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Dyke–Davidoff–Masson Syndrome: An Underdiagnosed Cause of Cerebral Hemiatrophy IN Children

Pintu Biswas1*, Janame Jeyan2,Sribas Das3and Akash Shikar Debbarma4

1senior Resident, 2 & 4post Graduate Student, 3associate Professor
1, 2 & 4 Department of Radiodiagnosis, Agartala Government Medical College, Agartala, West Tripura, India
3department of Paediatrics, Agartala Government Medical College, Agartala, West Tripura, India

*Corresponding author

*Pintu Biswas, Senior Resident, Department of Radiodiagnosis, Agartala Government Medical College, Agartala, West Tripura, India

Abstract

Dyke–Davidoff–Masson syndrome (DDMS) is a rare condition characterized by unilateral cerebral hemiatrophy or hypoplasia due to brain injury during fetal or early childhood. Key radiological features include cerebral hemiatrophy, thickening of the calvarium, hyperpneumatization of the paranasal sinuses, and elevation of the petrous ridge. A 12-year-old male presented to our institute with seizures, mental retardation, and left-sided hemiparesis. Imaging revealed right cerebral hemisphere atrophy, ex-vacuo dilatation of the ipsilateral lateral ventricle and hyperpneumatization of the paranasal sinuses, consistent with DDMS.

Key words: Dyke–Davidoff–Masson syndrome (DDMS), Magnetic resonance imaging (MRI), Computed tomography (CT), hyperpneumatization, hemiplegia, cerebral hemiatrophy

Introduction

Dyke–Davidoff–Masson Syndrome (DDMS) is a rare condition. In a similar case reported in recent past stated that, less than 100 cases had been reported worldwide till then [1]. It is characterized by hemiparesis, seizures, mental retardation, and facial asymmetry. It results from brain injury during fetal or early childhood, leading to cerebral hemiatrophy. DDMS can be congenital or acquired, with a higher prevalence in males and left-hemisphere involvement [2]. Clinical manifestations vary but typically include intellectual disabilities and motor deficits [3].Imaging reveals cerebral hemiatrophy, ipsilateral ventriculomegaly, skull hypertrophy, and enlarged sulcal spaces. Diagnosis is usually made in childhood, though it can be delayed [4]. A case of a 12-year-old male with seizures and progressive left-sided hemiparesis is presented, diagnosed via MRI and CT, highlighting DDMS’s clinical and radiographic features

Case Report

A 12-year-old male was brought in with worsening mental status and a history of left focal seizures with secondary generalization over the past 3years, without any history of head trauma. The seizures were followed by reduced movement on the left side and occasional drooling. Perinatal and birth histories were unremarkable. The patient was not attending school and showed signs of undernutrition and significant cognitive impairment. Physical examination revealed microcephaly, intellectual disability, and left-sided facial palsy. Neurological examination showed left hemiparesis, while other system evaluations were normal. Laboratory workups include baseline investigations were within normal limits. After admission, he was advised CT & MRI of brain.

Non-contrast computed tomography (Fig-1A & 1B) and magnetic resonance imaging (Fig-2A & 2B) of brain reveled hemiatrophy of right cerebral hemisphere, right half of midbrain and right cerebellar hemisphere with cystic encephalomalacia and gliosis involving right parieto-occipital region with prominence of sulci, dilation of occipital and temporal horns of ipsilateral lateral ventricle. Enlarged right frontal & sphenid sinus, elevated petrous ridge & ipsilateral flax cerebri shift were noted.

Based on the CT & MRI findings patient was diagnosed as a case of DDMS.

REPRESENTATIVE IMAGES OF THIS CASE:

Figure 1- A & B: A: Computed tomography (bone window) of the brain showing thickened right calvarium (green arrow), hyper pneumatized & enlarged right frontal sinus (purple arrow). B. Axial Computed tomography (brain window) showing diffuse atrophy of the right cerebral hemisphere with ex-vacuo dilatation of the right lateral ventricle, ipsilateral flax cerebri shift (orange arrow) and prominence of sulci over the right cerebral hemisphere(sky-blue arrow).

