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OBJECTIVE Non-traumatic headache is a frequent reason for visits to the emergency department (ED). We evaluated the performance of the Manchester Triage System (MTS) in prioritising patients presenting to the ED with non-traumatic headache. METHODS In this single-centre observational retrospective study, we compared the association of MTS priority classification codes with a final diagnosis of a severe neurological condition requiring timely management (ischaemic or haemorrhagic stroke, subarachnoid haemorrhage, cerebral sinus venous thrombosis, central nervous system infection or brain tumour). The study was conducted and reported according to the STROBE statement. The overall prioritisation accuracy of MTS was estimated by the area under the receiver operating characteristic (ROC) curve. The correctness of triage prediction was estimated based on the "very urgent" MTS grouping. An undertriage was defined as a patient with an urgent and severe neurological who received a low priority/urgency MTS code (green/yellow). RESULTS Over 30 months, 3002 triage evaluations of non-traumatic headache occurred (1.7% of ED visits). Of these, 2.3% (68/3002) were eventually diagnosed with an urgent and severe neurological condition. The MTS had an acceptable prioritisation accuracy, with an area under the ROC curve of 0.734 (95% CI 0.668-0.799). The sensitivity of the MTS for urgent codes (yellow, orange and red) was 79.4% (95% CI 74.5-84.3), with a specificity of 54.1% (95% CI 52.9-55.3). The triage prediction was incorrect in only 6.3% (190/3002) of patients with headache. CONCLUSION The MTS is a safe and accurate tool for prioritising patients with non-traumatic headache in the ED. However, MTS may need further specific tools for evaluating the more complicated symptoms and for correctly identifying patients with urgent and severe underlying pathologies. RELEVANCE TO CLINICAL PRACTICE The triage nurse using MTS may need additional tools to improve the assessment of patients with headache, although MTS provides a good safety profile.

Background The effectiveness of adjunctive perampanel has not been systematically assessed in seizure types other than its approved indications of focal seizures and primary generalised tonic–clonic seizures (PGTCS) in idiopathic generalised epilepsies (IGEs). Objective We aimed to identify and review available evidence on outcomes with perampanel in generalised seizures and epilepsies to examine its potential as a broad-spectrum anti-seizure medication. Methods Bibliographic databases of publications, clinical trials, and conference abstracts were searched up to August 2020 to identify studies reporting seizure or safety outcomes in patients of any age, with any type of epilepsy-associated generalised seizures treated with perampanel. Data extracted from selected records were tabulated by seizure type and syndrome, and analysed qualitatively (PROSPERO protocol CRD42020201564). Results Ninety-one reports met inclusion criteria and were selected: 15 reports of 1 randomised controlled trial (RCT), 8 reports of 4 non-randomised interventional studies, 37 reports of observational studies, 21 case reports and 10 systematic reviews and meta-analyses. Extracted data included 359 patients with PGTCS of any aetiology, 251 with myoclonic seizures, 112 with absence seizures, 50 with tonic seizures and 32 children with epileptic spasms. The most commonly reported epilepsy type was IGE (N = 378) and the most common syndromes were juvenile myoclonic epilepsy (N = 92), progressive myoclonic epilepsies (N = 59) and absence epilepsies (N = 43). The RCT provided Class I evidence of the efficacy and tolerability of adjunctive perampanel for PGTCS in patients aged ≥ 12 years with IGE. Data from other studies provides weaker (observational) evidence of its effectiveness in multiple generalised seizure types, including myoclonic, absence and tonic seizures. There were no patterns suggesting seizure worsening or aggravation in any seizure or epilepsy type. Conclusions The identified studies suggest the potential of perampanel as a broad-spectrum antiseizure medication. Much of the available data, however, come from non-randomised, non-controlled studies and are open to high risk of bias. Further studies are warranted to provide more robust evidence. Supplementary Information The online version contains supplementary material available at 10.1007/s40263-021-00831-y.

