Notes

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In the rare co-occurrence of childhood cancer and severe hemophilia, hemostatic management is of paramount therapeutic importance. We present the case of an 11-month-old boy with severe congenital hemophilia B, who was diagnosed with metastatic high-risk neuroblastoma. He consequently developed paraneoplastic coagulopathy with life-threatening tumor hemorrhage and intracranial hemorrhage, showing central nervous system relapse. Management consisted of factor IX replacement with extended half-life factor IX fusion protein, adjusted to bleeding risk. Additional interventions included factor XIII, fibrinogen, fresh frozen plasma, tranexamic acid, and platelet transfusions. The half-life of factor IX products was markedly reduced requiring close factor IX monitoring and adequate replacement. This intensified treatment allowed chemotherapy, autologous stem cell transplantation, and GD2 antibody immune therapy without bleeding or thrombosis.
This review covers several aspects our understanding of episodic manifestations and unusual symptoms that may be associated with migraine aura.

The episodic manifestation of migraine aura is typically visual in nature, although five other types are currently recognized sensory, speech and/or language, motor, brainstem, and retinal. Other transitory perceptions or experiences such as emotional, olfactory, or auditory have been reported as possible migraine auras. As underlined by the much higher reported prevalence of aura manifestation in individuals with professional knowledge of its possible manifestations, it appears that a number of migraine auras may remain unnoticed, unreported, or misdiagnosed.

Aura manifestations may be more common, complex, symptom-rich and variable than previously thought. Clinicians should proactively ask questions beyond those addressing visual symptoms when examining individuals with a potential diagnosis of migraine with aura.
Aura manifestations may be more common, complex, symptom-rich and variable than previously thought. Clinicians should proactively ask questions beyond those addressing visual symptoms when examining individuals with a potential diagnosis of migraine with aura.
With the introduction of new diagnostic criteria, the sensibility for multiple sclerosis (MS) diagnosis increased and the number of cases with the clinically isolated syndrome (CIS) decreased. Nevertheless, a misdiagnosis might always be around the corner, and the exclusion of a 'better explanation' is mandatory.There is a pressing need to provide an update on the main prognostic factors that increase the risk of conversion from CIS or from radiologically isolated syndrome (RIS) to MS, and on the potential 'red flags' to consider during the diagnostic workup.

We discuss diagnostic challenges when facing patients presenting with a first demyelinating attack or with a RIS, with a focus on recently revised diagnostic criteria, on other neuroinflammatory conditions to be considered in the differential diagnosis and on factors distinguishing patients at risk of developing MS.A correct definition of a 'typical' demyelinating attack, as well as a correct interpretation of MRI findings, remains crucial in the diagnostic process. The cerebrospinal fluid examination is warmly recommended to confirm the dissemination in time of the demyelinating process and to increase the diagnostic accuracy.

An early and accurate diagnosis of MS requires careful consideration of all clinical, paraclinical and radiological data, as well the reliable exclusion of other mimicking pathological conditions. This is advocated to promptly initiate an appropriate disease-modifying therapy, which can impact positively on the long-term outcome of the disease.
An early and accurate diagnosis of MS requires careful consideration of all clinical, paraclinical and radiological data, as well the reliable exclusion of other mimicking pathological conditions. This is advocated to promptly initiate an appropriate disease-modifying therapy, which can impact positively on the long-term outcome of the disease.
Thunderclap headache (TCH) is an abrupt-onset of severe headache that needs to be thoroughly investigated because the most common secondary cause is subarachnoid hemorrhage (SAH). There has been no consensus guideline regarding the diagnostic workup. This review aims to provide an update on the evaluation of TCH.

The most important update in the 2019 American College of Emergency Physicians guideline for evaluation of acute headache in the emergency department is that negative noncontrast brain computed tomography (CT) findings within 6 h from ictus essentially excludes SAH. Additionally, the updated guideline recommends that after a negative brain CT, CT angiogram is a reasonable alternative to lumbar puncture if clinical suspicion of an intracranial source of SAH is high. An important update of reversible vasoconstriction syndrome (RCVS), the second most common etiology of TCH, is the RCVS2 score development based on clinical and radiological features, providing high specificity and sensitivity for distinguishing RCVS from other intracranial arteriopathies.

Although the evaluation of TCH is exhaustive, the potentially catastrophic consequence of a missed diagnosis of sentinel headache justifies the efforts. Awareness of the clinical features and application of diagnostic tools specific for different pathological conditions can facilitate the diagnostic workup.
Although the evaluation of TCH is exhaustive, the potentially catastrophic consequence of a missed diagnosis of sentinel headache justifies the efforts. Awareness of the clinical features and application of diagnostic tools specific for different pathological conditions can facilitate the diagnostic workup.
Trigeminal neuralgia is a well-known facial pain syndrome with several treatment options. In contrast, non-neuralgiform idiopathic facial pain syndromes are relatively rare, reflected by the fact that, until 2020, no internationally accepted diagnostic classification existed. Like trigeminal neuralgia, these non-dental facial pain syndromes need to be managed by neurologists and pain specialists, but the lack of pathophysiological understanding has resulted in an underrepresented and undertreated patient group.

