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Dyke-Davidoff-Masson syndrome.

Manas R Behera¹, Sibabrata Patnaik, Aswini K Mohanty

¹Department of Paediatrics, Kalinga Institute of Medical Sciences (KIMS), KIIT University, Patia, Bhubaneswar, Orissa, India.

Journal of Neurosciences in Rural Practice

|November 29, 2012

Summary

A pediatric case of Dyke-Davidoff-Masson syndrome presented with seizures and developmental delays. Brain imaging confirmed infantile cerebral hemiatrophy, highlighting this rare neurological condition.

Keywords:

Dyke-Davidoff-Masson syndrome hemiatrophy

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Area of Science:

Neurology
Pediatrics
Radiology

Background:

Infantile cerebral hemiatrophy, also known as Dyke-Davidoff-Masson syndrome, is a rare congenital neurological disorder.
It is characterized by unilateral cerebral atrophy and ipsilateral hypertrophy of the skull and sinuses.

Observation:

A 14-month-old male infant exhibited recurrent generalized seizures.
The child presented with spastic hemiplegia, microcephaly, and significant delays in motor and speech development.

Findings:

Cerebral CT scans displayed features consistent with the infantile form of cerebral hemiatrophy.
These findings are diagnostic for Dyke-Davidoff-Masson syndrome.

Implications:

Early diagnosis of Dyke-Davidoff-Masson syndrome is crucial for timely intervention and management.
Understanding the neuroimaging characteristics aids in differentiating this condition from other pediatric neurological disorders.
Further research into the etiology and long-term outcomes of infantile cerebral hemiatrophy is warranted.