Intracranial Hypertension Syndrome in Systemic Lupus Erythematosus: Clinical Analysis and Review of the Literature

In order to better understand the clinical manifestation of systemic lupus erythematosus （SLE） with intracranial hypertension syndrome （IHS）, we analyzed the clinical features and treatment of a typical SLE patient with IHS. SLE is one of the most unpredictable autoimmune diseases involving multiple organ systems that is defined clinically and associated with antibodies directed against cell nuclei. IHS is an uncommon manifestation of neuropsychiatric SLE （NPSLE） and is characterized by an elevated intracranial pressure, papilledema, and headache with occasional abducens nerve paresis, absence of a space-occupying lesion or ventricular enlargement, and normal cerebrospinal fluid chemical and hematological constituents. IHS has been reported in a few sporadic cases in patients with SLE worldwide, but rarely has been reported in China. In this study, a 34-year-old female SLE patient with IHS was. reported and pertinent literature reviewed. The clinical presentation, image logical features, and investigatory findings were discussed.

Pharmacological potential of acetazolamide in traumatic intracranial hypertension

Traumatic brain injuries are an important cause of morbidity and mortality around the world. These types of lesions are often associated with increased intracranial pressure and cerebral edema, proper management of this can reduce tissue damage of the brain and improve brain perfusion. The use of acetazolamide is not indicated in guidelines for the management of intracranial hypertension, which is used to a great extent for the management of idiopathic intracranial hypertension. However, it is not yet known in the management of traumatic intracranial hypertension.

Idiopathic Intracranial Hypertension without Papilloedema “Case Report”

Purpose: The purpose of this case report is to share and spread the awareness of diagnosing and managing idiopathic intracranial hypertension in atypical scenarios as in this case, who presented with multiple other medical conditions, and had no papilloedema. The presenting signs and symptoms of mere headache were misleading. The other medical conditions could have explained the patient’s headache. These included cervical spine osteoporosis, and middle ear otolith disease. This case is unique because she was found to have IIH without papilloedema, and despite having multiple cancers in the uterus, breasts, and recently the thyroid, she had no malignant cells in the CSF. The patient was treated for IIH, and her malignancies were treated separately by the corresponding physicians. Method: The patient was assessed in 2019, and was followed up closely till 2021, in collaboration with her neurologist, oncologist, and orthopedist. Result: The patient had IIH after her neurologist attempted a lumbar puncture, which showed high CSF pressure. Conclusion: Headache is a common symptom presented by patients of different ages and backgrounds. The absence of papilloedema does not exclude the diagnosis of IIH. When possible etiologies for headache are ruled out (e.g. middle and inner ear infections, anemia, intracranial tumors, glaucoma, etc.), chronic headaches that do not respond to medications, and present with visual field changes may require a lumbar puncture.

Shunting,optic nerve sheath fenestration and dural venous stenting for medically refractory idiopathic intracranial hypertension:systematic review and meta-analysis

Background:Cerebrospinal fluid(CSF)-diversion procedures have traditionally been the standard of treatment for patients with medically refractive idiopathic intracranial hypertension(IIH).However,dural venous sinus stent(VSS)placement has been described as a safe and effective procedure for the management of medically refractive IIH.We performed a meta-analysis comparing outcomes and complications of CSF-diversion procedures,VSS and optic nerve sheath fenestration(ONSF)for the treatment of medically refractive IIH.Methods:Electronic searches were performed using six databases from 1988 to January 2017.Data was extracted and meta-analysed from the identified studies.Results:From 55 pooled studies,there were 538 CSF-diversion cases,224 dural venous stent placements,and 872 ONSF procedures.Similar improvements were found in terms of postoperative headaches(CSF vs.VSS vs.ONSF:84%vs.78%vs.62%,P=0.223),papilledema(CSF vs.VSS vs.ONSF:71%vs.86%vs.77%,P=0.192),whilst visual acuity changes favored venous stenting(CSF vs.VSS vs.ONSF:55%vs.69%vs.44%,P=0.037).There was a significantly lower rate of subsequent procedures with venous stent placement(CSF vs.VSS vs.ONSF:37%vs.13%vs.18%,P<0.001),but other complication rates were similar(CSF vs.VSS vs.ONSF:13%vs.8%vs.14%,P=0.28).Subgroup analysis of lumbar-peritoneal vs.ventriculoperitoneal shunts found no differences in symptom improvements,complications and subsequent procedure rates.Conclusions:Our findings suggest that dural venous sinus stenting may be a viable alternative to traditional surgical interventions in patients who are refractory to medical treatment.

