Novel Use of Time-Resolved Magnetic Resonance Angiography to Evaluate Retrograde Lumbar Vein Flow and Epidural Venous Plexus Enhancement in Chronic Headache Patients With and Without Nutcracker Physiology.

OBJECTIVE: Compression of the left renal vein by the superior mesenteric artery, known as nutcracker phenomenon (NCP), can cause retrograde flow and congestion in communicating venous systems. It has recently been speculated that NCP can result in retrograde flow and congestion of the lumbar veins and epidural venous plexus (EVP), thereby affecting the central nervous system. This study describes the novel use of time-resolved magnetic resonance angiography (trMRA) to evaluate for retrograde left second lumbar vein (L2LV) flow and early EVP enhancement in patients with chronic daily headache (CDH) with and without NCP. METHODS: A retrospective analysis was performed of 31 patients with CDH (27 females and 4 males; median age, 38 years [range, 18-63 years]) who underwent trMRA centered over the L2LV to evaluate the direction of blood flow and presence of early EVP enhancement from May 2020 to March 2022. Descriptive statistics were performed, and anatomic associations were analyzed in patients with and without retrograde L2LV flow and early EVP enhancement. The accuracy of magnetic resonance imaging findings in detecting these flow patterns was also assessed. RESULTS: Patients with NCP who demonstrated narrowing of the left renal vein, a positive beak sign ( P = 0.052), decreased aortomesenteric distance ( P = 0.038), and decreased SMA angle demonstrated increased rates of retrograde L2LV flow and early EVP enhancement. A positive beak sign was 83% specific, and an aortomesenteric distance of ≤6.5 mm was 61% sensitive and 83% specific for identifying retrograde L2LV flow with early regional EVP enhancement in patients with CDH. CONCLUSIONS: Retrograde L2LV flow with early EVP enhancement in CDH patients can be effectively evaluated using trMRA and was seen with greater propensity in those patients with NCP.

Prediction of vascular abnormalities on CT angiography in patients with acute headache.

OBJECTIVES: Patients with acute headache increasingly undergo CT-angiography (CTA) to evaluate underlying vascular causes. The aim of this study is to determine clinical and non-contrast CT (NCCT) criteria to select patients who might benefit from CTA. METHODS: We retrospectively included patients with acute headache who presented to the emergency department of an academic medical center and large regional teaching hospital and underwent NCCT and CTA. We identified factors that increased the probability of finding a vascular abnormality on CTA, performed multivariable regression analyses and determined discrimination with the c-statistic. RESULTS: A total of 384 patients underwent NCCT and CTA due to acute headache. NCCT was abnormal in 194 patients. Among these, we found abnormalities in 116 cases of which 99 aneurysms. In the remaining 190 with normal NCCT we found abnormalities in 12 cases; four unruptured aneurysms, three cerebral venous thrombosis', two reversible cerebral vasoconstriction syndromes, two cervical arterial dissections and one cerebellar infarction. In multivariable analysis abnormal NCCT, lowered consciousness and presentation within 6 hr of headache onset were independently associated with abnormal CTA. The c-statistic of abnormal NCCT alone was 0.80 (95% CI: 0.75-0.80), that also including the other two variables was 0.84 (95% CI: 0.80-0.88). If NCCT was normal no other factors could help identify patients at risk for abnormalities. CONCLUSIONS: In patients with acute headache abnormal NCCT is the strongest predictor of a vascular abnormality on CTA. If NCCT is normal no other predictors increase the probability of finding an abnormality on CTA and diagnostic yield is low.

Choroid plexus adenoma in a child: expanding the clinical and pathological spectrum.

Primary choroid plexus tumors encompass a variety of tumors, with choroid plexus papilloma and carcinoma being the most common. Also in the differential diagnosis is the rare benign choroid plexus adenoma. As these tumors are infrequently described, the histological profile continues to evolve. The authors present a case with unusual characteristics that will broaden the pathological spectrum for choroid plexus adenomas.

