocirugia. 2015 Oct 1. 41(2):167–74. Thakkar PA, Dave RH. Dyke-Davidoff-Masson syndrome: A rare cause of cerebral hemiatrophy in children. Journal of pediatric neurosciences. 2016 Jul 1. 11(3):252–4. doi: 10.4103/1817-1745.193365 Djimdé SO, Yalcouyé A, Koïta A, Samir H, Kebkiba P, Gueli CA, et al. An unusual case of Dyke–Davidoff–Masson syndrome revealed by status epilepticus in a Malian patient. Clinical Case Reports. 2022 Oct 1. 10(10):e6428. doi: 10.1002/CCR3.6428 Ayele BA, Zewde YZ. DYKE-DAVIDOFF-MASSON SYNDROME-A Rare Cause of Cerebral Hemiatrophy in a 17-Years-Old Ethiopian Patient: A Case Report. Ethiopian journal of health sciences. 2019 Mar 1. 29(2):287–90. doi: 10.4314/EJHS.V29I2.16 Adebayo PB, Bakare A, Bello MM, Olaewe OD, Wahab KW. Dyke-Davidoff-Masson syndrome in a Nigerian. Epilepsy & behavior case reports. 2016. 7:10–2. doi: 10.1016/J.EBCR.2016.09.003 Benjamin Nwako A, Emeka Nwolisa C, Francis Nwako O, Chidimma Nwako ML. Dyke-Davidoff-Masson Syndrome as a rare congenital hemiatrophy: a case report. ajol.infoAB Nwako, CE Nwolisa, OF Nwako, MLC NwakoTanzania Journal of Health Research, 2021•ajol.info. 2021. 22(1). doi: 10.4314/thrb.v22i1.4 Aguiar PH, Liu CW, Leitao H, Issa F, Lepski G, Figueiredo EG, et al. MR and CT imaging in the Dyke-Davidoff-Masson syndrome. Report of three cases and contribution to pathogenesis and differential diagnosis. Arquivos de neuro-psiquiatria. 1998. 56(4):803–7. doi: 10.1590/S0004-282X1998000500016 Ünal Ö, Tombul T, Çirak B, Anlar Ö, Incesu L, Kayan M. Left hemisphere and male sex dominance of cerebral hemiatrophy (Dyke-Davidoff-Masson Syndrome). Clinical Imaging. 2004 May. 28(3):163–5. doi: 10.1016/S0899-7071(03)00158-X Rondão MBA, Hsu BRRHS, Centeno RS, de Aguiar PHP. Dyke-Davidoff-Masson Syndrome: Main clinical and radiological findings- systematic literature review. Seizure. 2023 Aug 1. 110:58–68. doi: 10.1016/J.SEIZURE.2023.04.020 Behera MR, Patnaik S, Mohanty AK, Behera MR, Patnaik S, Mohanty AK. Dyke-Davidoff-Masson syndrome. Journal of Neurosciences in Rural Practice. 2012 Sep. 3(3):411–3. doi: 10.4103/0976-3147.102646 Jacoby CG, Go RT, Hahn FJ. Computed tomography in cerebral hemiatrophy. American Journal of Roentgenology. 1977. 129(1):5–9. doi: 10.2214/AJR.129.1.5 Sheybani L, … KSSAN, 2011 undefined. Rasmussen’s encephalitis: an update. pdfs.semanticscholar.orgL Sheybani, K Schaller, M SeeckSchweiz Arch Neurol Psychiatr, 2011•pdfs.semanticscholar.org. Behera MR, Patnaik S, Mohanty AK. Dyke-Davidoff-Masson syndrome. Journal of Neurosciences in Rural Practice. 2012 Sep. 3(3):411. doi: 10.4103/0976-3147.102646 Diestro JDB, Dorotan MKC, Camacho AC, Perez-Gosiengfiao KT, Cabral-Lim LI. Clinical spectrum of Dyke-Davidoff-Masson syndrome in the adult: an atypical presentation and review of literature. Case Reports. 2018 Jul 4. 2018:bcr-2018-224170. doi: 10.1136/BCR-2018-224170 Younas A, Saim M, Maqsood H, Younus S, Raza MH, Younas A, et al. Dyke-Davidoff-Masson Syndrome: A Case Report and Review of Literature. Cureus. 2020 Dec 5. 12(12). doi: 10.7759/CUREUS.11919 Sordia-Ramírez J, Infante-Valenzuela A, Hernández-Galarza IDJ, Costilla-Esquivel A. Neuropsychiatric symptoms in a patient with Dyke–Davidoff–Masson syndrome and systemic lupus erythematosus: a case report. Journal of Medical Case Reports. 2019 Apr 29. 13(1). doi: 10.1186/S13256-019-2039-2 Arora R, Rani JY. Dyke-Davidoff-Masson syndrome: imaging features with illustration of two cases. Quantitative Imaging in Medicine and Surgery. 2015 Jun. 5(3):469. doi: 10.3978/J.ISSN.2223-4292.2014.11.17 Thakkar PA, Dave RH. Dyke-Davidoff-Masson syndrome: A rare cause of cerebral hemiatrophy in children. Journal of pediatric neurosciences. 2016 Jul 1. 