Elio Agostoni

Atypical facial pain: clinical considerations and differential diagnosis

Atypical facial pain (ATFP), recently defined as persistent idiopathic facial pain by the revision of the Classification of the International Headache Society (IHS), is a poorly understood condition, which still lacks clear diagnostic criteria and proper treatment. The pain is described as “persistent facial pain that does not have the characteristics of cranial neuralgias and is not attributable to another disorder”. In general, however, according to the IHS criteria, a diagnosis of ATFP is possible when the pain in the face is present daily and persists for most or all of the day. The pain is confined at onset to a limited area on one side of the face, often in the nasolabial fold or side of the chin and may spread to the upper or lower jaw or a wider area of the face of neck and is deep and poorly localised. It is not associated with sensory loss or other physical signs. Laboratory investigations including X-ray of face and jaws do not demonstrate relevant abnormality. Pain may be initiated by operation or injury to face, teeth or gums but persists without any demonstrable local cause. But, the definition and the diagnostic criteria are over-simplified when we face the reality of the clinical practice. Many different disorders may be included in this diagnostic category, making differential diagnosis very complex. Diagnosis of ATFP is therefore, usually, a process of elimination. A targeted history and an accurate examination are crucial to correctly classify this facial pain.

Migraine and stroke

Abstract.The association between migraine and stroke is a dilemma for neurologists. Migraine is associated with an increased stroke risk and it is considered an independent risk factor for ischaemic stroke in a particular subgroup of patients. The pathogenesis is not known but several studies report some common biochemical mechanisms between the two diseases. A classification of migraine-related stroke that encompasses the full spectrum of the possible relationship between migraine and stroke has been proposed and it includes three main entities: coexisting stroke and migraine, stroke with clinical features of migraine, and migraine-induced stroke. The concept of migraine-induced stroke is well represented by migrainous infarction; it is described in the revised classification of the International Headache Society (IHS), and it represents the strongest demonstration of the relationship between ischaemic stroke and migraine. A very interesting common condition in stroke and migraine is patent foramen ovale (PFO) which could play a pathogenetic role in both disorders. The association between migraine and cervical artery dissection (CAD) is reported in recent studies. Migraine is more frequent in patients with CAD. This supports the hypothesis that an underlying arterial wall disease could be a predisposing condition for migraine. The neuroradiological evidence of subclinical lesions most typical in the white matter and in the posterior artery territories in patients with migraine, opens a new field of research. In conclusion the association between migraine and stroke remains conflicting. Solving the above mentioned issues is fundamental to understanding the epidemiologic, pathogenetic and clinical aspects of migraine-related stroke.

Migrainous cerebral infarction: case reports

Abstract.Migraine is a common and chronic disorder. It is considered benign but several studies have suggested it as a rare risk factor for ischaemic stroke. The association is still conflicting and seems to be restricted to particular subgroups of patients (i.e., women under the age of 45, with migraine with aura, and particularly ones who smoke and use oral contraceptives). The pathogenetic mechanisms underlying this condition are not known. We describe 6 cases of migrainous stroke fully meeting the diagnostic criteria of the International Headache Society (IHS). For each patient, demographic and anamnestic data, clinical features, results of laboratory tests and neuroimaging findings were recorded. Five of the 6 cases were women (median age of 29, range from 23 to 40). The man was 36. All patients fulfilled the IHS criteria for migraine with aura. At the time of the event, 2 patients were taking oral contraceptives and smoked, one patient smoked and three patients had no vascular risk factors. The stroke manifested as homonymous hemianopia in 3 patients, lower homonymous quadrantopia in 1 patient and sensory symptom in 1 patient. The neurological examination was normal in 1 case. All patients underwent several tests with negative results: blood test (antithrombin III, protein C or S, autoantibodies), transthoracic and transoesophageal echocardiography, extracranial and intracranial Doppler sonography, and angiography which was not performed in 1 patient. All patients had a cerebral infarct visible on neuroimaging study (MRI): posterior cerebral artery in 4, middle cerebral artery in 1 and anterior cerebral artery in 1. We support the findings reported by others that migrainous stroke is more common in young women affected by migraine with aura. In consideration of the high prevalence of migraine in the population, further research is indicated and necessary to establish if migraine is independent from other vascular risk factors.

Cluster Headache and Internal Carotid Artery Dissection : Two Cases and Review of the Literature

We describe 2 patients with cluster headache attacks associated with a dissection of the ipsilateral internal carotid artery at the extra–intracranial passage. These cases highlight the need for extensive neuroradiological investigation in cluster headache patients when atypical features are present. We also performed a PubMed search to review the current literature data about this association.

Persistent Idiopathic Facial Pain

CLINICAL HISTORY A 57-year-old woman was seen for a third neurological opinion with a 1 to 1 2 year history of facial pain. She described a dull ache or burning around the medial canthus of the left eye, the left side of the nose, the left naso-labial fold, and the left side of the lip. The pain typically started about 8 AM and had an intensity of 9/10 all day. Amitriptiline, nortriptyline, gabapentin, zonisimide, levetiracetam, duloxetine, and tramadol all in moderate-to-high doses have proven ineffective. Hydrocodone, it was reported, moderately decreased the pain. A left upper molar extraction did not help. Cryoablation of the left infraorbital nerve was of no benefit. An MRI scan of the brain with and without contrast was normal including views of the trigeminal

