Peritoneal catheter knot formation in ventriculoperitoneal shunting: an intraoperative artificial phenomenon?

BACKGROUND: Peritoneal catheter knot formation is a rare complication associated with ventriculoperitoneal shunting. In most reports, the knot formation was also considered to be the cause of shunt malfunction. DISCUSSION: In this study, we demonstrate the possible misinterpretation of peritoneal catheter knot formation in ventriculoperitoneal shunting. We found a knot in the peritoneal catheter intraoperatively, while no knot was noted on the abdominal X-ray taken 1 day prior to the operation. Our findings indicate that the knot had actually formed intraoperatively. This case suggests that we should not immediately conclude that a knot is the cause of shunt malfunction in such an operation.

Extracranial glioblastoma diagnosed by examination of pleural effusion using the cell block technique: case report.

Metastatic glioblastoma is a rare condition, and several studies have reported the involvement of multiple organs including the lymph nodes, liver, and lung. The lung and pleura are reportedly the most frequent sites of metastasis, and diagnosis using less invasive tools such as cytological analysis with fine needle aspiration biopsy is challenging. Cytological analysis of fluid specimens tends to be negative because of the small number of cells obtained, whereas the cell block technique reportedly has higher sensitivity because of a decrease in cellular dispersion. Herein, the authors describe a patient with a history of diffuse astrocytoma who developed intractable, progressive accumulation of pleural fluid. Initial cytological analysis of the pleural effusion obtained by thoracocentesis was negative, but reanalysis using the cell block technique revealed the presence of glioblastoma cells. This is the first report to suggest the effectiveness of the cell block technique in the diagnosis of extracranial glioblastoma using pleural effusion. In patients with a history of glioma, the presence of extremely intractable pleural effusion warrants cytological analysis of the fluid using this technique in order to initiate appropriate chemotherapy.

Choroid Plexus Hyperplasia with Intractable Ascites and a Resulting Communicating Hydrocele following Shunt Operation for Hydrocephalus.

Choroid plexus hyperplasia/papilloma and resulting hyperproduction of cerebrospinal fluid is a rare cause of hydrocephalus. In these patients, intractable ascites can occur after a ventriculoperitoneal (VP) shunting operation. However, shunt-related hydrocele is a rare complication of VP shunting. Previous reports have indicated catheter-tip migration to the scrotum as a cause of hydrocele. Here, we present the first documented case of choroid plexus hyperplasia that led to intractable ascites after shunting and a resulting hydrocele without catheter-tip migration into the scrotum.

Hypertensive Cerebral Hemorrhage in a Patient with Turner Syndrome Caused by Deletion in the Short Arm of the X Chromosome.

Turner syndrome is a chromosomal disorder usually caused by complete deletion of an X chromosome, with deletion in the short arm of the X chromosome being a rare cause of the condition. Patients with Turner syndrome commonly develop hypertension, and associated vascular complications such as aortic dissection or cerebral hemorrhage have been reported. Cerebral hemorrhage in Turner syndrome is a rare complication, and only a few reports have been published. In these reports, all patients have XO karyotypes or a mosaic type as the cause of Turner syndrome, while no other Turner syndrome types have been documented. In this report, we present for the first time a patient with Turner syndrome caused by deletion in the short arm of the X chromosome who experienced hypertensive hemorrhage as a late complication.

Adult-Onset Hemorrhagic Quasi-Moyamoya Disease with Unilateral Steno-occlusive Lesion in a Patient with Neurofibromatosis Type 1.

BACKGROUND: Quasi-moyamoya disease is a condition that occurs in association with a specific underlying condition or disease such as atherosclerotic disease or neurofibromatosis type 1 (NF1). Pediatric cases are frequently reported, and an ischemic and bilateral presentation is more common than a hemorrhagic and unilateral presentation. CLINICAL PRESENTATION: A 39-year-old woman previously diagnosed with NF1 presented to our department with nausea and left hemiparesis. She was diagnosed with right temporal intracerebral hemorrhage by initial computed tomography. Subsequent angiography showed an occlusion of the terminal portion of the right internal carotid artery, and magnetic resonance imaging showed multiple flow voids in the right basal ganglia, suggesting quasi-moyamoya disease. The hematoma was surgically removed, and her neurological condition improved after the operation. CONCLUSIONS: This is the first reported case of quasi-moyamoya disease with a rare combination of characteristics, including an adult-onset, hemorrhagic presentation and a unilateral lesion in a patient previously diagnosed with NF1.