11(3):252–4. doi: 10.4103/1817-1745.193365 Additional Declarations No competing interests reported. Cite Share Download PDF Status: Posted Version 1 posted You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-4095531","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":280819172,"identity":"925267ba-e14e-41d8-8bff-d1ee0758195c","order_by":0,"name":"Baraka Alphonce","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAABAklEQVRIiWNgGAWjYDACdgY2CEMCRBxgkANTD/BpYUbTYgymEkjRktgAovFp4W/mPfbg44478vzSzc8+/Dhjlz4/7PBDoC12croN2LVIHOZLN5x55pnhzDnHjGf23EjO3Xg7zQCoJdnY7AAOaw7zmEnzth1OMLiRYMzA84E5d+PsBJCWA4nbcGiRh2mxv5H+mfHPh/p0w9npH/BqMYDbIpFjzMxz43CCvHQOflsMD/OlSc5sO2w4486ZYmaZM8cNN0jnFBxIMMDtF7njvcckPrYdluef3b6Z8c2xann52embP3yosJPD6X0GHnSnglUa4FKOTYt8Az7Vo2AUjIJRMBIBAMGhZDoMZMpUAAAAAElFTkSuQmCC","orcid":"","institution":"Benjamin Mkapa Hospital","correspondingAuthor":true,"prefix":"","firstName":"Baraka","middleName":"","lastName":"Alphonce","suffix":""},{"id":280819173,"identity":"eb5d34a1-783e-4020-bb04-bc0898ef4197","order_by":1,"name":"Ng’weina Magitta","email":"","orcid":"","institution":"University of Dodoma","correspondingAuthor":false,"prefix":"","firstName":"Ng’weina","middleName":"","lastName":"Magitta","suffix":""},{"id":280819174,"identity":"fb14713a-3f81-49f6-bf62-9111676c965b","order_by":2,"name":"Francisca Komanya","email":"","orcid":"","institution":"Benjamin Mkapa Hospital","correspondingAuthor":false,"prefix":"","firstName":"Francisca","middleName":"","lastName":"Komanya","suffix":""},{"id":280819175,"identity":"4b3209e7-a604-4fda-b2a3-62b1c9600701","order_by":3,"name":"Mbelwa Bitesigilwe","email":"","orcid":"","institution":"Benjamin Mkapa Hospital","correspondingAuthor":false,"prefix":"","firstName":"Mbelwa","middleName":"","lastName":"Bitesigilwe","suffix":""},{"id":280819178,"identity":"4bc8ee0a-6588-4c37-b951-c6fe6b020d6b","order_by":4,"name":"John Meda","email":"","orcid":"","institution":"University of Dodoma","correspondingAuthor":false,"prefix":"","firstName":"John","middleName":"","lastName":"Meda","suffix":""},{"id":280819179,"identity":"38bad13c-2c2a-46ae-b586-a0a5b6876142","order_by":5,"name":"Azan Nyundo","email":"","orcid":"","institution":"University of Dodoma","correspondingAuthor":false,"prefix":"","firstName":"Azan","middleName":"","lastName":"Nyundo","suffix":""}],"badges":[],"createdAt":"2024-03-13 19:29:14","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-4095531/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-4095531/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":53193546,"identity":"f804c8b0-5412-47e6-9db8-3b81f1834233","added_by":"auto","created_at":"2024-03-21 18:01:30","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":624149,"visible":true,"origin":"","legend":"\u003cp\u003ePanel (A): Brain Axial CT non-enhanced soft tissue window shows diffuse right cerebral hemisphere volume loss with ex-vacuo dilatation of the right lateral ventricle (arrow head). Panel (B\u0026amp;C): Brain Axial CT non-enhanced bone window demonstrate thickening of the skull vault on the right (arrow) (B) and hyperpneumatization of right frontal sinus (asterisk) (C).\u003c/p\u003e","description":"","filename":"floatimage1.png","url":"https://assets-eu.researchsquare.com/files/rs-4095531/v1/b212a3c336e59a6db4c1c495.png"},{"id":53193545,"identity":"ea26201f-763c-4d82-b477-cd1e3b5e17d8","added_by":"auto","created_at":"2024-03-21 18:01:30","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":1442199,"visible":true,"origin":"","legend":"\u003cp\u003eEEG shows a low-amplitude background over the right hemisphere with an occasional burst of widespread sharp wave and spike\u003c/p\u003e","description":"","filename":"floatimage2.png","url":"https://assets-eu.researchsquare.com/files/rs-4095531/v1/2d7128d90e820e8dec1cd5a6.png"},{"id":58029798,"identity":"9a9b444e-bb12-4e4f-ba0b-1b39ee12ac04","added_by":"auto","created_at":"2024-06-10 07:34:12","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":2495019,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-4095531/v1/29e01570-3c67-4e60-8483-9994278a6a91.