Small spheno-ethmoidal meningoencephalocele versus ethmoidal mucocele in spontaneous intracranial hypotension.

Background: Meningoencephalocele is defined as an abnormal sac of brain tissue and meninges extending beyond natural skull margins, often leading to cerebrospinal fluid (CSF) leakage. When this condition arises in the spheno-ethmoidal region, the diagnosis becomes more challenging as it can be mistaken for other nasal pathologies, such as mucocele. Research question: We show in this case report a non-congenital sphenoethmoidal meningoencephalocele causing rhinoliquoral fistula and spontaneous intracranial hypotension. Results: this 65-year-old woman presented with sporadic rhinoliquorrhoea associated with orthostatic headache, nausea and dizziness. Brain MRI revealed a small lesion of an ethmoidal sinus, which was successfully treated with endoscopic endonasal surgery. Histology confirmed the presence of meningoencephalic tissue positive for S100 protein on immunohistochemistry. Conclusions: When dealing with lesions of the paranasal sinuses in contact with the anterior skull base, rhinoliquorrhoea presence suggests meningoencephalocele. In dubious cases, a proper workup, including a thorough clinical history and neurological examination, specific imaging, and a direct search of CSF-like markers, is essential to support the differential diagnosis. In such cases, a transnasal endoscopic surgical approach is recommended to obtain a final histological diagnosis and to perform eventual dural plastic surgery.

Headache attributed to IIH: clinical evolution in IHS criteria through the years.

Idiopathic intracranial hypertension (IIH) is a condition characterized by increased intracranial pressure without a detectable cause. The most common symptom of IIH is a headache, which occurs in almost all cases at the time of diagnosis with various headache phenotypes. In clinical practice, diagnosis of headache attributed to IIH is given referring to the International Headache Society (IHS) criteria of the International Classification of Headache Disorders. In the present publication, we consider how the IHS diagnostic criteria for headache attributed to intracranial idiopathic hypertension have evolved through the years.

When does the brain choose pain?

Why does the brain choose pain? Why does an organ that is able to mask pain, even when intense as in fractures or in fighting wounds, decide to let pain pass and begin conscious, such as that of migraine, when there is no noxa patogena and there is no threat to the integrity of the organism, failing in the main function of pain, that of protection? In this brief review, we retrace the journey that led to the identification of the first complex mechanism of regulation of painful input, the spinal gate control system, through the identification of the predominantly thalamocortical supraspinal centers of the neuromatrix, up to the recognition of a pain matrix extremely articulate and sophisticated that integrates elementary sensations with much more complex functions, related to memory, affectivity, emotion, autonomic self-regulation, and homeostasis systems and so on. Why does the protection system lose its fundamental function in migraine in a behavioral harakiri that periodically damages only itself? This is the challenge facing those dealing with primary headaches in the next future: why migraine? The great strides made in the last decades that have led to the understanding of complex pathogenetic mechanisms risk remaining orphans if we fail to identify the primum movens at the base of one of the most common pathologies in the human race.

Chiari malformation-related headache: outcome after surgical treatment.

The outcome of headache in a series of 135 operated CM1 is presented. Favorable results were obtained in 85% of atypical and 93% typical headache with the support of a multidisciplinary approach that restricted the indications for surgery.

Secondary chronic cluster headache treated by posterior hypothalamic deep brain stimulation: first reported case.

INTRODUCTION: Deep brain stimulation (DBS) of the posterior hypothalamus (pHyp) has been reported as an effective treatment for primary, drug-refractory and chronic cluster headache (CCH). We here describe the use of such a procedure for the treatment of secondary CCH due to a neoplasm affecting the soft tissues of the right hemiface. METHODS: A 27-year-old man affected by infiltrating angiomyolipoma of the right hemiface who subsequently developed drug refractory homolateral CCH underwent DBS of the right pHyp region at the Fondazione IRCCS Istituto Nazionale Neurologico Carlo Besta. RESULTS: After surgery, the patient presented a significant reduction in frequency of pain bouts. However, because of a subsequent infection, the entire system was removed. After re-implantation of the system, successful outcome was observed at 2 years follow-up. DISCUSSION: This brief report shows the feasibility of pHyp DBS in secondary drug-refractory CCH syndromes; future reports are needed in order to confirm our positive result.

Treatment of chronic headache of cervical origin with lipostructure: an observational study.

