Central tegmental tract hyperintensity: follow-up outcomes from a single-center study.

PURPOSE: To evaluate the follow-up outcomes of symmetrical central tegmental tract hyperintensity (CTTH) and discuss possible etiological factors involved. METHODS: Brain MRI scans of 7028 pediatric patients aged 0 to 18 years obtained between July 2015 and May 2020, were reviewed retrospectively for the presence of CTTH. Clinical data of the patients were retrieved from the hospital information system. Patients with follow-up MRI scans were evaluated separately. RESULTS: A total of 5113 patients meeting the study inclusion criteria were identified in whom the prevalence of CTTH was 4.02% (n = 206). Of the patients with CTTH, 40.3% (n = 83) were girls, and the median age was 19 months (range, 1-108). The most common MRI indication was seizures (40.3%, n = 83), and among those with a definitive diagnosis, epilepsy was the most prevalent etiology (7.8%, n = 16). 40.7% (n = 84) of the patients with CTTH had follow-up MRI scans. CTTH disappeared on follow-up in 28.6% (n = 24) of the patients. The median age at CTTH disappearance was 51.5 months, and the mean (± SD) time to CTTH disappearance was 31.50 (± 19.02) months. CONCLUSION: CTTH is a radiological finding commonly seen in early childhood but its clinical relevance has not been fully elucidated. While CTTH may be a transient phenomenon representing the maturation process, it may also be associated with a number of clinical conditions. Using a large patient series and follow-up MRI scans, our study shed light on the possible etiological factors of CTTH and its evolution over time.

Evaluation of the Differences in the MRI Findings Related to Primary and Secondary Adhesive Capsulitis.

Purpose: The aim of this study is to describe the radiological findings of adhesive capsulitis due to different etiological factors and reveal that different clues due to different etiological causes should be considered in the diagnosis of adhesive capsulitis. Methods: The study group comprised 24 primary and 22 supraspinatus tendon rupture-related adhesive capsulitis patients with 24 individuals without adhesive capsulitis and with normal shoulder magnetic resonance imaging. Independent samples t-test and one-way analysis of variance tests were used to compare the measurements between the groups. Paired sample t-test and Cohen's kappa statistic were used to determine inter-observer reliability. The chi-squared test was used to determine the relationships between groups and categorical variables. Study participants were evaluated both qualitatively and quantitatively. Quantitative measurements included the thickness of the humeral and glenoid segment of the joint capsule and the thickness of the anterior joint capsule and the fluid depth within the biceps tendon sheath. The qualitative assessments included the detection of any signal abnormality at the rotator interval, the grading of the humeral and glenoid segment of the joint capsule, and the detection of any signal abnormality in respect of the anterior joint capsule. Results: A statistically significant difference was found between the groups of patients with primary adhesive capsulitis and with supraspinatus tear-related adhesive capsulitis in terms of the thickness of the humeral segment of the joint capsule at the axillary recess level (AUC = .729) (P: .006). Furthermore, setting the cutoff value for the thickness of the humeral segment of the capsule to 4.6 mm allowed the differentiation of primary adhesive capsulitis and supraspinatus tear-related adhesive capsulitis with a sensitivity of 70.8% and a specificity of 87.3%. Conclusion: In conclusion, the humeral segment of the joint capsule thickness at the axillary recess was found to be affected by different degrees depending on whether it is a primary or supraspinatus tear-related adhesive capsulitis.

Challenging treatment of parameningeal rhabdomyosarcoma with leptomeningeal metastasis: Case report and review of literature.