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Dyke-Davidoff-Masson Syndrome in a Tanzanian patient: A case report","fulltext":[{"header":"Introduction","content":"\u003cp\u003eDyke-Davidoff-Masson Syndrome (DDMS), also known as cerebral hemiatrophy, was initially defined by Dyke, Davidoff, and Masson in 1933 [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e]. It is an uncommon condition that results after a developing brain injury leading to hypoplasia in one cerebral hemisphere [\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e]. Indeed, by the year 2022, there were only five cases that were reported in African population [\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e]. In four cases, the diagnosis was made in the second decade of life [\u003cspan additionalcitationids=\"CR4\" citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e], with the exception of one case in which the diagnosis was made at the age of three [\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e]. The disease is characterized by diverse neurological features and deficits. In the majority of patients, epileptic seizures, contralateral hemiparesis or hemiplegia, intellectual disability, facial asymmetry, language and speech disorders, learning problems, contralateral choreic movements, sensory disorders, and unstable gait are among the hallmark\u0026rsquo;s features of the disease [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e, \u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e, \u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eThe pathological insults in DDMS can either be congenital or acquired [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]. The congenital or primary type is marked by cerebral injury during the intrauterine or neonatal period, most often due to vascular etiologies, resulting in symptoms at birth or shortly afterwards. The acquired or secondary type develops during infancy or later in life and can be caused by trauma, infections, cerebral vascular abnormalities, cerebral bleeding or ischemia, immunological disorders, neoplasms, or an indeterminate cause[\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e, \u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]. The age of onset is determined by when the neurologic insult transpired, and distinct changes may not appear until puberty. For reasons which remains elusive, DDMS displays a male gender and left hemispheric predominance [\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e]. However, it is speculated that left hemisphere is more susceptible to cerebral vascular accidents in early life because its reduced blood flow compared to the right hemisphere [\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eComputed tomography (CT) scan, or magnetic resonance imaging (MRI) of the brain are the gold standard for diagnosing DDMS. The etiology influences the radiological findings: Congenital DDMS is characterized by cerebral volume loss, ipsilateral ventricular enlargement, gliosis, falx cerebri shift, hypoplasia of the cerebral peduncle, thalamus, or internal capsule, and contralateral cerebellar atrophy, in contrast acquired DDMS is distinguished by dilated sulci, encephalomalacia, calvarial thickening, and hyperpneumatization of the paranasal sinuses [\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e]. In this report, we present a 30-year-old Tanzanian female whose clinical and radiological findings are consistent with DDMS\u003c/p\u003e"},{"header":"Case presentation","content":"\u003cdiv id=\"Sec3\" class=\"Section2\"\u003e \u003ch2\u003eDemographic and medical history\u003c/h2\u003e \u003cp\u003eA thirty-year-old female presented with a history of recurrent seizures and intellectual disability. Her delivery was through the vaginal route with unremarkable post-natal history. Her developmental milestones were normal until the age of three years when she was hospitalized for two weeks due to a febrile illness and generalized seizures, with full recovery. However, her school age was notable for intellectual impairment lagging behind her peers in class, which prevented her from continuing with secondary education. There was no history of speech or language regression that was obtained. During adolescence, she noticed an insidious onset of inability to lift objects and maintain grip on the left upper limb, and the left lower limb was increasingly becoming weak as well.