Limb-shaking transient ischemic attacks in an adult PHACE syndrome: a case report and review of the literature

Chou, Ping-Song; Guo, Yuh Cherng

doi:10.1007/s10072-011-0671-8

Cited by 9 publications

(6 citation statements)

References 16 publications

(16 reference statements)

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“…The majority of our current knowledge about PHACE syndrome is based on studies of infants and young children, as diagnostic criteria were not available until 2009, and many adults with PHACE syndrome were likely previously misdiagnosed or have gone undiagnosed due to a lack of awareness. Most adults in the literature were not diagnosed with PHACE syndrome until they suffered from a transient ischemic attack or AIS . While many outcomes in adults with PHACE syndrome mirror those seen in children—such as neurodevelopmental anomalies—our study is the largest to date specifically examining the natural history of PHACE syndrome.…”

Section: Discussionmentioning

confidence: 99%

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Natural history of PHACE syndrome: A survey of adults with PHACE

Stefanko

Cossio

Powell

et al. 2019

Pediatric Dermatology

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Background Awareness of PHACE syndrome has increased; however, little information exists regarding its natural history, especially in patients over the age of 18. We aim to describe the natural history of PHACE to enhance clinical management and counseling of patients. Methods A cohort of patients ≥ 18 years was identified through the PHACE Syndrome Registry and a Vascular Anomalies Clinic Database. A cross‐sectional survey was designed after a review of the literature by PHACE experts (IF, JP, DS). Questions were selected by consensus, and the survey was conducted using the Qualtrics platform and via in‐person interviews. A 75% response rate was found. Results Eighteen adults—17 females and one transgender male—completed the survey. Respondents ranged in age from 18 to 59, with 24 being the mean age. Eighty‐nine percent reported experiencing headaches, and 17% reported experiencing acute but transient symptoms mimicking acute ischemic stroke, later diagnosed as atypical migraines. Thirty‐three percent reported hearing loss, and 67% endorsed dental issues. One patient experienced two arterial dissections. Three‐fourths who attempted conception were successful, and none of their children had clinical features of PHACE. Because results were based on a retrospective survey, data captured were prone to recall bias and not objective. Results were limited by a small sample size. Conclusions Health care providers should be aware of a possible increased risk of neurovascular complications, including atypical migraines mimicking transient ischemic attacks and arterial dissection, in adults with PHACE. Heritability has not been demonstrated, and future studies are needed to assess the risk of infertility.

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Section: Discussionmentioning

confidence: 99%

“…a transient ischemic attack or AIS. [5][6][7][8] While many outcomes in adults with PHACE syndrome mirror those seen in children-such as neurodevelopmental anomalies-our study is the largest to date specifically examining the natural history of PHACE syndrome.…”

Section: Discussionmentioning

confidence: 99%

Natural history of PHACE syndrome: A survey of adults with PHACE

Stefanko

Cossio

Powell

et al. 2019

Pediatric Dermatology

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“…Some experts have advised particular caution when using propranolol in individuals with PHACE with cervical or cerebral arteriopathy, because systemic hypotension has the theoretical risk of reducing blood flow through narrowed or occluded arteries supplying the brain . Recent reports of transient ischemic attacks in two adults with PHACE with severe cerebrovascular anomalies underscore the potential for hypoperfusion in PHACE . Although PHACE is not an absolute contraindication to the use of beta‐blockers, benefits must be carefully weighed against potential risks.…”

mentioning

confidence: 99%

Propranolol Use in PHACE Syndrome with Cervical and Intracranial Arterial Anomalies: Collective Experience in 32 Infants