Papillary glioneuronal tumors: A radiopathologic correlation.

OBJECTIVE: Papillary glioneuronal tumors (PGNT) are a rare and recently recognized tumor entity. The neuroimaging findings were reviewed to determine if any specific findings emerge to assist a preoperative diagnosis of PGNT. MATERIALS AND METHODS: Seven histologically confirmed cases of PGNT were evaluated from 2004 to 2014. Clinical, neuroimaging and histological findings were reviewed and tabulated. RESULTS: Headache and seizures were observed in 4 patients (57.1%) each. The majority (n=5, 71.4%) of lesions were periventricular and located in temporal lobe with 57.1% cases being solid cystic (n=4), and 42.9% being purely solid (n=3). Calcification and hemorrhage were noted in 3 cases (42.9%) and 5 cases (71.4%) respectively. The most frequent imaging feature was the presence of septations in the cystic component that enhanced on contrast which correlated with long pseudopapillary projections into the cyst cavity on histopathology. The solid inner component demonstrated heterogeneous enhancement. One case with tumor recurrence demonstrated hemorrhage with superficial siderosis, patchy diffusion restriction, raised choline and focal areas of raised perfusion which correlated on histopathology with increased cellularity and anaplasia. CONCLUSION: Presence of cystic mass in periventricular location with septations and a solid inner component should raise a suspicion of PGNT on neuroimaging.

Subependymomas Are Low-Grade Heterogeneous Glial Neoplasms Defined by Subventricular Zone Lineage Markers.

OBJECTIVE: Subependymomas are infrequent, low-grade gliomas associated with the ventricular system and the spinal cord. Little is known about the origin and natural history of these slow-growing lesions. METHODS: We identified all patients with pathologically proven subependymomas presenting to our institution between 1998 and 2016. We retrospectively reviewed clinical, radiographic, histologic, and surgical outcomes data in all patients who underwent surgical resection. Immunohistochemical analyses for cell lineage markers were performed. RESULTS: A total of 31 patients with pathologically proven subependymomas were identified. Of these, 7 asymptomatic lesions were discovered at autopsy and 24 symptomatic cases were treated surgically. There were 15 (48%) lateral ventricle tumors, 11 (35%) fourth ventricular tumors, and 5 (17%) spinal tumors. Symptomatic intracranial lesions most commonly presented with headaches and balance and gait abnormalities. Subependymomas had no distinguishing radiographic features that provided definitive preoperative diagnosis. At last follow-up, no patient treated surgically experienced recurrence. Immunohistochemical analyses demonstrated a diffusely GFAP-positive glial neoplasm with mixed populations of cells that were variably positive for Olig2, NHERF1, Sox2, and CD44. The Ki67 proliferation index was generally low (<1% in many of the tumors). CONCLUSIONS: Subependymomas demonstrate mixed populations of cells expressing glial lineage markers as well as putative stem cell markers, suggesting these tumors may arise from multipotent glial progenitors that reside in the subventricular zone. Definitive diagnosis requires surgical sampling. Although the clinical course of subependymomas appears benign, the inability to radiographically diagnose these lesions, and the possibility of an alternative malignant lesion support a low threshold for early and safe maximal resection.

A case of idiopathic hypertrophic pachymeningitis presenting with chronic headache and multiple cranial nerve palsies: A case report.

