OBJECTIVE: Shunt-dependent hydrocephalus (SdHCP) is a well-known complication of aneurysmal subarachnoid hemorrhage (SAH). The risk factors for SdHCP have been widely investigated, but few risk scoring systems have been established to predict SdHCP. This study was performed to investigate the risk factors for SdHCP and devise a risk scoring system for use before aneurysm obliteration.METHODS: We reviewed the data of 301 consecutive patients who underwent aneurysm obliteration following SAH from September 2007 to December 2016. The exclusion criteria for this study were previous aneurysm obliteration, previous major cerebral infarction, the presence of a cavum septum pellucidum, a midline shift of >10 mm on initial computed tomography (CT), and in-hospital mortality. We finally recruited 254 patients and analyzed the following data according to the presence or absence of SdHCP : age, sex, history of hypertension and diabetes mellitus, Hunt-Hess grade, Fisher grade, aneurysm size and location, type of treatment, bicaudate index on initial CT, intraventricular hemorrhage, cerebrospinal fluid drainage, vasospasm, and modified Rankin scale score at discharge.RESULTS: In the multivariate analysis, acute HCP (bicaudate index of ≥0.2) (odds ratio [OR], 6.749; 95% confidence interval [CI], 2.843–16.021; p=0.000), Fisher grade of 4 (OR, 4.108; 95% CI, 1.044–16.169; p=0.043), and an age of ≥50 years (OR, 3.938; 95% CI, 1.375–11.275; p=0.011) were significantly associated with the occurrence of SdHCP. The risk scoring system using above parameters of acute HCP, Fisher grade, and age (AFA score) assigned 1 point to each (total score of 0–3 points). SdHCP occurred in 4.3% of patients with a score of 0, 8.5% with a score of 1, 25.5% with a score of 2, and 61.7% with a score of 3 (p=0.000). In the receiver operating characteristic curve analysis, the area under the curve (AUC) for the risk scoring system was 0.820 (p=0.080; 95% CI, 0.750–0.890). In the internal validation of the risk scoring system, the score reliably predicted SdHCP (AUC, 0.895; p=0.000; 95% CI, 0.847–0.943).CONCLUSION: Our results suggest that the herein-described AFA score is a useful tool for predicting SdHCP before aneurysm obliteration. Prospective validation is needed.
Aneurysm ; Cerebral Infarction ; Cerebrospinal Fluid Leak ; Diabetes Mellitus ; Hemorrhage ; Hospital Mortality ; Humans ; Hydrocephalus ; Hypertension ; Multivariate Analysis ; Prospective Studies ; Risk Factors ; ROC Curve ; Septum Pellucidum ; Subarachnoid Hemorrhage ; Ventriculoperitoneal Shunt

Eosinophilic granulomatosis with polyangiitis (EGPA) is an immune related systemic disease that is caused by vasculitis affecting multiple organ systems. It is characterized by asthma, fever, eosinophilia, cardiac problems, renal injury, and peripheral neuropathy. In this report, we describe a patient with EGPA with concurrent cerebral infarction and acute polyneuropathy mimicking a Guillain-Barre syndrome (GBS). A 46-year-old man presented with rapidly progressing gait disturbance, muscular weakness, and tingling sensation in all four limbs. A nerve conduction study revealed sensorimotor polyneuropathy in all four limbs, and a test of the cerebrospinal fluid showed an albumin-cytologic dissociation. In addition, brain magnetic resonance imaging (MRI) using fluid-attenuated inversion recovery and diffusion weighted MRI revealed high signal intensity lesions with gadolinium enhancement on T1-weighted MRI in the right caudate nucleus. After performing laboratory tests, paranasal sinus computed tomography, and a nasal smear, the patient was diagnosed with EGPA and treated with high dose glucocorticoid and oral cyclophosphamide. In conclusion, our findings indicate that a diagnosis of EGPA should be considered when a patient presents with rapidly progressing polyneuropathy mimicking a GBS along with unusual systemic symptoms or brain lesions.
Asthma ; Brain ; Caudate Nucleus ; Cerebral Infarction ; Cerebrospinal Fluid ; Churg-Strauss Syndrome ; Cyclophosphamide ; Diagnosis ; Diffusion Magnetic Resonance Imaging ; Eosinophilia ; Eosinophils* ; Extremities ; Fever ; Gadolinium ; Gait ; Granulomatosis with Polyangiitis* ; Guillain-Barre Syndrome ; Humans ; Magnetic Resonance Imaging ; Middle Aged ; Muscle Weakness ; Neural Conduction ; Peripheral Nervous System Diseases ; Polyneuropathies* ; Sensation ; Vasculitis ; Vasculitis, Central Nervous System*