Figure 2 A & B: A: Brain MRI in coronal T2 section showing right cortical atrophy (orange arrow), dilated ipsilateral lateral ventricle (sky-blue arrow), calvarial thickening (red arrow) & elevated right petrous apex (green arrow). B. Brain MRI Axial T2 weighted section showing hyper pneumatized right frontal sinus (sky-blue arrow), atrophy involving right half of midbrain (orange arrow) and right cerebellar hemisphere (green arrow)

Discussion

Dyke-Davidoff-Masson Syndrome (DDMS) involves unilateral cerebral hemiatrophy due to prenatal or early-life brain damage, leading to compensatory changes in the calvarium. The condition can result from various causes, including prenatal cerebral infarctions, birth trauma, [5] or infections. Clinical features include hemiparesis, seizures, mental retardation, and facial asymmetry. Rarely, patients may also present with psychiatric disorders like schizophrenia.

First described in 1933 [6], DDMS is diagnosed through characteristic radiological findings such as cerebral hemiatrophy, enlarged ventricles, thickened calvarium, and sinus enlargement, visible in CT or MRI scans.

Differential diagnoses for DDMS include Sturge-Weber syndrome, Rasmussen encephalitis, Fishman syndrome, and Silver-Russell syndrome. These conditions lack the characteristic sinus hyperpneumatization and bone hypertrophy seen in DDMS, with features like facial malformations, cortical atrophy, cranial lipomas, and growth retardation. [7]

The syndrome can be congenital, occurring when the brain insult happens in utero, or acquired, due to factors like trauma or infections during early childhood. In congenital cases, sulcal prominence is absent, while acquired cases show prominent sulci due to post-birth insults. [8]

Management focuses on controlling seizures with anticonvulsants, along with physiotherapy, occupational, and speech therapies. In severe cases, hemispherectomy may be indicated, which has a high success rate. Early diagnosis is crucial for better outcomes, especially in cases with recurrent seizures or early-onset hemiparesis.

Radiologists, neurologists, and paediatricians must be aware of DDMS to ensure prompt diagnosis and treatment, as early intervention can significantly impact the patient's quality of life and developmental progress [9].

Conclusion

DDMS is a rare condition often misdiagnosed by inexperienced clinicians. Early diagnosis is crucial for proper mental and physical development, and advanced imaging, especially MRI, is key to accurate detection. Increased awareness is necessary to avoid misdiagnosis.

The condition can be mistaken for an ischemic lesion in patients with motor deficits. DDMS should be considered when imaging shows cerebral hemiatrophy and bone hypertrophy. Prognosis improves when hemiparesis occurs after age two and without recurrent seizures.

REFERENCES

  1. Djimdé SO, Yalcouyé A, Koïta A (2022) An unusual case of Dyke–Davidoff– Masson syndrome revealed by status epilepticus in a Malian patient. Clin Case Rep 10: e06428.
  2. Ayele BA, Zewde YZ (2019) Dyke–Davidoff–Masson syndrome-a rare cause of cerebral Hemiatrophy in a 17-years-old Ethiopian patient: a case report. Ethiopian journal of health sciences 29(2).
  3. Park KI, Chung JM, Kim JY (2014) Dyke-davidoff-masson syndrome: cases of two brothers and literature review. Journal of epilepsy research. 4(1):24
  4. Behera MR, Patnaik S, Mohanty AK (2012) Dyke-Davidoff-Masson syndrome. Journal of Neurosciences in Rural Practice. 3(3):411
  5. Goyal J, Shah V, Rao S, Jindal N (2009) Dyke Davidoff Masson syndrome in children. The Internet Journal of Pediatrics and Neonatology. 10(2):101-107.
  6. Duncan MA, Vázquez Flores S, Chávez Lluévamos EB, Cantú Salinas AC, De León Flores L, Villarreal Velázquez HJ (2014) Dyke-Davidoff-Masson syndrome: A case study. Medicina universitaria. 16(63):71-73.
  7. Razek AA, Kandell AY, Elsorogy LG, Elmongy A, Basett AA (2009) Disorders of cortical formation: MR imaging features. American Journal of Neuroradiology. 30(1):4-11
  8. Shetty DS, Lakhkar BN, John JR (2003) Dyke-Davidoff-Masson syndrome. Neurology India. 51(1):136
  9. Narain NP, Kumar R, Narain B (2008) Dyke-Davidoff-Masson syndrome. Indian Pediatrics. 45: 11.

JCMCRI
ISSN: 3064-8025

Journal of Clinical & Medical Case Reports, Images (JCMCRI) ISSN: 3064-8025 is a peer-reviewed journal dedicated to publishing clinical images from all sectors of medicine. The goal of this magazine is to disseminate information about new discoveries and treatments in science and medicine.

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