Optical coherence tomography use in idiopathic intracranial hypertension

Idiopathic intracranial hypertension(IIH)is a condition in which elevated pressure in the cerebrospinal fluid can lead to optic nerve head(ONH)dysfunction and subsequent visual impairment.Physicians are currently limited in their ability to monitor and manage this condition,as clinical symptoms and exam findings are often delayed in response to changes in intracranial pressure.In order to find other biomarkers of disease,researchers are using imaging modalities such as optical coherence tomography(OCT)to observe microscopic changes in the eye in this condition.OCT can create 2-dimensional and 3-dimensional high definition images of the retina of the ONH and has been used to study various conditions such as glaucoma and multiple sclerosis.Numerous studies have used OCT in IIH as well,and they have shown that certain retinal layers and the ONH change in thickness and shape in both the short and long term with intracranial pressure changes.OCT is a promising modality for clinical and scientific evaluation of IIH as it is a noninvasive and practical tool to obtain in depth images.This review will discuss how OCT can be used to assess a patient with IIH,both before and after treatment,along with its limitations and future applications.

Biological characteristics of the cerebral venous system and its hemodynamic response to intracranial hypertension

Background The role of the cerebral venous system （CVS） in intracranial pressure （ICP） regulation remains largely unclear. In the present study, the interaction between ICP and the cerebral venous system and its possible mechanism were investigated with respect to the biological characteristics of the cerebral venous system and its hemodynamic response under increased ICP. Methods We created intracranial hypertension animal model, measured and calculated the venous flow velocity and diameter of the outflow terminal of the CVS with color ultrasonic system and recorded the vascular morphology by 3-dimensional anatomical microscopy. Patients who suffered from raised ICP underwent MRI and digital subtraction angiography （DSA） examination to show the length in the vertical direction of the wall of the bridging vein representing the diameter value. Pathological autopsy was performed from bodies of patients who had died from non-cerebral causes to observe the juncture part between the venous sinuses and tributary vertical brain veins. Results Under increased ICP conditions, venous drainage through the outlet cuff segment, a unique structure between the bridge vein and sinus, was obstructed and in turn venous blood became congested. Therefore, the increased blood volume worsened the pre-existing ICP according to the well-accepted theory regarding volume-pressure relationship. This phenomenon was described as concurrent ＇k, enogenic intracranial hypertension＂, which is characterized by intracranial venous blood stasis responsive to and together with the original increased ICP. Conclusions The existence of this special pathophysiological process is prevalent, rather than rare, in various intracraniAI disorders. Thi.~ findinn would definitAIv nrovide new insinht into the. Area of cerebral venous svstem research.

Intracranial pressure monitoring in the perioperative period of patients with acute liver failure undergoing orthotopic liver transplantation

Acute liver failure(ALF)may result in severe neurological complications caused by cerebral edema and elevated intracranial pressure(ICP).Multiple pathogenic mechanisms explain the elevated ICP,and newer hypotheses have been described.While invasive ICP monitoring(ICPM)may have a role in ALF management,these patients are typically coagulopathic and at risk for intracranial hemorrhage.ICPM is the subject of much debate,and significant heterogeneity exists in clinical practice regarding its use.Contemporary ICPM techniques and coagulopathy reversal strategies may be associated with a lower risk of hemor-rhage;however,most of the evidence is limited by its retrospective nature and relatively small sample size.

Should a VP or LP Shunt be Used for the Treatment of Pseudotumorcerebri in Adults?

