Stereotactic Biopsy for Brainstem Lesions: A Meta-analysis with Noncomparative Binary Data.

OBJECTIVES: To evaluate the diagnostic yield and safety of brainstem stereotactic biopsy for brainstem lesions. METHODS: We performed a meta-analysis of English articles retrieved from the PubMed, Web of Science, Cochrane Library, and APA psycInfo databases up to May 12, 2021. A binary fixed-effect model, the inverse variance method, or a binary random-effect model, the Dersimonian Laird method, were utilized for pooling the data. This meta-analysis was registered with INPLASY, INPLASY202190034. FINDINGS: A total of 41 eligible studies with 2792 participants were included. The weighted average diagnostic yield was 97.0% (95% confidential interval [CI], 96.0-97.9%). The weighted average proportions of temporary complications, permanent deficits, and deaths were 6.2% (95% CI, 4.5-7.9%), .5% (95% CI, .2-.8%), and .3% (95% CI, .1-.5%), respectively. The subgroup analysis indicated a nearly identical weighted average diagnostic yield between MRI-guided stereotactic biopsy and CT-guided stereotactic biopsy (95.9% vs 95.8%) but slightly increased proportions of temporary complications (7.9% vs 6.0%), permanent deficits (1.9% vs .2%), and deaths (1.1% vs .4%) in the former compared to the latter. Moreover, a greater weighted average diagnostic yield (99.2% vs 97.6%) and lower proportions of temporary complications (5.1% vs 6.8%) and deaths (.7% vs 1.5%) were shown in the pediatric patient population than in the adult patient population. CONCLUSIONS: Brainstem stereotactic biopsy demonstrates striking accuracy plus satisfying safety in the diagnosis of brainstem lesions. The diagnostic yield, morbidity, and mortality mildly vary based on the diversity of assistant techniques and subject populations.

Rapidly progressive fatal idiopathic hypertrophic pachymeningitis with brainstem involvement in a child.

BACKGROUND: Hypertrophic pachymeningitis (HP) is a rare disorder characterized by diffuse thickening of the dura mater with resultant neurologic deficits. HP develops secondary to various conditions or idiopathically usually in adults but rarely in children. CASE REPORT: We describe a 3-year-old female child with idiopathic HP. Her HP involved the entire central nervous system with progression into the brainstem. The lesion responded poorly to pulsed steroids or any immunosuppressants. The brainstem lesion grew rapidly and formed various nodules that ultimately resulted in brain death. This is the first fatal case of HP in a child.

Stereotactic biopsy of brainstem lesions: 21 years experiences of a single center.

Stereotactic biopsies are procedures performed to obtain tumor tissue for diagnostic examinations. Cerebral lesions of unknown entities can safely be accessed and tissue can be examined, resulting in correct diagnosis and according treatment. Stereotactic procedures of lesions in highly eloquent regions such as the brainstem have been performed for more than two decades in our department. In this retrospective study we focus on results, approaches, modalities of anesthesia, and complications. We performed a retrospective analysis of our prospective database, including 26 patients who underwent stereotactic biopsy of the brainstem between April 1994 and June 2015. All of the patients underwent preoperative MRI. Riechert-Mundinger-frame was used before 2000, thereafter the Leksell stereotactic frame was used. After 2000 entry and target points were calculated by using BrainLab stereotactic system. We evaluated histopathological results as well as further treatment; additionally we compared complications of local versus general anesthesia and complications of a frontal versus a trans-cerebellar approach. Median age of all patients was 33 years, and median number of tissue samples taken was 12. In all patients a final histopathological diagnosis could be established. 5 patients underwent the procedure under local anesthesia, 21 patients in general anesthesia. In 19 patients a frontal approach was performed, while in 7 patients a trans-cerebellar approach was used. Complications occurred in five patients. Thereby no significant difference was found with regard to approach (frontal versus trans-cerebellar) or anesthesia (local versus general). Stereotactic biopsies even of lesions in the brainstem are a save way to obtain tumor tissue for final diagnosis, resulting in adequate treatment. Approach can be trans-cerebellar or frontal and procedure can be performed either under local or general anesthesia without significant differences concerning complication rate.

