Specific Neuroimaging Findings Research Articles

Minimizing unnecessary brain magnetic resonance imaging in pediatric endocrinology: a retrospective cohort analysis.

Brain magnetic resonance imaging (MRI) is mandatory or highly recommended in many pediatric endocrinological conditions to detect causative anatomic anomalies and rule out neoplastic lesions. However, MRI can also show findings associated with the underlying clinical condition, as well as unrelated "incidentalomas". These latter findings are often abnormalities with a high incidence in the general population for which there is no clear literature regarding their management, especially in pediatric patients. The present study aimed to evaluate the number of unnecessary performed MRIs in pediatric endocrinology. Retrospective analysis on 584 MRI scans performed in 414 patients (254 growth hormone deficiency, 41 other causes of short stature, 116 central precocious puberty). The MRI scans were completely normal in 67% of the individuals, and the prevalence of individuals who underwent more than one MRI was 18%, with no significant differences among the groups. The overall prevalence of incidentalomas was 17%. Among 170 repeated MRI scans, 147 (86%) were not required according to a dedicated protocol. Only five patients (four GHD, one Noonan) correctly repeated the MRI. All the repeated MRI scans did not reveal any progression in the findings. If we include the MRIs performed in cases of OCSS other than Noonan syndrome (n=32) and girls with CPP older than 6 years (n=89), an additional 121 MRIs could have been avoided, leading to a total number of unnecessary MRIs to 268 (46%). Only a few specific neuroimaging findings in endocrinologic pediatric patients warrant further investigation, while too often repeated imaging is carried out unnecessarily. We advocate the importance of guidelines to reduce costs for both the healthcare system and patients' families, as well as to alleviate physical and psychological distress for patients and caregivers.

Dyke–Davidoff–Masson Syndrome – A Case Series

Abstract Dyke–Davidoff–Masson syndrome (DDMS) refers to variable degrees of atrophy of one cerebral hemisphere, resulting from brain injury in intrauterine or early years of life. Clinical features of this rare syndrome include varying degrees of contralateral hemiplegia, facial asymmetry, seizures, and mental retardation. Specific neuroimaging findings include unilateral brain volume loss, ventriculomegaly, and compensatory bone hypertrophy. In addition to this, calvarial thickening and hyperpneumatization of frontal sinuses may occur. We hereby report three cases of this syndrome, with varied clinical and radiographic features, to highlight the wide spectrum of this syndrome.

Stroke Mimics: Insights from a Retrospective Neuroimaging Study

Objectives: The study’s goals are to evaluate the management of Stroke Mimics (SMs), conditions with stroke-like symptoms but non-vascular origins. It seeks to assess the effect of unnecessary intravenous thrombolysis on therapy delays and determine the best SMs diagnosis approach. Materials and methods: A review was conducted of all patients admitted to the Emergency Department under a “stroke code” from January 1, 2018, to January 31, 2019. Anamnestic and clinical data, along with information on neuroimaging protocols and findings, were collected. Advanced MRI sequences, such as Arterial Spin Labeling (ASL) MR perfusion and MR spectroscopy, were revised to confirm the diagnoses. Results: 513 stroke codes were found; a neurovascular disease was diagnosed in 414 cases: 282 ischemic strokes and 73 intracranial hemorrages and 59 TIAs. The 99 SMs included, 13 infections, 12 syncopes, 11 epileptic seizures, 11 hemodinamic conditions, 10 tumors, 9 metabolic disorders, 9 diziness, 7 migraines, 4 drug/alcohol intoxication, 3 functional disorders, 3 acute hydrocefalus, 2 multiple sclerosis, 2 arteriovenous malformations, 1 spinal cord compression and 2 unexplained conditions. Specific neuroimaging findings were evaluated for all cases. Positive and negative predictive values of clinical diagnosis of SM were respectively 0.23 and 0.35. 125 SM patients underwent MRI examination, 40 of which within 3 hours from the activation of stroke code. Advanced MRI sequences as ASL, MR perfusion, MR spectroscopy were used to reach the correct diagnosis. Conclusions: Advanced multimodal MRI can be a valuable tool in the assessment of, particularly in cases where conventional imaging techniques such as DWI-FLAIR mismatch are inconclusive. The novelty of this retrospective study is to demonstrate that the consistent use of arterial spin labeling perfusion in cases with stroke code leads to a rapid and accurate diagnosis of SMs. The implementation of an MRI-based pathway can expedite the diagnosis and treatment of underlying non-vascular causes such as SMs.

