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A 50-year-old man was referred to our hospital with myelitis associated with a 10-months history of progressive muscle weakness in the left leg. Neurological examinations demonstrated diffuse muscle weakness of the left leg, touch hypoesthesia of the right leg, reduced pain sensation below the right nipple, left pyramidal sign, and urinary incontinence. On the basis of thoracic spinal MRI and thoracic CT myelography, revealing anterior displacement of the spinal cord and enlargement of the posterior subarachnoid space at the Th4 vertebral level, we diagnosed the patient as having idiopathic spinal cord herniation with incomplete Brown-Séquard syndrome. After microsurgical release of the spinal cord and subsequent covering of the anterior dural defect with an artificial dura mater, the symptoms improved without progression. Clinicians should consider spinal cord herniation as a cause of slowly progressive thoracic myelopathy with Brown-Séquard syndrome.

Background Meningeal melanocytoma is a rare benign lesion found in the central nervous system. Preoperative diagnosis of meningeal melanocytoma is often a diagnostic challenge, as the clinical and neurologic features are often nonspecific. Various characteristics, including the natural course of this tumor, remain poorly understood. We report a case of a rapidly growing dumbbell-shaped melanocytoma compressing the spinal cord that manifested 2 years after a tumor was identified at the right C2-C3 foramen. Case Description A 40-year-old, right-handed man presented with a 2-month history of right palm and left leg numbness. Magnetic resonance imaging of the cervical spine showed a dumbbell-shaped tumor at the right C2-C3 foramen with extension into the central canal. The lesion was hyperintense on T1-weighted images and hypointense to isointense on T2-weighted images. Contrast enhancement was not visualized clearly. Fluorodeoxyglucose–positron emission tomography with computed tomography showed intense uptake in the lesion. The patient's history included a small lesion that had been localized at the right C2-C3 foramen 2 years before admission. The pathologic findings were consistent with melanocytoma. Conclusions It is important to include meningeal melanocytoma in the differential diagnosis of dumbbell tumors, as meningeal melanocytomas may show rapid progression.

AIM To determine the prevalence of a deep vein thrombosis (DVT) in osteoporotic vertebral fractures. MATERIAL AND METHODS Data were retrospectively collected from the medical records of 50 patients who were admitted to the Kameda Medical Center for osteoporotic vertebral fracture from 2019 to 2020. Inpatients were screened for DVT using D-dimer, and those who were screened positive underwent lower extremity venous ultrasonography to confirm DVT. Associations between various clinical factors and DVT were analyzed. RESULTS Six (12.0%) inpatients with osteoporotic vertebral fractures were found to have DVT. Two (33.3%) of the six had proximal DVT, although no pulmonary embolism was detected by chest computed tomography angiography. Univariate analysis showed that D-dimer values and duration from onset to hospitalization were predictive of DVT (p < 0.05). CONCLUSION The prevalence of DVT among inpatients with osteoporotic vertebral fractures was 12.0%. This finding emphasizes the importance of DVT screening using D-dimer in those with osteoporotic vertebral fractures.

Background Ganglioneuroma is a well-differentiated benign tumor that develops from the ganglion cells of the posterior mediastinum, retroperitoneum, cervical spine, and adrenal glands. The paravertebral body, in which the sympathetic trunk exists, is a common tumor site, and tumor sometimes invades the spinal canal through the intervertebral foramen. There have been no reports regarding tumors with intradural and extradural continuity. We report a paravertebral ganglioneuroma extending between the intradural and extradural spaces and its surgical treatment. Case Description A 33-year-old man was admitted to the hospital with progressive left lower limb numbness. A dumbbell-type tumor progressing to the spinal canal via the left intervertebral foramen from the paravertebral body at L1-2 was detected, and intradural calcified lesions were found. Pathologic examination of a computed tomography–guided biopsy sample revealed a ganglioglioma. The extradural tumor was removed; however, the left lower limb pain gradually worsened. As complete block was observed on myelography, the intradural tumor was removed 8 months later. Intraoperative findings revealed that the intradural and extradural tumors were continuous through the L1 nerve root. Conclusions This is the first known reported case of paravertebral ganglioneuroma presenting in continuity between the intradural and extradural spaces.

