In a patient presenting with clinical features of obstructing hydrocephalus, stenosis of aqueduct of Sylvius was demonstrated by contrast ventriculography and C-T scan. The case of aqueduct stenosis in a dult has been described by spiller and Allen, 1907. Ventriculoperitoneal shunting resulted in clinical improvement with significant change in ventricular size and subarachnoid space.
Cerebral Aqueduct ; Constriction, Pathologic* ; Humans ; Hydrocephalus ; Subarachnoid Space ; Ventriculoperitoneal Shunt*

An analysis of recent experiences for the brain stem glioma with surgery and autopsy established confirmation of the characteristic symptoms and signs, and of diagnostic criterias of Conray-ventriculogram and vertebral-angiogram. The cases consisted of 2 cases of exophytic brain stem glioma and 4 cases of pontomedullary glioma, and 5 of 6 patients had the signs of the increased intracranial pressure in this cases. A resurgence of interest in the Conray ventriculogram has been produced by cut-off sign of aqueduct of Sylvius in 2 cases of the exophytic brain stem glioma. The survival period in adults (1-3 years) were longer than that in children (less than 3 months) in this series.
Adult ; Autopsy ; Brain Stem* ; Brain* ; Cerebral Aqueduct ; Child ; Glioma* ; Humans ; Intracranial Pressure

Amenorrhea is rarely presented as a manifestation of endocrinological disturbances in patients of chronic hydrocephalus. We describe two cases of secondary amenorrhea caused by hydrocephalus due to aqueductal stenosis. Two female patients of age 30 and 20 yr presented with amenorrhea and increasing headache. Magnetic resonance images revealed marked, noncommunicating hydrocephalus without any tumorous lesion. In one patient, emergent extraventricular drainage was necessary because of progressive neurological deterioration. Each patient underwent surgical intervention for the hydrocephalus-ventriculoperitoneal shunt and endoscopic third ventriculostomy. Both resumed normal menstruation continuing so far with further normal menstrual bleeding. These two cases and others reported in the literature indicated that the surgical intervention for hydrocephalus resolves amenorrhea in all the cases of amenorrhea due to hydrocephalus. The suspected role of the surgery is the correction of increased intracranial pressure, which is an important pathogenetic factor in the development of amenorrhea.
Adult ; Amenorrhea/*etiology ; *Cerebral Aqueduct ; Cerebrospinal Fluid Shunts ; Female ; Gonadorelin/deficiency ; Human ; Hydrocephalus/*complications

BACKGROUND: Acute transient obstructive hydrocephalus is rare in adults. We describe a patient with intraventricular hemorrhage (IVH) who experienced the delayed development of acute transient hydrocephalus. CASE REPORT: A 33-year-old man with a previously diagnosed Spetzler-Martin Grade 5 arteriovenous malformation presented with severe headache, which was found to be due to IVH. Forty hours after presentation he developed significant obstructive hydrocephalus due to the thrombus migrating to the cerebral aqueduct, and a ventriculostomy placement was planned. However, shortly thereafter his headache began to improve spontaneously. Within 4 hours after onset the headache had completely resolved, and an interval head CT scan revealed resolution of hydrocephalus. CONCLUSIONS: In patients with IVH, acute obstructive hydrocephalus can develop at any time after the ictus. Though a delayed presentation of acute but transient obstructive hydrocephalus is unusual, it is important to be aware of this scenario and ensure that deterioration secondary to thrombus migration and subsequent obstructive hydrocephalus do not occur.
Adult ; Arteriovenous Malformations ; Cerebral Aqueduct ; Dietary Sucrose ; Head ; Headache ; Hemorrhage ; Humans ; Hydrocephalus ; Thrombosis ; Ventriculostomy