Resumen Introduccion y objetivos Tras el flujo migratorio procedente del Africa Subsahariana (ASS) en Europa, el numero de diagnosticos de esquistosomiasis urogenital (EUG) es cada vez mayor. Este fenomeno representa un desafio para los urologos que trabajan en zonas no endemicas. El objetivo de este estudio es describir el tratamiento urologico y los procedimientos quirurgicos de los pacientes con EUG en un centro de referencia terciario Pacientes Todos los sujetos del ASS diagnosticados con EUG entre enero del 2011 y noviembre del 2018 fueron inscritos retrospectivamente. Se recogio y analizo la informacion detallada de los pacientes con EUG tratados mediante procedimientos urologicos. Resultados Treinta pacientes fueron diagnosticados con EUG, 12 (42,8%) fueron tratados mediante cirugia. El procedimiento quirurgico mas comun fue la reseccion transuretral de vejiga (RTUV) para las lesiones sospechosas persistentes despues del tratamiento con praziquantel administrado en 7 casos (58%). Otros procedimientos quirurgicos (realizados una vez) fueron RTUV combinada con ureteroscopia laser por sospecha de neoplasia de vejiga con calculos renales, litotricia endoscopica y nefrolitotomia percutanea para calculos vesicales y renales, nefrectomia laparoscopica para enfermedad renal terminal, colocacion de nefrostomia bilateral para hidroureteronefrosis, cirugia testicular exploratoria por sospecha de torsion testicular. Cuatro pacientes (33%) se perdieron en el seguimiento. Conclusion Se ha observado un numero cada vez mayor de migrantes del ASS diagnosticados con EUG. Algunos pacientes requirieron intervencion quirurgica por sospecha de lesiones neoplasicas o danos en organos en fase terminal. En varios pacientes fue particularmente dificil realizar un seguimiento regular. Se necesitan mas estudios multicentricos para lograr un manejo estandar en terminos de diagnostico, tratamiento y seguimiento de los sujetos con EUG.

Introduction Cerebrovascular disease is the most common cause of seizures in adults and the elderly. So far, no drug is recommended as primary prevention of acute symptomatic poststroke seizures (ASPSS) or poststroke epilepsy (PSE). This systematic review aimed to evaluate the association between the use of statins after stroke and the risk of developing ASPSS or PSE following cerebral infarct or hemorrhage (primary prevention). Methods We included studies evaluating the poststroke use of statins as primary prevention of ASPSS or PSE, irrespective of stroke type. We excluded uncontrolled studies and studies with prestroke statin use. The main outcome included the occurrence of ASPSS or PSE and the effect of statins by type and dose. The odds ratios (ORs) or hazard ratios (HR) with 95% confidence intervals (CIs) were used as the measures of association between treatment and outcome. Results Four studies were included. One study showed a reduced risk of ASPSS after ischemic stroke (OR: 0.25; 95% CI: 0.10–0.59; p = 0.0016). Three studies consistently reported a reduced risk of PSE after ischemic stroke, and one study a reduced risk of PSE after hemorrhagic stroke (HR: 0.62; 95% CI: 0.42–0.90; p = 0.01). Conclusions Data from the literature suggest an association between statin use and a reduced risk of ASPSS after ischemic stroke and a reduced risk of PSE after ischemic and hemorrhagic stroke. Although the certainty of the evidence is low, these findings appear promising and worthy of further investigation.