Controversies in optic neuritis pain diagnosis

Optic neuritis (ON) refers to any inflammatory disorder of the optic nerve. In clinical practice ON is mainly diagnosed by ophthalmologists and less frequently by neurologists. ON diagnostic criteria are included in different classification systems both in neurologic and ophthalmologic fields. Diagnosis of ON is still very unsatisfactory. Indeed diagnostic criteria are not uniform and therefore the diagnosis is still mainly formulated according to the clinical experience only. A consensus on practice guidelines for ON diagnosis might be useful. Ocular pain is a milestone in ON diagnosis, but it is too often mistreated by both the patient and the clinician. The International Headache Society (IHS) Classification of Headache Disorders provides in its 1988 and 2004 versions the diagnostic criteria for ON. These criteria are not spread and followed by the large majority of neurologists, but they are mainly applied by the experts in headache disorders. On the other hand, ON is a disorder widely encountered by neurologists and ophthalmologists. The latest IHS version defines the criteria of the pain features more precisely, but it is still unsatisfactory. In a future revision, the pain should be further detailed. Further studies aimed at validation of the diagnostic criteria of ON are strongly needed.

Visual evoked potentials in migraine

Abstract.Migraine is a chronic disorder. Visual symptoms and hypersensitivity to light stimuli are common. The aim of this study is the analysis of visual system in migraineurs by visual evoked potentials (VEP). We studied 53 migraineurs (21 with prophylactic migraine treatment and 32 without preventive therapy) and 20 healthy control subjects. We found lower P100 latencies in migraineurs without therapy compared to controls. In treated patients, P100 latencies showed the same trend seen in the control group. We speculate a different responsiveness of the visual system in migraineurs probably due to a dysmodulation of sensor input leading to facilitation of visual processing.

Cerebral venous thrombosis

Cerebral venous thrombosis (CVT) was formerly considered a rare disorder, associated with an unfavorable outcome. More recent data based on modern imaging techniques, however, have changed our perception of this disorder. The use of angiography and, especially, MRI have allowed an early diagnosis and have proved that the incidence of CVT is, in fact, higher than previously thought, approximately 3–4 cases per million people per year, and that the majority of patients have a favorable outcome. At present, the most frequent causes are oral contraceptives assumption and pregnancy/puerperium; as a consequence, 75% of patients are females. CVT may cause isolated intracranial hypertension or lead to an ischemic stroke, which does not follow the distribution of an arterial vessel and has a relevant vasogenic edema. Venous strokes often have a hemorrhagic component, ranging from small petechiae to an actual intracerebral hemorrhage; the latter is associated with a worse clinical course. The clinical presentation of CVT is hihgly variable and includes patients with just a mild headache, others with focal neurological deficits and a few with a dramatic syndrome with coma; seizures are a frequent presenting symptom. The best radiological examination to confirm the suspicion of CVT is MRI of the brain, which can both demonstrate parenchymal lesions and directly show evidence of sinus occlusions. The available evidence suggests that anticoagulants are effective in reducing mortality and dependency in CVT patients; the possible role of systemic or localized thrombolysis is still to be established.

Intravenous thrombolysis and intra-arterial interventions in acute ischemic stroke: Italian Stroke Organisation (ISO)-SPREAD guidelines.

i.v. Thrombolysis (IVT) is the most important achievement of the last 20 years in the field of ischemic stroke management. In Italy, the evidence that stroke units were effective per se in improving stroke outcome was not sufficient to favor their implementation. Only the approval of IVT boosted the activation of stroke units, which are now 170 centers widespread over the country. The numbers of treatments, however, are still limited, amounting in 2014 to approximately 4200 out of the 10 000 which should theoretically be performed each year. Too strict exclusion criteria and/or their too restrictive interpretation are two of the main causes of this substantial undertreatment. Hence, a critical reappraisal of these criteria was necessary. On the other hand, recent evidences on the potentialities of intra-arterial interventions made it mandatory to better define the role of these techniques in the chain of treatments for acute ischemic stroke. A panel of vascular neurologists (D. T., D. I., A. C., E. A., P. C., A. Z.) and of interventional neuro-radiologists (S. M., S. V., M. B.) collected the data through a systematic review of the available literature, searching electronic databases including PubMed, EMBase, OVID, and Cochrane Library, up to May 2015. Reference lists of the selected articles were also scrutinized. Each panelist was assigned individual sections, then the panel assessed the complete guidelines. Recommendations were formulated by integrating the principles of the Scottish Intercollegiate Guideline Network with the statistical considerations suggested by the Centre for EvidenceBased Medicine methodology (Table 1). When literature data and practice experience data were not available or not considered to be sufficient, no specific recommendation was made. Consensus was reached during face-to-face discussions. In case of disagreement, a majority decision was taken. Recommendations were then revised by a larger group of experts pertaining to the fields of trial methodology, vascular neurology, cardiology, emergency medicine, neurosurgery, vascular surgery, neuro-radiology, and rehabilitation (see Appendix S1), which in particular checked for the congruence between the grading and the source data.

Migraine and hypertension

Although the possibility of a comorbidity between migraine and hypertension has long been suspected, the epidemiologic evidence is controversial, with studies demonstrating positive, negative or no correlation between the two diseases. A unifying view that takes into account the most recent evidence suggests that there might be a different effect of diastolic and systolic pressure, with the former having a positive and the latter a negative correlation with migraine. In this paper, the methodologic and clinical reasons for the discrepancies in epidemiologic studies are discussed, together with the possible biological mechanisms that might account for the migraine-hypertension correlation. One such mechanisms may be the renin angiotensin system, which is certainly involved in hypertension and has activities in the CNS that may be relevant for migraine pathogenesis. Despite the uncertainty still present in this field, the control of hypertension in migraine patients is an important factor for the success of migraine treatment and to lower cerebrovascular risk.