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Background. In some clinical situations, distinguishing between cerebellar medulloblastoma and brainstem glioma is important. We assessed whether diffusion kurtosis imaging (DKI) metrics could be used to distinguish cerebellar medulloblastomas from brainstem gliomas in children. Patients and methods. This prospective study was approved by the institutional review board. Seventy patients were separated into two groups according to eventual diagnosis: brainstem glioma (n = 30) and cerebellar medulloblastoma (n = 40). Both groups underwent brain magnetic resonance imaging (MRI), including DKI. The Kurtosis value for the tumor region and the ratio between Kurtosis values between the tumor and the normal parenchyma (rKurtosis) were compared between groups using the Mann-Whitney U test. Receiver operating characteristic curve analysis and the Youden's Index were applied to identify a cutoff value for distinguishing between the two tumor types, and the area under the curve (AUC), sensitivity, and specificity for the selected cutoff value were calculated. Results. Compared with brainstem gliomas, cerebellar medulloblastomas had significantly higher Kurtosis and rKurtosis values (p < 0.05). Medulloblastoma could be differentiated from brainstem gliomas using a Kurtosis value of 0.91 or an rKurtosis value of 0.90, both of which achieved 100% sensitivity, 96.7% specificity, and AUC values of 0.990 Conclusions. DKI measurements can contribute to distinguishing between cerebellar medulloblastoma and brainstem glioma in children. [ABSTRACT FROM AUTHOR]

For atypical brainstem lesions, histological diagnosis can have an impact on treatment, especially in cases where diffuse glioma is not found. Since radiotherapy is the only therapeutic modality that has shown clinical and radiographic improvement in patients with diffuse glioma, the misdiagnosis of diffuse glioma can have drastic consequences, particularly in patients with nontumorous lesions. Thus, the purpose of this study was to evaluate the impact of histological diagnosis on the treatment of atypical brainstem lesions. This was a retrospective study of 31 patients who underwent biopsy of atypical brainstem lesions. The procedures were performed between January 2008 and December 2018 at the Life Center Hospital and Santa Casa de Belo Horizonte, MG, Brazil. A diagnosis was obtained in 26 (83.9%) cases. Three patients presented complications: one presented bleeding with no clinical repercussions and two showed worsening of neurological deficit, only one of which was definitive. No mortality occurred due to the procedure. The histological diagnosis was diffuse glioma in seven cases (22.6%) and not diffuse glioma in 19 cases (61.3%). Thus, the histological diagnosis had an impact on the treatment of 19 patients (treatment impact rate: 61.3%). The histological diagnosis of intrinsic brainstem lesions is a safe, efficient procedure with a high diagnosis rate, and as such, it should be considered in the management of atypical lesions. [ABSTRACT FROM AUTHOR]

Brainstem tumors show invasive growth, and there is no obvious boundary with normal brain tissues, and most of them are not limited to one brain lobe, which destroys brain tissues with fingers outside the brain tissues. With the improvement of microscopes and microscopy instruments and the continuous improvement of microsurgery technology, microsurgery has significantly improved the total resection rate of gliomas, which can significantly improve clinical efficacy. Microneurosurgeiy will promote the classic neurosurgery "brain lobe range surgery" to the level of "focal surgery" to minimize interference with brain tissue and explore new surgical approaches. Microsurgical treatment of craniocerebral tumors not only has less trauma, less damage to brain tissue, high safety, but also helps to completely remove the tumor There are fewer postoperative complications and a low disability rate. Based on the above background, the research content of this article is the treatment and research of brainstem tumors during microsurgical resection. Based on the analysis of the characteristics and pathological characteristics of brainstem tumors and the basis of microsurgery technology, this article proposes the application of microsurgical resection to brainstem tumor resection and the experimental simulation of patients in a hospital. The results it shows that surgical removal of more than 98% of tumors can enable patients to obtain significant survival advantages. Even in gliomas in important functional areas of the brain, the use of modern surgical techniques and intraoperative monitoring techniques can achieve subtotal tumor resection and surgery. The degree of resection is proportional to the patient's survival time. [ABSTRACT FROM AUTHOR]

With no statutory definition of death, the accepted medical definition relies on brain stem death criteria as a definitive measure of diagnosing death. However, the use of brain stem death criteria in this way is precarious and causes widespread confusion amongst both medical and lay communities. Through critical analysis, this paper considers the insufficiencies of brain stem death. It concludes that brain stem death cannot be successfully equated with either biological death or the loss of integrated bodily function. The overemphasis of the brain-stem and its consequences leaves the criteria open to significant philosophical critique. Further, in some circumstances, the use of brain stem death criteria causes substantial emotional conflict for families and relatives. Accordingly, a more holistic and comprehensive definition of death is required. [ABSTRACT FROM AUTHOR]

Objective Dural arteriovenous fistulas (DAVFs) at the petrous apex are rare but may cause subarachnoid hemorrhage (SAH) or severe brainstem edema. This study aimed to summarize their clinical features and discuss the classification. Methods During a 15-year period, 64 consecutive patients with DAVF at the petrous apex were reviewed. According to their angioarchitecture, these cases were classified as follows: type I, no venous ectasia (48.4%); type II, venous ectasia but with normal vein proximal to the fistula (29.7%); and type III, venous ectasia at the site of the fistula (21.9%). Results There were 53 men and 11 women included. Presented symptoms were SAH in 8 patients (12.5%), nonhemorrhagic neurologic defects (NHNDs) in 53 patients (82.8%), and no symptoms in 3 patients (4.7%). There were 49 patients who received transarterial embolization, 8 patients who received microsurgery, and 7 patients who received embolization and microsurgery. Complications occurred in 9 patients (14.1%), including transient cranial nerve palsy (4.7%), rebleeding (6.3%), and respiratory failure (3.1%). Of the type I patients, 96.77% presented with NHNDs and 77.42% presented with infratentorial drainage. However, SAH occurred more often in type II (21.05%)/type III cases (28.57%), and most patients carried a supratentorial drainage (63.16% and 85.71%, respectively). In different types of DAVFs, the necessity for embolization combined with microsurgery (6.45% in type I, 10.53% in type II, 21.43% in type III) and the occurrence of rebleeding complications (0% in type I, 10.53% in type II, and 14.29% in type III) were varied. Conclusions Petrous apex DAVFs carried a high risk of embolization-related complications. Based on the vascular architecture, this classification may reflect their clinical features and provide some advice on the treatment of DAVFs at the petrous apex. Highlights • DAVFs at the petrous apex are rare but dangerous. • This is one of the largest case reports of this type of DAVF (N = 64). • Our recurrence and complication rates (0% and 14.1%) were much lower than previous reports (43% and 55%), respectively. • A classification based on anatomic features and clinical features was made. • We used different techniques to treat 3 types of DAVFs, which has not yet been reported. [ABSTRACT FROM AUTHOR]

Background. Cranial nerve palsies are well-known complications of basal meningitis, especially in patients with tuberculous meningitis. However, a minority of bacterial meningitis gets complicated with cranial nerve palsies. Although cerebral infarctions are known to occur with acute bacterial meningitis, infarctions occurring in the brainstem are infrequently described. Case Presentation. We report a 46-year-old healthy female who presented with dysarthria with fever, headache, and vomiting and was diagnosed to have acute pyogenic meningitis complicated with a brainstem infarction resulting in bilateral hypoglossal palsy. Her MRI revealed an infarction in the lower part of the medulla oblongata, probably involving the bilateral hypoglossal nuclei. Conclusion. Isolated bilateral hypoglossal nerve palsy is an extremely rare cranial nerve palsy, secondary to pyogenic meningitis. To our knowledge, this should be the first reported case of isolated bilateral hypoglossal nerve palsy due to a brainstem infarct in the background of pyogenic meningitis. [ABSTRACT FROM AUTHOR]