Background The main purpose of this study is to assess efficacy and tolerability of perampanel (PER), a noncompetitive α-amino-3-hydroxy-5-methyl-4-isoxazole propionic acid receptor antagonist, as an add-on treatment in adult patients with refractory focal-onset seizures. Patients and Methods A prospective, open label, observational study was conducted in patients with refractory focal-onset seizures treated with PER at our Epilepsy Unit, from May 2015 to February 2016. Patients were followed up for 1 year. Frequency of seizure and tolerability was assessed every 3 months. Patients were under a polytherapy, and the mean number of concomitant antiepileptic drugs (AEDs) at PER initiation was 2.9. Results We consecutively enrolled 52 patients (M/F = 18/34). Three were lost on follow-up. Mean age was 38.7 years, with a mean duration of disease of 28.1 years. After 1 year of treatment, 57.14% reported a 50% or greater reduction in seizure frequency; five (10.21%) were seizure free. Six (12.25%) patients reported a reduction lower than 50%. Mean dosage of PER was 7.57 mg. Thirty-one patients were taking enzyme-inducing AEDs (carbamazepine, oxcarbazepine, phenytoin). In this subgroup, the responder rate was 45.2%. Twenty-one patients reported side-effects, most frequently somnolence (11), vertigo/ataxia (6), and aggressiveness (5). Eleven (22.4%) patients reduced or discontinued at least one concomitant AED, while the electroencephalography improved in four (8.16%). Sixteen (32.65%) patients withdrew PER, after a mean duration of 163 days, the mean dosage being 6.4 mg (range 4–12). Conclusions Adjunctive PER can achieve clinically meaningful improvement, or even seizure freedom, in almost two-thirds of patients suffering from refractory focal-onset epilepsies. It seems similarly safe and well-tolerated. Enzyme-inducing AEDs may limit the efficacy of PER.

Photodermatoses are characterized by the development of skin eruptions following exposure to ultraviolet radiation or visible light. We report here the clinical findings and results of laboratory investigations and phototesting of 6 patients who experience debilitating and excruciating pain after sun exposure ("sun pain") in the absence of any skin eruption. Phototesting with sub-erythemal doses of ultraviolet A radiation triggered localized pain in 4 patients. At follow-up, 3 female patients were found to have developed fibromyalgia, 2 male patients experienced a major depressive disorder, and another male patient had a conversion disorder. One patient also developed allodynia to tactile stimuli and one developed allodynia to thermal and tactile stimuli. Psychiatric conditions should be taken into consideration in patients presenting with excruciating and debilitating pain on exposure to ultraviolet radiation, but with absence of skin eruption. Further research is needed to evaluate whether it represents a type of allodynia triggered by exposure to ultraviolet radiation.

Migrants may constitute a risk group and should have specific targets for health policy. To identify their health needs, it is important to investigate their epidemiological profile and their access to health services. The aim of this study was to identify the pattern of hospital and neurological services use among immigrants living in Brescia (Italy). The analysis took into account the records of 45,645 immigrants admitted to the ER (Emergency Room) as well as the discharge data of 6419 patients hospitalized in the Department of Neurology of the Azienda Ospedaliera Spedali Civili di Brescia, over a 3.5 years period. To take confounding factors into account, immigrant patients admitted to the department of Neurology were compared to a selection of Italian patients matched by age and sex. The main objectives were to explore causes of admission of the immigrant population-along with socio-demographic characteristics-to the Emergency Room and to the Neurology Units. Immigrants showed a similar pattern of hospital use to the Italian patients, although with a higher frequency of infective diseases and traumatic injuries. They also showed a higher mean Diagnosis-Related Group (DRG) weight than the Italians. Average length of hospitalization was longer in immigrant population. However, the use of neurological services by migrants is less than their demographic share. Poorer economic and social conditions, as well as a worse labor market experienced by immigrants may expose them to risk factors for injuries and infective diseases. Reducing the language and bureaucratic barriers, as well as enhancing cross-cultural skills of physicians, might be crucial in decreasing the length and the cost of hospitalization.

Introduction: Restless Legs Syndrome/Willis-Ekbom Disease (RLS/WED) is a sleep disorder characterized by an urge to move the legs, frequently associated or triggered by unpleasant sensations in the lower limbs that affects approximately 2.5% of adults. Therapy and management of RLS/WED require long-term interventions, since the typical manifestation of this disorder is chronic. Areas covered: In this review, we provide an update regarding the treatment of RLS/WED with particular attention to future challenges for its management. We reviewed a large variety of treatments studied in clinical trials and supported by the most updated guidelines. Alongside with first-line interventions other pharmacological options including opioids, benzodiazepines, iron therapy, and newly studied drugs are discussed. Furthermore, due to the occurrence of augmentation and worsening of symptoms we also reviewed the development of non-pharmacologic alternatives. Expert commentary: The management of RLS/WED is a challenge because of different long-term issues. Several complications, such as loss of the therapeutic effect of dopaminergic or non-dopaminergic agents and augmentation, are still unsolved concerns. However, the development of new drugs acting on adenosinergic and glutamatergic neurotransmission seems promising. Randomized controlled trials are needed in order to recognize effectiveness of new drugs or non-pharmacological treatment strategies.

