PURPOSE: Agenesis of the septum pellucidum (ASP) is a very rare disease that can be isolated or associated with other brain abnormalities. The neurological prognosis of isolated ASP remains controversial. The aim of this study was to evaluate the clinical outcome of neonates with ASP. METHODS: We retrospectively analyzed the medical records of 12 neonates with isolated ASP or ASP combined with other brain abnormalities who were born at Cheil General Hospital & Women's Healthcare Center between January 2007 and December 2017. RESULTS: Of the 12 neonates, six were identified prenatally and six were identified postnatally. Isolated ASP was found in eight neonates; of these, four were detected antenatally. ASP associated with other brain abnormalities was found in four neonates. ASP was complete in nine neonates, including six with isolated ASP, and partial in three, including two with isolated ASP. Six of the eight neonates with isolated ASP had normal neurological development, except two who were lost to follow-up. Among the four neonates with other associated brain abnormalities, two had delayed motor development and a seizure, one had normal development, and one was lost to follow-up. In all neonates, ophthalmological examination revealed no optic nerve abnormalities. CONCLUSION: Isolated ASP seems to have a good neurological prognosis without ocular problems. This result needs to be confirmed by larger prospective studies over a longer developmental timeline.
Brain ; Delivery of Health Care ; Hospitals, General ; Humans ; Infant, Newborn ; Lost to Follow-Up ; Medical Records ; Optic Nerve ; Prognosis ; Prospective Studies ; Rare Diseases ; Retrospective Studies ; Seizures ; Septum Pellucidum ; Viperidae

OBJECTIVE: Shunt-dependent hydrocephalus (SdHCP) is a well-known complication of aneurysmal subarachnoid hemorrhage (SAH). The risk factors for SdHCP have been widely investigated, but few risk scoring systems have been established to predict SdHCP. This study was performed to investigate the risk factors for SdHCP and devise a risk scoring system for use before aneurysm obliteration.METHODS: We reviewed the data of 301 consecutive patients who underwent aneurysm obliteration following SAH from September 2007 to December 2016. The exclusion criteria for this study were previous aneurysm obliteration, previous major cerebral infarction, the presence of a cavum septum pellucidum, a midline shift of >10 mm on initial computed tomography (CT), and in-hospital mortality. We finally recruited 254 patients and analyzed the following data according to the presence or absence of SdHCP : age, sex, history of hypertension and diabetes mellitus, Hunt-Hess grade, Fisher grade, aneurysm size and location, type of treatment, bicaudate index on initial CT, intraventricular hemorrhage, cerebrospinal fluid drainage, vasospasm, and modified Rankin scale score at discharge.RESULTS: In the multivariate analysis, acute HCP (bicaudate index of ≥0.2) (odds ratio [OR], 6.749; 95% confidence interval [CI], 2.843–16.021; p=0.000), Fisher grade of 4 (OR, 4.108; 95% CI, 1.044–16.169; p=0.043), and an age of ≥50 years (OR, 3.938; 95% CI, 1.375–11.275; p=0.011) were significantly associated with the occurrence of SdHCP. The risk scoring system using above parameters of acute HCP, Fisher grade, and age (AFA score) assigned 1 point to each (total score of 0–3 points). SdHCP occurred in 4.3% of patients with a score of 0, 8.5% with a score of 1, 25.5% with a score of 2, and 61.7% with a score of 3 (p=0.000). In the receiver operating characteristic curve analysis, the area under the curve (AUC) for the risk scoring system was 0.820 (p=0.080; 95% CI, 0.750–0.890). In the internal validation of the risk scoring system, the score reliably predicted SdHCP (AUC, 0.895; p=0.000; 95% CI, 0.847–0.943).CONCLUSION: Our results suggest that the herein-described AFA score is a useful tool for predicting SdHCP before aneurysm obliteration. Prospective validation is needed.
Aneurysm ; Cerebral Infarction ; Cerebrospinal Fluid Leak ; Diabetes Mellitus ; Hemorrhage ; Hospital Mortality ; Humans ; Hydrocephalus ; Hypertension ; Multivariate Analysis ; Prospective Studies ; Risk Factors ; ROC Curve ; Septum Pellucidum ; Subarachnoid Hemorrhage ; Ventriculoperitoneal Shunt

