Intercostal Neurolysis for The Treatment of Postsurgical Thoracic Pain: a Case Series.

INTRODUCTION: We investigated the possible role of intercostal surgical neurolysis in relieving chronic neuropathic pain refractory to other nonsurgical treatments in patients with postsurgical thoracic pain. METHODS: We retrospectively collected clinical data on patients referred to the Neurosurgery Unit of Policlinic Hospital of Milan. Ten patients (age range, 20-68 years) suffering from neuropathic pain for at least 2 months after thoracic surgery underwent intercostal neurolysis. RESULTS: Compared with preneurolysis, pain intensity decreased 1 month postneurolysis and remained stable 2 months postneurolysis (median score [interquartile range]: 8 [6-9] preneurolysis, 4 [3-5] 1 month after, and 3 [2-5] 2 months after, P < 0.001). Antiepileptic drugs for pain control decreased after neurolysis. DISCUSSION: Surgical intercostal neurolysis may be a promising therapeutic option in patients with chronic neuropathic pain associated with neurological deficits. Muscle Nerve 58: 671-675, 2018.

Nervous system involvement in Farber disease.

A 30 months-old boy with Farber disease developed nystagmus 12 months after hematopoietic stem cell transplantation (HSCT). At 40 months, gait ataxia was evident, and brain MRI showed increased size of pericerebellar sulci and 4th ventricle. EMG showed denervation in the tongue and upper limb muscles, consistent with motor neuron disease. HSCT improves the peripheral manifestations of Farber disease, but may not prevent the progressive neurological deterioration.

Increased levetiracetam clearance and breakthrough seizure in a pregnant patient successfully handled by intensive therapeutic drug monitoring.

We report a 36-year-old pregnant patient with subtherapeutic trough plasma levels of levetiracetam (LVT) and a breakthrough nocturnal seizure while assuming 3 times a day dosing of the drug. An intensive pharmacokinetic study was performed from immediately before to 11 hours after the morning LVT dose administration and suggested that the patient was not adequately exposed to the drug during the night. After changing the dosing of LVT to 4 times a day, the patient experienced no seizures and delivered a healthy newborn without complications. Afterward, trough plasma levels of LVT remained always within the therapeutic range until delivery, and no major increase of the drug daily dose was required.

Severe and isolated headache associated with hypertension as unique clinical presentation of posterior reversible encephalopathy syndrome.

BACKGROUND: Posterior reversible encephalopathy syndrome is a potentially reversible clinicoradiologic syndrome characterized by headache, mental confusion, visual disturbances and seizures associated with posterior cerebral lesions on radiological imaging. Prompt treatment of this condition is mandatory to avoid severe irreversible complications. CASE PRESENTATION: We report a 9-year-old boy with arterial hypertension and headache as unique clinical presentation of posterior reversible encephalopathy syndrome. CONCLUSIONS: Severe and isolated headache associated with arterial hypertension can be the unique clinical presentation of posterior reversible encephalopathy syndrome. This syndrome must be considered even in absence of all typical symptoms to prevent the progression of a potentially life threatening condition.

Electrophysiological study of medial and lateral branches of the superficial radial nerve.

INTRODUCTION: In this study we sought to establish a new technique for nerve conduction study (NCS) of medial and lateral branches of the superficial radial nerve (SRN). METHODS: Antidromic NCS were performed on 60 healthy subjects, recording the sensory nerve action potential (SNAP) from the dorsomedial and dorsolateral aspects of the phalanx of the thumb. The main trunk of SRN was also investigated. RESULTS: SNAPs were easily recorded in all subjects, and normative latency and amplitude data were collected. CONCLUSIONS: We propose a simple and reliable technique to investigate nerve conduction of the terminal branches of the SRN, which could be useful in some focal and systemic pathologies.

Corticosteroid Treatment in Sydenham Chorea: A 27-Year Tertiary Referral Center Experience.