RATIONALE: Idiopathic hypertrophic pachymeningitis (IHP) is a rare condition, characterized by a chronic fibrosing inflammatory process usually involving either the intracranial or spinal dura mater, but rarely both. Here, we report a rare case of IHP affecting both the intracranial and spinal dura mater. We also discussed the diagnosis, management, and outcome of IHP. PATIENT CONCERNS: We reviewed the case of a 60-year-old woman presenting with chronic headache, multiple cranial nerve palsies and gait disturbance. Magnetic resonance imaging (MRI) of her head revealed thickened and contrast-enhanced dura in the craniocervical region as well as obstructive hydrocephalus and cerebellar tonsillar herniation. The patient had a suboccipital craniectomy and posterior decompression through C1 plus a total laminectomy. The dura was partially resected to the extent of the bony decompression, and a duroplasty was performed. DIAGNOSES: Microscopic examination of the surgically resected sample showed chronic inflammatory changes, lymphoplasmacytic cell infiltration, fibrous tissue hyperplasia, and hyaline degeneration. Blood tests to evaluate the secondary causes of hypertrophic pachymeningitis (HP) were unremarkable. INTERVENTIONS: Steroid was used to treat suspected IHP. OUTCOMES: Postoperatively, the patient showed gradual improvement in her headache, glossolalia, and bucking. Prior to discharge, a follow-up MRI showed improvement of the dura mater thickening. LESSONS: IHP is a chronic inflammatory disorder of the dura mater that usually causes neurological deficits. Clinical manifestations of IHP, MRI findings, and laboratory abnormalities are the essential components for making an accurate diagnosis. When the radiological or laboratory evaluation is uncertain, but neurological deficits are present, a prompt surgical approach should be considered. Postoperative steroid therapy and close observation for recurrence are necessary to ensure a good long-term outcome.

Effect of Headache on the Pathologic Findings of Unruptured Cerebral Saccular Aneurysms.

BACKGROUND: Some patients with aneurysm exhibit warning headaches without minor bleeding, and this could be caused by stretching of the aneurysm wall. Recently, our pathologic study observed subintimal fibrin deposition in a majority of the ruptured aneurysms. However, these findings also were observed in some unruptured aneurysms. In this report, 2 unruptured aneurysms exhibited subintimal fibrin, and interestingly, one of the patients experienced severe headache within 1 month before neuroimaging. OBJECTIVE: We performed pathologic analysis of unruptured aneurysms and collected their various clinical variables, including severe headache, to clarify the clinical characteristics of "dangerous" unruptured aneurysms. METHODS: This study included unruptured saccular aneurysm samples (n = 17) that were resected after clipping. We compared the differences in clinical variables, including warning headache, between aneurysms with and without fibrin deposition. RESULTS: Fibrin deposition was present in the subintimal layer in 4 patients and in the periouter membrane in 4 patients. Three of the 4 former patients experienced warning headaches, and one presented aneurysm growth. Of the latter 4 patients, one exhibited aneurysm growth, whereas the others presented with relatively large aneurysms. In the remaining 9 aneurysms without fibrin deposition, monocyte infiltration was observed in one, all aneurysms were small, and no patients experienced warning headaches or aneurysm growth. CONCLUSIONS: Subintimal fibrin deposition is observed frequently in patients with aneurysm with warning headaches. These pathologic findings are clinically inspiring and may suggest that these aneurysms exhibit rapid stretching by newly formed aneurysms, which can result in rupture at an early stage.

Cortical thickness and functional connectivity abnormality in chronic headache and low back pain patients.

This study aims to characterize the psychological wellbeing of chronic headache (CH) patients, to identify cortical structural abnormalities and any associations of those abnormalities with resting state functional connectivity (rsFC), and to determine whether such rsFC abnormality is specific to CH patients. Compared with healthy controls (CONCH ), CH patients suffered from mild depression, sleep disturbances, and relatively poor quality of life. CH patients also exhibited widespread cortical thickness (CT) abnormalities in left premotor (BA6), right primary somatosensory (S1) and right prefrontal (BA10) cortices, as well as in regions of default mode and executive control networks. Using cortical regions with thickness abnormality as seeds, we found cortical region pairs showed strengthened rsFC in CH patients. Using the same seeds, rsFC analysis from chronic low back pain (CLBP) patients and their controls (CONCLBP ) identified abnormalities in non-overlapping cortical region pairs. Direct comparison of rsFC between CH and CLBP patients revealed significantly differences in thirteen cortical region pairs, including the four identified in CH and CONCH comparison. Across all three groups (CH, CLBP and CON), the rsFC between left multisensory association area (BA39) and left posterior cingulate cortex (BA23) differed significantly. Eight regions showed CT abnormality in CLBP patients, two of which overlapped with those of CH patients. Our observations support the notion that CH and CLBP pain are pathological conditions, under which the brain develops distinct widespread structural and functional abnormalities. CH and CLBP groups share some similar structural abnormalities, but rsFC abnormalities in several cortical region pairs appear to be pathology-specific. Hum Brain Mapp 38:1815-1832, 2017. © 2017 Wiley Periodicals, Inc.