Friedreich ataxia (FRDA) is an autosomal recessive neurodegenerative disorder characterized by progressive gait and limb ataxia, cerebellar, pyramidal and dorsal column involvement, visual defects, scoliosis, pes cavus and cardiomyopathy. It is caused by a homozygous guanine-adenine-adenine (GAA) trinucleotide repeat expansion in intron 1 of the frataxin gene (FXN) on chromosome 9q13-q21.1. Onset is usually in the first or second decade of life; however, late-onset cases of Freidreich ataxia (LOFA), after the age of 25 years, and very late-onset cases of Freidreich ataxia (VLOFA), after the age of 40 years, have been reported. VLOFA is quite rare and usually presents a milder progression of the disease. We report the case of a 64-year-old woman affected with VLOFA whose first symptoms (balance and gait disturbances) occurred at the age of 44 years. At the age of 62 years, she started complaining of a slowly progressive dysphonia showing the clinical aspects of laryngeal dystonia. Molecular analysis showed a 210- and 230-trinucleotide GAA repeat expansion in the two alleles of the FXN gene. Laryngeal dystonia has been reported only in very few cases of ataxia syndrome and never before in FRDA patients. It may represent a rare clinical manifestation of VLOFA thus confirming the high variability of the clinical spectrum of FRDA.

Aims: To prospectively assess the incidence and etiology (ie, primary vs symptomatic) of headache in women during the first month postdelivery, with particular emphasis on the type of presentation as a clue for identifying potentially harmful etiologies. A secondary aim was to evaluate the relative frequency of migraine- vs tension-type headache in cases of primary headache. Methods: A total of 900 consecutive women were enrolled in the study and examined within 3 days of delivery, both clinically and with transcranial color-coded sonography (TCCS). During the course of follow-up, all subjects presenting with headache suspected of being secondary to intracranial pathology underwent a complete clinical and instrumental assessment with TCCS and magnetic resonance imaging (MRI) and angiography. A telephone interview was administered to all subjects 1 month after delivery. Two-tailed t test, Mann-Whitney test, Pearson chi-square test, and multiple logistic regression were used to analyze the data. Results: At the end of the follow-up period, 241 women (26.8% of the sample) reported at least one headache attack. In 88 of these 241 cases (9.8%), the headache attack occurred soon after delivery and was already recorded at the first visit. Thunderclap headache occurred in 34 (3.8%) of the subjects. In all but one of these subjects, the course was spontaneously benign. None of the recorded variables allowed discrimination of the subjects with thunderclap headache from those without headache. Three subjects had thunderclap headache following dural anesthesia, and one subject was found to have reversible cerebral vasoconstriction syndrome. Headache with gradual onset was recorded in 207 subjects (23%). Three of these subjects fulfilled the criteria for pre-eclampsia, and 13 had postural headache after dural anesthesia. Migraine history and urinary protein were independent predictors of gradual onset headache, and migraine history and parity were significant independent predictors of pulsating pain with gradual onset headache. Conclusion: Headache appeared early in the first days postdelivery, and its incidence increased in the first month thereafter. Predictors were different according to whether the headache had a gradual onset or a thunderclap presentation. Primary headache accounted for the overwhelming majority of the recorded cases.

