Dyke Davidoff Masson syndrome

Dyke Davidoff
Mason syndrome. Left cerebral hemiatrophy with exvacuuo dilatation of left lateral ventricle.
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Radiological
findings in Dyke-Davidoff-Mason Syndrome (DDMS). CT brain bone window, the left frontal sinus hyper-pneumatisation is better illustrated.
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Radiological
findings in Dyke-Davidoff-Mason Syndrome (DDMS). CT brain, demonstrating severe left cerebral hemiatrophy with ipsilateral compensatory skull hypertrophy, enlarged frontal sinus consistent with DDMS. There are other findings of bilateral cerebellar atrophy.
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Radiological
findings in Dyke-Davidoff-Mason Syndrome (DDMS). CT brain, demonstrating severe left cerebral hemiatrophy with ipsilateral compensatory skull hypertrophy, enlarged frontal sinus consistent with DDMS. There are other findings of bilateral cerebellar atrophy.
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Dyke-Davidoff-Masson
syndrome • Dyke-Davidoff-Masson syndrome with mesial temporal sclerosis - Ganzer Fall bei Radiopaedia
Kabra U, Dyke-Davidoff-Masson syndrome with mesial temporal sclerosis. Case study, Radiopaedia.org (Accessed on 17 Nov 2022) https://doi.org/10.53347/rID-73919 • CC by-nc-sa 3.0 (modified)
Dyke Davidoff
Mason syndrome. Left cerebral hemiatrophy with calvarial thickening (white arrows). Compensatory contralateral cerebral hypertrophy and midline shift towards left side.
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Dyke-Davidoff-Masson
syndrome: case report of fetal unilateral ventriculomegaly and hypoplastic left middle cerebral artery. Two-month-old patient. Contrast enhanced-MRI-angiography. Three-dimensional multiplanar reconstruction image shows normal patency of the carotid arteries and hypoplasia of the left middle cerebral artery (white arrow).
ijponline.biomedcentral.com • CC-by-2.0
Dyke-Davidoff-Masson
syndrome: case report of fetal unilateral ventriculomegaly and hypoplastic left middle cerebral artery. Two-month-old patient. Brain MRI coronal FLAIR image shows atrophy of the entire left hemisphere with compensatory ipsilateral midline structure shifting.
ijponline.biomedcentral.com • CC-by-2.0
Dyke-Davidoff-Masson
syndrome: a case report. 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
bmcneurol.biomedcentral.com • CC-by-4.0
Dyke Davidoff
Mason syndrome. Left cerebral hemiatrophy, dilated ipsilateral occipital horn, ipsilateral midline shift and ipsilateral pneumosinus dilatans (frontal)
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Dyke Davidoff
Mason syndrome. Enlarged hyperpneumatized left frontal sinus (white arrow), exvacuuo dilatation of left frontal horn (yellow arrow) and widened diploic space (small white arrows).
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Dyke Davidoff
Mason syndrome. Elevation of the left petrous ridge along with larger ipsilateral mastoid air cells.
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Radiological
findings in Dyke-Davidoff-Mason Syndrome (DDMS). There is hyper-pneumatisation of the left frontal sinus, elevated left petrous ridge, left skull thickening and falx displaced to the left.
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Radiopaedia • CC-by-nc-sa 3.0 • de

Dyke-Davidoff-Masson syndrome is a condition characterized by hemicerebral atrophy/hypoplasia 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:

  • thickening of the skull vault (compensatory)
  • enlargement of the frontal sinus (also ethmoidal and mastoid air-cells)
  • elevation of the petrous ridge
  • ipsilateral falcine displacement
  • capillary malformations (are a novel finding for children with Dyke-Davidoff-Masson syndrome) 

In some sources, it is equated to hemispheric infarction, whereas in other sources any cause of cerebral hemiatrophy is included.

Clinical presentation

  • seizures
  • facial asymmetry
  • contralateral hemiparesis
  • intellectual disability

Some authors divide the condition into two types mainly dependent on clinical presentation age

  • infantile (congenital): 
    • results from various etiologies such as infection, neonatal or gestational vascular occlusion involving the middle cerebral artery, unilateral cerebral arterial circulation anomalies, and coarctation of the mid aortic arch.
    • patient becomes symptomatic in the perinatal period or infancy.
  • acquired
    • main causes of acquired type are trauma, tumor, infection, ischemia, hemorrhage, and prolonged febrile seizures

Radiographic features

Imaging spectrum includes varying degrees of cerebral hemiatrophy of the affected hemisphere (with dilatation of the ipsilateral lateral ventricle and ipsilateral sulcal prominence) accompanied by homolateral hypertrophy of the skull and sinuses. Elevation of the petrous ridge and ipsilateral falcine displacement may also be present.

The spectrum of findings include

  • Wallerian degeneration of the mesencephalon and middle fossa hypoplasia
  • atrophy in basal ganglia
  • atrophy in the brain stem
  • capillary malformations: may be detected in some situations
  • calvarial thickening (affected side)
  • hyperpneumatisation of mastoid cells (affected side)

History and etymology

It was initially described as changes in the skull seen on skull x-ray in patients with cerebral hemiatrophy but is now applied more broadly to cross-sectional imaging. It was initially described by C G Dyke, L M Davidoff and C B Masson in 1933 .

Differential diagnosis

General imaging differential considerations include:

Siehe auch:
Assoziationen und Differentialdiagnosen zu Dyke-Davidoff-Masson syndrome:
Sturge-Weber-Krabbe-Syndrom
 
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Hemimegalencephalie
 
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einseitige
Hirnatrophie
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Rasmussen-Enzephalitis
 
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