Headache in the first manifestation of Multiple Sclerosis - Prospective, multicenter study.

Objectives: Multiple sclerosis (MS) is the most frequent immune-mediated inflammation of the central nervous system that can lead to early disability. Headaches have not been considered as MS-related symptoms initially, whereas higher prevalence rates were reported since 2000. Postmortem histological analyses of MS patients' brains revealed lymphoid follicle-like structures in the cerebral meninges which suggest a possible pathophysiological explanation for the high headache prevalence in MS. The aim of this study was to evaluate headache characteristics during the first clinical event of MS. Methods: In a prospective, multicenter study, 50 patients with the diagnosis of CIS or MS were recruited. All participants were screened for the presence of headache within the last 4 weeks with help of the Rostock Headache Questionnaire (Rokoko). Results: Thirty-nine of fifty questioned patients (78%) reported headaches within the last 4 weeks. Most patients suffered from throbbing and pulsating headaches (25, 50%), 15 (30%) reported stabbing, 14 (28%) dull and constrictive headaches. Conclusions: Headaches were prevalent in 78% of patients in our population with newly diagnosed CIS and MS. It is among the highest prevalence rates reported so far in patients with CIS or MS. Thus, headache, especially of a migraneous subtype, is a frequent symptom within the scope of the first manifestation of multiple sclerosis. If it were possible to define a MS-typical headache, patients with these headaches and with typical MRI results would be classified as CIS or early MS instead of radiologically isolated syndrome and treated accordingly with an immunomodulatory therapy.

Postpartum vertebral artery dissection.

We report a case of a right vertebral artery dissection in a 35-year-old woman, 3 weeks post partum, with manifestations of vertebrobasilar disease. She was 3 weeks out from the uneventful delivery of her fourth child, with presentation of acute neurological symptoms, predominantly intractable vertigo. Vertigo can have many non-specific generalised symptoms and clinical findings. Postpartum women have a lengthy list of possible aetiologies of vertigo not limited to our initially suspected preeclampsia, dural venous thrombosis and vertebral dissection.

Neural Plasticity in Common Forms of Chronic Headaches.

Headaches are universal experiences and among the most common disorders. While headache may be physiological in the acute setting, it can become a pathological and persistent condition. The mechanisms underlying the transition from episodic to chronic pain have been the subject of intense study. Using physiological and imaging methods, researchers have identified a number of different forms of neural plasticity associated with migraine and other headaches, including peripheral and central sensitization, and alterations in the endogenous mechanisms of pain modulation. While these changes have been proposed to contribute to headache and pain chronification, some findings are likely the results of repetitive noxious stimulation, such as atrophy of brain areas involved in pain perception and modulation. In this review, we provide a narrative overview of recent advances on the neuroimaging, electrophysiological and genetic aspects of neural plasticity associated with the most common forms of chronic headaches, including migraine, cluster headache, tension-type headache, and medication overuse headache.