Background and Importance Lesions located lateral to the lower brainstem, such as proximal posterior inferior cerebellar artery (PICA) aneurysms, are surgically challenging. We report a case of a patient with a left proximal PICA aneurysm that was successfully clipped via a so-called “extended” suboccipital subtonsillar approach, which allowed us to obtain proper vascular control without removal of the atlas. The anatomy relevant for this approach has been studied. Methods Three adult cadaveric heads were studied. The relevant neurovascular anatomy related to this approach was exposed. Hence, this technique was applied on the patient herewith reported. Case Presentation A 60-year-old man with sudden onset of severe headache, nausea, and vomiting was admitted to our hospital. Computed tomography of the brain showed diffuse subarachnoid hemorrhage, mainly distributed at the level of the perimesencephalic cisterns. Cerebral angiography revealed a 3-mm aneurysm arising at the origin of the left PICA. The aneurysm was considered unsuitable for coil embolization, so it was treated via a “modified” posterolateral suboccipital subtonsillar route. The modification consisted of accomplishing proper proximal vascular control at the extracranial segment of the vertebral artery (V3), without the need of further removal of the posterior portion of the atlas. The patient was discharged neurologically intact. Conclusions The technique we suggested allowed no unnecessary removal of bone, with no need to drill the occipital condyle or remove the atlas, offered proper proximal vascular control in the early stage of the surgical procedure, and limited the quantity of temporary vascular clips inside the intracranial surgical field. [ABSTRACT FROM AUTHOR]

Goal: Automatic segmentation of brainstem gliomas and prediction of genotype (H3 K27M) mutation status based on magnetic resonance (MR) images are crucial but challenging tasks for computer-aided diagnosis in neurosurgery. In this paper, we present a novel cascaded deep convolutional neural network (CNN) to address these two challenging tasks simultaneously. Methods: Our novel segmentation task contains two feature-fusion modules: the Gaussian-pyramid multiscale input features-fusion technique and the brainstem-region feature enhancement. The aim is to resolve very difficult problems in brainstem glioma segmentation. Our prediction model combines CNN features and support-vector-machine classifier to automatically predict genotypes without region-of-interest labeled-MR images and is learned jointly with the segmentation task. First, Gaussian-pyramid multiscale input feature fusion is added to our glioma-segmentation task to solve the problems of size variety and weak brainstem-gliomas boundaries. Second, the two feature-fusion modules provide both local and global contexts to retain higher frequency details for sharper tumor boundaries, handling the problem of the large variation of tumor shape, and volume resolution. Results and Conclusion: Experiments demonstrate that our cascaded CNN method achieves not only a good tumor segmentation result with a high Dice similarity coefficient of 77.03%, but also a competitive genotype prediction result with an average accuracy of 94.85% upon fivefold cross-validation. [ABSTRACT FROM AUTHOR]

Objectives: To examine short-term outcome of brainstem auditory function in term infants after discharge from the neonatal intensive care unit (NICU).Methods: Brainstem auditory evoked response (BAER) was recorded and analysed at various click rates at 2-4 months of age in 43 term NICU graduates. The data were compared with those in age-matched normal controls.Results: The threshold of BAER in the NICU graduates was marginally elevated. The graduates also showed an increase in wave III latency at 91/s and wave V latency at all 21-91/s. The I-V interval was increased at 51 and 91/s, whereas the I-III and III-V intervals were slightly increased, with no significant differences from normal controls at any click rates. The amplitudes of waves I and V in the NICU graduates were moderately reduced at all click rates. Wave III amplitudes were reduced at 51 and 91/s. None of the slopes of BAER variables-rate functions in the NICU graduates differed significantly from normal controls.Conclusions: The BAER was moderately abnormal in the NICU graduates, suggesting that brainstem auditory function is moderately impaired at 2-4 months of age after discharge. There is a need to monitor postnatal auditory and neural development for NICU graduates. [ABSTRACT FROM AUTHOR]

The hypothalamus-pituitary-adrenal axis (HPA) is the main regulator of the stress response. The key of the HPA is the parvocellular paraventricular nucleus of the hypothalamus (pPVN) controlled by higher-order limbic stress centers. The reactivity of the HPA axis is considered to be a function of age, but to date, little is known about the background of this age-dependency. Sporadic literature data suggest that the stress sensitivity as assessed by semi-quantitation of the neuronal activity marker c-Fos may also be influenced by age. Here, we aimed at investigating the HPA activity and c-Fos immunoreactivity 2 h after the beginning of a single 60 min acute restraint stress in eight age groups of male Wistar rats. We hypothesized that the function of the HPA axis (i.e., pPVN c-Fos and blood corticosterone (CORT) level), the neuronal activity of nine stress-related limbic areas (i.e., magnocellular PVN (mPVN), medial (MeA), central (CeA), basolateral nuclei of the amygdala, the oval (ovBNST), dorsolateral (dlBNST), dorsomedial (dmBNST), ventral and fusiform (fuBNST) divisions of the bed nucleus of the stria terminalis (BNST)), and two brainstem stress centers such as the centrally projecting Edinger-Westphal nucleus (cpEW) and dorsal raphe nucleus (DR) show age dependency in their c-Fos response. The somatosensory barrel cortex area (S1) was evaluated to test whether the age dependency is specific for stress-centers. Our results indicate that the stress-induced rise in blood CORT titer was lower in young age reflecting relatively low HPA activity. All 12 stress-related brain areas showed c-Fos response that peaked at 2 months of age. The magnitude of c-Fos immunoreactivity correlated negatively with age in seven regions (MeA, CeA, ovBNST, dlBNST, dmBNST, fuBNST and pPVN). Unexpectedly, the CeA, ovBNST and cpEW showed a considerable basal c-Fos expression in 1-month-old rats which decreased with age. The S1 showed a U-shaped age-related dynamics in contrast to the decline observed in stress centers. We conclude that the age- and brain area dependent dynamics in stress-induced neuronal activity pattern may contribute to the age dependance of the stress reactivity. Further studies are in progress to determine the neurochemical identity of neurons showing age-dependent basal and/or stress-induced c-Fos expression. [ABSTRACT FROM AUTHOR]

Background: Early detection and intervention for post-stroke dysphagia could reduce the incidence of pulmonary complications and mortality. The aims of this study were to investigate the benefits of swallowing therapy in swallowing function and brain neuro-plasticity and to explore the relationship between swallowing function recovery and neuroplasticity after swallowing therapy in cerebral and brainstem stroke patients with dysphagia. Methods: We collected 17 subacute stroke patients with dysphagia (11 cerebral stroke patients with a median age of 76 years and 6 brainstem stroke patients with a median age of 70 years). Each patient received swallowing therapies during hospitalization. For each patient, functional oral intake scale (FOIS), functional dysphagia scale (FDS) and 8-point penetration-aspiration scale (PAS) in videofluoroscopy swallowing study (VFSS), and brain functional magnetic resonance imaging (fMRI) were evaluated before and after treatment. Results: FOIS (p = 0.003 in hemispheric group and p = 0.039 in brainstem group) and FDS (p = 0.006 in hemispheric group and p = 0.028 in brainstem group) were both significantly improved after treatment in hemispheric and brainstem stroke patients. In hemispheric stroke patients, changes in FOIS were related to changes of functional brain connectivity in the ventral default mode network (vDMN) of the precuneus in brain functional MRI (fMRI). In brainstem stroke patients, changes in FOIS were related to changes of functional brain connectivity in the left sensorimotor network (LSMN) of the left postcentral region characterized by brain fMRI. Conclusion: Both hemispheric and brainstem stroke patients with different swallowing difficulties showed improvements after swallowing training. For these two dysphagic stroke groups with corresponding etiologies, swallowing therapy could contribute to different functional neuroplasticity. [ABSTRACT FROM AUTHOR]

