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

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.

Limb-shaking TIA: a case of cerebral hypoperfusion in severe cerebrovascular disease in a young adult.

BACKGROUND: Limb-shaking transient ischaemic attacks (TIAs) are an under recognised presentation of severe cerebrovascular disease resulting from cerebral hypoperfusion. Patients present with jerking, transitory limb movements precipitated by change in position or exercise that are often confused with seizure. Cerebral perfusion imaging studies are an important tool available to aid diagnosis. CASE PRESENTATION: We present the case of a young female who developed limb-shaking TIA in the context of progressive severe intracranial atherosclerotic disease (ICAD). Previous cortical infarction raised suspicion for seizure as a cause of her symptoms. However, single photon emission CT (SPECT) with CT acetazolamide challenge identified severe left hemisphere cerebral hypoperfusion and a diagnosis of limb-shaking TIA was made. Symptoms improved with maximal medical management. CONCLUSIONS: This case highlights the importance of cerebral perfusion imaging for diagnostic confirmation as well as therapeutic options available to alleviate symptoms and reduce stroke risk in patients with limb-shaking TIA.

Brachiocephalic Arterial Occlusive Disease Presenting as Limb-Shaking Transient Ischemic Attacks.

Limb-shaking transient ischemic attacks (LSTIAs) are a phenomenon that occurs due to transient hypoperfusion to a cerebral motor territory with a chronically outstripped autoregulatory vascular reserve. First described in 1962 by Miller Fisher, the pathogenesis and the global understanding of this presentation have undergone a significant advancement throughout the years. Typically, patients will present with this syndrome of transient hypoperfusion in the context of extracranial carotid intrinsic vessel stenosis or by intracranial vascular stenosis to select motor pathways. We present within this case report a novel mechanism by which LSTIAs may emerge. Through this knowledge, clinicians may need to consider expansion of their diagnostic breadth to include proximal vasculature luminal integrity.

Inducible limb-shaking transitory ischemic attacks: a video-documented case report and review of the literature.

BACKGROUND: Limb-shaking transient ischemic attack (TIA) is a well-recognized, but rare observation in contralateral carotid steno-occlusive disease. Consequently, most clinicians have not had the chance to witness an attack. CASE PRESENTATION: We present the story of a 64-year old gentleman with exercise-induced weakness associated with tremor in his right arm. His left internal carotid artery was occluded at the bifurcation. Administration of statin and antiplatelet did not relieve his symptoms, and his stereotypic, exercise-induced "limb-shaking" episodes persisted. He underwent successful extracranial to intracranial (EC-IC) bypass, which stopped his symptoms. The patient, however, returned to our department and reported that he was able to recreate his original symptoms by compressing the bypass graft manually. CONCLUSION: To our knowledge, this is the first case with video documentation of the clinical appearance of a limb-shaking TIA. We hope this case report will increase the physicians' understanding of the clinical nature of limb-shaking TIAs.

Bilateral asymmetrical asterixis as limb-shaking transient ischemic attack in bilateral carotid stenosis.

Asterixis as limb-shaking transient ischemic attack (TIA) is rare and poorly understood. Bilateral asymmetrical asterixis as limb-shaking TIA has not been reported in carotid stenosis. A 69-year-old gentleman presented with a TIA episode (dysarthria, right-arm weakness, and numbness). Bilateral asterixis was observed and was more severe on the right side. No prior infarcts were noted in the thalamus. Liver function was normal. A computerized tomography angiogram revealed 85%stenosis of the right internal carotid artery (ICA) and 65% stenosis of the left ICA. Three days after left ICA endarterectomy, the patient had complete disappearance of bilateral asterixis, with the right side showing initial improvement. The bilateral asterixis observed is proposed to be secondary to hemodynamic impairment and hypoperfusion of certain brain territory with resolution on revascularization.

Long-segment common carotid occlusion presenting with limb-shaking transient ischemic attack: Case report.