This work provides a brief overview of the most common primary facial pain syndromes, namely, the facial attack-like facial pain, which corresponds to attack-like headache, the persistent idiopathic facial pain (formerly 'atypical facial pain'), and trigeminal neuropathy. What these disorders have in common is that they should all be treated conservatively.

On the basis of pragmatic classifications, permanent and attack-like primary facial pain can be relatively easily differentiated from one another. The introduction of the new International Classification of Orofacial Pain offers the opportunity to better coordinate and concentrate scientific efforts, so that in the future the therapy strategies that are still inadequate, can be optimized.
On the basis of pragmatic classifications, permanent and attack-like primary facial pain can be relatively easily differentiated from one another. The introduction of the new International Classification of Orofacial Pain offers the opportunity to better coordinate and concentrate scientific efforts, so that in the future the therapy strategies that are still inadequate, can be optimized.
Central nervous system (CNS) tuberculosis is the most devastating form of tuberculosis (TB), with mortality and or neurological sequelae in over half of individuals. We reviewed original research and systematic reviews published since 1 January 2019 for new developments in CNS TB pathophysiology, diagnosis, management and prognosis.

Insight in the pathophysiology is increasing steadily since the landmark studies in 1933, focussing on granuloma type classification, the relevance of the M. tuberculosis bacterial burden and the wide range of immunological responses. Although Xpert/RIF has been recommended by the WHO for extrapulmonary TB diagnosis, culture is still needed to increase the sensitivity of TB meningitis diagnosis. Sequential MRIs can improve understanding of neurological deficits at baseline and during treatment. Pharmacokinetic/pharmacodynamic modelling suggests that higher doses of rifampicin and isoniazid in TB meningitis could improve survival.

Recent studies in the field of CNS-TB have largely focussed on TB meningitis. The outcome may improve by optimizing treatment dosing. This needs to be confirmed in clinical trials. Due to the important role of inflammation, these trials should be used as the platform to study the inflammatory and metabolomic responses. This could improve understanding of the biology of this disease and improve patient outlook by enabling individualised host-directed therapy.
Recent studies in the field of CNS-TB have largely focussed on TB meningitis. The outcome may improve by optimizing treatment dosing. This needs to be confirmed in clinical trials. Selleckchem BIX 02189 Due to the important role of inflammation, these trials should be used as the platform to study the inflammatory and metabolomic responses. This could improve understanding of the biology of this disease and improve patient outlook by enabling individualised host-directed therapy.
The objective of this study was to investigate the efficacy of high-intensity focused ultrasound (HIFU) combined with gonadotropin-releasing hormone agonist or levonorgestrel-releasing intrauterine system (LNG-IUS) in treating dysmenorrhea in patients with severe adenomyosis.

A retrospective analysis was performed on 243 patients diagnosed with severe adenomyosis. Patients were divided into H (received HIFU alone), H-G (received HIFU combined with gonadotropin-releasing hormone agonist), and H-L (received HIFU combined with LNG-IUS) groups. Their clinical results were compared at 3 months, 6 months, and 12 months after treatment.

The effective rates of dysmenorrhea relief in the 3 groups after 3 months were 95.24% in the H group, 98.8% in the H-G group, and 94.74% in the H-L group; those after 6 months were 88.10% in the H group, 95.18% in the H-G group, and 84.21% in the H-L group; those after 12 months were 77.38% in the H group, 79.52% in the H-G group, and 96.05% in the H-L group. There was significant difference in effective rates of dysmenorrhea relief among 3 groups after 12 months of treatment, but not 3 or 6 months. In addition, at 12 months after treatment, there were significant differences in the efficacy of dysmenorrhea between patients of different ages or different ablation rates in group H. However, there was no significant difference in the H-G group and the H-L group.

High-intensity focused ultrasound alone is effective in alleviating the symptoms of dysmenorrhea in short term. However, HIFU combined with LNG-IUS improves the therapeutic effect for a longer period.
High-intensity focused ultrasound alone is effective in alleviating the symptoms of dysmenorrhea in short term. However, HIFU combined with LNG-IUS improves the therapeutic effect for a longer period.
Standing waves are a phenomenon of uncertain etiology seen on imaging. We present the first case demonstrating standing waves on computed tomography angiography in multiple vessels in a single patient with imaging evidence of resolution in some of the vessels. Our case further supports the literature that standing waves are a physiologic phenomenon, likely because of flow mechanics, rather than modality.
Standing waves are a phenomenon of uncertain etiology seen on imaging. We present the first case demonstrating standing waves on computed tomography angiography in multiple vessels in a single patient with imaging evidence of resolution in some of the vessels. Our case further supports the literature that standing waves are a physiologic phenomenon, likely because of flow mechanics, rather than modality.
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