Extracranial arteriovenous malformation mimicking a peripheral nerve tumor: illustrative case.

BACKGROUND: Arteriovenous malformations (AVMs) are vascular malformations that are more commonly found intracranially, followed by the head, neck, limbs, and trunk. Extracranially, AVMs can mimic peripheral nerve tumors, leading to misdiagnosis. OBSERVATIONS: A 19-year-old female, who presented with left lateral lower leg pain, was preoperatively thought to have a peripheral nerve tumor; at surgery, however, she was found to have an extracranial AVM. The distinct margins of the tumor on preoperative magnetic resonance imaging suggested that the patient might have a peripheral nerve tumor; however, the clinical symptoms of focal pain at rest and the absence of Tinel's sign should have raised questions about this diagnosis. LESSONS: This case highlights the difficulty in differentiating a peripheral nerve tumor from an extracranial AVM in certain clinical scenarios. It is important to use a multifaceted diagnostic approach to get a correct preoperative diagnosis and plan treatment appropriately.

Quantitative Ultrasound Assessment of Hepatic Steatosis.

Background/Aims: Non-alcoholic fatty liver disease (NAFLD) is widespread chronic disease of the live in humans with the prevalence of 30% of the United States population.1,2 The goal of the study is to validate the performance of quantitative ultrasound algorithms in the assessment of hepatic steatosis in patients with suspected NAFLD. Methods: This prospective study enrolled a total of 31 patients with clinical suspicion of NAFLD to receive liver fat measurements by quantitative ultrasound and reference MRI measurements (proton density fat-fraction, PDFF). The following ultrasound (US) parameters based on both raw ultrasound RF (Radio Frequency) data and 2D B-mode images of the liver were analyzed with subsequent correlation with MRI-PDFF: hepatorenal index, acoustic attenuation coefficient, Nakagami coefficient parameter, shear wave viscosity, shear wave dispersion and shear wave elasticity. Ultrasound parameters were also correlated with the presence of hypertension and diabetes. Results: The mean (± SD) age and body mass index of the patients were 49.03 (± 12.49) and 30.12 (± 6.15), respectively. Of the aforementioned ultrasound parameters, the hepatorenal index and acoustic attenuation coefficient showed a strong correlation with MRI-PDFF derivations of hepatic steatosis, with r-values of 0.829 and 0.765, respectively. None of the remaining US parameters showed strong correlations with PDFF. Significant differences in Nakagami parameters and acoustic attenuation coefficients were found in those patients with and without hypertension. Conclusions: Hepatorenal index and acoustic attenuation coefficient correlate well with MRI-PDFF-derived measurements of hepatic steatosis. Quantitative ultrasound is a promising tool for the diagnosis and assessment of patients with NAFLD.

Professional and Demographic Profile of Spanish-Speaking Child Neurologists in the United States.

>Objective: To ascertain the prevalence of culturally native Spanish-speaking child neurologists in the United States. Methods: Prevalence statistics regarding demographic and work profile were applied to data obtained from a cross-sectional electronic survey of Child Neurology Society (CNS) members. Results: Demographics of the 135 respondents were comparable to a similar CNS survey except for ethnicity as shown in Table 1. Fifty- three percent were male and 24% were over age 60. Approximately a quarter were represented each from East, South, Midwest, and Western US. 42% self-identified as Spanish, Hispanic, or Latino. 62% spoke English as their primary language and 39% spoke Spanish as their primary language. Two-thirds graduated from a US medical school, 51% practice general neurology, and epilepsy was the most common subspecialty (18%). Two-thirds of respondents practice at a major teaching hospital, and 93% hold university academic appointments. 79% are AAN members. 76% did not have medical student debt at the time of the survey. 29% report signs consistent with burnout. 87% would choose Child Neurology again and 96% would recommend Child Neurology to a medical student. Conclusion: 40% of survey respondents self-identified as Hispanic, Latino or Spanish and spoke Spanish as the primary language and the majority practice in Academic Medicine. Nearly a third of those in the current survey identify burnout symptoms. Consideration of distinctive language and cultural characteristics across the US may lead to provision of a more patient-centered and equitable care.

