Arachnoid Cyst of the Cerebellopontine Angle: A Systematic Literature Review.

OBJECTIVE: The role of surgical management of arachnoid cyst (AC) of the cerebellopontine angle (CPA) is uncertain. This topic has remained controversial with varying contradictory recommendations in the literature, which is limited to mostly case reports. We aimed to provide a comprehensive summary and analysis of symptoms, operative techniques, outcomes, and recurrence of all available surgical cases of AC of the CPA to date. METHODS: A systematic literature search was performed in May 2022 querying several scientific databases. Inclusion criteria specified all studies and case reports of patients with AC located at the CPA for which any relevant surgical procedures were performed. RESULTS: A total of 55 patients from the literature and 5 treated at our institution were included. Mean patient age was 29 years (range, 0.08-79 years), with nearly twice (1.7×) as many female as male patients (37 female, 22 male). Headaches (35%), hearing loss (30%), vertigo (22%), and ataxia (22%) were the most common presentations. Following surgery, 95% experienced symptom improvement, with complete resolution in 64%. Of patients with hearing loss, 44% reported a return to normal. The rate of mortality was 1.69%, and 10% of tumors recurred (mean follow-up 2.3 years [range, 0-15 years]. CONCLUSIONS: Symptomatic AC of the CPA is rare. It exhibits a proclivity for females and commonly manifests with headache, hearing loss, vertigo, and ataxia. While careful selection for surgical candidacy is needed and intervention should be reserved for patients with severe symptoms, surgical decompression is an effective tool for symptom alleviation and recovery.

Micro-structural white matter abnormalities in new daily persistent headache: a DTI study using TBSS analysis.

BACKGROUND: New daily persistent headache (NDPH) is a rare primary headache disorder characterized by daily and persistent sudden onset headaches. The pathogenesis of NDPH remains unclear, and there are few white matter imaging studies related to NDPH. The purpose of this study was to investigate the micro-structural abnormalities of white matter in NDPH and provided insights into the pathogenesis of this disease based on tract-based spatial statistics (TBSS). METHODS: Twenty-one patients with NDPH and 25 healthy controls (HCs) were included in this study. T1 structural and diffusion magnetic resonance imaging (MRI) were acquired from all participants. Differences in the fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (AD), and radial diffusivity (RD) between patients with NDPH and HCs were investigated using TBSS analysis. RESULTS: Significantly decreased FA, increased MD and RD were found in patients with NDPH compared to HCs. White matter regions overlaid with decreased FA, increased MD and RD were found in 16 white matter tracts from the Johns Hopkins University ICBM-DTI-81 White-Matter Atlas and Johns Hopkins University White-Matter Tractography Atlas. Specifically, these white matter regions included the right anterior thalamic radiation (ATR), body of the corpus callosum (BCC), bilateral cingulum, left hippocampal cingulum (CGH), left corticospinal tract (CST), forceps major, fornix, left inferior fronto-occipital fasciculus (IFOF), bilateral inferior longitudinal fasciculus (ILF), left posterior limb of the internal capsule (PLIC), right retrolenticular part of the internal capsule (RPIC), splenium of the corpus callosum (SCC), right superior longitudinal fasciculus (SLF) and left uncinate fasciculus (UF). After Bonferroni correction, there were no correlations between the FA, MD, AD and RD values and the clinical characteristics of patients with NDPH (p > 0.05/96). CONCLUSION: The results of our research indicated that patients with NDPH might have widespread abnormalities in the white matter of the brain.

Giant Arachnoid Granulations: Diagnostic Workup and Characterization in Three Symptomatic Adults.

