Septooptic dysplasia is a rare anterior midline anomaly considered to be a mild form of lobar holoprosencephaly. We describe a case with unilateral optic nerve hypoplasia and the absence of a septum pellucidum.
Holoprosencephaly ; Optic Nerve ; Septo-Optic Dysplasia* ; Septum Pellucidum

Septo-optic dysplasia is an uncommon developmental disorder consisting of septum pellucidum angenesis, optic nerve hypoplasia, congenital nystagmus, seizure and multiple endocrine dysfunction. Clinically mild forms of septo-optic dysplasia and syndrome of absent septum pellucidum are now easily detected by computed tomography. We report two cases of septo-optic dysplasia with consistent radiographic findings with the anomaly.
Nystagmus, Congenital ; Optic Nerve ; Seizures ; Septo-Optic Dysplasia* ; Septum Pellucidum

Central neurocytoma, a cerebral intraventricular tumor, is usually benign. It frequently develops in the area of the foramen of Monro, and is usually attached to the septum pellucidum. Mild to moderate contrast enhancement is common. We encountered a case of central neurocytoma in a 60-year-old woman; the tumor arose from the atrium of the lateral ventricle, and extraventricular extension and malignant transformation were apparent. CT and MRI revealed a well-defined, slightly heterogeneous mass measuring 2.5x3x5cm with surrounding edema. There was strong contrast enhancement of the mass.
Cerebral Ventricles ; Edema ; Female ; Humans ; Lateral Ventricles ; Magnetic Resonance Imaging ; Middle Aged ; Neurocytoma* ; Septum Pellucidum

OBJECTIVE: To assess the visualization rate and size of the cavum septum pellucidum (CSP) by transabdominal sonography in normal fetuses in the second and third trimesters of pregnancy. METHODS: The CSP was prospectively researched and measured using an axial transventricular plane in 308 consecutive uncomplicated singleton pregnancies between 15 and 41 weeks of gestation. RESULTS: The CSP was seen in 42.5% of cases at 15 weeks, 84.78% at 16-17 weeks, 100% at 18-37 weeks and 81.25% at 38-41 weeks. Compared to biparietal diameter (BPD), the visualization rate was 35.29% between 31 and 32 mm, 47.8% between 33 and 34 mm, 84% between 35 and 43 mm, 100% between 44 and 88 mm and 87.5% between 89 and 101 mm. Mean CSP width was 5.5+/-1.7 mm (range 2-9 mm). The CSP width was increased with gestational age and BPD, but with a slight decrease around term. CONCLUSION: In normal fetuses the CSP should always be visualized between 18 and 37 weeks, or with a BPD of 44-88 mm. Failure to observe the CSP in this interval, or possibly the presence of a large CSP, may indicate abnormal cerebral development and warrant further investigation. Conversely, absence of the CSP prior to 18 weeks, or later than 37 weeks, is a normal finding.
Female ; Fetus* ; Gestational Age ; Humans ; Pregnancy ; Pregnancy Trimester, Third* ; Pregnancy* ; Prospective Studies ; Septum Pellucidum*

OBJECTIVE: The authors study a relationship between the presence of cavum septum pellucidum(CSP) and the development of epilepsy by comparing the presence of CSP, which has been known to be a normal variation, in normal control group and epilepsy patients. METHODS: This study included 377 patients with epilepsy and 252 controls without epilepsy. Of epilepsy patients, 168 patients underwent surgery due to intractability and 209 patients was on medication of antiepileptic drugs. Control group had only headache and no visible lesion in MRI. Of 168 surgical patients, 102 patients had temporal lobe epilepsy and 66 patients had extratemporal lobe epilepsy. Ninty five patients showed a neuronal migration disorder in histopathologic findings. Definition of "CSP" and "partial CSP" was followed by Pauling's classification. RESULTS: CSP was present 8.2% of epilepsy patients and 1.6% of control group(p<0.01). CSP was detected in 11.3% of patients with surgical treatment and in 5.7% of patients with medical treatment. CSP was noticed in 8.9% of temporal lobe epilepsy, in 15.2% of extratemporal lobe epilepsy, in 13.7% of patients with neuronal migration disorder, and in 8.2% of patients with no neuronal migration disorder. CONCLUSION: Presence of CSP is statistically higher in epilepsy patients than in control group. This results indicates that the presence of CSP may not be a simple normal variation, and it can be considered a developmental anomaly that may contribute to epileptogenesis.
Anticonvulsants ; Classification ; Epilepsy* ; Epilepsy, Temporal Lobe ; Headache ; Humans ; Magnetic Resonance Imaging ; Neuronal Migration Disorders ; Septum Pellucidum*