It is difficult to treat atlantoaxial instability in patients with a high-riding vertebral artery or anomalies of the craniocervical junction. We report 2 successful cases in which the transspinal canal screwing technique was used because of difficulties performing conventional fixation methods. Case 1: A 78-year-old woman suffered from progressive myelopathy due to severe spinal cord compression with a congenital anomaly of the craniovertebral junction. Bilateral transspinal canal screws from the axis body with spondylolisthesis to the dens were inserted by retracting the dural sac medially after foramen magnum decompression and cervical laminoplasty. Case 2: A 20-year-old man with a spinal deformity due to Loeys-Dietz syndrome presented to our hospital for treatment of syringomyelia. He had no obvious neurological deficits, but spinal cord compression due to right atlantoaxial rotating dislocation was observed. A screw was inserted from the vertebral body of the axis to the right lateral mass of the atlas via the spinal canal after laminectomy of the atlas. The transspinal canal screwing technique is useful for treating atlantoaxial instability in cases where other fixation methods are difficult.

Background: Normal pressure hydrocephalus (NPH) associated with tumors of the cauda equina is rare. Here, we report two cases of NPH attributed to cauda equina ependymomas. Case Description: A 63-year-old male presented with progressive gait disturbance, dementia, and urinary incontinence. When the lumbar MR documented an intradural tumor involving the cauda equina at the L2-L3 level; the tumor was excised; pathologically, it proved to be a myxopapillary ependymoma. Postoperatively, however, the patient’s continued gait disturbance led to a brain CT that documented ventricular dilation consistent with NPH; following ventriculoperitoneal (VP) shunt placement his symptoms improved. A 65-year-old female also presented with gait disturbance, dementia, and urinary retention. Here, procedures were performed in reverse. When a brain CT showed hydrocephalus, a VP shunt was placed. When symptoms persisted, a lumbar MR demonstrated a T12-L2 intradural tumor; following a lumbar laminectomy for tumor excision, symptoms stabilized. The pathological diagnosis was also consistent with a conus/cauda equina ependymoma. Over the next 10 years, the patient had residual bladder dysfunction (e.g., requiring straight catheterization), but had no shunt dysfunction. Conclusion: We observed two cases of ependymomas of the cauda equina and brain CTs documenting NPH that was successfully surgically managed with stabilization of neurological deficit. In the first case, L2-L3 laminectomy for tumor removal was succeeded by shunting for NPH, while in the second case, initial VP shunting for NPH was followed by a T12-L2 laminectomy for tumor excision.

To analyze the clinical characteristics of thoracic ossified ligamentum flavum (OLF) and clarify prognostic factors.We retrospectively collected clinical data from the medical records of 29 patients who underwent decompressive surgery for myelopathy caused by thoracic OLF from 2011 to 2019. Associations between various clinical and radiological factors and surgical outcomes were analyzed.The mean preoperative and final follow-up modified Japanese Orthopaedic Association scores were 6.7 and 7.9 points, respectively (mean follow-up period, 22.4 months; P 0.0001). Univariate analysis revealed that preoperative symptom duration and postoperative epidural hematoma on magnetic resonance imaging (MRI) were significant surgical outcome predictors. The recovery rate was higher in patients without postoperative epidural hematoma (n = 16) than in those with postoperative epidural hematoma (n = 13; 38.7% ± 28.1% vs. 12.9% ± 24.4% mm; P = 0.01). Multiple regression analysis showed that postoperative epidural hematoma on MRI was a significant surgical outcome predictor (P 0.05).Posterior decompressive laminectomy and resection of OLF can be considered effective. Postoperative epidural hematoma on MRI may be an important surgical outcome predictor.

