Low-Pressure Hydrocephalus (LPH) is a distinct type of hydrocephalus characterized by persistently low intraventricular pressure below 70 mmH₂O (1 mmH₂O=0.009 8 kPa). Its pathophysiological mechanisms and treatment strategies differ significantly from normal-or high-pressure hydrocephalus, with fourth ventricular outflow tract obstruction and altered brain compliance potentially serving as critical pathogenesis. LPH predominantly occurs in patients with severe consciousness impairment, typically secondary to severe neurological conditions such as severe brain injuries or chronic intracranial infections. Given that the LPH usually has a long course of primary disease and insidious onset, it was poorly diagnosis and get insufficient recognition in clinic. To date, there is no accurate epidemiology of LPH, and existing studies suggest that its actual incidence may be systematically underestimated. To standardize the early identification, precise diagnosis and reasonable intervention of LPH and improve the treatment effect, the Chinese Neuroscience Society organized experts in related fields to formulate the "Consensus on the diagnosis and treatment of low pressure hydrocephalus（2025 edition）", based on the latest evidence-based medicine and specific practices in the diagnosis and treatment of LPH. The diagnosis of LPH is confirmed based on: (1) high-risk clinical history; (2) progressive ventricular enlargement accompanied by worsening neurological deficits: (3) characteristic imaging findings (ventricular system dilation, disappearance or marked narrowing of sylvian fissure cisterns and convexal subarachnoid spaces, fourth ventricular enlargement potentially demonstrating a "ball sign"); (4) ventricular puncture confirming pressure between 0 to <70 mmH₂O. For LPH management, ventriculoperitoneal shunt placement using non-antisiphon devices is recommended as the primary treatment. This should be combined with dynamic progressive valve pressure adjustments guided by serial imaging of ventricular morphology and neurological status, ultimately achieving personalized target pressure levels to ensure effective cerebrospinal fluid diversion and improved clinical outcomes.
Humans ; Hydrocephalus, Normal Pressure/therapy* ; Consensus ; Hydrocephalus/therapy*

Midbrain gliomas are relatively rare neoplasms with a generally benign prognosis, with dissemination or metastasis not previously reported. We describe here a woman, in whom magnetic resonance imaging scans showed hydrocephalus and a tegmental lesion in the upper aqueduct. Endoscopic third ventriculostomy and biopsy were performed; during surgery, a second small lesion was observed in the infundibular recess. Histologically, the two lesions had the characteristics of low grade astrocytoma, suggesting that the midbrain astrocytoma may have been disseminated via the cerebral spinal fluid to the infundibular recess. Postoperatively this patient received radiotherapy for nearly one month. Although patients with these tumors are not usually administered adjunctive therapy, radiation and, combined modality therapy, including surgery, radiotherapy, and chemotherapy, may be beneficial in patients with midbrain gliomas with dissemination.
Adult* ; Astrocytoma* ; Biopsy ; Cerebrospinal Fluid* ; Combined Modality Therapy ; Drug Therapy ; Female ; Glioma ; Humans ; Hydrocephalus ; Magnetic Resonance Imaging ; Mesencephalon ; Neoplasm Metastasis ; Neuroendoscopes ; Prognosis ; Radiotherapy ; Ventriculostomy

Central neurocytoma (CN) is a rare, benign brain tumor often located in the lateral ventricles. CN may cause obstructive hydrocephalus and manifest as signs of increased intracranial pressure. The goal of treatment for CN is a gross total resection (GTR), which often yields excellent prognosis with a very high rate of tumor control and survival. Adjuvant radiosurgery and radiotherapy may be considered to improve tumor control when GTR cannot be achieved. Chemotherapy is also not considered a primary treatment, but has been used as a salvage therapy. The radiological features of CN are indistinguishable from those of other brain tumors; therefore, many histological markers, such as synaptophysin, can be very useful for diagnosing CNs. Furthermore, the MIB-1 Labeling Index seems to be correlated with the prognosis of CN. We also discuss oncogenes associated with these elusive tumors. Further studies may improve our ability to accurately diagnose CNs and to design the optimal treatment regimens for patients with CNs.
Brain Neoplasms ; Drug Therapy ; Humans ; Hydrocephalus ; Intracranial Pressure ; Lateral Ventricles ; Neurocytoma* ; Oncogenes ; Prognosis ; Radiosurgery ; Radiotherapy ; Salvage Therapy ; Synaptophysin

