Hemiatrophy of the brain may occur in adults and in old age as a result of degenerative or vascular disorders, but the name is more commonly applied to the congenital or the infantile variety of the condition. It was proposed by Alpers and Dear (1939) that the two varieties be designated secondary and primary respectively. The condition is, however, always secondary in the sense that it is due ...

Alström syndrome (AS) is a rare autosomal recessive disorder, characterized by retinal degeneration, progressive hearing impairment, truncal obesity and non-insulin dependent diabetes mellitus. A 6-year-old girl was admitted with aphasia, deafness, strabismus, abdominal distention, and weakness on the right body side. The physical and laboratory examination revealed psychomotor retardation, rig...

Dyke-Davidoff-Masson Syndrome (DDMS) is a syndrome associated with refractory epilepsy. DDMS is a rare syndrome characterized by seizures, facial asymmetry, contralateral hemiplegia and mental retardation. The characteristic radiologic features are cerebral hemiatrophy with homolateral hypertrophy of the skull and sinuses. The case was an 18 years old female with seizures, hemiparesis of the ri...

Dyke-Davidoff-Masson Syndrome (DDMS) is a rare syndrome characterized with specific clinical and radiological findings due to involvement of the developing brain cerebral hemiatrophy one hemisphere. The was first described from Dyke, Davidoff Masson in 1933 series nine patients. has two forms, congenital acquired forms etiological factors vary brain. Most common symptom are focal or secondary g...

The nervous system (NS) in its complexity still has some surprises store for us. developmental disorder of the NS during embryonic period is at origin malformations cortical development (CDM) which represent a major cause mental and motor handicaps as well severe epilepsy. Many anomalies latter (SN) are not yet described will continue to be over time given significant advances science observed ...

Incontinentia pigmenti is a systemic disorder affecting the skin, teeth, eyes, nervous tissue, hair, nails, musculoskeletal system, and heart. We describe an 11-month-old girl with incontinentia pigmenti associated with a ventricular septal defect, left hemiatrophy, hemangiomas, an abnormal labial frenum, and spastic cerebral palsy manifested as left hemiplegia and developmental delay. We belie...

Acta Neurologica Taiwanica Vol 13 No 4 December 2004 From the Department of Neurology, Chang Gung Memorial Hospital, Linkou Medical Center, Taoyuan, Taiwan. Received November 19, 2004. Revised and Accepted November 29, 2004. Reprint requests and correspondence to: Nai-Shin Chu, MD. Department of Neurology, Chang Gung Memorial Hospital, No. 199, Tung-Hwa N. Road, Taipei, Taiwan. E-mail: chu060@c...


 Lennox-Gastaut syndrome (LGS) is a form of severe epileptic encephalopathy in children. LGS with encephalomalacia cysts rare We report six-year-old mentally retarded boy who was referred for an intractable seizure. Seizures were tonic, atonic, and dialeptic frequency. EEG showed generalized SSW discharges 1.5-2 Hz, polyspikes, burst suppression typical LGS. Head MRI cyst the right subcor...