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Dyke-davidoff-masson syndrome: cases of two brothers and literature review.

Park KI, Chung JM, Kim JY - J Epilepsy Res (2014)

Bottom Line: Sometimes, he noted an auditory aura.MRI demonstrated the hemispheric atrophy with the adjacent bony hypertrophy.Our sibling cases might have a familial predisposition and support the idea that clinical courses and radiological findings of DDMS are varied even within one family.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, Inje University College of Medicine, Seoul Paik Hospital, Seoul, Korea.

ABSTRACT
Dyke-Davidoff-Masson syndrome (DDMS) has cerebral hemiatrophy and compensatory ipsilateral skull thickening, and is manifested by recurrent seizures and hemiparesis. We present one case with typical DDMS, who had a brother suffering from epilepsy with mild imaging abnormality relevant to DDMS and similar seizure semiology. A 26-year-old man had a history of developmental delay, mental retardation, hemiparesis and recurrent seizures. His brother, 23-year-old man had also experienced recurrent seizures, but he had no neurological deficits. Older brother experienced focal motor seizures with/without secondary generalization. Sometimes, he noted an auditory aura. MRI demonstrated the hemispheric atrophy with the adjacent bony hypertrophy. The seizures of younger brother were mainly of the auditory type and the MRI showed mild hemispheric atrophy with hippocampal sclerosis without any bony change. Our sibling cases might have a familial predisposition and support the idea that clinical courses and radiological findings of DDMS are varied even within one family.

No MeSH data available.


Related in: MedlinePlus

MRIs of the two epilepsy brothers. A, B, C (case 1) The T2-weighted images of the axial view and the oblique coronal view demonstrate the cortical hemiatrophy, including hippocampal sclerosis, ipsilateral skull thickening and ventricular enlargement on the right side, which is typical feature of Dyke-Davidoff-Masson syndrome. D, E, F (case 2) The images show showed the asymmetry in lateral ventricle, hippocampal atrophy in the right side (arrow). The volume of right caudate body was smaller than that of contralateral side, whereas no difference of cortical and skull thickness.
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f1-er-4-1-24-6: MRIs of the two epilepsy brothers. A, B, C (case 1) The T2-weighted images of the axial view and the oblique coronal view demonstrate the cortical hemiatrophy, including hippocampal sclerosis, ipsilateral skull thickening and ventricular enlargement on the right side, which is typical feature of Dyke-Davidoff-Masson syndrome. D, E, F (case 2) The images show showed the asymmetry in lateral ventricle, hippocampal atrophy in the right side (arrow). The volume of right caudate body was smaller than that of contralateral side, whereas no difference of cortical and skull thickness.

Mentions: A 26-year-old man was referred for consultation about his recurrent seizures and hemiparesis. He was born by cesarian section for breech position, but in good condition. However, he had displayed developmental delay since infancy. He could express meaningful words at the age of 3, and showed a hemiplegic gait since he first started walking. The patient had experienced seizures since the age of 4. From the age of 4 to the time of referral, his seizures were yearly events regarding generalized tonic-clonic (GTC) seizure and weekly regarding aura-only. Neither febrile convulsion, nor meningoencephalitis occurred. The neurological examination revealed mild hemifacial weakness and hemiparesis on the left side. The intelligence quotient was 66. He experienced focal motor seizures in the left arm and leg, independently or prior to a GTC seizure. In some instances, he noted an auditory aura with or without being followed by a brief alteration of consciousness or secondary generalization. His brain magnetic resonance image (MRI) is demonstrated in Fig. 1A, B, and C. Repetitive electroencephalographies (EEGs) demonstrated continuous irregular theta slow activities and lower amplitude over the right hemisphere (Fig. 2A).


Dyke-davidoff-masson syndrome: cases of two brothers and literature review.

Park KI, Chung JM, Kim JY - J Epilepsy Res (2014)

MRIs of the two epilepsy brothers. A, B, C (case 1) The T2-weighted images of the axial view and the oblique coronal view demonstrate the cortical hemiatrophy, including hippocampal sclerosis, ipsilateral skull thickening and ventricular enlargement on the right side, which is typical feature of Dyke-Davidoff-Masson syndrome. D, E, F (case 2) The images show showed the asymmetry in lateral ventricle, hippocampal atrophy in the right side (arrow). The volume of right caudate body was smaller than that of contralateral side, whereas no difference of cortical and skull thickness.
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4066624&req=5

f1-er-4-1-24-6: MRIs of the two epilepsy brothers. A, B, C (case 1) The T2-weighted images of the axial view and the oblique coronal view demonstrate the cortical hemiatrophy, including hippocampal sclerosis, ipsilateral skull thickening and ventricular enlargement on the right side, which is typical feature of Dyke-Davidoff-Masson syndrome. D, E, F (case 2) The images show showed the asymmetry in lateral ventricle, hippocampal atrophy in the right side (arrow). The volume of right caudate body was smaller than that of contralateral side, whereas no difference of cortical and skull thickness.
Mentions: A 26-year-old man was referred for consultation about his recurrent seizures and hemiparesis. He was born by cesarian section for breech position, but in good condition. However, he had displayed developmental delay since infancy. He could express meaningful words at the age of 3, and showed a hemiplegic gait since he first started walking. The patient had experienced seizures since the age of 4. From the age of 4 to the time of referral, his seizures were yearly events regarding generalized tonic-clonic (GTC) seizure and weekly regarding aura-only. Neither febrile convulsion, nor meningoencephalitis occurred. The neurological examination revealed mild hemifacial weakness and hemiparesis on the left side. The intelligence quotient was 66. He experienced focal motor seizures in the left arm and leg, independently or prior to a GTC seizure. In some instances, he noted an auditory aura with or without being followed by a brief alteration of consciousness or secondary generalization. His brain magnetic resonance image (MRI) is demonstrated in Fig. 1A, B, and C. Repetitive electroencephalographies (EEGs) demonstrated continuous irregular theta slow activities and lower amplitude over the right hemisphere (Fig. 2A).

Bottom Line: Sometimes, he noted an auditory aura.MRI demonstrated the hemispheric atrophy with the adjacent bony hypertrophy.Our sibling cases might have a familial predisposition and support the idea that clinical courses and radiological findings of DDMS are varied even within one family.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, Inje University College of Medicine, Seoul Paik Hospital, Seoul, Korea.

ABSTRACT
Dyke-Davidoff-Masson syndrome (DDMS) has cerebral hemiatrophy and compensatory ipsilateral skull thickening, and is manifested by recurrent seizures and hemiparesis. We present one case with typical DDMS, who had a brother suffering from epilepsy with mild imaging abnormality relevant to DDMS and similar seizure semiology. A 26-year-old man had a history of developmental delay, mental retardation, hemiparesis and recurrent seizures. His brother, 23-year-old man had also experienced recurrent seizures, but he had no neurological deficits. Older brother experienced focal motor seizures with/without secondary generalization. Sometimes, he noted an auditory aura. MRI demonstrated the hemispheric atrophy with the adjacent bony hypertrophy. The seizures of younger brother were mainly of the auditory type and the MRI showed mild hemispheric atrophy with hippocampal sclerosis without any bony change. Our sibling cases might have a familial predisposition and support the idea that clinical courses and radiological findings of DDMS are varied even within one family.

No MeSH data available.


Related in: MedlinePlus