[Surgical Management of Idiopathic Normal Pressure Hydrocephalus].

Cerebrospinal fluid shunting, including ventriculo-abdominal/atrial and lumbar-abdominal shunts, is the only treatment available for idiopathic normal pressure hydrocephalus (iNPH). Hybrid valves that combine a programmable pressure valve and an antisiphon device are being widely used for treatment of iNPH, which has significantly minimized surgical complications, such as shunt revision and subdural hematomas associated with overdrainage. Development of novel technologies that can maintain a more physiological intracranial environment after shunting presents a challenge for future research.

[Basic Knowledge and Current Evidence on Cerebrospinal Shunt Devices].

Revision surgeries after cerebrospinal shunt placement are sometimes unavoidable mainly because of the mechanical limitations of current shunt devices despite their technological improvements. This article discusses evidence on existing shunt devices, basic knowledge on their structure and function, and possible future issues.

Guidelines for Management of Idiopathic Normal Pressure Hydrocephalus (Third Edition): Endorsed by the Japanese Society of Normal Pressure Hydrocephalus.

Among the various disorders that manifest with gait disturbance, cognitive impairment, and urinary incontinence in the elderly population, idiopathic normal pressure hydrocephalus (iNPH) is becoming of great importance. The first edition of these guidelines for management of iNPH was published in 2004, and the second edition in 2012, to provide a series of timely, evidence-based recommendations related to iNPH. Since the last edition, clinical awareness of iNPH has risen dramatically, and clinical and basic research efforts on iNPH have increased significantly. This third edition of the guidelines was made to share these ideas with the international community and to promote international research on iNPH. The revision of the guidelines was undertaken by a multidisciplinary expert working group of the Japanese Society of Normal Pressure Hydrocephalus in conjunction with the Japanese Ministry of Health, Labour and Welfare research project. This revision proposes a new classification for NPH. The category of iNPH is clearly distinguished from NPH with congenital/developmental and acquired etiologies. Additionally, the essential role of disproportionately enlarged subarachnoid-space hydrocephalus (DESH) in the imaging diagnosis and decision for further management of iNPH is discussed in this edition. We created an algorithm for diagnosis and decision for shunt management. Diagnosis by biomarkers that distinguish prognosis has been also initiated. Therefore, diagnosis and treatment of iNPH have entered a new phase. We hope that this third edition of the guidelines will help patients, their families, and healthcare professionals involved in treating iNPH.

Development of a Lumbar Extradural Arachnoid Cyst Associated with a Lumbar Catheter of Lumboperitoneal Shunt: A Case Report.

A 78-year-old man, who had undergone lumboperitoneal shunt (LPS) placement for idiopathic normal-pressure hydrocephalus eight years prior, presented with intermittent claudication, lower back pain, and radicular pain on the inside of the right thigh. Magnetic resonance imaging (MRI) revealed an extradural arachnoid cyst (EDAC) above the lumbar catheter of the LPS. The EDAC compressed the spinal dural sac and cauda equina toward the anterior side at level L3/4, triggering his clinical manifestations. The LPS was removed and simultaneously converted into a ventriculoperitoneal shunt (VPS), which immediately improved the neurological deficits. Postoperative MRI showed shrinkage of the cyst and restoration of the compressed cauda equina. Spinal EDAC is a rare entity resulting from arachnoid membrane herniation due to a small defect in the dura mater. This is the first report showing that symptomatic EDAC can be accompanied by the lumbar catheter of the LPS and that a mere conversion from LPS to VPS or ventriculoatrial shunt might be sufficient to shrink LPS-related EDAC without invasive lumbar surgeries.

Receptor Tyrosine Kinases: Principles and Functions in Glioma Invasion.

