Dykedavidoffmasson syndrome, brain atrophy, computerized tomography, magnetic resonance image. T1weighted mri illustrates hypoplasia and extensive encephalomalacia of the left hemisphere arrowheads on axial a and coronal b images, enlargement of the left frontal sinus and increased pneumatization arrow on axial image a, and hyperplasia of the left mastoid on coronal image arrow b. Dyke davidoff masson syndrome ddms is a rare disease which was first described in 1933. We present here a case of a 37year old man who presented with. Dyke davidoff masson syndrome is a syndrome characterized by cerebral hemiatrophy and a contralateral hemiplegia, hemiatrophy, epilepsy and mental retardation with characteristic skull changes. The clinical features are variable and depend on the extent of brain injury. Mundle, ketan chaturvedi, yogesh dhoble and anand bakre. Pdf cerebral hemiatrophy or dykedavidoffmasson syndrome is a. Abstract cerebral hemiatrophy or dyke davidoff masson. Clinical spectrum of dykedavidoffmasson syndrome in the. This report follows the case of a 22yearold man presenting with seizures and hemiatrophy and hemiparesis. Dyke davidoff masson syndrome ddms is a syndrome associated with refractory epilepsy and was first described by dyke, davidoff and masson in 1933.
Dykedavidoffmasson syndrome ddms is a rare clinical condition characterized by seizures, mental retardation, facial asymmetry, contralateral hemiplegia. Time to revisit case series shubhakaran1, bharat bhushan2 1associate professor, 2resident, department of neurology, m. Dykedavidoffmasson syndrome is a rare condition of unknown frequency resulting from brain injury due to a multitude of causes. Case report dykedavidoff masson syndrome presenting. Dyke davidoff masson syndrome was first described by dyke, davidoff, and masson1 in 1933. Oct 11, 2018 postnatal brain and skull growth in an apert syndrome mouse model. Neuroimaging features are characterized by unilateral cerebral atrophy with ipsilateral hypertrophy of paanr in sauasl ses 2.
British biomedical bulletin case report dyke davidoff masson syndrome a case report. Dyke davidoff masson syndrome ddms is a rare disorder characterized by recurrent seizures, facial asymmetry, contralateral hemiplegia, radiologic features of cerebral hemiatrophy, and ipsilateral compensatory hypertrophy of the skull bone and sinuses. Dykedavidoffmasson syndrome is a constellation of clinical features that consists of hemiparesis, seizure, facial asymmetry, and intellectual disability with distinct neuroimaging features. Cerebral hemiatrophy or dyke davidoffmasson syndrome ddms is a rare clinical picture, characterized by unilateral cerebral atrophy and facial asymmetry, hemiparesis, seizures and mental retardation. Emiatrofia cerebrale di dykedavidoffmasson wikipedia. Case report a 2 year old male child, 1st by birth order and born of. Dyke davidoff masson syndrome ddms is a rare clinical condition characterized by seizures, facial asymmetry, contralateral hemiplegia or hemiparesis, mental retardation, sensorineural hearing loss and behavioral changes. We present here a case of a 22 year old man who presented with altered sensorium and on. Pdf mr and ct imaging in the dykedavidoffmasson syndrome. In 1933, ddms was first described in a case series by dyke, davidoff and mason. Three drugs are commonly used and administered orally. Lemiatrofia cerebrale di dykedavidoffmasson o sindrome di dykedavidoffmasson o dai nomi dei neurologi americani leo max davidoff, cornelius gysbert dyke e c. Two types of ddms have been identified, congenital and acquired.
