Dyke-Davidoff-Masson syndrome

Case contributed by Dr Heba Abdelmonem

Presentation

A well being child till an encephalitis attack 4 years ago, followed by progressive right side weakness and recent onset of seizure.

Patient Data

Age: 7 years
Gender: Female
CT

CT demonstrates left cerebral hemiatrophy, dilated left lateral ventricle and Wallerian degeneration of the left cerebral peduncle, bone window shows hypertrophied calvarium more on the left side with dilated left frontal and ethmoidal sinuses. The right temporal and parietal lobes demonstrate encephalomalacia with dilated right temporal horn. Multiple areas of dystrophic gyral calcification are demonstrated on both sides.

Case Discussion

A healthy child with history of encephalitis 4 years ago now describes progressive right side weakness and intractable seizures. The CT findings, history and clinical presentation fit the criteria of Dyke-Davidoff-Masson syndrome.

The etiology of the syndrome is abnormal insult that happens either  intrauterine or early in life results in hypoplasia / atrophy of a cerebral hemisphere. The main differential of the syndrome is Rasmussen encephalitis which isn't characterized by hypertrophied skull and Sturge-Weber syndrome which was excluded by the normal pre insult imaging findings.

 

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Case information

rID: 63032
Published: 15th Sep 2018
Last edited: 14th Aug 2019
Inclusion in quiz mode: Included
Institution: Zagazig University Hospital

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