Protein tyrosine kinases are enzymes that are capable of adding a phosphate group to specific tyrosines on target proteins. A receptor tyrosine kinase (RTK) is a tyrosine kinase located at the cellular membrane and is activated by binding of a ligand via its extracellular domain. Protein phosphorylation by kinases is an important mechanism for communicating signals within a cell and regulating cellular activity; furthermore, this mechanism functions as an "on" or "off" switch in many cellular functions. Ninety unique tyrosine kinase genes, including 58 RTKs, were identified in the human genome; the products of these genes regulate cellular proliferation, survival, differentiation, function, and motility. Tyrosine kinases play a critical role in the development and progression of many types of cancer, in addition to their roles as key regulators of normal cellular processes. Recent studies have revealed that RTKs such as epidermal growth factor receptor (EGFR), platelet-derived growth factor receptor (PDGFR), c-Met, Tie, Axl, discoidin domain receptor 1 (DDR1), and erythropoietin-producing human hepatocellular carcinoma (Eph) play a major role in glioma invasion. Herein, we summarize recent advances in understanding the role of RTKs in glioma pathobiology, especially the invasive phenotype, and present the perspective that RTKs are a potential target of glioma therapy.

Aqueductal Developmental Venous Anomaly Presenting with Mimic Symptoms of Idiopathic Normal Pressure Hydrocephalus in an Elderly Patient: A Case Report.

Developmental venous anomalies (DVAs) are generally asymptomatic; however, they can sometimes cause central nervous disorders. Aqueductal stenosis caused by DVAs is so rare that only 14 cases have been reported to date. Moreover, most patients are children or young adults, presenting with headaches or consciousness disturbances, associated with raised intracranial pressure. Here, we report on an 83-year-old man presenting with mimic symptoms of idiopathic normal pressure hydrocephalus (cognitive disorder, gait disturbance, and urinary urgency: Hakim's triad) because of obstructive hydrocephalus caused by a DVA located in the aqueduct. Endoscopic third ventriculostomy (ETV) was performed to relieve his symptoms, and the opening pressure of the lateral ventricle was recorded to be 10 cm-H2O. Endoscopic examination of the intraventricular system clearly revealed a vein within the aqueduct converging with the adjacent subependymal vessels. These findings were compatible with the characteristics of DVAs. His symptoms improved after the ETV. This case suggested that DVAs within the aqueduct, despite of their congenital nature, could give rise to decompensated obstructive hydrocephalus even in elderly patients, resulting in Hakim's triad.

Contribution of Intrasellar Pressure Elevation to Headache Manifestation in Pituitary Adenoma Evaluated With Intraoperative Pressure Measurement.

BACKGROUND: Headache frequently occurs in patients with pituitary adenoma and is reported in large as well as small adenomas. However, the exact mechanism of headache derived from pituitary adenoma remains unknown. OBJECTIVE: To evaluate the contribution of intrasellar pressure (ISP) to headache manifestation by using intraoperative ISP measurement. METHODS: The records of 108 patients who had first-time transsphenoidal surgery for pituitary adenoma were reviewed retrospectively. Measurement of intraoperative ISP was undergone using intracranial pressure monitoring sensors and compared with radiological assessment. RESULTS: Among 30 patients with headache, 29 (96.7%) presented with significant headache (Headache Impact Score-6, 50 or greater). Intraoperative ISP measurement was conducted successfully in all cases, and revealed higher ISP in patients with headache (35.6 ± 9.2 mm Hg) than in those without headache (15.8 ± 5.2 mm Hg). The ISP reduction after sella floor decompression was greater in patients with headache than that in patients without headache. In patients with headache, the frequency of invasion into the cavernous sinus or sphenoid sinus was significantly lower, and the diameter of the foramen at the diaphragm sellae was narrower. In addition, intratumoral cyst or hematoma was more common in patients with headache. Postoperatively, headache was either diminished or improved in 28 patients (93.3%). CONCLUSION: Headache in patients with pituitary adenomas associated with ISP elevation, results from compromised dural integrity at the sella and intratumoral hemorrhage. The increased stretch force of the sella dura may be a notable etiology of headache in patients with pituitary adenoma.

Directional Regulation of Extrasellar Extension by Sellar Dura Integrity and Intrasphenoidal Septation In Pituitary Adenomas.