Background and purpose: Neuromelanin of the brainstem, which is considered a marker of neurodegeneration in Parkinson's disease (PD), can be detected by T1‐weighted neuromelanin‐sensitive magnetic resonance imaging (NM‐MRI). Our aim was to investigate the NM‐MRI features of de novo PD and to determine whether these features are associated with motor and non‐motor symptoms in de novo PD patients. Methods: Fifty‐one patients with de novo PD (Hoehn and Yahr stage 1–2) and 28 healthy controls were recruited. All subjects underwent clinical and MRI examinations including an NM‐MRI sequence. The width and contrast‐to‐noise ratio (CNR) of the substantia nigra pars compacta (SNc) and the CNR of the locus coeruleus (LC) were measured on NM‐MRI images. Results: Both the width and CNR values of the high intensity signals in the SNc were significantly decreased in the lateral, central and medial SNc parts in de novo PD patients compared to control subjects. The changes in the SNc on NM‐MRI were not significantly different between the motor subgroups. The CNR values of the left LC were significantly lower in the PD group than in the control group. Specifically, the subtype of PD patients with depressive symptoms exhibited a significantly lower CNR in the left LC than the control group and PD patients without depressive symptoms. Conclusions: Substantia nigra pars compacta neuromelanin changes occur across both motor and non‐motor (with and without depressive symptoms) subtypes, whilst LC changes are more notable in PD patients with depressive symptoms. Our results may provide new evidence to understand the pathophysiology of non‐motor symptoms in PD. [ABSTRACT FROM AUTHOR]

Transplantation of Human Organs is guided by laid down specific Laws in India. The organs which are targeted to be transplanted are liver, kidney and cornea. The waiting list is enormous but the donor pool is meagre. This document has been made with a view that the donor pool can be enlarged by identifying patients who are ‘Brain Dead’ while still not having ‘Cardiac Death’. The steps include the prerequisite conditions which must be satisfied by patients who have suspicion of being brain dead, detailed examination of the patient, confirmation of the Brain Death and Counselling of the relatives for organ donation. [ABSTRACT FROM AUTHOR]

The topic of brain stem computational simulation still seems understudied in contemporary scientific literature. Current advances in neuroscience leave the brain stem as one of the least known parts of the human central nervous system. Brain stem lesions are particularly damaging to the most important physiological functions. Advances in brain stem modeling may influence important issues within the core of neurology, neurophysiology, neurosurgery, and neurorehabilitation. Direct results may include both development of knowledge and optimization and objectivization of clinical practice in the aforementioned medical areas. Despite these needs, progress in the area of computational brain stem models seems to be too slow. The aims of this paper are both to recognize the strongest limitations in the area of computational brain stem simulations and to assess the extent to which current opportunities may be exploited. Despite limitations, the emerging view of the brain stem provided by its computational models enables a wide repertoire of functions, including core dynamic behavior. [ABSTRACT FROM AUTHOR]

Purpose: Incidence of BS primitive neuroectodermal tumors (BS-PNET) in children is not reported to date. Our main objectives were to estimate the incidence and report the outcome of BS-PNET in children.Methods: Data were collected using the Surveillance Epidemiology and End Results cancer registry.Results: From 1973 to 2013, we identified 83 pediatric patients (aged 0–21 years). Patients were divided into two age groups (0–3 years and 4–21 years). Median overall survival was 53 months. Patients in the older age group had a significant survival advantage (P < 0.001), as did those who received three modalities of therapy (surgery, chemotherapy, and radiation therapy) (P < 0.001) and patients with gross or subtotal tumor resection (P < 0.001).Conclusions: This study presents the first estimate of incidence and the largest cohort of pediatric BS-PNETs to date. A high index of suspicion of BS-PNET in similar cases is crucial for diagnosis, treatment, and outcome. [ABSTRACT FROM AUTHOR]

Background and purpose: To assess the anatomical distribution of the ischemic strokes of the brainstem, the effect of anatomical distribution on clinical features and prognosis, and the association between etiology and anatomical involvement. Methods: A retrospective search of the patient database of our institution was performed for a total of 227 patients who were admitted to the Department of Neurology, Medical Faculty of Bezmialem Vakif University between January 2012 and September 2014. Patients with adequate diagnostic data and 3-month follow-up visit were included in the study. Results: Twenty-one (9%), 136 (60%), and 65 (29%) patients had an infarction only at the mesencephalon, pons, and medulla, respectively. However, a single patient (0.5%) had an infarction both at the mesencephalon and pons, 3 (1.5%) at the pons and medulla, and 1 (0.5%) at the mesencephalon, pons, and medulla. While anterior involvement was more common in the mesencephalon and pons, posterior and lateral involvement occurred more frequently in the medulla. Large arterial atherothrombosis was the predominant cause of the strokes in all anatomical sites, particularly in infarcts involving the pons. Cardioembolic events were more common in patients with mesencephalic infarcts. Also, ischemia due to dissection was more common in infarctions involving the medulla, especially the lateral medulla. In subjects with simultaneous infarcts at other sites in addition to the brainstem, there was a significantly higher co-occurrence of medullary infarcts with cerebellar infarcts, mesencephalic infarcts with posterior cerebral artery infarcts, and pons infarcts with anterior circulation and multiple infarcts. Conclusion: Determination of risk factors and infarct localization as well as prediction of etiological parameters may assist in improving survival rates and therapeutic approaches. [ABSTRACT FROM AUTHOR]

Background and purpose: Non‐alcoholic fatty liver disease (NAFLD) is closely correlated to visceral obesity, dyslipidaemia, insulin resistance and type 2 diabetes mellitus. We sought to assess the association between a specific stroke subgroup, brainstem infarctions (BSIs) and NAFLD. Furthermore, we evaluated whether NAFLD is an independent risk factor in patients with BSIs. Methods: Non‐alcoholic fatty liver disease was assessed in 306 patients with radiologically confirmed BSIs via liver ultrasound. Differences between patients with and without NAFLD were compared. Data associated with stroke severity and progression after admission were collected. Results: Non‐alcoholic fatty liver disease was found in 130 (42.5%) patients with acute BSIs; 58 (19.0%) had National Institutes of Health Stroke Scale scores >7 and 57 (18.6%) had progression after admission. Initial National Institutes of Health Stroke Scale scores, incidence of progression and stroke severity, and modified Rankin Scale scores at discharge were significantly higher in patients with NAFLD than in those without NAFLD. NAFLD was associated with stroke severity [Cox regression: hazard ratio (HR), 2.243; 95% confidence interval (CI), 1.254–4.013, P < 0.01]. This risk remained statistically significant after controlling for age, gender, diabetes mellitus and C‐reactive protein (HR, 2.327; 95% CI, 1.252–4.324, P < 0.01). In addition, NAFLD was associated with progression (HR, 2.155; 95% CI, 1.201–3.865, P < 0.05) and remained significant after controlling for age, gender, diabetes mellitus, fibrinogen and C‐reactive protein (HR, 2.378; 95% CI, 1.260–4.486, P < 0.01). Conclusions: These results suggest that NAFLD is a potential risk factor when evaluating the severity and progression of acute BSIs. This relationship is independent of classic risk factors and metabolic syndrome features. [ABSTRACT FROM AUTHOR]