OBJECTIVE: Elevated cell counts and protein levels in cerebrospinal fluid (CSF) result from disease activity in patients with leptomeningeal carcinomatosis (LMC). Previous studies evaluated the use of CSF profiles to monitor a treatment response or predict prognosis. CSF profiles vary, however, according to the sampling site and the patient's systemic condition. We compared lumbar and ventricular CSF profiles collected before intraventricular chemotherapy for LMC and evaluated the association of these profiles with patients' systemic factors and LMC disease activity. METHODS: CSF profiles were retrospectively collected from 228 patients who underwent Ommaya reservoir insertion for intraventricular chemotherapy after a diagnosis of LMC. Lumbar samples taken via lumbar puncture were used for the diagnosis, and ventricular samples were obtained later at the time of Ommaya reservoir insertion. LMC disease activity was defined as the presence of LMC-related symptoms such as increased intracranial pressure, hydrocephalus, cranial neuropathy, and cauda equina syndrome. RESULTS: Cell counts (median : 8 vs. 1 cells/mL) and protein levels (median : 68 vs. 17 mg/dL) significantly higher in lumbar CSF than in ventricular CSF (p<0.001). Among the evaluated systemic factors, concomitant brain metastasis and previous radiation were significantly correlated with higher protein levels in the lumbar CSF (p=0.01 and <0.001, respectively). Among the LMC disease activity, patients presenting with hydrocephalus or cauda equina syndrome showed higher lumbar CSF protein level compared with that in patients without those symptoms (p=0.049 and p<0.001, respectively). The lumbar CSF cell count was significantly lower in patients with cranial neuropathy (p=0.046). The ventricular CSF cell counts and protein levels showed no correlation with LMC symptoms. Carcinoembryonic antigen (CEA), which was measured from ventricular CSF after the diagnosis in 109 patients, showed a significant association with the presence of hydrocephalus (p=0.01). CONCLUSION: The protein level in lumbar CSF indicated the localized disease activity of hydrocephalus and cauda equina syndrome. In the ventricular CSF, only the CEA level reflected the presence of hydrocephalus. We suggest using more specific biomarkers for the evaluation of ventricular CSF to monitor disease activity and treatment response.
Biomarkers ; Brain ; Carcinoembryonic Antigen ; Cell Count ; Cerebrospinal Fluid* ; Cranial Nerve Diseases ; Diagnosis ; Drug Therapy ; Humans ; Hydrocephalus ; Intracranial Pressure ; Meningeal Carcinomatosis* ; Neoplasm Metastasis ; Polyradiculopathy ; Prognosis ; Retrospective Studies* ; Spinal Puncture