Introduction: Pseudotumorcerebri(PTC) is a condition characterized by false brain tumor symptoms, caused by high intracranial pressure (ICP). Treatment options include medication, weight loss, surgery, and shunting. Shunting, either ventriculoperitoneal (VP) or lumboperitoneal (LP), emerged as the preferred method of treatment, but there is an ongoing debate as to which technique should be prioritized. The aim of this study is to gather additional evidence to determine the optimal type of shunt for treating PTC. Materials and Methods: Ninety patients with PTC were studied at Damascus University between 2016 and 2021. The study monitored symptoms before and after treatment, with improvement related to the technique used (VP or LP shunts). Of all patients, 83 were women and 7 were men. In addition, complications were analyzed. Results: Both shunts showed similar postoperative rates of symptom improvement, but VP shunts were utilized more frequently overall in this study. Patients who received LP shunt surgery had a higher rate of postoperative complications compared to those who received VP shunt surgery, but the chi-squared analysis did not provide sufficient evidence to confirm a significant relationship between the type of surgery and the occurrence of postoperative complications. Conclusion: Despite ongoing controversy about the optimal treatment for benign intracranial hypertension (BTC), most authors approved the trend of using VP (ventriculoperitoneal) shunts, given a lower rate of complications. However, there is no statistically significant difference between outcomes of VP and LP (lumboperitoneal) shunting techniques, according to our research.

Treatment of malignant glioma using hyperthermia

Thirty pathologically diagnosed patients with grade Ⅲ-Ⅳ primary or recurrent malignant glioma （tumor diameter 3-7 cm） were randomly divided into two groups. The control group underwent conventional radiotherapy and chemotherapy. In the hyperthermia group, primary cases received hyperthermia treatment, and patients with recurrent tumors were treated with hyperthermia in com- bination with radiotherapy and chemotherapy. Hyperthermia treatment was administered using a 13.56-MHz radio frequency hyperthermia device. Electrodes were inserted into the tumor with the aid of a CT-guided stereotactic apparatus and heat was applied for 1 hour. Dudng 3 months a^er hyperthermia, patients were evaluated with head CT or MRI every month. Gliomas in the hyper- thermia group exhibited growth retardation or growth termination. Necrosis was evident in 80% of the heated tumor tissue and there was a decrease in tumor diameter. Our findings indicate that ra- dio frequency hyperthermia has a beneficial effect in the treatment of malignant glioma.

Murine model to study brain,behavior and immunity during hepatic encephalopathy

AIM:To propose an alternative model of hepatic encephalopathy(HE) in mice,resembling the human features of the disease.METHODS:Mice received two consecutive intraperitoneal injections of thioacetamide(TAA) at low dosage(300 mg/kg).Liver injury was assessed by serum transaminase levels(ALT) and liver histology(hematoxylin and eosin).Neutrophil infiltration was estimated by confocal liver intravital microscopy.Coagulopathy was evaluated using prolonged prothrombin and partial thromboplastin time.Hemodynamic parameters were measured through tail cuff.Ammonia levels were quantified in serum and brain samples.Electroencephalography(EEG) and psychomotor activity score were performed to show brain function.Brain edema was evaluated using magnetic resonance imaging.RESULTS:Mice submitted to the TAA regime developed massive liver injury,as shown by elevation of serum ALT levels and a high degree of liver necrosis.An intense hepatic neutrophil accumulation occurred in response to TAA-induced liver injury.This led to mice mortality and weight loss,which was associated with severe coagulopathy.Furthermore,TAA-treated mice presented with increased serum and cerebral levels of ammonia,in parallel with alterations in EEG spectrum and discrete brain edema,as shown by magnetic resonance imaging.In agreement with this,neuropsychomotor abnormalities ensued 36 h after TAA,fulfilling several HE features observed in humans.In this context of liver injury and neurological dysfunction,we observed lung inflammation and alterations in blood pressure and heart rate that were indicative of multiple organ dysfunction syndrome.CONCLUSION:In summary,we describe a new murine model of hepatic encephalopathy comprising multiple features of the disease in humans,which may provide new insights for treatment.

An Unusual Cause of Irritability in a Single Ventricle Patient after Bidirectional Glenn Shunt

The differential diagnosis for irritability in children is broad.In patients with congenital heart disease,one must strongly consider cardiac etiologies such as low cardiac output or elevated central venous pressure(CVP).In patients with single-ventricle physiology,the second stage of palliation includes bidirectional Glenn,which involves anastomosis of the superior vena cava to the pulmonary artery resulting in volume offloading of the single systemic ventricle.Typically,early in the post-operative period,patients may experience a headache due to the acute increase in CVP,and symptoms improve over time.Idiopathic intracranial hypertension(IIH),also known as pseudotumor cerebri,is a rare neurologic disorder in children,characterized by raised intracranial pressure(ICP)in the absence of brain parenchymal lesions or cerebrospinal fluid(CSF)abnormalities.While the pathogenesis of IIH is unknown,early recognition and treatment of IIH are important to prevent permanent vision loss.There are only rare reports of IIH in patients with Fontan circulation.To our knowledge,we report the first case of IIH in a 2-year-old female after bidirectional Glenn.