A posttraumatic pontomedullary rent with good outcome.

Posttraumatic pontomedullary rents have been described mainly as postmortem histopathological findings in patients who died immediately or within the first hours after trauma. To the best of our knowledge, no long-term survivors of this condition have been described, and those surviving initially were always severely impaired. We present the first patient with this condition and with corresponding lesions on imaging who survived longer than 3 months. Moreover, the patient regained almost complete independence 1 year after the trauma. We briefly discuss the proposed mechanisms of this injury. We conclude that this lesion, when incomplete, is not always lethal and can exceptionally have a good clinical outcome. Prevention of respiratory failure is of utmost importance in these patients.

Cerebral Cavernous Malformation Bleeding Following Cerebrospinal Fluid Diversion Surgery: A Case Report and Literature Review.

Cavernous malformations (CM) are rare intracerebral vascular lesions occurring in the brain, or less commonly in the spine, with an annual bleeding risk of up to 1.1%. These lesions can be occult or present to signs and symptoms based on location or, more frequently, are a result of hemorrhagic events. The most challenging aspect of managing these cases is weighing the risks and benefits of surgical treatment and intervening before the onset of a devastating hemorrhagic event. Here, we present the second case of CM haemorrhage following the cerebrospinal fluid (CSF) diversion procedure with a literature review of theories explaining this phenomenon. We present a 37-year-old female who has a known case of brainstem cavernoma and underwent left sub-temporal resection with stable residual since 2011, then was managed conservatively due to patient preference till she had a deterioration in December 2021 manifested as confusion, diplopia, dysarthria, and significant left sided weakness leaving her wheelchair bound. CT showed supratentorial hydrocephalus with extensive periventricular transependymal edema and no clear haemorrhage. A ventriculoperitoneal (VP) shunt was inserted, with no intraoperative complications. A few hours post-VP shunt insertion, she experienced a worsening in her mental status, hemiparesis, and dysarthria. Subsequent imaging found evidence of acute haemorrhage in the location of the previously noted residual. She was managed by supportive care. Causative factors of CM haemorrhage are poorly understood, and current data only suggest that prior haemorrhage and CM location could increase bleeding risk. Only one case of CM bleeding post-shunt insertion was reported; however, studies on other types of intracranial vascular lesions suggest that alterations in transmural pressure (including cerebrospinal fluid diversion procedures) can increase the risk of haemorrhage by changing the hemodynamic flow in these abnormally formed and weak vascular structures.

Locked-In Syndrome: A Rare Manifestation of Neuropsychiatric Lupus.

Neuropsychiatric systemic lupus erythematosus (SLE) is a rare condition that has a multitude of mechanisms resulting in the emergence of variable clinical presentations. We describe a peculiar case of a 33-year-old female with a history of SLE presented with two weeks of fever, headache, and vomiting. On admission, she became obtunded and was emergently intubated. Initial lumbar puncture revealed pleocytosis (46% neutrophils, 320 corrected nucleated cells/µL), elevated protein (244 mg/dL; normal, 15-40 mg/dL), normal glucose (63 mg/dL), and negative cultures. Empiric acyclovir, ampicillin, ceftriaxone, and vancomycin were initiated without clinical improvement. Neurological examination was notable for limited ability to follow commands, vertical nystagmus, horizontal gaze palsy, diffuse hyperreflexia, and quadriparesis. Electroencephalogram (EEG) was consistent with diffuse encephalopathy. Brain magnetic resonance imaging demonstrated restricted diffusion and contrast enhancement in the posterior and central pons with edema. A cerebral angiogram showed no signs of vasculitis. Treatment with intravenous (IV) methylprednisolone 1 g and IV immunoglobulin 2 g/kg was initiated for five days. Despite these interventions, no discernible clinical improvement was observed, prompting the commencement of 500 mg/m2 cyclophosphamide and daily maintenance of IV methylprednisolone at 2 mg/kg. A repeat MRI three weeks later revealed a marked reduction in the size of the lesion involving the pons. The patient also improved clinically over the month with successful extubation, complete return in mental capabilities, and the ability to ambulate short distances with assistance.

Treatment of brainstem and fourth ventricle lesions by the full neuroendoscopic telovelar approach.