Normal variant residual germinal matrix in extremely premature infants: radiographic features and imaging pitfalls.

BACKGROUND: As radiology volume from premature patients increases, previously undescribed imaging findings may be identified, posing diagnostic dilemma to the pediatric radiologist. OBJECTIVE: The primary goal of our study is to characterize the previously undescribed imaging finding of subependymal echogenicity at the floor of the frontal horns, which we postulate represents normal variant embryologic remnant residual germinal matrix. Furthermore, we hope to equip the pediatric radiologist with diagnostic criteria to distinguish this normal variant from pathology. MATERIALS AND METHODS: Retrospective review of neonates at our institution over a 10 year period was performed to identify extremely premature infants who received head ultrasounds during their hospital stay. Clinical data from EPIC was collected on these patients in addition to retrospective review of their head ultrasound images. RESULTS: Literature review of neuroembryology and observed involution of the frontal horn subependymal echogenicity on sequential imaging inform our hypothesis that this imaging finding represents normal variant residual germinal matrix. Two-thirds of the 210 included extremely premature infants demonstrated this finding, which was frequently misinterpreted as grade 1 germinal matrix, intra-choroidal or intra-ventricular hemorrhage. Residual matrix was concomitantly present with additional pathology in 29.4% of the patients. CONCLUSION: Previously undescribed subependymal echogenicity at the floor of the frontal horns is favored to represent normal variant embryologic remnant residual germinal matrix. Since this finding may be misinterpreted as germinal matrix, intra-choroidal or intra-ventricular hemorrhage, it is essential for the interpreting radiologist to be aware of this normal variant and not confuse it for pathology.

Pineal Dysgerminoma: A Misleading Clinical Course With Potential Life-Threatening Consequences.

Pineal dysgerminomas are sporadic pediatric intracranial tumors that usually grow as midline lesions around the third ventricle, most frequently the pineal gland and the pituitary regions of the brain. The severity of symptoms is dependent on the location of the lesion and can present with increased intracranial symptoms. We report a 20-year-old man who presented with new-onset headaches over the past month that would wake him from his sleep at night. The headaches, however, resolved completely one week prior to his first neurological evaluation. A thorough neurological examination was normal. A careful review of the literature does not show a case of a pineal tumor presenting with spontaneous regression of intracranial pressure, and therefore we would like to raise awareness among clinicians about this potential course. A delay in obtaining imaging could have been life-threatening; thus, we recommend a high index of suspicion when patients present with recent symptoms suggesting increased intracranial pressure. Our patient had an excellent outcome two years after his presentation, with appropriate management including drainage of the cerebrospinal fluid, chemotherapy, and radiotherapy.

Amyloidosis in the head and neck: CT findings with clinicopathological correlation.

PURPOSE: To characterize the CT imaging features of head and neck amyloidosis and correlate with extent of disease and clinical outcomes. MATERIALS AND METHODS: This retrospective study included 80 patients with head and neck amyloidosis who underwent soft tissue neck CT imaging between November 2003 and April 2018. The CT imaging features including lesion distribution, morphology (focal, diffuse/circumferential, or combined), presence and pattern of calcification, (punctate or diffuse), and thickness of airway lesion were evaluated and compared with the extent of amyloidosis (localized or systemic), and clinical course (stable, no recurrence, or progression requiring repeated surgical treatment). RESULTS: Localized disease (83.8%, 67/80) was most common with AL type (97.6%, 41/42) representing nearly all cases of head and neck amyloidosis. The larynx was the most frequently affected organ (60.0%, 48/80), specifically the glottis (43.8%, 35/80). Calcification was seen in 65.0% of cases (52/80). Non-airway or tongue lesions were significantly associated with systemic (92.3%, 12/13) as opposed to localized amyloidosis (4.5%, 3/67; P < 0.001). Repeated surgical treatment was significantly associated with laryngeal amyloidosis (35.3%, 12/34; P = 0.002) and multi-centric disease (33.3%, 10/30; P = 0.048). Airway wall thickness in patients who required repeated surgical treatment was significantly greater than in patients with stable or no recurrent disease (P = 0.016). CONCLUSION: Knowledge of the imaging features of head and neck amyloidosis can aid the diagnosis, disease monitoring, and prediction of patients requiring repeated surgical intervention.