Septo-optic dysplasia, as first described by de Morsier, is a rare developmental anomaly involving optic nerve hypolasia, midline anomalies of the brain and variable hypothalamic-pituitary dysfunction. We experienced a case of septo-optic dysplasia in 56-day old male infant who presented with prolonged jaundice. A magnetic resonance imaging of the brain showed absence of the septum pellucidum and the ophthalmoscopic examination disclosed left optic nerve hypoplasia. Thus, we reported it with a brief review of literatures.
Brain ; Humans ; Infant ; Jaundice ; Magnetic Resonance Imaging ; Male ; Optic Nerve ; Septo-Optic Dysplasia* ; Septum Pellucidum

The authors report a case of oligodendroglioma in the septum pellucidum. A 22-year-old male presented with symptoms of headache and decreased visual acuity. Simple skull series showed calcified density near the midline. Brain C-T scan revealed huge calcified mass involving right lateral ventricle, 3rd ventricle and obstructive hydrocephalus. The authors diagnosed oligodendroglioma in operative findings and pathologic examination.
Brain ; Headache ; Humans ; Hydrocephalus ; Lateral Ventricles ; Male ; Oligodendroglioma* ; Septum Pellucidum* ; Skull ; Visual Acuity ; Young Adult

PURPOSE: To evaluate the significance of high signal intensity of the septum pellucidum in hydrocephalus on proton density-weighted brain MR images. MATERIALS AND METHODS: Authors reviewed the MR images of 418 cases of patients with normal(175 cases), hydrocephalic(35 cases), atrophic(58 cases), and other groups(150 cases) retrospectively. We analyzed the signal intensity of the septum pellucidum in the normal group and the incidences of high signal intensities of periventricular area of frontal horn of lateral ventricle(area 1), periventricular area except area 1(area 2), callososeptal area(area 3), and septum pellucidum(area 4) in the normal and abnormal groups. RESULTS: In the normal group, the septurn pellucidum was isointense to the head of caudate nucleus on proton density-weighted image. High signal intensity of the septurn pellucidurn was seen in 31 cases (22 cases of hydrocephalus, 5 cases of brain atrophy, and 4 cases of others), and showed high specificity(91.4%) for hydrocephalus in spite of low sensitivity(62.9%), as compared with periventricular hyperintensities of other areas. CONCLUSION: High signal intensity of the septum pellucidum on proton density-weighted image may be caused by transependymal CSF migration in the patients with hydrocephalus, and considered as an additional finding of hydrocephalus in the cases of ventriculomegaly.
Animals ; Atrophy ; Brain ; Caudate Nucleus ; Head ; Horns ; Humans ; Hydrocephalus* ; Incidence ; Protons ; Retrospective Studies ; Septum Pellucidum*

Septo-optic dysplasia, first described by de Morsier in 1956, is an uncommon congenital midline structural abnormality of the brain, including agenesis of septum pellucidum, primitive optic ventricle and dysplasia of the optic chiasm, optic nerves, and infundibulum. Agenesis of septum pellucidum, initially thought to be an integral part of this syndrome, is inconstant and not an essential part of the disorder. Also, it reveals a variable spectrum of associated abnormalities in disturbed hypothalamic-pituitary function. We recently experienced 3 patients of septo-optic dysplasia who showed bilateral optic disc hypoplasia clinically and midline structural abnormalities in computerized tomography(CT) or magnetic resonance imaging(MRI).
Brain ; Humans ; Optic Chiasm ; Optic Nerve ; Septo-Optic Dysplasia* ; Septum Pellucidum

De Morsier syndrome, or septo-optic dysplasia is a developmental anomaly characterized by unilateral or bilaterral optic nerve hypoplasia, agenesis of septum pellucidum, visual disturbance, and a variable spectrum of disturbed hypothalamic-pituitary function. We experienced a 7-month-old female with de Morsier syndrome whose presenting manifestation was congenital exotropia of right eye, and subsequent evaluation revealed optic nerve hypoplasia of right eye, absence of septum pellucidum, and hypothyroidism.
Exotropia* ; Female ; Humans ; Hypothyroidism ; Infant ; Optic Nerve ; Septo-Optic Dysplasia ; Septum Pellucidum