Neural correlates of distorted body images in adolescent girls with anorexia nervosa: How is it different from major depressive disorder?

Body image disturbance is closely linked to eating disorders including anorexia nervosa (AN). Distorted body image perception, dissatisfaction and preoccupation with weight and shape are often key factors in the development and maintenance of these disorders. Although the pathophysiological mechanism of body image disorder is not yet fully understood, aberrant biological processes may interfere with perceptive, cognitive and emotional aspects of body image. This study focuses on the neurobiological aspects of body image disturbance. The sample consisted of 12 adolescent girls diagnosed with AN, nine girls with major depressive disorder (MDD) and 10 without psychiatric diagnoses (HC, the healthy control group). We applied a block-design task in functional magnetic resonance imaging using participants' original and distorted overweight and underweight images. After imaging, the participants scored the images for resemblance, satisfaction and anxiety levels. The findings of this study demonstrate that overweight images elicited dissatisfaction and increased occipitotemporal activations across all participants. However, no difference was found between the groups. Furthermore, the MDD and HC groups showed increased activations in the prefrontal cortex and insula in response to underweight images compared to their original counterparts, whereas the AN group exhibited increased activations in the parietal cortex, cingulate gyrus and parahippocampal cortex in response to the same stimuli.

Flocculus Herniation into the Internal Acoustic Canal in Incomplete Partition Type I: A Case Report.

In this study, we present the first case with cerebellar herniation into the internal acoustic canal in incomplete partition type I anomaly. Cerebellar herniation into the internal acoustic canal is very rare with only a few cases reported in the literature. Although it is a rare clinical situation, cerebellar herniation into the internal acoustic canal may be seen in patients with incomplete partition type I. We presented magnetic resonance imaging findings of a 3-year-old girl with a history of meningitis, middle ear effusion, and bilateral congenital sensorineural hearing loss. Magnetic resonance imaging showed bilateral incomplete partition type I malformation and an additional flocculus herniation into the right internal acoustic canal. In the presented case, predisposition to cerebrospinal fluid leak in incomplete partition type I anomaly may be the reason for cerebellar herniation into internal acoustic canal. Also, possible increased intracranial pressure due to meningitis may be a contributing factor.

The Effects of Respiratory Muscle Training on Resting-State Brain Activity and Thoracic Mobility in Healthy Subjects: A Randomized Controlled Trial.

BACKGROUND: Although inspiratory muscle training (IMT) is an effective intervention for improving breath perception, brain mechanisms have not been studied yet. PURPOSE: To examine the effects of IMT on insula and default mode network (DMN) using resting-state functional MRI (RS-fMRI). STUDY TYPE: Prospective. POPULATION: A total of 26 healthy participants were randomly assigned to two groups as IMT group (n = 14) and sham IMT groups (n = 12). FIELD STRENGTH/SEQUENCE: A 3-T, three-dimensional T2* gradient-echo echo planar imaging sequence for RS-fMRI was obtained. ASSESSMENT: The intervention group received IMT at 60% and sham group received at 15% of maximal inspiratory pressure (MIP) for 8 weeks. Pulmonary and respiratory muscle function, and breathing patterns were measured. Groups underwent RS-fMRI before and after the treatment. STATISTICAL TESTS: Statistical tests were two-tailed P < 0.05 was considered statistically significant. Student's t test was used to compare the groups. One-sample t-test for each group was used to reveal pattern of functional connectivity. A statistical threshold of P < 0.001 uncorrected value was set at voxel level. We used False discovery rate (FDR)-corrected P < 0.05 cluster level. RESULTS: The IMT group showed more prominent alterations in insula and DMN connectivity than sham group. The MIP was significantly different after IMT. Respiratory rate (P = 0.344), inspiratory time (P = 0.222), expiratory time (P = 1.000), and inspiratory time/total breath time (P = 0.572) of respiratory patterns showed no significant change after IMT. All DMN components showed decreased, while insula showed increased activation significantly. DATA CONCLUSION: Differences in brain activity and connectivity may reflect improved ventilatory perception with IMT with a possible role in regulating breathing pattern by processing interoceptive signals. EVIDENCE LEVEL: 2 TECHNICAL EFFICACY: Stage 4.

Craniocervical junction involvement in inflammatory arthritis: a single-center radiologic study.

