Angioimmunoblastic T-cell lymphoma harboring a t(8;14)(q24;q11.2)/TCR::MYC translocation that presented with intestinal infiltration.

Although rearrangement of the MYC oncogene (MYC-R) is frequently observed in aggressive B-cell lymphomas, it is extremely rare in T-cell malignancies. A 64-year-old man who had been under observation for several years because of asymptomatic pulmonary extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALToma) was admitted to our hospital because of poor general condition and hypotension. Blood tests revealed thrombocytopenia and elevated serum lactate dehydrogenase levels, whereas computed tomography revealed systemic lymphadenopathy and splenomegaly.

Successful Allogeneic Hematopoietic Stem Cell Transplantation for Nodal Epstein-Barr Virus-positive T/NK-cell Lymphoma.

Nodal Epstein-Barr virus-positive T/NK-cell lymphoma (EB-nTNKL) is an extremely rare disease characterized by an aggressive clinical course and poor prognosis, for which treatment strategies have not yet been established. We herein report a young man with EB-nTNKL. Although initial chemotherapies, including L-asparaginase, failed to produce a good response, subsequent myeloablative allogeneic hematopoietic stem cell transplantation (alloHSCT) resulted in favorable disease control and a long-term disease-free survival.

Successful Cord Blood Transplantation for Myeloid/Natural Killer Precursor Acute Leukemia: A Case Report and Literature Review.

A 21-year-old man was diagnosed with myeloid/natural killer precursor leukemia (MNKPL) with bone marrow infiltration of blasts of cyCD3, CD7, CD33, CD34, CD56, HLA-DR, cyMPO, and TdT immunophenotypes. Although hyper-CVAD therapy was unsuccessful, induction treatment with idarubicin and cytarabine resulted in complete remission (CR). The patient subsequently underwent cord blood transplantation with a myeloablative conditioning regimen, which resulted in durable CR and complete donor chimerism.

Brainstem Surgery: Functional Surgical Anatomy with the Use of an Advanced Modern Intraoperative Neurophysiological Procedure.

Intraoperative neurophysiology (ION) in brainstem surgery evolved as brainstem surgery advanced.The original idea of brainstem mapping (BSM) is a neurophysiological procedure to locate cranial nerve motor nuclei (CNMN) on the floor of the fourth ventricle. With the introduction of various skull base approaches to the brainstem, BSM is carried out on any surface of the brainstem to expose the safe entry zone to the intrinsic brainstem lesion.

Surgery for spina bifida occulta: spinal lipoma and tethered spinal cord.

The technical evolution of the surgery for spina bifida occulta (SBO) over the course of a half-century was reviewed with special foci placed on the spinal lipoma and tethered spinal cord. Looking back through history, SBO had been included in spina bifida (SB). Since the first surgery for spinal lipoma in the mid-nineteenth century, SBO has come to be recognized as an independent pathology in the early twentieth century.

Ventral anchoring of the conus medullaris: a new surgical technique preventing the radiographic recurrence of retethering after surgery for tethered spinal cord.

Objective: Retethering is not an unusual operation for a congenital lumbosacral dysraphic spinal lesion. The present study aimed to assess a new surgical technique for preventing retethering.

Surgical Technique: After untethering the spinal cord, the pia mater or scar tissue at the caudal end of the conus medullaris is anchored to the ventral dura mater loosely using 8-0 thread, and the dura mater is closed directly.

A Case of Synchronous Occurrence of Intracranial Germinoma and Systemic Sarcoidosis.

Although the synchronous occurrence of testicular seminoma and systemic sarcoidosis has been reported, that of intracranial germinoma and systemic sarcoidosis is unknown. A 26-year-old man presented with symptoms of panhypopituitarism and consciousness disturbance. Imaging demonstrated a large nodule in the upper right lung field and swelling of multiple bilateral pulmonary and mediastinal lymph nodes in addition to the bifocal pineal and suprasellar tumors with obstructive hydrocephalus.

Deleterious fibronectin type III-related gene variants may induce a spinal extradural arachnoid cyst: an exome sequencing study of identical twin cases.

Purpose: Despite numerous studies, the etiology of spinal extradural arachnoid cyst (SEDAC), a lesion associated with neurological symptoms, remains unknown. In this genomic twin study, we investigated the genetic etiology of SEDACs.

Methods: The subjects were identical twins who developed notably similar SEDACs at the same vertebral level.

Basal encephalocele: surgical strategy and functional outcomes in the Tokyo experience.

Objective: The basal encephalocele (BEC) is the rarest form of encephalocele, with an incidence of about 1/35,000 live births. The incidence of its subtype, sphenoidal BEC, is even lower at about 1/700,000 live births. The aim of this study was to propose the optimal surgical approach to repairing BEC, with special attention to the reconstruction of the skull base bone defect.

Voxel-Based Morphometry Reveals a Correlation Between Bone Mineral Density Loss and Reduced Cortical Gray Matter Volume in Alzheimer's Disease.

: Decreased bone mineral density (BMD) was associated with poorer cognitive function and increased risk of Alzheimer's disease (AD). However, objective evidence for the relationship between osteoporosis and AD in humans has not been extensively described. : We aimed to evaluate the relationships between BMD and the cortical volumes of brain regions vulnerable to AD; hippocampus, parahippocampal gyrus, precuneus, posterior cingulate, and angular gyrus, using voxel-based morphometry (VBM), to investigate the association between bone loss and AD.

