Idiopathic Moyamoya Disease Presenting as Isolated Hemichorea

Author(s):  
Omer Abdul Hamid ◽  
Paul Klimo ◽  
Asim F. Choudhri ◽  
Namrata Shah

AbstractThe study aimed to describe a case of a 7-year-old Caucasian girl who developed isolated chorea in her right upper and lower extremities progressively increasing over 2 years. This study is a case report and conducted at tertiary care center. A 7-year-old Caucasian girl who gradually developed worsening choreiform movements in her right upper and lower extremities over the course of 2 years until medical attention was sought. Literature review of children presenting with chorea as the only manifestation in moyamoya disease. A 7-year-old right-handed Caucasian girl presented with progressively worsening choreiform movements in her right upper and lower extremities affecting her fine motor skills and gait impairment There was no weakness, hyperreflexia, or spasticity on her neurological exam. Neuroimaging studies showed “ivy sign,” asymmetric prominence of vessels within the subarachnoid spaces overlying the left cerebral hemisphere with corresponding serpiginous T2 fluid-attenuated inversion recovery abnormality, but no parenchymal volume loss or diffusion restriction. Magnetic resonance angiography showed focal moderate-to-severe stenosis at the junction of the paraophthalmic and supraclinoid segments of the left internal carotid artery, with poststenotic dilatation. Angiography demonstrated focal stenosis involving the ophthalmic segment of the left internal carotid artery with 50% stenosis. There was compensation through a medium sized left posterior communicating artery as well as posterior cerebral artery to middle cerebral artery and anterior cerebral artery collateralization. Brain single-photon emission computerized tomography scan showed no evidence of perfusion defects in the cerebral hemispheres, basal ganglia, or thalami. She underwent successful revascularization procedure (left pial synangiosis) with resolution of her choreiform movements and normalization of her gait. Our case demonstrates that moyamoya disease should be suspected when evaluating a child with hemichorea and describes resolution of symptoms after revascularization surgery. Neuroimaging and vascular studies should be obtained in children with unilateral movement disorder.

2017 ◽  
Vol 23 (6) ◽  
pp. 601-604 ◽  
Author(s):  
Jonathan Pace ◽  
Jeffrey Nelson ◽  
Abhishek Ray ◽  
Yin Hu

A middle-aged patient presented for elective embolization of an incidentally found right internal carotid aneurysm. An angiogram was performed, during which the left internal carotid artery was visualized to evaluate a second, small aneurysm. During the embolization of the right internal carotid artery aneurysm, a catheter-induced vasospasm was identified that prompted treatment with intra-arterial verapamil. The procedure was uncomplicated; a postoperative rotational flat-panel computed tomography scan was performed on the angiography table that demonstrated right hemisphere contrast staining. The patient developed a right middle cerebral artery (MCA) syndrome after extubation with repeat cerebral angiography negative for occlusion and magnetic resonance imaging negative for stroke. The patient was observed for 48 hours, during which time the patient had slowly improved. At a six-week follow up visit, the patient had fully recovered. We present an interesting case of a verapamil-induced breakdown of the blood–brain barrier and self-limited right MCA syndrome.


2021 ◽  
Vol 12 ◽  
Author(s):  
Shuai Zheng ◽  
Peicong Ge ◽  
Zhiyong Shi ◽  
Jingzhe Wang ◽  
Yi Li ◽  
...  