Contrast-induced encephalopathy after cerebral angiography is a rare complication and until now, only few cases have been reported. This paper reports on contras-induced encephalopathy mimicking meningoencephalitis after cerebral angiography by using iodixanol, an iso-osmolar non-ionic contrast agent. A 58-year-old woman underwent cerebral angiography for the evaluation of multiple intracranial aneurysms. A few hours later, she had persistent headache, vomiting, fever, and seizures. Brain computed tomography (CT) showed sulcal obliteration of right cerebral hemisphere and cerebrospinal fluid profile was unremarkable. The next day, she developed left side hemiparesis, sensory loss, and left-sided neglect with drowsy mentality. Brain magnetic resonance imaging (MRI) showed cerebral swelling with leptomeningeal enhancement in the right parieto-occipital lobe without sign of ischemia or hemorrhage. The patient was managed with intravenous dexamethasone, mannitol and anticonvulsant. There was a progressive neurological improvement with complete resolution of the symptoms at day 6. This observation highlights that contrast-induced encephalopathy can be caused by an iso-osmolar non-ionic contrast agent. This rare entity should be suspected if neurologic deterioration after cerebral angiography is not explained by other frequent causes such as acute infarction or hemorrhage.
Angiography ; Brain ; Brain Diseases* ; Cerebral Angiography* ; Cerebrospinal Fluid ; Cerebrum ; Dexamethasone ; Female ; Fever ; Headache ; Hemorrhage ; Humans ; Infarction ; Intracranial Aneurysm ; Ischemia ; Magnetic Resonance Imaging ; Mannitol ; Meningoencephalitis ; Middle Aged ; Paresis ; Seizures ; Vomiting

Cerebral amyloid angiopathy (CAA) involves cerebrovascular amyloid deposition and is classified into several types according to the amyloid protein involved. Of these, sporadic amyloid beta-protein (Abeta)-type CAA is most commonly found in older individuals and in patients with Alzheimer's disease (AD). Cerebrovascular Abeta deposits accompany functional and pathological changes in cerebral blood vessels (CAA-associated vasculopathies). CAA-associated vasculopathies lead to development of hemorrhagic lesions [lobar intracerebral macrohemorrhage, cortical microhemorrhage, and cortical superficial siderosis (cSS)/focal convexity subarachnoid hemorrhage (SAH)], ischemic lesions (cortical infarction and ischemic changes of the white matter), and encephalopathies that include subacute leukoencephalopathy caused by CAA-associated inflammation/angiitis. Thus, CAA is related to dementia, stroke, and encephalopathies. Recent advances in diagnostic procedures, particularly neuroimaging, have enabled us to establish a clinical diagnosis of CAA without brain biopsies. Sensitive magnetic resonance imaging (MRI) methods, such as gradient-echo T2* imaging and susceptibility-weighted imaging, are useful for detecting cortical microhemorrhages and cSS. Amyloid imaging with amyloid-binding positron emission tomography (PET) ligands, such as Pittsburgh Compound B, can detect CAA, although they cannot discriminate vascular from parenchymal amyloid deposits. In addition, cerebrospinal fluid markers may be useful, including levels of Abeta40 for CAA and anti-Abeta antibody for CAA-related inflammation. Moreover, cSS is closely associated with transient focal neurological episodes (TFNE). CAA-related inflammation/angiitis shares pathophysiology with amyloid-related imaging abnormalities (ARIA) induced by Abeta immunotherapies in AD patients. This article reviews CAA and CAA-related disorders with respect to their epidemiology, pathology, pathophysiology, clinical features, biomarkers, diagnosis, treatment, risk factors, and future perspectives.
Alzheimer Disease ; Amyloid ; Amyloid beta-Peptides ; Biomarkers ; Biopsy ; Blood Vessels ; Brain ; Cerebral Amyloid Angiopathy* ; Cerebrospinal Fluid ; Cerebrovascular Disorders ; Dementia ; Diagnosis ; Epidemiology ; Humans ; Immunotherapy ; Infarction ; Inflammation ; Leukoencephalopathies ; Ligands ; Magnetic Resonance Imaging ; Neuroimaging ; Pathology ; Plaque, Amyloid ; Positron-Emission Tomography ; Risk Factors ; Siderosis ; Stroke ; Subarachnoid Hemorrhage