Fronto-orbital advancement in a patient with Marshall-Smith syndrome: a case report and review of the literature.

Objective: The present report aimed to document the clinical features of a case of Marshall-Smith syndrome (MSS), an extremely rare embryonic developmental disorder with associated craniosynostosis.

Patient And Method: We presented herein a case of a 2-year-old female patient with MSS who underwent fronto-orbital advancement for multisuture craniosynostosis.

Results: The patient's proptosis improved after surgery, and no further surgical intervention was required for corneal exposure.

Pudendal mapping of S1 rootlets in a functional posterior rhizotomy: when an S1 posterior root shows a high pudendal dorsal action potential-a technical note.

Background: The standard level for lesioning in a functional posterior rhizotomy (FPR) ranges from L2 to S1/S2. Lesioning of the S1 and S2 rootlets strongly correlates with a reduction in ankle spasticity. In the Japanese population, the S2 root often shows the highest dorsal root action potentials (DAPs) in the afferent fibers of the pudendal nerve and is not lesioned to preserve postoperative urinary function.

Characteristics of Recurrent Congenital Sinus Pericranii: Case Report and Review of the Literature.

Introduction: Sinus pericranii (SP) involves transosseous vessels that connect the intra- and extracranial venous systems. Accessory-type SP can be cured by surgical or endovascular treatment. Reports of recurrence are, however, rare.

Real prevalence of neural tube defects in Japan: How many of such pregnancies have been terminated?

The vital role of folic acid is to reduce the risk of having a neonate afflicted with neural tube defects. The prevalence of neural tube defects (myelomeningocele and anencephaly) has been reported in an incomplete form over the last 40 years in Japan. We aimed to evaluate the total number of neural tube defects including those delivered or terminated, to clarify the proportion of those terminated, and to internationally compare their prevalence.

Intraoperative neurophysiological monitoring of the bulbocavernosus reflex during surgery for conus spinal lipoma: what are the warning criteria?

Objective: Despite the surge in the intraoperative use of the bulbocavernosus reflex (BCR) during lumbosacral surgeries, there are as yet no widely accepted BCR warning criteria for use with intraoperative neurophysiological monitoring (IONM). The author's aim was to find clinically acceptable warning criteria for use in IONM of the BCR.

Methods: Records of IONM of the BCR in 164 operations in 163 patients (median age 5 months) with a conus spinal lipoma who underwent surgery between August 2002 and May 2016 were retrospectively analyzed.

Clinically practical formula for preoperatively estimating the cutting rate of the spinal nerve root in a functional posterior rhizotomy.

Objective: A functional posterior rhizotomy (FPR) ideally involves minimal cutting of the posterior root while providing maximal reduction of disabling spasticity. However, the ideal cutting rate has yet to be determined. It was hypothesized that the cutting rate of the posterior root would negatively correlate with preoperative motor function in children with spasticity.

Surgical management of cervical spine deformity in chondrodysplasia punctata.

OBJECTIVE Chondrodysplasia punctata (CDP), a rare skeletal dysplasia, can lead to cervical spine instability and deformity. However, an optimal neurosurgical intervention has yet to be established. Thus, a retrospective study was conducted to assess the efficacy of various surgical interventions for children with CDP.

Pediatric Craniovertebral Junction Surgery.

The craniovertebral junction (CVJ) has attracted more attention in pediatric medicine in recent years due to the progress in surgical technologies allowing a direct approach to the CVJ in children. The CVJ is the site of numerous pathologies, most originating in bone anomalies resulting from abnormal CVJ development. Before discussing the surgical approaches to CVJ, three points should be borne in mind: first, that developmental anatomy demonstrates age-dependent mechanisms and the pathophysiology of pediatric CVJ anomalies; second, that CT-based dynamic simulations have improved our knowledge of functional anatomy, enabling us to locate CVJ lesions with greater certainty; and third, understanding the complex structure of the pediatric CVJ also clarifies the surgical anatomy.

Impact of magnetic resonance imaging and urodynamic studies on the management of sacrococcygeal dimples.

OBJECTIVE Sacrococcygeal dimples in neonates and infants are of uncertain pathological import. Previously they were believed to be rarely associated with intraspinal anomalies. Recent studies using MRI, however, revealed that 6%-7% of pediatric cases of sacrococcygeal dimples were associated with anatomical tethered spinal cord (TSC).

Neural tube defects: Risk factors and preventive measures.

For the last 25 years, it has been proven that the occurrence or recurrence of neural tube defects can be prevented with the administration of folic acid before and early pregnancy. At present, over 80 countries in the world, except Japan, have mandated the fortification of wheat flour and/or rice with folic acid, which has resulted in a significant reduction in the prevalence of neural tube defects. In 2000, the Japanese government recommended folic acid 400 μg daily for young women of childbearing age and women who are planning to conceive.

New classification of spinal lipomas based on embryonic stage.

OBJECTIVE Spinal lipomas are generally thought to occur as a result of failed primary neurulation. However, some clinical features cannot be explained by this theory. The authors propose a novel classification of spinal lipomas based on embryonic changes seen during primary and secondary neurulation.