OBJECTIVE: To test feasibility, safety, and efficacy of local transplant of stromal fraction of adipose tissue in the treatment of chronic headaches of cervical origin. BACKGROUND: Chronic headaches of cervical origin (chronic cervicogenic headache and occipital neuralgia) are characterized by persistent pain due to the involvement of the great occipital nerve, with concurrent myofascial spasm and the consequent nerve entrapment within the trapezoid tunnel. METHODS: Tolerability and effectiveness of treatment of chronic cervicogenic headaches refractory to conventional therapies were evaluated in 24 patients. The visual analog scale of pain and the medication use diary were used in the 3 months preceding treatment; moreover, in order to verify the quality of life, patients are required to fill before surgery the neck pain disability index, the headache disability index, migraine disability assessment scale questionnaire, and the short-form 12 standard v1 questionnaire. Follow-up examination was performed at 3 and 6 months. RESULTS: In 19 cases (79.2%), a good clinical response was recorded. At 6-month follow-up analysis, recurrence of occipital pain was recorded in 7 cases (29.2%); there is a significant reduction in disability and pain scores, and also a significant reduction of need for pharmacologic treatment and a fast return to previous work capacities. CONCLUSIONS: The key point of our therapeutic strategy might be the regenerative role of stromal fraction of adipose tissue transplanted in the area of the occipital nerve entrapment; the results of the present study are encouraging both in terms of reduction of pain scores and in terms of quality of life improvement. The technique is minimally invasive, and no complications were recorded; indeed, the procedure seems to be safe and effective, and thus, a randomized study with larger follow-up and in a large series will be started.

How to investigate and treat: headache and hyperprolactinemia.

Hyperprolactinemia is a condition characterised by an increase of prolactin blood levels (more than 100-200 ng/ml). It is the most common endocrine disorder of the hypothalamic-pituitary axis. The clinical characteristics of the headache-hyperprolactinemia-hypophyseal-adenoma association are discussed, the various diagnostic and treatment possibilities are explored and the etiology of the headache is considered in the light of several pathogenetic possibilities. We present two cases. (1) A 35-year-old woman suffering from chronic tension-type headache interspersed with occasional episodes of migraine without aura (as defined by the International Headache Society criteria). She had also suffered menstrual cycle alterations since the age of 16. At the age of 30 she developed amenorrhea with hyperprolactinemia. Computed tomography (CT) and magnetic resonance imaging (MRI) scans revealed a median-left intrasellar mass. Treatment with cabergoline resulted in complete resolution of both types of headache and the menstrual cycle and prolactin levels returned to normal. The therapy also reduced the volume of the tumour. (2) The second case relates to a 47-year-old man who had been suffering from tension-type headaches for almost 3 months. The patient had never previously suffered from headaches. CT and MRI scans showed a large sellar and suprasellar lesion with raised serum prolactin levels. Treatment with cabergoline had significantly reduced the prolactin levels and had also improved the patient's headaches. High-resolution CT, with and without contrast, or MRI is necessary to visualise microprolactinomas (and other sellar lesions) and confirm the diagnosis.

ICF in neurology: functioning and disability in patients with migraine, myasthenia gravis and Parkinson's disease.

PURPOSE: To report and compare functional features of patients with migraine, myasthenia gravis (MG) and Parkinson's disease (PD) with the International Classification of Functioning, Disability and Health (ICF). METHOD: Adult patients with migraine, MG and PD were enrolled and the ICF checklist administered. Count-based indexes were calculated for each ICF chapter and domain. Indexes were compared across conditions by means of ANOVA; relationships between ICF domains were evaluated using Spearman's correlation; group based on disability status were defined through cluster analysis and compared with disease groups using chi(2) test. Finally, most prevalent ICF categories were identified. RESULTS: A total of 300 patients were enrolled and specific differences in BF, BS, A&P and EF indexes are reported. Spearman's correlations reported moderate relationships between BF and A&P indexes, whereas the correlation between A&P and EF is lower. Cluster analysis and chi(2) test show that patients with Migraine and MG are more likely to report moderate and low disability, whereas patients with PD are more likely to report moderate or severe disability. A total of 60 ICF relevant categories, mostly from A&P, were identified. CONCLUSIONS: Our study provided a description of functioning and disability domains in migraine, MG and PD and enabled to report the impact of EF in determining the actual disability experience.

A novel de novo nonsense mutation in ATP1A2 associated with sporadic hemiplegic migraine and epileptic seizures.

Familial hemiplegic migraine (FHM) is a severe dominant form of migraine with aura associated with transient hemiparesis. Several other neurological signs and symptoms can be associated with FHM such as cerebellar abnormalities, cerebral edema and coma after minor head trauma, epileptic seizures and mental retardation. The sporadic form of hemiplegic migraine named SHM, presents with identical clinical symptoms. Here we report a case of a young hemiplegic migraine patient, 11 years old, who had the first hemiplegic attack at the age of 10 years. This patient has a clinical history of epileptic seizures in the childhood successfully controlled with drug therapy. No familiarity for any type of migraine or seizures can be observed within the paternal or maternal line. The patient who can therefore be considered a sporadic case, carries a novel de novo nonsense mutation p.Tyr1009X in the ATP1A2 gene (FHM2), leading to a truncated alpha-2 subunit of the Na+/K+-ATPase pump thus lacking the last 11 amino acids. The novel mutation identified confirms the role of FHM2 gene in forms of hemiplegic migraine associated with epilepsy with both familial and sporadic occurrence, and expands the spectrum of mutations related to these forms of the disease.

