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

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*

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

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

Septo-optic dysplasia, as first described by de Morsier, is a rare developmental anomaly of absent or hypoplastic septum pellucidum, a primitive optic vesicle, and dysplasia of the optic nerve, chiasm, and optic tracts. It is a common cause of hypopituitarism in children and has variable hypothalamic-pituitary dysfunction. We experienced a case of septo-optic dysplasia in 5 month old female baby who presented with developmental delay and seizure. A magnetic resonance imaging of the brain showed absence of the septum pellucidum and the fundoscopic examination disclosed the absence of left optic disc and atrophy of right optic disc.
Atrophy ; Brain ; Child ; Female ; Humans ; Hypopituitarism ; Infant ; Magnetic Resonance Imaging ; Optic Nerve ; Seizures ; Septo-Optic Dysplasia* ; Septum Pellucidum ; Visual Pathways

The septo-optic dysplasia, or de Morsier syndrome is a developmental anomaly characterized by involvement of the septum pellucidum, optic system and hypothalamic-pituitary axis. We had experienced a case of septo-optic dysplasia in 8 month-old female. A magnetic resonance imaging of the brain showed isolated absent septum pellucidum. And ophthalmoscopic examination showed right optic nerve hypoplasia, exotropia of right eye.
Axis, Cervical Vertebra ; Brain ; Exotropia ; Female ; Humans ; Infant ; Magnetic Resonance Imaging ; Optic Nerve ; Septo-Optic Dysplasia* ; Septum Pellucidum

Although the incidence of epidemic encephalitis has decreased mardekly nowadays, sporadic cases are stillobserved in Korea. In this study, CT findings of 17 patients with clinically and serologically proven epidemicencephalitis were analized. In principle, CT scans were done at the acute and subacute stage, whithin 20 days fromonset. The results were follows; 1. Of all 17 patients with CT scan, normal scan were demonstrated in 3 cases andabnormal scans in 14 cases. 2. The abnormal CT findings were ; area of low density in 9 cases, brain edema in 7cases, nodular and gyral contrast enhancement in 3 cases, central brain atrophy in 3 cases and hemorrhagic highdensity in 2 cases in order of frequency. 3. The CT findings, according to date of CT scan from onset, were; brainedema and are of low density within 10 days from onset, normal or central brain atrophy between 11 days and 20days, are of hemorrhagic high density and contrast enhanced lesions at both stage. 4. The locational distributionswere; thalamus and cerebral white matter in 7 cases respectively, basal ganglia in 5 cases, cerebral cortex in 4cases and septum pellucidum in 1 case in order of frequency.
Atrophy ; Basal Ganglia ; Brain ; Brain Edema ; Cerebral Cortex ; Encephalitis, Arbovirus ; Humans ; Incidence ; Korea ; Septum Pellucidum ; Thalamus ; Tomography, X-Ray Computed ; White Matter

About one tenth of all CNS neoplasms involves the brain. Due to their location in the ventricles they often present similar nonspecific clinical manifestation. Localization and differential diagnosis are dependent on radiological investigation. For the identification of specific CT characteristics of the intraventricular tumors and the differental diagnosis, we retrospectively analyzed 22 pathologically proved cases seen on CT. Important differential features included age and sex of the patient, the location within the ventricle, and the morpholgic appearance of the mass and density on CT before and after intravenous administration of contrast material. Meningiomas (4 cases) and a germinoma showed increased density on the precontrast CT scans, and demonstrated dense uniform enhancement of the postenhanced scan. Choroid plexus papillomas (3 cases) showed dense uniform contrast enhancement. Intraventricular neurocytomas (3 cases) demonstrated characteristic attachment to the septum pellucidum, confinement of the lateral and third ventricle, and calcification within the mass Colloid cysts (2 cases) showed characteristic location of anterosuperior aspect of the third ventricle. In conclusion, CT findings of intraventricular tumors are usually nonspecific. The location of the mass and the patient's age are the most helpful information in the differential diagnosis.
Administration, Intravenous ; Brain ; Colloid Cysts ; Diagnosis ; Diagnosis, Differential ; Germinoma ; Humans ; Meningioma ; Neurocytoma ; Papilloma, Choroid Plexus ; Retrospective Studies ; Septum Pellucidum ; Third Ventricle ; Tomography, X-Ray Computed

PURPOSE: To report the incidence and new findings of abnormal brain imaging studies associated with patients initially diagnosed with Leber's congenital amaurosis (LCA) without definite systemic abnormalities and to determine the need for brain imaging studies in these patients. METHODS: A retrospective review of medical records was performed in 83 patients initially diagnosed as LCA and without definite systemic abnormalities before the age of 6 months in 2 tertiary referral centers. Brain magnetic resonance imaging was performed in 31 of 83 patients (37.3%). RESULTS: Six of 31 patients (19%) had radiologically documented brain abnormalities. Two patients had cerebellar vermis hypoplasia, 1 patient showed an absence of septum pellucidum, 2 subjects showed mild external hydrocephalus, and 1 patient was found to have a small cerebellum. CONCLUSIONS: Approximately one fifth of the LCA patients in whom brain imaging was performed were associated with brain abnormalities, including the absence of septum pellucidum, which has not been documented in the literature. Brain imaging is mandatory in patients primarily diagnosed with LCA, even without definite neurologic or systemic abnormalities.
Brain/*pathology ; Cerebellum/pathology ; Female ; Humans ; Hydrocephalus/pathology ; Infant ; Leber Congenital Amaurosis/*diagnosis ; *Magnetic Resonance Imaging ; Retrospective Studies ; Septum Pellucidum/pathology

Holoprosencephaly is a rare CNS developmental defect with midline cleavage of embryonal forebrain during the fourth week of fetal development characterized with s single ventricle. Holoprosencephaly is divided into three types by the degree of the brain cleavage : alobar, semilobar, and lobar. Depending on the degree of defected regions, there exist variable clinical symptoms and signs such as stillbirth, hypotonia, apnea, convulsion, delayed development and visual disturbance. It was frequently associated with the deformity of the brain as well as the midline facial structures. Advances in neuroimaging over the past decades have led us to a better understanding of the pathogenesis and the variability of holoprosencephaly. As a result, a lot of cases of holoprosencephaly with various clinical and pathologic findings have been reported. We experienced a case of isolated interhemisphere fusion of frontal lobes with intact septum pellucidum in a child with febrile status epilepticus. Is it a variant of holoprosencephaly?
Apnea ; Brain ; Child ; Congenital Abnormalities ; Fetal Development ; Frontal Lobe* ; Holoprosencephaly* ; Humans ; Muscle Hypotonia ; Neuroimaging ; Prosencephalon ; Seizures ; Septum Pellucidum* ; Status Epilepticus ; Stillbirth