Malignant neuroleptic syndrome following deep brain stimulation surgery: a case report

<p>Abstract</p> <p>Background</p> <p>The neuroleptic malignant syndrome is an uncommon but dangerous complication characterized by hyperthermia, autonomic dysfunction, altered mental state, hemodynamic dysregulation, elevated serum creatine kinase, and rigor. It is most often caused by an adverse reaction to anti-psychotic drugs or abrupt discontinuation of neuroleptic or anti-parkinsonian agents. To the best of our knowledge, it has never been reported following the common practice of discontinuation of anti-parkinsonian drugs during the pre-operative preparation for deep brain stimulation surgery for Parkinson's disease.</p> <p>Case presentation</p> <p>We present the first case of neuroleptic malignant syndrome associated with discontinuation of anti-parkinsonian medication prior to deep brain stimulation surgery in a 54-year-old Caucasian man.</p> <p>Conclusion</p> <p>The characteristic neuroleptic malignant syndrome symptoms can be attributed to other, more common causes associated with deep brain stimulation treatment for Parkinson's disease, thus requiring a high index of clinical suspicion to timely establish the correct diagnosis. As more centers become eligible to perform deep brain stimulation, neurologists and neurosurgeons alike should be aware of this potentially fatal complication. Timely activation of the deep brain stimulation system may be important in accelerating the patient's recovery.</p

Pitfalls in the diagnosis of a tumefactive demyelinating lesion: A case report

<p>Abstract</p> <p>Introduction</p> <p>In rare instances, demyelinating disorders manifest as tumefactive lesions that simulate brain tumors. We report a patient with a space-occupying lesion in the parietal lobe, which presented a serious diagnostic dilemma, between a rare tumefactive demyelinating disease, such as Balo concentric sclerosis and a glioma. This case report highlights important diagnostic clues in the differential diagnosis of Balo concentric sclerosis.</p> <p>Case presentation</p> <p>A 20-year-old Caucasian woman with acute onset of left-sided weakness and numbness was admitted to hospital with neurologic signs of left-sided hemiparesis and hypoesthesia. Brain magnetic resonance imaging showed a mass lesion of abnormal signal intensity with concentric enhancing rings in the right parietal lobe, without perifocal edema. The characteristic concentric pattern detected on the magnetic resonance images was highly suggestive of Balo disease, and corticosteroids were administered. Evoked potentials, cerebrospinal fluid analysis, and magnetic spectroscopy findings were not specific, and glioma was also included in the differential diagnosis. A stereotactic biopsy was not diagnostic.</p> <p>After one month the patient showed moderate clinical improvement, and during 12 months follow-up, no further relapses occurred. In the follow-up magnetic resonance imaging, the concentric pattern had completely disappeared, and only a low-signal, gliotic lesion remained.</p> <p>Conclusion</p> <p>We hope this case presentation will advance our understanding of clinical and radiologic appearance of Balo concentric sclerosis, which is a rare demyelinating disease. Although this is a specific entity, it has a broader clinical impact across medicine, because it must be differentiated from other space-occupying lesions in the central nervous system.</p

Prevalence of Clinical and Neuroimaging Markers in Cerebral Amyloid Angiopathy: A Systematic Review and Meta-Analysis

