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Linear scleroderma of the head and face is a rare connective tissue disorder characterized by linear depressed scarring in the frontoparietal area of the face. Here, we report a patient with linear scleroderma of the head and face with neurological symptoms such as spontaneous epilepsy and numbness of the right limb as well as the presence of white matter lesions. The patient underwent computed tomography and 3.0-T magnetic resonance examinations including diffusion weighted imaging, diffusion tensor imaging, and perfusion imaging. The imaging findings suggested a disrupted fiber tract and decreased relative cerebral blood flow. Our observation may help to improve the diagnosis and treatment of linear scleroderma of the head and face.

As a result of population growth and the development of tube wells, humans’ exposure to arsenic has increased over the past few decades. The natural course of organ damage secondary to arsenic exposure is not yet well understood. In Toroku, Japan, an arsenic mine was intermittently operated from 1920 to 1962, and residents were exposed to high concentrations of arsenic. In this paper, we analyzed 190 consecutive residents for whom detailed records of neurological symptoms and findings were obtained from 1974 to 2005. All participants were interviewed regarding the presence of general, skin, hearing, respiratory, and neurological symptoms. Neurological symptoms were classified into extremity numbness or pain, constipation, dyshidrosis, sensory loss, and muscle atrophy. Superficial and vibratory sensation was also evaluated. More than 80% of participants experienced extremity numbness, and numbness was the most common neurological symptom. Numbness was associated with superficial sensory disturbance, and was correlated with the subsequent development of other neurological symptoms, including autonomic and motor symptoms. No previous studies have investigated the natural course of chronic arsenic intoxication, thus, these data serve as a guide for detecting early symptoms due to arsenic exposure.

Background Evidence show that Matrix metalloproteinases (MMPs) have been associated with neurological complications in the viral infections. Here in the current investigation, we intended to reveal if MMPs are potentially involved in the development of neurological symptoms in the patients with Coronavirus disease 2019 (COVID-19). Methods The levels of MMPs, inflammatory cytokines, chemokines, and adhesion molecules were evaluated in the serum and cerebrospinal fluid (CSF) samples from 10 COVID-19 patients with neurological syndrome (NS) and 10 COVID-19 patients lacking NS. Monocytes from the CSF samples were treated with TNF-α and the secreted levels of MMPs were determined. Results The frequency of monocytes were increased in the CSF samples of COVID-19 patients with NS compared to patients without NS. Levels of inflammatory cytokines IL-1β, IL-6, and TNF-α, chemokines CCL2, CCL3, CCL4, CCL7, CCL12, CXCL8, and CX3CL1, MMPs MMP-2, MMP-3, MMP-9, and MMP-12, and adhesion molecules ICAM-1, VCAM-1, and E-selectin were significantly increased in the CSF samples of COVID-19 patients with NS compared with patients without NS. Treatment of CSF-derived monocytes obtained from COVID-19 patients with NS caused increased production of MMP-2, MMP-3, MMP-9, and MMP-12. Conclusions Higher levels of inflammatory cytokines might promote the expression of adhesion molecules on blood-CSF barrier (BCSFB), resulting in facilitation of monocyte recruitment. Increased levels of CSF chemokines might also help to the trafficking of monocytes to CSF. Inflammatory cytokines might enhance production of MMPs from monocytes, leading to disruption of BCSFB (and therefore further infiltration of inflammatory cells to CSF) in COVID-19 patients with NS.

Between 2016 and 2017, several U.S. Diplomats in Havana, Cuba, experienced perplexing vestibular and neurological symptoms attributed to an unknown source. They presented with significant vestibular and headache symptoms similar to individuals who experience vestibular migraine (VM). As such, we hypothesize that VM may be a possible explanation for the Havana cohort's presenting symptoms. To evaluate this hypothesis, we compared symptoms reported by the affected individuals in Havana, Cuba, to symptoms reported by corresponding patients from a tertiary academic neurotology clinic with a chief complaint of vertigo or disequilibrium, who met the International Classification of Headache Disorders criteria for VM. The prevalence of the Havana subjects experiencing various symptomatic domains was compared with the VM cohort, leading to 26 unique domains and statistical comparisons between the cohorts. Of the 26 domains compared between the two cohorts, 18 were not significantly different. This included the two most important components of meeting criteria for VM, namely the co-existence of headache and vestibular symptoms. On regression analysis, the only feature which kept its significant difference between the two cohorts was acute intense head pressure (P=0.007). The domains with similar occurrence ratios included dizziness, headache, light sensitivity, hearing reduction, and tinnitus. In other words, multiple headache and vestibular symptoms, consistent with VM criteria, were similar between the two cohorts. The considerable similarities across various domains between VM patients and Havana subjects could be due to migraine as a possible common etiology for both groups. We propose VM as a potential etiology for the symptomatology in the U.S. diplomats in Cuba.