OBJECTIVE: To explore the surgical techniques, advantages, and disadvantages of neuroendoscopic telovelar approach in the treatment of brainstem and fourth ventricle lesions. METHODS: The clinical data of 5 patients treated by neuroendoscopic telovelar approach from March 2020 to March 2022 were analyzed retrospectively. RESULTS: Among the 5 patients, there were 3 cavernous hemangiomas in pontine arm and 2 tumors in brainstem and fourth ventricle. All patients could successfully complete the operation, and 4 patients recovered well, other 1 patient discharged automatically for serious complications of other systems after the operation. CONCLUSION: The telovelar approach has gained popularity as a safe and effective strategy for lesions in fourth ventricular and brainstem. However, without removing the posterior arch of the atlas, it is difficult to enter the upper part of the fourth ventricle under a microscope. Transcranial neuroendoscopy can effectively compensate for the shortcomings of microscopy, whether used as an auxiliary measure for microsurgery or alone with proficient endoscopic techniques, it will provide greater application in minimally invasive surgery for fourth ventricle and brainstem lesions. By utilizing the excellent degree of freedom of transcranial neuroendoscopy, there is no need to open the posterior arch of the atlas, making the surgery more minimally invasive. However, the sample size of this study is small, and it was completed under the very mature neuroendoscopic technology of our team. Its general safety and practicality still require extensive clinical research validation.

Recurrent Stroke as a First Presentation in Behçet Disease: A Case Report.

Behçet disease (BD) is a multisystemic relapsing autoimmune vascular disorder. It is clinically characterized by recurrent oral ulcers, genital ulcers, eye, and skin manifestations. Development of neurological symptoms in BD cases is rare and occurs several years after the initial diagnosis. We describe a rare case of a 39-year-old Saudi male who presented with isolated neurological manifestations as the first sign of BD. The patient had recurrent strokes, both ischemic and hemorrhagic, over an 11-month period before developing typical BD features. A thorough investigation excluded other potential etiologies of his neurological disorders. Imaging showed multiple brainstem lesions compatible with parenchymal neuro-BD (NBD). The patient was positive for HLA-B51, a genetic marker linked to BD, but had a negative pathergy test. Treatment with corticosteroids and infliximab resulted in symptom improvement. The diagnosis of NBD requires a comprehensive clinical, imaging, and laboratory assessment to rule out other possible causes. This case demonstrates the need to include NBD in the differential diagnosis of young patients with unexplained neurological manifestations, especially if they are followed by an onset of BD features. Treatment with corticosteroids and biologic agents can achieve favorable outcomes. NBD can present with isolated neurological symptoms, emphasizing the need for a high level of suspicion and a multidisciplinary approach for accurate diagnosis and effective management.

Brainstem auditory evoked potential in cognitive impairment in patients with type 2 diabetes mellitus.

PURPOSE: Type 2 diabetes mellitus (T2DM) can cause mild cognitive impairment (MCI) which threatens the health of patients. So the diagnosis of MCI is particularly important. It is reported that brainstem auditory evoked potential (BAEP) is a sensitive tool to detect the brainstem function in patients with T2DM. This study aimed to investigate the relationship between BAEP and MCI in patients with T2DM. METHODS: A total of 244 T2DM patients with normal hearing, including 117 normal cognition patients and 127 MCI patients, were recruited in this cross-sectional study. Each subject underwent the BAEP examination. The diagnosis of MCI was based on the diagnostic guideline developed by the National Institute on Aging-Alzheimer's Association workgroups. The Montreal Cognitive Assessment (MoCA) was used to assess the cognitive function of the subjects. RESULTS: Compared with the normal cognition group, the patients in the MCI group had longer latencies of waves III and V and interpeak latencies (IPL) I-V in both ears (P < 0.05). The significant negative correlations were found between the latencies of waves III, V, IPL I-V, and MoCA score in both ears (P < 0.05). Logistic regression showed that the prolongations of latunits of waves III and V and IPL I-V in both ears were still associated with MCI after adjustment for mixed factors (P < 0.05). CONCLUSION: These results indicate abnormal auditory pathway in brainstem of T2DM patients with MCI. BAEP may contribute to the clinical diagnosis of MCI in patients with T2DM.

