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Mucormycosis infection of the maxillofacial region and brain has been associated with coronavirus disease 2019 (COVID-19) infection. Mucormycosis was relatively a rare infection before COVID-19, and imaging findings are not very well described.A retrospective imaging study of 101 patients diagnosed with COVID-19-associated mucormycosis by histopathology and/or culture was performed. All patients underwent computed tomography and/or magnetic resonance imaging based on the clinical condition of the patient and on consensus decision by the team of treating physicians. A simple 3-stage classification system based on imaging findings was adopted.One hundred one cases were included in the final analysis (mean age = 55.1 years; male/female ratio = 67:34). The affected patients had diabetes in 94% of the instances (n = 95), 80.1% (n = 81) received steroids), whereas 59.4% (n = 60) patients received supplemental oxygen. The majority underwent surgical intervention, whereas in 6 cases, patients were treated with antibiotic regimens. Sixty subjects improved following therapy, whereas 18 eventually succumbed to the illness. We noted a significant positive correlation between the imaging stage and outcomes. No association was seen between other clinical parameters and final clinical outcomes. Salient imaging findings include lack of normal sinonasal mucosal enhancement, perisinus inflammation, ischemic optic neuropathy, perineural spread, pachymeningeal enhancement, and presence of strokes.We describe the imaging findings in the largest cohort of patients with rhino-orbito-cerebral mucormycosis in the context of the current COVID-19 pandemic. A simplified staging system described here is helpful for standardized reporting and carries prognostic information.

The use of standardized imaging protocols is paramount in order to facilitate comparable, reproducible images and, consequently, to optimize patient care. Standardized MR protocols are lacking when studying head and neck pathologies in the pediatric population. We propose an international, multicenter consensus paper focused on providing the best combination of acquisition time/technical requirements and image quality. Distinct protocols for different regions of the head and neck and, in some cases, for specific pathologies or clinical indications are recommended. This white paper is endorsed by several international scientific societies and it is the result of discussion, in consensus, among experts in pediatric head and neck imaging.

Unlike adult neurofibromatosis type 2 (NF2), which presents with symptoms related to bilateral vestibular schwannomas, children with NF2 most frequently present with ocular, dermatological, and neurological symptoms. Arteriopathy, a well-established feature in neurofibromatosis type 1, is not a widely recognized feature of NF2. Here we report three children with NF2 with cerebral arteriopathy and/or arterial ischaemic stroke. Bevacizumab, a vascular endothethial growth factor inhibitor, is an established treatment for rapidly growing vestibular schwannomas; however, it carries a risk of both ischaemic and haemorrhagic stroke. Thus, the role of screening and risk to benefit ratio of bevacizumab in NF2 merit further consideration. WHAT THIS PAPER ADDS: Children with neurofibromatosis type 2 (NF2) may be at increased risk of cerebral vasculopathy and arterial ischaemic stroke. Targeted magnetic resonance angiography should be performed in children with NF2 who are being considered for bevacizumab therapy.

Type 1 Neurofibromatosis (NF1) is a common autosomal dominant condition, with a major impact on the nervous system, eye, bone, and skin, and a predisposition to malignancy. At present it is not possible to predict clinically or on imaging, whether a brain tumour will remain indolent or undergo high-grade change. There are no consensus guidelines on the follow-up of non-optic pathway glioma (non-OPG) tumours in NF1. One hundred patients from the National NF1 Service with generalised NF1 and a diagnosis of non-OPG glioma were followed up for a median time of 63 months after glioma detection. Forty-two patients underwent surgical intervention. Ninety-one percent (38) of those requiring surgery did so within 5 years of diagnosis of glioma. Serial neuroimaging was undertaken in 88 patients. In 66 (75%), the lesion on the scan was stable or had improved at follow-up. High-grade lesions were present in five patients and were strongly associated with tumours in the thalamus (p = 0.001). Five patients died during follow-up. The diagnosis of high-grade glioma had a HR of 99.7 (95% CI 11.1–898.9, p

Background We present an unusual neuroimaging finding in a young girl with genetically confirmed vanishing white matter disease and a possible response to immunotherapy. Methods and results 2.5 yr old girl, presented with acute onset unsteadiness and encephalopathy following a viral illness. MRI showed global symmetric white matter abnormality, with symmetric enhancement of cranial nerves (III and V) and of cervical and lumbar roots. She received immunotherapy for her encephalopathic illness with white matter changes. Follow up neuroimaging showed resolution of white matter edema and resolution of the change in the brainstem. Genetic testing confirmed a diagnosis of vanishing white matter disease (VWMD). Conclusion Craniospinal nerve enhancement and possible response to immunotherapy has not been described in vanishing white matter disease.

