Dyke Davidoff Masson Syndrome

Classification according to ICD-10
I63	stroke
ICD-10 online (WHO version 2019)

The Dyke-Davidoff-Masson syndrome is a characteristic change in the skull (brain and facial skull) after unilateral brain atrophy in early childhood. The name refers to the first authors of the first description from 1933 by the US neuroradiologist Cornelius Gysbert Dyke and the US neurosurgeons Leo Max Davidoff and Clement B. Masson.

root cause

It is based on unilateral atrophy or hypoplasia of the cerebrum, usually due to an infarction during pregnancy , in newborns or infants.

Clinical manifestations

Clinical criteria are:

Seizures
Facial asymmetry
Hemiparesis of the opposite side
Mental disability

diagnosis

The following changes can be found in the X-ray image or in the computed tomography :

asymmetrically thickened skullcap ( compensatory )
asymmetrically enlarged pneumatization of the paranasal sinuses and mastoid
unilateral brain atrophy
Elevation of the temporal bone edge
Displacement of the falx cerebri to the affected side

Differential diagnosis

The following are to be distinguished:

Hemimegalencephaly
Sturge-Weber syndrome , possibly also associated
Rasmussen's encephalitis

literature

JD Diestro, MK Dorotan, AC Camacho, KT Perez-Gosiengfiao, LI Cabral-Lim: Clinical spectrum of Dyke-Davidoff-Masson syndrome in the adult: an atypical presentation and review of literature. In: BMJ Case Reports. Volume 2018, July 2018, S., doi: 10.1136 / bcr-2018-224170 , PMID 29973410 .
KI Park, JM Chung, JY Kim: Dyke-davidoff-masson syndrome: cases of two brothers and literature review. In: Journal of epilepsy research. Volume 4, number 1, June 2014, pp. 24-27, PMID 24977128 , PMC 4066624 (free full text).

Individual evidence

↑ ^a ^b ^c Bernfried Leiber (founder): The clinical syndromes. Syndromes, sequences and symptom complexes . Ed .: G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger. 7., completely reworked. Edition. tape 2 : symptoms . Urban & Schwarzenberg, Munich et al. 1990, ISBN 3-541-01727-9 .
↑ ^a ^b ^c ^d Radiopaedia
^ CG Dyke, LM Davidoff, CB Masson Cerebral hemiatrophy with homolateral hypertrophy of the skull and sinuses In: Surgery, Gynecology and Obstetrics, Chicago , 1933, Volume 57, pp. 588-600.

[Leiber-1] Bernfried Leiber (founder): The clinical syndromes. Syndromes, sequences and symptom complexes . Ed .: G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger. 7., completely reworked. Edition. tape 2 : symptoms . Urban & Schwarzenberg, Munich et al. 1990, ISBN 3-541-01727-9 .

[radio-2] Radiopaedia

[3] CG Dyke, LM Davidoff, CB Masson Cerebral hemiatrophy with homolateral hypertrophy of the skull and sinuses In: Surgery, Gynecology and Obstetrics, Chicago , 1933, Volume 57, pp. 588-600.