Valentina Favoni

SUNCT/SUNA and neurovascular compression: New cases and critical literature review

Background Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache with cranial autonomic symptoms (SUNA) are primary headache syndromes. A growing body of literature has focused on brain magnetic resonance imaging (MRI) evidence of neurovascular compression in these syndromes. Objective The objective of this article is to assess whether SUNCT is a subset of SUNA or whether the two are separate syndromes and clarify the role of neurovascular compression. Method We describe three new SUNCT cases with MRI findings of neurovascular compression and critically review published SUNCT/SUNA cases. Results We identified 222 published SUNCT/SUNA cases. Our three patients with neurovascular compression added to the 34 cases previously described (16.9%). SUNCT and SUNA share the same clinical features and therapeutic options. At present, there is no available abortive treatment for attacks. Lamotrigine was effective in 64% of patients; topiramate and gabapentin in about one-third of cases. Of the 34 cases with neurovascular compression, seven responded to drug therapies, 16 patients underwent microvascular decompression of the trigeminal nerve (MVD) with effectiveness in 75%. Conclusions We suggest that SUNCT and SUNA should be considered clinical phenotypes of the same syndrome. Brain MRI should always be performed with a dedicated view to exclude neurovascular compression. The high percentage of remission after MVD supports the pathogenetic role of neurovascular compression.

Migraine and sleep disorders

The burden of migraine strongly increases, considering its linkage with sleep disorders. Migraine is positively associated with many sleep-complaint disorders; some are confirmed by several studies, such as restless leg syndrome, whereas others still remain uncertain or controversial, e.g. narcolepsy. Many studies have investigated the association between headache and other sleep disturbances such as daytime sleepiness, insomnia, snoring and/or apnea, but only a few have focused on migraine. Highlighting the comorbidity between migraine and sleep disorders is important to improve treatment strategies and to extend the knowledge of migraine pathophysiology.

Nociception and autonomic nervous system.

The International Association for the Study of Pain (IASP) defines pain as “an unpleasant sensory and emotional experience associated with actual or potential tissue damage, or described in terms of such damage”. Pain may also be experienced in absence of noxious stimuli and together with temperature and other bodily feelings constitute the interoception redefined as the sense of the physiological condition of the entire body, not just the viscera. The main characteristic of these feelings is the affective aspect. Emotion, motivation, and consequent behavior connected with these feelings characterize their homeostatic role. This implies an interaction between neural structures involved in pain sensation and autonomic control. The aim of this review is to focus on pain perception, mainly on pain matrix structures’ connections with the autonomic nervous system.

Cerebrospinal fluid hypocretin-1 levels during the active period of cluster headache.

Background: Hypocretins (orexins) are hypothalamic neuropeptides which are involved in a wide range of physiological processes in mammals including central pain processing. Genetic studies in humans evidenced a role for the hypocretinergic system in cluster headache (CH). Patients and methods: We tested cerebrospinal fluid (CSF) hypocretin-1 (orexin-A) levels in 10 CH patients during an active cluster period. CSF hypocretin-1 levels were measured by radioimmunoassay. Results: CSF hypocretin-1 levels were within the normal range (mean 457.3 ± 104.98 pg/ml, range 304–639) in our 10 patients, with a slight reduction in one case (304 pg/ml). There were no associations between CSF hypocretin-1 levels and the clinical features of CH. A trend towards higher hypocretin-1 levels was disclosed in patients with chronic CH compared to episodic CH. Conclusions: CSF hypocretin-1 levels seem not to influence the clinical course of CH, but our results cannot completely exclude a functional involvement of the hypothalamic hypocretinergic system in the pathogenesis of CH.

Rotigotine Objectively Improves Sleep in Parkinson's Disease: An Open-Label Pilot Study with Actigraphic Recording

Sleep disturbances represent important predictors of poor quality of life (QoL) in Parkinsons disease (PD). This open-label pilot study aimed to objectively assess, by means of actigraphic recording, effect of rotigotine on sleep in PD patients with self-reported sleep complaints. 15 PD patients underwent one-week actigraphic recording before (T0) and during (T1) rotigotine treatment, which was titrated to the dose subjectively improving motor symptoms (4–8 mg/24 h). Sleep disturbances, daytime sleepiness, cognitive performance, QoL, and depression were also evaluated with questionnaires. Actigraphic recordings showed a significant reduction in nocturnal motor activity and mean duration of wake episodes after sleep onset during rotigotine treatment compared to baseline. In 10 patients presenting objective evidence of poor sleep quality at T0 (sleep efficiency ≤ 85%), rotigotine also significantly improved other sleep parameters and further reduced nocturnal motor activity and mean duration of wake episodes. A significant decrease in number and duration of daytime sleep episodes was also observed at T1. Finally we confirmed that rotigotine significantly improves perceived sleep quality and QoL. Our study showed for the first time that rotigotine is associated with an objective improvement of nocturnal and diurnal sleep disturbances in PD patients with self-reported sleep complaints. This study is registered with AIFA-observational study registry number 12021.

Migraine and cardiovascular diseases

Migraine has complex relationships with cerebrovascular and cardiovascular disorders but also with cardiac anomalies. Patients affected by migraine with aura have an increased prevalence of right-to-left shunt due to patent foramen ovale or pulmonary arteriovenous malformations. The association between ischemic heart disease, cardiovascular mortality and migraine remains unsettled. The debate focuses on a physiopathological link between migraine and cardiovascular diseases or a higher prevalence of risk factors in migraineurs.

Superficial siderosis associated with peripheral autonomic failure and tetraventricular hydrocephalus: a case report

We describe the case of a man whose initial clinical presentation included sensorineural hearing loss and orthostatic hypotension. The patient was diagnosed with superficial siderosis associated with peripheral autonomic failure and tetraventricular hydrocephalus.