OBJECTIVE: The purpose of this study was to investigate the effectiveness of corticosteroid therapy for children suffering from Sydenham chorea (SC). METHODS: The design of the study was observational, retrospective and conducted at the single center of the Rheumatology Unit of Policlinic Hospital of Milan, Italy, from May 1995 to May 2022. All data about the patients were collected from medical records. RESULTS: From a total of 59 patients enrolled in the study (44 females and 15 males; median age 9.3 years, range 7.4-10.6 years), 49 were eligible for primary outcome analysis (10 patients were excluded due to incomplete data). Overall, 75% of patients received steroid therapy, while the remaining cases were treated with symptomatic drugs, including neuroleptics and antiseizure drugs. We found that the duration of chorea was significantly shorter in patients treated with corticosteroids in comparison to those receiving symptomatic treatment (median time: 31 vs. 41 days, p = 0.023). Additionally, patients with arthritis at the onset of the disease had a longer duration of chorea than those without arthritis (median time 90.5 vs. 39 days, p = 0.02). We also found that chorea recurred in 12% of the patients and seemed to be linked to a younger age at onset (p = 0.01). CONCLUSIONS: The study suggests that corticosteroid therapy can lead to a faster resolution of SC when compared to neuroleptics and antiseizure drugs treatment.

Febrile seizures and convulsions with mild gastroenteritis: age-dependent acute symptomatic seizures.

Background: Febrile seizures (FS) and benign convulsions in children with mild gastroenteritis (CwG) are acute symptomatic seizures, transiently occurring in infants and young children, probably related to the immaturity of the brain. Our paper aims to review the literature data on patients with FS and CwG. Methods: A review of series of patients with FS and CwG was performed by literature search on PubMed January 1960 to October 2022. Several parameters were considered, including epidemiology, pathophysiology, clinical features, electroencephalographic findings and other diagnostic studies, and treatment. Results: FS and CwG share an age-dependent course, but they show significant differences in the pathophysiology, clinical features, diagnostic studies, and treatment. Conclusion: Acute symptomatic seizures include seizures that are caused by acute structural brain pathologies, such as stroke, as well as seizures that are provoked by a reversible factor, such as hyponatraemia, although the two groups should be not equated. Furthermore, FS and CwG should be set apart as "age-dependent acute symptomatic seizures", reinforcing the concept of their self-limited course over a certain period.

Questions and Controversies in Neonatal Seizures.

Neonatal seizures are relatively common, but their diagnosis and management remain challenging. We reviewed the scientific literature on neonatal seizures from July 1973 to November 2023. Several parameters were considered, including pathophysiology, diagnostic criteria, electroencephalographic findings and treatment. Recent classification system of seizures and epilepsies in the newborn, as well as treatment recommendations of neonatal seizures, have been proposed. Nonetheless, the approach to neonatal seizures varies among clinicians and centres, including detection, investigation, treatment and follow-up of patients. There are still many issues on the diagnosis and treatment of neonatal seizures, including the meaning or relevance of some electroencephalographic findings, the precise estimation of the seizure burden, the limited efficacy and side effects risk of antiseizure medications, and the best measures to establish the outcome.

How Can Specialist Advice Influence the Neuroimaging Practice for Childhood Headache in Emergency Department?

Differentiating between primary and secondary headaches can be challenging, especially in the emergency department (ED). Since symptoms alone are inadequate criteria for distinguishing between primary and secondary headaches, many children with headaches undergo neuroimaging investigations, such as brain CT and MRI. In various studies, the frequency of neuroimaging utilization is influenced by several factors, including teaching status, ownership, metropolitan area, insurance status, and ethnicity of patients. However, only a few studies have considered the role of specialist consultations in ordering neuroimaging studies on childhood headaches. We report the contributions of different specialists to the evaluation of children with headaches admitted to the ED and their influence on neuroimaging decisions. We retrospectively reviewed the medical reports of paediatric patients who presented with headaches to the paediatric ED of the Ospedale Maggiore Policlinico of Milano between January 2017 and January 2022. Overall, 890 children with headaches were evaluated (mean age: 10.0 years; range: 1 to 17 years). All patients were examined by the ED paediatricians, while specialist consultations were required for 261 patients, including 240 neurological (92.0%), 46 ophthalmological (17.6%), and 20 otorhinolaryngological (7.7%) consultations. Overall, 173 neuroimaging examinations were required, of which 51.4 and 48.6% were ordered by paediatricians and neurologists, respectively. In particular, paediatricians required 61.4% of brain CT scans, and neurologists required 92.0% of brain MRI scans. In conclusion, paediatricians were responsible for the management of most children with headaches admitted to the ED, while specialist consultations were required only in about a third of the cases. Although there was no significant difference in the number of neuroimaging studies ordered by specialists, brain CT scans were most often used by paediatricians, and MRI scans by neurologists.