Intrathecal Baclofen Pump Implantation for Type 2 Gaucher Disease.

Gaucher disease (GD) is the most common type of lysosomal storage disease, with type 2 being the most severe subtype. Type 2 GD patients suffer significant progressive neurological impairment, including spasticity, opisthotonus, seizure, and apnea. The recently developed enzyme replacement therapy (ERT) has shown therapeutic benefit for GD. However, as the enzymes do not cross the blood-brain barrier, ERT does not ameliorate neurological impairment in GD. Intrathecal baclofen therapy (IBT) is indicated for spastic neurological diseases, such as cerebral palsy, and studies have shown its therapeutic benefit in improving several manifestations of GD, such as scoliosis caused by muscle spasticity and respiratory function. To date, the potential benefits of IBT for treating lysosomal storage diseases such as GD have not been examined. Here we provide the first report of a patient with type 2 GD treated with IBT, and demonstrate its therapeutic benefit in ameliorating the neurological aspects of this disease.

Eosinopenia in Children following Traumatic Intracranial Hemorrhage Is Associated with Poor Prognosis and Prolonged Hospital Admission.

BACKGROUND: Neutrophilia is associated with brain injury and is frequently accompanied by eosinopenia. Although eosinopenia is a poor prognostic indicator for various diseases, its significance in intracranial events has not been investigated. METHODS: We retrospectively included 22 pediatric patients (≤18 years old) who experienced traumatic intracranial hemorrhage between 2002 and 2015. Patients were divided into two groups based on the presence or absence of eosinopenia on admission, i.e. the proportion of eosinophils to total white blood cells <1.0%. RESULTS: The mean Glasgow Coma Scale score was marginally lower in the eosinopenia group (14.1 vs. 12.0, p = 0.06). The mean Glasgow Outcome Scale-Extended (GOSE) score was significantly lower in the eosinopenia group (7.5 vs. 5.7, p = 0.02), and the mean length of hospital stay tended to be longer in patients with eosinopenia (7.8 vs. 28.4, p = 0.10). In our multivariate logistic regression analysis, eosinopenia was the only significant risk factor for poor outcome (GOSE score 1-7, OR 29.7, p = 0.03) and prolonged hospital stay (>2 weeks, OR 7.1, p = 0.047). CONCLUSION: These results demonstrate the significance of eosinopenia as a novel prognostic factor in traumatic intracranial hemorrhage in children.

Clinical Usefulness of Preoperative MCA Anatomical Scanning MRI in Thrombectomy Therapy for Acute Anterior Circulation Vessel Occlusion.

Objective: The aim of the present study was to evaluate the usefulness of the T2-weighted three-dimensional sequence method, known as "basi-parallel anatomical scanning (BPAS)-magnetic resonance imaging (MRI)," in demonstrating the running course of the obstructed middle cerebral artery (MCA) before acute mechanical thrombectomy. Methods: Patients whose M1 part and internal carotid artery (ICA) were occluded on preprocedural MRA, but well demonstrated on MCA anatomical scanning (MAS)-MRI were enrolled in this study. The MAS-MR images for patients in whom thrombectomy was performed were compared with the post-thrombectomy angiography. We compared the running course of the C1-M2 bifurcation on MAS-MRI and angiography after thrombectomy, and the results were classified into 3 groups (Excellent, Good, and Poor). Results: A total of 13 patients (range: 54-89) were enrolled, among whom 12 underwent thrombectomy. We compared MAS-MRI and post-thrombectomy angiography in 10. On comparison between MAS-MRI and post-procedural angiography, visualization was excellent in six (60%) patients. The mean age was 75.7 years, ranging from 54 to 89, and 6 were males. 3 patients had ICA occlusion and seven had MCA occlusion. Conclusion: MAS-MRI was considered useful to clarify the running course of the MCA before acute mechanical thrombectomy.

Elevated Serum Fibrinogen Degradation Products on Admission Is a Novel Predictive Factor for Recurrence of Chronic Subdural Hematoma.

