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Dyke-Davidoff-Masson syndrome.

Nigam Prakash Narain1, Rakesh Kumar, Bhupendra Narain

  • 1Department of Pediatrics, Patna Medical College, Patna, Bihar, India. nigampn@gmail.com

Indian Pediatrics
|November 26, 2008
PubMed
Summary

Dyke Davidoff Masson syndrome (DDMS) involves seizures, facial asymmetry, and hemiplegia. This case highlights DDMS in an 18-month-old girl with specific neurological and developmental symptoms.

Area of Science:

  • Neurology
  • Pediatrics
  • Radiology

Background:

  • Dyke Davidoff Masson syndrome (DDMS) is a rare congenital neurological disorder.
  • It is characterized by a triad of seizures, facial asymmetry, and contralateral hemiplegia, often with intellectual disability.

Observation:

  • This report details a case of DDMS in an 18-month-old female infant.
  • The patient presented with right-sided focal seizures and ipsilateral hemiparesis.
  • Delayed developmental milestones were also noted.

Findings:

  • Radiological findings consistent with DDMS include cerebral hemiatrophy on the affected side.
  • Homolateral hypertrophy of the skull and sinuses are characteristic imaging features.
  • The presented case aligns with these typical radiological manifestations.

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Implications:

  • Early diagnosis of DDMS is crucial for timely intervention and management.
  • Understanding the clinical and radiological spectrum aids in differentiating DDMS from other neurological conditions.
  • This case contributes to the literature on pediatric presentations of DDMS.