Impact of COVID-19 Pandemic on Headache Evaluations in the Pediatric Emergency Department.

BACKGROUND: To evaluate the impact of COVID-19 on evaluations in the pediatric emergency department (ED) because of headache as main symptom. METHODS: Number and clinical features of patients evaluated in the pediatric ED of a single site in Milan, Italy, were collected between January 2017 and January 2022. The impact of COVID-19 on evaluation rates was quantified by using the incidence rate ratio (IRR) and 95% confidence intervals (CI) between the pandemic (March 2020 to January 2022) and the prepandemic period (January 2017 to February 2020). RESULTS: During the study period, 890 evaluations were registered: 689 over the prepandemic period and 201 over the pandemic period. Mean age at evaluation was 10 years (range: 1 to 17 years). Evaluation rates per month were 18.1 during the prepandemic period and 8.7 during COVID-19 pandemic, with peaks in autumn and winter months and considerable drops in the summer. The IRR was 0.49 (95% CI, 0.40-0.61). The reduction in evaluation rate was higher for secondary headache (IRR, 0.31; 95% CI, 0.23-0.42) when compared with primary headache (IRR, 0.56; 95% CI, 0.40-0.78). CONCLUSIONS: We found a remarkable reduction in the number of evaluations in the pediatric ED for headache during the pandemic period.

A Comparative Study of Febrile Seizures and Febrile Convulsions Associated With Mild Gastroenteritis.

BACKGROUND: Children presenting with complex febrile seizures (FS) have an increased risk of developing epilepsy. This study aimed to investigate the occurrence of complex seizures in children presenting with FS and those with both convulsions associated with mild gastroenteritis (CwG) and fever. METHODS: Children admitted to our Pediatric Emergency Department between January 2017 and April 2019 with seizures were enrolled in this cross-sectional study. Patients were grouped according to the etiology as FS and febrile CwG. FS classification criteria of simple FS and complex FS was applied to both groups to allow a comparison between them. Prevalence ratios (PRs) of complex seizures, estimated through a log binomial model, were used to compare the occurrence of complex seizures between the two groups, using the FS group as reference category. RESULTS: A total of 294 patients were enrolled: 231 with FS and 63 with febrile CwG. Complex seizures occurred in 31 patients with FS (13.4%) and 21 patients (33.3%) with febrile CwG. The PR of complex seizures was 2.48 (95% confidence interval, 1.54 to 4.01). CONCLUSIONS: Children with febrile CwG showed a higher rate of complex seizures when compared with those with FS.

Bathing epilepsy: a video case report.

Bathing epilepsy is a rare form of reflex epilepsy triggered by bathing in room temperature water. It predominates in boys with a mean age of 15 months and its evolution is benign. Diagnosis of bathing epilepsy requires the exclusion of other paroxysmal disorders triggered by water contact. Video-EEG confirmation of the seizures is necessary to reach a diagnosis of certainty and to allow adequate management. We present the case of a one-year-old boy who experienced recurrent episodes of unresponsiveness and cyanosis while bathing in lukewarm water. The diagnosis of bathing epilepsy was confirmed by the video-EEG recording of a seizure, showing left-sided frontotemporal delta activity with rapid contralateral spread. Therapy with levetiracetam was effective, subsequently allowing bathing without further seizures.

Steroid Pulse Therapy for Severe Central Nervous System Involvement in Shiga Toxin-Producing Escherichia coli-Related Hemolytic Uremic Syndrome.

We report on the case of a 7-year-old boy with Shiga toxin-producing Escherichia coli-related hemolytic uremic syndrome (STEC-HUS), initially presenting with abdominal pain as the only clinical feature and thus requiring differential diagnosis with a surgical emergency. Diagnosis of STEC-HUS was made with the appearance of bloody diarrhea and renal function impairment, and the clinical picture rapidly progressed to multiorgan failure. Relatively late and severe central nervous system (CNS) involvement was present, characterized by subacute encephalitis progressing to coma, which became apparent when the acute phase of thrombotic microangiopathy was resolving. Therefore, neurologic manifestations were thought to be related to reperfusion damage to the CNS and high-dose IV steroid pulse therapy was empirically administered. Following this therapeutic scheme, neurologic involvement resolved with no sequelae. This case offers several points of discussion on the clinical presentation and the diagnostic approach to STEC-HUS, on the related neurologic complications, and on a novel approach to their management.

