Images: Sleep-related painful erection with concomitant hypnic headache.

Sleep-related painful erection (SRPE) is a parasomnia defined by the repetition of painful erections during rapid eye movement (REM) sleep. Hypnic headache (HH) is a primary headache occurring exclusively at night, often during REM sleep. We report the observation of a 33-year-old man with simultaneous SRPE and HH. Physical examination was normal. Comprehensive urological and endocrine explorations excluded other organic differential diagnoses. Polysomnography revealed several awakenings in REM, due to SRPE and concurrent HH. Medication by baclofen at bedtime seemed to have resulted in a decrease in SRPE episodes, confirmed by polysomnography, but at the cost of excessive daytime sleepiness, and was discontinued by the patient. Caffeine intake at bedtime was proposed, but the patient was reluctant because he was concerned about worsening insomnia. At 9-month follow-up, the patient had accepted his medical condition and was coping with both SRPE and HH. He felt reassured and wished no "overmedicalization." To our knowledge, the coexistence of both conditions has not yet been reported, yet their frequencies might be underestimated. We hypothesize a common underlying pathophysiology with a possible dysfunction of the vascular control and/or the autonomic nervous system and that could involve the hypothalamus. Somnologists should be aware of SRPE, potentially overlapping with HHs. SRPE should be considered in case of sleep-maintenance insomnia. Patient reassurance seems to be central in the care process of SRPE. CITATION: Moreau A, Monnier L, Medde A, Bourgin P, Ruppert E. Images: sleep-related painful erection with concomitant hypnic headache. J Clin Sleep Med. 2024;20(5):837-839.

Other Primary Headache Disorders.

PURPOSE: This article provides an overview of a diverse group of primary headache disorders that are categorized in the International Classification of Headache Disorders, 3rd Edition (ICHD-3), as "other primary headache disorders." This article provides clinicians with a distilled understanding of the diagnoses and their epidemiology, pathophysiology, and management. RECENT FINDINGS: Cough-induced headache requires neuroimaging to exclude posterior fossa pathology and recently has been reported as a common symptom in patients with CSF-venous fistula. Clinical overlap is observed between patients with primary exercise headache and primary headache associated with sexual activity. Patients with recurrent thunderclap headache associated with sexual activity should be presumed to have reversible cerebral vasoconstriction syndrome until proven otherwise. De novo external-pressure headache is a common sequela among health care workers using personal protective equipment during the COVID-19 pandemic. New daily persistent headache is an important mimicker of chronic migraine or chronic tension-type headache and is distinguished by a daily-from-onset progression of persistent headache; a treatment-refractory course is often observed, and early involvement of a multidisciplinary team, including a psychotherapist, is advised. SUMMARY: Patients with primary headache disorders that are classified as "other primary headache disorders" have presentations with unique diagnostic and management considerations. The disorders are highly recognizable, and an appreciation of the diagnoses will aid clinicians in providing safe and effective care for patients presenting with headache.

RCVS-TCH score can predict reversible cerebral vasoconstriction syndrome in patients with thunderclap headache.

Reversible cerebral vasoconstriction syndrome (RCVS) is one of the most important differential diagnosis in patients with thunderclap headache (TCH). We aimed to develop a new scoring system for RCVS in patients with TCH. We retrospectively analyzed 72 patients enrolled in the prospective study of TCH conducted in 2015-2016 (derivation set). We identified possible predictors for the diagnosis of RCVS and constructed a prediction model (RCVS-TCH score) using the multivariable logistic regression model. Diagnostic performance was validated to an independent validation set from our headache registry. The derivation set comprised 41 patients with RCVS and 31 with non-RCVS, and the validation set included 253 patients with TCH (165 with RCVS and 88 with non-RCVS). The RCVS-TCH score (range: 0-12) contained four predictors: recurrent TCHs, female sex, triggering factor for TCH (single or multi) and blood pressure surge. The C-index of RCVS-TCH score was 0.929 (95% CI = 0.874-0.984). The RCVS-TCH score ≥ 7 had a sensitivity of 80% and a specificity of 97% in discriminating RCVS from non-RCVS. In the validation set, RCVS-TCH score showed a C-index of 0.861 (95% CI = 0.815-0.908). In our study, the RCVS-TCH showed good performance, which may aid the diagnosis of RCVS among patients with TCH.

Characterization of thunderclap headache in a reversible cerebral vasoconstriction syndrome case series.