Until six decades ago, death was considered to be a specific point in time, referring to the moment at which life ends. With the availability of ventilators, even determining when death has occurred is becoming difficult, as cessation of life functions is often not simultaneous across organ systems. With increasing accessibility to intensive care units (ICUs) even in Tier II and Tier III cities, and the government making it mandatory to notify brain death to facilitate cadaveric organ transplants, it behooves the neurosurgeon and neurologist to totally understand the minutiae of brain death. The author reflects on his personal experience in certifying brain death, over two decades, in a quaternary care hospital. Knowing the pathophysiology of brain stem dysfunction will help the clinician better understand the rationale of the mandatory clinical tests prescribed. The necessity for an early diagnosis and the pitfalls in the clinical diagnosis of brain death, the limitations of the so-called confirmatory tests, and the concerns and ethical issues will be highlighted. Review of the world literature demonstrates that there is no international consensus even on what constitutes brain death. An individual could be considered legally dead in one country but not in another! [ABSTRACT FROM AUTHOR]

Background: Leukoencephalopathies with prominent involvement of cerebellum and brainstem, henceforward called prominent infratentorial leukoencephalopathies (PILs), encompass a variety of inherited and acquired white matter diseases. Erdheim–Chester disease (ECD) is a rare non-Langerhans cell histiocytosis likely under-diagnosed as cause of adult PIL.Methods: We reviewed the clinical and laboratory information of ten consecutive sporadic adult patients with PIL of unknown origin, who were investigated for ECD.Results: There were seven males and three females; mean age at clinical onset was 49.6 years (range 38–59); cerebellar ataxia with or without other neurological symptoms was the only or the main clinical manifestation; diabetes insipidus was present in three individuals. Eight patients had white matter focal supratentorial abnormalities, in addition to the infratentorial white matter changes. Six out of eight patients had spinal cord lesions. Thoraco-abdominal CT showed periaortic sheathing in two patients, whole-body FDG-PET revealed increased glucose uptake in the long bones of the legs in five patients, brain FDG-PET showed overt infratentorial hypermetabolism in one patient. In eight patients, ECD was confirmed by bone scintigraphy, pathological data, or both. Two ECD patients treated with vemurafenib showed a marked improvement of neurological symptoms and brain MRI abnormalities at 1 year follow-up.Conclusions: Symptoms of PIL can be the only clinical manifestation of ECD. Adult patients with PIL of unknown origin should undergo investigations aimed at unveiling ECD, including bone scintigraphy and whole-body FDG-PET. The early diagnosis allows starting disease-modifying therapies of an otherwise life-threatening disease. [ABSTRACT FROM AUTHOR]

Objective: To report a rare case of dorsal brainstem syndrome in an infant after hypoxic-ischemic episode due to severe sepsis and the use of neurally adjusted ventilatory assist (NAVA) to aid in diagnosis and in the removal of mechanical ventilation. Case description: A 2-month-old male infant, previously healthy, presented with severe sepsis that evolved to dorsal brainstem syndrome, which usually occurs after hypoxic-ischemic injury in neonates and infants, and is related to very specific magnetic resonance images. Due to neurological lesions, thei nfant remained in mechanical ventilation. A NAVA module was installed to keep track of phrenic nerve conduction to the diaphragm, having successfully showed neural conduction and helped removing mechanical ventilation. Comments: Dorsal brainstem syndrome is a rare condition that should be considered after hypoxic-ischemic episode in infants. [ABSTRACT FROM AUTHOR]

Background: Spontaneous regressions of brain stem gliomas are extremely rare. Only six cases have been reported in the literature. Case presentation: We describe the case of a patient who was diagnosed with a pontomedullary dorsal brain stem glioma at the age of 15 years. An open biopsy showed the presence of an anaplastic glioma. Because the patient and her parents refused conventional therapies, including radiation and chemotherapy, we followed up the patient by performing magnetic resonance imaging scans on her every 3 months. At 3 months after biopsy, we observed the radiological disappearance of her tumor. One year after biopsy, the tumor retained the spontaneous complete regression observed earlier. Conclusion: In this case report, we present the first report of the spontaneous complete regression of a brain stem glioma that was histologically proven to be a high-grade glioma and we believe that this regression was the natural progression of this case, as may be the scenario in a few other cases of brain stem gliomas. [ABSTRACT FROM AUTHOR]

Diffuse intrinsic pontine glioma (DIPG) is an aggressive type of brainstem cancer occurring mainly in children, for which there currently is no effective therapy. Current efforts to develop novel therapeutics for this tumor make use of primary cultures of DIPG cells, maintained either as adherent monolayer in serum containing medium, or as neurospheres in serum-free medium. In this manuscript, we demonstrate that the response of DIPG cells to targeted therapies in vitro is mainly determined by the culture conditions. We show that particular culture conditions induce the activation of different receptor tyrosine kinases and signal transduction pathways, as well as major changes in gene expression profiles of DIPG cells in culture. These differences correlate strongly with the observed discrepancies in response to targeted therapies of DIPG cells cultured as either adherent monolayers or neurospheres. With this research, we provide an argument for the concurrent use of both culture conditions to avoid false positive and false negative results due to the chosen method. [ABSTRACT FROM AUTHOR]

Hyperekplexia is a rare movement disorder characterized by pathologically exaggerated response to unexpected stimuli. It is differentiated from the normal startle reflex by its lower threshold, higher intensity, and resistance to habituation. Many of the acquired hyperekplexias result from brainstem involvement such as encephalitis, infarct, hemorrhage, pontocerebellar hypoplasia and medullary compression. This case report depicts a rare manifestation of hyperekplexia. The unusual aspect of this case was the vocalization that was reproduced in response to startling stimuli. Startle induced vocalization is not a part of the classical hyperekplexia description. When faced with a patient with pathologically exaggerated response to unexpected stimuli, the physician should consider this rare condition in the differential diagnosis, and also keep in mind that the disease may present with features different from those listed in the textbooks. [ABSTRACT FROM AUTHOR]

Listeria monocytogenes is associated with rhombencephalitis. However, the exact mechanisms of brainstem invasion remains poorly understood. Here, we demonstrate clinical and radiological data suggesting that Listeria may invade the brainstem via the trigeminal nerve. Three females (41, 64 and 70 years) with culture proven L. monocytogenes bacteremia and rhombencephalitis were investigated in the period of 2014-16. T2-weighted and contrast-enhanced T1-weighted MRI revealed a cerebellopontine abscess in all three patients, including the involvement of the trigeminal nerve root. In two patients, MRI also revealed selective contrast enhancement of the sensory trigeminal tract in the pons and medulla oblongata. Prior to any other neurological symptoms, two patients complained of hypoesthesia and a tingling sensation in the ipsilateral half of the face, consistent with sensory trigeminal nerve dysfunction on that side. In addition, we identified another 120 cases of Listeria rhombencephalitis following a systematic review. Cranial nerves VII, V, IX, and X, respectively, medulla oblongata, cerebellum and pons, were the most frequently involved brain structures. The present clinical and radiological findings corroborate earlier data from animal experiments, indicating that L. monocytogenes may be capable of retrograde intra-axonal migration along the cranial nerves. We suggest that in a subset of patients with rhombencephalitis L. monocytogenes enters the cerebellopontine angle through the trigeminal nerve, invading the brainstem via the sensory trigeminal nuclei. [ABSTRACT FROM AUTHOR]

Key Clinical Message Opalski syndrome is a rare vascular brainstem syndrome which is accepted as a variant of Wallenberg syndrome. Opalski syndrome should be considered in acute conditions in which typical symptoms of lateral medullary infarct are accompanied by ipsilateral hemiparesis. Other brain stem syndromes are distinguished from Opalski syndrome by the presence of contralateral hemiparesis. [ABSTRACT FROM AUTHOR]

The article discusses the degeneration of motor neurons in the progressive and paralytic condition called amyotrophic lateral sclerosis (ALS). It is characterized by insidious focal weakness and stiff muscles and spasticity due to failure of motor neurons in the brain stem and spinal cord. It also discusses the epidemiologic features, genetic features, and pathological characteristics of ALS.