Background and Aim of the Study Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by severe “thunderclap” headache, with or without associated neurological symptoms and neuroimaging findings of reversible vasoconstriction of cerebral arteries. Puerperium is a recognized precipitant, but the incidence of puerperal RCVS is unknown. We conducted a prospective study to assess incidence, risk factors and clinical features of RCVS. Material and Method Nine-hundred consecutive puerperae were prospectively enrolled within three days of delivery. Past medical history, basal demographic, anthropometric and biological variables were recorded. Transcranial Colour Coded Sonography (TCCS) was performed to assess early signs of vasospasm in brain vessels. A structured telephone interview was planned in all subjects one month post delivery. Results Thunderclap headache was recorded in 8 subjects (0.9%) on the first visit. At the one month follow-up interview 27 more patients reported having had at least one episode of thunderclap headache. In these 33 (3.8%) patients the course was spontaneously benign. One patient presented to the Emergency ward with throbbing thunderclap headache three weeks after delivery. Diagnostic work-up ended up in the diagnosis of RCVS, the outcome was favorable Conclusion In normally coursing pregnancies and after uncomplicated delivery the risk of puerperal RCVS is negligible (0.1%). On the other way thunderclap headache may occur in a measurable proportion of (3.4%), although in the vast majority of cases ( 33/34 = 97%) it is of benign course. Transcranial Doppler sonography may be helpful to pick up those cases in whom further neuroradiological investigation is warranted.

Hashimoto's encephalopathy (HE) is a rare neuropsychiatric syndrome associated with antithyroid antibodies. It may have an acute onset (episodes of cerebral ischemia, seizure, and psychosis) or it may present as an indolent form (depression, cognitive decline, myoclonus, tremors, and fluctuations in level of consciousness). We here describe a case of encephalopathy presenting as non-convulsive status epilepticus associated with Hashimoto's thyroiditis (HT), unresponsive to corticosteroid therapy, with improvement after plasma exchange treatment. A previously healthy 19-year-old woman, presented generalized tonic-clonic seizures. About a month later, she manifested a speech disorder characterized by difficulties in the production and comprehension of language. Within a few days she also developed confusion and difficulties in recognizing familiar places, with gradual worsening over time. EEG revealed a non-convulsive status epilepticus (NCSE). CSF examination showed slightly elevated cell count and four oligoclonal bands. MRI was unremarkable, and (18)F-FDG brain PET showed widespread hypometabolism, mostly in posterior regions bilaterally. Laboratory and ultrasound findings showed signs of HT. Treatment with steroid was introduced without any improvement. After five sessions of plasma exchange there was a decrease of antithyroid antibodies, as well as EEG and clinical improvement. Three months after discharge (18)F-FDG brain PET showed a complete normalization of the picture, and the patient was asymptomatic. This report emphasizes the successful treatment of HE with plasma exchange in a patient who presented with NCSE. Based on the actual evidence, the term "Encephalopathy associated with Hashimoto's thyroiditis" may be the most proper. Furthermore, to our knowledge, this is the first case of an adult patient studied twice with an (18)F-FDG brain PET: prior to treatment with plasma exchange, and at 3 months follow-up when the patient was clinically completely asymptomatic. Studies in more patients are needed to clarify the relevance of (18)F-FDG brain PET as a possible diagnostic tool for HE.

Background: Behavioral and cognitive impairment are common in amyotrophic lateral sclerosis (ALS) and represent a continuum with frontotemporal dementia (FTD). Olfactory dysfunction has been described in a subset of ALS patients and might be associated with frontotemporal and insular cortex dysfunction. Objective: To evaluate olfaction dysfunction in ALS patients and its relationship with either cognition or behavioral impairment. Methods: 28 consecutive ALS patients underwent an extensive cognitive and behavioral battery and were classified as patients with normal cognition (ALS-N, n = 11) or with part of the ALS-FTD spectrum (n = 17), including either cognitive or behavioral impairment or dementia. Odor verbal and visual identification and discrimination were investigated in patients and age-matched controls using a test adapted from the Sniffin' Sticks. Results: Olfactory function was significantly different between ALS-FTD spectrum patients and controls (p < 0.001) and inversely correlated with behavioral and cognitive performance. The 10-point cutoff distinguished ALS-N from ALS-FTD spectrum patients with a sensitivity and specificity of 71 and 100%, respectively. Conclusions: Hyposmia is common in a subset of ALS patients and strongly associated with behavioral and cognitive impairment. Olfactory testing may represent an early screening tool in order to identify ALS subjects with cognitive/behavioral dysfunction. Further studies in larger series are mandatory in order to better investigate clinical and pathological aspects in this group of patients.