Objective This study aimed to investigate the clinical characteristics of patients with aortic dissection presenting with neurological symptoms as the initial symptoms. Methods Clinical characteristics, radiological abnormalities, and prognosis were analyzed in two female patients with aortic dissection. The main clinical symptoms were sudden coma and paraplegia. A detailed clinical examination was performed and computed tomography scans were evaluated. Results The initial symptoms of the patients involved the nervous system. Chest and abdominal computed tomography scans were performed to confirm the diagnosis of aortic dissection. Both patients died. Conclusion Patients who present with nervous system symptoms as the first symptoms of aortic dissection, which lead to misdiagnosis and missing the diagnosis, are rare. Improvement in the understanding of this disease is required to ensure that patients receive a timely diagnosis in the early stages.

Mastocytosis is a rare disease of clonal hematological disorders characterized by a pathological accumulation of Mast Cells (MCs) in different tissues, with variable symptomatology and prognosis. Signs and symptoms of Systemic Mastocytosis (SM) are due to pathological infiltration of MCs and to the release of chemical mediators, mainly histamine. Patients with SM may also present with neurological symptoms or complications. The pathophysiology of these neurological disorders remains uncertain to this day, but it can be associated with the infiltration of tissue mastocytes, release of mastocytes' mediators or both. Moreover, there is a lot to understand about the role of neurological symptoms in SM and knowing, for example, what is the real frequency of neurological disorders in SM and if is present a relation between other SM subtypes, because it has been noted that the alteration of the histamine expression may be an initiating factor for susceptibility, gravity and progression of the epigenetic disease. In this review we explain the possible pathophysiological mechanism about neurological symptomatology found in some patients affected by SM, describing the role of histamine and its receptors in the nervous system and, in light of the results, what the future prospects may be for a more specific course of treatment.

Objective This study aimed to analyze the risk factors of intracranial hemorrhage (ICH) after deep brain stimulation (DBS) for idiopathic Parkinson’s disease (PD). Methods Patients who received DBS from March 2014 to December 2016 were retrospectively analyzed. The hemorrhage index was derived by combining the hemorrhagic volume and clinical manifestations of ICH. All patients with IHC were followed up for 2 years. Results Computed tomography showed 13 events of ICH in 11 patients (nine cases in the subthalamic nucleus), including eight cases with symptomatic hemorrhage (seven cases in the subthalamic nucleus). Hemorrhage was characterized by intracranial hematoma in the electrode puncture tract. Male sex and hypertension were significant risk factors for ICH. Hemorrhage in the preferred puncture side was significantly higher than that in the non-preferred puncture side. The mean hemorrhage index was 2.23 ± 0.83 in 11 patients, and no permanent neurological impairment was found during the 2-year follow-up. The effect of DBS on motor symptoms was similar in patients with and without ICH. Conclusion Male sex and hypertension are risk factors of ICH after DBS in PD. The risk of hemorrhage on the first puncture site is significantly higher than that on the second puncture site.

Purposes Brain metastases (BM) are a frequent complication in small cell lung cancer (SCLC), resulting in a reduced survival prognosis. Precise prognostic assessment is an important foundation for treatment decisions and clinical trial planning. Methods Patients with newly diagnosed SCLC BM were identified from the Vienna Brain Metastasis Registry and evaluated concerning prognostic factors. Results 489 patients (male 62.2%, female 37.8%; median age 61 years) were included. Neurological symptoms were present in 297/489 (60.7%) patients. A- to oligosymptomatic patients (5 vs. 9 months, p = 0.030) as well as patients with synchronous diagnosis of BM and primary tumor (5 vs. 9 months, p = 0.008) presented with improved overall survival (OS) prognosis. RPA (HR 1.66; 95% CI 1.44–1.91; p