Giant arachnoid granulations (GAGs) are poorly investigated. Here, we document clinical findings associated with five new GAGs and illustrate the anatomical composition of these structures as well as diagnostic considerations in three symptomatic adults. The GAGs ranged from 1.1 to 3.6 cm (mean, 2.2 cm) in maximum dimension and manifested in middle-aged individuals who presented with long-standing brain mass and/or chronic headache. On imaging examinations, the tissues appeared as irregular parasagittal and/or perisinus structures that demonstrated heterogeneous internal elements. The GAGs abutted dura, extended through calvarial marrow spaces, and impinged on dural venous sinuses, causing their stenosis. The histologic workup of two GAG specimens resected from separate individuals revealed central collagen with pronounced internal vascular proliferation. One specimen additionally exhibited reactive changes within the lesion, including venous thrombosis, hemorrhage, and conspicuous inflammation. The salient immune component consisted of a foam cell-rich infiltrate that obstructed subcapsular and internal sinusoidal GAG spaces. Within this specimen, meningothelial hyperplasia was also appreciated. Notably, proliferated lymphatic vascular elements were additionally observed within the structure, extending into deep central collagen regions and engulfing many extravasated erythrocytes in the subcapsular space. In both surgically treated patients, symptoms resolved completely following resection. This report is the first to definitively depict reactive vascular and immunological changes within GAGs that were clinically associated with headache. The frequency of reactive changes within these meningeal structures is unclear in the literature, as GAGs are rarely sampled and investigated. Further systematic analyses are warranted to elucidate the causes and consequences of GAG genesis and their roles in physiology and disease states.

Dots and spots: A retrospective review of T2-hyperintense white matter lesions in pediatric patients with and without headache.

OBJECTIVE: We aimed to determine if T2-weighted hyperintense white matter lesions (WMLs) on brain magnetic resonance imaging (MRI) occur more frequently in pediatric patients with migraine and other primary headache disorders compared to the general pediatric population. BACKGROUND: Small foci of T2 hyperintensity in the white matter are frequently identified on brain MRI during the workup of pediatric headache. Such lesions have been reported to be more common among adults with migraine versus adults without migraine; however, this association has not been well established in the pediatric population. METHODS: We performed a retrospective cross-sectional single-center study of electronic medical records and radiologic studies, examining pediatric patients from 3 to 18 years old who underwent brain MRI between 2016 and 2021. Patients with existing intracranial disease or abnormalities were excluded. Patients with reports of headache were categorized. Imaging was reviewed to determine the number and location of WMLs. Headache-associated disability scores (Pediatric Migraine Disability Assessment) were noted, when available. RESULTS: Brain MRI of 248 patients with a diagnosis of headache (144 with migraine, 42 with non-migraine primary headache, and 62 with headache that could not be further classified) and 490 controls were reviewed. WMLs were encountered commonly among all study participants, with a prevalence of 40.5% (17/42) to 54.1% (265/490). There was no statistically significant difference comparing the number of lesions between each of the headache groups and the control group: migraine group versus control group median [interquartile range (IQR)], 0 [0-3] versus 1 [0-4], incidence rate ratio [95% confidence interval (CI)], 0.99 [0.69-1.44], p = 0.989, non-migraine headache group versus control group median [IQR], 0 [0-3] versus 1 [0-4], 0.71 [0.46-1.31], p = 0.156, headache not otherwise specified group versus control group median [IQR], 0 [0-4] versus 1 [0-4], 0.77 [0.45-1.31], p = 0.291. There was no significant correlation between headache-associated disability and the number of WMLs (0.07 [-0.30 to 0.17], rho [95% CI]). CONCLUSION: T2 hyperintense WMLs are common within the pediatric population and are not encountered more frequently in pediatric patients with migraine or other primary headache disorders. Thus, such lesions are presumably incidental and unlikely related to headache history.

Temporal Artery Biopsy: When Is It Worth the Headache?

BACKGROUND: Temporal artery biopsy is ordered when clinical symptoms and an elevated C-reactive protein values and/or erythrocyte sedimentation rates suggest giant cell arteritis. The percentage of temporal artery biopsies positive for giant cell arteritis is low. The objectives of our study were to analyze the diagnostic yield of temporal artery biopsies at an independent academic medical center and to develop a risk stratification model for triaging patients for possible temporal artery biopsy. METHODS: We retrospectively reviewed the electronic health records of all patients who underwent temporal artery biopsy in our institution from January 2010 through February 2020. We compared clinical symptoms and inflammatory marker (C-reactive protein and erythrocyte sedimentation rate) values of patients whose specimens were positive for giant cell arteritis with those of patients with negative specimens. Statistical analysis included descriptive statistics, chi-square test, and multivariable logistic regression. A risk stratification tool, which included point assignments and measures of performance, was developed. RESULTS: Of 497 temporal artery biopsies for giant cell arteritis performed, 66 were positive and 431 were negative. Jaw/tongue claudication, elevated inflammatory marker values, and age were associated with a positive result. Using our risk stratification tool, 3.4% of low-risk patients, 14.5% of medium-risk patients, and 43.9% of high-risk patients were positive for giant cell arteritis. CONCLUSIONS: Jaw/tongue claudication, age, and elevated inflammatory markers were associated with positive biopsy results. Our diagnostic yield was much lower when compared with a benchmark yield determined in a published systematic review. A risk stratification tool was developed based on age and the presence of independent risk factors.