\u003c/p\u003e \u003cp\u003eShe had recurrent seizures four years prior to presentation; they started with the right side of her body shortly afterward spread to involve the rest of her body. Seizures were tonic-clonic, with brief episodes of loss of consciousness and confusion afterwards. Following seizures, either tongue biting or urine and fecal incontinence were noticed. Since then, two seizure episodes occurred every two years. There was no history of trauma, surgery, or similar presentation within the family or close relatives.\u003c/p\u003e \u003c/div\u003e \u003cdiv id=\"Sec4\" class=\"Section2\"\u003e \u003ch2\u003eClinical findings\u003c/h2\u003e \u003cp\u003eA general examination revealed a young asthenic female who appeared small for her age. There was no port-wine stain on her face. Her body weight was 46 kg, and her height was 1.63m (BMI\u0026thinsp;=\u0026thinsp;17.3 kg/m2). She earned a score of 14/60 on the Ravens Standard Progressive Matrices (RSPM) [\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e] indicating an intellectual disability. The neurological examination revealed constructional apraxia, facial asymmetry consistent with a facial nerve upper motor neuron lesion (UMNL), a slow, sinuous writhing movement involving all fingers, and left spastic paresis; power was 4 out of 5 in both upper and lower limbs. Other examination findings were essentially normal.\u003c/p\u003e \u003c/div\u003e \u003cdiv id=\"Sec5\" class=\"Section2\"\u003e \u003ch2\u003eInvestigations\u003c/h2\u003e \u003cdiv id=\"Sec6\" class=\"Section3\"\u003e \u003ch2\u003eComputed Tomography\u003c/h2\u003e \u003cp\u003eA brain CT scan revealed a decrease in right cerebral hemisphere volume with gross dilatation of the right lateral ventricle with ex-vacuo dilatation of the right lateral ventricle (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003eA). Furthermore, the right side had calvarial thickening (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003eB) and hyperpneumatization of the right frontal sinus compared to the left side (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003eC).\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv id=\"Sec7\" class=\"Section2\"\u003e \u003ch2\u003eElectroencephalogram (EEG)\u003c/h2\u003e \u003cp\u003eHer EEG (Fig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003e) revealed a low-amplitude background over the right hemisphere with an occasional burst of widespread sharp wave and spike consistent with structural-metabolic causes [\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e].\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003c/div\u003e \u003cdiv id=\"Sec8\" class=\"Section2\"\u003e \u003ch2\u003eDifferential diagnosis\u003c/h2\u003e \u003cp\u003eThe clinical features of the case study were consistent with DDMS due to a history of recurrent seizures, intellectual disability, constructional apraxia, a facial nerve UMNL, left spastic hemiparesis, and choreic movement involving the left hand's fingers. A CT scan of the head backed up DDMS by revealing diffuse right cerebral hemisphere volume loss, \u003cem\u003eex-vacuo\u003c/em\u003e dilatation of the right lateral ventricle, hyperpneumatization of the right frontal sinus, and thickening of the right skull vault [\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e]. Rasmussen encephalitis, basal ganglia germinoma, Sturge-Weber syndrome, Silver-Russel syndrome, linear nevus syndrome, and Fisherman syndrome were also considered as differential diagnoses due to their similar presentation to DDMS [\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e]. The clinical characteristics of Sturge-Weber syndrome include midline shift, intracranial tram track calcifications, and port-wine facial nevus, all of which were absent in this case except for seizures, mental retardation, and hemiparesis [\u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e]. Uncontrolled focal seizures, cognitive decline, and unilateral hemiatrophy are all associated with Rasmussen encephalitis. However, the