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The outcome of spontaneous intracranial hypotension (SIH) years after onset is largely unknown. The objective of our study was to describe our clinical experience and long-term outcomes in a case series of patients with SIH. From March 2007 to March 2022, demographic variables, clinical symptoms, neuroimaging findings, and response to treatment were retrospectively analyzed in patients with confirmed SIH and in a subgroup of patients with clinical symptoms but not confirmed by MR or LP, probable SIH (pSIH). We have included 37 SIH and 13 pSIH patients. The average age at onset was 44 years, and 59% (pSIH 46%) were women. All patients presented with a new-onset orthostatic headache. In the SIH group, brain MRI showed signs of intracranial hypotension in all patients, spinal MR was performed in 70%, and pathological findings were identified in 73%. The range of EBP was 1-8 (average 2.2). Good outcome after single or 2 EBPs had 42% (pSIH 46%) of patients. At follow-up, 81% (pSIH 54%) of patients had a favorable outcome. Relapse occurred in 16% of patients in the SIH group and none in the pSIH group. The mean follow-up time was 60 months. EBP is an effective and minimally invasive treatment, and efficacy seems independent of disease duration. The long-term prognosis is favorable in 80% of SIH patients and in half of pSIH patients. Despite the lack of MRI signs of low intracranial pressure on neuroimaging, pSIH patients should also be offered EBP, and more awareness of SIH is needed. [ABSTRACT FROM AUTHOR]

Objective: To systematically investigate previously examined biomarkers in blood, urine, cerebrospinal fluid, tear fluid, and saliva of patients with cluster headache. Background: Cluster headache is a condition with extensive clinical challenges in terms of diagnosis and treatment. Identification of a biomarker with diagnostic implications or as a potential treatment target is highly warranted. Methods: We conducted a systematic review including peer reviewed full text of studies that measured biochemical compounds in either blood, urine, cerebrospinal fluid, tear fluid, or saliva of patients with cluster headache diagnosed after the implementation of the International Classification of Headache Disorders (1988) written in English, Danish, Swedish, or Norwegian. Inclusion required a minimum of five participants. The search was conducted in PubMed and EMBASE, in September 2022, and extracted data were screened by two authors. Preferred Reporting Items for Systematic Reviews and Meta‐Analyses guidelines for reporting systematic reviews were followed. The Newcastle–Ottawa Scale was used to assess the risk of bias in case–controlled studies. Results: We included 40 studies involving 832 patients with cluster headache and 872 controls, evaluating 80 potential biomarkers. The risk of bias for case–controlled studies was a median of 6 (range: 3–8) and 20 studies out of 40 (50%) were of fair or good quality. Most studies were identified within three groups: hypothalamic‐regulated hormones, inflammatory markers, and neuropeptides. Among the hypothalamic hormones, cortisol was the most frequently investigated (N = 7) and was elevated in cluster headache in most of the studies. The most frequently examined inflammatory marker was interleukin 1 (N = 3), but findings were divergent. Calcitonin gene–related peptide was the most investigated neuropeptide (N = 9) and all studies found increased levels during attacks. Conclusion: Biomarker findings have been inconsistent and widely non‐specific for cluster headache, which explains why none of the previous studies succeeded in identifying a unique biomarker for cluster headache, but instead contributed to substantiating the underlying pathophysiologic mechanisms. Several of the examined biomarkers could hold promise as markers for disease activity but are unfit for a clear distinction from both controls and other headaches. [ABSTRACT FROM AUTHOR]

Background and Purpose: Cluster headache is a relatively rare, disabling primary headache disorder with a major impact on patients' quality of life. This work presents evidence‐based recommendations for the treatment of cluster headache derived from a systematic review of the literature and consensus among a panel of experts. Methods: The databases PubMed (Medline), Science Citation Index, and Cochrane Library were screened for studies on the efficacy of interventions (last access July 2022). The findings in these studies were evaluated according to the recommendations of the European Academy of Neurology, and the level of evidence was established using GRADE (Grading of Recommendations Assessment, Development, and Evaluation). Recommendations: For the acute treatment of cluster headache attacks, there is a strong recommendation for oxygen (100%) with a flow of at least 12 L/min over 15 min and 6 mg subcutaneous sumatriptan. Prophylaxis of cluster headache attacks with verapamil at a daily dose of at least 240 mg (maximum dose depends on efficacy and tolerability) is recommended. Corticosteroids are efficacious in cluster headache. To reach an effect, the use of at least 100 mg prednisone (or equivalent corticosteroid) given orally or at up to 500 mg iv per day over 5 days is recommended. Lithium, topiramate, and galcanezumab (only for episodic cluster headache) are recommended as alternative treatments. Noninvasive vagus nerve stimulation is efficacious in episodic but not chronic cluster headache. Greater occipital nerve block is recommended, but electrical stimulation of the greater occipital nerve is not recommended due to the side effect profile. [ABSTRACT FROM AUTHOR]