Neonatal Encephalopathies: A Clinical Perspective.

Seizures are the most acute evident manifestation of central nervous system dysfunction in neonates. The incidence is higher in very low weight neonates, about 58/100 live births, as opposed to full-term infants, estimated about 3.5/100 live births. Neonatal seizures represent the clinical manifestation of a non-specific disorder of cortical cerebral dysfunction, which could lead to permanent brain injury. The etiology is multifactorial and requires a judicious assessment of each clinical scenario. The diagnosis and its management are further complicated as most neonatal seizures may have very subtle or no clinical changes and the diagnosis may be just based on EEG findings, so-called subclinical. The treatment is dependent on the etiology, but early and opportune intervention can prevent further brain damage and improve prognosis. Although early identification and treatment are essential, the diagnosis of neonatal seizures can be further complicated by the clinical presentations, possible etiologies, and treatments. Nevertheless, research studies and clinical evidence have shown that early treatment with anti-seizure medications can change the outcome.

Idiopathic Recurrent Polyneuritis Cranialis: A Rare Entity.

Recurrent polyneuritis cranialis is a rare disorder that can affect multiple cranial nerves. We describe a young man who presented with recurrent cranial nerve (CN) palsies. His first episode at 17 years of age involved the right VI cranial nerve, the second episode at age 21 involved the left V and VII cranial nerves while the last episode six months later affected the left IV cranial nerve. Based on the clinical findings and laboratory test results to exclude other possibilities, a diagnosis of idiopathic recurrent polyneuritis cranialis was made. This is a very rare disorder in childhood and adolescence. This is the youngest patient ever reported with recurrent polyneuritis cranialis of unknown etiology.

Brain MRI in Children with Mild Traumatic Brain Injury and Persistent Symptoms in Both Sports- and Non-sports-related Concussion.

AIM: To evaluate the utility of magnetic resonance imaging (MRI) in children with mild traumatic brain injury (mTBI), persistent post-concussive syndrome (PPCS), and normal neurologic examination. MATERIALS AND METHODS: This was a retrospective review of pediatric patients, who were evaluated in a Pediatric Concussion Clinic between August 2013 and November 2018, with documented persistent post-concussive symptoms, normal neurological examination, and available brain MRI. RESULTS: In our analysis of 86 cases we found seven MRI studies with abnormal findings, but none were clinically significant. CONCLUSION: We conclude that MRI has a low diagnostic yield in this population, and based on these results we recommend that clinicians should avoid ordering MRI studies in this group. Further research is necessary to validate these results in larger and prospective studies.

Mixoma auricular asociado a evento cerebrovascular isquémico / Atrial myxoma related with ischemic cerebrovascular event

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[Treatment of neonatal seizures]. / Tratamiento de las convulsiones neonatales.

Neonatal seizures are among the most dramatic manifestations of acute central nervous system dysfunction. The incidence is much higher in very low weight neonates than in full term infants (˜ 58 and 3.5 per 100 live births, respectively). Neonatal seizures represent the clinical manifestation of a non-specific cortical cerebral dysfunction which can lead to permanent brain injury. The etiology is multifactorial and requires a judicious assessment for each clinical scenario. The diagnosis is further complicated by the fact that most neonatal seizures are subclinical, that is, may display very subtle or no clinical changes and the diagnosis may just be based on EEG findings. The treatment depends on the etiology, but an early and opportune intervention prevents further brain damage, thus improving prognosis. Although early identification and treatment are critical, the diagnosis of neonatal seizures is complicated by several factors such as different clinical presentations, possible etiologies and several treatment options. Nevertheless, research studies and clinical evidence have shown that early treatment with anti-seizure medications can change the outcome.

