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Development Disturbance Of Schizencephaly Type II (Gangguan perkembangan pada Schizencephaly tipe II)

Development Disturbance Of Schizencephaly Type II (Gangguan perkembangan pada Schizencephaly tipe II)
Halomoan Saragi, Uni Gamayani , Siti Aminah
Unpad
Indonesia
Unpad
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Background : Schizencephaly is defined as a malformation with clefts of the cerebral cortex. Neuroimaging confirmed the diagnosis.

Insident in USA estimated to the numbers about 200.000 (ORD of NIH 2007 ). No data in Indonesia There are no difference of incident between race and gender, and more susceptible in newborn child. Clinical feature delayed developmental in language, mental retardation, paralyses, hypotonus, seizure and hydrocephalus.

Case : A girl 7 years old care in neurology department of Hasan Sadikin Hospital, When childhood deleyed of motor and speech not progressive. At first year of elementary regular school there was cognitive disturbance, The history of pregnancy, delivery were normal. Examination found : CP motorik tetraparese 4+| 4 ; speech combined 5 words ; ADL alone ; IQ 83.

At the age of 6 year 9 month there was once partial general seizure. EEG showed there were epileptogenik wave at right parietocentral area and cortical disfunction at right temporoacipital area. Head MRI indicate schizencephaly tipe II, described cyst lesion at right temporal communicated to arachnoid space and right ventrikel lateralis cornu posterior.

Prognosa : Quo ad vitam: ad bonam, Quo ad functionam : dubia ad bonam

Conclution : The etiologi of developmental disturbance must be search to estabilish prognosis and right management.

Latar Belakang : Schizencephaly adalah malformasi berupa celah pada korteks serebri, dapat didiagnosa dengan neuroimaging. Insidensi di USA sekitar 200.000 orang dari seluruh populasi ( ORD of NIH 2007 ). Data di Indonesia belum ada. Tidak terdapat perbedaan ras, jenis kelamin, sering mengenai anak baru lahir. Gambaran klinis berupa keterlambatan perkembangan berbicara, berbahasa, retardasi mental, paresis, hipotonus, kejang, dan hidrocefalus.

Kasus : Seorang anak perempuan 7 tahun dirawat dibagian saraf RSHS. dengan riwayat keterlambatan motorik dan berbicara sejak kecil yang tidak progressif. Di SD kelas 1 reguler terdapat gangguan kognitif, Riwayat kehamilan, kelahiran, normal ; pada pemeriksaan : CP tetraparese motorik 4+| 4 ; bicara merangkai 5 kata ADL mandiri ; IQ 83.

Pada usia 6 tahun 9 bulan kejang satu kali bentuk parsial umum sekunder. EEG menunjukkan gelombang epileptogenik di parietosentral kanan dan disfungsi kortikal di temporoksipital kanan. MRI kepala ditemukan Schizencephaly tipe II, berupa lesi kistik didaerah temporal kanan, berhubungan dengan ruang arachnoid dan ventrikel lateralis kanan cornu posterior.

Prognosa : Quo ad vitam: ad bonam, Quo ad functionam : dubia ad bonam.

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