Posterior ischaemic optic neuropathy is a rare cause of visual loss believed to be due to infarction in the territory of the pial branches of the ophthalmic artery. The disorder most commonly occurs in the context of prolonged surgery or giant cell arteritis, and the absence of clinical signs in the eye means that the diagnosis is one of exclusion. Here, we present two cases studies of patients who developed posterior ischaemic optic neuropathy confirmed by the observation of secondary changes on diffusion-weighted imaging sequences. In the first case visual loss followed robotic pelvic surgery, and in the second case it was associated with multiorgan dysfunction secondary to severe pancreatitis. Our cases demonstrate that in the right clinical context, diffusion-weighted imaging can provide a positive diagnosis of acute posterior ischaemic optic nerve injury in the acute phase.

We describe two women presenting with severe postpartum headache associated with hypertension but with no other signs or investigation results to suggest pre-eclampsia. In one case, the headache was associated with atypical subarachnoid haemorrhage. The variable nature of the headache and the degree of associated hypertension raised the clinical suspicion of reversible cerebral vasoconstriction syndrome, confirmed on MR angiography. Both patients took nimodipine until the cerebral vasoconstriction had resolved radiologically.

Objectives:(1) To study the prevalence and characteristics of large endolymphatic sac internal compartments on thin-section T2- and T2*-weighted magnetic resonance imaging, and to relate these to other large endolymphatic sac magnetic resonance imaging features, and (2) to correlate the compartment imaging features, endolymphatic sac size and labyrinthine anomalies with the patients' clinical and audiological data.Method:Magnetic resonance imaging studies for 38 patients with large endolymphatic sac anomalies were retrospectively reviewed in a tertiary referral centre. Endolymphatic sac compartment presence, morphology and imaging signal were assessed. Endolymphatic sac size and labyrinthine anomalies were also recorded. Endolymphatic sac compartments and other imaging features were correlated with clinical and audiological data.Results:Compartments were present in 57 per cent of the imaged endolymphatic sacs, but their presence alone did not correlate with other imaging features or clinical data. The endolymphatic sac : internal auditory meatus signal ratio was associated with a history of sudden or fluctuating hearing loss. Hearing loss correlated with opercular and extraosseous endolymphatic sac size measurements. A larger midpoint intraosseous endolymphatic sac size was associated with clear fluid loss at cochlear implantation.Conclusion:The magnetic resonance imaging characteristics of large endolymphatic sac compartments have been defined. The endolymphatic sac size and distal compartment signal should be recorded, as these provide prognostic information and assist the planning of appropriate interventions.

Objectives Deep Brain Stimulation (DBS) of the Globus Pallidus Internus produces dramatic benefits in primary dystonia. Improvements in patients with secondary dystonia are smaller and vary markedly between individuals. Predictive markers are lacking. This study tests the hypothesis that Somatosensory Evoked Potentials (SEPs) and Central Motor Conduction Times (CMCT) may predict outcome from DBS in childhood dystonia. Methods Data were obtained from 180 consecutive children with dystonia undergoing multidisciplinary assessment for DBS (mean age 10 years; range 2.5–19). CMCT to each limb was obtained using Transcranial Magnetic Stimulation and the F-wave method. Median and posterior tibial nerve SEPs were recorded over contralateral and midline centro-parietal scalp. Technically unsatisfactory data were excluded. Structural abnormalities were assessed with cranial MRI. Outcome from DBS at 1 year was assessed as percentage improvement in Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS-m). Results Satisfactory CMCT data were obtained from 146 children, of whom 28 (19%) had an abnormal CMCT to at least one limb. Satisfactory cortical SEP data were obtained from 100 children, of whom 47 had at least one abnormal cortical potential. Abnormal CMCTs and SEPs were both observed more frequently in secondary than primary/primary plus dystonia (CMCT abnormal in 22% secondary versus 9% primary patients; SEP abnormal in 53% secondary vs 24% primary patients). Of children proceeding to DBS, improvement in BFMDRS-m was greater in those with normal (n = 78) than abnormal CMCT (n = 11) (Mann Whitney test p = 0.002) and was also greater in those with normal (n = 35) versus abnormal SEPs (n = 16) (Mann Whitney test p = 0.001). On sub-group analysis, the relationships between CMCT/SEPs and BFMDRS-m scores were independent of aetiology (primary versus secondary) and MRI findings (normal versus abnormal). Discussion/Conclusion CMCTs and SEPs provide objective evidence of motor and sensory pathway dysfunction in children with dystonia and relate to DBS outcome. These markers may therefore contribute to patient selection and counselling of families about potential benefit from neuromodulation. Significance We identify neurophysiological markers that can guide the selection of patients for DBS.

We describe three children with genetically different sodium channel alpha 1 subunit (SCN1A) mutation associated epilepsy who experienced a sudden and sustained neurologic regression following status epilepticus in two and acute sepsis in one. Neuroimaging showed evidence of cerebral ischemia in one, but the other two cases showed cerebellar signal abnormalities. The selectivity of cerebellar white matter change suggests a different mechanism of injury or increased susceptibility of this brain region to injury in at least some patients with SCN1A mutations. This report adds to the spectrum and mechanism of acute neurologic deterioration and functional deficit associated with SCN1A mutations, which remains to be fully understood.