A proper clinical history and ctmri findings provide the correct diagnosis the treatment is symptomatic, and should target. Patient was controlled on medication for next 4 months but then, unfortunately, lost to followup. Dyke davidoff masson syndrome is an uncommon congenital or childhoodonset acquired neurologic syndrome, presenting mainly with complete hemiparesis, recurrent seizures and nonprogressive chronic encephalopathy1,2. Dykedavidoffmasson syndrome in adulthood neurology. The magnetic resonance mr findings of three patients with cerebral hemiatrophy, the socalled dykedavidoffmasson syndrome, which is characterized by variable degrees of unilateral loss of. Radiologia vol 49, issue 4, pages 221293 july 2007. A 15yearold female presented with seizures, rightsided hemiparesis, hemiatrophy of the right side of the body and mental retardation. Based on characteristic radiological finding patient was diagnosed as a case of dyke davidoff masson syndrome ddms and was treated with valproic acid and levetiracetam. The evaluated parameters were the location of the lesions, midline structural shift effect, pathological and morphological changes on the ipsilateral calvarium, paranasal sinuses and mesencephalon, presence of compensatory contralateral hypertrophy. Dyke davidoff masson syndrome in adulthood a 50year diagnostic delay a 54yearold woman was seen due to weakness and gait difficulty. Dyke davidoff masson syndrome is characterized by unilateral atrophy of cerebral hemispheres, enlargement of the ipsilateral sulci, ventricles, and cisternal spaces, decrease in the size of ipsilateral cranial fossae, overdevelopment of paranasal sinuses and mastoid air cells, and unilateral thickening of the skull.
To classify cerebral hemiatrophy on the basis of childhood febrile seizures and magnetic resonance mr imaging findings of mesial temporal sclerosis. Dykedavidoffmasson syndrome ddms refers to atrophy or hypoplasia of one cerebral hemisphere hemiatrophy, which is usually due to an insult to the. Ddms or hemispheric infarction is an atrophy or hypoplasia. Medical college, jodhpur, rajasthan sir we read a case report by manghera et al japi, vol 62 page no. Dyke davidoff masson pdf dyke davidoff masson syndrome is characterized by unilateral atrophy of cerebral hemispheres, enlargement of the ipsilateral sulci, ventricles, and. Dykedavidoffmasson syndrome ddms is an uncommon condition, in which the diagnosis is mainly done by various clinical presentations along with. It is a rare syndrome characterized by seizures, facial asymmetry, contralateral hemiplegia and mental retardation.
Dyke davidoff masson syndrome ddms refers to atrophy or hypoplasia of one cerebral hemisphere cerebral hemiatrophy which is secondary to brain insult in fetal or early childhood period. Physical examination revealed spasticity ashworth 23, weakness 35, hyperactive deep tendon reflexes on the right side, and facial asymmetry. Cerebral hemiatrophy or dyke davidoff masson syndrome is a condition characterized by seizures, facial asymmetry, contralateral hemiplegia or hemiparesis, and mental retardation. Dyke davidoff masson syndrome is a rare epilepsy syndrome with predominant manifestations of seizures, facial asymmetry, hemiatrophy, contralateral hemiparesis, skull vault thickening and mental retardation. These patients can potentially have complex medical management that requires multidisciplinary team based approach. Dyke davidoff masson syndrome ddms is a rare neurological condition of unknown frequency, with available literature mostly from case reportsseries. Dyke davidoff masson syndrome is an uncommon neurological condition, characterized by epileptic. Dykedavidoffmasson syndrome canan a med j dy patil univ. Jul 11, 2019 balantidiasis tratamiento pdf treatment of balantidiasis. Regalado chico, hector alfredo montenegro rosales, javier valdes garcia, patricia sevilla flores, alfredo lopez parra, ali diaz castillejos, alejandro zavala reina, guy broc haro p 167 ver pdf. We present two cases of this uncommon condition and discuss its imaging features, differential. However first manifestation at the age of 28 years or more has not been reported so far in literature.