Activation of Meningeal Afferents Relevant to Trigeminal Headache Pain after Photothrombotic Stroke Lesion: A Pilot Study in Mice.

Stroke can be followed by immediate severe headaches. As headaches are initiated by the activation of trigeminal meningeal afferents, we assessed changes in the activity of meningeal afferents in mice subjected to cortical photothrombosis. Cortical photothrombosis induced ipsilateral lesions of variable sizes that were associated with contralateral sensorimotor impairment. Nociceptive firing of mechanosensitive Piezo1 channels, activated by the agonist Yoda1, was increased in meningeal afferents in the ischemic hemispheres. These meningeal afferents also had a higher maximal spike frequency at baseline and during activation of the mechanosensitive Piezo1 channel by Yoda1. Moreover, in these meningeal afferents, nociceptive firing was active during the entire induction of transient receptor potential vanilloid 1 (TRPV1) channels by capsaicin. No such activation was observed on the contralateral hemi-skulls of the same group of mice or in control mice. Our data suggest the involvement of mechanosensitive Piezo1 channels capable of maintaining high-frequency spiking activity and of nociceptive TRPV1 channels in trigeminal headache pain responses after experimental ischemic stroke in mice.

[Mild encephalopathy with a reversible splenial lesion in a child]. / Sindrom umerennoi entsefalopatii s obratimym porazheniem valika mozolistogo tela u rebenka.

Mild encephalopathy with a reversible splenial lesion (MERS) is a clinical and radiological syndrome that can be caused by infectious and non-infectious factors. The most common neurological symptoms are impaired consciousness, impaired speech, convulsions, muscle weakness, ophthalmoplegia, facial paralysis, and headache. A case of a 5-year-old child with a leading clinical symptom of bilateral transient blindness and radiological characteristics of MERS syndrome is presented.

Hypophysitis.

OBJECTIVE: Hypophysitis is considered a rare inflammatory disease of the pituitary gland. For a long time, primary autoimmune hypophysitis has stood out as the most relevant type of hypophysitis. However, with the advent of immunotherapy for the treatment of malignancies and identification of hypophysitis as an immune-related adverse event, hypophysitis has garnered increasing interest and recognition. Therefore, awareness, early recognition, and appropriate management are becoming important as the indication for immunomodulatory therapies broaden. METHODS: In this review, we discuss the epidemiology, diagnosis, and treatment of hypophysitis with a focus on recent data and highlight subtypes of particular interest while recognizing the gaps in knowledge that remain. RESULTS: Regardless of cause, symptoms and signs of hypophysitis may be related to mass effect (headache and visual disturbance) and hormonal disruption that warrant prompt evaluation. In the vast majority of cases, a diagnosis of hypophysitis can be made presumptively in the appropriate clinical context with radiologic findings consistent with hypophysitis and after the exclusion of other causes. CONCLUSION: Although subtle differences currently exist in management and outcome expectations between primary and secondary causes of hypophysitis, universally, treatment is aimed at symptom management and hormonal replacement therapy.

Cavernous Hemangioma of Cavernous Sinus: An Outcome Report of 45 Surgically Treated Patients.