Cardiac Myxoma as a Rare Cause of an Ischemic Stroke of the Vertebrobasilar Territory in a Young Adult: A Case Report.

An ischemic stroke is characterized by the brutal installation of a focal functional deficit. Myxomas are the most common primitive cardiac tumors. Neurological manifestations of myxomas are mostly related to cardioembolic events, either caused by a migrating fragment of the tumor or by an attached clot that got detached from the tumor This article describes the case of a 55-year-old male who presented with an ischemic stroke of the vertebrobasilar territory. Cardiac exploration revealed the presence of a cardiac mass. The patient was surgically treated and the anatomopathological study confirmed the diagnosis of cardiac myxoma. This case emphasizes the obligation to research the etiology of ischemic strokes, and more importantly the realization of a complete cardiologic exploration.

Resection of Pontine Cavernoma with Hemorrhage via Unilateral Uvulotonsillar Keyhole Approach.

The telovelar approach and its modifications are widely used to remove lesions of the fourth ventricle and brainstem. We offer a variant of a unilateral uvulotonsillar approach, which is performed using an open-door keyhole suboccipital craniotomy (Video 1). A 56-year-old woman presented with symptoms of 2 months' duration. Preoperative examination revealed paresis of the left sixth cranial nerve, left seventh nerve paralysis, right-sided hemihypoesthesia, right-sided severe hemiparesis owing to which the patient could not move independently, and dynamic ataxia in the left limbs. Magnetic resonance imaging showed a cavernoma with hemorrhage in the left side of the pons. The surgery was performed in the prone position. After a median skin and soft tissue incision about 6-7 cm long, an open-door keyhole craniotomy was performed. Dura over the left cerebellar hemisphere was opened in a semilunar fashion. The dissection of the uvulotonsillar fissure was performed from its cranial part, where the medial trunk of the cortical segment of the posterior inferior cerebellar artery was the reference point. Following the dissection, the tela choroidea was incised, and the left half of the rhomboid fossa was exposed. After the mapping, the floor of the fourth ventricle was incised in the suprafacial triangle. Cavernoma and hematoma were removed. Hemostasis was achieved. Dura was closed. The attached bone flap was then turned upward and reattached using nonresorbable sutures. Postoperatively, the patient had resolution of sensory and motor disturbances on the right half of the body, and coordination was improved in the left limbs. Postoperative imaging confirmed complete removal of the lesion.

Development of Leigh syndrome with a high probability of cardiac manifestations in infantile-onset patients with m.14453G > A.

The m.14453G > A mutation in MT-ND6 has been described in a few patients with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes or Leigh syndrome.However, the clinical spectrum and molecular characteristics are unclear.Here, we present four infantile-onset patients with m.14453G > A-associated Leigh syndrome. All four patients had brainstem lesions with basal ganglia lesions, and two patients had cardiac manifestations. Decreased ND6 protein expression and immunoreactivity were observed in patient-derived samples. There was no clear correlation between heteroplasmy levels and onset age or between heteroplasmy levels and phenotype; however, infantile onset was associated with Leigh syndrome.

Gamma Knife Radiosurgery to Four Brainstem Lesions After Whole Brain Radiation Therapy.

Our patient was a 58-year-old female with a history of extensive stage small cell lung cancer initially diagnosed in November 2018. She received palliative radiation to the right hip and whole brain in December of 2018 and then received chemotherapy. Unfortunately, in October 2019, the repeat brain magnetic resonance imaging (MRI) showed recurrent lesions and she was referred for Gamma Knife Radiosurgery (GKRS). At the time of the treatment, she was found to have four brainstem lesions as well as a left frontal lobe and a right frontal lobe lesion. She completed GKRS to all six lesions without any neurological complications seen in her short-term follow-up. This case report adds to the growing body of literature showing safety of GKRS for multiple brainstem lesions.

Unusual Presentation of a Posterior Reversible Encephalopathy Syndrome With Brainstem Involvement and Subarachnoid Haemorrhage.