Tumoral calcinosis involving the spine is rare. The involvement of the thoracic spine is rarer than that of the cervical or lumbar spine. We report a case of thoracic tumoral calcinosis accompanied by vertebral fracture with increased concentrations of inflammatory markers and no abnormalities in serum calcinosis and phosphorus concentration. A 60-year-old woman presented with complete paraplegia. Her white blood cell count and C-reactive protein (CRP) concentration were elevated. The thoracic magnetic resonance imaging revealed vertebral fracture and an epidural mass that demonstrated low intensity on both T2- and T1-weighted images at the T9/10 dorsal side of the central canal. This lesion is larger in size than that observed in the previous 2 months. Her laboratory data showed signs of infection, and only decompression surgery without fixation for treatment and diagnosis was performed. Histopathological examination was consistent with tumoral calcinosis. Postoperatively, the patient’s white cell count and CRP concentration were normalized. We found that tumoral calcinosis can occur at the thoracic level on the basis of the spinal instability due to the vertebral compression fracture and the accompanying increase in inflammation indicated by increased white blood cell count and CRP concentration.

Spinal deformity is a characteristic feature of Loeys-Dietz syndrome (LDS). Surgical correction in LDS is indicated when the deformity is progressive to avoid neurological deficits, respiratory impairment, and back pain. However, few reports exist on the surgical treatment of spinal deformity in LDS, and no therapeutic standards have been established. We described the clinical and radiological outcomes of a patient with LDS receiving surgery for severe kyphoscoliosis. A 21-year-old male patient with LDS underwent posterior spinal fusion with an all-pedicle screw construct from T10 to L5 for a preoperative main curve Cobb angle of 70 degrees and kyphotic angle of 49 degrees. The postoperative Cobb angle of the main curve and kyphotic angle improved to 36 and 8 degrees, respectively. Correction surgery was performed with frequent motor evoked potential testing, taking care not to cause motor paralysis. Ameliorated low back pain and improvements in clinical questionnaire scores were noted at 21 months after surgery. No perioperative complications were reported. Based on the present case, posterior spinal fusion represents a good correction option for severe spinal deformity in LDS with syringomyelia. Careful preoperative examination and treatment for neurovascular and neurological lesions is advised to prevent severe complications.

Spinal subdural abscesses are rare lesions. We report the case of surgical site infection complicated with meningitis and rapidly progressive spinal subdural abscess caused by P. aeruginosa following transforaminal lumbar interbody fusion (TLIF). A 72-year-old woman was admitted to our hospital complaining of drop foot syndrome and sciatica caused by stenosis of the L5/6 intervertebral foramen accompanied by L5 lumbar vertebral fracture. Accordingly, TLIF of L5-L6 and balloon kyphoplasty of L5 were performed. On the 3rd postoperative day (POD), she was diagnosed with surgical site infection complicated with bacterial meningitis. Subcutaneous fluid, blood, and cerebrospinal fluid cultures indicated P. aeruginosa. On the 7th POD, a repeat MRI showed a large dorsal fluid collection consistent with a subdural infection and massive cauda equina compression. We performed debridement and instrument removal and found a dural laceration that was not observed during the first operation. An intraoperative insensible dural laceration may cause bacteria intrusion into the subdural space.