Medulloblastoma is a common pediatric tumor typically diagnosed before the age of fifteen. Initial therapy includes surgical resection and radiation of the entire neuro-axis. Recurrence is common and typically occurs within 2 years of initial diagnosis. Those fitting Collin's Law is considered tumor-free. We report a case of single supratentorial recurrence 13 years after initial diagnosis. Here we present a 22 year old male presenting 13 years after initial diagnosis with isolated septum pellucidum recurrence. He underwent complete resection of the tumor. Medulloblastoma is a common in the pediatric population. Late recurrence to the ventricular system is uncommon. Long term follow-up is recommended in these patients.
Diagnosis ; Follow-Up Studies ; Humans ; Jurisprudence ; Male ; Medulloblastoma* ; Neuroectodermal Tumors, Primitive ; Recurrence* ; Septum Pellucidum

A 27-year-old male with nonobstructive hydronephrosis was referred from the urology department for polyuria evaluation and management. The patient was hospitalized for urinary tract infection and cystostomy was performed due to neurogenic bladder of unknown origin. The patient was of short stature and had visual impairment. From the interview, we discovered he had been suffering from polyuria and polydipsia for more than 20 years. Urine output was 13 L/day and urine osmolarity was 85 mOsm/kg. The results of a water deprivation test were consistent with central diabetes insipidus. Septo-optic dysplasia (SOD) was observed on brain magnetic resonance imaging (MRI). SOD is a very rare condition characterized by agenesis of the septum pellucidum or corpus callosum, which may cause optic nerve aplasia or hypoplasia, midbrain abnormalities and/or hypopituitarism. After desmopressin treatment, polyuria and hydronephrosis were improved. We report a case of a 27-year-old male diagnosed with SOD including diabetes insipidus, resulting in nonobstructive hydronephrosis.
Adult ; Brain ; Corpus Callosum ; Cystostomy ; Deamino Arginine Vasopressin ; Diabetes Insipidus ; Diabetes Insipidus, Neurogenic* ; Humans ; Hydronephrosis* ; Hypopituitarism ; Magnetic Resonance Imaging ; Male ; Mesencephalon ; Optic Nerve ; Osmolar Concentration ; Polydipsia ; Polyuria ; Septo-Optic Dysplasia* ; Septum Pellucidum ; Urinary Bladder, Neurogenic ; Urinary Tract Infections ; Urology ; Vision Disorders ; Water Deprivation

Computed tomography angiography (CTA) is commonly used in setting of subarachnoid hemorrhage, but imaging features of aneurysm rupturing taking place at the time of scanning has rarely been described. The author reports a case of actively rebleeding aneurysm of the anterior communicating artery with intraventricular extravasation on the hyperacute CTA imaging. The rebleeding route, not into the third ventricle but into the lateral ventricles, can be visualized by real-time three-dimensional CT pictures. The hemorrhage broke the septum pellucidum and the lamina rostralis rather than the lamina terminalis.
Aneurysm ; Angiography* ; Arteries ; Hemorrhage* ; Hypothalamus ; Intracranial Aneurysm* ; Lateral Ventricles* ; Septum Pellucidum* ; Subarachnoid Hemorrhage ; Third Ventricle

Abnormalities, Multiple ; diagnosis ; genetics ; pathology ; Brain ; diagnostic imaging ; pathology ; Humans ; Infant ; Magnetic Resonance Imaging ; Male ; Optic Atrophies, Hereditary ; diagnostic imaging ; pathology ; Radiography ; Septo-Optic Dysplasia ; diagnosis ; genetics ; pathology ; Septum Pellucidum ; diagnostic imaging ; pathology