OBJECTIVE: Removal of blood from subarachnoid space with a lumbar drainage (LD) may decrease development of cerebral vasospasm. We evaluated the effectiveness of a LD for a clinical vasospasm and outcomes after clipping of aneurysmal subarachnoid hemorrhage (SAH). METHODS: Between July 2008 and July 2013, 234 patients were included in this study. The LD group consisted of 126 patients, 108 patients in the non LD group. We investigated outcomes as follow : 1) clinical vasospasm, 2) angioplasty, 3) cerebral infarction, 4) Glasgow outcome scale (GOS) score at discharge, 5) GOS score at 6-month follow-up, and 6) mortality. RESULTS: Clinical vasospasm occurred in 19% of the LD group and 42% of the non LD group (p<0.001). Angioplasty was performed in 17% of the LD group and 38% of the non LD group (p=0.001). Cerebral infarctions were detected in 29% and 54% of each group respectively (p<0.001). The proportion of GOS score 5 at 6 month follow-up in the LD group was 69%, and it was 58% in the non LD group (p=0.001). Mortality rate showed 5% and 10% in each group respectively. But, there was no difference in shunt between the two groups. CONCLUSION: LD after aneurysmal SAH shows marked reduction of clinical vasospasm and need for angioplasty. With this technique we have shown favorable GOS score at 6 month follow-up.
Aneurysm ; Angioplasty ; Cerebral Infarction ; Cerebrospinal Fluid* ; Drainage* ; Follow-Up Studies ; Glasgow Outcome Scale ; Humans ; Mortality ; Subarachnoid Hemorrhage* ; Subarachnoid Space ; Surgical Instruments* ; Vasospasm, Intracranial*

Cases of incomplete Kawasaki disease (KD), wherein the patient does not fulfill the full diagnostic criteria for KD, are often detected in infants younger than 6 months of age. The clinical manifestations in infants with incomplete KD may resemble other infectious diseases, including meningitis. For this reason, clinicians may have difficulty differentiating incomplete KD from other infectious diseases in this population. Various neurological features are associated with KD, including aseptic meningitis, subdural effusion, facial nerve palsy, cerebral infarction, encephalopathy, and reversible corpus callosum splenial lesions on magnetic resonance imaging. We report a case of a 5-month-old girl with incomplete KD, associated with cerebrospinal fluid pleocytosis and an epidural fluid collection. Echocardiography indicated dilatation of the main coronary arteries. The girl made a complete recovery, with resolution of both the epidural fluid collection and coronary artery aneurysms. In this case, the child is well, and showed normal developmental milestones at the 7-month follow-up.
Aneurysm ; Cerebral Infarction ; Cerebrospinal Fluid* ; Child ; Communicable Diseases ; Coronary Vessels ; Corpus Callosum ; Dilatation ; Echocardiography ; Epidural Abscess ; Facial Nerve ; Female ; Follow-Up Studies ; Humans ; Infant* ; Leukocytosis* ; Magnetic Resonance Imaging ; Meningitis ; Meningitis, Aseptic ; Mucocutaneous Lymph Node Syndrome* ; Paralysis ; Subdural Effusion