Febrile infection-related epilepsy syndrome (FIRES), or acute encephalitis with refractory, repetitive partial seizures (AERRPS), is an epileptic encephalopathy beginning with fever-mediated seizures. The etiology remains unclear. To elucidate the genetic background of FIRES/AERRPS (hereafter FIRES), we recruited 19 Japanese patients, genotyped polymorphisms of the IL1B , IL6 , IL10 , TNFA , IL1RN , SCN1A and SCN2A genes, and compared their frequency between the patients and controls. For IL1RN , the frequency of a variable number of tandem repeat (VNTR) allele, RN2, was significantly higher in the patients than in controls ( p =0.0067), and A allele at rs4251981 in 5′ upstream of IL1RN with borderline significance (p =0.015). Haplotype containing RN2 was associated with an increased risk of FIRES (OR 3.88, 95%CI 1.40–10.8, p =0.0057). For SCN1A , no polymorphisms showed a significant association, whereas a missense mutation, R1575C, was found in two patients. For SCN2A , the minor allele frequency of G allele at rs1864885 was higher in patients with borderline significance ( p =0.011). We demonstrated the association of IL1RN haplotype containing RN2 with FIRES, and showed a possible association of IL1RN rs4251981 G>A and SCN2A rs1864885 A>G, in Japanese patients. These preliminary findings suggest the involvement of multiple genetic factors in FIRES, which needs to be confirmed by future studies in a larger number of FIRES cases.

Objective: To investigate the clinical and epidemiologic features of pediatric acquired demyelinating syndromes (ADS) of the CNS in Japan. Methods: We conducted a nationwide survey and collected clinical data on children with ADS aged 15 years or younger, who visited hospitals between 2005 and 2007. Results: Among 977 hospitals enrolled, 723 (74.0%) responded to our inquiries and reported a total of 439 patients as follows: 244 with acute disseminated encephalomyelitis (ADEM), 117 with multiple sclerosis (MS), 14 with neuromyelitis optica (NMO), and 64 with other ADS. We collected and analyzed detailed data from 204 cases, including those with ADEM (66), MS (58), and NMO (10). We observed the following: (1) the estimated annual incidence rate of pediatric ADEM in Japan was 0.40 per 100,000 children (95% confidence interval [CI], 0.34–0.46), with the lowest prevalence in the north; (2) the estimated prevalence rate of MS was 0.69 per 100,000 children (95% CI, 0.58–0.80), with the lowest prevalence in the south; (3) NMO in Japan was rare, with an estimated prevalence of 0.06 per 100,000 children (95% CI, 0.04–0.08); and (4) the sex ratio and mean age at onset varied by ADS type, and (5) male/female ratios correlated with ages at onset in each ADS group. Conclusions: Our results clarify the characteristic clinical features of pediatric ADS in the Japanese population.

Background context Neuroendocrine tumors (NETs) from tailgut cysts are rare; only 15 cases have been reported until now. A tailgut cyst with spinal cord tethering has not been previously reported, although both diseases are congenital anomalies in the early stage of gestation. Purpose To report a rare case of NET from tailgut cyst associated with spinal cord tethering and review the literature. Study design Case report and literature review. Methods We describe the clinical course of a 53-year-old man, who presented with gluteal pain and bladder dysfunction. Magnetic resonance images showed that a tumor of the sacral spinal canal extended into the retrorectal space and connected to a thickened fatty filum terminale, which was tethering the spinal cord. Results Because of tumor malignancy on a computed tomography-guided biopsy and the imaging data of involvement of presacral lymph nodes, we performed total removal of the tumor. Pathologic examination revealed NET (Grade 2) arising from a tailgut cyst. The patient received somatostatin analog therapy after surgery, followed by local radiation because of the further enlargement of the lymph nodes. Later, we started everolimus therapy for the metastases to the retroperitoneal lymph nodes. He presented with no local recurrence or further disease progression at 28 months after surgery. The review indicated that tumors in Grade 2 or 3 showed progressive clinical course after surgery and three of seven patients with biopsy were misdiagnosed. Conclusions The correct preoperative diagnosis of NETs from tailgut cysts is difficult, but extremely important because Grade 2 or 3 tumors show disease progression even after surgery. Presacral congenital tumors, such as tailgut cysts, have the potential of malignant transformation into neuroendcrine tumors or adenocarcinomas. Comorbidity of spinal cord tethering and tailgut cyst suggests some relationship to common developmental errors in embryogenesis.

