Limb-shaking syndrome derived from the contralateral hemisphere following unilateral revascularisation for moyamoya disease

Background: Moyamoya disease is a rare chronic steno-occlusive cerebrovascular disease. It may have variable clinical symptoms associated with cerebral stroke, including motor paralysis, sensory disturbances, seizures, or headaches. However, patients with moyamoya disease rarely present with involuntary movement disorders, including limb-shaking syndrome, with no previous reports of limb-shaking syndrome occurring after revascularization procedures for this disease. Although watershed shifts can elicit transient neurological deterioration after revascularisation, symptoms originating from the contralateral hemisphere following the revascularization procedure are rare. Here, we report the case of moyamoya disease wherein the patient developed limb-shaking syndrome derived from the contralateral hemisphere after unilateral revascularisation. Case Description: A 16-year-old girl presented with transient left upper and lower limb numbness and headache. Based on digital subtraction angiography, she was diagnosed with symptomatic moyamoya disease. Single-photon emission computed tomography (SPECT) showed decreased cerebral blood flow (CBF) on the right side, and she underwent direct and indirect bypasses on this side. Involuntary movements appeared in her right upper limb immediately postoperatively. SPECT showed decreased CBF to the bilateral frontal lobes. Subsequently, the patient was diagnosed with limb-shaking syndrome. After performing left-hemispheric revascularisation, the patient’s symptoms resolved, and SPECT imaging confirmed improvements in CBF to the bilateral frontal lobes. Conclusion: Revascularization for moyamoya disease can lead to watershed shifts, which can induce limb-shaking syndrome derived from abnormalities in the contralateral hemisphere of the revascularized side. For patients with new-onset limb-shaking syndrome after moyamoya revascularisation procedures, additional revascularization may be warranted for treatment of low perfusion areas.

Successful multiple burr hole openings for limb-shaking transient ischemic attack due to moyamoya disease: illustrative case

BACKGROUND Limb-shaking transient ischemic attacks (LS-TIAs) are a rare form of TIAs that present as involuntary movements of the limbs and indicate severe cerebral hypoperfusion. LS-TIAs are often reported in patients with carotid artery stenosis but can also affect patients with intracranial artery stenosis and moyamoya disease (MMD). OBSERVATIONS A 72-year-old woman presented with repeated episodes of involuntary shaking movements of the right upper limb. Cerebral angiography revealed complete occlusion of the M1 segment of the left middle cerebral artery (MCA), and the left hemisphere was supplied by moyamoya vessels. She was treated with left direct revascularization without complications, and her involuntary movements subsided. However, she demonstrated involuntary shaking movements of the right lower limb 2 months postoperatively. Cerebral angiography revealed complete occlusion of the A1 segment of the left anterior cerebral artery (ACA). The multiple burr hole opening (MBHO) procedure was performed to improve perfusion in the left ACA territory and after 3 months, the patient’s symptoms resolved. LESSONS This case demonstrated that LS-TIAs can also develop as ischemic symptoms due to MMD. Moreover, instances of LS-TIA of the upper and lower limbs developed separately in the same patient. The patient’s symptoms improved with direct revascularization and MBHO.

Stepwise improvement in limb shaking achieved by staged angioplasty for severe carotid stenosis

Cerebral hyperperfusion syndrome is a rare but severe complication of carotid artery stenting or carotid endarterectomy. Staged angioplasty is reportedly an effective strategy to avoid cerebral hyperperfusion syndrome. We encountered a case of internal carotid artery stenosis with a rare clinical presentation of limb shaking that was successfully improved by staged angioplasty. To our knowledge, there are no reported cases of limb shaking treated with staged angioplasty. A 76-year-old woman presented with continuous chorea in her left lower limb and shoulder. Medical examination revealed a tiny cerebral infarction in the right corona radiata and severe right internal carotid artery stenosis. Angiography showed near occlusion of the right internal carotid artery. Staged angioplasty was performed to avoid the risk of cerebral hyperperfusion syndrome. The first angioplasty resulted in an expanded diameter of 2.5 mm and was followed by definitive carotid artery stenting using a closed-cell stent 3.5 weeks later. Limb shaking improved in a stepwise manner along with an improvement in internal carotid artery stenosis and distal flow state with no signs of cerebral hyperperfusion syndrome. Patients with internal carotid artery stenosis or occlusion presenting with limb shaking have been suggested to have impaired cerebrovascular reactivity, which is also thought to be a risk factor for cerebral hyperperfusion syndrome. The stepwise improvement in limb shaking observed in this case supports the idea that the pathophysiology of limb shaking is related to cerebral haemodynamic impairment. Measures to prevent cerebral hyperperfusion syndrome, including staged angioplasty, should be actively considered in patients with limb shaking because the symptoms themselves suggest severe hypoperfusion.

Limb-Shaking Transient Ischemic Attack Presenting with Myoclonus

Limb-shaking transient ischemic attack is a rare disease, associated with the steno-occlusion of the internal carotid artery. It is caused by hemodynamic dysfunction in the anterior circulation. It is difficult to observe patients directly in clinical settings since they visit the hospital after symptoms have subsided which usually last less than 5 minutes. Here we report a atient who developed right arm dominant myoclonus related to left internal carotid artery occlusion along with a recorded video.

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.

Limb-Shaking Transient Ischemic Attacks in a Patient with Previous Bilateral Neck Irradiation: The Role of Collateral Flow

Nasopharyngeal carcinoma (NPC) is commonly treated with bilateral neck radiation, which is closely associated with the complication of carotid-occlusive disease. This leads to cerebral hemodynamic compromise and possible ischemic stroke. Another manifestation is limb-shaking transient ischemic attacks (LS-TIAs), characterized by rhythmic jerks which can be easily mistaken as a focal motor seizure. We describe a case of unilateral LS-TIAs from bilateral carotid occlusion that resolved with contralateral carotid revascularization. Our patient is a 65-year-old gentleman who had no significant co-morbidities other than a past history of bilateral neck irradiation for NPC 8 years before. He presented with left-sided limb weakness and subsequently left-sided limb involuntary movements whenever he sat up or stood. His symptoms did not respond to anti-epileptic therapy. Clinical and neurological examination was significant for a left pronator drift and weak left finger abduction. Computed tomography and magnetic resonance imaging of the brain revealed infarcts in the right periventricular and watershed areas; MR angiogram showed bilateral internal carotid artery occlusion. Single photon emission computed tomography showed reduced blood flow in the right frontal, temporal, and parietal regions, that reduced further after acetazolamide challenge. He was diagnosed as having LS-TIA secondary to carotid-occlusive disease. Attempts at endovascular opening the right internal carotid artery failed. Following successful left carotid angioplasty and stenting, his symptoms gradually resolved. The left internal carotid artery remained patent at the 3-month follow-up; the right side remained occluded. Our case supports the hypothesis that LS-TIAs are due to hemodynamic compromise and may respond to improved collateral cerebral blood flow.