Malignant cerebral edema following ischemic stroke is life threatening, as it can cause inadequate blood flow and perfusion leading to irreversible tissue hypoxia and metabolic crisis. Increased intracranial pressure and brain shift can cause herniation syndrome and finally brain death. Multiple randomized clinical trials have shown that preemptive decompressive hemicraniectomy effectively reduces mortality and morbidity in patients with malignant middle cerebral artery infarction. Another life-saving decompressive surgery is suboccipital craniectomy for patients with brainstem compression by edematous cerebellar infarction. In addition to decompressive surgery, cerebrospinal fluid drainage by ventriculostomy should be considered for patients with acute hydrocephalus following stroke. Medical treatment begins with sedation, analgesia, and general measures including ventilatory support, head elevation, maintaining a neutral neck position, and avoiding conditions associated with intracranial hypertension. Optimization of cerebral perfusion pressure and reduction of intracranial pressure should always be pursued simultaneously. Osmotherapy with mannitol is the standard treatment for intracranial hypertension, but hypertonic saline is also an effective alternative. Therapeutic hypothermia may also be considered for treatment of brain edema and intracranial hypertension, but its neuroprotective effects have not been demonstrated in stroke. Barbiturate coma therapy has been used to reduce metabolic demand, but has become less popular because of its systemic adverse effects. Furthermore, general medical care is critical because of the complex interactions between the brain and other organ systems. Some challenging aspects of critical care, including ventilator support, sedation and analgesia, and performing neurological examinations in the setting of a minimal stimulation protocol, are addressed in this review.
Analgesia ; Anoxia ; Brain ; Brain Death ; Brain Edema ; Brain Stem ; Cerebrospinal Fluid ; Coma ; Critical Care* ; Drainage ; Head ; Humans ; Hydrocephalus ; Hypothermia ; Infarction ; Infarction, Middle Cerebral Artery ; Intracranial Hypertension ; Intracranial Pressure ; Mannitol ; Mortality ; Neck ; Neurologic Examination ; Neuroprotective Agents ; Perfusion ; Stroke* ; Ventilators, Mechanical ; Ventriculostomy

We report here the case of a suprasellar cystic germ cell tumor (GCT) initially diagnosed as an arachnoid cyst. A 10-year-old boy experienced headache, dizziness, and diplopia, and was shown to have an approximately 2 cm suprasellar cyst. Two months after endoscopic third ventriculostomy was performed, a 5-6 cm cystic mass with an internal enhancing component was observed in the suprasellar cistern. Serum human chorionic gonadotropin levels were slightly increased in the serum and cerebrospinal fluid (55 and 162 IU/L, respectively) but were strikingly elevated in the cystic fluid (14,040 IU/L). The patient showed complete remission, with only a very small cystic lesion remaining after surgery, chemotherapy, and radiation treatment for a suprasellar mixed GCT. However, follow-up after treatment was complicated by moyamoya syndrome and cerebral infarction. GCT can be considered as a rare differential diagnosis in the case of a suprasellar cystic mass. Evaluation of tumor markers and close follow-up will be necessary.
Arachnoid* ; Central Nervous System Cysts* ; Cerebral Infarction ; Cerebrospinal Fluid ; Child ; Chorionic Gonadotropin ; Diagnosis, Differential ; Diplopia ; Dizziness ; Drug Therapy ; Follow-Up Studies ; Germ Cells* ; Headache ; Humans ; Male ; Moyamoya Disease ; Neoplasms, Germ Cell and Embryonal* ; Biomarkers, Tumor ; Ventriculostomy