Acute necrotizing encephalopathy (ANE) is a rare and severe syndrome of acute encephalopathy triggered by viral infections. Cytokine storm is considered as the main pathogenetic mechanism of ANE. ANE is prevalent in East Asia, suggesting the association of host genetic factors. To elucidate the genetic background of Japanese ANE, we examined genotypes of human leukocyte antigen (HLA)-A, C, B, DRB1, DQB1 and DPB1 in 31 patients. Significant positive association was observed in both the allele frequency and positivity of DRB1*09:01 (P=0.043 and 0.025, respectively), as well as those of DQB1*03:03 (P=0.034 and 0.026, respectively). The carrier frequency of DRB1*09:01 and DQB1*03:03 alleles was higher in the patients (45.16%) than in controls (28.57%). These alleles are more common in East Asian than in European populations, and are reportedly associated with various autoimmune diseases in Japanese patients. Our data provide further evidence that altered immune response based on individual HLA genotypes may contribute to ANE pathogenesis.

Four lysyl oxidase family genes (LOXL1, LOXL2, LOXL3, and LOXL4), which catalyze cross-linking of collagen and elastin, were considered to be functional candidates for intracranial aneurysms (IA) and were extensively screened for genetic susceptibility in Japanese IA patients. Total RNA was isolated from four paired ruptured IA and superficial temporal artery (STA) tissue and examined by real-time RT-PCR. The expression of LOXL2 in the paired IA and STA tissues was elevated in the IA tissue. A total of 55 single nucleotide polymorphisms (SNPs) of LOXL1-4 were genotyped for an allelic association study in 402 Japanese IA patients and 462 Japanese non-IA controls. Allelic associations were evaluated with the chi-square test and the permutation test especially designed for adjustment of multiple testing. SNPs of LOXL1 and LOXL4 were not significantly associated with IA, while several SNPs of LOXL2 and LOXL3 showed nominally significant associations in IA patients. We detected an empirically significant association with one SNP of LOXL2 in familial IA patients after adjustment for multiple testing [chi(2) = 10.23, empirical P = 0.023, OR (95% CI) = 1.49 (1.17, 1.90)]. Furthermore, multilocus interaction was evaluated by multifactor dimensionality reduction analysis. We found that the SNPs of LOXL2 have an interactive effect with elastin (ELN) and LIM kinase 1 (LIMK1) that have been previously found to be associated with IA. In conclusion, one SNP of LOXL2 showed a significant association with IA individually, and we also detected a gene-gene interaction of LOXL2 with ELN/LIMK1, which may play an important role in susceptibility to IA.

The rupture of an intracranial aneurysm (IA) results in subarachnoid hemorrhage, a catastrophic neurological condition with high morbidity and mortality. Following-up on our previous genome-wide linkage study in Japanese population, we extensively analyzed a 4.6 Mb linkage region around D7S2472 on 7q11 by genotyping 168 single nucleotide polymorphisms (SNPs). SNP association and window scan haplotype-based association studies revealed a susceptibility locus for IA on a single LD block covering the 3'-untranslated region (3'-UTR) of ELN and the entire region of LIMK1. An association study with 404 IA patients and 458 non-IA controls revealed that the ELN 3'-UTR G(+659)C SNP has the strongest association to IA (P=0.000002) and constitutes a tag-SNP for an at-risk haplotype, which contains two functional SNPs, the ELN 3'-UTR (+502) A insertion and the LIMK1 promoter C(-187)T SNP. These allelic and haplotype-based associations were confirmed in a Korean population. Ex vivo and in vitro analyses demonstrate that the functional impact of both SNPs is the decrease of transcript levels, either through accelerated ELN mRNA degradation or through decreased LIMK1 promoter activity. Elastin and LIMK1 protein are involved in the same actin depolymerization signaling pathway; therefore, these lines of evidence suggest a combined effect of the SNPs in the at-risk haplotype possibly by weakening the vascular wall and promoting the development of IA.