Dykedavidoffmasson syndrome ddms is a rare, but important cause of drugresistant seizures. Dykedavidoffmasson syndrome ddms is a condition characterized by hemicerebral atrophyhypoplasia secondary to brain insult usually in fetal or early childhood period and is accompanied by ipsilateral compensatory osseous hypertrophy and contralateral hemiparesis it is characterized by. It refers to the characteristic holohemispheric gl. Balantidium coli infection can be treated effectively with antibiotics. Classical oral manifestations of dykedavidoffmasson. The magnetic resonance mr findings of three patients with cerebral hemiatrophy, the socalled dyke davidoff masson syndrome, which is characterized by variable degrees of unilateral loss of. Radiological findings include cerebral atrophy on one side. Enviar correspondencia, observaciones y sugerencias a gaxiola higuera al telefono 667228 20 78. Dyke davidoff masson syndrome ddms is a rare neurological condition of unknown frequency, with available literature mostly from case reportsseries 1,2,3. Dyke davidoff masson syndrome in type1 diabetes mellitus. Pant hospital and associated maulana azad medical college, new delhi, india.
They described the plain skull radiographic and pneumatoencephalographic changes in a series of nine patients characterized clinically by. Masson e una condizione caratterizzata da problemi di lateralizzazione, asimmetria faciale, emiplegia controlaterale, difficolta allapprendimento e ritardo mentale. Dyke davidoff masson syndrome ddms is a rare neurological disorder characterized by cerebral asymmetry secondary to unilateral brain atrophy, ipsilateral ventricular dilatation, convulsions, mental retardation, learning disabilities and behavioural changes. Dyke davidoff masson syndrome ddms refers to atrophy or hypoplasia of one cerebral hemisphere hemiatrophy which is secondary to brain insult in fetal or early childhood period. Dykedavidoffmasson syndrome in a nigerian sciencedirect. Baba farid university of health sciences, faridkot, saudi arabia.
Case report current pediatric research 2019 volume 23, issue 4. Hemiconvulsionhemiplegia epilepsy syndrome hhe is a clinical syndrome of infancy or early childhood that is associated with seizures, cerebral hemiatrophy and transient or permanent epilepsy. Ddms usually occurs in fetal life or early childhood due to brain damage. A 34 year old male patient was admitted with complaints of the seizure episode in spite of being on anti epileptics. A rare case report find, read and cite all the research you need on. Radiological findings in dykedavidoffmason syndrome. The purpose of the journal of the belgian society of radiology is the publication of articles dealing with diagnostic and interventional radiology, related imaging techniques, allied sciences, and continuing education.
Imagine findings briefly show left cerebral atrophy, ipsilateral calvarian thickening and hyperpneumatisation of the left frontal sinus. Mri brain revealed characteristic features diagnostic of congenital type of cerebral hemiatrophy or dyke davidoff masson syndrome. Its a very rare condition presenting in childhood ranging from infancy to late adolescence 2. Dyke davidoff masson syndrome ddms refers to atrophy or hypoplasia of one cerebral hemisphere hemiatrophy, which is usually due to an insult to the developing brain in fetal or early childhood. May 10, 2015 as we consider dyke davidoff masson syndrome, as seizure was present while facial asymmetry and contralateral hemiparesis was absent in our patient. Most affected patients are among the pediatric population. Arora a, kapoor a, upreti l, singh s, puri sk department of radiodiagnosis, g. Dyke davidoff mason syndrome ddms, rasmussen syndrome and sturgwebers syndrome. Ct scan brain showed left cerebral hemiatrophy suggestive of dyke davidoffmasson syndrome ddms. Dykedavidoffmasson syndrome congenital defects jama. Dyke davidoff masson syndrome is a rare neurological disorder that can manifest with cerebral hemiatrophy, ventricular dilatation, seizures, hemiparesis, behavioural. Dyke davidoff masson syndrome ddms, also called cerebral hemiatrophy, is a clinical condition characterized by hypoplasia or atrophy of one of the cerebral hemispheres during the fetal period or. Due to its rarity, it may be misdiagnosed or underreported by the majority of clinicians.