ATP1A2 gene mutations are not present in two sisters with basilar-type migraine associated with menses.

Basilar-type migraine (BM) and hemiplegic migraine are clinically distinct subtypes of migraine with aura, however they do share clinical features and it is possible they may share genetic bases. In recent years, ATP1A2 and other gene mutations have been discovered in familial and sporadic hemiplegic migraine. More recently, an ATP1A2 mutation has been identified in an Italian family with BM. In this study we document the absence of ATP1A2 mutations in two Italian sisters with menstrual BM, suggesting that other genes are involved in the condition.

Hypothalamic stimulation for intractable cluster headache: long-term experience.

The authors report long-term results of continuous hypothalamic stimulation in 16 chronic drug-refractory patients with cluster headache (CH). At a mean follow-up of 23 months, 13 patients are persistently pain-free or almost pain-free, and the other 3 are improved. There are no persistent side effects. Hypothalamic stimulation is an effective, safe, and well-tolerated alternative to surgery for chronic patients with drug-refractory CH.

Migraine, celiac disease, and cerebral calcifications: a new case.

Celiac disease is sometimes associated with neurological complications. Only one case of celiac disease associated with migraine and cerebral calcifications has been reported. We present a patient with migraine (with and without aura) in whom neuroimaging revealed cerebral calcifications in the occipital and parietal regions bilaterally. Visual examination showed bilateral double scotoma, and further investigations revealed celiac disease. Migraine may on occasion be a sentinel symptom of mild celiac disease.

Plasma cytokine levels in migraineurs and controls.

BACKGROUND: The vasoactive peptide, calcitonin gene-related peptide (CGRP), is released from primary afferent neurons in the trigemino-vascular circulation during migraine headache. CGRP at physiological concentrations and possibly via stimulation of its selective receptors on T-cells, triggers the secretion of cytokines. Cytokines play an important role in several physiological and pathological settings such as immunology, inflammation, and pain. OBJECTIVE: To investigate plasma levels of pro- and anti-inflammatory cytokines in migraineurs and healthy controls. METHODS: We studied 25 migraine patients, during and outside attacks, and 18 healthy control subjects measuring plasma levels of IL-6, IL-10, tumor necrosis factor alpha (TNFalpha), IL-4, IL-1 beta, and IL-2 using ELISA. RESULTS: Circulating levels of IL-10, TNFalpha, and IL-1 beta during attacks were significantly higher in comparison to their levels outside attacks (P=.0003, P=.03, and P=.05, respectively). IL-10 and TNF serum levels were higher in patients studied soon after headache onset and lower over time (P=.004 and P=.05). CONCLUSION: Our results suggest that TNFalpha, IL-1 beta, and IL-10 may be involved in the pathogenesis of migraine attacks.

Cervicogenic headache: a critical review of the current diagnostic criteria.

Opinions are divided on the use of the term cervicogenic headache (CGH) in cases with no evidence of cervical damage. According to Sjaastad et al. (1990), CGH is diagnosed from three features: (1) unilateral headache triggered by head/neck movements or posture; (2) unilateral headache triggered by pressure on the neck; (3) unilateral headache spreading to the neck and the homolateral shoulder/arm. Other characteristics are not essential for CGH diagnosis, including pain improvement after greater occipital nerve (GON)/C2 block. However, other authors give different definitions of CGH, and this may explain why reported frequencies for this headache vary so widely. In this paper we critically review the major diagnostic criteria of Sjaastad et al. for CGH in the light of clinical studies conducted at our institute and other literature findings. In a study of 500 headaches we found only two patients with unilateral headache triggered by head/ neck movements or posture, and no cases of neck pressure-induced headache. No clear-cut criteria are given in the literature for differentiating CGH trigger points from myofascial trigger points. In another study of 440 primary headache patients we found that in the unilateral long-lasting headache group (64 migraines and 10 tension-type headaches), a pain involving the occiput/neck was present in 30 migraine and seven tension headache patients; thus, according to the CGH major criteria, 10% (30/307) of 'migraines' and 7% (7/96) of 'tension headaches' could be diagnosed as CGH. However, one cannot exclude that the association of unilateral pain with posterior irradiation is due to the high prevalence of migraine, tension-type headache and chronic neck pain. The relation between CGH and whip-lash injury has been put in doubt by a recent study which found no difference in headache frequency between trauma and control groups and reported no specific headache pattern in the trauma group. Other reports suggest that, when it occurs, CGH usually disappears within a year of whip-lash, throwing doubt on the appropriateness of surgery for post-traumatic CGH. The lack of specificity of GON/C2 block as a treatment for CGH adds further difficulties to the diagnosis of this headache. We conclude that, although neck structures play a role in the pathophysiology of some headaches, clinical patterns indicating a neck-headache relationship have still not been adequately defined. We believe that further rigorous studies are needed to definitively confirm the validity of CGH as a nosological entity.