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Dyke-Davidoff-Masson syndrome--typical imaging features/Dajk-davidov-masonov sindrom--tipicne radioloske karakteristike sindroma.

UDK 616.831-007.23:537.635


Dyke-Davidoff-Masson syndrome (DDMS) is a rare neurologic condition clinically characterized by a different degree of mental retardation, facial or body asymmetry, hemiparesis or hemiplegia and epileptic seizures [1-3]. The syndrome was first reported in 1933. Using plain skull radiography and pneumoencephalography in the series of nine patients with the aforementioned symptoms, Dyke, Davidoff and Masson reported thickening of skull bones and asymmetry of lateral ventricles [3]. The development of more sophisticated diagnostic imaging modalities, such as computed tomography and, especially magnetic resonance imaging led to a more detailed evaluation of this condition. Classical imaging findings include hypoplasia of one brain hemisphere (hemiatrophy), often with reduced volume of a corresponding cranial fossa, and consecutive thickening of nearby bony structures and enlargement of equilateral paranasal sinuses, the frontal sinus being most often involved [1-3]. In more severe cases, hyperpneumatization of equilateral mastoid cells and elevation of a petrous ridge and orbital roof can be seen [3]. The purpose of this report was to present a rare cause of seizures revealed by magnetic resonance imaging (MRI).

Case Report

A 17-year-old boy was admitted to neurology department due to a sudden onset of severe frontal headache. Since the age of 11 he had been treated because of partial seizures manifested as short episodes of right arm tonic seizures, frequently accompanied with a stutter. He has had episodes of stutter ever since he was a little child. The seizures had not recurred since the introduction of the medications and the treatment ended two years ago. The parents gave information that the delivery had happened outside of a hospital and that the child had been resuscitated at birth.

Neuropsychological assessment revealed mild mental retardation, mild dyslexia and dyscalculia, as well as discrete right-sided hemiparesis. MRI was ordered.

Magnetic resonance imaging examination revealed a volume loss of the left frontal lobe with a gliosis of subcortical and deep frontal white matter and consequent widening of the extracerebral cerebrospinal fluid (CSF) spaces and the frontal horn of the left lateral ventricle (Figure 1A). Thickening of the squama of the frontal bone was evident as well as hyper-pneumatization of the left frontal sinus (Figures IB and 1C). Mild enlargement of the left mastoid cells and discrete elevation of the left petrous ridge were also present (Figures 2A and 2B). The decreased volume of left anterior cranial fossa, with a shift of midline structures toward the left was evident (Figure 1).


After the first report of the syndrome given by Dyke, Davidoff and Masson in 1933, Alpers and Dear defined two types of cerebral hemiatrophy in 1939, according to the probable etiology and the time of the insult [4]. In the primary or congenital type, an entire cerebral hemisphere is atrophic or hypoplastic while in the secondary or acquired type there is a localized volume loss of the brain parenchyma [1, 5]. In the primary type, a potential insult occurs in an early pregnancy, causing a development of an entire brain hemisphere to slow down. There is no brain volume loss, but the brain never develops to the normal size, therefore this condition is known as hemi-hypoplasia or unilateral cerebral hypoplasia [6, 7]. The vast majority of the primary type is of an unknown origin (idiopathic) and the cerebrovascular origin in the early pregnancy is thought to be the most probable cause of the disorder.

In the secondary type, cerebral insults occur in the perinatal period or later and a possible cause could be either cerebrovascular insult, both ischemic or hemorrhagic, inflammatory, infectious process or head trauma [6]. Any of these factors can cause a delay in the development of the brain, most probably due to reduced perfusion and hypoxic-ischemic injury [7]. Recent fluorodeoxyglucose-positron emission tomography (FDG-PET) studies proved hypometabolism of the affected hemisphere [8, 9], whereas other studies have suggested that a reduced blood flow through carotid artery due to co-arctation could be the cause of cerebral hemiatrophy [9, 10].

As the brain increases its volume during the development, it pushes the bony tables outwards and causes them to laminate. In case of retarded brain growth the bones and paranasal sinuses tend to invert its growth inwards, to enlarge and thus occupy the free intracranial space [6, 7, 11]. Therefore, the frontal sinus, ethmoid and mastoid cells equilateral to brain hemiatrophy get larger, the squama of the frontal bone becomes thicker and large sphenoid wing and petrous ridge elevate. The lateral ventricle and extracerebral CSF spaces also get larger ex vacuo due to the brain volume loss. Both genders and hemispheres may be affected, although there is a propensity for male and left hemisphere [1]. The frontal lobes are most commonly affected. Cross cerebellar atrophy can be seen in patients with long-standing and extensive lesions, usually in the primary type of DDMS [1].

Conditions associated with cerebral hemiatrophy, such as Sturge-Weber syndrome, Rasmussen encephalitis, Linear nevus syndrome, Fishman syndrome, Silver-Russell syndrome and unilateral complete occlusion of the middle cerebral artery (MCA) could mimic DDMS on imaging. The clinical manifestation of this disorder may become evident at any time, depending on the time the insult occurred, and its severity. It most frequently becomes evident in the teenage or adolescent period. The clinical presentation may also be variable. A person most frequently experiences simple or complex partial seizures, varying degree of hemiparesis, cognitive disorder, language and learning disorders, depending upon the severity of the brain damage [1, 7, 12, 13].


Magnetic resonance imaging is the key imaging modality that confirms clinical suspicion of Duke-Davidoff-Masson syndrome based on a proper physical and neurological examination.

Prognosis is better if the onset of symptoms is after the age of 2 years with medically controllable seizures. In the case of a pharmacoresistant epilepsy, a hemispherectomy is a treatment of choice with a success rate of 85% in carefully selected cases.