OBJECTIVE: Pituitary macroadenomas extend into the extrasellar space, such as the sphenoid sinus, cavernous sinuses, and suprasellar region. However, factors that regulate the direction of their extensions into the surrounding anatomical structures remain unknown. METHODS: This retrospective study included 162 patients who were treated for pituitary adenomas that had maximum diameters greater than 20 mm. According to the direction of adenoma extension, patients were divided into 4 groups: group A, inferior into the sphenoid sinus; group B, lateral into the cavernous sinus; group C, suprasellar region with enlarged sella turcica; and group D, supraellar region without enlarged sella turcica. Several anatomical structures surrounding the sella turcica were evaluated statistically, and multivariate logistic regression analysis was performed if the structures met the determining factors of adenomas extensions. RESULTS: The maximum diameter of adenomas was significantly larger in groups A and D. The maximum diameter of the diaphragmatic foramen was largest in group C (19.3 mm) and was significantly narrower in groups B (12.7 mm) and D (12.5 mm). Intrasphenoid septation, attached on the midline of the sella turcica, was observed most frequently in group D (78.6%) and was not detectable in group A (0%). Extension into the cavernous sinus, classified as dural discontinuity, was highly prevalent in group B (80.0%) and was uncommon in group C (12.3%). Erosion of the posterior clinoid process was most apparent in group B (92.0%). CONCLUSIONS: The integrity of the sella dura and the intrasphenoid septation can regulate adenoma extension by encouraging their growth towards paths of least resistance.

Evaluation of Soft Tissue Hypertrophy at the Retro-Odontoid Space in Patients with Chiari Malformation Type I on Magnetic Resonance Imaging.

INTRODUCTION: Chiari malformation type I (CM-I) is a well-known hindbrain disorder in which the cerebellar tonsils protrude through the foramen magnum. The soft tissues, including the transverse ligament and the tectorial membrane at the retro-odontoid space, can compress the cervicomedullary junction if they become hypertrophic. METHODS: Twenty-two symptomatic patients with CM-I (aged 5-19 years) were treated between 2007 and 2017 at our institute. The retro-odontoid soft tissue was evaluated using T2-weighted magnetic resonance imaging. Anteroposterior (AP) distances and craniocaudal distances of the soft tissue were measured in patients with CM-I and 48 normal control children. Modified clivoaxial angles were also evaluated as the index of ventral compression of the cervicomedullary junction. RESULTS: Of the 18 patients treated with foramen magnum decompression, 16 patients improved postoperatively, whereas the condition of 2 remained unchanged. The AP distances in the CM-I group (6.0 mm) were significantly larger than those in the control group (3.5 mm), whereas there were no apparent differences in the craniocaudal distances. Modified clivoaxial angles were obviously smaller in the CM-I group (131.5°) than in the control group (146.9°). Moreover, the AP distances were significantly reduced postoperatively (5.5 mm), although the other parameters did not change significantly. CONCLUSIONS: The retro-odontoid soft tissue in symptomatic patients with CM-I can be hypertrophic enough to compress the cervicomedullary junction ventrally even if there are no combined osseous anomalies. Foramen magnum decompression works to reduce the hypertrophic changes significantly, suggesting that downward tonsil movement might participate in hypertrophic soft tissue formation at the retro-odontoid space.

Outcome of diabetes insipidus in patients with Rathke's cleft cysts.