Purpose: We sought to evaluate the long-term functional outcomes and identify the potential risk factors for rebleeding in patients with brain stem cavernous malformations (BCMs) who presented with hemorrhages and were surgically or conservatively treated and prospectively monitored. Methods: From January 1990 to July 2015, we included patients with first hemorrhagic episodes secondary to single BCMs. Modified Rankin score (mRS) was used for neurological status assessment. Univariate and multivariate regression statistics were used to identify the risk factors for rebleeding. Results: A total of 99 patients with BCMs hemorrhages were included (59 [59.6%] women, mean age 37± 13 years). As initial treatments, 37 patients (37.4%) underwent surgery and 62 (62.6%) received conservative treatment. The median follow- up was 3.33 years (interquartile range 1.16-7 years; 408.3 patient/years). The rebleeding rate by patient/year was 10% in conservatively treated patients. Deterioration was significantly more frequent in patients with rebleeding (p = 0.0001). At the end of the follow-up, the mRS were favorable in 49 patients (65.3%) without rebleeding, whereas only 8 (33.3%) with rebleeding evolved to favorable outcomes (p = 0.006). Lesion size >18 mm (hazards ratio, HR 3.34, 95% CI 1.54-7.26; p = 0.0001) and ventral location or crossing the brain stem's midpoint (HR 2.5, 95% CI 1.14-5.46; p = 0.022) were associated with a major risk of rebleeding in the univariate analysis, but only a lesion >18 mm remained statistically significant (HR 2.7, 95% CI 1.2-6.21; p = 0.016) in the multivariate analysis. Conclusion: A lesion size >18 mm was the principal factor associated with hemorrhage recurrence. The overall functional outcome was good. However, significant morbidity was attributable to rebleeding. [ABSTRACT FROM AUTHOR]

The article presents scientific evidences that notes the deregulated expression of enhancer of zeste homolog 2 (EZH2) gene in cases of congenital brainstem disconnection (CBSD), an embryo-fetal defect focusing on the role of Polycomb Repressive Complex 2 (PRC2) as a promotor gene. Topics include interference with developing rhombencephalon, cerebral pathological analysis of patients with CBSD, and role of EZH2 in development of nervous system.

Brain MRI in adult patients with Alexander disease (AxD) mainly shows atrophy in the medulla oblongata. However, currently there is no quantitative standard for assessing this atrophy. In this study, we quantitatively evaluated the brain stem of AxD patients with glial fibrillary acidic protein (GFAP) mutation using conventional MRI to evaluate its usefulness as an aid to diagnosing AxD in daily clinical practice. Nineteen AxD patients with GFAP mutation were compared with 14 patients negative for GFAP mutation in whom AxD was suspected due to "atrophy of the medulla oblongata." In the GFAP mutation-positive group, the sagittal diameter of the medulla oblongata, the ratio of the diameter of the medulla oblongata to that of the midbrain (MO/MB), and the ratio of the sagittal diameter of the medulla oblongata to that of the pons (MO/Po) were significantly smaller compared to those of the GFAP mutation-negative group (p < 0.01). The sensitivity and specificity of each parameter were 87.5 and 92.3%, 91.7 and 81.3%, and 88.2 and 100% with a sagittal diameter of the medulla oblongata <9.0 mm, MO/MB <0.60, and sagittal MO/Po <0.46, respectively. These parameters can provide very useful information to differentially diagnose AxD from other disorders associated with brain stem atrophy in adult patients. [ABSTRACT FROM AUTHOR]

Background: Isolated brainstem tuberculomas are rare lesions and account for up to 5% of all intracranial tuberculomas in endemic areas. The difficulties in diagnosis and management of this condition are sparsely reported. The aim of this study is to illustrate the nuances in managing brainstem tuberculomas, define prognosis, and demonstrate a shift in management strategies with newer imaging modalities. Method: A retrospective review of 14 patients diagnosed and treated with a diagnosis of 'isolated brainstem tuberculoma' between 2011 and 2015 was done. Diagnosis was made after combining the findings at clinical history, examination, as well as imaging features. Patients were treated with steroids for 6 weeks or until they made a meaningful clinical recovery, and antitubercular therapy (ATT) for a minimum of 18 months or until there was resolution of the tuberculoma. Confirmation of tubercular pathology was done by observing if response to treatment resulted in clinical improvement, which happened in all of our cases. Results: Mean age at diagnosis was 24.7 years and nine were males. Twelve patients had a combination of cranial nerve deficits with pyramidal weakness or sensory symptoms. Mean duration of symptoms was 4.7 months and tests for human immunodeficiency virus (HIV) infection were negative in all patients. Only two patients had a previous history of tubercular meningitis. Most lesions were located in the pons with size ranging from 1 to 22.2 cm. Eight patients showed complete resolution of the lesion at latest follow-up and the rest were still on ATT. Mean duration of ATT received for resolution of the lesion was 22 months. Almost all of our patients improved clinically on steroids and ATT. Conclusions: Intracranial tuberculomas may present with or without meningitis. A high index of suspicion is essential, especially in endemic areas. A combination of clinical symptoms, investigations, and imaging features help in coming to a diagnosis. Biopsy of a brainstem lesion is fraught with complications. Antitubercular therapy has a very good prognosis, though the duration of therapy required may be longer. [ABSTRACT FROM AUTHOR]

Background and Purpose Although diffusion-weighted imaging ( DWI) is a very sensitive technique for the detection of small ischemic lesions in the human brain, in particular in the brainstem it may fail to demonstrate acute ischemic infarction. In this study, we sought to evaluate the value of additional thin-section coronal DWI for the detection of brainstem infarction. Methods In 155 consecutive patients (median age 69 [interquartile range, IQR 57-78] years, 95 [61.3%] males) with isolated brainstem infarction, MRI findings were analyzed, with emphasis on ischemic lesions on standard axial (5 mm) and thin-section coronal (3 mm) DWI. Results On DWI, we identified ischemic lesions in the mesencephalon in 12 (7.7%), pons in 115 (74.2%), and medulla oblongata in 31 (20%) patients. In 3 (1.9%) cases-all of these with medulla oblongata infarction-the ischemic lesion was detected only on thin-section coronal DWI. Overall, in 35 (22.6%) patients the ischemic lesion was more easily identified on thin-section coronal DWI in comparison to standard axial DWI. In these, the ischemic lesions were significantly smaller (0.06 [ IQR 0.05-0.11] cm3 vs. 0.25 [ IQR 0.13-0.47] cm3; p < .001) in comparison to those patients whose ischemic lesion was more easily (6 [3.9%]) or at least similarly well identified (114 [73.5%]) on standard axial DWI. Conclusions Since thin-section coronal DWI may facilitate the diagnosis of brainstem infarction, we suggest its inclusion in standard stroke MRI protocols. [ABSTRACT FROM AUTHOR]