CT scan finding towards calvarial thickening ruled out Rasmussen encephalitis in this case [\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e]. Similarly, given the history of recurrent seizures, intellectual disability, and unilateral ventricular dilatation, the diagnosis of linear nevus syndrome was most likely drawn in this instance. Nevertheless, it was excluded due to the lack of facial nerve palsy and the presence of radiological signs indicative of DDMS [\u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e]. On the other hand, the absence of distinguishing facial phenotypes, such as a triangle face, broad forehead, short, pointed chin, and downturned corner of the mouth ruled out Silver-Russel syndrome. Conversely, the presence of hemiatrophy and seizures in this instance prompted an inquiry of Fishman syndrome, an uncommon neurocutaneous condition. It is less likely, though, because there were no cutaneous symptoms, and brain imaging showed neither calcified cortex nor unilateral cranial lipoma [\u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e].\u003c/p\u003e \u003c/div\u003e \u003cdiv id=\"Sec9\" class=\"Section2\"\u003e \u003ch2\u003eTherapeutic intervention\u003c/h2\u003e \u003cp\u003eThe patient was initially prescribed 200 mg of phenobarbital once daily. One year later, the dosage was reduced to 120 mg per day. Sodium valporate was started and phenobarbital was gradually tapered after additional tonic-clonic seizure episodes. She remained seizure free for the next two years while on sodium valproate 500mg twice daily, as a monotherapy.\u003c/p\u003e \u003c/div\u003e\n\u003ch3\u003eOutcome and follow up\u003c/h3\u003e\n\u003cp\u003eShe is currently taking 500mg of sodium valporate twice daily, in addition to cognitive and physical rehabilitation. She visits the neurology clinic on a monthly basis where blood counts, hepatic and renal function are checked. Furthermore, concerns about medication use, seizure control, and potential side effects are addressed.\u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003eDDMS is a rare neurological condition named after the three physicians who first jointly described it in 1933. It occurs after brain injury and causes hypoplasia in one cerebral hemisphere. DDMS can be either congenital or acquired. Congenital DDMS usually has no obvious cause. Symptoms typically appear at birth or soon after. The acquired type is caused by a central nervous system injury arising during or after pregnancy. Trauma, ischemic and hemorrhagic conditions, infection, and vascular abnormalities have all been reported as etiological factors [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]. Even though DDMS is primarily thought of as a pediatrics illness, adult cases have been documented in the past; a recent review found only 21 cases in this group [\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e]. In the adult population, the mean age of seizure onset was 6.7\u0026thinsp;\u0026plusmn;\u0026thinsp;8.2 (range: 0.52-29) years, and the mean age of diagnosis was 39\u0026thinsp;\u0026plusmn;\u0026thinsp;18 (range: 18\u0026ndash;83) years. The right hemisphere was primarily affected, while 57.7% of cases were female, in contrast to a study that revealed male sex dominance in a predominantly pediatrics population [\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e]. Our patient had her first seizure in the second decade that was preceded by an acute illness characterized by febrile illness at age of 3 years. She was 30 years old at the time of diagnosis, and the right hemisphere was primarily affected. Clinically, DDMS can present with a range of symptoms that vary in severity with regard to the extent of injury. Some of the more noticeable features include seizures (both focal and generalized), facial asymmetry, contralateral hemiparesis, language disorders, and intellectual disability, as well as delayed milestones. Contralateral choreic movements, sensory disorders, and unstable gait have been reported as well [\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e]. The index case presented with typical symptoms of DDMS except for well controlled epilepsy on monotherapy.