PREDICTORS OF CLINICAL OUTCOMES IN INDIAN PATIENTS WITH ACUTE ENCEPHALOPATHY: A PROSPECTIVE OBSERVATIONAL STUDY FROM A TERTIARY CARE CENTER IN INDIA

Objective: Objective of the study was to evaluate the predictors of poor disease outcome at discharge and at 1 month in patients with acute encephalopathy. Methods: This prospective, observational, single center study included adult patients meeting the diagnostic criteria for acute confusion state and admitted in the intensive care unit of a tertiary care hospital. A modified Rankin Scale (mRS) score of <3 was considered as “good outcome,” while mRS ≥3 was considered as an indicator of “poor outcome.” Results: Among the total population of 219, 52.5% (n=115) were male, the mean age was 41.58 (±18.10) years and mean disease duration was 14.30 (±10.05) days (range: 1–30 days). Lethargy was the most common history at presentation (84.93%), while sleep abnormalities were least common (4.57%), and tuberculous meningitis was the most common etiology (21%). Diminution of vision, diplopia, dysarthria, cranial nerve symptoms, abdominal pain, difficulty in breathing, seizures, high-risk behavior, loss of appetite and the diagnosis of posterior reversible encephalopathy, retroviral disease, stroke and tuberculous meningitis were significant predictors of “poor outcome” at discharge (p<0.05). A diagnosis of tuberculous meningitis, history of headache, diminution of vision, diplopia, dysarthria, seizures, sensory deficits and loss of appetite and neuroimaging findings of atrophy, intracranial bleeding, demyelination, and space-occupying lesion were found to be significant predictors of “poor outcome” at 1 month post-discharge in this population (p<0.05). Conclusion: In patients with acute encephalopathy, tuberculous etiology, the presence of focal brainstem deficits and specific neuroimaging findings indicate poor outcomes at discharge as well as at 1 month follow-up.

Neuroimaging Findings in Patients with COVID-19: A Systematic Review and Meta-Analysis.

ObjectiveCentral nervous system involvement in coronavirus disease 2019 (COVID-19) has been increasingly reported. We performed a systematic review and meta-analysis to evaluate the incidence of radiologically demonstrated neurologic complications and detailed neuroimaging findings associated with COVID-19.Materials and MethodsA systematic literature search of MEDLINE/PubMed and EMBASE databases was performed up to September 17, 2020, and studies evaluating neuroimaging findings of COVID-19 using brain CT or MRI were included. Several cohort-based outcomes, including the proportion of patients with abnormal neuroimaging findings related to COVID-19 were evaluated. The proportion of patients showing specific neuroimaging findings was also assessed. Subgroup analyses were also conducted focusing on critically ill COVID-19 patients and results from studies that used MRI as the only imaging modality.ResultsA total of 1394 COVID-19 patients who underwent neuroimaging from 17 studies were included; among them, 3.4% of the patients demonstrated COVID-19-related neuroimaging findings. Olfactory bulb abnormalities were the most commonly observed (23.1%). The predominant cerebral neuroimaging finding was white matter abnormality (17.6%), followed by acute/subacute ischemic infarction (16.0%), and encephalopathy (13.0%). Significantly more critically ill patients had COVID-19-related neuroimaging findings than other patients (9.1% vs. 1.6%; p = 0.029). The type of imaging modality used did not significantly affect the proportion of COVID-19-related neuroimaging findings.ConclusionAbnormal neuroimaging findings were occasionally observed in COVID-19 patients. Olfactory bulb abnormalities were the most commonly observed finding. Critically ill patients showed abnormal neuroimaging findings more frequently than the other patient groups. White matter abnormalities, ischemic infarctions, and encephalopathies were the common cerebral neuroimaging findings.