DDMS        --Dyke-Davidoff-Masson syndrome
MRI         --magnetic resonance imaging
CSF         --cerebrospinal fluid
T2W FLAIR   --T2-weighted fluid attenuated inversion recovery
T1W         --T1-weighted
T2W         --T2-weighted

DOI: 10.22 98/MPNS1612373B


[1.] 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. Arq Neuropsiquiatr. 1998; 56(4):803-7.

[2.] Narain NP, Kumar R, Narain B. Dyke-Davidoff-Masson syndrome. Indian Pediatr. 2008; 45(11):927-8.

[3.] Dyke CG, Davidoff LM, Masson CB. Cerebral hemiatrophy with homolateral hypertrophy of the skull and sinuses. Surg Gynecol Obstet. 1933; 57:588-600.

[4.] Alpers BJ, Dear RB. Hemiatrophy of the brain. J Nerv Ment Dis. 1939; 89:653-71.

[5.] Parker JC Jr, Gaede JT. Occurrence of vascular anomalies in unilateral cerebral hypoplasia. "Cerebral hemiatrophy". Arch Pathol. 1970; 90(3):265-70.

[6.] Sharma S, Goyal D, Negi A, Sood RG, Jhobta A, Surya M. Dyke-Davidoff-Masson syndrome. Indian J Radiol Imaging. 2006; 16:165-6.

[7.] Jain D, Aggarwal HK, Goyal S, Mittal A. Dyke-Davidoff-Masson syndrome: a rare case report. Iran J Neurol. 2014; 13(4):255-6.

[8.] Basu S, Chouhan A. Striking asymmetry in cerebral metabolism in Dyke-Davidoff-Masson (DDM) syndrome: FDG-PET and MRI correlation. Acta Neurochir (Wien). 2013; 155(3):519-21.

[9.] Shrestha B. Acquired cerebral hemiatrophy: Dyke-Davidoff-Masson syndrome: a case report. Turk Neurosurg. 2013; 23(1):117-21.

[10.] Unal O, Tombul T, Cirak B, Anlar O, Incesu L, Kayan M. Left hemisphere and male sex dominance of cerebral hemiatrophy (Dyke-Davidoff-Masson Syndrome). Clin Imaging. 2004; 28(3):163-5.

[11.] Malik P, Garg R, Gulia AK, Kario J. Dyke-Davidoff-Masson syndrome: a rare cause of refractory epilepsy. Iran J Psychiatry. 2014; 9(1):42-4.

[12.] Koshy B, Surendrababu NR. Image in medicine. Dyke-Davidoff-Masson syndrome. Ann Acad Med Singapore. 2010; 39(6):501-2.

[13.] Behera MR, Patnaik S, Mohanty AK. Dyke-Davidoff-Masson syndrome. J Neurosci Rural Pract. 2012; 3(3):411-3.

Rad je primljen 25. VIII2015.

Recenziran 2. VI 2016.

Prihvacenza stampu 15. VI 2016.

BIBLID.0025-8105:(2016):LXIX: 11-12:373-375.

Mladen BJELAN (1, 2), Dusko KOZIC (1, 2), Vesna NJAGULJ (1, 2), Aleksandar RAGAJI (1), Katarina KOPRIVSEK (1,2) and Milos LUCIC (1, 2)

Institute for Oncology of Vojvodina, Sremska Kamenica

Diagnostic Imaging Center (1)

University of Novi Sad, Faculty of Medicine, Novi Sad, Serbia (2)

Corresponding Author: Dr Mladen Bjelan, Institut za onkologiju, Centar za imidzing dijagnostiku, 21204 Sremska Kamenica, Put Dr. Goldmana 4, E-mail:

Caption: Figure 1. T2W FLAIR image in the axial plane (A) showing atrophy of the left frontal lobe with gliosis of sub-cortical and deep frontal white matter as well as "ex vacuo" widening of the extracerebral CSF spaces and frontal horn of the left lateral ventricle. Thickening of the squama of the frontal bone is evident on the T1W axial image (arrows in B) and shift of midline structures toward the left. T1W image in the axial plane (C) shows hyper-pneumatization of the left frontal sinus. Decreased volume of the left anterior cranial fossa is visible on all three images. Slika 1. T2W FLAIR snimak u trcmsverzalnoj ravni (A) pokazuje atrofiju levogfrontalnog lobusa sa gliozom supkortiklane i duboke bele mase, kao i "ex vacuo" prosirenjem ekstracerebralnih likvorskih prostora i frontalnog roga leve lateralne mozdane komore. Na T1W snimku u transverzalnoj ravni uocava se zadebljanje skvame frontalne kosti (strelica na slid B), kao i pomeranje struktura sredisnje linije iilevo. T1W snimak u transverzalnoj ravni (C) pokazuje hiperpneumatizaciju levogfrontalnog sinusa. Smanjen volumen levog dela prednje lobanjske jame uocava se na sva tri snimka.

Caption: Figure 2. T2W images in coronal plane (A and B) showing mild enlargement of the left mastoid cells and discrete elevation of the left petrous ridge (arrows in A). Slika 2. T2W snimak u transverzalnoj ravni (A i B) pokazuje blago uvecanje mastoidnih celija s leve strane, kao i diskretnu elevaciju levog petroznog grebena (strelica na Slici A).
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Author:Bjelan, Mladen; Kozic, Dusko; Njagulj, Vesna; Ragaji, Aleksandar; Koprivsek, Katarina; Lucic, Milos
Publication:Medicinski Pregled
Article Type:Report
Date:Nov 1, 2016
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