Posterior reversible encephalopathy syndrome is an increasingly recognized disorder characterized by a headache, visual disturbances, and seizures. It is a reversible neurotoxic state, with multiple risk factors in which endothelial injury and compromised brain perfusion are the common characteristics. Diagnosis is usually made by cerebral magnetic resonance imaging that typically shows early-stage bilateral symmetrical parieto-occipital hyperintensities on T2 and fluid-attenuated inversion recovery (FLAIR) sequences. However, other locations have been described where the disease may appear less frequently. We describe the case of a 62-year-old man, with a medical history of hypertension, who presented with anisocoria with mydriatic non-reactive pupil and ptosis of the left eye. CT head showed a slightly hypodense brainstem, in relation to vasogenic edema. This was confirmed with magnetic resonance imaging. The angiography did not identify cerebral artery aneurysms. The symptoms and radiological findings were almost completely reversible after improving the patient's blood pressure. This case highlights a rare single presentation of posterior reversible encephalopathy syndrome associated with subarachnoid hemorrhage. A high index of suspicion, careful examination, and exploration with imaging techniques were essential to reach this diagnosis.

Extensive Brainstem Posterior Reversible Encephalopathy Syndrome in a Hemodialysis Non-Adherent Patient.

Posterior reversible encephalopathy syndrome (PRES) refers to a disorder of reversible vasogenic edema caused by rapid hyperperfusion of the brain that classically involves areas supplied by the posterior circulation such as the parieto-occipital region. It may present with atypical features such as brainstem and spinal cord involvement. Common causes include renal failure, pre-eclampsia/eclampsia among pregnant women, rapid changes in systemic blood pressure, and autoimmune diseases. The most prevalent presenting signs and symptoms are encephalopathy, seizures and headache. A 64-year-old female presented to a dialysis unit after missing several sessions with twitching in her extremities and elevated blood pressure. Additionally, she recently terminated clonidine use and was likely experiencing rebound hypertension. The continuous electroencephalogram (EEG) demonstrated generalized, non-convulsive seizures. MRI findings were notable for hyperintensities in the pons, middle cerebellar peduncles, cerebellar hemispheres, and periventricular and subcortical matter with medulla and proximal spinal cord involvement. A notable clinical sequela of PRES in this patient was coma. Aggressive blood pressure control led to significant improvement and return to her neurologic baseline. PRES can present with extensive brainstem involvement with a clinical sequela of coma. Multiple underlying causes such as dialysis non-adherence and rebound hypertension following clonidine discontinuation contributed to the development of this condition in this patient.

[A case of refractory generalized atonic seizure and hemifacial spasm with the possible causative pontocerebellar lesion].

The patient was a 35-year-old woman. At the age of 1, she had undergone resection and radiation therapy for neoplastic lesions in the pons. She had a history of gelastic seizures when she was in elementary school, and brief lapses of the neck and truncal muscular tone and convulsions on the left face occurred at the age of 23. After a generalized sharp wave in the ictal electroencephalogram and electromyogram recording, left orbicularis oris muscle contraction was observed followed by sudden cervical extensor atonia. Seizure propagation was noted in the cerebral cortex, left facial nerve nucleus, and brainstem reticular formation. In a simultaneous electroencephalography with functional MRI, the blood oxygen level-dependent effect related to generalized sharp waves was observed in the vicinity of brainstem lesions in addition to a decrease in bilateral frontal and parietal lobes signals, as detected in generalized seizures. These findings suggest that the lesion could be a part of the epilepsy network. Although most epileptic seizures are derived from the cerebral cortex, it is important to note that brainstem lesions are involved in seizures in the patient presented in this study.

Diffuse infiltrative pontine glioma biopsy in children with neuronavigation, frameless procedure: A single center experience of 10 cases.