BACKGROUND: Limited data exist regarding the prevalence of clinical and neuroimaging manifestations among patients diagnosed with cerebral amyloid angiopathy (CAA). We sought to determine the prevalence of clinical phenotypes and radiological markers in patients with CAA. METHODS: Systematic review and meta-analysis of studies including patients with CAA was conducted to primarily assess the prevalence of clinical phenotypes and neuroimaging markers as available in the included studies. Sensitivity analyses were performed based on the (1) retrospective or prospective study design and (2) probable or unspecified CAA status. We pooled the prevalence rates using random-effects models and assessed the heterogeneity using the Cochran Q and I2 statistics. RESULTS: We identified 12 prospective and 34 retrospective studies including 7159 patients with CAA. The pooled prevalence rates were cerebral microbleeds (52% [95% CI, 43%-60%]; I2=93%), cortical superficial siderosis (49% [95% CI, 38%-59%]; I2=95%), dementia or mild cognitive impairment (50% [95% CI, 35%-65%]; I2=97%), intracerebral hemorrhage (ICH; 44% [95% CI, 27%-61%]; I2=98%), transient focal neurological episodes (48%; 10 studies [95% CI, 29%-67%]; I2=97%), lacunar infarcts (30% [95% CI, 25%-36%]; I2=78%), high grades of perivascular spaces located in centrum semiovale (56% [95% CI, 44%-67%]; I2=88%) and basal ganglia (21% [95% CI, 2%-51%]; I2=98%), and white matter hyperintensities with moderate or severe Fazekas score (53% [95% CI, 40%-65%]; I2=91%). The only neuroimaging marker that was associated with higher odds of recurrent ICH was cortical superficial siderosis (odds ratio, 1.57 [95% CI, 1.01-2.46]; I2=47%). Sensitivity analyses demonstrated a higher prevalence of ICH (53% versus 16%; P=0.03) and transient focal neurological episodes (57% versus 17%; P=0.03) among retrospective studies compared with prospective studies. No difference was documented between the prevalence rates based on the CAA status. CONCLUSIONS: Approximately one-half of hospital-based cohort of CAA patients was observed to have cerebral microbleeds, cortical superficial siderosis, mild cognitive impairment, dementia, ICH, or transient focal neurological episodes. Cortical superficial siderosis was the only neuroimaging marker that was associated with higher odds of ICH recurrence. Future population-based studies among well-defined CAA cohorts are warranted to corroborate our findings

Stereotactic Anatomy of the Human Subthalamic Nucleus: Providing Coordinates for Accurate Electrode Placement

Background and Study Aims Stereotactic targeting of the subthalamic nucleus (STN) for insertion of deep brain stimulation (DBS) electrodes became popular in the last two decades. We present a study focused on the detailed stereotactic anatomy of the STN with respect to its targeting. Material and Methods We used cerebral magnetic resonance (MR) images from 26 patients (50 STNs) for the imaging study. The material of the anatomical study consisted of 24 cerebral hemispheres (24 STNs) from 16 normal human brains. We measured and analyzed the X, X’, Y, Y’, and Z, Z’ stereotactic coordinates of the STN at specific clinically important transverse, coronal, and sagittal planes. Results The STN extended almost 1.5 mm more lateral and almost 1 mm more posterior in patients &lt; 60 years of age. The Y coordinate was more posterior and the X coordinate more lateral on MRI than in anatomical specimens. The left STN was located more lateral than the right STN. Analyzing our data we found a standard coronal zone 1.1 mmwide (zone T), a standard coronal plane (thus providing the most accurate Y target coordinate, plane M), and a standard transverse area (area C) for accurate right STN targeting. Conclusions Our study offers new insights into the stereotactic anatomy of the human STN. These data should be taken into account if performing STN DBS

Exploring the neurosurgical anatomy of the human insula: a combined and comparative anatomic-radiologic study

The human insula constitutes the invaginated portion of the cerebral cortex and is less well understood than other cortical areas because of its hidden location. Our purpose was to study the neurosurgical anatomy of the human insula via a combined and comparative study of its gross and imaging anatomy. We totally studied the anatomy of 148 normal insulae in anatomic specimens and radiologic images. We evaluated the number of all insular gyri (short and long), we statistically analyzed our results, and we made comparisons among hemispheres and gender. We also compared the two studies. Finally, we searched in the literature to make comparisons with other authors and to add our experience to the today existing knowledge of the insular anatomy. We found a significantly greater value of the insular gyri number for males, potential underestimation of the real insular gyri number by MRI and that the classic insular gyri pattern can rarely be absent. The middle short insular gyrus can be indistinguishable more likely on MRIs than during surgery, the long insular gyri are less curved than the short gyri, and finally, the insular perforating vessels usually originate at the inferior insular part. Deep knowledge of the gross, imaging, and surgical anatomy of the insula is of paramount importance for neurosurgeons dealing with disorders in this area. The male insula is larger (increased gyri pattern) than the female. Moreover, the classic insular gyri pattern can rarely be absent, probably as a normal anatomic variation

The Role of Antisiphon Devices in the Prevention of Central Ventricular Catheter Obliteration for Hydrocephalus: A 15-Years Institution&rsquo;s Experience Retrospective Analysis