Background/aim: Craniocervical junction (CCJ) can be involved in inflammatory arthritis. We aimed to define types of CCJ involvement in rheumatoid arthritis (RA), spondyloarthritis (SpA), and psoriatic arthritis (PsA) and compare them with patients without inflammatory arthritides. Materials and methods: In this retrospective analysis, cervical CT or MRIs of patients with RA, SpA, or PsA, taken for any reason between 2010 and 2020, according to ICD-10 codes, were scanned. Demographic data of the patients were recorded. CCJ involvements (atlantoaxial, vertical, or subaxial subluxation, odontoid process involvement) were reevaluated by an experienced radiologist. The control group consisted of consecutive patients without inflammatory arthritis. Results: Exactly 459 patients (204 RA, 200 SpA, and 55 PsA) and 78 patients in the control group were included in the study. CCJ involvement was detected in 101 (49.5%) RA, 53 (26.5%) SpA, 10 (18.2%) PsA, and 4 patients (5.1%) in the control group (p < 0.001). The odontoid process was one of the main targets, especially in RA patients (69 (33.8%)), which was significantly higher than in the SpA, PsA, and control groups. Although vertical subluxation (VS) was numerically higher in the RA and SpA groups compared to the control group, VS-related brainstem compression was relatively uncommon: 6 (2.9%) in RA, 1 (0.5%) in AS, and none in the PsA and control groups. Conclusion: CCJ involvement can often be detected in patients with inflammatory arthritis, especially in RA and SpA patients. The odontoid process is the main target of inflammation.

A splice site mutation in the TSEN2 causes a new syndrome with craniofacial and central nervous system malformations, and atypical hemolytic uremic syndrome.

Recessive mutations in the genes encoding the four subunits of the tRNA splicing endonuclease complex (TSEN54, TSEN34, TSEN15, and TSEN2) cause various forms of pontocerebellar hypoplasia, a disorder characterized by hypoplasia of the cerebellum and the pons, microcephaly, dysmorphisms, and other variable clinical features. Here, we report an intronic recessive founder variant in the gene TSEN2 that results in abnormal splicing of the mRNA of this gene, in six individuals from four consanguineous families affected with microcephaly, multiple craniofacial malformations, radiological abnormalities of the central nervous system, and cognitive retardation of variable severity. Remarkably, unlike patients with previously described mutations in the components of the TSEN complex, all the individuals that we report developed atypical hemolytic uremic syndrome (aHUS) with thrombotic microangiopathy, microangiopathic hemolytic anemia, thrombocytopenia, proteinuria, severe hypertension, and end-stage kidney disease (ESKD) early in life. Bulk RNA sequencing of peripheral blood cells of four affected individuals revealed abnormal tRNA transcripts, indicating an alteration of the tRNA biogenesis. Morpholino-mediated skipping of exon 10 of tsen2 in zebrafish produced phenotypes similar to human patients. Thus, we have identified a novel syndrome accompanied by aHUS suggesting the existence of a link between tRNA biology and vascular endothelium homeostasis, which we propose to name with the acronym TRACK syndrome (TSEN2 Related Atypical hemolytic uremic syndrome, Craniofacial malformations, Kidney failure).

Does Combined Fractionated Stereotactic Radiotherapy and Immunotherapy Change the Outcome of Recurrent High-Grade Gliomas?

Background Radiotherapy (RT) with immune checkpoint inhibitors (ICI) has yielded good responses in many cancers. We aimed to report the results of combined fractionated stereotactic radiotherapy (FSRT) and ICI in patients with recurrent high-grade glioma. Methodology Patients were treated with FSRT and nivolumab which were continued until progression or toxicity. The Response Assessment in Neuro-oncology and Immunotherapy Response Assessment in Neuro-oncology criteria were used to assess treatment response on magnetic resonance imaging. Treatment-related toxicity was noted in all patients. Results A total of eight patients were included. Recurrence was detected after a median of 5.8 months following the first RT, all in the treatment field. FSRT (3 × 8 Gy) was applied with neoadjuvant, concurrent, and adjuvant nivolumab. After a median follow-up of 21.3 months from diagnosis and 12.6 months from recurrence, one patient was alive and seven succumbed to the disease. The median overall survival was 20.9 months after diagnosis and 12.9 months after recurrence. The median progression-free interval was 2.3 months after FSRT. The local control (LC) rate was 62.5% with a median local recurrence-free survival of nine months. Progression in other regions of the brain was observed in four patients with a median progression-free survival of 2.1 months. Acute toxicity was not observed. ICI-related grade 3 late pneumonitis was observed in two patients, and grade 1 late thyroid toxicity in two patients. One patient with pneumonitis also developed osteoporosis and radiation necrosis. Conclusions A high LC rate was achieved with concurrent FSRT and ICI with a severe late toxicity rate of 25%. This combination can be an option in recurrent high-grade gliomas.