OBJECTIVES: It is well-known that Rathke's cleft cysts (RCCs) infrequently cause headache, endocrinological dysfunction, and visual disturbance, and in rare cases, cause diabetes insipidus (DI). Although surgical evacuation of the cyst content can result in high rates of symptomatic improvement, not only the treatment efficacy but also the pathophysiology of DI with RCC are undetermined. The aim of this study is to elucidate the underlying mechanisms and outcomes of DI associated with RCCs. PATIENTS AND METHODS: We retrospectively studied 109 patients with RCCs treated at Kanazawa University Hospital between 2000 and 2016. Their age, sex, symptoms, endocrinological status, DI, visual disturbance, neuroradiological findings, pathological appearances, and pre-/post-operative hormone levels and status of anti-diuretic hormone replacements were assessed. RESULTS: Among 109 cases of RCCs, five cases (4.6%, 2 males and 3 females) manifested with DI as initial presentation were included. These five cases could be divided into two types: the acute type and the chronic type, based on the onset and duration of symptoms. Three acute onset cases presented with not only strong thirst but also sudden headaches without pituitary dysfunction, whereas the two chronic onset cases presented with chronic headaches and hypopituitarism. Pathological examination in the acute type revealed inflammatory cell infiltration into only the posterior lobe of the pituitary and disruption of the cyst wall adjacent to the posterior lobe, which might suggest RCC rupture. In contrast, the chronic type showed inflammatory cell infiltration into both the anterior and posterior lobes of the pituitary and thickened fibrosis beneath the cyst wall. Postoperatively, two cases of the acute type could be controlled with a smaller amount of 1-deamino-8-D-arginine vasopressin (DDAVP) than that required preoperatively, whereas no change was observed in the cases of the chronic type. CONCLUSION: The cases of DI onset caused by RCCs could be divided into the acute type and the chronic type. In the chronic type, surgical treatment could not affect the status of DI. However, in acute type, urgent surgical intervention partially relieved DI.

Delayed Occurrence of Diabetes Insipidus After Transsphenoidal Surgery with Radiologic Evaluation of the Pituitary Stalk on Magnetic Resonance Imaging.

BACKGROUND: Diabetes insipidus (DI) is a major complication of transsphenoidal surgery (TSS). DI usually occurs within a couple of days after TSS. Delayed occurrence of postoperative DI is rarely observed and its developing mechanisms remain unknown. METHODS: Six patients were identified as having postoperative delayed DI, which was defined as DI that first occurred 2 or more weeks after TSS. They consisted of 1 male and 5 females, and their mean age was 38.3 years (range, 10-76 years). Five patients were histologically diagnosed with Rathke cleft cyst (RCC), and one had RCC coexisting with prolactin-secreting adenoma. Sequential T1-weighted magnetic resonance imaging was evaluated for hyperintensity (HI) in the pituitary stalk and the posterior lobe, indicating the location of antidiuretic hormone. RESULTS: No patients had any DI before TSS. Delayed DI occurred 2 weeks to 3 months after TSS and persisted for 2 weeks to 5 months. T1-weighted magnetic resonance imaging showed that the HI in the posterior lobe became faint but did not disappear after DI occurrence, and their intensities increased with recovery from DI. In contrast, the HI in the pituitary stalk was found faintly preoperatively and turned clear postoperatively and decreased with recovery from DI. The morphologic patterns were dependent on DI duration. CONCLUSIONS: In the delayed occurrence of DI, it was suggested that preoperative antidiuretic hormone transport was mildly congested yet not completely blocked when DI manifested postoperatively. Gradual spreading of inflammation to the infundibulum after RCC removal was considered as 1 possible mechanism of this delayed DI development.

Contribution of sellar dura integrity to symptom manifestation in pituitary adenomas with intratumoral hemorrhage.

PURPOSE: Although hemorrhage within pituitary adenomas frequently exacerbates the symptoms, there are many grades of severity. Moreover, the contributing factors for symptom severity are still controversial. METHODS: This retrospective study included 82 patients who underwent transsphenoidal surgery for pituitary adenomas with intratumoral hemorrhage. The grades of preoperative symptoms were classified into group A, asymptomatic or minor symptoms; group B, moderate symptoms sufficient for complain; and group C, severe symptoms disturbing daily life. RESULTS: The hemorrhage volume within an adenoma was significantly higher in group C (92.6%) than in groups A (48.6%) and B (58.7%). Both headache and diplopia were dominant in group C, occurring in 72.2% and 27.8% of the patients, respectively. In group C, there was no significant difference in frequency between adenoma extensions into the sphenoid sinus (0%) and involvement of the cavernous sinus of Knosp grade 4 (0%), and extensions into the suprasellar region were not common (38.9%). The most distinctive feature was that "no extrasellar extension" was found only in group C (41.2%), and "multidirectional extension" was not detected in this group (0%). Multiple regression analysis revealed that the most powerful determining factors were the high frequencies of intratumoral hemorrhage and lack of extrasellar and multidirectional extensions. CONCLUSION: Rapid volume expansion of a hematoma and lack of extension or unidirectional extension might lead to significant compression of the sellar and surrounding structures. Of note, the integrity of the sellar dura might contribute to the acute onset of symptom manifestations caused by hemorrhage in pituitary adenomas.