Sonographic Measurement of the Optic Nerve Sheath Diameter (ONSD) and Eyeball Transversal Diameter (ETD) in Benin Healthy African Adults

Background: Measuring optic nerve sheath diameter (ONSD), a relatively re-cent technique, allows an indirect and non-invasive diagnosis of intracranial hypertension. The ONSD ratio to eyeball transverse diameter (ETD) increases this reliability of the technique. The objective of this study was to determine the normal ONSD and its ratio with ETD in black African adults in Benin. Methods: A descriptive cross-sectional study was conducted between May 2019 and August 2019. Ultrasound ONSD and ONSD/ETD ratio were meas-ured in 210 healthy adults received in the medical imaging department of the Borgou and Alibori University Hospital Center in Benin. The ONSD corre-sponded to the average of 12 measurements (03 horizontal and 03 vertical for each eye) taken 3 mm behind the papilla. The transverse ETD corresponded to the average of 03 measurements. Results: The mean ONSD was 4.20 ± 0.28 mm (95% CI = [4.17;4.24] mm and 95th percentile = 4.70 mm). The mean ETD was 24.25 ± 1.10 mm. The ONSD/ETD ratio was 0.17 ± 0.01 (95% CI = [0.17;0.17] and 95th percentile = 0.19). There was no difference between ver-tical and horizontal ONSD and between right and left ONSD. The ONSD and the ONSD/ETD ratio were not correlated with any of the socio-demographic and anthropometric characteristics studied (sex, age, ethnicity, weight, height, body mass index, head circumference and waist circumference). The ONSD was associated with ETD (p < 0.001 and r = 0.55). Conclusion: An ONSD and an ONSD/ETD ratio respectively greater than 4.70 mm and 0.19 should sus-pect intracranial hypertension. The ONSD/ETD ratio may be a better indicator of HICT because of its small variation.

Pediatric neuro-ophthalmology:not simply neuro-ophthalmology for small adults

Pediatric neuro-ophthalmology is a subspecialty within neuro-ophthalmology.Pediatric neuro-ophthalmic diseases must be considered separate from their adult counterparts,due to the distinctive nature of the examination,clinical presentations,and management choices.This manuscript will highlight four common pediatric neuro-ophthalmic disorders by describing common clinical presentations,recommended management,and highlighting recent developments.Diseases discussed include pediatric idiopathic intracranial hypertension(IIH),pseudopapilledema,optic neuritis(ON)and optic pathway gliomas(OPG).The demographics,diagnosis and management of common pediatric neuro-ophthalmic disease require a working knowledge of the current research presented herein.Special attention should be placed on the differences between pediatric and adult entities such that children can be appropriately diagnosed and treated.

Empty Sella Turcica Syndrome: About a Case Revealed by Adrenal Insufficiency

Background: Empty sella turcica corresponds to an arachnocele which may be complete or partial, primary or secondary. The primary form is rarer. Its cause is unknown, but etiological factors have been identified. The prevalence of this condition is variable, depending on techniques of diagnosis. It may be detected fortuitously on imaging or in the presence of neurological, ocular or endocrine manifestations. Aim: We aim to report a case of empty sella turcica syndrome revealed by adrenal insufficiency in a young African black woman. Case presentation: It was an overweight young woman with signs of intracranial hypertension associated with central adrenal insufficiency. The diagnosis of empty sella was confirmed by magnetic resonance imaging. Secondarily, she developed a hypo metabolism syndrome and biology confirmed central hypothyroidism. After correction of the adrenal cortical deficit, the treatment of the thyroid deficit was administered. Evolution was favourable clinically and biologically. Conclusion: The empty sella turcica is rare and its revelation by a central adrenal insufficiency is even more. The case we report is a perfect illustration.