Background: Morphologic changes of the brainstem in major depressive disorder (MDD) have rarely been reported in neuroimaging studies, even though, monoaminergic neurotransmitters are synthesized in several brainstem regions. We aimed to investigate volume changes in each region of the brainstem and their association with antidepressant use or the remission status of MDD.Methods: A total of 126 patients with MDD and 101 healthy controls underwent T1-weighted structural magnetic resonance imaging. We analyzed volumes of each brainstem region, including the medulla oblongata, pons, midbrain, and superior cerebellar peduncle, and the volume of the whole brainstem using the FreeSurfer.Results: The patients with MDD had significantly greater midbrain volumes (P=0.013) compared to healthy controls. In particular, drug-naïve patients with MDD had significantly greater brainstem volumes compared to healthy controls (P=0.007), while no significant findings were observed between the antidepressant treatment group and healthy controls. The remitted patient group had reduced pons (P=0.002) and midbrain (P=0.005) volumes compared to healthy controls, while the non-remitted MDD patient group had significantly greater midbrain volumes compared to the healthy controls (P=0.017).Limitations: We could not distinguish gray versus white matter volumes changes in our analysis.Conclusions: We observed that the midbrain is enlarged in patients with a current depressive episode, who are not undergoing antidepressant treatment. This volume then returns to normal after antidepressant treatment, and is even reduced, when the patient is in remission. Further studies are needed to confirm our observations. [ABSTRACT FROM AUTHOR]

Background: Acute-onset neurodegenerative diseases in older patients are rare clinical cases, especially when the degeneration only affects specific regions of the nervous system. Several neurological disorders have been described in which the degeneration of brain parenchyma originates from and/or primarily affects the brain stem. Clinical diagnosis in these patients, however, is often complicated due to a poor understanding of these diseases and their underlying mechanisms. Case presentation: In this manuscript we report on a 73-year-old female who had experienced a sudden onset of complex neurological symptoms that progressively worsened over a period of 2 years. Original evaluation had suggested a MRI-negative stroke as underlying pathogenesis. The combination of patient's medical history, clinical examination and exceptional pattern of brain stem degeneration presenting as "kissing swan sign" in MR imaging was strongly suggestive of acute onset of Alexander's disease. This leukoencephalopathy is caused by GFAP(glial fibrilary acidic protein) gene mutations and may present with brain stem atrophy and stroke-like onset of symptoms in elderly individuals. However, a pathognomonic GFAP gene mutation could not be identified by Sanger sequencing. Conclusions: After an extended differential diagnosis and exclusion of other diseases, a definite diagnosis of the patient's condition presently remains elusive. However, whole-exome sequencing performed from patient's blood revealed 12 potentially disease-causative heterozygous variants, amongst which several have been associated with neurological disorders in vitro and in vivo-in particular the axon degeneration-related NMNAT2 gene. [ABSTRACT FROM AUTHOR]

Aim: To examine brainstem auditory function at 36-37weeks of postconceptional age in preterm infants who are diagnosed to have neonatal chronic lung disease (CLD).Study Design: Preterm infants, born at 31 and less weeks of gestation, were studied at 36-37weeks of postconceptional age when they were diagnosed to have neonatal CLD. Brainstem auditory evoked response (BAER) was recorded and analyzed at different click rates.Results: Compared with healthy controls at the same postconceptional age, the CLD infants showed a slightly increase in BAER wave V latency. However, the I-V, and III-V interpeak intervals in the CLD infants were significantly increased. The III-V/I-III interval ratio was also significantly increased. The amplitudes of BAER waves III and V in the CLD infants tended to be reduced. These BAER findings were similar at all 21, 51 and 91/s clicks, although the abnormalities tended to be more significant at higher than at low click rates.Conclusion: At 36-37weeks of postconceptional age, BAER was abnormal in preterm infants who were diagnosed to have neonatal CLD. This suggests that at time when the diagnosis of CLD is made there is functional impairment, reflecting poor myelination, in the brainstem auditory pathway in preterm infants with neonatal CLD. [ABSTRACT FROM AUTHOR]

Abnormality in balance is one of the most important causes of gait disturbance which has a direct impact to disability and medical cost in multiple sclerosis (MS) and neuromyelitis optica (NMO). However, characteristics of imbalance in these two diseases have not been fully elucidated. The aim of this study was to evaluate the degree and features of imbalance using stabilography, the degree of deep sensation disturbance using tibial nerve somatosensory evoked potentials (SEP), and their association with clinical impairment, in patients with MS and NMO. Seven NMO patients and seven MS patients with balance disturbance were examined. The relationship among stabilography measurements representing the degree and features of imbalance, height-adjusted P38 peak latency of SEP, and neurological functional disability, were analyzed. Stabilography evaluation showed a significantly severer degree of imbalance in NMO than in MS. Romberg quotient of the patients with brainstem lesions was significantly larger than those without them. In all patients, length of excursion per second significantly correlated positively with anterio-posterior-axis power spectra at intermediate frequency band. In all patients and in NMO, P38 peak latency adjusted by height significantly correlated positively with anterio-posterior-axis power spectra at intermediate frequency band. These findings suggest that the degree of imbalance of MS and NMO possibly correlate with deep sensation disturbance, which could be evaluated by anterio-posterior-axis power spectra at intermediate frequency band by stabilography. Severer imbalance in NMO than MS may be associated with the severe longitudinally extensive spinal cord lesions. [ABSTRACT FROM AUTHOR]

Unilateral hyperhidrosis of neurological origin has been associated with head trauma, cerebral palsy, spinal cord injury, peripheral neuropathy, lesions of the hypothalamus, and cerebral or brainstem strokes. In this report, we describe a 61-year-old man with isolated sweating on the left side of his entire body. A right-sided brainstem meningioma producing mass effect is suspected as the underlying etiology. [ABSTRACT FROM AUTHOR]

Background To report the diversity of MRI features of brainstem encephalitis (BE) induced by Enterovirus 71. This is supported by implementation and testing of our new classification scheme in order to improve the diagnostic level on this specific disease. Methods Neuroimaging of 91 pediatric patients who got EV71 related BE were hospitalized between March, 2010 to October, 2012, were analyzed retrospectively. All patients underwent preand post-contrast MRI scan. Thereafter, 31 patients were randomly called back for followup MRI study during December 2013 to August 2014. The MRI signal patterns of BE primary lesion were analyzed and classified according to MR signal alteration at various disease stages. Findings in fatal and non-fatal cases were compared, and according to the MRI scan time point during the course of this disease, the patients' conditions were classified as 1) acute stage, 2) convalescence stage, 3) post mortem stage, and 4) long term follow-up study. Results 103 patients were identified. 11 patients did not undergo MRI, as they died within 48 hours. One patient died on 14th day without MR imaging. 2 patients had postmortem MRI. Medical records and imaging were reviewed in the 91 patients, aged 4 months to 12 years, and two cadavers who have had MRI scan. At acute stage: the most frequent pattern (40 patients) was foci of prolonged T1 and T2 signal, with (15) or without (25) contrast enhancement. We observed a novel pattern in 4 patients having foci of low signal intensity on T2WI, with contrast enhancement. Another pattern in 10 patients having foci of contrast enhancement without abnormalities in T1WI or T2WI weighted images. Based on 2 cases, the entire medulla and pons had prolonged T1 and T2 signal, and 2 of our postmortem cases demonstrated the same pattern. At convalescence stage, the pattern observed in 4 patients was foci of prolonged T1 and T2 signal without contrast enhancement. Follow-up MR study of 31 cases showed normal in 26 cases, and demonstrated foci of prolonged T1 and T2 signal with hyper-intensity on FLAIR in 3 cases, or of prolonged T1 and T2 signal with hypo-intensity on FLAIR in 2 cases. Most importantly, MR findings of each case were thoroughly investigated and classified according to phases and MRI signal alteration. Conclusions This study has provided enhanced and useful information for the MRI features of BE induced by EV71, apart from common practice established by previous reports. In addition, a classification scheme that summarizes all types of features based on the MRI signal at the four different stages of the disease would be helpful to improve the diagnostic level. [ABSTRACT FROM AUTHOR]