Diferencias entre migraña crónica con y sin uso excesivo de medicación: experiencia en una serie hospitalaria de 434 pacientes / Chronic migraine with and without medication overuse: Experience in a hospital series of 434 patients

Introducción: La migraña crónica (MC) es una evolución de la migraña episódica favorecida por factores de riesgo, entre los que se encuentra el uso excesivo de medicación (UEM). Pretendemos comparar características clínicas y demográficas de una serie de casos de MC, con y sin UEM. Métodos: Pacientes con MC (criterios revisados 2006) atendidos en una consulta monográfica de cefaleas de un hospital terciario entre enero del 2008 y mayo del 2012. Recogimos datos demográficos, evolución, utilización previa de preventivos e impacto de la migraña medido con la escala HIT-6. esultados: Cuatrocientos treinta y cuatro pacientes (357 mujeres, 77 varones) fueron diagnosticados de MC entre un total de 1,868 (23,2%) atendidos en la mencionada consulta durante el periodo de inclusión. Doscientos cincuenta y ocho (72,2%) presentaban UEM. El 59,8% de los casos con UEM recibieron previamente tratamiento preventivo frente al 41,1% sin UEM (p < 0,001). La edad de inicio de la migraña fue menor en los pacientes con UEM (21,2 ± 10,1 vs. 23,8 ± 12,5 años; p = 0,02) y el tiempo de evolución al llegar a nuestra consulta mayor en los pacientes con UEM (23,8 ± 14,1 vs. 18,3 ± 14,8 años; p < 0,001). No encontramos diferencia en la puntuación HIT-6 o el porcentaje de casos con HIT-6 mayor de 55 entre ambos grupos. Conclusiones: La MC con o sin UEM es una afección frecuente y discapacitante en una consulta de cefaleas. En nuestra población, los pacientes con UEM llegan a la consulta tras una evolución más larga, en la que ya recibieron tratamientos preventivos

Introduction: Chronic migraine (CM) is a complication of episodic migraine, favored by risk factors as medication overuse (MO). We intend to compare demographic and clinic characteristics of a series of CM patients, with and without MO. Methods: The study included patients with CM (2006 revised criteria) attended in a headache outpatient office located in a tertiary hospital between January 2008 and May 2012. We recorded demographic characteristics, age at migraine onset, time from onset, previous use of symptomatic or preventive therapy, and headache impact measured with six-item headache impact test (HIT-6). Results: A total of 434 patients (357 women, 77 men) were diagnosed with CM out of the 1868 (23.2%) that attended our clinic. Of these, 258 (72.2%) fulfilled criteria of MO, and 59.8% of those with MO, and 41.1% of cases without MO had previously received preventative treatment (P<.001). Age at onset of migraine was lower in MO patients (21.2 ± 10.1 vs 23.8 ± 12.5 years, P=.02) and time from onset to headache clinic consultation was higher in MO cases (23.8 ± 14.1 vs 18.3 ± 14.8 years, P<.001). We found no difference between both groups in average HIT-6 score and the percentage of patients with a HIT-6 score over 55. Conclusions: CM, with or without MO, is a burdensome group of patients in our headache clinic. Patients with MO are referred later and have more frequently received preventive treatments

Macular pattern dystrophy and homonymous hemianopia in MELAS syndrome.

We report an unusual association of a pattern dystrophy of the retinal pigment epithelium and homonymous hemianopia in a woman diagnosed with mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes syndrome.

[Clinical diagnostics and imaging in headache disorders]. / Diagnostik und Einsatz von bildgebenden Verfahren bei Kopfschmerzen.

Headache diagnosis is based on the criteria of the International Headache Society. Migraine and tension type headache are the most frequent primary headache disorders. Diagnosis can be made based on clinical criteria and further diagnostics are not mandatory. Imaging is primarily needed in suspected case of secondary headache disorders. Distinct imaging methods are used for research purposes especially for research about underlying pathophysiology of headache disorders.

Low-pressure/spinal fluid leak headache.

