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Dyke-Davidoff-Masson syndrome.

N Vinay Kumar1, T S Gugapriya1, Arun T Guru2

  • 1Department of Anatomy, Chennai Medical College Hospital and Research Centre, Trichy, Tamil Nadu, India.

International Journal of Applied & Basic Medical Research
|March 10, 2016
PubMed
Summary
This summary is machine-generated.

Dyke-Davidoff-Masson syndrome (DDMS) involves brain hemisphere atrophy from early insults. This report details a rare case in a female adolescent with right-sided cerebral changes, deviating from typical male, left-sided presentations.

Keywords:
Cerebral atrophyparanasal air sinusespetrous ridgeprominent sulci

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Area of Science:

  • Neurology
  • Radiology
  • Pediatric Neurology

Background:

  • Dyke-Davidoff-Masson syndrome (DDMS) is characterized by cerebral hemiatrophy resulting from prenatal or early childhood brain insults.
  • While typically affecting males and the left hemisphere, DDMS presentation varies with insult timing and can manifest in adolescence.

Observation:

  • A 17-year-old female presented with refractory seizures, hemiplegia, and cognitive deficits.
  • Clinical examination revealed facial asymmetry, delayed milestones, and spastic hemiplegia.

Findings:

  • Computed tomography (CT) scans demonstrated right cortical atrophy, ventricular dilatation, prominent sulci, and midline shift.
  • Bone window CT images revealed skull asymmetry, including diploic space width and paranasal sinus size differences.

Implications:

  • This case highlights the atypical presentation of DDMS in a female adolescent with right-sided cerebral involvement.
  • It underscores the importance of considering DDMS in adolescents with neurological deficits, even with non-classical findings.
  • Radiological assessment is crucial for diagnosing DDMS and understanding its varied manifestations.