\u003c/p\u003e \u003cp\u003eEstablishing the diagnosis is largely dependent on the radiological results of CT or MRI scans, patients will exhibit different findings, but commonly, there is atrophy of the cerebral hemisphere adjacent to the lesion, lateral ventricular dilatation, and prominent sulci. Hyperpneumatization of air sinuses, such as the frontal sinus, and compensatory hypertrophy of the skull bones are two other notable abnormalities [\u003cspan citationid=\"CR15\" class=\"CitationRef\"\u003e15\u003c/span\u003e]. It has been suggested that acquired DDMS mostly present with dilated sulci, calvarial thickening, and hyperpneumatization of the paranasal sinuses and/or mastoid cells. In the present case, the brain imaging findings were hypoplasia of right cerebral hemisphere, skull thickening on right side and frontal sinus enlargement keeping with compensatory changes in acquired type of DDMS.\u003c/p\u003e \u003cp\u003eThe goal of DDMS management is to optimize anticonvulsant to attain seizure control through dosage adjustments. In cases of debilitating seizures, hemispherectomy\u0026mdash;which is successful in 85% of cases\u0026mdash;should be the last alternative [\u003cspan citationid=\"CR16\" class=\"CitationRef\"\u003e16\u003c/span\u003e]. Occupational therapy, speech therapy, and physical rehabilitation are some of the intervention employed depending on patient needs [\u003cspan citationid=\"CR17\" class=\"CitationRef\"\u003e17\u003c/span\u003e]. The prognosis is favorable when hemiparesis occurs beyond the age of two years and there is an absence of protracted or recurring seizures [\u003cspan citationid=\"CR18\" class=\"CitationRef\"\u003e18\u003c/span\u003e].\u003c/p\u003e"},{"header":"Conclusion","content":"\u003cp\u003eIn the setting of recurrent seizures, intellectual disability, hemiparesis, or facial asymmetry, a clinician should have a high index of suspicion for Dyke-Davidoff Masson Syndrome (DDMS), because late diagnosis is often related to intractable seizures to anticonvulsants, necessitating hemispherectomy, which is not a readily available option in limited resource settings.\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eConsent for publication\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eWritten informed consent was obtained from the patient to publish this report in accordance with the journal\u0026rsquo;s patient consent policy.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAvailability of data and materials\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThis case report\u0026apos;s complete data is available on a justifiable request.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCompeting interests\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe authors declare there is no conflict of interest\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eFunding\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThere is no funding attained for this work\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAuthor contribution\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eBaraka Alphonce: Writing \u0026ndash; original draft; writing \u0026ndash; review and editing. Francisca Komanya: \u0026nbsp;Writing \u0026ndash; original draft; writing \u0026ndash; review and editing. Ng\u0026apos;weina Magitta: Writing \u0026ndash; original draft; writing \u0026ndash; review and editing. Mbelwa Bitesigilwe: Writing \u0026ndash; original draft; writing \u0026ndash; review and editing. John R Meda: Supervision; writing \u0026ndash; review and editing. Azan Nyundo: Supervision; writing \u0026ndash; review and editing.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAcknowledgements\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eWe thank the patient in question for her consent to publish this case report. We are grateful to the Departments of Internal Medicine, Psychiatry, and Radiology \u0026amp; Imaging at Benjamin Mkapa Hospital for their valuable support.