Low cerebrospinal fluid (CSF) pressure results in neurologic deficits, of which the most common manifestation is headache. Typically, the headache is postural - and specifically, orthostatic - in presentation. There are three hypotheses to explain the occurrence of headache associated with low CSF fluid. The first is traction on pain-sensitive intracranial and meningeal structures; the second is CSF hypovolemia; and the third is spinal loss of CSF resulting in increased compliance at the caudal end of the CSF space. Spontaneous intracranial hypotension (SIH), once believed to be rare, is now more commonly recognized. It is typically associated with orthostatic headache (although initially it may not be) and one or more other symptoms such as alterations in hearing, nausea, vomiting, neck stiffness, diplopia, and visual field cuts. Magnetic resonance imaging (MRI) of the brain with gadolinium is the first study of choice, which typically reveals diffuse pachymeningeal enhancement and, frequently, cerebellar tonsillar descent and posterior fossa crowding. Epidural blood patch (EBP) is the treatment of choice. Surgery and epidural fibrin glue injection are options for those who fail conservative therapy and/or EBP.

Peripheral endothelial function and arterial stiffness in patients with chronic migraine: a case-control study.

BACKGROUND: Migraine is a complex and disabilitating neurovascular disorder predominantly affecting women. There is strong evidence that nitric oxide is critically involved in migraine pathophysiology. The aim of the present study was to test the hypothesis that chronic migraine is associated with ultrasonographic endothelial dysfunction and increase in arterial stiffness. These parameters were assessed using a novel plethysmograph by peripheral arterial tonometry. METHODS: Twenty-one patients with chronic migraine and twenty-one healthy controls matched by sex and gender were recruited. Measurement of the ultrasonographic endothelial function and augmentation index were made according to manufacturer's protocol. RESULTS: The mean of patient's peripheral arterial tonometry ratios was 1.93 ± 0.39 and for healthy control 2.21 ± 0.44 (p=0.040). The median of patients' augmentation index was -6,0 (IQR: 6.5 to -15) in healthy controls and 9.0 (IQR: 4 to 12) in chronic migraine, (p=0.002). CONCLUSIONS: Patients with chronic migraine have ultrasonographic endothelial dysfunction and increase in the arterial stiffness. An improved understanding of the role in the endothelial system of migraine may provide a basis for preventive drugs in migraine and restore the endothelial function.

International Paediatric Stroke Study: stroke associated with cardiac disorders.

BACKGROUND AND HYPOTHESIS: The aetiologies of arterial ischaemic stroke in children are diverse and often multifactorial. A large proportion occurs in children with cardiac disorders. We hypothesized that the clinical and radiographic features of children with arterial ischaemic stroke attributed to cardiac disorders would differ from those with other causes. METHODS: Using the large population collected in the prospective International Paediatric Stroke Study, we analysed the characteristics, clinical presentations, imaging findings, and early outcomes of children with and without cardiac disorders. RESULTS: Aetiological data were available for 667 children with arterial ischaemic stroke (ages 29 days to 19 years). Cardiac disorders were indentified in 204/667 (30.6%), congenital defects in 121/204 (59.3%), acquired in 40/204 (19.6%), and isolated patent foramen ovale in 31/204 (15.2%). Compared to other children with stroke, those with cardiac disorders were younger (median age 3.1 vs. 6.5 years; P < 0.001) and less likely to present with headache (25.6% vs. 44.6%; P < 0.001), but were similar in terms of gender and presentation with focal deficits, seizures, or recent infection. Analysis of imaging data identified significant differences (P = 0.005) in the vascular distribution (anterior vs. posterior circulation or both) between groups. Bilateral strokes and haemorrhagic conversion were more prevalent in the cardiac disorders group. CONCLUSIONS: Cardiac disorders were identified in almost one-third of children with arterial ischaemic stroke. They had similar clinical presentations to those without cardiac disorders but differed in age and headache prevalence. Children with cardiac disorders more frequently had a 'cardioembolic stroke pattern' with a higher prevalence of bilateral strokes in both the anterior and posterior circulations, and a greater tendency to haemorrhagic transformation.