Hypertensive intracranial hematomas: endoscopic-assisted keyhole evacuation and application of patent viewing dissector

To study the effect of endoscopic assisted keyhole operation (EAKO) on treating hypertensive intracranial hematomas and the value of our patent dissector appli ed during the operation Methods A total of 25 patients with hypertensive intracranial hematomas underwent endos copic assisted keyhole evacuation, during which, the viewing dissector, which h ad recently achieved national patent, was connected to the tip of endoscope and used to help dissect hematomas The outcome of this procedure were compared wit h those of 22 comparable cases undergone conventional surgical treatment (large or smaller craniotomy) The items for comparison included the volum e of remaining hematoma, the duration of operation, postsurgical Glasgow Coma Sc ale (GCS) and Glasgow Outcome Scale (GOS) Results Remaining hematoma was ascertained 48 h after operation with the use of comp uterized tomography (CT) scans In the case of EAKO, nearly complete evacuation (>84%) was achieved in 21 cases; GCS was evaluated at 7 d postsurgery result ing in GCS >12 in 9 patients, GCS 9-12 in 12 patients and GCS <9 in 4 patients The follow up period ranged from 6 to 21 mon GOS was estimated at half a year and good recovery rate as defined by GOS was assigned to 76% of the EAKO pa tients There are significant differences in the volumes of remaining hematomas and the duration of operation between the EAKO and craniotomy group ( P <0 0 5) In addition, better clinical outcomes were obtained in EAKO Conclusion EAKO has the advantage of being minimally invasive, improving surgical results a nd the prognosis of hypertensive intracranial hematoma patients We conclude th at keyhole operation is a safe, effective alternative for removal of hypertensiv e intracranial hematoma, particularly during acute stages

Intensive Care Management of Acute Liver Failure:Considerations While Awaiting Liver Transplantation

Acute liver failure is a unique clinical phenomenon charac-terized by abrupt deterioration in liver function and altered mentation.The development of high-grade encephalopathy and multisystem organ dysfunction herald poor prognosis.Etiologic-specific treatments and supportive measures are routinely employed;however,liver transplantation remains the only chance for cure in those who do not spontaneously recover.The utility of artificial and bioartificial assist therapies as supportive care—to allow time for hepatic recovery or as a bridge to liver transplantation—has been examined but stud-ies have been small,with mixed results.Given the severity of derangements,intensive critical care is needed to success-fully bridge patients to transplant,and evaluation of candi-dates occurs rapidly in parallel with serial reassessments of operative fitness.Psychosocial assessment is often subopti-mal and relative contraindications to transplant,such as ventilator-dependence may be overlooked.While often em-ployed to guide evaluation,no single prognostic model dis-criminates those who will spontaneously recover and those who will require transplant.The purpose of this review will be to summarize approaches in critical care,prognostic model-ing,and medical evaluation of the acute liver failure trans-plant candidate.

Diagnose and treatment of traumatic pleural-subarachnoid fistula in children: A case report and systematic review

Background:Pleural-subarachnoid fistula(PSF)is a rare disease that is difficult to diagnose and treat.Secondary intracranial hypertension and the treatment are seldom mentioned previously among PSF.Case presentation:A 1-year-old boy diagnosed PSF developed into secondary intracranial hypertension after conservative treatment.He was finally cured by down-step treatment of mannitol,avoiding form ventricleperitoneal shunt.Then,we reviewed the literature of pleural-subarachnoid fistula.Fifty-six cases have been reported so far.Most of the cases(51.8%)were caused by surgery;only 17.9%were caused by car accidents.Regarding the treatment,half of the cases cured by surgery and the other by conservative measures.Our case is the first one involving secondary intracranial hypertension and cured by down-step treatment of mannitol.Conclusions:A comprehensive examination should be performed before the treatment to avoid any inappropriate medical strategies.Secondary acute intracranial hypertension may be cured by down step treatment of mannitol,evading from the long-term ventriculoperitoneal shunt.

Decompressive craniectomy in herpes simplex encephalitis

Intracranial hypertension is a common cause of morbidity in herpes simplex encephalitis(HSE).HSE is the most common form of acute viral encephalitis.Hereby we report a case of HSE in which decompressive craniectomy was performed to treat refractory intracranial hypertension.A 32-year-old male presented with headache,vomiting,fever,and focal seizures involving the right upper limb.Cerebrospinal fluid-meningoencephalitic profile was positive for herpes simplex.Magnetic resonance image of the brain showed swollen and edematous right temporal lobe with increased signal in gray matter and subcortical white matter with loss of gray,white differentiation in T2-weighted sequences.Decompressive craniectomy was performed in view of refractory intracranial hypertension.Decompressive surgery for HSE with refractory hypertension can positively affect patient survival,with good outcomes in terms of cognitive functions.