Background: Acute brainstem syndrome (ABS) may herald multiple sclerosis (MS), neuromyelitis optica (NMO), or occur as an isolated syndrome. The aquaporin 4 (AQP4)-specific serum autoantibody, NMO-IgG, is a biomarker for NMO. However, the role of anti-AQP4 antibody in the conversion of ABS to NMO is unclear. Methods: Thirty-one patients with first-event ABS were divided into two groups according to the presence of anti-AQP4 antibodies, their clinical features and outcomes were retrospectively analyzed. Results: Fourteen of 31 patients (45.16 %) were seropositive for NMO-IgG. The 71.43 % of anti-AQP4 (+) ABS patients converted to NMO, while only 11.76 % of anti-AQP4 (-) ABS patients progressed to NMO. Anti-AQP4 (+) ABS patients demonstrated a higher IgG index (0.68 ± 0.43 vs 0.42 ± 0.13, p < 0.01) and Kurtzke Expanded Disability Status Scale (4.64 ± 0.93 vs 2.56 ± 0.81, p < 0.01) than anti-AQP4 (-) ABS patients. Area postrema clinical brainstem symptoms occurred more frequently in anti-AQP4 (+) ABS patients than those in anti-AQP4 (-) ABS patients (71.43 % vs 17.65 %, p = 0.004). In examination of magnetic resonance imaging (MRI), the 78.57 % of anti-AQP4 (+) ABS patients had medulla-predominant involvements in the sagittal view and dorsal-predominant involvements in the axial view. Conclusions: ABS represents an inaugural or limited form of NMO in a high proportion of anti-AQP4 (+) patients. [ABSTRACT FROM AUTHOR]

Stillbirth is one of the most stressful life events affecting over 3 million pregnancies per year throughout the world. An accurate autopsy of the stillborn fetus, including the placenta and umbilical cord examination, should be performed promptly after delivery. A thorough maternal history also should be taken, including exposures to risk factors. In many cases a death cause, attributable to fetal, maternal, or placental pathology, is clearly identified. However, in 50% or more of cases the cause remains unknown. The purpose of this study is to highlight possible developmental alterations of the autonomic nervous system in unexplained stillbirths to provide an explanation of the pathogenetic mechanism of their death. We conducted a careful neuropathological study of the brainstem, where the main vital centers are located, in 85 unexplained stillbirths and 52 age-matched controls died of known cause. Information on the maternal lifestyle, including the smoking habit, was collected in all cases. Hypodevelopment of neuronal centers involved in breathing control, all connected together in a “respiratory network”, precisely hypoplasia of the facial/parafacial complex, Kölliker-Fuse nucleus, pre-Bötzinger nucleus and intermediolateral nucleus, were frequently observed in unexplained deaths, significantly related to maternal cigarette smoking. We support the hypothesis of a strong action of maternal smoking during pregnancy on the development of brainstem respiratory nuclei and suggest an explanation of the high incidence of the respiratory network alterations in unexplained fetal death, when breathing not represents a vital function. [ABSTRACT FROM AUTHOR]

We report case of a 48 years old woman bitten on her right foot by a Bothrops atrox viper. As a result, she developed a severe coagulopathy which improved with application of polyvalent antivenom. Four days after bite she suffered a devastating brainstem ischemic stroke. Possible pathogenetic mechanisms are discussed. [ABSTRACT FROM AUTHOR]

While brain stem involvement in multiple sclerosis (MS) is relatively common, isolated cranial nerve palsies are rare, especially when they represent the initial presenting sign of a new diagnosis of MS. This report describes a patient with no prior history of MS whose sole presenting sign was an isolated abducens palsy. An enhancing pontine lesion was found on MRI which correlated with his abducens palsy, and additional nonactive lesions on MRI led to a diagnosis of MS. This case demonstrates the importance of considering MS as part of the differential diagnosis of patients with isolated cranial nerve palsies. [ABSTRACT FROM AUTHOR]

Brainstem dysfunctions are associated to high risk of developing severe disability in patients with multiple sclerosis (PwMS), often undetected by conventional routine assessments. In this view, the purpose of this study was to monitor brainstem function over a short-term period in PwMS, comparing clinical and magnetic resonance imaging (MRI) examinations with evoked potentials (EPs) and brainstem reflexes (BSRs). Forty-five PwMS were evaluated at baseline and after 15.1 ± 4.2 months through Expanded Disability Status Scale (EDSS) score, MRI, EPs, vestibulo-masseteric (VMR), acoustic-masseteric (AMR), vestibulo-collic (VCR) and trigemino-collic (TCR) reflexes. At baseline, brainstem alterations were detected by EDSS, MRI, EPs and BSRs in 40, 77.8, 84.4 and 82.2 % of patients, respectively. At follow-up, EDSS and MRI remained unchanged, while EP and BSR deteriorated in 86.7 and 91.1 % of patients, respectively. Changes from 1 to 3 altered EPs and from 1 to 4 altered BSRs were significant only for EPs (p = 0.028). The analysis of grading severity for each test disclosed significant worsening of the VMR, AMR, TCR and P14 wave of the median somatosensory EP. Combined EP/BSR recordings were significantly more sensitive than paired EDSS/MRI assessments at baseline (93.3 versus 80 %; p = 0.006) and follow-up (97.8 versus 82.2 %; p = 0.008). In the short-term VMR, AMR, TCR and P14 wave disclosed a significant functional brainstem deterioration by detecting lesions that remained clinically and MRI silent. Our findings provide evidence for a valuable role of neurophysiological methods, especially BSRs, in investigating and monitoring brainstem dysfunctions in MS, in comparison with the standard clinical and MRI procedures. [ABSTRACT FROM AUTHOR]

Brainstem gliomas are not nearly as common in adults as they are in children. They are likely the final common consequence not of a single disease process but of several. They can be difficult to diagnose, and are challenging to treat. Clinical studies of this diagnosis are few and generally small. Because of these factors, our understanding of the biology of adult brainstem glioma is incomplete. However, the knowledge base is growing and progress is being made. In this article, we review the current state of knowledge for brainstem glioma in adults and identify key areas for which additional information is required. [ABSTRACT FROM AUTHOR]