MRI of large pituitary adenomas has revealed that a posterior pituitary bright spot (PPBS), comprising ADH-containing neurosecretory granules, is commonly ectopic before surgery and attached to the tip of the pituitary stalk late after surgery. Although the PPBS indicates functional integrity of the posterior lobe, transient diabetes insipidus (DI), caused by deficiency of ADH, is frequent early after surgery. We attempted to clarify how the shape, signal intensity and site of the PPBS before surgery are related to transient DI in the early postoperative period. We carried out MRI on 15 patients with a large adenoma and an ectopic PPBS before surgery and then within 1 week (early), 1-2 months (intermediate) and 6 or more months (late) after the operation. There were nine who had transient DI, which subsided by the intermediate study; none had permanent DI. Regardless of transient DI, the PPBS was visible, and its signal intensity was similar, on all postoperative studies. Although 11 did not change in shape, four showed a remarkable change from a flat shape before surgery to a rounded one postoperatively. On the intermediate MRI, the PPBS had descended to the level of the diaphragma as mass effect disappeared.

To evaluate the disappearance or relocation patterns of posterior pituitary bright spot (PPBS) in the infundibuloneurohypophyseal (INH) system and endocrinologic implications in large pituitary adenomas.Sixty-nine patients with adenoma and supradiaphragmatic extension were classified into PPBS-visible and PPBS-nonvisible groups on the basis of findings at preoperative T1-weighted magnetic resonance (MR) imaging. The adenoma shapes were classified into hourglass type with indentation and barrel type without indentation at the diaphragmatic level.The PPBS-visible group included 55 (80%) patients. PPBS most commonly occurred at the distal pituitary stalk immediately above the diaphragm in 48 patients with hourglass-type adenoma. In the remaining seven patients with barrel-type adenoma, PPBS occurred in the sella or in varying sites along the pituitary stalk. Postoperatively, two patients, whose PPBS became nonvisible, developed persistent diabetes insipidus. The PPBS-nonvisible group included 14 (20%) patients. Five had hourglass-type and nine had barrel-type adenoma. Occurrence of the barrel type was marked. In these patients, four developed postoperative permanent diabetes insipidus.The diaphragm, a probable major anatomic determinant of indentation, may serve as a transportation blockade and facilitate proximal accumulation of PPBS material, as evidenced in the hourglass-type adenoma. PPBS was more commonly nonnvisible in the barrel-type adenoma. The presence of PPBS in the INH system indicates its functional maintenance in large adenomas.

Objective. Location of anterior optic pathways in sellar and parasellar tumours was preoperatively evaluated, by use of heavily T2 weighted MR images. Methods. Heavily T2 and conventional T1 weighted images were studied in 20 patients with sellar and parasellar tumours who underwent craniotomy. Pathology revealed pituitary adenoma in 5 patients, craniopharyngioma in 8 and parasellar meningioma in 7. Maximum sizes ranged from 15 mm to 58 mm. Sequence parameters of TR/TE for heavily T2 weighted and T1 weighted images were 5800/220 msec and 600/20 msec, respectively, and slice thickness was 3 mm for both. Results. The anterior optic pathway was detected in 95% on heavily T2 weighted images and 50% on T1 weighted images. All preoperative heavily T2 weighted images were compatible with operative findings. The optic chiasms were most commonly supero-posterior in pituitary adenomas, anterior (prefixed) in craniopharyngiomas and posterior in meningiomas. The optic nerves were commonly located superior or lateral to the tumours. However, parasellar meningiomas, off the midline, revealed the optic nerves in various locations, depending on the tumour origin. In such tumours, heavily T2 weighted images provided surgical information on the width of the working space through prechiasmal and/or optico-carotid spaces in the pterional approach. Spatial relation of the tumours to the lamina terminalis, anterior commissure and anterior communicating artery complex was clearly shown in craniopharyngioma patients, who underwent the anterior interhemispheric approach. Conclusion. Heavily T2 weighted MR images are useful in determining the location of optic pathways and surgical approach and in individual prediction of the anatomy for even large sellar and parasellar tumours.