Development and Preliminary Validation of an Electromyography-Scoring Protocol for the Assessment and Grading of Muscle Involvement in Patients With Juvenile Idiopathic Inflammatory Myopathies.

INTRODUCTION: We performed a pilot study in order to investigate the feasibility of an electromyography (EMG)-scoring protocol for the assessment of disease activity in juvenile idiopathic inflammatory myopathies (JIIM). METHODS: Children with JIIM followed up in a tertiary-level care center underwent standardized clinical, laboratory, and EMG assessment. An EMG-scoring protocol was devised by a consensus panel including a pediatric neurophysiologist and two pediatric rheumatologists, based on a combined score obtained as the sum of (1) the presence of denervation signs (fibrillation potentials) and (2) motor unit remodeling (mixed pattern of short- and long-duration motor unit action potentials). The EMG-scoring protocol was then validated following the Outcome Measures in Rheumatoid Arthritis Clinical Trials filter for outcome measures in rheumatology and the consensus-based standards for the selection of health measurement instruments methodology. RESULTS: Thirteen children (77% females) were included in the study, with a median age of 10 years (interquartile range: 7-17 years) and median disease duration of 11.8 months (interquartile range: 2.1-44.5). A total of 39 EMG examinations were evaluated. A strong positive association between a standardized tool for muscle strength assessment and the combined score was observed. No significant associations were found with both creatine kinase and erythrocyte sedimentation rate levels. DISCUSSION: Our EMG-scoring protocol is the first standardized and reproducible tool for the neurophysiologic evaluation and grading of muscle involvement in patients with JIIM and could provide relevant additional information in the assessment and follow-up of these rare conditions.

Infant Botulism: Checklist for Timely Clinical Diagnosis and New Possible Risk Factors Originated from a Case Report and Literature Review.

Infant botulism is a rare and underdiagnosed disease caused by BoNT-producing clostridia that can temporarily colonize the intestinal lumen of infants less than one year of age. The diagnosis may be challenging because of its rareness, especially in patients showing atypical presentations or concomitant coinfections. In this paper, we report the first infant botulism case associated with Cytomegalovirus coinfection and transient hypogammaglobulinemia and discuss the meaning of these associations in terms of risk factors. Intending to help physicians perform the diagnosis, we also propose a practical clinical and diagnostic criteria checklist based on the revision of the literature.

A cortically blind patient with preserved visual imagery.

BACKGROUND/OBJECTIVE: The loss or preservation of visual imagery in patients with cortical blindness may be helpful in resolving the controversial roles assigned by some researchers to the early visual cortex during the process of visual imagery. PATIENT AND METHODS: Here we report a patient with complete permanent cortical blindness coupled with denial of the blindness (Anton syndrome) as a result of bilateral occipital infarction. RESULTS: Interestingly, the patient's ability to visualize objects, color, and spatial imagery was preserved, although cerebral computed tomography, magnetic resonance imaging, and positron emission tomography scans detected what was likely complete bilateral damage to the primary visual cortex. CONCLUSIONS: Our findings may support the hypothesis that the primary visual cortex, in which retinal spatial geometry is preserved, is not critical for visual imagery.

Autoimmune rhomboencephalitis: A pediatric case report.

Rhomboencephalitis is a potentially life-threatening condition due to inflammation of the hindbrain with an unpredictable outcome depending on the wide spectrum of etiologies and the promptness of diagnosis and treatment. A 23-month-old Caucasian male presented with fever, clouding of consciousness, and trunk ataxia. Three weeks earlier he received active immunization against varicella-zoster virus. Routine laboratory tests were unremarkable. Cerebrospinal fluid showed might elevation of leukocytes. The infection panel was negative. Brain magnetic resonance showed signal hyperintensity in the dorsal portion of the pons, which was consistent with a rhomboencephalitis. Autoimmune pathogenesis was supposed and a high dose of steroids was started. The patient's neurologic status progressively improved till full recovery and complete regression of previous magnetic resonance lesions after 1 year. Nevertheless, longer follow-up is needed in order not to miss any possible signs of an underlying autoimmune neurologic disorder.