Cortical connectivity in the face of congenital structural changes-A case of homozygous LAMC3 mutation.

The homozygous LAMC3 gene mutation is associated with severe bilateral smoothening and thickening of the lateral occipital cortex . Despite this and further significant changes in gray matter structure, a patient harboring this mutation exhibited a range of remarkably intact perceptual abilities . One possible explanation of this perceptual sparing could be that the white matter structural integrity and functional connectivity in relevant pathways remained intact. To test this idea, we used diffusion tensor and functional magnetic resonance imaging to investigate functional connectivity in resting-state networks in major structural pathways involved in object perception and visual attention and corresponding microstructural integrity in a patient with homozygous LAMC3 mutation and sex, age, education, and socioeconomically matched healthy control group. White matter microstructural integrity results indicated widespread disruptions in both intra- and interhemispheric structural connections except inferior longitudinal fasciculus. With a few exceptions, the functional connectivity between the patient's adjacent gray matter regions of major white matter tracts of interest was conserved. In addition, functional localizers for face, object, and place areas showed similar results with a representative control, providing an explanation for the patient's intact face, place, and object recognition abilities. To generalize this finding, we also compared functional connectivity between early visual areas and face, place, and object category-selective areas, and we found that the functional connectivity of the patient was not different from the control group. Overall, our results provided complementary information about the effects of LAMC3 gene mutation on the human brain including intact temporo-occipital structural and functional connectivity that are compatible with preserved perceptual abilities.

International consensus classification of hippocampal sclerosis and etiologic diversity in children with temporal lobectomy.

INTRODUCTION: The distribution of hippocampal sclerosis (HS) subtypes, according to the classification of the International League Against Epilepsy (ILAE), has been reported mainly in adult patients. We aimed to review the pathological findings in children who had anterior temporal lobectomy accompanied with amygdalohippocampectomy, in view of the current classification, and evaluate postsurgical outcome with respect to HS subtypes in childhood. METHODS: Seventy children who underwent temporal resections for treatment of medically refractory epilepsy, with a minimum follow-up of 2 years, were included; the surgical hippocampus specimens were re-evaluated under the HS ILAE classification. RESULTS: Neuropathological evaluations revealed HS type 1 in 38 patients (54.3%), HS type 2 in 2 (2.8%), HS type 3 in 21 patients (30%), and no HS in 9 patients (12.9%). Of 70 patients, 23 (32.9%) had dual pathology, and the most common pattern was HS type 3 with low-grade epilepsy-associated brain tumors (LEAT). The distribution of HS types with respect to age revealed that HS type 3 and no HS subgroups had significantly more patients younger than 12 years, compared with those of HS type 1 (90.5%, 77.8% vs 47.4%, respectively). History of febrile seizures was higher in HS type 1. Prolonged/recurrent febrile seizures were most common in patients 12 years and older, whereas LEAT was the most common etiology in patients under 12 years of age (p < 0.001). Patients with HS type 1 had longer duration of epilepsy and an older age at the time of surgery compared with patients with HS type 3 and no HS (p: 0.031, p: 0.007). At final visit, 74.3% of the patients were seizure-free. Seizure outcome showed no significant difference between pathological subtypes. CONCLUSIONS: Our study presents the distribution of HS ILAE subtypes in an exclusively pediatric series along with long-term seizure outcome. The study reveals that the leading pathological HS subgroup in children is HS type 1, similar with adult series. Hippocampal sclerosis type 2 is significantly less in children compared with adults; however, HS type 3 emerges as the second most predominant group because of dual pathology, particularly LEAT. Further studies are required regarding clinicopathological features of isolated HS in pediatric cohort. Seizure-free outcome was favorable and similar in all HS types in children. The proportion of HS types may be better defined in pediatric patients with temporal resections, as the current HS ILAE classification becomes more widely used, and may help reveal the surgical and cognitive outcome with respect to HS types.

