Search

Background: We report a rare case of split cord malformation., Case Description: A female neonate presented with split cord malformation (SCM) manifesting as right lower limb paresis. Myelomeningocele and meningocele were found in the lumbosacral region at birth. Magnetic resonance imaging (MRI) demonstrated division of the spinal cord into two hemicords below the T5 level. The right hemicord formed a hemimyelomeningocele and the left hemicord terminated in the low-lying conus. Three-dimensional computed tomography (3D CT) showed extensive vertebral body abnormalities. The hemimyelomeningocele was repaired 1 day after birth, and septectomy and the repair of the meningocele were performed when the patient was 1 year old. The patient has been followed up as an outpatient, and has residual right lower limb paresis., Conclusion: SCM can be associated with multiple spinal abnormalities. MRI and 3D-CT are useful for identifying such abnormalities and planning the surgical treatment.

Turcot's syndrome is a combination of multiple adenomatous gastrointestinal polyps and neuroepithelial tumors of the central nervous system (CNS). A 16-year-old man presented with Turcot's syndrome consisting of nonfamilial colorectal polyposis and multiple fibrillary astrocytomas accompanied by ruptured intracranial arteriovenous malformation (AVM) manifesting as generalized epileptic activity. Multiple CNS tumors are extremely unusual in Turcot's syndrome. This is the first description of Turcot's syndrome associated with an intracranial AVM. This case may correspond to a new phenotypic variant of colonic polyposis syndrome.

Background: Central benzodiazepine (BZD) receptor imaging is effective to evaluate neuron density in the pathological brain cortex. This study used BZD receptor imaging to evaluate neuronal damage in adult ischemic moyamoya disease., Methods: Single photon emission computed tomography and a novel tracer, 123I-iomazenil, were used to measure BZD receptor density in the brain. Evaluation of early and late images was performed in three asymptomatic, unoperated patients, and six mildly symptomatic, operated patients., Results: Uptakes in the frontal, parietal, and occipital lobes of symptomatic patients were significantly lower (p<0.05) than those of asymptomatic patients. The late image/ early image count ratios (L/E ratios) of asymptomatic patients were relatively constant (mean, 0.571; range, 0.550-0.581) in all regions of interest. In contrast, the L/E ratios of symptomatic patients were not uniform., Conclusions: The neuron density was preserved in adult asymptomatic patients despite harboring moyamoya disease. In contrast, the neuronal density was decreased in symptomatic patients even though their symptoms were mild and they had undergone revascularization.

A newborn girl was admitted suffering from convulsive seizures in both legs. A computed tomography (CT) scan revealed bilateral symmetrical middle cranial fossa arachnoid cysts and intraventricular hemorrhage. In the course of a year, only the cyst on the right side became gradually larger; the cyst on the left side was reduced in size. At the age of 1 year and 2 months, her intellectual development was normal, but she could not stand by herself. On metrizamide-enhanced CT cisternography, the cyst on the right side was of a noncommunicating type. Membranectomy of the right-sided cyst was performed and the cyst proved to be of the intraarachnoid type. The postoperative course was uneventful and a CT scan, taken 10 months after the operation, revealed no abnormality on either side. The intellectual and motor development was normal, and no abnormality was observed by electroencephalography. This may be the first report of arachnoid cysts found in a neonate. It gives us clues for understanding the etiology of this disease, which has not been fully clarified.