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n\u003cli\u003eAngel UEB, Chico JAR, Rosales HAM, Garc\u0026amp;iacute;a JV, Flores PS, Parra AL, et al. Dyke Davidoff Masson: reporte de cuatro casos y revisi\u0026amp;oacute;n de la literatura. Revista Chilena de Neurocirugia. 2015 Oct 1. 41(2):167\u0026ndash;74. \u003c/li\u003e\n\u003cli\u003eThakkar PA, Dave RH. Dyke-Davidoff-Masson syndrome: A rare cause of cerebral hemiatrophy in children. Journal of pediatric neurosciences. 2016 Jul 1. 11(3):252\u0026ndash;4. doi: 10.4103/1817-1745.193365\u003c/li\u003e\n\u003cli\u003eDjimd\u0026eacute; SO, Yalcouy\u0026eacute; A, Ko\u0026iuml;ta A, Samir H, Kebkiba P, Gueli CA, et al. An unusual case of Dyke\u0026ndash;Davidoff\u0026ndash;Masson syndrome revealed by status epilepticus in a Malian patient. Clinical Case Reports. 2022 Oct 1. 10(10):e6428. doi: 10.1002/CCR3.6428\u003c/li\u003e\n\u003cli\u003eAyele BA, Zewde YZ. DYKE-DAVIDOFF-MASSON SYNDROME-A Rare Cause of Cerebral Hemiatrophy in a 17-Years-Old Ethiopian Patient: A Case Report. Ethiopian journal of health sciences. 2019 Mar 1. 29(2):287\u0026ndash;90. doi: 10.4314/EJHS.V29I2.16\u003c/li\u003e\n\u003cli\u003eAdebayo PB, Bakare A, Bello MM, Olaewe OD, Wahab KW. Dyke-Davidoff-Masson syndrome in a Nigerian. Epilepsy \u0026amp; behavior case reports. 2016. 7:10\u0026ndash;2. doi: 10.1016/J.EBCR.2016.09.003\u003c/li\u003e\n\u003cli\u003eBenjamin Nwako A, Emeka Nwolisa C, Francis Nwako O, Chidimma Nwako ML. Dyke-Davidoff-Masson Syndrome as a rare congenital hemiatrophy: a case report. ajol.infoAB Nwako, CE Nwolisa, OF Nwako, MLC NwakoTanzania Journal of Health Research, 2021\u0026bull;ajol.info. 2021. 22(1). doi: 10.4314/thrb.v22i1.4\u003c/li\u003e\n\u003cli\u003eAguiar PH, Liu CW, Leitao H, Issa F, Lepski G, Figueiredo EG, et al. MR and CT imaging in the Dyke-Davidoff-Masson syndrome. Report of three cases and contribution to pathogenesis and differential diagnosis. Arquivos de neuro-psiquiatria. 1998. 56(4):803\u0026ndash;7. doi: 10.1590/S0004-282X1998000500016\u003c/li\u003e\n\u003cli\u003e\u0026Uuml;nal \u0026Ouml;, Tombul T, \u0026Ccedil;irak B, Anlar \u0026Ouml;, Incesu L, Kayan M. Left hemisphere and male sex dominance of cerebral hemiatrophy (Dyke-Davidoff-Masson Syndrome). Clinical Imaging. 2004 May. 28(3):163\u0026ndash;5. doi: 10.1016/S0899-7071(03)00158-X\u003c/li\u003e\n\u003cli\u003eRond\u0026atilde;o MBA, Hsu BRRHS, Centeno RS, de Aguiar PHP. Dyke-Davidoff-Masson Syndrome: Main clinical and radiological findings- systematic literature review. Seizure. 2023 Aug 1. 110:58\u0026ndash;68. doi: 10.1016/J.SEIZURE.2023.04.020\u003c/li\u003e\n\u003cli\u003eBehera MR, Patnaik S, Mohanty AK, Behera MR, Patnaik S, Mohanty AK. Dyke-Davidoff-Masson syndrome. Journal of Neurosciences in Rural Practice. 2012 Sep. 3(3):411\u0026ndash;3. doi: 10.4103/0976-3147.102646\u003c/li\u003e\n\u003cli\u003eJacoby CG, Go RT, Hahn FJ. Computed tomography in cerebral hemiatrophy. American Journal of Roentgenology. 1977. 129(1):5\u0026ndash;9. doi: 10.2214/AJR.129.1.5\u003c/li\u003e\n\u003cli\u003eSheybani L, \u0026hellip; KSSAN, 2011 undefined. Rasmussen\u0026rsquo;s encephalitis: an update. pdfs.semanticscholar.orgL Sheybani, K Schaller, M SeeckSchweiz Arch Neurol Psychiatr, 2011\u0026bull;pdfs.semanticscholar.org. \u003c/li\u003e\n\u003cli\u003eBehera MR, Patnaik S, Mohanty AK. Dyke-Davidoff-Masson syndrome. Journal of Neurosciences in Rural Practice. 2012 Sep. 3(3):411. doi: 10.4103/0976-3147.102646\u003c/li\u003e\n\u003cli\u003eDiestro JDB, Dorotan MKC, Camacho AC, Perez-Gosiengfiao KT, Cabral-Lim LI. Clinical spectrum of Dyke-Davidoff-Masson syndrome in the adult: an atypical presentation and review of literature. Case Reports. 2018 Jul 4. 2018:bcr-2018-224170. doi: 10.1136/BCR-2018-224170\u003c/li\u003e\n\u003cli\u003eYounas A, Saim M, Maqsood H, Younus S, Raza MH, Younas A, et al. Dyke-Davidoff-Masson Syndrome: A Case Report and Review of Literature. Cureus. 2020 Dec 5. 