OBJECTIVES: To empirically address how thunderclap headache (TCH) is described in a relevant real-world setting. BACKGROUND: TCH refers to a highly recognizable description of a severe headache that reaches maximum severity within 1 minute and endures for at least 5 minutes. The use of a numerical rating scale (NRS) to appraise TCH severity, as well as assessment of TCH progression in patients with pre-existing headache at the time of TCH onset has not been previously evaluated. METHODS: This was a retrospective case series of adults with a diagnosis of reversible cerebral vasoconstriction syndrome (RCVS), identified through a search of the electronic health record. Individuals meeting International Classification of Headache Disorders, 3rd Edition criteria for acute headache attributed to RCVS were included. Attacks described using a verbal descriptor scale (VDS), NRS, or both were recorded to evaluate acute headache characteristics. RESULTS: In all, 56 individuals with available descriptions of 120 acute headaches were included in the study analysis. Patients were female (35, 62.5%) with a median age of 46 (range: 19-67). The majority of patients reported a RCVS trigger (39, 69.6%). Acute headaches were characterized using a VDS (52, 43.3%), NRS (51, 42.5%), or both (17, 14.1%). Acute headaches were always described as severe when a VDS was utilized, and with a median NRS of 10 (range: 4-10). Four patients (7%) did not have a single headache characterized as either severe or with a NRS 8 or greater. In the 10 cases for which there was a pre-TCH baseline headache, it was either rated as mild or with a median NRS of 3 (range: 2-6). CONCLUSIONS: TCH in RCVS can be recognized using either VDS or NRS, with a broader range of peak intensities than previously recognized. TCH remains recognizable despite pre-existing baseline headache.

Narrative Review: Headaches After Reversible Cerebral Vasoconstriction Syndrome.

PURPOSE OF REVIEW: Reversible cerebral vasoconstriction syndrome (RCVS) is a disorder with distinct features: recurrent thunderclap headaches with reversible vasoconstriction of intracranial arteries. Substantial studies regarding outcomes after RCVS were conducted, showing favorable functional outcomes in most patients despite the potentially life-threatening complications of RCVS, including ischemic stroke, intracranial hemorrhage, or convexity subarachnoid hemorrhage. However, patients may report headaches after the resolution of RCVS while relative studies were scarce. RECENT FINDINGS: Two prospective studies from different cohorts consistently revealed that RCVS recurred in at least 5% of patients. Patients with prior migraine history and patients whose thunderclap headaches are elicited by sexual activity or exertion are at higher risk for RCVS recurrence. On the other hand, several retrospective studies and case reports reported that chronic headaches are common in RCVS patients after the resolution of acute bouts. The chronic headaches after RCVS are sometimes disabling in certain patients. Headaches after RCVS are not uncommon but usually overseen. Medical attention and examinations are warranted in patient with RCVS who reported recurrence of thunderclap headaches or chronic headaches after RCVS.

Reversible cerebral vasoconstriction syndrome and dissection in the setting of COVID-19 infection.

The current COVID-19 pandemic has recently brought to attention the myriad of neuro- logic sequelae associated with Coronavirus infection including the predilection for stroke, particularly in young patients. Reversible cerebral vasoconstriction syndrome (RCVS) is a well-described clinical syndrome leading to vasoconstriction in the intracra- nial vessels, and has been associated with convexity subarachnoid hemorrhage and oc- casionally cervical artery dissection. It is usually reported in the context of a trigger such as medications, recreational drugs, or the postpartum state; however, it has not been described in COVID-19 infection. We report a case of both cervical vertebral ar- tery dissection as well as convexity subarachnoid hemorrhage due to RCVS, in a pa- tient with COVID-19 infection and no other triggers.

The Relationship Between Parental Care and Pain in Children With Headache: A Narrative Review.

PURPOSE: In migraine or primary headache in children, parents play a fundamental role in pain management. For this narrative review, PubMed, Google Scholar, and Psych Info were searched using the terms "parent headache", "mother/father headache", "parental impact headache", "alexithymia parents headache", "catastrophizing parent headache", "family headache", "children parent headache", and "quality of life family headache". Articles were chosen for inclusion based on their relevance in to the topic. OVERVIEW: Several parental and psychological characteristics can influence in children and adolescent headache, such as parental attitudes as oppressive or overprotective; punitive parenting styles; familial psychological symptoms, especially anxiety and depression; catastrophizing about their child's pain or excessive worry about their child's headache; inability to express emotions; and feelings that may lead to somatization problems. DISCUSSION: Parents' attitudes and behaviors toward their child's headache have a strong relation with the severity of headache attacks. Mothers seem to have more influence than fathers on children's pain and emotional regulation. We suggest that the presence of caregiver-child transmission of maladaptive coping strategies, arising from difficulties expressing emotion, may lead to incorrect management of headache pain, further facilitating headache chronification.