Parameningeal rhabdomyosarcomas (PM RMSs) are rarely seen childhood tumors. Their treatment might be challenging and prognosis is poor compared to other head and neck RMS. Here we report a PM RMS presenting with leptomeningeal seeding metastasis a year after diagnosis. A five-year-old girl presented with an enlarging mass protruding from the right ear and right facial paralysis. Magnetic resonance imaging (MRI) revealed a large mass extending from right external auditory canal to the temporal lobe, pterygoid fossa and nasopharynx with an intracranial component indenting the right temporal lobe and extending into the right cavernous sinus. Trucut biopsy revealed embryonal rhabdomyosarcoma. Cerebrospinal fluid (CSF) cytology was negative for malignant cells. Chemotherapy was started since it was found unresectable. At second week of chemotherapy, radiotherapy was applied to primary tumor location with intensity-modulated radiation therapy (IMRT) technique in 1.8 Gy fractions to total dose of 50.4 Gy. At week 27, MRI showed significant response. At week 36, the patient presented with vomiting and tendency to sleep. MRI was found to be compatible with meningitis and antibacterial therapy was started. At week 39, chemotherapy was stopped. But MRI performed one month later revealed linear contrast enhancements around the spinal cord compatible with leptomeningeal metastases. Chemotherapy and craniospinal irradiation were applied. But the patient did not improve and received palliative treatment. Six months after the completion of radiotherapy the patient died. Treatment of parameningeal rhabdomyosarcomas require multidisciplinary approach including surgery, radiotherapy, and chemotherapy. Prognosis is poor for patients with leptomeningeal spread.

Evaluation of ocular blood flow by Doppler ultrasound in patients with essential blepharospasm.

PURPOSE: Benign essential blepharospasm (EB) is a focal facial dyskinesia that occurs with the involuntary contraction of muscles around the eyes. In the literature, studies on blepharospasm focus on elucidating the pathophysiology of this condition in the brain. To the best of our knowledge, there is no research evaluating the orbital imaging findings of patients with EB. Therefore, the current study aimed to determine whether there was any change in the blood supply of muscles around the eye or ocular blood flow in patients with blepharospasm and investigate flow changes that may be caused by spasm. METHODS: Thirty patients with EB and 30 controls were included in the study. Orbital Doppler ultrasound was performed in all cases to measure ophthalmic and temporal artery peak systolic velocity and end diastolic velocity and calculate resistive index values. Superior ophthalmic vein blood flow velocity (SOVBFV) was also measured. RESULTS: There was no significant difference between the groups in terms of age and gender distribution (P = 0.345 and 0.870, respectively). SOVBFV was found to be significantly higher in the EB group (P = 0.001). No significant difference was observed in the remaining investigated parameters. CONCLUSIONS: In conclusion, our study suggested that ocular spasm in patients with EB had no effect on blood flow other than SOVBFV. When SOVBFV was compared between the EB and control groups, it was found to be increased in the EB group. We consider that this statistical difference may be clinically and pathophysiologically significant.

ATYPICAL MRI FINDINGS IN CEREBRAL ADRENOLEUKODYSTROPHY: A CASE REPORT.

Adrenoleukodystrophy is a rare X-linked hereditary disease that results in accumulation of very-long-chain fatty acids in all body tissues, thus causing demyelination of the white matter. Magnetic resonance imaging (MRI) is a reliable radiological modality to demonstrate the extension of brain lesions and severity of the disease. In the classic form, the parieto-occipital white matter is affected. Besides, atypical MRI findings such as primary frontal lobe involvement are rarely described. We report a case of adrenoleukodystrophy presenting with rare MRI findings such as bilateral symmetric frontal lobe white matter changes suggesting anterior predominance.

Acute transverse myelitis after inactivated COVID-19 vaccine.

Vaccines against the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) have been rapidly developed to prevent coronavirus disease 2019 (COVID-19) pandemic. There is increasing safety concerns regarding COVID-19 vaccines. We report a 78-year old woman who was presented with tetraparesis, paresthesias of bilateral upper extremities, and urinary retention of one-day duration. Three weeks before these symptoms, she was vaccinated with CoronaVAC vaccine (Sinovac Life Sciences, China). Spine magnetic resonance imaging showed longitudinally extensive transverse myelitis (TM) from the C1 to the T3 spinal cord segment. An extensive diagnostic workup was performed to exclude other possible causes of TM. We suggest that longitudinally extensive TM may be associated with COVID-19 vaccination in this case. To the best of our knowledge, this is the first report of longitudinally extensive TM developing after CoronaVac vaccination. Clinicians should be aware of neurological symptoms after vaccination of COVID-19.