OBJECTIVE: To report an outcome analysis following surgical treatment of 45 patients with cavernous hemangiomas involving cavernous sinus. METHODS: From 1992 to 2020, 45 cases of cavernous hemangiomas involving the cavernous sinus were surgically treated. These patients were retrospectively analyzed. RESULTS: Patients included 12 males and 33 females; the average age was 34 years (age range, 15-61 years). Two patients had only headache as presenting symptom, and 43 patients had headache and diplopia as presenting symptoms. Investigations showed characteristic radiological imaging and encasement of internal carotid artery (35 cases), extension toward the sella, and displacement of cranial nerves III-V. An entirely extradural surgical approach was adopted in 39 cases. Cranial nerves III-V had a discrete dural covering and were always displaced over the dome of the tumor. The sixth cranial nerve was displaced on the dome of the tumor adjacent to the floor of the cavernous sinus. Three patients died in the early postoperative period, all related to excessive bleeding. In 36 patients, ocular movement dysfunction did not recover or worsened. During an average follow-up period of 110 months (range, 6 months to 27 years), 3 patients had tumor recurrence after postoperative imaging had shown complete tumor resection. CONCLUSIONS: Surgery on cavernous hemangiomas of cavernous sinus can be a challenge owing to the vascular profile and complex anatomical location. An extradural approach provides satisfactory exposure for radical tumor resection.

Gray matter alteration in medication overuse headache: a coordinates-based activation likelihood estimation meta-analysis.

Medication overuse headache (MOH) is a prevalent secondary headache, bringing heavy economic burden and neuropsychological damage. Neuroimaging studies on the disease reported divergent results. To merge the reported neuroimaging alterations in MOH patients and explore a pathophysiological mechanism of this disorder. A meta-analytic activation likelihood estimation (ALE) analysis method was used. We systematically searched English and Chinese databases for both morphological and functional neuroimaging studies published before Nov 18, 2021. Reported altered brain regions and the stereotactic coordinates of their peaks were extracted and pooled by GingerALE using Gaussian probability distribution into brain maps, illustrating converged regions of alteration among studies. We identified 927 articles, of which five studies on gray matter changes, using voxel-based morphometry (VBM) were eventually included for ALE analysis, with 344 subjects and 54 coordinates put into GingerALE. No functional magnetic resonance imaging (fMRI) or positron emission topography (PET) studies were included for pooling. Compared with healthy controls (HCs), MOH featured increased gray matter density in midbrain, striatum, cingulate, inferior parietal cortex and cerebellum (P < 0.001 uncorrected), whereas decreased gray matter density in orbitofrontal cortex (P < 0.05, family-wise error), frontal, insular and parietal cortices (P < 0.001 uncorrected). Withdrawal of analgesics led to decreased gray matter density in superior temporal gyrus, cuneus, midbrain and cerebellum (P < 0.001 uncorrected). This meta-analysis confirmed that medication overuse headache is associated with morphologic alteration in the reward system, the prefrontal cortex and a reversible modification in the pain network. Further functional imaging paradigms and longitudinal studies are required for a more definite conclusion and a causal mechanism.

Lateral Medullary Infarction with Contralateral Segmental Dysesthesia and Ipsilateral Headache: A Case Report.

INTRODUCTION: The medulla oblongata is the lowest segment of the brain stem, located adjacent to the spinal cord, with a complex anatomical structure. Thus, a small injury to the medulla oblongata can show complex clinical manifestations. CASE PRESENTATION: A patient experienced dysesthesia, which manifested as numbness in her right lower limb and decreased temperature sense, and dizziness 20 days before admission. The numbness worsened 1 week before admission, reaching the right Thoracic (T) 12 dermatomes. Her thermoception below the T12 dermatomes decreased, and the degree of dizziness increased, accompanied by nausea and vomiting. Magnetic Resonance Imaging (MRI) of the neck, chest, and abdomen performed at a local hospital showed no abnormalities. MRI of the brain was performed after admission. One week after admission, she experienced a severe headache in the upper left periorbital area. The numbness extended to T4, and thermoception decreased below T4. DIAGNOSIS: Lateral medullary infarction. INTERVENTIONS: Anti-platelet aggregation and mitochondrial nutritional therapies were performed along with treatments for improving circulation and establishing collateral circulation. OUTCOMES: The intensity of limb numbness decreased, and the symptoms of headache and dizziness resolved. CONCLUSION: Lesions leading to segmental sensory disorders can occur in the medulla oblongata. Ipsilateral headaches with contralateral segmental paresthesia can be a specific sign of lateral medullary infarction.