Other primary headaches-thunderclap-, cough-, exertional-, and sexual headache.

This article reviews the disorders of thunderclap, cough, exertional and sexual headache. These are a group of paroxysmal and precipitated headaches, which often occur in bouts with prolonged remissions. Indometacin seems to be the most effective preventative. Each can occur in primary and secondary form. Thunderclap headache is the most frequently reported headache syndrome associated with a secondary pathology. Discussed are the complexities of whether all patients with thunderclap headache should have further investigation if timely computerised tomography is normal and, the relevance of abnormal imaging in these disorders, differentiating what is deemed to be secondary and managing the pain.

Review on the Diagnosis and Treatment of Reversible Cerebral Vasoconstriction Syndrome in Children and Adolescents.

Reversible cerebral vasoconstriction syndrome (RCVS) is a clinical-radiologic diagnosis that affects children and adolescents, but it is much more frequently reported in adults. Clinically, patients present with severe and commonly recurrent thunderclap headaches. Typical precipitating triggers include vasoactive substances, serotonergic agents, and the postpartum period. There may be associated neurologic complications at presentation or in the weeks following, such as convexity subarachnoid hemorrhage, stroke, cerebral edema, cervical artery dissection (CeAD), and seizures. Angiographically, the cerebral arteries demonstrate segmental vasoconstriction and dilation, although imaging early in the clinical course may be normal. Work-up is performed to exclude intracranial disorders such as vasculitis, subarachnoid hemorrhage due to ruptured aneurysm, meningitis, and intracranial venous sinus thrombosis. Within 1 month of initial symptom onset, clinical symptoms such as severe headache have ceased, and within 3 months, the cerebral vasoconstriction is much improved or resolved. Management involves avoidance of precipitating triggers and potentially short-term pharmacotherapy with calcium channel blockers for patients with associated neurologic complications. Steroids are not recommended and may worsen the clinical outcome. Prognosis is excellent in the large majority of patients, and only 5% of patients experience a recurrence of RCVS.

[Clinical and pathophysiological description of nummular headaches: a case series]. / Descripción clínica y fisiopatológica de la cefalea numular: serie de casos.

INTRODUCTION: Nummular headache was initially described as an infrequent primary headache characterised by chronic circumscribed mild-to-moderate pain in a small area of the head that may present with exacerbations. Since its initial description, dozens of cases of nummular headache have been reported which broaden the clinical and pathophysiological spectrum. AIMS: To review and establish the clinical characteristics of nummular headache, to analyse the epidemiology in our setting and to clarify some unanswered pathophysiological issues. PATIENTS AND METHODS: The study involved 83 cases (42 women and 41 men) who were diagnosed with nummular headache from July 2008 and followed up until 2018 at the Fundacion Alcorcon Hospital. The mean age of onset was 46 years. The time to diagnosis was seven months. RESULTS: The pain was moderate-intense and oppressive, with exacerbations in 62.5% of cases. The average size of the symptomatic area was 2.4 ± 0.7 cm, with a parietal (47%), temporal (24%), frontal (12%) or occipital (6%) location. It presented a chronic (82%) or episodic (18%) pattern. Symptoms of local sensory dysfunction were observed in 25 patients and three others showed skin alterations. Spontaneous remission was observed in 36% of cases, and the most commonly used drugs were non-steroidal anti-inflammatory drugs, paracetamol and gabapentin. Botulinum toxin was effective. CONCLUSIONS: Nummular headache is a disorder with well-defined characteristics and is considered an entity in its own right. Our data indicate that its origin lies in the epicranial structures. The conjunction of pain, sensory dysfunction and trophic disorders suggests a local form of a complex regional syndrome.