Shunt over-drainage in patients harboring a ventriculoperitoneal shunt constitutes one of the most devastating, and difficult to manage, side effects associated with this operation. Siphoning is one of the most important contributing factors that predispose to this complication. Based on the fact that the predisposing pathophysiologic mechanism is considerably multiplicated, amelioration of that adverse condition is considerably difficult to achieve. A lot of evidence suggests that the widespread utilization of gravitational valves or antisiphon devices is of utmost importance, in order to minimize or even avoid the occurrence of such complications. The recent literature data highlight that gravity-related, long-lasting shunt over-drainage consists of a momentous factor that could be considered one of the main culprits of central shunt failure. A lot of efforts have been performed, in order to design effective means that are aimed at annihilating siphoning. Our tenet was the investigation of the usefulness of the incorporation of an extra apparatus in the shunt system, capable of eliminating the impact of the siphoning effect, based on the experience that was gained by their long-term use in our institution. A retrospective analysis was performed, based on the data that were derived from our institution&rsquo;s database, centered on patients to which an ASD was incorporated into their initial shunt device between 2006 and 2021. A combination of clinical, surgical, radiological findings, along with the relevant demographic characteristics of the patients were collected and analyzed. We attempted to compare the rates of shunt dysfunction, attributed to occlusion of the ventricular catheter, in a group of patients, before and after the incorporation of an anti-siphon device to all of them. A total number of 120 patients who have already been shunted due to hydrocephalus of different etiologies, were managed with the insertion of an ASD. These devices were inserted at different anatomical locations, which were located peripherally to the initially inserted valvular mechanism. The data that were collected from a subpopulation of 17 of these patients were subjected to a separate statistical analysis because they underwent a disproportionately large number of operations (i.e., &gt;10-lifetime shunt revisions). These patients were studied separately as their medical records were complicated. The analysis of our records revealed that the secondary implementation of an ASD resulted in a decrease of the 1-year and 5-year central catheter dysfunction rates in all of our patients when compared with the relevant obstruction rates at the same time points prior to ASD insertion. According to our data, and in concordance with a lot of current literature reports, an ASD may offer a significant reduction in the obstruction rates that is related to the ventricular catheter of the shunt. These data could only be considered preliminary and need to be confirmed with prospective studies. Nevertheless, this study could be considered capable of providing supportive evidence that chronic shunt over-drainage is a crucial factor in the pathophysiology of shunt malfunction. Apart from that, it could provide pilot data that could be reviewed in order to organize further clinical and laboratory studies, aiming toward the assessment of optimal shunt valve systems that, along with ASD, resist siphoning

The Role of Antisiphon Devices in the Prevention of Central Ventricular Catheter Obliteration for Hydrocephalus: A 15-Years Institution’s Experience Retrospective Analysis

Shunt over-drainage in patients harboring a ventriculoperitoneal shunt constitutes one of the most devastating, and difficult to manage, side effects associated with this operation. Siphoning is one of the most important contributing factors that predispose to this complication. Based on the fact that the predisposing pathophysiologic mechanism is considerably multiplicated, amelioration of that adverse condition is considerably difficult to achieve. A lot of evidence suggests that the widespread utilization of gravitational valves or antisiphon devices is of utmost importance, in order to minimize or even avoid the occurrence of such complications. The recent literature data highlight that gravity-related, long-lasting shunt over-drainage consists of a momentous factor that could be considered one of the main culprits of central shunt failure. A lot of efforts have been performed, in order to design effective means that are aimed at annihilating siphoning. Our tenet was the investigation of the usefulness of the incorporation of an extra apparatus in the shunt system, capable of eliminating the impact of the siphoning effect, based on the experience that was gained by their long-term use in our institution. A retrospective analysis was performed, based on the data that were derived from our institution’s database, centered on patients to which an ASD was incorporated into their initial shunt device between 2006 and 2021. A combination of clinical, surgical, radiological findings, along with the relevant demographic characteristics of the patients were collected and analyzed. We attempted to compare the rates of shunt dysfunction, attributed to occlusion of the ventricular catheter, in a group of patients, before and after the incorporation of an anti-siphon device to all of them. A total number of 120 patients who have already been shunted due to hydrocephalus of different etiologies, were managed with the insertion of an ASD. These devices were inserted at different anatomical locations, which were located peripherally to the initially inserted valvular mechanism. The data that were collected from a subpopulation of 17 of these patients were subjected to a separate statistical analysis because they underwent a disproportionately large number of operations (i.e., >10-lifetime shunt revisions). These patients were studied separately as their medical records were complicated. The analysis of our records revealed that the secondary implementation of an ASD resulted in a decrease of the 1-year and 5-year central catheter dysfunction rates in all of our patients when compared with the relevant obstruction rates at the same time points prior to ASD insertion. According to our data, and in concordance with a lot of current literature reports, an ASD may offer a significant reduction in the obstruction rates that is related to the ventricular catheter of the shunt. These data could only be considered preliminary and need to be confirmed with prospective studies. Nevertheless, this study could be considered capable of providing supportive evidence that chronic shunt over-drainage is a crucial factor in the pathophysiology of shunt malfunction. Apart from that, it could provide pilot data that could be reviewed in order to organize further clinical and laboratory studies, aiming toward the assessment of optimal shunt valve systems that, along with ASD, resist siphoning