Preoperative Evaluation of the Interface Between Tuberculum Sellae Meningioma and the Optic Nerves on Fast Imaging with Steady-State Acquisition for Extended Endoscopic Endonasal Transsphenoidal Surgery.

INTRODUCTION: Endoscopic endonasal transsphenoidal surgery (EETS) is increasingly applied to treat tuberculum sellae meningiomas. However, if the tumor adheres firmly to the optic nerve, dissection of the interface between both structures should be prudent to preserve visual function. The purpose of this study was to investigate whether tumor adhesion to the optic nerve can be predicted preoperatively by fast imaging with steady-state acquisition (FIESTA). METHODS: Twenty-two patients with tuberculum sellae meningioma treated with EETS were retrospectively identified. Clinical characteristics, radiologic studies, intraoperative findings, and outcomes were reviewed from their clinical charts. RESULTS: Patients' symptoms included visual function impairment in 18 patients and headaches in 4 patients. Symptoms were resolved in 19 patients after operation. Preoperative radiologic evaluation was performed in 44 sides (22 patients) of the interface between tumors and the optic nerves and showed absence of peritumoral hyperintensity on FIESTA in 7 sides in 7 patients. In 5 of the 7 sides, tumor dissection was complicated by firm adhesion to the optic nerves. Among these cases, visual functions were unchanged in 1 patient after complete removal of the adhesion but substantially improved in 3 patients after partial resection. In the remaining 37 sides with preoperative peritumoral hyperintensity, no adhesion was found between both structures intraoperatively. CONCLUSIONS: Absence of peritumoral hyperintensity between tuberculum sellae meningioma and the optic nerve on FIESTA may indicate firm adhesion at the interface, severely complicating complete removal. Preoperative recognition of this adhesion is important for safe tumor removal and preservation of visual functions.

Significant Improvement in Chronic Persistent Headaches Caused by Small Rathke Cleft Cysts After Transsphenoidal Surgery.

PURPOSE: Rathke cleft cysts (RCC) usually are asymptomatic and can be observed via the use of conservative methods. Some patients with RCCs, however, have severe headaches even if they are small enough to be confined to the sella, and these small RCCs seldom have been discussed. This study presents an investigation into clinical characteristics of small RCCs associated with severe headaches, demonstrating efficacy and safety of endoscopic transsphenoidal surgery (ETSS) to relieve headaches. METHODS: In this study, 13 patients with small RCCs (maximum diameter <10 mm) who presented with headaches and were treated by ETSS at our institute from 2009 to 2014 were recruited. These RCCs were treated Headache Impact Test-6 (HIT-6) score was calculated both pre- and postoperatively to evaluate headache severity. RESULTS: All patients complained of severe headaches, which disturbed their daily life. Most headaches were nonpulsating and localized in the frontal area. Characteristically, 6 patients (46%) experienced severe headaches with sudden onset that continued chronically. HIT-6 score was 64 on average, meaning headaches affected daily life severely. After surgical decompression of the cyst, headache in all of the patients improved dramatically and HIT-6 score decreased significantly to 37, suggesting that headaches were diminished. No newly developed deficiencies of the anterior pituitary lobe function were detected. Postoperative occurrence of diabetes insipidus was found in 2 patients, both of which were transient. No recurring cysts were found. CONCLUSIONS: Severe headaches can develop from small RCCs. In the present study, ETSS was performed on such patients effectively and safely to relieve their headaches.