TITLE: Descripción clínica y fisiopatológica de la cefalea numular: serie de casos.Introducción. La cefalea numular se describió inicialmente como una cefalea primaria infrecuente caracterizada por dolor circunscrito crónico en una pequeña área craneal, de intensidad leve-moderada y que puede presentar exacerbaciones. Desde su descripción inicial se han publicado decenas de casos de cefalea numular que amplían el espectro clínico y fisiopatológico. Objetivos. Revisar y establecer las características clínicas de la cefalea numular, analizar la epidemiología en nuestro medio y aclarar incógnitas fisiopatológicas. Pacientes y métodos. Se estudiaron 83 casos (42 mujeres y 41 varones), que fueron diagnosticados de cefalea numular desde julio de 2003 a julio de 2008, y seguidos hasta 2018 en el Hospital Universitario Fundación Alcorcón. La edad media de inicio fue de 46 años. El tiempo de evolución hasta el diagnóstico fue de siete meses. Resultados. El dolor fue moderado-intenso y opresivo, con exacerbaciones en el 62,5% de los casos. El tamaño medio del área sintomática fue de 2,4 ± 0,7 cm, con una localización parietal (47%), temporal (24%), frontal (12%) u occipital (6%). Presentó un patrón crónico (82%) o episódico (18%). Mostraron síntomas de disfunción sensitiva local 25 pacientes, y otros tres, cambios en la piel. La remisión espontánea se observó en el 36% de los casos, y los fármacos más utilizados fueron los antiinflamatorios no esteroideos, el paracetamol y la gabapentina. La toxina botulínica fue eficaz. Conclusiones. La cefalea numular es un trastorno con características bien definidas y con entidad propia. Nuestros datos indican que su origen está en las estructuras epicraneales. La conjunción de dolor, disfunción sensitiva y alteraciones tróficas indica una forma local de síndrome regional complejo.

[Reversible cerebral vasoconstriction syndrome - a common cause of thunderclap headache].

Reversible cerebral vasoconstriction is characterized by thunderclap headache and vasoconstriction of cerebral arteries, with or without focal neurologic symptoms. The syndrome is three times more common in women with a mean age around 45 years. In approximately 60% of cases a cause can be identified, commonly after intake of vasoactive substances. The pathophysiology of reversible cerebral vasoconstriction syndrome is unknown, though temporary dysregulation in cerebral vascular tone is thought to be a key underlying mechanism. The syndrome typically follows a benign course; however, complications such as ischemic stroke or intracranial hemorrhage can cause permanent disability or death in a small minority of patients. Vascular imaging reveals alternating cerebral vasoconstriction and vasodilation that normalizes within 12 weeks. Calcium channel antagonists such as nimodipine reduce the frequency of thunderclap headaches but do not decidedly affect the risk of cerebral ischemia or hemorrhage. In this article the epidemiology, risk factors, pathophysiology, symptoms, diagnosis and treatment of RCVS is reviewed.

Primary stabbing headache in children and adolescents.

AIM: To examine the characteristics of primary stabbing headache (PSH) in typically developing children and adolescents. METHOD: In this prospective non-interventional hospital-based study, we examined 42 eligible individuals (24 females, 18 males; 26 white; mean age 12y 1mo, range 6y-16y 1mo) with spontaneous transient stabs. A headache focused history and/or a headache diary was obtained from each patient. All patients had normal neurological examination. Diagnosis of primary headache was based on the International Classification of Headache Disorders, Third Edition (ICHD-3) beta version. RESULTS: Duration of stabs ranged from a few seconds to up to 10 minutes. Stabs were located in a variety of sites on the cranium but mostly in the extra-trigeminal regions (n=28). Intensity of stabs varied from moderate (n=4) to severe (n=38). The frequency of the stabs ranged from daily to monthly. There were up to 50 stabs per headache attack. Stabs among our patients occurred independently from those caused by other primary headache types. Accompanying symptoms during stabs were reported by eight patients. Family history of primary headaches was identified (n=17). Familial transmission of PSH was recognized among two patients. All patients had normal brain magnetic resonance imaging. INTERPRETATION: The presentation and nature of PSH in children and adolescents varies widely. PSH in children may be a different entity to that in adults, and there is a need for further research to support changes in the ICHD-3 criteria for PSH in children and adolescents. WHAT THIS PAPER ADDS: Presentation of childhood primary stabbing headache (PSH) varies widely. Duration of PSH could last from a few seconds up to 10 minutes.

Nummular headache: a gender-oriented perspective on a case series from the RegistRare Network.