Focal Cortical Dysplasia as Cause of Refractory Epilepsy- A Case Report

Focal Cortical Dysplasia (FCD) is one of the most common causes of refractory epilepsy in children as well as adult where malformed cortical development occurs resulting from abnormal neuronal migration due to both genetic and acquired factors. Herein, a 22-year-old male presented with recurrent secondary generalized tonic seizure with aura since childhood. Despite adequate anti-epileptic medications with good compliance the seizure was uncontrolled. As a cause, Type II FCD was diagnosed by specific neuroimaging findings supported by EEG abnormalities. Till now in refractory epilepsy FCD is rarely diagnosed but there remains a good hope of cure by surgical intervention.
 Bangladesh Journal of Neuroscience 2019; Vol. 35 (1): 46-50

Paroxysmal sympathetic hyperactivity syndrome caused by fat embolism syndrome

Paroxysmal sympathetic hyperactivity represents an uncommon and potentially life-threatening complication of severe brain injuries, which are most commonly traumatic. This syndrome is a clinical diagnosis based on the recurrent occurrence of tachycardia, hypertension, diaphoresis, tachypnea, and occasionally high fever and dystonic postures. The episodes may be induced by stimulation or may occur spontaneously. Underdiagnosis is common, and delayed recognition may increase morbidity and long-term disability. Trigger avoidance and pharmacological therapy can be very successful in controlling this complication. Fat embolism syndrome is a rare but serious complication of long bone fractures. Neurologic signs, petechial hemorrhages and acute respiratory failure constitute the characteristic presenting triad. The term cerebral fat embolism is used when the neurological involvement predominates. The diagnosis is clinical, but specific neuroimaging findings can be supportive. The neurologic manifestations include different degrees of alteration of consciousness, focal deficits or seizures. Management is supportive, but good outcomes are possible even in cases with very severe presentation. We report two cases of paroxysmal sympathetic hyperactivity after cerebral fat embolism, which is a very uncommon association.

Neuromyelitis Optica.

Neuromyelitis optica (NMO), formerly known as Devic disease, is an autoimmune astrocytopathic disease characterized by transverse myelitis and optic neuritis. Most patients demonstrate a relapsing course with incomplete recovery between attacks, resulting in progressive disability. The pathogenesis involves production of aquaporin-4 antibodies (AQP4-IgG) by plasmablasts in peripheral circulation, disruption of the blood-brain barrier, complement-mediated astrocyte injury, and secondary demyelination. The diagnosis relies on characteristic clinical manifestations in the presence of serum AQP4-IgG positivity or specific neuroimaging findings, and exclusion of alternative etiologies. Current treatment involves aggressive immunosuppression with pulse-dose steroids during acute attacks and long-term immunosuppression for attack prevention.

Central Nervous System Manifestations of Systemic Lupus Erythematosus.

Neuropsychiatric systemic lupus erythematosus (NPSLE) encompasses a variety of phenomena. Manifestations are focal or diffuse, and correlate with disease mechanisms. Recent understanding of the contribution of blood-brain barrier dysfunction to the passage of circulating antineuronal antibodies into the brain parenchyma has shed light on pathogenesis. Correct attribution of neuropsychiatric manifestations to SLE remains a challenge, but validated attribution models will help. Diagnosis relies on characteristic clinical manifestations, SLE disease activity, antibody measurements, cerebrospinal fluid findings, specific neuroimaging findings, and exclusion of alternative etiologies. Current treatment encompasses the identification and management of the inciting event, symptomatic treatment, and anticoagulation or immunosuppression.

Headache in Neuromyelitis Optica.