BACKGROUND: Previous studies reported an association of hematologic parameters, including white blood cells, neutrophil, eosinophils, or coagulation-related factors, with prognosis in cerebrovascular disorders. However, an association of recurrence rate with serum coagulation-related factors (e.g., D-dimer or fibrinogen degradation products [FDP]) in chronic subdural hematoma (CSDH) is unclear. METHODS: Ninety-two patients who experienced first-time CSDH treated with burr-hole hematoma evacuation were included in this study. Laboratory data on admission were used to divide patients into 2 groups: serum FDP >5 µg/mL or FDP ≤5 µg/mL (within normal range), based on the reference range of our institute. We retrospectively compared the recurrence rate of CSDH within 90 days after the first operation between these groups. Statistical significance was accepted at P < 0.05. RESULTS: Patients with an FDP greater than 5 µg/mL showed a significantly increased recurrence rate compared with those with a normal FDP (≤5 µg/mL; 27.3% vs. 10.2%, respectively; P = 0.03). Patients with an FDP greater than 5 µg/mL also showed a significantly higher recurrence rate within 30 days after the operation (15.2% vs. 3.4%, respectively; P = 0.04), but no difference in the recurrence rate at 31-90 days after the operation (12.1% vs. 6.8%, respectively; P = 0.38). In multivariable analysis, monolayer hematoma (odds ratio, 7.61; P = 0.003) and an FDP >5 µg/mL (odds ratio, 5.04; P = 0.01) were independent predictive factors for recurrence within 90 days. CONCLUSIONS: Elevated serum FDP on admission is a novel predictive factor for the recurrence of CSDH. These patients require careful follow-up, and recurrence within 30 days after the first operation should be considered.

Primary Central Nervous System Hodgkin Lymphoma-Like Posttransplant Lymphoproliferative Disorder.

BACKGROUND: Posttransplant lymphoproliferative disorder (PTLD) is a rare condition occurring after organ transplantation. PTLD comprises 4 subtypes, of which Hodgkin lymphoma (HL) type and HL-like type (currently included in polymorphic type) account for only about 1%-3% of cases. Primary central nervous system PTLD is also rare; most cases are Epstein-Barr virus-positive, B-cell PTLD. To our knowledge, no case of HL-like PTLD has been documented. CASE DESCRIPTION: A 43-year-old woman who underwent kidney transplantation for IgA nephropathy 14 years previously presented to the emergency department with seizure. Gadolinium-enhanced T1-weighted magnetic resonance imaging showed a ring-enhancing mass in the left temporal lobe. Gross total removal of the tumor was performed, and pathologic examination revealed findings consistent with HL-like PTLD. The patient's immunosuppressants were subsequently reduced, and she received postoperative systemic therapy with rituximab and radiation therapy. Follow-up magnetic resonance imaging showed no signs of relapse. CONCLUSIONS: This represents an extremely rare case of a patient with HL-like PTLD occurring as a primary central nervous system lesion.

Joint Capsule-Like Intracranial Osteochondroma Mimicking Cystic Meningioma.

This report provides the first representative images of an intracranial osteochondroma with a cystic component mimicking cystic meningioma. A 40-year-old male presented at our outpatient clinic with decreased sensation in his right upper extremity. Preoperative computed tomography showed a tumor with a cystic meningioma-like appearance and prominent calcifications. On magnetic resonance imaging, calcification of the lesion was suggested by the T2-weighted image; weak enhancement was seen on the T1-weighted image. Following surgical removal of the tumor, the pathologic examination showed findings consistent with osteochondroma. Cystic meningioma is a rare tumor with a cystic component. Intracranial osteochondroma is also a rare, benign tumor that can mimic meningioma when presenting in the dural convexity. Our report describes the joint capsule-like appearance of a convex cystic osteochondroma including a calcified cap, bonelike structure, and fluid-containing intracapsular space. The tumor was evaluated by imaging and pathologic studies.

Presence of a Malignant Tumor as a Novel Predictive Factor for Repeated Recurrences of Chronic Subdural Hematoma.