Micturition-triggered severe headache associated with bladder paraganglioma: A case report.

BACKGROUND: Paragangliomas are rare neuroendocrine tumors, especially in the bladder. Although many paragangliomas are non-functional, functioning paragangliomas present with many non-specific signs and symptoms, such as hypertension and headache, complicating their diagnosis. Here, we report a case of micturition-induced severe headache associated with a bladder paraganglioma.Case description: This report describes a severe headache disorder triggered by micturition and associated with a bladder paraganglioma in a middle-aged woman. Her pain occurred consistently after micturition, beginning from the left side of the nose and gradually extended to the forehead and the parietal and occipital regions. The headaches lasted 5-10 min. Removal of the paraganglioma completely eliminated the patient's pain syndrome. CONCLUSION: This case elucidates the association between micturition-triggered headaches and bladder paragangliomas. The presence of a post-micturition severe headache should suggest the possibility of a bladder paraganglioma.

Occipital Neuralgia.

PURPOSE OF REVIEW: Occipital neuralgia is a painful condition that affects the posterior aspect of the head and can be difficult to distinguish from other common forms of headaches. This article reviews the anatomy, pathophysiology, clinical presentation, differential diagnosis, diagnostic testing, and management approaches for occipital neuralgia. RECENT FINDINGS: Non-pharmacological treatments aim to alleviate muscle tension and improve posture. Acupuncture shows some promise. The occipital nerve block is considered the first line in a minimally invasive intervention, but the duration of relief may be short term. An onabotulinum toxin A injection may improve the sharp but not the dull component of the pain of occipital neuralgia. Radiofrequency ablation and occipital nerve stimulation may provide effective long-term relief in refractory patients. Surgical decompression, neurotomies, and neurolysis are last-resort treatment options. Occipital neuralgia is a debilitating condition that can be difficult to treat. Studies with larger sample sizes and randomized control trials are needed to further determine the effectiveness and safety of different therapies.

Rhinogenic and sinus headache - Literature review.

BACKGROUND: Headache is a common, yet challenging symptom to evaluate given its wide range of clinical presentations and different etiologies. For centuries, conceptual understanding of headache causation has been attributed to anatomic abnormalities of the nose and paranasal sinuses. METHODS: Structured literature review. RESULTS: The number of cases, categorized as migraines or other primary headaches, misdiagnosed as a "sinus headache" is high in the literature, ranging from 50 to 80%. The potential mechanisms for rhinogenic headaches were classically described as pain secondary to prolonged mucosal contact points, hypoxia in the paranasal sinuses secondary to poor ventilation, or pressure caused by the growth of nasal polyps. Additionally, other mechanisms were described and are still being studied. Corrective surgery for mucosal contact points in the nasal cavity is deemed necessary for relieving the headache, although patient outcomes are variable. CONCLUSION: Delay in proper diagnosis and treatment negatively impact patient quality of life. Most cases of "sinus headache" or "rhinogenic headache" seen in clinical practice are in fact misdiagnosed as either primary headaches or migraines. Because of increased misdiagnoses, Otolaryngologists should establish a direct and precise diagnosis congruent with a chief complaint being a headache. Vital information such as a good clinical history, well-performed nasal endoscopy, and occasional CT scan may decrease misdiagnosis probability.

Population pharmacokinetic model development and its relationship with adverse events of oxcarbazepine in adult patients with epilepsy.

This study aimed to develop a pharmacokinetic (PK) model of oxcarbazepine (OXC) and analyse the relationship between monohydroxylated derivative (MHD), an active metabolite of OXC, and the adverse events of OXC. We obtained 711 OXC samples from 618 patients with epilepsy who were enrolled in the Epilepsy Registry Cohort of Seoul National University Hospital from February 2011 to January 2014. The plasma PK model was developed using a nonlinear mixed-effect modelling method with NONMEM (ver 7.3). A one-compartment model with a first-order absorption model and proportional residual error adequately described the MHD concentration-time profiles. The only covariate incorporated for CL/F and V/F was body weight. Of the 447 patients analysed, 28 (6.26%) had dose-related adverse events (DRAEs), which were dizziness, somnolence, headache, and diplopia. For DRAE occurrence, the cut-off values of the MHD trough and AUC were 12.27 mg/L (specificity 0.570, sensitivity 0.643) and 698.5 mg h/L (specificity, sensitivity 0.571), respectively. Multivariate analysis showed the sole dizziness symptom was significantly associated with both the MHD trough and the AUC (p = 0.013, p = 0.038, respectively). We newly developed a population PK model using sparse sampling data from patients with epilepsy, and the model better reflects the actual clinical situation.

