Spinal cord deformity with aggravation of tethering in saccular limited dorsal myeloschisis during the first 2 months of life.

Background: Although the optimal timing of prophylactic untethering surgery for limited dorsal myeloschisis (LDM) with intact or subtle neurological findings diagnosed at birth remains undetermined, intentional delayed surgery is commonly used for flat and tail-like LDM. Conversely, for saccular LDM, early surgery is indicated during the postnatal period because it prevents rupture of the sac. We treated a saccular LDM patient, in whom intentional delayed surgery was selected because the sac was thickly covered with normal skin.

Two Cases of Large Filar Cyst Associated with Terminal Lipoma: Relationship with Retained Medullary Cord.

Background: A small, incidental filar cyst associated with terminal lipoma is thought to be caused by failure of secondary neurulation; however, the precise embryologic background is not fully understood. Retained medullary cord (RMC) also originates from late arrest of secondary neurulation. The central feature of RMC histopathology is a central canal-like ependyma-lined lumen with surrounding neuroglial core.

Retained medullary cord with sacral subcutaneous meningocele and congenital dermal sinus.

Background: A retained medullary cord (RMC) is a rare closed spinal dysraphism with a robust elongated cord-like structure extending continuously from the conus medullaris to the dural cul-de-sac that is caused by late arrest of secondary neurulation. Five patients with RMC extending to an associated sacral subcutaneous meningocele have been reported.

Case Presentation: We report an additional patient with RMC, in whom a congenital dermal sinus (CDS) was found in the caudal portion of the RMC.

A patient with a dysembryoplastic neuroepithelial tumor who underwent epilepsy surgery after initial seizure.

Background: Since dysembryoplastic neuroepithelial tumors (DNTs) are benign tumors that are frequently associated with long-standing medically intractable epilepsy, it is well known that the surgical strategy is resection of the associated epileptogenic zone as well as the tumor. However, the surgical strategy for DNT with a single seizure has not been fully discussed.

Methods: We report an 8-year-old boy with DNT in the nondominant frontal lobe who underwent epilepsy surgery at 3 months after his initial seizure.

[A pregnant case on which EC-IC bypass was successfully performed in the acute phase of brain infarction].

A 30 year-old, 28 weeks-pregnant woman (gravida 2, para 2) suffered from a sudden onset of aphasia when she was having a chat in the upright position. Although the initial symptom soon disappeared, transient attacks of aphasia combined with weakness in the right arm occurred intermittently, particularly when she uprose. MR image disclosed a small acute infarcted focus at the genu of the left internal capsule, while MRA showed an occlusion of the intracranial portion of the left internal carotid artery.

[Case of Gerstmann-Sträussler-Scheinker syndrome (GSS-P102L) mimicking variant Creurtzfeldt-Jakob disease in clinical manifestation and MRI findings].

We reported a 51-year-old woman with Gerstmann-Sträussler-Scheinker syndrome (GSS P102L) manifesting characteristic MRI findings. At the age of 45, She developed gait disturbance with muscle atrophy in the lower limbs and positive plantar flexor sign. Subsequently, sensory disturbance such as refractory pain in the lower limbs and ataxic gait were developed at the age of 49.