INTRODUCTION: This study presented pediatric DIPG 's biopsy with frameless Neuronavigation. PATIENTS AND METHODS: We report our experience about 10 patients who had Diffuse Intrinsic Pontine Glioma between 2014 and 2018. All patients were biopsied with BrainLab Varioguide Neuronavigation®. We always used fusion between specific CT Scan and MRI to selected target, made planning and biopsies. All patients were included in BIOMEDE after scientific and ethic discussions. We always selected a trans-cerebellar trajectory and made same procedure (lot of biopsies at one level). All patients have MRI at J1 to verify site of biopsy and to eliminate complication. RESULTS: The average age was 8.1 years. Symptoms were common with principally headaches and nystagmus. All biopsies were contributive for histopathological diagnosis and establish molecular profile for molecular study. We have no definitive morbidity and procedure duration was 93minutes in average. All MRI didn't showed intracranial complication after procedure and showed great precision of biopsy compared with the selected target. DISCUSSION: We reviewed the literature and compare our results with series of DIPG biopsies using stereotactic frame or robotic assisted frameless. It was a safe, accuracy and easiness procedure. We always have histopathological and molecular result to proceed next step of treatment. This modality is an alternative possibility to biopsy very young patients with low morbidity.

Oblique Positioning of the Stereotactic Frame for Biopsies of Cerebellar and Brainstem Lesions.

BACKGROUND: Frame-based stereotactic biopsy has proven to be a safe procedure with a high diagnostic yield for patients. Different supratentorial localizations can easily be accessed by standardized stereotactic approaches. Cerebellar and brainstem lesions, however, are not easy to reach because the positioning of the frame pins often makes it impossible to address the entry point properly. METHODS: By oblique positioning of the frame, cerebellar and brainstem lesions also can easily be accessed to take tissue samples. CONCLUSION: Modification of the standardized positioning of the frame allows for safe and straightforward stereotactic access even to cerebellar and brainstem lesions.

Extensive ischemic brainstem lesions and pneumocephalus after application of hydrogen peroxide (H2O2) during lumbar spinal surgery.

BACKGROUND CONTEXT: The hemostatic properties of hydrogen peroxide (H2O2) are often used in neurosurgical practice. CASE REPORT: We present the case of an 81-year-old woman who underwent lumbar spinal surgery (microsurgical decompression) in an external hospital. H2O2 was used during the procedure. The patient was transferred to our hospital. She remained unconscious postoperatively, with progressive loss of brainstem reflexes. Computed tomography showed intra- and extradurally trapped air ascending from the operated lumbar segment up to frontal lobe. Magnetic resonance imaging demonstrated severe brainstem lesions on T2- and diffusion-weighted series. The patient died 10 days after surgery. Autopsy was not performed. CONCLUSIONS: Our case demonstrates a fatal complication with ischemic brainstem lesions and pneumocephalus after the use of hydrogen peroxide. Therefore, H2O2 should only be used in cases without any signs of dural injury.

Stereotactic biopsy of brainstem lesions: Techniques, efficacy, safety, and disease variation between adults and children: A single institutional series and review.

BACKGROUND: Stereotactic biopsy of brainstem lesions have been performed with varying indications, with most of the literature reporting on children. MATERIALS AND METHODS: The present study retrospectively analyzed all cases that underwent stereotactic biopsy for brainstem lesion in both adult and pediatric population between 1994 and 2009 in a single tertiary neurosurgical center. The clinical and radiological features, technique of the procedure, morbidity, diagnostic accuracy, spectrum of diagnosis, and variations in adult and pediatric population were analyzed. RESULTS: Eighty-two patients were included in the study. Computed tomography (CT) was used as guidance in 73 (38 children and 35 adults) patients and magnetic resonance imaging (MRI) in 9 (3 children and 6 adults). The biopsy was performed in a procedure room under local anesthesia in most adults, while children required sedation. Glioblastoma comprised 29.3% of all pathologies in children, compared with only 4.9% of the pathologies in adult population (P = 0.007). Tuberculosis was the next major diagnosis (9.8%). In 12 patients, initial biopsy was inconclusive. Following a repeat biopsy in 5 of these patients, a diagnosis was possible for 75/82 (91.5%) patients by STB. The location of the target, the choice of entry, the radiological characteristic of the lesion, enhancement pattern, and age group did not significantly correlate with the occurrence of inconclusive biopsy. Permanent complications occurred in two patients (2.4%). There was no mortality in this series. CONCLUSION: Stereotactic biopsy has an important role in brainstem lesions, more significantly in adults, due to wider pathological spectrum. It can be performed safely under local anesthesia through a twist drill craniostomy in most of the adults.