The treatment of refractory schizophrenia has been a clinical challenge for most psychiatrists; the possible reasons include diagnostic errors, medical conditions and brain dysgenesis. Here, we described a patient with childhood-onset schizophrenia who had severe psychiatric symptoms such as auditory hallucinations and persecutory delusions, and etc. We reexamined all his possible medical conditions and found that the patient had an abnormally enlarged cavus septum pellucidum (CSP) combined with cavum vergae (CV) (maximum length >30 mm). Some reports suggested that abnormal CSP (length >6 mm) has a significant association with schizophrenia. However, abnormally large CSP or CSP/CV and related prognosis were reported rarely. This case suggested that abnormally enlarged CSP or CSP/CV may worsen the prognosis.
Adolescent ; Antipsychotic Agents ; therapeutic use ; Benzodiazepines ; therapeutic use ; Clozapine ; therapeutic use ; Dibenzothiazepines ; therapeutic use ; Humans ; Male ; Quetiapine Fumarate ; Schizophrenia, Childhood ; diagnosis ; drug therapy ; pathology ; Septum Pellucidum ; pathology

OBJECTIVE: To evaluate the surgical efficacy of and factors associated with decompressive craniectomy in patients with an internal carotid artery (ICA) territory infarction. METHODS: Seventeen patients (8 men and 9 women, average age 61.53 years, range 53-77 years) were treated by decompressive craniectomy for an ICA territory infarction at our institute. We retrospectively reviewed medical records, radiological findings, and National Institutes of Health Stroke Scale (NIHSS) at presentation and before surgery. Clinical outcomes were assessed using the Glasgow Outcome Scale (GOS). RESULTS: Of the 17 patients, 15 (88.24%) achieved a poor outcome (Group A, GOS 1-3) and 2 (11.76%) a good outcome (Group B, GOS 4-5). The mortality rate at one month after surgery was 52.9%. Average preoperative NIHSS was 27.6+/-10.88% in group A and 10+/-4.24% in group B. Mean cerebral infarction fraction at the septum pellucidum level before surgery in group A and B were 33.67% and 23.72%, respectively. Mean preoperative NIHSS (p=0.019) and cerebral infarction fraction at the septum pellucidum level (p=0.017) were found to be significantly associated with a better outcome. However, no preexisting prognostic factor was found to be of statistical significance. CONCLUSION: The rate of mortality after ICA territory infarction treatment is relatively high, despite positive evidence for surgical decompression, and most survivors experience severe disabilities. Our findings caution that careful consideration of prognostic factors is required when considering surgical treatment.
Carotid Artery, Internal ; Cerebral Infarction ; Decompression, Surgical ; Decompressive Craniectomy ; Female ; Glasgow Outcome Scale ; Humans ; Infarction ; Male ; Medical Records ; National Institutes of Health (U.S.) ; Retrospective Studies ; Septum Pellucidum ; Stroke ; Survivors

We report a case of intracranial dissemination developing approximately 4 months after partial removal of a spinal cord anplastic astrocytoma in a 22-year-old male. He presented with paraplegia on initial admission at a local hospital. Spinal magnetic resonance (MR) images disclosed multiple intramedullary lesions at the T3-11. The tumor was partially removed. The final histologic diagnosis was anaplastic astrocytoma. Four months after the operation, he was admitted with the symptoms of headache and deterioration of consciousness. MR images showed enhanced lesions in the anterior horn of the left lateral ventricle, and septum pellucidum. He underwent computed tomography-guided stereotactic biopsy and histological appearance was consistent with anaplastic astrocytoma. The clinical course indicates that the tumor originated in the spinal cord and extended into the subarachnoid space, first the spinal canal and later intracranial.
Animals ; Astrocytoma ; Biopsy ; Consciousness ; Headache ; Horns ; Humans ; Lateral Ventricles ; Magnetic Resonance Spectroscopy ; Male ; Paraplegia ; Septum Pellucidum ; Spinal Canal ; Spinal Cord ; Subarachnoid Space ; Young Adult

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