The role of lateralisation and sex on insular cortex: 3D volumetric analysis

Background/aim: The insula has attracted the attention of many neuroimaging studies because of its key role between brain structures. However, the number of studies investigating the effect of sex and laterality on insular volume is insufficient. The aim of this study was to investigate the differences in insular volume between sexes and hemispheres. Materials and methods: A total of 47 healthy participants [24 males (20.08 ± 1.44 years) and 23 females (19.57 ± 0.90 years)] underwent magnetic resonance imaging (MRI). Imaging was performed using the 3T MRI scanner. The insular volume was measured using the Individual Brain Atlases using Statistical Parametric Mapping (IBASPM); total intracranial, cerebral, grey and white matter volumes were measured using volBrain. Results: The right insular volume was significantly higher than the left insular volume in the participants, and the left cerebral volume was significantly higher than the right cerebral volume (p < 0.05). The total brain, total cerebral, left and right insular, and cerebral volumes were significantly larger in males than in females (p < 0.001). Also, the ratios of the insular volume to total brain and cerebral volume were significantly higher in males than in females (p < 0.05). Conclusion: This study shows that insular volume differs with laterality and sex. This outcome may be explained by the anatomical relationship between the insula and behavioural functions and emotional reactions and the fact that the right side of the brain is best at expressive and creative tasks.

Contribution of the Mesencephalon Indices to Differential Diagnosis of Parkinsonian Disorders.

BACKGROUND AND PURPOSE: This study aimed to assess the sensitivity and specificity of brain stem morphologic changes to differentiate the progressive supranuclear palsy (PSP) from Parkinson disease (PD) and multiple system atrophy (MSA), by single and combined magnetic resonance imaging (MRI) measurements. MATERIALS AND METHODS: Peduncle angle (PA), pons area (P), mesencephalon area (M), middle cerebellar peduncles (MCPs), and superior cerebellar peduncles (SCPs) were measured in 14 PSP, 43 PD, 8 MSA patients, and 45 age-matched control participants on T1-weighted MRI. Neurologists clinically diagnosed all patients. Additionally, P/M ratio, MCPs/SCPs ratio, the previously defined Magnetic Resonance Parkinsonism Index, MRPI: (P/M) · (MCP/SCP), and also the Akdeniz Index (AKI) that we termed were calculated, AKI: (P/M) · (PA/180). Two blinded radiologists evaluated all MR images and inter-/intraobserver variations were measured. RESULTS: Both M and SCPs were significantly lower and P/M, MCPs/SCPs, and PA were significantly higher in PSP patients than the other groups (P < .001). This significance was related to patients with PSP and PD. But all single measurements showed some overlapping values. Therefore, previously defined MRPI was calculated and shown to distinguish patients (negative predictive values: 92%, sensitivity: 78%, specificity: 82%). In this study, interobserver correlation (0.68) was found low for MRPI. Therefore, we identified a more practical index: the Akdeniz Index, which has same diagnostic power with MRPI and higher interobserver correlation (0.91). CONCLUSION: The Akdeniz Index identified in our study is a practical index with high diagnostic power and can reinforce radiological distinguishing of PSP and PD, which are clinically difficult to distinguish.

Pediatric Tonsillar Synovial Sarcoma- Very Rare Localization: A Case Report and Review of the Literature.

Tonsillar synovial sarcoma is an extremely rare entity and only 9 adult patients have been reported up to now. Here, we describe the first pediatric tonsillar synovial sarcoma of the literature in a patient who presented with a 2-month history of dysphagia and snoring. Clinical and radiological examinations showed that the tumor arose from the right palatine tonsil and narrowed the parapharyngeal space. An incisional biopsy from the palatine tonsil revealed the diagnosis of synovial sarcoma. The patient has underwent total tonsillectomy and received radiotherapy and chemotherapy because of the positive surgical margins. The patient is clinically in good condition and free of tumor 30 months after the initial diagnosis. We achieved a long-term complete remission with a combination of surgery, radiotherapy and chemotherapy in our case. Tonsillar synovial sarcoma should be kept in mind while dealing with tonsillar masses. We can conclude that a multidisciplinary approach is warranted while treating synovial sarcoma with this localization.

An infant with intradural extramedullary synovial sarcoma: the youngest case in the literature.