Background Because circulating antibodies against a variety of antigens have been detected in patients with coronary heart disease, carotid atherosclerosis and those who have suffered a stroke, it is suspected that immune response may be one of the mechanisms of atherogenesis The objective of this study is to identify novel antibodies in ischemic stroke patients by screening the expressed recombinant proteins using a human cDNA library (SEREX). Methods To identify the candidate antigens, cDNA library was screened by SEREX using plasma from ten patients with ischemic stroke. Subsequently, via ELISA using recombinant proteins and synthetic peptides, the serum antibody levels were measured in two independent patient/healthy donor (HD) cohorts (142 and 78 in the 2nd screening and a validation cohort, respectively). Results The initial screening resulted in the identification of six candidate antigens. Of these antigens, replication protein A2 (RPA2) was determined to be the antigen associated with stroke (P

The indication of preventive surgery for patients who harbor asymptomatic unruptured intracranial aneurysms remains controversial. To evaluate the benefit of this treatment, we investigated the management outcome in 128 patients with 157 unruptured aneurysms. Surgery was planned in patients 70 years old or younger without serious systemic complications. A total of 77 patients underwent surgery including four endovascular interventions, and conservative management was chosen in 51 patients. There was no mortality and 6.5% morbidity as postoperative results, and no complication was found after endovascular treatment. Among the patients in conservative management, four patients suffered from subsequent rupture during the total follow-up period of 148 person-years. The annual rupture rate was estimated at 2.7%. According to the clinical decision analysis based on our data, preventive surgery is beneficial for a Japanese 70 years old or younger. However, the expected utility decreases if the rupture rate is set at 0.5% or 0.05%, posing a doubt about the benefit of the surgery. Decision analysis provides an aid for logical and objective choice in the management of unruptured aneurysms. The actual risk of rupture has a major impact on decision making in therapeutic strategy.

The MRI finding of oedema along the optic tract has been reported in patients with craniopharyngioma, as a useful diagnostic sign among common pituitary tumours. We report two patients with pituitary metastasis with this MRI finding. A 59-year-old woman and a 54-year-old man had diabetes insipidus and extraocular impairment due to a pituitary metastasis from adenocarcinoma of lung and colon, respectively. Several months later, they noticed rapidly deteriorating visual field and acuity impairment. MRI revealed enlarged pituitary mass accompanied by marked oedema along the bilateral optic tracts. To our knowledge, this is the first MRI report of oedema along the optic pathway due to pituitary metastasis. Our cases and review of literature indicate that this MRI sign is non-specific. Appearance of the oedema well corresponded to the initiation of visual complaint, which make a good contrast to reported clinical presentations of craniopharyngioma. Clinical significance and pathomechanism of this MRI sign are ...

Patients with moyamoya disease verified by conventional cerebral angiography (CCA) were evaluated by means of magnetic resonance angiography (MRA), in order to clarify its advantages and limitations in comparison with CCA. This retrospective study was carried out in 13 hemispheres of seven patients with moyamoya disease (including one atypical patient). In MRA, magnetisation transfer contrast (MTC) and maximal intensity projection (MIP) techniques were used. A good correspondence between MRA and CCA was noted in 11 hemispheres (86.4%) on evaluating stenotic lesions and overestimation in the remaining 2. Underestimation of moyamoya vessels in MRA was noted in 3 hemispheres, since well developed moyamoya vessels were detectable, while poorly developed ones were not. No difference between MRA and CCA findings was found in 10 hemispheres (76.9%). MRA tends to overestimate the stenosis and underestimate moyamoya vessels. These characteristics may lead to wrong classification in the angiographic grade of patients with early and advanced stages and should be taken into consideration in interpreting MRA images of moyamoya disease (compatible rate of grade between MRA and CCA; 84.6%). In conclusion MRA with MTC and MIP techniques offers an acceptable quality of assessment of stenotic lesions and moyamoya vessels. MRA is a useful follow up method at present and in the near future it may replace CCA as the initial diagnostic tool.