Descent of the anterior communicating artery after removal of pituitary macroadenoma using transsphenoidal surgery.

BACKGROUND: After removal of pituitary macroadenoma, the anterior communicating artery (AComA) descends toward the original position. However, the process and contributing factors of this descent are not elucidated. METHODS: This retrospective study included 102 patients who underwent transsphenoidal surgery (TSS) for macroadenomas with maximum diameters of >2 cm. Sequential T2-weighted magnetic resonance images were used to assess the AComA flow void and its distance from the planum sphenoidale before and after TSS. The AComA position in relation to the adenoma was divided into four groups as follows: anterior, anterosuperior, superior, and posterior. The descent was compared to the presence of intratumoral hemorrhage or adenoma extension into the sphenoid sinus. RESULTS: One week after TSS, the AComA descent was more pronounced than when originally in the superior position (6.5 ± 3.7 mm vs 4.4 ± 3.5 mm, P < 0.0001). The postoperative descents of the AComA were well correlated with those of residual adenomas only when in the superior position (P = 0.030). The AComA descent was more significant at 1 week (4.4 ± 3.5 mm) than at 1 week to 3 months (0.7 ± 1.0 mm) in all the groups. Both intratumoral hemorrhage and sphenoid sinus extension of adenoma did not affect the AComA descent in each group. CONCLUSION: AComA descent was most influenced when it was superior to the macroadenoma and progressed mostly within 1 week after TSS, probably initiating during TSS. The position of the AComA in relation to a macroadenoma should be considered preoperatively to avoid vascular injury.

A Segmental Copy Number Loss of the SFMBT1 Gene Is a Genetic Risk for Shunt-Responsive, Idiopathic Normal Pressure Hydrocephalus (iNPH): A Case-Control Study.

Little is known about genetic risk factors for idiopathic normal pressure hydrocephalus (iNPH). We examined whether a copy number loss in intron 2 of the SFMBT1 gene could be a genetic risk for shunt-responsive, definite iNPH. Quantitative and digital PCR analyses revealed that 26.0% of shunt-responsive definite iNPH patients (n = 50) had such a genetic change, as compared with 4.2% of the healthy elderly (n = 191) (OR = 7.94, 95%CI: 2.82-23.79, p = 1.8 x 10-5) and 6.3% of patients with Parkinson's disease (n = 32) (OR = 5.18, 95%CI: 1.1-50.8, p = 0.038). The present study demonstrates that a copy number loss within intron 2 of the SFMBT1 gene may be a genetic risk factor for shunt-responsive definite iNPH.

Treatment Result in the Initial Stage of Kanazawa Mobile Embolectomy Team for Acute Ischemic Stroke.

Five recent multicenter randomized controlled trials (RCTs) have clearly shown the superiority of mechanical thrombectomy in large vessel occlusion acute ischemic stroke compared to systemic thrombolysis. Although 14 hospitals in Ishikawa prefecture have uninterrupted availability of systemic thrombolysis, mechanical thrombectomy is not available at all of these hospitals. Therefore, we established a Kanazawa mobile embolectomy team (KMET), which could travel to these hospitals and perform the acute reperfusion therapy. In this article, we report early treatment outcomes and validate the effectiveness of a network between affiliated hospitals and KMET. Between January 2014 and December 2015, 48 patients, aged 45-92 years (mean: 73.0 years), underwent acute reperfusion therapy provided by KMET in 10 affiliated hospitals of Kanazawa University Hospital. The pre-treatment NIHSS scores ranged from 5 to 39 (mean: 19.1). ASPECTS+W ranged from 1 to 11 (mean: 7.3). Successful revascularization, defined as thrombolysis in cerebral infarction (TICI) 2b or 3, was achieved in 38/48 cases (80%), and a good outcome, defined as modified Rankin Scale (mRS) score from 0 to 2 at 90 days after the treatment, was achieved in 24/48 cases (50%). There were two cases of intracranial bleeding (4%). Mean time from onset to recanalization was 297 min. These results, which are similar to those of five previous RCTs, suggest that a collaborative network between affiliated hospitals and KMET is effective for acute reperfusion therapy in local areas wherein experienced neuroendovascular specialists are insufficient.