Long-term effects of vagus nerve stimulation in refractory pediatric epilepsy: A single-center experience.

INTRODUCTION: Vagus nerve stimulation (VNS) has been used as an adjunctive therapy for both children and adults with refractory epilepsy, over the last two decades. In this study, we aimed to evaluate the long-term effects and tolerability of VNS in the pediatric drug-resistant epilepsy (DRE) and to identify the predictive factors for responsiveness to VNS. METHODS: We retrospectively reviewed the medical records of pediatric patients who underwent VNS implantation between 1997 and 2018. Patients with ≥50% reduction of seizure frequency compared with the baseline were defined as "responders". The clinical characteristics of responders and nonresponders were compared. RESULTS: A total of 58 children (male/female: 40/18) with a mean follow-up duration of 5.7 years (3 months to 20 years) were included. The mean age at implantation was 12.4 years (4.5 to 18.5 years). Approximately half (45%) of our patients were responders, including 3 patients (5.8%) who achieved seizure freedom during follow-up. The age of seizure-onset, duration of epilepsy, age at implantation, and etiologies of epilepsy showed no significant difference between responders and nonresponders. Responders were more likely to have focal or multifocal epileptiform discharges (63%) on interictal electroencephalogram (EEG), when compared to nonresponders (36%) (p = .07). Vocal disturbances and paresthesias were the most common side effects, and in two patients, VNS was removed because of local reaction. CONCLUSION: Our series had a diverse etiological profile and patients with transition to adult care. Long-term follow-up showed that VNS is an effective and well-tolerated treatment modality for refractory childhood onset epilepsy. Age at implantation, duration of epilepsy and underlying etiology are not found to be predictors of responsiveness to VNS. Higher response rates were observed for a subset of patients with focal epileptiform discharges.

Combination of Paclitaxel and R-flurbiprofen loaded PLGA nanoparticles suppresses glioblastoma growth on systemic administration.

Malignant gliomas are highly lethal. Delivering chemotherapeutic drugs to the brain in sufficient concentration is the major limitation in their treatment due to the blood-brain barrier (BBB). Drug delivery systems may overcome this limitation and can improve the transportation through the BBB. Paclitaxel is an antimicrotubule agent with effective anticancer activity but limited BBB permeability. R-Flurbiprofen is a nonsteroidal antienflammatory drug and has potential anticancer activity. Accordingly, we designed an approach combining R-flurbiprofen and paclitaxel and positively-charged chitosan-modified poly-lactide-co-glycolic acid (PLGA) nanoparticles (NPs) and to transport them to glioma tissue. NPs were characterized and, cytotoxicity and cellular uptake studies were carried out in vitro. The in vivo efficacy of the combination and formulations were evaluated using a rat RG2 glioma tumor model. Polyethylene glycol (PEG) modified and chitosan-coated PLGA NPs demonstrated efficient cytotoxic activity and were internalized by the tumor cells in RG2 cell culture. In vivo studies showed that the chitosan-coated and PEGylated NPs loaded with paclitaxel and R-flurbiprofen exhibited significantly higher therapeutic activity against glioma. In conclusion, PLGA NPs can efficiently carry their payloads to glioma tissue and the combined use of anticancer and anti-inflammatory drugs may exert additional anti-tumor activity.

Paediatric Behçet's disease with sinus venous thrombosis: experience from three centres in Turkey.