Metry

Frieden

Hess

et al. 2012

Pediatric Dermatology

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1. Objective Combine collective clinical experience using oral propranolol therapy in PHACE syndrome infants with cerebrovascular anomalies. 2. Design Retrospective study of patients evaluated between July 2008 and October 2011. 3. Setting Seven pediatric dermatology centers. 4. Patients 32 infants with definite PHACE syndrome and cervical and/or intracranial arterial anomalies. 5. Intervention Oral propranolol: average dose of 1.8 mg/kg/day divided t.i.d. or b.i.d., for an average duration of 12.3 months. 6. Main Outcome Measure Adverse neurologic events. 7. Results 7/32 (22%) patients were categorized as higher-risk for stroke, defined on MRA as severe, long-segment narrowing or non-visualization of major cerebral or cervical vessels without anatomic evidence for collateral circulation, often in the presence of concomitant cardiovascular comorbidities. Only 1 patient developed a change in neurologic status during propranolol treatment: a mild right hemiparesis that remained static and improved while propranolol was continued. An additional 3 patients had worsening hemangioma ulceration and/or tissue necrosis during therapy. 8. Conclusions This is the largest report thus far of PHACE patients treated with propranolol. While no catastrophic neurologic events occurred, serious complications, particularly severe ulcerations were seen in a minority of patients, and given the sample size we cannot negate the possibility that propranolol could augment the risk of stroke in this population. We continue to advise caution in using systemic beta-blockers, particularly for children with vascular anomalies at higher risk for stroke. Use of the lowest possible dosage, slow dosage titration, and t.i.d. dosing in order to minimize abrupt changes in blood pressure and close follow-up, including neurologic consultation as needed, are recommended.

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“…Rare aetiologies were also reported. A novel case of limb shaking TIA associated with PHACE, an abbreviation for malformations of the posterior fossa, facial haemangiomas, arterial anomalies, cardiac anomalies, and abnormalities of the eye, was reported [ 14 ]. The 23-year-old female patient visited the hospital because of suffering from an insidious onset of hemiparesis since infancy.…”

Section: Discussionmentioning

confidence: 99%

A case of limb shaking transient ischaemic attack due to internal carotid artery dissection: an unusual presentation of fibromuscular dysplasia

Liu

Lei

et al. 2023

BMC Neurol

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Background Fibromuscular dysplasia (FMD) has a high prevalence of associated nontraumatic carotid artery dissection, which could further result in transient ischaemic attack (TIA) or stroke. Limb shaking TIA is an unusual form of TIA that is commonly discribed in elderly patients with atherosclerotic backgrounds, while there are limited data about it in patients with FMD. Furthermore, discussions of limb shaking TIA in nonelderly patients are scarce. Case presentation An Asian 47-year-old female presented with intermittent involuntary movement of the left upper limb accompanied by neck torsion. The episode stopped soon after changing to the supine position. On native source images of time-of-flight magnetic resonance angiography (TOF-MRA), the right internal carotid artery showed a "dual lumen sign" with an intimal flap. On contrast-enhanced magnetic resonance angiography and sagittal black-blood T1WI, an intravascular haematoma with irregular lumen stenosis was observed, which overall indicated right internal carotid artery dissection. Digital subtraction angiography showed the characteristic “string-of-beads” appearance in the left internal carotid artery, and the presence of this sign pointed to the diagnosis of FMD. The patient was finally diagnosed with limb shaking TIA due to internal carotid dissection with fibromuscular dysplasia. The patient was prescribed dual anti-platelet therapy. The limb shaking vanished soon after admission with no reoccurrence in the three-month follow-up. Conclusions This case demonstrates that limb shaking TIA can present in patients with FMD. Limb shaking TIA in nonelderly patients can be caused by multiple diseases, and more detailed patient guidance is required in clinical practice.

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Limb-shaking transient ischemic attacks in an adult PHACE syndrome: a case report and review of the literature

Cited by 9 publications

References 16 publications

Natural history of PHACE syndrome: A survey of adults with PHACE

Natural history of PHACE syndrome: A survey of adults with PHACE

Propranolol Use in PHACE Syndrome with Cervical and Intracranial Arterial Anomalies: Collective Experience in 32 Infants

A case of limb shaking transient ischaemic attack due to internal carotid artery dissection: an unusual presentation of fibromuscular dysplasia

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