WOS: 000428044700008, PubMed ID: 27858390, AIM: Eagle syndrome is a rare entity that causes recurrent throat pain, neck pain, dysphagia, or facial pain due to an elongated styloid process or calcified stylohyoid ligament. Clinical findings related to lower cranial nerve compression have also been reported. In some cases, it is reported that carotid artery compression or dissection can be seen due to elongated styloid process and this is called carotid artery syndrome. Carotid artery compression causes flow reduction and carotidynia or neurological symptoms can be seen. Dural sinuses and the jugular vein can be compressed. Eagle syndrome with neurological symptoms has been rarely reported. MATERIAL and METHODS: The data of 5 patients (aged between 22 and 68 years), who presented to the hospital with various neurological symptoms, were retrospectively reviewed. Each patient underwent computed tomography (CT) that revealed a long styloid process. RESULTS: An elongated styloid process caused neurological symptoms. Two patients had venous compression by the styloid process and the other patients had transient ischemic attacks due to internal carotid artery compression by the styloid process. Only one patient underwent surgical removal. All patients' outcomes were good after treatment and no symptoms remained. CONCLUSION: Eagle syndrome may be presented with neurological symptoms. It should be kept in mind in the differential diagnosis of patients who have neurological symptoms without any objective etiological factors. To the best of our knowledge, Eagle syndrome with arterial and venous compression due to elongated styloid process has not been previously presented in the literature.

Background Isolated adrenocorticotropic hormone (ACTH) deficiency is a pituitary disorder characterized by reduction only in the secretion of ACTH. Although the underlying mechanism remains to be elucidated, numbers of cases with this entity have been increasing. We experienced a case presenting with gait disturbance necessitating differential diagnosis from idiopathic normal pressure hydrocephalus (iNPH). Case presentation A 69-year-old female with a complaint of difficulty walking and suspected to have iNPH at a prior hospital was referred to our department. For the prior three years, she had suffered from a progressive gait disturbance. Magnetic resonance imaging (MRI) revealed global ventricular dilatation. The typical features of the gait in iNPH cases were all identifiable. Neuropsychological dementia scale tests showed deterioration. However, the major feature of a disproportionately enlarged subarachnoid-space on MRI was not obvious. The patient developed progressively worsening fatigue during hospitalization. Her symptoms resembled those of hypothalamic-pituitary tumor patients. Serum ACTH and cortisol levels were low. While corticotrophin releasing hormone stress tests showed no response, other stress tests using thyrotropin releasing hormone, luteinizing hormone releasing hormone, and growth hormone releasing hormone yielded normal responses, indicating a diagnosis of isolated ACTH deficiency. We initiated corticosteroid therapy, and her gait disturbance improved promptly. Conclusion Isolated ACTH deficiency may have major significance to the differential diagnosis of iNPH. Early consideration of this entity is anticipated to facilitate making an early diagnosis.

Patients with essential thrombocythemia (ET) often suffer from neurological symptoms (NS) not ever resulting from previous thrombotic cerebral events (TCE). We reported NS occurred in 282 patients, in order to identify the factors influencing ET-related NS in the absence of TCE, and the response to therapy. Overall, 116 of 282 patients (41%) presented NS; 101 of them (87%) reported subjective transient and fluctuating NS, without concurrent TCE, which we defined as ET-related NS, by frequency: cephalalgia, chronic paresthesias, dizziness or hypotension, visual disturbances, and tinnitus. In univariate analysis, ET-related NS resulted more frequently in young people (P=0.017) and in females (P=0.025). We found a higher prevalence of JAK2V617F mutation in ET-related NS patients (P=0.021). In multivariate analysis, gender (P=0.024) and JAK2V617F mutation (P=0.041) remained significantly associated with the development of ET-related NS, with a risk of about four times higher for JAK2V617F-mutated patients (OR=3.75). Ninety-seven of 101 patients with ET-related NS received an antiplatelet (AP) agent at the time of NS, whereas only selected high-risk ET-related NS patients were treated with a cytoreductive drug, according to the published guidelines and similarly to patients without NS. We observed that only 32 of 97 (33%) patients with ET-related NS achieved a complete response after AP treatment. Among the 65 non-responder patients, 36 (55.4%) improved NS after the introduction of cytoreductive therapy; therefore, the addition of cytoreductive treatment should be considered in this setting.

The neurobiological basis of psychogenic movement disorders (PMDs) has been elusive, and they remain difficult to treat. In the last few years, functional neuroimaging studies have provided insight into their pathophysiology and neural correlates. Here, we review the various methodological approaches that have been used in both clinical and research practice to address neural correlates of functional disorders. We then review the dominant hypotheses generated from the literature on psychogenic paralysis. Overall, these studies emphasize abnormalities in the prefrontal and anterior cingulate cortices. Recently, functional neuroimaging has been used to specifically examine PMDs. These studies have addressed a major point of controversy: whether higher frontal brain areas are directly responsible for inhibiting motor areas or whether they reflect modulation by attentional and/or emotional processes. In addition to elucidating the mechanism and cause, recent work has also explored the lack of agency that characterizes PMDs. We describe the results and implications of the results of these imaging studies and discuss possible interpretations.