12(12). doi: 10.7759/CUREUS.11919\u003c/li\u003e\n\u003cli\u003eSordia-Ram\u0026iacute;rez J, Infante-Valenzuela A, Hern\u0026aacute;ndez-Galarza IDJ, Costilla-Esquivel A. Neuropsychiatric symptoms in a patient with Dyke\u0026ndash;Davidoff\u0026ndash;Masson syndrome and systemic lupus erythematosus: a case report. Journal of Medical Case Reports. 2019 Apr 29. 13(1). doi: 10.1186/S13256-019-2039-2\u003c/li\u003e\n\u003cli\u003eArora R, Rani JY. Dyke-Davidoff-Masson syndrome: imaging features with illustration of two cases. Quantitative Imaging in Medicine and Surgery. 2015 Jun. 5(3):469. doi: 10.3978/J.ISSN.2223-4292.2014.11.17\u003c/li\u003e\n\u003cli\u003eThakkar PA, Dave RH. Dyke-Davidoff-Masson syndrome: A rare cause of cerebral hemiatrophy in children. Journal of pediatric neurosciences. 2016 Jul 1. 11(3):252\u0026ndash;4. doi: 10.4103/1817-1745.193365\u003c/li\u003e\n\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":true,"highlight":"","institution":"","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true},"keywords":"Dyke-Davidoff-Masson Syndrome, Recurrent Seizures, Cerebral Hemiatrophy, Paranasal sinus Hyperpneumatization","lastPublishedDoi":"10.21203/rs.3.rs-4095531/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-4095531/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003ch2\u003eBackground\u003c/h2\u003e \u003cp\u003eDyke-Davidoff-Masson Syndrome (DDMS) is a rare neurological disorder resulting from an insult to the developing brain in utero or during early life which is characterized by refractory seizures and a wide variety of deficits. Due to its rarity, the condition is often overlooked although a detailed history and imaging can help distinguish it from other similar conditions and provide early diagnosis.\u003c/p\u003e\u003ch2\u003eCase presentation:\u003c/h2\u003e \u003cp\u003eA thirty-year-old female presented with a history of recurrent seizures and intellectual disability. Her delivery was through the vaginal route with unremarkable post-natal history. Her developmental milestones were normal until the age of three years when she was hospitalized for two weeks due to a febrile illness and generalized seizures, with full recovery. She earned a score of 14/60 on the Ravens Standard Progressive Matrices (RSPM) indicating an intellectual disability. The neurological examination revealed constructional apraxia, facial nerve upper motor neuron lesion (UMNL). A slow, sinuous writhing movement involving all fingers on the left hand was observed, and left spastic paresis. She underwent a computed tomography (CT) of the head which confirmed DDMS due to presence of right cerebral hemisphere volume loss with gross dilatation of the right lateral ventricle with ex-vacuo dilatation of the right lateral ventricle, right calvarial thickening and hyperpneumatization of the right frontal sinus. She was managed conservatively with anticonvulsants and had her seizure well controlled.\u003c/p\u003e\u003ch2\u003eConclusion\u003c/h2\u003e \u003cp\u003eIn the setting of recurrent seizures, intellectual disability, hemiparesis, or facial asymmetry, a clinician should have a high index of suspicion for DDMS. Late diagnosis is often related to intractable seizures to anticonvulsants, necessitating hemispherectomy, which is not a readily available option in limited resource settings.\u003c/p\u003e","manuscriptTitle":"Dyke-Davidoff-Masson Syndrome in a Tanzanian patient: A case report","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2024-03-21 18:01:25","doi":"10.21203/rs.3.rs-4095531/v1","editorialEvents":[{"type":"communityComments","content":0}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"515a98ce-b709-4ef9-8006-e4fdfd39db04","owner":[],"postedDate":"March 21st, 2024","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2024-06-10T07:26:04+00:00","versionOfRecord":[],"versionCreatedAt":"2024-03-21 18:01:25","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-4095531","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-4095531","identity":"rs-4095531","version":["v1"]},"buildId":"8U1c8b4HqxoKbykW_rLl7","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}