Evaluation of intracranial CSF flow was accomplished by the use of cine MR technique. In the cine MR, there were two methods of evaluation in CSF flow pattern. Qualitative and quantitative methods were called magnitude reconstruction and phase contrast mapping method, respectively. The image of magnitude reconstruction method can demonstrate areas of decreased CSF flow and help explain the cause of hydrocephalus. The image of phase contrast mapping method is more sensitive to fluid motion and may increase utility in the future for analysis of fluid flow. Cine MR is capable of showing both normal and abnormal intracranial CSF flow. Such a study can be easily added as an extra pulse sequence at the end of a routinely acquired MR examination. We evaluated 2 cases of the normal pattern of pulsatile flow within subarachnoid space and 3 cases of abnormal patterns of CSF flow(communicating hydrocephalus: 2 cases, arachnoid cyst in posterior fossa: 1 case). These observations were compared with pre- and post-operative CSF flow state. In conditions which result in alterations of flow, cine MR shows either obstruction or excessively turbulent flow within the CSF pathways. In our studies, the most distinctive pathological finding was bulk flow in the aqueduct of Sylvius. The authors suggest that this technique can be applied in a wide range of conditions where CSF pathway is altered including hydrocephalus, evaluation of the function of the shunt system and communication between arachnoid cyst and subarachnoid space . Moreover correct diagnosis is possible in patients with hydrocephalus, in whom the exact level of CSF obstruction can be determined. We believe that surgical decisions can be aided by careful analysis of these CSF cine MR studies. We discuss the normal and abnormal CSF flow findings and indications of cine MR CSF flow technique with literature review.
Arachnoid ; Cerebral Aqueduct ; Diagnosis ; Humans ; Hydrocephalus ; Magnetic Resonance Imaging ; Pulsatile Flow ; Subarachnoid Space

OBJECTIVE: To analyze L1CAM gene mutation in a family featuring X-linked recurrent fetal hydrocephalus. METHODS: The family had three pregnancies where a male fetus was detected at 22 weeks with hydrocephalus by ultrasonography. DNA was extracted from peripheral blood samples from the parents as well as fetal tissue from the third abortion. The fetal DNA was subjected to testing of folic acid metabolism ability gene and chromosomal microarray analysis (CMA). Next-generation sequencing (NGS) was employed to detect potential mutation of related genes. Suspected mutation was verified by Sanger sequencing. RESULTS: Testing of folic acid metabolism ability gene (MTHFR C677T) and CMA were both normal. A c.512G>A (p.Trp171Ter) hemizygous mutation of the L1CAM gene was detected in the fetal tissue, which was inherited from the phenotypically normal mother. The novel mutation was predicted to be pathogenic. CONCLUSION The c.512G>A (p.Trp171Ter) mutation of the L1CAM gene probably underlies the X-linked hydrocephalus in this family. Screening of L1CAM gene variations should be carried out for couples experiencing recurrent fetal hydrocephalus affecting the male gender.
Cerebral Aqueduct ; Female ; Humans ; Hydrocephalus ; genetics ; Male ; Mutation ; Neural Cell Adhesion Molecule L1 ; genetics ; Pedigree ; Pregnancy

Isolated fourth(IV) ventricle in shunted patients has been reported with increasing frequency. Symptomatic isolated IV ventricular hydrocephalus in adults, however, has seldom been described. We report five such cases among total of 420 shunted cases in our institution from January 1992 to December 1995. The causes of initial hydrocephalus were postsurgical meningitis(SAH, teratoma and abscess of posterior fossa), tuberculous meningitis and neurocysticercosis of the IV ventricle. All cases were symptomatic with clinical findings related to posterior fossa lesions. Two patients developd symptoms in 2 months after V-P shunts and the others between 17 and 118 months after V-P shunts. These 5 patients required IV ventricular shunting. All patients improve postoperatively except one patient who developed 6th nerve palsy related to secondary irritation of the brainstem by the IV ventricular catheter. Inflammatory changes in the ependyma of both aqueduct of Sylvius, foramina Luschka and Magendi have been regarded as the most important factors in the development of the isolation of IV ventricle, especially in adults. It is generally recommended to shunt in cases of the adult symptomatic isolated IV ventricle. Alternative surgical techniques and prevention of such complications are discussed.
Abducens Nerve Diseases ; Abscess ; Adult* ; Brain Stem ; Catheters ; Cerebral Aqueduct ; Diagnosis* ; Ependyma ; Humans ; Hydrocephalus* ; Neurocysticercosis ; Teratoma ; Tuberculosis, Meningeal