Background: Pontine infarcts are common and often attributed to small vessel disease (''small deep infarcts'') or basilar branch atherosclerosis ("wedge shaped"). A well-described morphological differentiation using magnetic resonance images has not been reported. Furthermore, whether risk factors and outcomes differ by morphology, or whether infarct morphology should guide secondary prevention strategy, is not well characterized. Methods: All participants in the Secondary Prevention of Small Subcortical Strokes Study with magnetic resonance imaging-proven pontine infarcts were included. Infarcts were classified as well-circumscribed small deep (small deep infarct, i.e. lacunar), paramedian, atypical paramedian, or other based on diffusion-weighted imaging, T2/fluid-attenuated inversion recovery, and T1-magnetic resonance images. Inter-rater reliability was high (90% agreement, Cohen's kappa=0.84). Clinical and radiologic features independently associated with small deep infarct versus paramedian infarcts were identified (multivariable logistic regression). Differences in stroke risk and death were assessed using Cox proportional hazards. Results: Of the 3020 patients enrolled, 644 had pontine infarcts; 619 images were available: 302(49%) small deep infarct, 245 (40%) paramedian wedge, 35 (6%) atypical paramedian, and 37 (6%) other. Among vascular risk factors, only smoking (OR 2.1, 95% CI 1.3-3.3) was independently associated with small deep infarct versus paramedian infarcts; on neuroimaging, old lacunes on T1/fluid-attenuated inversion recovery (OR 1.8, 1.3-2.6) and intracranial stenosis (any location) -50% (OR 0.62, 0.41-0.96). Small deep infarct versus paramedian was not predictive of either recurrent stroke or death, and there was no interaction with assigned treatment. Conclusions: Pontine infarcts can be reliably classified based on morphology using clinical magnetic resonance images. Few risk factors differed between small deep infarct and paramedian infarcts with no differences in recurrent stroke or mortality. There was no difference in response to different antiplatelet or blood pressure treatment strategies between these two groups. [ABSTRACT FROM AUTHOR]

Background: We describe a unique case of expansive diffuse brainstem lesion diagnosed prenatally by magnetic resonance imaging (MRI) with long-term survival. Findings of fetal and postpartum MRI were highly consistent with the characteristics of diffuse brainstem glioma. Methods: Diagnosis was based on the features of MRI, and histopathology was not confirmed by biopsy. Although the prognosis of diffuse brainstem tumor is usually poor, this child was asymptomatic at birth and the neurological condition is still normal at 4 years of age without any treatment. Results: During routine imaging follow-up, diameters of the expansion have remained stable, while the size of the lesion compared to the posterior fossa size has diminished. In addition to brainstem tumor, a skin lesion of the back was observed and MRI of the thoracic spine showed a large asymptomatic extradural cystic lesion suggesting an arachnoid cyst. The pontine tumor of this infant, in agreement with a few previously reported cases, suggests a subgroup of beneficial outcome of expansive diffuse brainstem lesions, particularly in the neonatal period. Discussion: In this article, we discuss the prognosis and characteristics of pediatric brainstem tumors and differential diagnosis of neonatal brainstem lesions. [ABSTRACT FROM AUTHOR]

The static subjective visual vertical (SVV) was assessed in 24 healthy volunteers with different preset angles (i.e., 10, 20, and 30 degrees), and in 20 other volunteers, the static SVV was tested and retested 1 week later. The static SVV results are influenced by the side of the preset angle (Wilcoxon test,p≤ 0.001), but not by the preset angle deviation. The test-retest static SVV outcomes are stable at a group level; however, they show statistically relevant variability at an individual level (−0.240 ≤ intraclass correlation coefficient [ICC] ≤ 0.508). A robust static SVV protocol is described in this paper. [ABSTRACT FROM PUBLISHER]

Autism spectrum disorder (ASD) is a group of neurodevelopmental conditions characterized by difficulties in communication and social interactions, restricted, repetitive behaviors and sensory abnormalities. Notably, the vast majority of individuals with ASD experience some degree of auditory dysfunction and we have recently reported consistent hypoplasia and dysmorphology in auditory brainstem centers in individuals with ASD. Prenatal exposure to the antiepileptic drug valproic acid (VPA) is associated with an increased risk of ASD. In rodents, prenatal exposure to VPA is employed as an animal model of ASD and is associated with a number of anatomical, physiological and behavioral deficits, including hypoplasia and dysmorphology of auditory brainstem centers. Based on these observations, we hypothesized that such dysmorphology in VPA-exposed animals would translate into abnormal neuronal activity in brainstem circuits and irregular tonotopic maps. Herein, we have subjected control and VPA-exposed animals to 4- or 16-kHz tones and examined neuronal activation with immunohistochemistry for c-Fos. After these exposures, we identified significantly more c-Fos-positive neurons in the auditory brainstem of VPA-exposed animals. Additionally, we observed a larger dispersion of c-Fos-positive neurons and shifted tonotopic bands in VPA-exposed rats. We interpret these findings to suggest hyper-responsiveness to sounds and disrupted mapping of sound frequencies after prenatal VPA exposure. Based on these findings, we suggest that such abnormal patterns of activation may play a role in auditory processing deficits in ASD. [ABSTRACT FROM AUTHOR]

The article discusses the role of variable brain phenotypes in recessively mutated proto-oncogene protein WNT1 that causes the fragile bones disease osteogenesis imperfecta (OI). It states that brain phenotypes cerebellar and brainstem hypoplasia that leads to developmental brain defects are associated with WNT1 mutations. Several topics are discussed including spreading of Wnt1 from developing midbrain, types of mutations in WNT1 like missense, and no relation between genotypes and phenotypes.

Background: It is extremely rare to see cerebrospinal fluid dissemination of intraventricular meningioma, particularly with the development of acute, progressive brainstem/cerebellar dysfunction with an absence of mass formation in the corresponding anatomical sites.Case Presentation: An 81-year-old man was admitted because of double vision, right facial nerve palsy and truncal ataxia. Brain magnetic resonance imaging showed normal findings except for a tumor mass in the left lateral ventricle, which had been noted over 6 months previously. The patient developed hiccups, hyperventilation, and drowsiness, which worsened progressively, and did not respond to corticosteroid or intraventricular immunoglobulin therapy. Cerebrospinal fluid study revealed a mild elevation of protein, and cytology was negative. The patient died and an autopsy was performed. Postmortem investigation disclosed a malignant transformation of benign fibroid meningioma with cerebrospinal fluid dissemination of the malignant cells, diversely involving the surface of brainstem, cerebellum, and spinal cords, secondarily resulting in extensive ischemia in the brain parenchyma by vessel occlusion.Conclusion: If a patient with an intraventricular tumor develops acute, progressive neurological symptoms, the possibility that it is be caused by cerebrospinal fluid dissemination of tumor cells, after malignant transformation, should be considered. [ABSTRACT FROM AUTHOR]

Introduction. Acute complete external ophthalmoplegia is a rare finding in clinical practice that is associated with diseases affecting the neuromuscular junction, the oculomotor nerves, or the brainstem. Ophthalmoplegia has been reported with acute ataxia in Miller Fisher syndrome (MFS) and Bickerstaff brainstem encephalitis (BBE). Up to 95% of these cases are associated with anti-GQ1b antibodies. Only a small number of cases of anti-GQ1b negative MFS have been documented in pediatric patients. This is the first case reporting a recurrence of ocular symptoms in an anti-GQ1b antibody negative patient with BBE. Case Presentation. An 8-year-old Caucasian boy presented with complete external ophthalmoplegia without ptosis, cerebellar ataxia, and a disturbance of consciousness. He had recently recovered from a confirmed Campylobacter jejuni infection. On subsequent laboratory testing he was anti-GQ1b antibody negative. He had a recurrence of diplopia at four-week follow-up. Conclusions. This patient’s recurrence of diplopia was treated with a five-week course of oral corticosteroids which did not worsen his condition, and this may be a therapeutic option for similar patients. We will discuss the symptoms and treatment of reported pediatric cases of anti-GQ1b antibody negative cases of MFS and the variation between cases representing a spectrum of illness. [ABSTRACT FROM AUTHOR]