Tratamiento de las convulsiones neonatales / Treatment of neonatal seizures

Las convulsiones neonatales están entre las manifestaciones más dramáticas de enfermedad neurológica y deben ser consideradas una emergencia. La incidencia es 3.5 por cada 100 nacidos a término y en prematuros asciende a 58 por cada 100 nacidos vivos. Las convulsiones neonatales son una manifestación clínica de disfunción cortical no específica que puede dar lugar a daño permanente del cerebro. La etiología es multifactorial y requiere una evaluación cuidadosa de cada escenario clínico. El diagnóstico es más complejo por el hecho de que la mayoría de convulsiones son sub-clínicas o sutiles y a veces no tienen correlación con el electroencefalograma. Aunque la identificación temprana y el tratamiento son críticos, el diagnóstico se complica por algunos factores como la variedad de presentaciones clínicas, diferentes etiologías y varias alternativas de tratamiento. De todas maneras, los estudios de investigación y la evidencia clínica disponible han demostrado que el tratamiento precoz con fármacos anticonvulsivantes puede mejorar el pronóstico.

Neonatal seizures are among the most dramatic manifestations of acute central nervous system dysfunction. The incidence is much higher in very low weight neonates than in full term infants (~58 and 3.5 per 100 live births, respectively). Neonatal seizures represent the clinical manifestation of a non-specific cortical cerebral dysfunction which can lead to permanent brain injury. The etiology is multifactorial and requires a judicious assessment for each clinical scenario. The diagnosis is further complicated by the fact that most neonatal seizures are subclinical, that is, may display very subtle or no clinical changes and the diagnosis may just be based on EEG findings. The treatment depends on the etiology, but an early and opportune intervention prevents further brain damage, thus improving prognosis. Although early identification and treatment are critical, the diagnosis of neonatal seizures is complicated by several factors such as different clinical presentations, possible etiologies and several treatment options. Nevertheless, research studies and clinical evidence have shown that early treatment with anti-seizure medications can change the outcome.

Incidentaloma suprarrenal, patología emergente en la era de los avances imaginológicos / Adrenal Incidentaloma, Emerging Pathology in the Age of the Imaging Advances

El incidentaloma adrenal es una masa encontrada de forma incidental durante un estudio de imagen, con múltiples implicaciones clínicas según su etiología; por esto es importante discutir su abordaje diagnóstico y el manejo médico correspondiente. Se presenta el caso de un paciente masculino de 64 años de edad, hospitalizado por cuadro clínico de síncope, hipertensión no controlada e hiperglucemia, a quien además se le había detectado previamente una masa suprarrenal en estudio ambulatorio de imagen. Durante su estancia se realizaron estudios imaginológicos y laboratorios para determinar la etiología de esta masa, su relación con el cuadro clínico actual y su tratamiento. Se concluye que el incidentaloma adrenal es una entidad frecuente cuyo reto diagnóstico consiste en establecer si se trata de una entidad maligna o benigna y si es funcionante o no, con el fin de determinar si requiere manejo quirúrgico o solo observación.

Introduction: Adrenal incidentaloma is a mass found incidentally during an imaging study with multiple clinical implications according etiology. For this reason, it is important to discuss the diagnostic approach and appropriate medical management. Case: 64 year old male patient, hospitalized for clinical symptoms of syncope, uncontrolled hypertension and hyperglycemia; in addition, a suprarenal mass was previously detected in an outpatient image study. During his stay, imaging and laboratory studies were conducted to determine the etiology of this mass, relative to the current clinical condition and treatment. Conclusion: The adrenal incidentaloma is a frequent diagnosis. Its challenge is to determine whether it is malignant or a benign entity and whether it is functioning or not, in order to determine if it requires surgical treatment or if it only requires observation.