BACKGROUND: Nummular headache (NH) is a rare headache disorder characterized by a small, circumscribed painful area of the scalp. The description of many cases in the last years has supported its re-classification as a primary headache from the International Headache Society, moving it from its previous placement in the Appendix of the International Criteria of Headache Disorders. METHODS: Data were collected from a retro-prospective observational study about rare headaches promoted by the RegistRare Network, a collaborative group of seven Italian Headache Centres. According to the gender-biased profile of certain primary headaches, we have looked further NH patients from a gender perspective. RESULTS: Nineteen NH patients (11 men, 8 women) have been enrolled in the study. Headache onset was at 39 years and preceded approximately 8 years the diagnosis. No clinically evident differences between men and women have been found, including treatment prescriptions and headache resolution. Of note, the mean time from the onset of NH to the first visit in a Headache Centre was longer in men, compared with women (13.5 vs. 0.9 years). NH attacks were efficaciously treated with nonsteroidal anti-inflammatory drugs in 60% of patients receiving treatment. Headache prophylaxis with pregabalin and amitriptyline has been reported as effective in 40% and 67% of the treated patients, respectively. CONCLUSIONS: NH is a primary headache clinically heterogeneous in terms of temporal patterns and pain characteristics. Further research is needed to investigate the existence of male and female phenotypes, by clarifying whether it may be relevant for therapeutic purposes.

Diagnostic Testing for Migraine and Other Primary Headaches.

Most primary headaches can be diagnosed using the history and examination. Judicious use of neuroimaging and other testing, however, is indicated to distinguish primary headaches from the many secondary causes that may share similar features. This article evaluates the reasons for diagnostic testing and the use of neuroimaging, electroencephalography, lumbar puncture, and blood testing. The use of diagnostic testing in adults and children who have headaches and a normal neurologic examination, migraine, trigeminal autonomic cephalalgias, hemicrania continua, and new daily persistent headache are reviewed.

Other Primary Headaches: An Update.

"Other Primary Headaches" in the ICHD-3 encompasses activity-related headaches, headaches due to direct physical stimuli, epicranial headaches and a miscellanea, including hypnic headache and new daily-persistent headache. They can be primary or secondary and their etiologies differ depending on headache type. For instance, activity-related headaches can be induced by Valsalva maneuvers ("cough headache") or prolonged exercise ("exercise and sexual headaches"). Almost half of cough headaches are secondary to posterior fossa abnormality, whereas only 20% of exertional/sexual headaches are secondary, with subarachnoid hemorrhage the most frequent etiology. This article reviews the clinical diagnosis and management of these heterogeneous headaches.

Body Mass Index and Primary Headache: A Hospital-Based Study in China.

OBJECTIVE: Primary headache and obesity are highly prevalent disorders in the general population. Although many studies have reported an association between the two, there is still no overall comprehension about this relationship. To gain a more accurate understanding in this regard, we analyzed data from a 2011 cross-sectional study in Chongqing, China. METHODS: Patients with a chief complaint of headache were administered a headache questionnaire and diagnosed by neurology doctors in accordance with the International Classification of Headache Disorders 2nd Edition (ICHD-II) criteria. Patients aged < 18 years or diagnosed with secondary headache were excluded. RESULTS: Of 1327 patients who cited headache as the chief complaint, 16 were excluded for missing data, while 396 were diagnosed with chronic headache (177 chronic migraine [CM], 186 chronic tension-type headache [CTTH], and 33 other chronic headache) and 915 with episodic headache (369 episodic migraine [EM], 319 episodic tension-type headache [ETTH], and 227 other episodic headache). Chronic headache patients had a higher number of headache days per month, longer duration of headache history, and greater tendency to overuse analgesics than episodic headache patients. The CM and ETTH patients were more apt to be overweight and had a significantly greater body mass index (BMI; p < 0.05) than the EM and CTTH patients. Overweight (odds ratio [OR] = 3.64; 95% confidence interval (CI), 1.19-8.81) and obesity (OR = 28.63; 95% CI, 2.96-276.6) were independently associated with CM but not with other headaches, and this association was not influenced by other factors such as medication overuse. CONCLUSIONS: The relationship between headache and overweight/obesity varies depending on the type of primary headache. CM patients are more likely to have a higher body mass index than EM patients, while ETTH patients are more likely to be overweight/obese than CTTH patients.