BACKGROUND: Various risk factors for recurrence of chronic subdural hematomas (CSDHs) have been reported, including alcohol addiction and diabetes mellitus. However, the significance of malignant tumors with respect to CSDH recurrence remains unclear. METHODS: We retrospectively evaluated 281 patients with a first-time CSDH from 2006 to 2016. The difference in the recurrence rate within 100 days postoperatively was compared between patients with a past or present extracranial malignant tumor and those with neither a past nor present extracranial malignant tumor at presentation. Patients in the former group were further divided into 2 subgroups: those with present tumors and those with past tumors. Statistical significance was defined as P < 0.05. RESULTS: A significantly greater repeated recurrence rate (>2 recurrences) was observed in patients with than without a past or present malignant tumor (8.5% vs. 1.7%, respectively; P = 0.01); no significant difference in the first recurrence rate was observed (19.1% vs. 16.2%, respectively; P = 0.63). Furthermore, patients with a present malignant tumor showed a marginally increased repeated recurrence rate than did patients with a past malignant tumor (20.0% vs. 3.1%, respectively; P = 0.053). In the multivariate analysis, a monolayer hematoma was the only risk factor for first recurrence (odds ratio, 3.16; P = 0.003), while a present malignant tumor was the only significant risk factor for repeated recurrences (odds ratio, 16.49; P = 0.002). CONCLUSIONS: The presence of a malignant tumor can be a novel predictive factor for repeated CSDH recurrences. Patients with malignant tumors should be carefully followed, and treatment options such as subcutaneous reservoir placement may be considered to prevent further recurrences.

Mechanical ventilation for comatose patients with inoperative acute intracerebral hemorrhage: possible futility of treatment.

BACKGROUND: Comatose patients with acute intracerebral hemorrhage (ICH) diagnosed as inoperative due to their severe comorbidity will be treated differently between countries. In certain countries including Japan, aggressive medical care may be performed according to the patients' family requests although the effects on the outcome are obscure. For respiratory distress in comatose patients with inoperative acute ICH, the role of mechanical ventilation on the outcome is unknown. We speculated that the efficacy of a ventilator in such a specific condition is limited and possibly futile. METHODS: We retrospectively evaluated the in-hospital mortality and further outcome of 65 comatose patients with inoperative ICH. Among the patients, 56 manifested respiratory distress, and the effect of the ventilator was evaluated by comparing the patients treated with and without the ventilator. RESULTS: The in-hospital mortality was calculated as 80%. A statistically significant parameter affecting the mortality independently was the motor subset on the Glasgow Coma Scale (P = 0.015). Among the patients who manifested respiratory distress, 7.7% of patients treated with a ventilator and 14.0% of patients not treated with a ventilator survived; an outcome is not significantly different. The mean survival duration of patients treated with a ventilator was significantly longer than the mean survival duration of patients not treated with a ventilator (P = 0.021). Among the surviving 13 patients, 7 patients died 5 to 29 months after onset without significant consciousness recovery. Another 6 patients suffered continuous disablement due to prolonged severe consciousness disturbances. CONCLUSION: The current results indicate that treating comatose patients resulting from inoperative acute ICH may be futile. In particular, treating these patients with a ventilator only has the effect of prolonging unresponsive life, and the treatment may be criticized from the perspective of the appropriate use of public medical resources.

Single administration of GDNF into the striatum induced protection and repair of the nigrostriatal dopaminergic system in the intrastriatal 6-hydroxydopamine injection model of hemiparkinsonism.

Purpose: Neurotrophic factor delivery into the brain is a promising approach in the treatment of Parkinson's disease. Glial cell line-derived neurotrophic factor (GDNF) is one of the most potent neurotrophic factors for dopaminergic neurons. Although multiple injections of GDNF into the brain are commonly performed in experimental studies, the present study investigates the efficacy of using a single injection of GDNF, which may be useful in elinically applying this treatment. Methods: Unilateral 6-hydroxydoparnine (6-OHDA) administration into the striatum was perforrned in Sprague-Dawley rats to create a partial lesion of the nigrostriatal DA system. These parkinsonian model rats received a single injection of human recombinant GDNF into the same portion of the striatum either 24 h before or 4 weeks after 6-OHDA treatrnent. Results: GDNF injected into the striatum before 6-OHDA administration potently protected the dopaminergic system, as shown by the numbers of mesencephalic dopaminergie neuron cell bodies and dopaminergic nerve terminal densities in the striatum. Dopaminergic neuron cell bodies and fiber densities were also significantly restored when GDNF was given after 6-OHDA administration, although the degree of restoration was lower than in the protective experiment. ODNF administration ameliorated apomorphine-induced rotational behavior in animals receiving it either before or after 6-OHDA treatment. However, the degree of improvement was less prominent when GDNF was iniected after 6-OHDA. Conclusion: Intracerebral GDNF adininistration exerts both protective and regenerative effects on the nigrostriatal dopaminergic system, a finding which may have implications for the development of new treatment strategies for Parkinson's disease.