The characteristic radiologic features are cerebral hemiatrophy with compensatory homolateral hypertrophy of the skull and sinuses. These findings are due to cerebral injury that may occur early in life or in utero. Dykedavidoffmasson syndrome is a noninherited rare condition that presents during childhood and is characterized by seizures, hemiplegia, mental retardation, cerebral hemiatrophy, calvarial. Pdf on oct 6, 2014, deepak jain and others published dykedavidoffmasson syndrome. Dykedavidoffmasson syndrome revisited ps manghera1, bharat bhushan sharma2, virendra singh 3 1postgraduate student, 2associate professor, 3professor, department of medicine, sms medical college, jaipur sir, we read the article by ola et al on dyke davidoffmasson syndrome published in january 2014 issue of the. Dyke davidoff masson syndrome is a relatively rare syndrome with its typical clinical and radiological features including facial asymmetry, hemiplegia, cerebral hemiatrophy, mental retardation with calvarial thickening, hypertrophy of sinuses and elevated petrous ridge on imaging. Hemiconvulsionhemiplegia epilepsy syndrome radiology. Dyke davidoff masson syndrome was described as skull radiographic and pneumatoencephalographic changes in their series of 9 patients whose clinical characteristics included hemiparesis, seizures, facialasymmetry, and. It is classified on basis of the etiological factors. The congenital type involves utero vascular occlusion of the middle cerebral. We reported five cases of dykedavidoffmasson syndrome ddms with different clinical and radiological findings. Dyke davidhoff masson syndrome is a very rare condition presentingin infancy or early chil dhood generally.
Dyke davidhoff masson syndrome ddms was first described by dyke davidhoff and masson in 1933 in a series of 9 patients 1. Sindrome dykedavidoffmasson by mariela soto on prezi. It is characterised by cerebral hemiatrophy, unilateral ventriculomegaly, calvarial thickening, skull and facial asymmetry, sometimes even contralateral hemiplegia in. Cerebral hemiatrophy is not frequently encountered in clinical practice. Some cases with late presentation up to the age of 19 years have been reported 3. In their original work, dyke, davidoff and masson described the radiological skull findings in 9 patients with infantile hemiplegia. Dyke davidoff masson syndrome should be differentiated from basal cell germinoma, sturge weber syndrome, linear nevus syndrome, fishman syndrome, silver russell syndrome and rasmussen encephalitis 8. The clinical features are variable and depend on the extent of brain injury in fetal or early childhood period.
The plain skull radiographic and pneumatoencephalographic changes in nine patients with ddms were reported by dyke et al. Dyke davidoff masson syndrome ddms is a syndrome associated with refractory epilepsy and was first described by dyke et al. The dyke davidoff masson syndrome results from an insult to the growing brain in utero or early infancy. Mr and ct imaging in the dykedavidoffmasson syndrome. Dyke davidhoff masson syndrome presenting in adulthood. Autoplay when autoplay is enabled, a suggested video will automatically play next.
There was no need to recommend a magnetic resonance scanning. Dykedavidoffmasson syndrome is characterized by unilateral atrophy of cerebral hemispheres, enlargement of the ipsilateral sulci, ventricles, and cisternal spaces, decrease in the size of ipsilateral cranial fossae, overdevelopment of paranasal sinuses and mastoid air cells, and unilateral thickening of the skull. Dyke davidoff masson syndrome ddms is a condition characterized by hemicerebral atrophyhypoplasia secondary to brain insult usually in fetal or early childhood period and is accompanied by ipsilateral compensatory osseous hypertrophy and contr. Dyke davidoff masson syndrome is a rare entity characterized by hemi cerebral atrophyhypoplasia secondary to brain insult in fetal or early childhood period along with ipsilateral compensatory osseous hypertrophy and contralateral hemiparesis. Dyke davidoff masson syndrome ddms in 1933 dyke, davidoff and masson described, on radiographs of the skull and pneumoencephalographs, changes in a series of nine patients characterized clinically by hemiparesis hemiplegia, seizures, facial asymmetry and mental retardation 8. Due to its rarity, it may be misdiagnosed or underreported by the. Seema rai, harshvardhan gupta, gurmeet k sethi, savneet kaur. Dyke davidoff masson syndrome ddms is a rare condition usually diagnosed in paediatric patients with clinical features of hemiparesis, seizures, mental retardation and contralateral cerebral hemiatrophy on neuroimaging.
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