The Entity of Cerebellar Mutism Syndrome: A Narrative Review Centered on the Etiology, Diagnostics, Prevention, and Therapeutic Options

Cerebellar mutism syndrome (CMS), also known as posterior fossa syndrome, is an entity that entails a constellation of signs and symptoms which are recorded in a limited number of pediatric patients who have been operated on mainly for tumors involving the posterior cranial fossa, and more precisely, the region of the vermis. Medulloblastoma seems to constitute the most commonly recognized pathological substrate, associated with this entity. The most prevalent constituents of this syndrome are noted to be a, often transient, although protracted, language impairment, emotional lability, along with cerebellar and brainstem dysfunction. Apart from that, a definite proportion of involved individuals are affected by irreversible neurological defects and long-lasting neurocognitive impairment. A bulk of literature and evidence based on clinical trials exist, which reflect the continuous effort of the scientific community to highlight all perspectives of this complex phenomenon. There are several circumstances that intervene in our effort to delineate the divergent parameters that constitute the spectrum of this syndrome. In summary, this is implicated by the fact that inconsistent nomenclature, poorly defined diagnostic criteria, and uncertainty regarding risk factors and etiology are all constituents of a non-well-investigated syndrome. Currently, a preliminary consensus exists about the identification of a group of diagnostic prerequisites that are managed as sine qua non, in our aim to document the diagnosis of CMS. These include language impairment and emotional lability, as proposed by the international Board of the Posterior Fossa Society in their consensus statement. It is common concept that midline tumor location, diagnosis of medulloblastoma, younger age at diagnosis, and preoperatively established language impairment should be accepted as the most determinant predisposing conditions for the establishment of this syndrome. A well-recognized pathophysiological explanation of CMS includes disruption of the cerebellar outflow tracts, the cerebellar nuclei, and their efferent projections through the superior cerebellar peduncle. Despite the relative advancement that is recorded regarding the diagnostic section of this disease, no corresponding encouraging results are reported, regarding the available treatment options. On the contrary, it is mainly targeted toward the symptomatic relief of the affected individuals. The basic tenet of our review is centered on the presentation of a report that is dedicated to the definition of CMS etiology, diagnosis, risk factors, clinical presentation, and clinical management. Apart from that, an effort is made that attempts to elucidate the paramount priorities of the scientific forum, which are directed toward the expansion our knowledge in the era of diagnostics, prevention, and therapeutic options for patients suffering from CM, or who are at risk for development of this syndrome

Asymptomatic massive subdural hematoma in a patient with bitemporal agenesis and bilateral temporal arachnoid cysts

The case of a 38-year-old man with a history of chronic migraine is reported. Despite a 3 week history of changes in his migraine pattern, a normal neurologic examination led to conservative treatment. He later presented with worsening headaches and imbalance; tendon reflexes were increased on the right side, and brain computed tomography and magnetic resonance imaging revealed a massive subacute subdural hematoma over the left hemisphere, developing on the grounds of bilateral temporal agenesis. The presence of bilateral temporal arachnoid cysts along with biternporal agenesis altered clinical findings, causing only mild symptoms where an otherwise acute and devastating neurologic deterioration would be expected