Neuromyelitis optica (NMO) classically features a clinical presentation that includes longitudinally extensive transverse myelitis and optic neuritis. However, many other pathognomonic phenomena have more recently been described in patients diagnosed with NMO, including intractable hiccups, vomiting, and painful tonic spasms, but less has been reported regarding the relationship between NMO and headache. Though headache is well established as both a symptom and comorbidity of multiple sclerosis (MS), it has been much less described thus far in the NMO literature and warrants more careful evaluation. Many questions remain unanswered about the relationship between NMO and headache, including headache prevalence in certain groups, distribution of primary and symptomatic headache disorders that are seen most frequently and the specific neuroimaging findings that are associated with an increased risk of headache. Various types of headache, such as cervicogenic headache and trigeminal autonomic cephalalgia-like headache, have been reported as the initial clinical presentation of NMO. Other publications have emphasized the association of NMO and other etiologies of headache, such as trigeminal neuralgia, PRES, and preeclampsia. Certain MR imaging findings such as medullary lesions in patients with NMO have also been associated with headache. The link between headache and NMO is evident not only in limited case reports and clinical studies but also with both MR imaging and even with some potential common underlying biomarkers such as pentraxin-3 and interleukin-6. Developing a further understanding in the association between these two diseases may lead to better management of headache in patients with NMO and potentially lead to earlier diagnosis of NMO in whom headache may serve as an initial presenting symptom and may even herald a disease exacerbation.

Associated ultrasonographic findings in fetuses with microcephaly because of suspected Zika virus (ZIKV) infection during pregnancy.

To describe fetal ultrasonographic findings and outcomes in a series of cases of fetal microcephaly associated with Zika virus infection. Retrospective case series of microcephaly with definite (laboratory evidence) or highly probable (specific neuroimaging findings and negative laboratory results) maternal Zika virus infection. Microcephaly was graded as mild if the head circumference was between 2 and 3 standard deviation (SD) below the mean, and severe if 3 or more SD below the mean. Associated central nervous system (CNS) and extracranial malformations are described. Nineteen singleton pregnancies fulfilling the inclusion criteria were identified. Severe microcephaly and mild microcephaly were identified in 14 and 5 fetuses, respectively. Additional CNS malformations were present in 17 cases and 7 had extracranial congenital anomalies. Symptoms were reported in 13/19 cases at a gestational age between 5 and 16 weeks. Mean (±SD) gestational age at ultrasound diagnosis was 32.3 ± 5.1 weeks. Amniocentesis was performed in five cases at a median gestational age of 31 weeks (range 28-38) and was positive for Zika virus RT-PCR in two cases. There were three neonatal deaths and one stillbirth. In the presence of fetal microcephaly associated with Zika virus infection, CNS malformations are frequently detected. © 2016 John Wiley & Sons, Ltd.

Congenital Zika virus syndrome in Brazil: a case series of the first 1501 livebirths with complete investigation

In November, 2015, an epidemic of microcephaly was reported in Brazil, which was later attributed to congenital Zika virus infection. 7830 suspected cases had been reported to the Brazilian Ministry of Health by June 4, 2016, but little is known about their characteristics. We aimed to describe these newborn babies in terms of clinical findings, anthropometry, and survival. We reviewed all 1501 liveborn infants for whom investigation by medical teams at State level had been completed as of Feb 27, 2016, and classified suspected cases into five categories based on neuroimaging and laboratory results for Zika virus and other relevant infections. Definite cases had laboratory evidence of Zika virus infection; highly probable cases presented specific neuroimaging findings, and negative laboratory results for other congenital infections; moderately probable cases had specific imaging findings but other infections could not be ruled out; somewhat probable cases had imaging findings, but these were not reported in detail by the local teams; all other newborn babies were classified as discarded cases. Head circumference by gestational age was assessed with InterGrowth standards. First week mortality and history of rash were provided by the State medical teams. Between Nov 19, 2015, and Feb 27, 2015, investigations were completed for 1501 suspected cases reported to the Brazilian Ministry of Health, of whom 899 were discarded. Of the remainder 602 cases, 76 were definite, 54 highly probable, 181 moderately probable, and 291 somewhat probable of congenital Zika virus syndrome. Clinical, anthropometric, and survival differences were small among the four groups. Compared with these four groups, the 899 discarded cases had larger head circumferences (mean Z scores -1·54 vs -3·13, difference 1·58 [95% CI 1·45-1·72]); lower first-week mortality (14 per 1000 vs 51 per 1000; rate ratio 0·28 [95% CI 0·14-0·56]); and were less likely to have a history of rash during pregnancy (20·7% vs 61·4%, ratio 0·34 [95% CI 0·27-0·42]). Rashes in the third trimester of pregnancy were associated with brain abnormalities despite normal sized heads. One in five definite or probable cases presented head circumferences in the normal range (above -2 SD below the median of the InterGrowth standard) and for one third of definite and probable cases there was no history of a rash during pregnancy. The peak of the epidemic occurred in late November, 2015. Zika virus congenital syndrome is a new teratogenic disease. Because many definite or probable cases present normal head circumference values and their mothers do not report having a rash, screening criteria must be revised in order to detect all affected newborn babies. Brazilian Ministry of Health, Pan American Health Organization, and Wellcome Trust.