Muscle Fascia Changes in Patients with Occipital Neuralgia, Headache, or Migraine.

SUMMARY: In an ongoing effort to understand the pathogenesis of occipital neuralgia/headache/migraine, it is critical to describe the anatomical/tissue changes encountered during surgery. Greater occipital nerve anatomical studies mainly focus on the greater occipital nerve course through muscle/fascial planes and interaction with the occipital vessels. However, structural soft-tissue changes have not been described in detail. Anecdotally, trapezius fascia is thickened at the greater occipital nerve trigger site. This study further investigates this observation. Patients undergoing greater occipital nerve decompression surgery were enrolled prospectively in this observational study (n = 92). Tissue changes were recorded intraoperatively. The resulting data were examined. Trapezius fascia was more than 3 mm thick and appeared fibrotic in 86 patients (94 percent), whereas semispinalis muscle appeared normal in all subjects. The greater occipital nerve was macroscopically abnormal, defined as edematous, flattened, and discolored in 29 cases (32 percent). The occipital artery interacted significantly with the greater occipital nerve in 88 percent of cases. The authors conclude that the tissue structure is abnormal in patients undergoing greater occipital nerve decompression surgery. This is the first study that describes the prevalence of thickened and fibrotic appearing trapezius fascia at the occipital trigger site, a phenomenon encountered in the vast majority of patients (94 percent). This structural anomaly has a resemblance to thickened fascial tissues seen in other nerve compression syndromes, and could be related to microtrauma/overuse or actual trauma in the head and neck region.

Headaches in hypermobility syndromes: A pain in the neck?

Headache and neck pain (cervicalgia) are frequently reported among patients with joint hypermobility but the prevalence and scope of these symptoms has not been studied in the era of contemporary Ehlers-Danlos and hypermobility disorder nosology. We performed a single-center retrospective study on the incidence of head and neck symptoms in 140 patients with hypermobility disorders over a 2-year period. Overall, 93 patients (66%) reported either headache or neck pain with 49 of those (53%) reporting both. Migraine (83%) was the most common headache type among those with headache disorders and cervical spondylosis (61%) the most common pathology among those with neck symptoms. Fifty-nine percent of spondylosis patients who underwent cervical facet procedures reported significant improvement in neck and head symptoms. Of patients with both head and neck complaints, 82% had both migraine and spondylosis, which, when combined with the high response rate to injections raises the possibility of cervicogenic headache. In this large multidisciplinary retrospective study of patients with hypermobility disorders, head and neck symptoms were highly prevalent, with migraine and cervical spondylosis common, often coexisting, and frequently responsive to targeted therapy for the cervical spine suggesting that degenerative spinal pathology may cause or contribute to headache symptoms in some patients with hypermobility disorders.

Listeria rhombencephalitis mimicking acute disseminated encephalomyelitis in a patient without predisposing medical conditions.

Listeria rhombencephalitis (L. rhombencephalitis) is an uncommon form of central nervous system infection caused by Listeria monocytogenes (LM). It often occurs to immunocompetent individuals. Here, we described the case of a 45-year-old female patient without medical histories, who presented for high-grade fever, headache, and focal neurological manifestations. She was initially empirically diagnosed with acute disseminated encephalomyelitis (ADEM) because of clinical symptoms, acute clinical course, and neuroimaging. However, the biochemical analysis of cerebral spinal fluid (CSF) questioned the diagnosis of ADEM. The final diagnosis of L. rhombencephalitis was based on CSF culture for LM. Thus, L. rhombencephalitis should be preferentially and empirically considered for a patient with significantly elevated lactic acid and moderately increased red cells in CSF at early time, accompanied with rapidly progressive neurological dysfunctions involved in the brain stem.