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.

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.

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.

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.
CONCLUSIONS: 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.

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.

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.

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.
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.
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).
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.

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.

OBJECTIVE: 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.
RESULTS: 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.

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.