This is the first report that the authors are aware of concerning fluctuating visual field defects in patients with Rathke's cleft cysts. Among 11 patients with histologically proven Rathke's cleft cyst, three patients presented with a visual field defect which spontaneously resolved. The symptom became overt again in the subsequent 1 to 14 month follow-up period and a surgical procedure was then chosen. Magnetic resonance imaging (MRI) observation clearly revealed change in the cyst size without signal intensity change in two cases. In the remaining case, multiple spotty high intensities on T1-weighted images were found corresponding to fluctuations in the clinical course. In the last case, enlargement of the cyst was considered to be due to repeated haemorrhage. In the other two cases, minor haemorrhage, undetectable on MRI, may have played a role in the increased cyst size. Another possible mechanism is an imbalance between secretion from, and absorption into, the cyst wall. The size change and neurological fluctuations were previously unknown aspects of the natural history of this lesion. Surgical indications for such patients with transient symptoms followed by cyst size reduction have been controversial, since pituitary cystic lesions may enlarge again or disappear. Our observations suggest that this change in cyst size may be more frequently noted in cases with iso- or high intensity cysts on T1-weighted images, indicating the need for closer observation of patients with this MRI finding.

A 56-year-old female presented with a paraganglioma in the left anterior cranial fossa who manifesting as persistent headache. Computed tomography and magnetic resonance imaging showed a solid, enhanced tumor with a cystic component located medially. The tumor was attached to the left frontal base and the sphenoid ridge. Angiography demonstrated a hypervascular tumor fed mainly by the left middle meningeal artery at the left sphenoid ridge. The preoperative diagnosis was meningioma of the left frontal base. The tumor was totally resected via a left frontotemporal craniotomy. Histological examination revealed the characteristic cellular arrangement of paraganglioma generally designated as the "Zellbaren pattern" on light microscopy. Only 10 patients with supratentorial paraganglioma have been reported, seven located in the parasellar area. The origin of the present tumor may have been the paraganglionic cells which strayed along the middle meningeal artery at differentiation.

There have been few clinical studies in the area of cervical spine that focused on surgery for treating degenerative lumbar disease in patients with rheumatoid arthritis (RA). High rates of wound complications and instrumentation failure have been reported more for RA than for non-RA patients, although clinical outcomes are similar between the two groups. Lumbar canal stenosis in RA is caused not only by degeneration but also by RA-related spondylitis, which includes facet arthritis and inflammation around the vertebral endplate. The pitfalls in surgical management of lumbar canal stenosis in RA patients are highlighted in this study. The study reviewed 12 patients with RA, who were surgically treated for lumbar canal stenosis. Two out of five patients with pulmonary fibrosis died of worsened pulmonary condition, even though there were no perioperative pulmonary complications. Two patients with pedicle screw fixation showed no instrumentation failure, but two patients with spinous process fixation needed re-operation or vertebral fracture. Surgical treatment for lumbar canal stenosis in RA patients needs to be individually adjusted. Preoperative assessments and treatments of pulmonary fibrosis and osteopenia are essential. Surgery for lumbar canal stenosis with RA should be deferred for patients with advanced pulmonary fibrosis because of its potential life-threatening risk. Fusion surgery is indicated only in patients with kyphosis or severe symptoms caused by intervertebral instability. Pedicle screw fixation with hydroxyapatite granules or sublaminar tape is recommended. Closer follow-up after surgery is necessary because of possible delayed wound infection, instrumentation failure, pathological fracture, and respiratory deterioration.