Yalçin K, Tüysüz G, Kazan S, Gürer EI, Karaali K, Küpesiz A, Güler E. An infant with intradural extramedullary synovial sarcoma: the youngest case in the literature. Turk J Pediatr 2019; 61: 765-770. Spinal cord involvement of synovial sarcoma is extremely rare. So far only two cases have been reported. Herein we describe the youngest case in the literature. She is 14-month-old and first presented with difficulty in walking ongoing for a week. Imagining showed a spinal cord mass at C5-T3 levels. The patient had gone under Decompressive surgery and histopathologic examination of the specimen revealed the presence of synovial sarcoma. Although the tumor regressed after chemotherapy, she was lost due to viral pneumonia. Synovial sarcoma should be kept in mind while evaluating spinal tumors even in infantile group.

Disseminated Cryptococcosis With Severe Increased Intracranial Pressure Complicated With Cranial Nerve Palsy in a Child.

Cryptococcosis is less common in children than in adults but remains an important cause of pneumonia and meningoencephalitis in both immunocompromised and immunocompetent patients. Intracranial hypertension commonly complicates cryptococcal meningitis and may cause significant visual and neurologic morbidity and mortality. Early and aggressive management of intracranial hypertension in accordance with established guidelines reduces the risk of long-term complications and death. In this case report, we present a 12-year-old girl with cryptococcal meningitis, pneumonitis and dermatitis complicated with cranial nerve palsy and loss of vision. She was successfully treated with serial cerebrospinal fluid drainage, antifungal and interferon gamma therapy.

The interaction between breathing and swallowing in amyotrophic lateral sclerosis.

The aim of the study is to determine the association between respiratory swallow patterns in amyotrophic lateral sclerosis (ALS) patients. Furthermore, it aims to clarify the role of the dysphagia limit in defining the relationship between swallowing disorders and respiratory disorders. Functional rating scales were used to describe swallowing and respiratory function. Swallowing was observed using the dysphagia limit. Dysphagia limit is the volume at which a second or more swallows are required to swallow the whole bolus. Laryngeal and chest movement sensors, pulmonary function tests, submental, and diaphragm electromyography activity were used to evaluate the relationship between swallowing and respiratory phase. Of the 27 patients included in the study, 14 were dysphagic and 13 were non-dysphagic. Tests showed normal respiratory function in 11 of the non-dysphagic patients and 3 of the dysphagic patients. There was a high correlation between the dysphagia limit and Amyotrophic Lateral Sclerosis Functional Rating Scale swallowing parameters. Non-dysphagic patients were able to swallow during inspiration but only six patients in the dysphagic group were able to swallow during inspiration. The occurrence of dysphagia in ALS is related to piecemeal deglutition and respiration consistency during swallowing. Detecting the timing of disturbances in the relationship between swallowing and respiration may be a way of identifying dysphagia. Dysphagia limit may be a useful, complementary test for assessing swallowing disturbances in amyotrophic lateral sclerosis.

Central Neurogenic Hyperventilation Related to Post-Hypoxic Thalamic Lesion in a Child.

Central neurogenic hyperventilation (CNH) is a rare clinical condition, whose mechanism is still unclear. Here, we report a 3-year-old male patient, who had bilateral thalamic, putaminal and globus pallideal infarction resulted in CNH without brainstem involvement. This case may illustrate a possible role for the thalamus in regulating ventilation.

UNUSUAL CLINICAL CASES THAT MIMIC ACUTE DISSEMINATED ENCEPHALOMYELITIS.

Acute disseminated encephalomyelitis (ADEM) is an immune-mediated monophasic inflammatory demyelinating disorder of the central nervous system which poses a diagnostic challenge. We report on six cases of different etiologies that mimicked the clinical and radiologic findings of ADEM. The cases were collected from four different reference hospitals in Turkey. The same radiologist from the Akdeniz University Faculty of Medicine examined the magnetic resonance images of all patients. Three (50%) patients had antecedent infections. Initial symptoms of the patients were as follows: fever in 50%, altered consciousness in 33.3% and convulsions in 16.7% of patients. Neurologic examination showed long tract signs in 83.3%, ataxia in 50% and altered consciousness in 50% of patients. Cerebrospinal fluid examination revealed lymphocytic pleocytosis only in case 6. Four patients received steroid pulse therapy and one of these initially underwent intravenous immunoglobulin therapy. The patients' definitive diagnoses were as follows: paraspinal neuroblastoma-associated paraneoplastic syndrome; histiocytic sarcoma; mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes; and cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy in one patient each, while two patients had hemophagocytic syndrome. The present case series demonstrated difficulties in diagnosing ADEM while revealing extremely rare disorders that mimic ADEM radiologically and clinically.