OBJECTIVES: To report our experiences of the juvenile Behçet's disease (BD) patients with cerebral venous sinus thrombosis (CVST) and to review previous studies reporting the clinical characteristics and outcomes of juvenile BD with CVST. METHODS: Clinical characteristics and outcomes of paediatric patients with CVST who met the Paediatric Behçet's Disease (PEDBD) classification criteria for juvenile BD from 3 referral centres in Turkey were reviewed retrospectively. A systematic review of literature of all published data was conducted. RESULTS: The study group consisted of 12 juvenile BD patients with CVST. At the time of CVST diagnosis, the most common symptom was headache (100%), followed by vomiting (25%), blurred vision (16.7%), and disturbances in eye movements (16.7%). Six (50%) patients presented with CVST. Transverse sinus was the most frequently affected sinus (9/12, 75%) followed by superior sagittal sinus. The mean (±2SD) BDCAF at the CVST diagnosis was 6 (±3.8). Four children (33.3%) had another venous thrombosis apart from CVST. All patients received pulse methylprednisolone for three consecutive days continued with oral prednisolone. Steroid treatment was tapered and discontinued minimum in six months. Eleven patients received azathioprine concomitant to steroid treatment at the time of CVST. All the patients received anticoagulant therapy concomitantly. Only one patient who did not receive azathioprine relapsed. Median follow-up period was 4 years (IQR: 2-5.4). In the literature review, we identified nine articles, describing 35 pediatric CVST patients associated with BD. Thirty patients achieved remission, while five patients had residual neurologic deficit. CONCLUSIONS: Neuroimaging is very important in the diagnosis of NBD. We suggest that treatment with immunosuppressants and steroid treatment is essential to decrease the adverse events of corticosteroids in the pediatric population and decrease relapses. Further multicenter studies with prospective follow-up may guide us in better management of these patients.

Epilepsy in neurofibromatosis type 1: Diffuse cerebral dysfunction?

INTRODUCTION: Neurofibromatosis type 1 (NF1) is accompanied by epileptic seizures in 4-7% of patients. We examined clinical, electrophysiological, and radiological features associated with epilepsy in our NF1 series in order to identify risk factors. METHODS: We reviewed data of 641 pediatric patients with NF1 diagnosis according to National Institutes of Health (NIH) criteria in Hacettepe University records from January 2008-August 2018. Demographic features, NF1-related clinical and imaging characteristics, age at onset of epilepsy, seizure semiology, and frequency, electroencephalogram (EEG) findings, and response to treatment were noted. RESULTS: Twenty-six patients with NF1, 15 male, 11 female, had epilepsy. Age at seizure onset was 6 months to 13 years. Seizure semiology was focal with impaired awareness (n = 9, 34%), focal aware motor (n = 2, 8%), focal to bilateral tonic-clonic (n = 3, 12%), generalized tonic-clonic (n = 7, 28%), absence (n = 3, 12%), infantile spasms (n = 1), and unclassified type (n = 1). None had a history of status epilepticus. The EEG findings were normal for age in ten patients (38%). Others had focal (n = 8, 30%), generalized (n = 7, 27%), or multifocal (n = 1, 4%) discharges. On brain magnetic resonance imaging (MRI) signal intensity changes typical for NF1 (neurofibromatosis bright objects, NBOs) were the most common finding (80%), followed by normal MRI (20%). There was no relation between the localization of NBOs and discharges on EEG. Seventeen patients (65%) were seizure-free at the time of the study; 11 of them still under medication including four on multiple antiepileptic drugs. The rate of learning problems and NBO were significantly higher in patients with NF1 with epilepsy compared to those without. DISCUSSION: Epilepsy in NF1 is associated with relatively infrequent seizures and good response to treatment. Learning disorders are markedly frequent in this group, irrespective of the severity of epilepsy. The absence of correlation between the localizations of epileptiform discharges and lesions on MRI support the role of cellular or synaptic mechanisms rather than structural causes in the pathogenesis of epilepsy.

Farnesylthiosalicylic acid-loaded lipid-polyethylene glycol-polymer hybrid nanoparticles for treatment of glioblastoma.

OBJECTIVES: We aimed to develop lipid-polyethylene glycol (PEG)-polymer hybrid nanoparticles, which have high affinity to tumour tissue with active ingredient, a new generation antineoplastic drug, farnesylthiosalicylic acid (FTA) for treatment of glioblastoma. METHOD: Farnesylthiosalicylic acid-loaded poly(lactic-co-glycolic acid)-1,2 distearoyl-glycerol-3-phospho-ethanolamine-N [methoxy (PEG)-2000] ammonium salt (PLGA-DSPE-PEG) with or without 1,2-dioleoyl-3-trimethylammonium-propane (DOTAP) hybrid nanoparticles has been prepared and evaluated for in-vitro characterization. Cytotoxicity of FTA-loaded nanoparticles along with its efficacy on rat glioma-2 (RG2) cells was also evaluated both in vitro (in comparison with non-malignant cell line, L929) and in vivo. KEY FINDINGS: Scanning electron microscopy studies showed that all formulations prepared had smooth surface and spherical in shape. FTA and FTA-loaded nanoparticles have cytotoxic activity against RG2 glioma cell lines in cell culture studies, which further increases with addition of DOTAP. Magnetic resonance imaging and histopathologic evaluation on RG2 tumour cells in rat glioma model (49 female Wistar rats, 250-300 g) comparing intravenous and intratumoral injections of the drug have been performed and FTA-loaded nanoparticles reduced tumour size significantly in in-vivo studies, with higher efficiency of intratumoral administration than intravenous route. CONCLUSION: Farnesylthiosalicylic acid-loaded PLGA-DSPE-PEG-DOTAP hybrid nanoparticles are proven to be effective against glioblastoma in both in-vitro and in-vivo experiments.