This study was carried out to investigate the role of intrinsic neuroprotective mechanisms in the occurrence and development of vascular cognitive impairment (VCI) with the goal of providing a target for the treatment and prevention of VCI. Inpatients with proven cerebral infarction on cranial computed tomography (CT) were recruited as the ischemic cerebrovascular diseases (ICVD) group, and the patients with mixed stroke were excluded. In ICVD group, 12 patients were diagnosed as having VCI and served as VCI group. Inpatients undergoing surgical operation in our hospital were enrolled as control group. Double-antibody sandwich enzyme-linked immunosorbent assay (ELISA) was employed to detect the levels of hypoxia-inducible factor 1-alpha (HIF-1α), vascular endothelial growth factor (VEGF), nerve growth factor (NGF) and brain-derived neurotrophic factor (BDNF) in the cerebrospinal fluid of patients with ICVD. Associations between the levels of these factors and the Mini-Mental State Examination (MMSE) score were evaluated. In ICVD and VCI groups, the levels of HIF-1α and NGF in the cerebrospinal fluid were markedly lower than those in control group (P=0.037 and P=0.000; P=0.023 and P=0.005). In ICVD and VCI groups, the MMSE score was negatively related to VEGF level in the cerebrospinal fluid (r=-0.327, P=0.021; r=-0.585, P=0.046). In VCI group, HIF-1α level was correlated with NGF level (r=0.589, P=0.044). HIF-1α and NGF are involved in ischemic and hypoxic cerebral injury. The HIF signaling pathway plays an important role in intrinsic neuroprotection. Upregulation and maintenance of HIF-1α and NGF expression may attenuate VCI. Changes in VEGF levels are related to the occurrence and development of cognitive impairment.
Biomarkers ; cerebrospinal fluid ; Brain-Derived Neurotrophic Factor ; cerebrospinal fluid ; Cerebral Infarction ; cerebrospinal fluid ; complications ; diagnosis ; Cognition Disorders ; cerebrospinal fluid ; complications ; diagnosis ; Female ; Humans ; Hypoxia-Inducible Factor 1, alpha Subunit ; cerebrospinal fluid ; Male ; Middle Aged ; Nerve Growth Factor ; cerebrospinal fluid ; Reproducibility of Results ; Sensitivity and Specificity ; Vascular Endothelial Growth Factor A ; cerebrospinal fluid

The aim of the present study was to assess the clinical and histopathological findings in a canine model of ischemic stroke. Cerebral ischemic stroke was induced by middle cerebral artery occlusion in four healthy beagle dogs using silicone plugs. They showed neurological signs of forebrain dysfunction such as reduced responsiveness, head turning, circling, postural reaction deficits, perceptual deficits, and hemianopsia. These signs gradually regressed within 4 weeks without therapy. On magnetic resonance imaging, T2 hyperintensity and T1 hypointensity were found in the cerebral cortex and basal ganglia. These lesions were well-defined and sharply demarcated from adjacent brain parenchyma with a homogenous appearance. No abnormalities of the cerebrospinal fluid were observed. At necropsy, atrophic and necrotic lesions were observed in the cerebral cortex. The cerebral cortex, basal ganglia, and thalamus were partially unstained with triphenyl-tetrazolium chloride. Histopathologically, typical features of infarction were identified in cortical and thalamic lesions. This study demonstrates that our canine model resembles the conditions of real stroke patients.
Animals ; Behavior, Animal/physiology ; Brain/metabolism/pathology ; Cerebral Infarction/*etiology/*pathology ; Cerebrospinal Fluid/chemistry/cytology ; Disease Models, Animal ; *Dogs ; Infarction, Middle Cerebral Artery/*complications/*pathology ; Magnetic Resonance Imaging ; Male