PURPOSE: Diencephalic syndrome is an uncommon cause of failure to thrive in early childhood that is associated with central nervous system neoplasms in the hypothalamic-optic chiasmatic region. It is characterized by complex signs and symptoms related to hypothalamic dysfunction; such nonspecific clinical features may delay diagnosis of the brain tumor. In this study, we analyzed a series of cases in order to define characteristic features of diencephalic syndrome. METHODS: We performed a retrospective study of 8 patients with diencephalic syndrome (age, 5-38 months). All cases had presented to Seoul National University Children's Hospital between 1995 and 2013, with the chief complaint of poor weight gain. RESULTS: Diencephalic syndrome with central nervous system (CNS) neoplasm was identified in 8 patients. The mean age at which symptoms were noted was 18+/-10.5 months, and diagnosis after symptom onset was made at the mean age of 11+/-9.7 months. The mean z score was -3.15+/-1.14 for weight, -0.12+/-1.05 for height, 1.01+/-1.58 for head circumference, and -1.76+/-1.97 for weight-for-height. Clinical features included failure to thrive (n=8), hydrocephalus (n=5), recurrent vomiting (n=5), strabismus (n=2), developmental delay (n=2), hyperactivity (n=1), nystagmus (n=1), and diarrhea (n=1). On follow-up evaluation, 3 patients showed improvement and remained in stable remission, 2 patients were still receiving chemotherapy, and 3 patients were discharged for palliative care. CONCLUSION: Diencephalic syndrome is a rare cause of failure to thrive, and diagnosis is frequently delayed. Thus, it is important to consider the possibility of a CNS neoplasm as a cause of failure to thrive and to ensure early diagnosis.
Astrocytoma ; Brain Neoplasms ; Central Nervous System ; Central Nervous System Neoplasms ; Diagnosis ; Diarrhea ; Drug Therapy ; Early Diagnosis ; Failure to Thrive* ; Follow-Up Studies ; Head ; Humans ; Hydrocephalus ; Hypothalamic Diseases ; Infant* ; Optic Nerve Glioma ; Palliative Care ; Retrospective Studies ; Seoul ; Strabismus ; Vomiting ; Weight Gain

Intracranial infections are one of the most common neurological diseases in children and are associated with high mortality and morbidity. Intracranial hypertension and hydrocephalus are the common, fatal complications of intracranial infections, so early diagnosis and timely treatment are the keys to saving patients' lives and reducing neurological sequelae. This paper introduces the progress in the etiology, diagnosis, and treatment of intracranial hypertension and hydrocephalus in children with intracranial infections.
Central Nervous System Infections ; complications ; Child ; Humans ; Hydrocephalus ; diagnosis ; etiology ; therapy ; Intracranial Hypertension ; diagnosis ; etiology ; therapy

lnflammatory pseudotumor (IPT) is a rare, non-neoplastic inflammatory process. It is most commonly occurs in the orbit, but extension into brain parenchyma is uncommon. In a confirmed case of IPT, most cases show good improvement with steroid theraphy. A 50-year-old man with progressive left-eye visual disturbance and mass lesion was admitted in a hospital. A left orbital mass biopsy revealed what was highly suspected as an inflammatory pseudotumor. Steroid pulse therapy with dexamethasone, radiation therapy, and chemotherapy with amphotericin B were performed, but they were not effective in improving the condition of the patient. Revision open surgery was then performed. A follow-up brain enhancement computerized tomography showed an enlarged mass volume and hydrocephalus with periventricular enhancement. As an additional procedure, ventriculoperitoneal shunt and tuberculosis medication were administered. About 2 weeks later, clinical symptoms and radiologic findings improved. We present a case of intra-cranial IPT and discuss further treatment methods.
Amphotericin B ; Biopsy ; Brain ; Cavernous Sinus ; Central Nervous System ; Combined Modality Therapy* ; Dexamethasone ; Drug Therapy ; Follow-Up Studies ; Granuloma ; Granuloma, Plasma Cell* ; Humans ; Hydrocephalus ; Middle Aged ; Orbit ; Skull Base* ; Skull* ; Sphenoid Sinus ; Tuberculosis ; Ventriculoperitoneal Shunt