A Rare Pediatric Case of Neurocysticercosis Misdiagnosed As Brain Abscess.

Neurocysticercosis is a parasitic infection of the central nervous system caused by the larval stage of Taenia solium. Although this zoonotic infection is one of the major public health problems in some developing countries, it is extremely rare in Turkey. In this article, we present the case of a pediatric patient with neurocysticercosis who was misdiagnosed with brain abscess because of focal seizures in another hospital.

Is white matter affected in adolescents with anorexia nervosa? A study using magnetization transfer imaging.

The aim of the study was to assess potential changes in structural integrity of the brain in adolescent patients with Anorexia Nervosa (AN) using the magnetization transfer imaging technique and re-evaluating after nutritional rehabilitation was accomplished. Magnetization transfer ratio (MTR) was used for the detection and quantification of histological changes to the white matter of 9 adolescents diagnosed with AN at diagnosis and after weight gain. Using the MTR technique 6 regions were measured: the pons, left cerebellar hemisphere, amygdala, thalamus, corona radiata and white matter adjacent to the left dorsolateral prefrontal cortex. Scans from the patients with AN were also compared with scans from an age-matched subset of 9 healthy controls. Compared with control subjects, the MTR of participants with AN was no different either at the early stages of diagnosis (p=0.62) or after weight recovery (p=0.81). Similarly, comparison of MTR between patients with AN at diagnoses compared with MTR after weight recovery yielded no statistically significant difference (P=0.33). We conclude that MTR was not able to detect any neuropathological alterations in adolescent patients with AN at either diagnosis or after nutritional recovery and the achievement of weight gain.

A late-diagnosed phenylketonuria case presenting with autism spectrum disorder in early childhood.

Phenylketonuria is one of the most prevalent autosomal recessive hereditary disorders in Turkey. If untreated, it results in severe brain damage and can also be associated with autism in certain patients. We present a three-year old boy who exhibited the symptoms of autism and was subsequently diagnosed with phenylketonuria. This case illustrates that because the majority of autism cases are idiopathic, an occasional patient with a metabolic disorder might be overlooked especially in the era of newborn screening. We also discuss the possible pathogenetic processes leading to autistic symptoms in phenylketonuria, and wish to draw attention to the possibility of cases missed in the screening program because of less than 100% coverage or insufficient food intake before blood sampling. Clinicians should keep in mind the possibility of treatable disorders in children with autism even when such disorders appear unlikely.

Conventional and advanced MR imaging in infantile Refsum disease.

We report magnetic resonance (MR) imaging findings including diffusion-weighted imaging and proton MR spectroscopy findings in a patient with infantile Refsum disease. The initial diagnosis was made on the basis of history, clinical findings and biochemical studies. Bilateral and symmetrical involvement of the peritrigonal white matter, centrum semiovale, thalami, corpus callosum and corticospinal tracts as assessed by increased T2 signal was highly suggestive of a peroxisomal disorder. Facilitated diffusion was observed in diseased parenchyma. Long echo-time (TE: 270 ms) MRS showed decreased N-acetyl-aspartate/creatine and elevated choline/creatine and lactate; short echo-time MRS (TE: 30 ms) revealed increased myoinositol at 3.56 ppm and lipid peaks at 0.9 and 1.3 ppm. A major contribution to the differential diagnosis came from MR imaging and proton MRS, as discussed in this report.

A Rare Association of Trigeminal Autonomic Cephalgia: Pontine Capillary Telangiectasia.

This report describes a case of pontine capillary telangiectasia in a 43-year-old woman with a clinical diagnosis of trigeminal autonomic cephalgia. The possible association with pontine capillary telangiectasia and trigeminal autonomic cephalgia is discussed.