Answer for BIR CoW 25 Oct 2020
Dyke- davidoff masson syndrome
Findings
Hemicerebral atrophy on right side with ex vacuo dilatation of right lateral ventricle noted. Right frontal sinus, sphenoid sinus and right mastoid air cells are relatively enlarged. Relative right frontal and right fronto parieto temperal calvarial thickening noted. Mr angiogram shows stenosis of c3 segment of right internal carotid artery
Discussion
Dyke-davidoff-masson syndrome is a condition characterised 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 characterised 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) clinical presentation • seizures • facial asymmetry • contralateral hemiparesis • intellectual disability
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)
Differential considerations include: • hemimegalencephaly • sturge-weber syndrome: o can also be an association o almost always have facial port-wine stain (cutaneous naevus) in the territory of ophthalmic division (v1) of the trigeminal nerve • rasmussen encephalitis: tends not to have calvarial changes
Findings
Hemicerebral atrophy on right side with ex vacuo dilatation of right lateral ventricle noted. Right frontal sinus, sphenoid sinus and right mastoid air cells are relatively enlarged. Relative right frontal and right fronto parieto temperal calvarial thickening noted. Mr angiogram shows stenosis of c3 segment of right internal carotid artery
Discussion
Dyke-davidoff-masson syndrome is a condition characterised 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 characterised 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) clinical presentation • seizures • facial asymmetry • contralateral hemiparesis • intellectual disability
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)
Differential considerations include: • hemimegalencephaly • sturge-weber syndrome: o can also be an association o almost always have facial port-wine stain (cutaneous naevus) in the territory of ophthalmic division (v1) of the trigeminal nerve • rasmussen encephalitis: tends not to have calvarial changes
Note:
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!