Holmes’ tremor (also known as rubral or midbrain tremor) is a rare movement disorder characterized by a low frequency tremor of the upper extremity at rest and upon voluntary movement. This report presents the case of a patient who developed a tremor of her right arm, with hemiparesis and ataxia as a result of systemic and cerebral toxoplasmosis in association with AIDS. MRI brain imaging showed a lesion in the contralateral red nucleus in the rostral midbrain, consistent with toxoplasmosis, and confirming the diagnosis of a Holmes’ tremor. The patient responded well, symptomatically, to botulinum toxin (Botox) injections. This case illustrates the importance of neuroimaging in diagnosis of tremor disorders.

Arterial spin labeling is a fully noninvasive magnetic resonance perfusion imaging method, ideally suited to pediatric perfusion imaging. We describe an 8-year-old boy with sickle cell disease, extensive right-hemispheric cerebral infarction, and crossed-cerebellar diaschisis apparent on arterial spin labeling perfusion magnetic resonance imaging. To our knowledge, this is the first case of crossed-cerebellar diaschisis demonstrated with arterial spin labeling, highlighting the potential value of perfusion magnetic resonance imaging in the clinical evaluation and follow-up of crossed-cerebellar diaschisis, and the suitability of arterial spin labeling methods for routine perfusion imaging in pediatric patients.

Introduction DBS of the Globus pallidus internus has dramatic benefits in primary dystonia. Responsiveness of secondary dystonia is more modest and varies markedly between individuals. Predictive markers are lacking and the underlying pathophysiology of secondary dystonia is poorly understood. We report Somatosensory Evoked Potentials (SEPs) and Central Motor Conduction Times (CMCT) in children with dystonia and test the hypothesis that these parameters relate to outcome from DBS. Methods Data were obtained from 180 consecutive children with dystonia undergoing multidisciplinary DBS assessment (mean age 10 years; range 2.5–19). Transcranial Magnetic Stimulation was applied to motor cortex and Motor Evoked Potentials were recorded in the activated contralateral hand and foot muscles. CMCT to each limb was calculated using the F-wave method. Median nerve SEPs were recorded over ipsilateral Erb’s point, 7th and 2nd cervical vertebrae and contralateral centroparietal scalp. Posterior tibial nerve SEPs were recorded over ipsilateral popliteal fossa and midline centroparietal scalp. A mid-frontal reference was used. Electrical stimuli of 0.2millisecond duration were applied at 2.1 Hz, just above motor threshold. SEPs were classed as abnormal if delayed, absent or of abnormal waveform. Technically unsatisfactory recordings were excluded. Structural abnormalities were assessed with cranial MRI. Outcome from DBS at 1 year was assessed as percentage improvement in Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS-m). Results Of the 146 children with satisfactory CMCT data, 28(19%) had an abnormal CMCT to at least one limb. Of the 100 children with satisfactory cortical SEP data, 47 had at least one abnormal cortical potential. Abnormal CMCTs and SEPs were both observed more frequently in secondary than primary/primary plus dystonia (CMCT: 22% vs. 9%, SEP: 53% vs. 24%). Of children proceeding to DBS, improvement in BFMDRS-m was greater in those with normal (n = 78) than abnormal CMCT (n = 11) (Mann Whitney p = 0.002) and in those with normal (n = 35) versus abnormal SEPs (n = 16) (Mann Whitney p = 0.001). On sub-group analysis, these relationships were preserved regardless of aetiology (primary versus secondary) or cranial imaging (normal vs abnormal MRI). Conclusion CMCTs and SEPs provide objective evidence of motor and sensory pathway dysfunction in children with dystonia. Abnormal CMCTs and SEPs relate to DBS outcome, therefore contributing to patient selection and counselling of families about potential benefit from neuromodulation.

An overwhelming majority of pharyngeal and laryngeal tumours are squamous cell carcinomas.A knowledge of the imaging anatomy and normal appearances of different tissues is crucial to diagnosis and staging.Early mucosal lesions can be easily missed on imaging and are best detected by direct visualization. Imaging appearances always require correlation with endoscopic appearances.Submucosal spread is best visualized on cross sectional imaging and the primary role of the radiologist is to accurately stage the extent of disease which directly influences treatment planning.Identification of cartilage involvement is crucial but is not without pitfalls.Both CT and MRI are widely used for tumour staging and are sometimes complementary.Post treatment imaging appearances can mimic tumour. Endoscopy, follow up imaging, PET imaging and biopsy all play a complementary role.Perineural and intracranial extension is often seen with nasopharyngeal and occasionally oropharyngeal tumours. The radiologist should be alert to ...

The pseudotumor cerebri syndrome (PTCS) arises as a consequence of raised intracranial pressure in the absence of a space occupying lesion. It can be associated with poor cranial cerebral venous outflow, typically in the context of venous sinus thrombosis. Increased intracranial pressure secondary to extrinsic compression and constriction of the jugular veins by inflammatory or fibrotic tissue is rare. We describe this phenomenon as a consequence of Wegener's granulomatosis (WG) in which venous compression is confirmed using non-invasive cranial imaging.