CASE 17942 Published on 29.11.2022

Dyke-Davidoff-Masson Syndrome [Cerebral Hemiatrophy]

Section

Head & neck imaging

Case Type

Clinical Cases

Authors

Ranjith Mohan, Shivadarshan Manjunatha, MR Shashikumar

1. Dept of Radiodiagnosis, Adichunchanagiri Institute of Medical Sciences, B G Nagar, Mandya, Karnataka, India

Patient

20 years, male

Categories
Area of Interest CNS, Neuroradiology brain ; Imaging Technique MR
Clinical History

Here is a 20-year-old male presented to the emergency department with status epilepticus which was initially managed by anticonvulsants and then the patient was sedated with injectable anaesthetics and intubated when the seizures persisted. The patient is a previously known case of generalized epilepsy since childhood on pharmacotherapy. But poor compliance led to the current presentation. No history of mental retardation. Clinical examination revealed no focal neurological deficit or facial asymmetry. Deep tendon reflexes were brisk. Laboratory tests revealed normal counts and serum electrolytes were within normal limits. The patient was advised MRI brain for further evaluation.

Imaging Findings

MRI brain study shows right cerebral atrophy (Fig1) with areas of gliotic and encephalomalacic changes predominantly in right anterior frontal lobe, parieto- occipital and posterior temporal lobes with exvacuo-dilatation of posterior and occipital horn of right lateral ventricle. Compensatory thickening of skull vault noted on right side (Fig 1 & 4). Enlargement of right frontal and hemisphenoid sinuses. There is elevation of right petrous ridge (Fig 4) with hyperpneumatisation of right mastoid air cells and petrous part of right temporal bone. Right hippocampal and parahippocampal regions show mild atrophy (Fig 3). No acute infarct is seen on diffusion sequences. No focal mass lesion.

Discussion

Dyke-Davidoff-Masson syndrome [DDMS], also known as cerebral hemiatrophy, is a neurological disorder which typically affects children and young adults with unknown frequency. Dyke, Davidoff, and Masson were the first to define this syndrome in 1933 [1]. The disease is categorized into 2 types based on its etiology: congenital and acquired. Congenital form is due to obstruction of fetal vascularization. The acquired form manifests in childhood and precipitating factors are perinatal hypoxia, infections, cerebrovascular abnormalities, and cranial trauma [2].

Patients typically present with contralateral hemiplegia or hemiparesis. Seizures, facial asymmetry, and mental retardation are common.

General imaging features include diffuse volume loss with encephalomalacia and gliosis on the affected hemisphere. Left-sided hemiatrophy is more common [70%] than right-sided hemiatrophy [3].

NECT scans show an atrophic hemisphere with enlarged sulci and exvacuodilatation of the ipsilateral ventricle. Ipsilateral falcine displacement may also be present [33-70]. Calvarial thickening, elevation of the sphenoid wing and petrous temporal bone, and expanded sinuses and mastoids are other features.

On MRI, T1WI shows hemispheric volume loss with prominent sulci and cisterns. T2/FLAIR scans demonstrate encephalomalacia with shrunken hyperintense gyri and subcortical WM [4]. Atrophy in basal ganglia, contralateral cerebellum and brain stem may be seen secondary to diaschisis. DDMS neither enhances on T1 C+ nor demonstrates restricted diffusion.

In uncontrolled seizures, hemispherectomy is the treatment of choice. If seizures are under control,

patients are advised anticonvulsant therapy and strict adherence to it is required to prevent further episodes of seizures. Physiotherapy, occupational, and linguistic therapy are advised in long term.

In our case, the refractory seizures were managed by various intravenous anticonvulsants. Mechanical ventilation was continued for the next 4 days, then weaned off and extubated. Patient was prescribed oral anticonvulsant on discharge and regular follow-up.

Due to the rarity of the syndrome, misdiagnosis and mismanagement is quite common. Hence imaging plays an important role in the diagnosis. In children with hemiparesis, facial asymmetry, or seizure disorder, DDMS should always be considered as one of the differentials.

Differential Diagnosis List
Dyke-Davidoff-Masson Syndrome
Sturge-Weber syndrome [SWS]
Rasmussen encephalitis
Haberland syndrome
Hemimegalencephaly
Large territorial MCA infarcts
Final Diagnosis
Dyke-Davidoff-Masson Syndrome
Case information
URL: https://www.eurorad.org/case/17942
DOI: 10.35100/eurorad/case.17942
ISSN: 1563-4086
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