White matter lesions progression in migraine with aura: a clinical and MRI longitudinal study.

OBJECTIVE: To prospectively evaluate longitudinal changes in white matter lesions (WMLs) in migraineurs with aura, by magnetic resonance imaging (MRI), and to correlate WMLs modifications with patients' clinical characteristics. METHODS: Forty-one consecutive migraineurs with aura were followed for a mean time of 33.2 months. Patients underwent MRI at baseline and follow-up and were evaluated for cerebrovascular risk factors. Presence of WMLs on MRI was assessed by two neuroradiologists. RESULTS: WMLs were present in 26 subjects (63.4%) at baseline MRI. At follow-up a total of 8 patients had new WMLs (19.5%). There was a significant correlation between aura duration and number of new WMLs, and between the number of migraine attacks with aura and new WMLs. CONCLUSIONS: Our study demonstrates that in migraine with aura WMLs number can progress over time and suggests an association between aura features and WMLs progression. Studies with a higher number of patients are required to confirm these findings.

Cortical subarachnoid hemorrhage associated with reversible cerebral vasoconstriction syndrome after elective triplet cesarean delivery.

Reversible cerebral vasoconstriction syndromes (RCVS) comprise a group of disorders characterized by prolonged, but reversible vasoconstriction of the cerebral arteries, usually associated with acute-onset, severe, recurrent headaches, with or without additional neurological signs and symptoms. Various complications of this condition have been observed, such as cortical subarachnoid hemorrhages (cSAH), intracerebral hemorrhages, reversible posterior leukoencephalopathy, ischaemic strokes and transient ischaemic attacks. It is important to include RCVS in thunderclap headache differential diagnosis and among non-aneurismatic subarachnoid hemorrhage causes. In the past years, thanks to the major diffusion of new diagnostic tools such as magnetic resonance, computed tomography and digital subtraction angiography, RCVS have been demonstrated to be more frequent than previously thought. We report an illustrative case of a woman affected by a small cSAH, associated to RCVS, after elective triplet cesarean delivery. To our knowledge, this is the first case of cSAH associated to RCVS after a triplet pregnancy.

Post-coital intra-cerebral venous hemorrhage in a 78-year-old man with jugular valve incompetence: a case report.

INTRODUCTION: Spontaneous intra-cerebral hemorrhage can occur in patients with venous disease due to obstructed venous outflow. CASE PRESENTATION: We report the case of a 78-year-old Caucasian man with jugular valve incompetence who experienced an intra-cerebral temporo-occipital hemorrhage following sexual intercourse. He had no other risk factors for an intra-cerebral hemorrhage. CONCLUSIONS: To the best of our knowledge, this is the first case of intra-cerebral hemorrhage due to jugular valve incompetence in association with the physical exertion associated with sexual intercourse.

Fabry disease in patients with migraine with aura.

Anderson-Fabry disease (AFD) is an X-linked recessive lysosomal disease caused by alpha-galactosidase A (alpha-gal) deficiency, causing progressive glycosphingolipid storage in various organ systems. Headache is a frequent symptom. Cerebral magnetic resonance imaging (MRI) often shows multiple white matter lesions (WML), like those seen in patients affected by migraine, in particular with aura (MA). To our knowledge, there are no reports about the prevalence of AFD in patients with MA. The objective of the study was to determine AFD prevalence, as assessed by alpha-gal activity and genetic tests, in MA patients. We evaluated 73 consecutive patients followed by the Headache Centre of our Department with a diagnosis of MA. They were screened for migraine characteristics and cerebrovascular risk factors. Gaseous contrast transcranial Doppler was used to diagnose right-to-left shunt and MRI to detect WML. All patients underwent blood test to evaluate peripheral alpha-gal activity and to identify alpha-gal gene mutations. Of 73 consecutive screened subjects (59 females, 14 males; mean age 38.3 +/- 11.8 years), the known GLA pathologic mutation p.[Asp313Tyr] was found in a 38-year-old woman, with a history of MA, deep venous thrombosis and abdominal pain. Cerebral MRI showed small WML. This is the first study reporting AFD prevalence in a cohort of MA patients. We found a relatively high prevalence (about 1.37%) among the examined patients, even if this finding needs to be confirmed in a larger sample. Despite this high prevalence, it seems not necessary to screen systematically all MA patients for AFD, but since it is a treatable genetic disorder, it is worthwhile to consider it for the subgroup of patients presenting WML and other typical AFD symptoms.

Diagnosis of right-to-left shunt with transcranial Doppler and vertebrobasilar recording.

BACKGROUND AND PURPOSE: Right-to-left shunt (RLS) due to patent foramen ovale is a well-established risk factor for cryptogenic stroke and is highly prevalent in cases of migraine, cluster headache, and obstructive apnea. It can be diagnosed by gaseous-contrast transcranial Doppler, yet in a small percentage of cases it cannot be done owing to an insufficient temporal window. The aim of the study was to compare transtemporal with transoccipital approaches for gaseous-contrast transcranial Doppler for RLS diagnosis. METHODS: We evaluated 183 subjects with a standard protocol for RLS diagnosis by simultaneously monitoring the right middle cerebral and vertebrobasilar circulations. RESULTS: Vertebrobasilar recording reached high specificity (100%) and good sensitivity (83.72%) for the diagnosis of RLS after the Valsalva maneuver. For only medium and large shunts, both sensitivity and specificity reached 100%. Time to bubble appearance after injection was higher in the vertebrobasilar circulation (4.36+/-1.7 vs 6.77+/-2.5 seconds; P<0.001). There was a positive correlation between the number of bubbles in the right middle cerebral and vertebrobasilar circulation (kappa=0.97). CONCLUSIONS: Transcranial Doppler with vertebrobasilar monitoring is highly sensitive and specific in detecting RLS, particularly when medium or large. It can be proposed for subjects with an insufficient temporal bone window.