BACKGROUND AND PURPOSE: The oculomotor nerve fascicles arise along its entire length and sweep ventrally to exit the midbrain at the medial edge of the crus cerebri. A rostro-caudal topography among the fascicular fibers is relatively well established. There are, however, some controversies whether medio-lateral topography also exists. METHODS: We retrospectively reviewed the clinical records and MRI of the 8 patients showing isolated oculomotor nerve palsy due to midbrain infarction. Brain MRI was performed using a 1.5-T magnet with 2mm thickness and 0.1 mm slice interval. The anterior-posterior axis(X) was defined as the midline crossing the center of the cerebral aqueduct and the medio-lateral axis(Y) as the line crossing the same point. For rostro-caudal measurement, the intercommissural line was used as base line of the Z axis. The location of the lesions was defined by measuring actual distance of the margins of the lesions in millimeter from each axis; anterior, right, and caudal direction was defined as positive values in X, Y and Z coordinates, respectively. RESULTS: The mean values and range of the X, Y and Z are as follows: X=7.56+/-4.34, 1< or =X< or =15; Y=3.43+/-1.37, 0< or =Y< or =6; Z=6.51+/-3.91, 0< or =Z< or =12.5. CONCLUSIONS: The distribution of all the MRI lesions was 0< or =|Y|< or =6 (mm), 0< or =|Z|< or =12.5 (mm) in mediolateral and rostrocaudal direction respectively, which is almost the same as the previously reported divergent range of the oculomotor fascicles in midbrain tegmentum. We suggest that our method of three dimensional measurements of the MRI lesion in midbrain tegmentum could be a useful tool for the study of oculomotor fascicular arrangement.
Axis, Cervical Vertebra ; Brain ; Cerebral Aqueduct ; Humans ; Infarction ; Magnets ; Mesencephalon ; Oculomotor Nerve ; Oculomotor Nerve Diseases ; Retrospective Studies

The sylvian aqueduct syndrome is a global rostral midbrain dysfunction induced by a transtentorial pressure gradient through the aquaeductus. Several months after ventriculoperitoneal shunt, a patient with hydrocephalus began experiencing a constellation of midbrain dysfunction symptoms, including bradykinesia, medial longitudinal fasciculus syndrome, third nerve palsy, and mutism. These were indicative of cerebral aqueduct syndrome. In addition, the patient showed posture-dependent underdrainage or overdrainage. All symptoms were resolved after distal catheter elongation and floating cranioplasty. We present a case of reversible parkinsonism, which developed in a patient with shunted hydrocephalus and aqueductal stenosis, and discuss the diagnosis and treatment of the sylvian aqueduct syndrome. We also review the literature to address problems of drainage and potential treatment modalities.
Catheters* ; Cerebral Aqueduct ; Diagnosis ; Drainage ; Humans ; Hydrocephalus ; Hypokinesia ; Mesencephalon ; Mutism ; Oculomotor Nerve Diseases ; Parkinsonian Disorders* ; Ventriculoperitoneal Shunt

OBJECTIVE: To evaluate whether the results of cerebrospinal fluid (CSF) flow quantification differ according to the anatomical location of the cerebral aqueduct that is used and the background baseline region that is selected. MATERIALS AND METHODS: The CSF hydrodynamics of eleven healthy volunteers (mean age = 29.6 years) were investigated on a 1.5T MRI system. Velocity maps were acquired perpendicular to the cerebral aqueduct at three different anatomical levels: the inlet, ampulla and pars posterior. The pulse sequence was a prospectively triggered cardiac-gated flow compensated gradient-echo technique. Region-of-interest (ROI) analysis was performed for the CSF hydrodynamics, including the peak systolic velocity and mean flow on the phase images. The selection of the background baseline regions was done based on measurements made in two different areas, namely the anterior midbrain and temporal lobe, for 10 subjects. RESULTS: The mean peak systolic velocities showed a tendency to increase from the superior to the inferior aqueduct, irrespective of the background baseline region, with the range being from 3.30 cm/sec to 4.08 cm/sec. However, these differences were not statistically significant. In the case of the mean flow, the highest mean value was observed at the mid-portion of the ampulla (0.03 cm3/sec) in conjunction with the baseline ROI at the anterior midbrain. However, no other differences were observed among the mean flows according to the location of the cerebral aqueduct or the baseline ROI. CONCLUSION: We obtained a set of reference data of the CSF peak velocity and mean flow through the cerebral aqueduct in young healthy volunteers. Although the peak systolic velocity and mean flow of the CSF differed somewhat according to the level of the cerebral aqueduct at which the measurement was made, this difference was not statistically significant.
Adult ; Cerebral Aqueduct/anatomy & histology/*physiology ; Cerebrospinal Fluid/*physiology ; Female ; Human ; *Magnetic Resonance Imaging, Cine ; Male ; Reference Values ; Rheology