Prediction of postoperative diabetes insipidus using morphological hyperintensity patterns in the pituitary stalk on magnetic resonance imaging after transsphenoidal surgery for sellar tumors.

PURPOSE: Diabetes insipidus (DI) remains a complication of transsphenoidal surgery (TSS) for sellar and parasellar tumors. Antidiuretic hormone (ADH) appears as hyper intensity (HI) in the pituitary stalk and the posterior lobe of the pituitary gland on T1-weighted magnetic resonance (MR) imaging. Its disappearance from the posterior lobe occurs with DI, indicating a lack of ADH. The appearance of HI in the pituitary stalk indicates disturbances in ADH transport. METHODS: This retrospective study included 172 patients undergoing TSS for sellar tumors at our institute from 2006 to 2014. Sequential T1-weighted MR images without enhancement were evaluated for HI in the pituitary stalk and the posterior lobe to assess the localization of ADH before and at intervals after TSS. DI was assessed pre- and postoperatively. HI in the pituitary stalk showed the following morphology: (1) ovoid in the distal end of the pituitary stalk (group A), (2) linear in the distal part of the pituitary stalk (group B), (3) linear in the whole pituitary stalk (group C). RESULTS: Preoperative DI occurred in 6 patients (3.5 %) with no HI observed in the posterior lobe. Postoperative DI was transient in 82 patients (47.7 %), and permanent in 11 (6.4 %). One week after surgery, HI was absent in the posterior lobe in 74 patients (43.0 %), and present in the pituitary stalk in 99 patients (57.6 %); both were significantly correlated with postoperative DI (p < 0.001). The absence of HI in the posterior lobe (A, 48.9 %; B, 68.3 %; C, 92.3 %), persistence of DI (A, 3.7 days; B, 45.9 days; C, 20.5 months), and duration until HI recovery in the posterior lobe (A, 3.6 months; B, 6.8 months; C, 22.9 months) were greatest in group C, followed by group B, and then group A. Fourteen group A patients did not have postoperative DI despite having HI in the pituitary stalk and the posterior lobe. Four group C patients developed permanent DI with persistence HI in the pituitary stalk. CONCLUSION: HI in the pituitary stalk and its absence in the posterior lobe indicated postoperative DI, which was transient if HI was detected in the pituitary stalk. DI duration could be predicted by the length of HI in the pituitary stalk, which corresponded to the degree of ADH transport obstruction.

Xanthomatous hypophysitis associated with autoimmune disease in an elderly patient: A rare case report.

BACKGROUND: Xanthomatous hypophysitis (XH) is an extremely rare form of primary hypophysitis characterized by infiltration of the pituitary gland by mixed types of inflammatory cells, including foamy cells, plasma cells, and small mature lymphocytes. XH manifests as varying degrees of hypopituitarism. Although several previous reports have denied a possible contribution of autoimmune mechanism, the exact pathogenesis of XH remains unclear. CASE DESCRIPTION: We describe the case of a 72-year-old woman with a history of rheumatoid arthritis and Sjögren's syndrome who presented with panhypopituitarism and diabetes insipidus. At the time of her visit, she also experienced relapsed rheumatoid arthritis and Sjögren's syndrome, manifesting as arthralgia. Magnetic resonance imaging (MRI) showed a multicystic mass in the sellar and suprasellar regions. In the course of steroid replacement therapy for hypocortisolism, the patient's arthralgia diminished, and MRI revealed shrinkage of the mass. XH was diagnosed histologically following a transsphenoidal endoscopic biopsy, and it was the oldest case of XH. CONCLUSION: To the best of our knowledge, this patient is the oldest of reported patients diagnosed with XH. Steroid therapy may be effective to XH temporarily. XH should be considered when diagnosing pituitary cystic lesions in elderly patients with autoimmune disease.