Restless Legs Syndrome/Willis-Ekbom Disease (RLS/WED) is a common condition characterized by an irresistible urge to move the legs, concomitant with an unpleasant sensation in the lower limbs, which is typically relieved by movement. Symptoms occur predominantly at rest and prevail in the afternoon or evening. Treatment of patients with RLS/WED is indicated for those patients who suffer from clinically relevant symptoms. The management of mild forms of RLS/WED is mainly based on dopamine agonists (DA) therapy (including pramipexole and ropinirole) and α-2-δ calcium-channel ligand. Nevertheless, with passing of time, symptoms tend to become more severe and the patient can eventually develop pharmacoresistance. Furthermore, long-term treatment with dopaminergic agents may be complicated by the development of augmentation, which is defined by an increase in the severity and frequency of RLS/WED symptoms despite adequate treatment. Here, we discuss which are the best therapeutic options when RLS/WED becomes intractable, with a focus on advantages and side effects of the available medications. Prevention strategies include managing lifestyle changes and a good sleep hygiene. Different drug options are available. Switching to longer-acting dopaminergic agents may be a possibility if the patient is well-tolerating DA treatment. An association with α-2-δ calcium-channel ligand is another first-line approach. In refractory RLS/WED, opioids such as oxycodone–naloxone have demonstrated good efficacy. Other pharmacological approaches include IV iron, benzodiazepines such as clonazepam, and antiepileptic drugs, with different level of evidence of efficacy. Therefore, the final decision regarding the agent to use in treating severe RLS/WED symptoms should be tailored to the patient, taking into account the symptomatology, comorbidities, the availability of treatment and the history of the disease.

The pathophysiology of restless legs syndrome (RLS) isn’t completely understood. Small Fiber Neuropathy (SFN) patients may have RLS-like sensory symptoms. The aim of this study was to assess the prevalence and clinical features of RLS in patients with SFN, compared with Large Fibres Neuropathy (LFN) patients and healthy controls. All patients underwent nerve-conduction studies and electromyography, and a skin biopsy to confirm SFN. RLS was diagnosed according to international criteria. We compared: frequency of RLS, age of onset, severity as measured by IRLS, insomnia and diabetes comorbidity. We included 39 SFN patients, 37 LFN patients and 40 matched controls. Peripheral nerve-conduction velocities were normal in SFN patients and controls. SFN patients had a 25.60% prevalence of RLS, compared to 10.80% in LFN and 7.50% in controls. Age of onset was 42.91 years, versus 62.67 in LFN and 58.50 in controls. Mean IRLS was 15.6 versus 19.5 in LFN and 18.6 in controls. The occurrence of insomnia was similar between SFN and LFN (43.58% and 43.24%), but higher than in controls (20%). Biopsy-proven SFN patients had a higher risk for an early-onset RLS. They show a less severe RLS, but it can frequently cause insomnia.

The three-dimensional (3D) temporomandibular joint (TMJ) model derives from a study of the cranium by 3D virtual reality and mandibular function animation. The starting point of the project is high-fidelity digital acquisition of a human dry skull. The cooperation between the maxillofacial surgeon and the cartoonist enables the reconstruction of the fibroconnective components of the TMJ that are the keystone for comprehension of the anatomic and functional features of the mandible. The skeletal model is customized with the apposition of the temporomandibular ligament, the articular disk, the retrodiskal tissue, and the medial and the lateral ligament of the disk. The simulation of TMJ movement is the result of the integration of up-to-date data on the biomechanical restrictions. The 3D TMJ model is an easy-to-use application that may be run on a personal computer for the study of the TMJ and its biomechanics.

