An Association Between Migrainous Aura and Hereditary Haemorrhagic Telangiectasia

Steele, Julie R.; Nath, Preetam; Burn, John; Porteous, Mary

doi:10.1111/j.1526-4610.1993.hed3303145.x

Cited by 58 publications

(44 citation statements)

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“…12,40,50,[52][53][54] Others have noted a high occurrence of cases of migraine headaches in patients with HHT and pulmonary AVFs. 55 It remains unsettled whether this frequent finding represents an increase over the normal incidence of migraine headaches in the population. 56 Our data confirm the high risk of cerebral ischemic complications and abscesses in patients with HHT that is related to pulmonary AVFs.…”

Section: Discussionmentioning

confidence: 99%

Cerebrovascular Manifestations in 321 Cases of Hereditary Hemorrhagic Telangiectasia

Maher¹,

Piepgras²,

Brown³

et al. 2001

Stroke

215

104

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Background and Purpose-Patients with hereditary hemorrhagic telangiectasia (HHT) are at risk for developing cerebral vascular malformations and pulmonary arteriovenous fistulae. We assessed the risk of neurological dysfunction from these malformations and fistulae. Methods-Three hundred twenty-one consecutive patients with HHT seen at a single institution over a 20-year period were studied. Any evidence of prior neurological symptoms or presence of an intracranial vascular malformation was recorded. All cases of possible cerebral arteriovenous malformation were confirmed by conventional arteriography. Results-Twelve patients (3.7%) had a history of cerebral vascular malformations. Ten patients had arteriovenous malformations, 1 had a dural arteriovenous fistula, and 1 had a cavernous malformation. Seven patients (2.1%) presented with intracranial hemorrhage, 2 presented with seizures alone, and 3 were discovered incidentally. The average age at the time of symptomatic intracranial hemorrhage was 25.4 years. All patients with a history of intracranial hemorrhage were classified as Rankin grade I or II at a mean follow-up interval of 6.0 years. A history of cerebral infarction or transient ischemic attack was found in 29.6% of patients with HHT and a pulmonary arteriovenous fistula. Conclusions-The risk of intracranial hemorrhage is low among people with HHT. Furthermore, a majority of these patients have a good functional outcome after hemorrhage. The data do not suggest a compelling indication for routine screening of patients with HHT for asymptomatic cerebral vascular malformations. 1 The estimated prevalence is 1 or 2 cases per 100 000. 1,2 Chromosomes 9q and 12q have been implicated in the inheritance of this disease. 3-7 A family history of HHT, although not required to make the diagnosis, is almost universally present. The vascular malformations of HHT may occur in multiple organs, including the lung, liver, kidney, and brain. 8,9 Epistaxis is the most common presentation, followed by gastrointestinal bleeding. 10,11 Pulmonary arteriovenous fistulae (AVFs) are often accompanied by dyspnea or hemoptysis and, less frequently, ischemic neurological symptoms. [12][13][14] Telangiectasias are frequently noted on the skin and mucous membranes, often not appearing until the second or third decade. 8,9 Brown et al 15,16 have reported that the detection rate of intracranial vascular malformations in a population-based study in the most recent time period was 2.75 per 100 000 person-years. The prevalence of intracranial hemorrhage from a vascular malformation was 7.50 per 100 000 people in the population at large. 15,16 Patients with HHT are thought to be at increased risk for harboring cerebral vascular malformations compared with the entire population. Arteriovenous malformations (AVMs), cavernous malformations, dural arteriovenous fistulae, and aneurysms have all been reported in these patients ( Figure 1). 14,17-24 Vascular malformations are found throughout the central nervous system, including the spinal cor...

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Section: Discussionmentioning

confidence: 99%

Cerebrovascular Manifestations in 321 Cases of Hereditary Hemorrhagic Telangiectasia

Maher¹,

Piepgras²,

Brown³

et al. 2001

Stroke

215

104

View full text Add to dashboard Cite

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“…In a study by STEELE et al [31], it is suggested that CAVM might play a role in the pathogenesis of migraine in HHT patients. In the present study, we found no association between the presence of a CAVM and overall prevalence of no migraine or MA.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary arteriovenous malformations associated with migraine with aura

Post

Gent²,

Plokker³

et al. 2009

European Respiratory Journal

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Migraine with aura (MA) is associated with cardiac right-to-left shunt. We prospectively studied the association between pulmonary arteriovenous malformations (PAVMs) and MA in hereditary haemorrhagic telangiectasia (HHT).All 220 consecutive HHT patients who underwent high-resolution chest computed tomography for PAVM screening were included prospectively. Prior to screening, a structured validated headache questionnaire was completed by 196 patients (57% female; mean¡sd age 44.6¡15.2 yrs). Two neurologists diagnosed migraine according to the International Headache Society Criteria.A PAVM was present in 70 (36%) patients. The prevalence of MA was 24% in the presence of a PAVM compared with 6% in the absence of a PAVM (OR 4.6, 95% CI 1.84-11.2; p50.001), and MA was an independent predictor for the presence of PAVM using multivariate analysis (OR 3.6, 95% CI 1.21-10.5; p50.02). A PAVM was present in 68% of the patients with MA compared with 32% in the non-migraine controls (OR 4.6, 95% CI 1.84-11.2; p50.001), and a PAVM was an independent predictor for MA using multivariate analysis (OR 3.0, 95% CI 1.00-9.20; p50.05).In conclusion, PAVMs are associated with MA in HHT patients.

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“…As many as 71% of patients with PAVMs secondary to HHT experience headache symptoms and y55% satisfy criteria for the diagnosis of migraine [3]. Though frequency of headache is unknown for patients with sporadic PAVMs, the presentation of the patient with headache, which resolved after PAVM embolization, suggests that the aetiology of her headache was related to the PAVM.…”

Section: Discussionmentioning

confidence: 94%