Background: Limb-shaking transient ischemic attack (LS-TIA) is a rare manifestation of carotid artery occlusion. Common carotid artery occlusion (CCAO) is a relatively rare condition, and both its natural history and recommendations for treatment are still unclear. Case description: A 67-year-old female suffered from transient episodes of unilateral limb shaking. Computer tomographic angiography (CTA) showed long-segment occlusion of the right common carotid artery. Computer tomographic perfusion (CTP) demonstrated hypoperfusion of the corpus striatum, which suggests that hemodynamic failure is a potential mechanism underlying the LS-TIA secondary to common carotid artery occlusion. The occlusion was successfully recanalized by retrograde common carotid endarterectomy, and the episodes of left limb shaking disappeared after surgery. Conclusions: The occlusion was successfully recanalized by retrograde common carotid endarterectomy, and the episodes of left limb shaking disappeared after surgery. Hypoperfusion of the corpus striatum might be a potential mechanism underlying the LS-TIA secondary to common carotid occlusion.

Limb-shaking TIA in Moyamoya angiopathy.

BACKGROUND: Limb shaking TIA(LS-TIA) is an rare manifestation seen in high grade steno-occlusive carotid disease. LS-TIA is rarely recognized as a presenting manifestation of MMA and has never been previously studied prospectively in MMA cohort. METHODS: Of 104 cases of MMA collected in our Indian center in last 4 years, 5 new patients had LS-TIA. MMA was diagnosed by MR angiography and LS-TIA was confirmed by three different neurologist by detailed scrutiny of history, neurological examination and EEG-recording (to rule out seizure). Seven previously reported cases of LS-TIA in MMA were retrieved by literature search in PubMed using keywords "Moyamoya" AND "Limb shaking TIA", "Moyamoya" AND "Limb shaking". Subsequently all data were analyzed and compared using descriptive statistics. RESULTS: Analysis of our 5 cases and those 7 found in the literature showed mean age at the time of diagnosis of MMA was 12.2 ± 8.9(Mean ± S.D.) years in our cases and 32.7 ± 13.6 years in previous cases. The commonest precipitating factors included prolonged crying and hyperventilation(3 of 5 our cases each) and exercise (3 of 7 previous cases). CT-perfusion demonstrated frontal white matter hypoperfusion in 2 of our 5 cases, corroborative with fronto-parietal hypoperfusion in previous cases. Medical management showed decreased symptoms in 4 of our 5 cases in a follow-up of 15.8 ± 11.6 months, while all previous patients had underwent revascularization surgery with resolution of symptoms. CONCLUSION: LS-TIA in MMA may not be so uncommon, especially in children. Early recognition is imperative to avoid future catastrophe. Steno-occlusive posterior circulation lesions might be an independent predictor of development of LS-TIA in MMA. In a resource-limited setting, careful avoidance of triggers and anti-platelet therapy can lead to decreased symptomatology of LS-TIA in MMA.

Limb-shaking transient ischemic attack associated with focal electroencephalography slowing: case report.

BACKGROUND: Limb shaking is a rare form of transient ischemic attack (TIA) that can easily be confused with focal motor seizures. CASE: We report a case of a 61-year-old man with rhythmic jerky movements of his left limb, without loss of awareness, that have occurred about once per month for the past four months, precipitated by standing up and extending the neck. The electroencephalography test showed right temporal slow activity, without epileptiform features. No evidence of a noteworthy structural lesion was found on magnetic resonance imaging of the brain. Doppler ultrasound and magnetic resonance angiography of the neck disclosed an 80% stenosis of the right internal carotid artery. The patient underwent an endarterectomy of the right internal carotid artery and remained asymptomatic in the 12-month follow-up period. DISCUSSION: Both hypoperfusion and reduction of vasomotor reactivity to hypercapnia of corresponding cerebral territories, without the structural lesions of the brain, were observed in patients with limb-shaking syndrome (LSS). Electroencephalographic studies have failed to show epileptiform activity associated with LSS, although some patients have controlateral slow activity. In our patient, we observed a resolution of the attacks after endarterectomy of controlateral internal carotid artery, suggesting that a quick diagnosis of this form of TIA is important both to abolish the attacks and to reduce the risk of major stroke.