Headache is a common presenting complaint in the Emergency Department. The aim of this study was to delineate the demographic profile of patients presenting a chief complaint of headache and to assess the application of diagnostic algorithms for the management of these patients. We examined patients admitted to the Spedali Civili Hospital ED between January 2005 and December 2009 who complained of headache not related to trauma and all patients hospitalized for headache in Neurological Clinic, from ED, between January 2008 and December 2009. 7495 patients were examined at ED for headaches. 72 % of patients were discharged, 22 % were admitted. From 2005 to 2009, there was a definite decrease in the rate of hospitalization due to headache (15 vs 9.9 % in Department of Neurology and 26 vs 18.9 % in all Departments). Considering the decrease year by year, this reduction was significant from 2007 to 2008, when the algorithms were adopted. The most common diagnosis in the ED was “Non-specific headache” (41 %), followed by “Primary headaches and complications of primary headaches” (20.8 %), “Secondary headaches not associated with risk of serious disease” (20.4 %) and “Secondary headache associated with risk of serious disease” (5 %). Over 2-year period (2008–2009) we found an increase in the diagnosis of “Primary headaches and complications of primary headaches” and “Secondary headaches associated with risk of serious disease” compared with a decrease of “nonspecific headache” and “secondary headaches not associated with risk of serious disease”. The use of the diagnostic algorithms and collaborative network between the ED and the Headache Center can improve the management of patients with headache in ED.

sporadic motor neuron disorder that differs from the spinal ALS because it remains largely restricted to the upper limbs over time. Only a few cases are described in the literature with a follow-up ranging between two and 11 years (3,4). We report on a 69-years-old patient having a 35-year history of progressive bilateral weakness of the proximal segments of upper limbs and shoulders, which started insidiously on the left side and involved the right side 15 years later. No lower limb involve-ment was reported. No injury of rotator cuff muscles and tendons was noted. Neuromuscular family history was unremarkable. Clinical evaluation showed severe weakness and atrophy of muscles of the upper limb girdle (Figure 1). Distal segments of upper limbs as well as lower limbs were normal. Fasciculations were not present. Deep tendon refl exes were absent at the upper limbs and normal at the lower ones. Dear Sir, Man-in-the-barrel syndrome (MIBS) is characterized by brachial diplegia with motor function preserved in the legs and facial musculature, giving to a patient the clinical appearance of seeming constrained in a barrel (1). MIBS was early observed in patients with bilat-eral ischaemia in the border zones of the anterior and medial cerebral arteries, pontine lesion, multiple sclerosis lesions, cerebral metastases and head trauma. A less recognized variant of MIBS is the neurogenic form that can result from different causes, i.e. amyotrophic lateral sclerosis and myelopathies with purely motor presentation including cervical spinal cord infarction, cervical spondylosis and post radiation (1,2). The term ‘ brachial amyotrophic diplegia ’ has been used to identify a MIBS related to a primary

Background.—Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) and trigeminal neuralgia (TN) are unilateral painful conditions that can share the same triggering factors, autonomic features and the main location, as well as the cyclically recurrent crises. Both these syndromes are associated with a high percentage of findings of vascular malformation touching the trigeminal nerve, suggesting a pathophysiological relationship. Case.—In this paper, we report a new case with the main purpose to shine a light on the pathophysiology of these conditions. Conclusion.—Many authors described a SUNCT case deriving from TN or vice versa, suggesting that these conditions are strongly related. Every case of transformed TN or SUNCT should therefore be reported to gather and compare further information.

Paraproteinemic neuropathy (PPN) is often under-diagnosed because of its clinical and electrophysiological variability. Progression of neuropathy is considered an alarm bell for possible malignant conversion of underlying monoclonal gammopathy (MG).To report clinical presentation, course, and evolution in a group of patients with PPN in order to identify findings useful for achieving the diagnosis, suspecting progression, and recognizing the underlying hematological conditions.Thirty-nine patients with PPN underwent clinical examination, electrodiagnostic studies, cerebrospinal fluid analysis, and laboratory tests. These parameters were compared between the different peak groups.IgM MG was found in 51.4%, IgG MG in 33.3%, and IgA MG in 10.3% of our cohort. PPN appeared as mainly sensory, demyelinating, mildly progressive neuropathy, regardless of the type of peak or light chain. However, axonal findings were present in many IgG patients and in part of the IgM patients and a small number of the IgG patients may have presented with motor symptoms at the onset. The IgM patients had a significant tendency toward clinical worsening and IgG subjects had a more elevated rate of malignancy. IgA-related neuropathies were rare, heterogenous, and with a high tendency to evolution and malignancy.Most of PPN often present a relatively monomorphic clinical picture but they can be clinically heterogenous and must be suspected even if sensory impairment and demyelination are not the dominant features. Tendency to malignancy seems globally elevated and needs intensive follow-up. Diagnostic approach to patients presenting with peripheral neuropathy should always include the typing of monoclonal immunoglobulins in serum and urine. In contrast, patients presenting with MG should be submitted to nerve conduction study/electroneurography and neurological evaluation.