We report a case of a 31-year-old woman with glioblastoma multiforme (GBM) in the pineal region with associated leptomeningeal dissemination and lumbar metastasis. The patient presented with severe headache and vomiting. Magnetic resonance imaging (MRI) of the brain showed a heterogeneously enhanced tumor in the pineal region with obstructive hydrocephalus. After an urgent ventricular-peritoneal shunt, she was treated by subtotal resection and chemotherapy concomitant with radiotherapy. Two months after surgery, MRI showed no changes in the residual tumor but leptomeningeal dissemination surrounding the brainstem. One month later, she exhibited severe lumbago and bilateral leg pain. Thoracico-lumbar MRI showed drop like metastasis in the lumbar region. Finally she died five months after the initial diagnosis. Neurosurgeons should pay attention to GBM in the pineal region, not only as an important differential diagnosis among the pineal tumors, but due to the aggressive features of leptomeningeal dissemination and spinal metastasis.
Adult ; Brain ; Brain Stem ; Diagnosis ; Diagnosis, Differential ; Drug Therapy ; Female ; Glioblastoma* ; Headache ; Humans ; Hydrocephalus ; Leg ; Low Back Pain ; Lumbosacral Region ; Magnetic Resonance Imaging ; Neoplasm Metastasis* ; Neoplasm, Residual ; Pinealoma ; Radiotherapy ; Vomiting

Primary spinal cord melanoma is a rare central nervous system malignant tumor. Usually it resembles an intradural extramedullary (IDEM) nerve sheath tumor or melanoma. We experienced a patient with upper thoracic primary IDEM spinal cord melanoma who was diagnosed to be with hydrocephalus and without intracranial lesions. Initial symptoms of the patient were related to the hydrocephalus and the primary spinal cord melanoma was diagnosed eight months later. At the first operation, complete resection was impossible and the patient refused additional radiotherapy or chemotherapy. At 22 months after surgery, the patient revisited our institution with recurrent both leg weakness. Leptomeningeal dissemination was present in the whole spinal cord and only partial resection of tumor was performed. The symptoms slightly improved after surgery. Primary spinal cord melanoma is extremely rare but complete resection and additional radiotherapy or chemotherapy can prolong the disease free interval. Hydrocephalus or signs of increased intracranial pressure may be the diagnostic clue of spinal cord malignancy and progression.
Central Nervous System ; Drug Therapy ; Humans ; Hydrocephalus* ; Intracranial Pressure ; Leg ; Melanoma* ; Radiotherapy ; Spinal Cord Neoplasms ; Spinal Cord* ; Spine*

The major aims of this study were to estimate the infection rate and recognize the risk factor for ventriculoperitoneal (VP) shunt infections in children. To analyze shunt infection rate and identify risk factors, a retrospective cohort analysis of 333 consecutive VP shunt series was performed at Seoul National University Children's Hospital in Korea between January 2005 and February 2011. Overall, 35 shunts (10.5%) were infected, which represented an infection rate of 0.075 infection cases per shunt per year. VP shunt infection occurred at a median of 1 month (range, 6 days to 8 months) after insertion. An independent risk factor for shunt infection was undergoing an operation before the first year of life (relative risk 2.31; 95% confidence interval, 1.19-4.48). The most common causative microorganism was coagulase-negative staphylococci in 16 (45.7%) followed by Staphylococcus aureus in 8 (22.9%). Methicillin resistance rate was 83.3% among coagulase-negative staphylococci and S. aureus. In this study, cerebrospinal fluid shunt infection rate was 10.5%. Infection was frequently caused by methicillin-resistant coagulase-negative staphylococci and S. aureus within two months after shunt surgery. Vancomycin may be considered as the preoperative prophylaxis for shunt surgery in a situation where methicillin resistance rate is very high.
Anti-Bacterial Agents/therapeutic use ; Bacterial Infections/drug therapy/*epidemiology/microbiology ; Child ; Child, Preschool ; Cohort Studies ; Humans ; Hydrocephalus/surgery ; Incidence ; Infant ; Infant, Newborn ; Male ; Methicillin-Resistant Staphylococcus aureus/isolation & purification ; Retrospective Studies ; Risk Factors ; Staphylococcus/isolation & purification ; Surgical Wound Infection/drug therapy/*epidemiology/etiology ; Vancomycin/therapeutic use ; Ventriculoperitoneal Shunt/*adverse effects