Magnetic resonance imaging findings of two sisters with Van der Knaap leukoencephalopathy.

Megalencephalic leukoencephalopathy with subcortical cysts, or Van der Knaap leukoencephalopathy, is a rare disease which is characterised by macrocephaly and neurological disorders with autosomal recessive inheritance. Magnetic resonance imaging is very helpful for determining distinctive findings and distinguishing other diseases. We present the radiological findings of two sisters (aged 6 and 10 years) diagnosed with Van der Knaap leukoencephalopathy.

Perinatal Diagnostic Approach to Fetal Skeletal Dysplasias: Six Years Experience of a Tertiary Center.

Skeletal dysplasias (SDs) constitute a group of heterogeneous disorders affecting growth morphology of the chondro-osseous tissues. Prenatal diagnosis of SD is a considerable clinical challenge due to phenotypic variability. We performed a retrospective analysis of the fetal autopsies series conducted between January 2006 and December 2012 at our center. SD was detected in 54 (10%) out of 542 fetal autopsy cases which included; 11.1% thanatophoric dysplasia (n = 6), 7.4% achondroplasia (n = 4), 3.7% osteogenesis imperfect (n = 2), 1.9% Jarcho-Levin Syndrome (n = 1), 1.9% arthrogryposis (n = 1), 1.9% Dyggve-Melchior-Clausen syndrome (n = 1), 72.1% of dysostosis cases (n = 39). All SD cases were diagnosed by ultrasonography. In 20 of the cases, amniocentesis was performed, 4 cases underwent molecular genetic analyses. Antenatal identification of dysplasia is important in the management of pregnancy and in genetic counseling. Our data analysis showed that SD is usually detected clinically after the 20th gestational week. Genetic analyses for SD may provide early diagnosis and management.

Epidural capillary hemangioma: A review of the literature.

Hemangiomas in the spinal epidural area are very rare lesions, and most of these lesions are of the cavernous type. Only seven cases of capillary hemangiomas have been reported in the English literature, and all of these cases occurred in adulthood. Here, we report on a 17-month-old girl who presented with an inability to walk. MRI revealed an epidural mass, which was diagnosed as an epidural capillary hemangioma in the thoracic region. To our best knowledge, this case is the first epidural capillary hemangioma case to occur in childhood that has been reported.

Venous drainage patterns in carotid cavernous fistulas.

Purpose. The carotid-cavernous fistula (CCF) is an abnormal arteriovenous communication and its drainage pathways may affect the clinic presentation and change treatment approach. We evaluated drainage patterns of CCFs by digital subtraction angiography (DSA) and categorized drainage pathways according to their types and etiology. Materials and Methods. Venous drainage patterns of 13 CCFs from 10 subjects were studied and categorized as anterior, posterior, superior, inferior, and contralateral on DSA. Drainage patterns were correlated to types and etiology of CCFs. Diagnosis of CCFs was first made by noninvasive imaging techniques. Results. On DSA, traumatic CCFs were usually high flow, direct type while spontaneous CCFs were usually slow flow, indirect type. Bilaterality and mixed types were observed among the indirect spontaneous CCFs. In all CCFs, anterior and inferior drainages were the most common. Contrary to the literature, posterior and superior drainages were noted only in high flow and long standing direct fistulas. Contralateral drainage was not observed in all, supporting plausible compartmentalization of cavernous sinuses. Conclusion. Types, etiology, and duration of the CCFs may affect their drainage patterns. DSA is valuable for categorization of CCFs and verification of drainage patterns. Drainage pathways may affect the clinic presentation and also change treatment approach.

A late-onset seizure in a child due to intracranial needle.

Placing of sewing needles in the brain through the anterior fontanel is a rare entity. There are very few cases reported in literature. Most of them were asymptomatic, but some of them presented with seizure. We report here a 14-year-old boy, who was admitted to the Pediatric Neurology Department with a history of generalized tonic-clonic seizures due to sewing needle located in the frontal lobe.