P037. Headache in multiple sclerosis: prevalence and clinical features in a case control-study

Methods One hundred and fifty adults (F/M = 98/52; mean age 40 years) with a diagnosis of MS and 150 sex and agematched controls (F/M = 101/49; mean age 40 years) from the general population were evaluated by means of an ad hoc semi-structured interview according to the International Classification Headache Disorders (ICHD-3-beta) criteria. All subjects filled out validated questionnaires about fatigue, Fatigue Severity Scale (FSS) and Modified Fatigue Impact Scale (MFIS). The c2 and Kruskal-Wallis tests were used when appropriate. Results The two groups differed significantly for education level and employment. Among the 150 patients with MS, 1 (0.7%) presented a radiologically isolated syndrome (RIS), 17 (11.3%) a clinically isolated syndrome (CIS), 20 (13.3%) a primary progressive form (PPMS), 96 (64%) a relapsing remitting form (RR), and 16 (10.7%) a secondary progressive form (SPMS). Headache was reported by 80 (53.3%) MS cases and 71 controls (47.3%), (p = 0.356); migraine was reported by 47 (31.33%) cases and 51 (34%) controls, tension-type headache was present in 21 (14%) MS affected vs 14 (9.33%) controls (p = 0.245). The simultaneous presence of migraine and tension-type headache was statistically higher (p = 0.002) in MS (28.8%) compared to controls (8.5%). Women with MS presented a low correlation between migraine and menstruation compared to controls while migraine normally improves during pregnancy as much as in controls (p = 0.65). The preliminary analysis of FSS and MFIS scores showed that fatigue resulted overall higher in MS patients with or without headache. Conclusions

Recurrent Reversible Cognitive Impairment in a Cluster Headache Patient: A Case Report: Headache

Cluster headache (CH) is a disabling primary headache disorder sometimes dubbed the “suicide headache.” CH is characterized by a severe or very severe unilateral head pain lasting from 15 to 180 minutes with a frequency ranging from once every other day to 8 times daily, accompanied by ipsilateral autonomic symptoms and/or restlessness or agitation. Behavior during attacks may range to include not just restlessness but a variety of complex, even violent, self-injurious behaviors and even suicide attempts. CH has been previously associated with psychiatric comorbidities such as depression, anxiety disorders, and aggressiveness. However, little is known about cognition in CH patients. Here we report a patient presenting with progressive worsening of cognitive functions and behavioral modifications during CH bouts. An extensive neuropsychological assessment, including global cognition indexes, verbal and visual memory, attention, executive and visuospatial function, language, and depression, was performed during 2 active CH periods, and during a pain-free period. Selective deficits in attention and executive functions were found only during the active CH bouts. We describe a 50-year-old man with a medical history of hypertension presenting with a 6-year history of episodic CH. He described strictly right-sided episodes of severe pain occurring in the periorbital area, accompanied by ipsilateral lacrimation, conjunctival injection, eyelid edema, and nasal congestion. The attacks had prominent psychiatric features with agitation and violent self-injurious behaviors. The pain occurred only during the daytime. The onset was never at night. The attacks usually lasted from 30 minutes to 2 hours when untreated, occurring with a frequency of one every other day, for nearly a month once a year. Attacks responded to subcutaneous sumatriptan 6 mg and high-flow inhaled oxygen. A trial with quietapine (up to 100 mg/die) was added to a From the IRCCS Institute of Neurological Sciences of Bologna, UOC Clinica Neurologica, Bologna, Italy (V. Favoni, L. Sambati, F. Oppi, M. Stanzani Maserati, S. Cevoli, and G. Pierangeli); Department of Biomedical and NeuroMotor Sciences (DiBiNeM), Alma Mater Studiorum University of Bologna, Italy (V. Favoni, L. Sambati, and G. Pierangeli).

Idiopathic Intracranial Hypertension Without Papilledema (IIHWOP) in Chronic Refractory Headache

Background: To determine the prevalence of Idiopathic intracranial hypertension without papilledema (IIHWOP) testing revised diagnostic criteria by Friedman in refractory chronic headache (CH) patients. Methods: This is a prospective observational study. Each patient underwent ophthalmologic evaluation and Optical Coherence Tomography; brain magnetic resonance venography (MRV) and a lumbar puncture (LP) with opening pressure (OP) measurement. CSF withdrawal was performed in patients with CSF OP > 200 mmH20. IIHWOP was defined according Friedmans diagnostic criteria. Effect of CSF withdrawal was evaluated clinically in a 6-month follow-up and with a MRV study at 1 month. Results: Forty-five consecutive patients were enrolled. Five were excluded due to protocol violations. Analyses were conducted in 40 patients (32 F, 8 M; mean age 49.4 ± 10.8). None had papilledema. Nine patients (22.5%) had OP greater than 200 mmH2O, two of them above 250 mmH2O. Two (5%) had neuroimaging findings suggestive of elevated intracranial pressure. One of them (2.5%) met the newly proposed diagnostic criteria by Friedman for IIHWOP. After CSF withdrawal seven (77.8%) of the nine patients improved. No changes in neuroimaging findings were found. Conclusions: We found a low prevalence (2.5%) of IIHWOP in refractory CH patients according to current diagnostic criteria. In agreement with Friedmans criteria, our results confirm that a diagnosis of IIHWOP should be based on CSF OP and the combination of neuroradiological findings. However, where to set the CSF OP upper limit in IIHWOP needs further field testing. Although IIHWOP is a rare clinical condition, it should be considered and treated in refractory CH patients.