Neuroimaging endophenotypes in autism spectrum disorder.

Autism spectrum disorder (ASD) is a neurodevelopmental disorder that has a strong genetic basis, and is heterogeneous in its etiopathogenesis and clinical presentation. Neuroimaging studies, in concert with neuropathological and clinical research, have been instrumental in delineating trajectories of development in children with ASD. Structural neuroimaging has revealed ASD to be a disorder with general and regional brain enlargement, especially in the frontotemporal cortices, while functional neuroimaging studies have highlighted diminished connectivity, especially between frontal-posterior regions. The diverse and specific neuroimaging findings may represent potential neuroendophenotypes, and may offer opportunities to further understand the etiopathogenesis of ASD, predict treatment response, and lead to the development of new therapies.

Childhood central nervous system leukemia: historical perspectives, current therapy, and acute neurological sequelae

During the past three decades, improvements in the treatment of childhood leukemia have resulted in high cure rates, particularly for acute lymphoblastic leukemia (ALL). Unfortunately, successful therapy has come with a price, as significant morbidity can result from neurological affects which harm the brain and spinal cord. The expectation and hope is that chemotherapy, as a primary means of CNS therapy, will result in acceptable disease control with less CNS morbidity than has been observed with combinations of chemotherapy and radiotherapy over the past several decades. In this review we discuss the poignant, historical aspects of CNS leukemia therapy, outline current methods of systemic and CNS leukemia therapy, and present imaging findings we have encountered in childhood leukemia patients with a variety of acute neurological conditions. A major objective of our research is to understand the neuroimaging correlates of acute and chronic effects of cancer and therapy. Specific features related to CNS leukemia and associated short-term toxicities, both disease- and therapy-related, are emphasized in this review with the specific neuroimaging findings. Specific CNS findings are similarly important when treating acute myelogenous leukemia (AML), and details of leukemic involvement and toxicities are also presented in this entity. Despite contemporary treatment approaches which favor the use of chemotherapy (including intrathecal therapy) over radiotherapy in the treatment of CNS leukemia, children still occasionally experience morbid neurotoxicity. Standard neuroimaging is sufficient to identify a variety of neurotoxic sequelae in children, and often suggest specific etiologies. Specific neuroimaging findings frequently indicate a need to alter antileukemia therapy. It is important to appreciate that intrathecal and high doses of systemic chemotherapy are not innocuous and are associated with acute, specific, recognizable, and often serious neurological consequences.

Medulloblastoma or cerebellar dysplastic gangliocytoma (Lhermitte-Duclos disease)?

Dysplastic cerebellar gangliocytoma is a rare benign tumor associated with specific neuroimaging findings of abnormal laminated or folial pattern in the posterior fossa. Some authors thus proposed that it could be diagnosed by neuroimaging studies alone. We encountered a patient with medulloblastoma in which the neuroimaging findings mimicked those of dysplastic gangliocytoma. In patients with a posterior fossa tumor suggestive of a dysplastic gangliocytoma on neuroimaging studies, a pathologic confirmation is necessary.