Very few data exist on causes and outcomes of hospitalization of immigrants in Italy. Even though immigration is a real challenge for the western countries, we are still unaware of how it reflects on the costs and the management of an acute care department. This study was aimed to compare the patterns of hospital use by immigrants incoming to the Acute Care Department of Neurology in Brescia, Italy, with those of the resident Italian people. The study was based on the hospital discharge data. Discharges of immigrants were compared to those of a random selection of Italian patients matched by age and sex. The length of the study period was of 2.5 years. A similar pattern of hospital use by age was observed between foreigners and Italian patients; however, average length of hospitalization was significantly longer in immigrant population.

Here we describe the imaging findings in a 73-year-old woman who had pain in the right inguinal region, followed by progressive weakness of muscles innervated by the right femoral and obturator nerves, diagnosed as nondiabetic lumbosacral radiculoplexus neuropathy. Magnetic resonance neurography showed thickening and increase in signal intensity of the right femoral and obturator nerves.

Purpose In March 2020, the World Health Organization declared the SARS-CoV-2 infection-related coronavirus Disease (COVID-19) a pandemic. During the first and second waves of the pandemic spread, there have been several reports of COVID-19-associated neurological manifestations, including acute seizures and status epilepticus (SE). In this systematic review, we summarized the available data on clinical features, diagnosis, and therapy of COVID-19-related SE. Methods We performed a systematic search of the literature to identify data on demographics, clinical, neurophysiological, and neuroradiological data of patients with COVID-19-related SE. We used regression models (linear or logistic) with a stepwise forward method to identify features associated with mortality or severity of SE. Results Thirty-nine articles were included with a total of 47 cases of SE associated with COVID-19. Age, time between the acute respiratory phase of SARS-CoV-2 infection and SE onset, and hospitalization correlated with a higher SE severity as assessed by quantitative validated scales. Conclusions SE can be a neurological manifestation of SARS-CoV-2 infection. Although a possible association between SE and COVID-19 has been reported, the exact mechanisms are still not fully understood. Systemic inflammatory syndrome due to cytokine release could play a role in COVID-19-related SE.

Restless Legs Syndrome/Willis Ekbom Disease (RLS/WED) is a common neurological disorder characterized by uncomfortable and unpleasant sensations in the legs, with an urge to move. The symptoms typically begin or worsen during periods of rest, in particular during the evening and at night, while the activity may typically relieve them. The majority of patients complains of poor sleep. Recent studies reported the prevalence is ranging from 5 to 10%. RLS/WED can be divided into primary (patients without associated conditions that may explain the symptoms) and secondary forms (mostly iron deficiency). RLS/WED is typically a chronic condition. The clinical course varies according to the age of onset. A great load of accumulating research and clinical data have led to an extended consensus for a need to enhance the diagnostic criteria. The aim of this paper is to provide a critical comparison among different diagnostic criteria, taking into account respectively the International Classification of Sleep Disorders (ICSD), the International RLS Study Group (IRLSSG) and the Diagnostic and Statistical Manual of Mental Disorders (DSM). There are several remarkable distinctions between the IRLSSG revised criteria, ICSD-3 and DSM-V. Contrary to the DSM-V criteria, ICSD-3 diagnostic criteria are more aligned to the IRLSSG ones. In fact, the five essential criteria of the IRLSSG are also required for the diagnosis of RLS/WED according to ICSD-3. The new IRLSSG criteria provide a more rigorous approach to case ascertainment and a better characterization of patients by specifying clinical significance and course. Future ascertainment of correct diagnosis should include documentation that all five diagnostic criteria are considered.