Objective: To investigate the hemodynamic changes using ultrasound according to digital subtraction angiography (DSA) findings and explore the association between ultrasound parameters and clinical symptoms of moyamoya disease (MMD).Methods: Hemodynamic parameters of extracranial internal carotid artery (EICA) and posterior cerebral artery (PCA) were evaluated by ultrasound. According to DSA findings, EICA parameters among Suzuki stages (stage I-II, III-IV, and V-VI), and PCA parameters among leptomeningeal system scores (score 0–2, 3–4, and 5–6) were compared, respectively. ROC analysis was performed based on the ultrasound parameters to distinguish stroke from non-stroke patients.Results: Forty patients with MMD were included in our study (16 men; median age, 37 years). The diameter (D), peak systolic velocity (PSV), end diastolic velocity (EDV) and flow volume (FV) of EICA decreased as the Suzuki stage advanced (D: P < 0.001; PSV: P < 0.001; EDV: P < 0.001; FV: P < 0.001). The PSV and EDV of PCA increased as the leptomeningeal system scores advanced (PSV: P < 0.001; EDV: P < 0.001). ROC analysis showed that the area under the curves (AUCs) based on the D and FV of EICA, the PSV and EDV of PCA and their combination were 0.688, 0.670, 0.727, 0.684, and 0.772, respectively, to distinguish stroke from non-stroke patients.Conclusions: Ultrasound parameters were related to Suzuki stages and leptomeningeal system scores. Ultrasound may be useful in predicting the occurrence of stroke in patients with MMD. Future prospective studies with large sample sizes and long-term follow-up are needed to confirm our preliminary findings.


BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Zhizhong Yan ◽  
Zhonghua Shi ◽  
Yuhai Wang ◽  
Chunlei Zhang ◽  
Huize Liu ◽  
...  

Abstract Background Abnormal hypoperfusion on the surgical side after carotid artery stenting is rare. Neurological deterioration caused by it is deceptive, which can easily lead to misdiagnosis. The mechanism of hypoperfusion has rarely been demonstrated. We present here a fully studied case with a high probability of intracerebral steal phenomenon. Case presentation A 68-year-old male with severe right internal carotid artery stenosis and left internal carotid artery occlusion underwent right stenosis stent implantation. Restlessness and left limb hemiplegia occurred within 24 h after the procedure, which was similar to hyperperfusion syndrome. However, postoperative computerized tomography perfusion (CTP) revealed abnormal hypoperfusion in the right hemisphere. Transcranial Doppler (TCD) also showed decreased flow velocity in the right middle cerebral artery, and increased flow velocity in the right anterior cerebral artery. We considered that intracerebral steal phenomenon might be the cause, then hypervolemic therapy was accepted and the symptoms completely resolved after 3 days. Conclusions Ipsilateral hypoperfusion is rarely seen after carotid artery stenting. Intracerebral steal phenomenon may be the underlying mechanism. CTP or TCD is helpful for the early detection of this adverse event.


VASA ◽  
2011 ◽  
Vol 40 (6) ◽  
pp. 491-494 ◽  
Author(s):  
Vávrová ◽  
Slezácek ◽  
Vávra ◽  
Karlová ◽  
Procházka

Internal carotid artery pseudoaneurysm is a rare complication of deep neck infections. The authors report the case of a 17-year-old male who presented to the Department of Otorhinolaryngology with an acute tonsillitis requiring tonsillectomy. Four weeks after the surgery the patient was readmitted because of progressive swallowing, trismus, and worsening headache. Computed tomography revealed a pseudoaneurysm of the left internal carotid artery in the extracranial segment. A bare Wallstent was implanted primarily and a complete occlusion of the pseudoaneurysm was achieved. The endovascular approach is a quick and safe method for the treatment of a pseudoaneurysm of the internal carotid artery.


Author(s):  
Walid Elshamy ◽  
Burcak Soylemez ◽  
Sima Sayyahmelli ◽  
Nese Keser ◽  
Mustafa K. Baskaya

AbstractChondrosarcomas are one of the major malignant neoplasms which occur at the skull base. These tumors are locally invasive. Gross total resection of chondrosarcomas is associated with longer progression-free survival rates. The patient is a 55-year-old man with a history of dysphagia, left eye dryness, hearing loss, and left-sided facial pain. Magnetic resonance imaging (MRI) showed a giant heterogeneously enhancing left-sided skull base mass within the cavernous sinus and the petrous apex with extension into the sphenoid bone, clivus, and the cerebellopontine angle, with associated displacement of the brainstem (Fig. 1). An endoscopic endonasal biopsy revealed a grade-II chondrosarcoma. The patient was then referred for surgical resection. Computed tomography (CT) scan and CT angiogram of the head and neck showed a left-sided skull base mass, partial destruction of the petrous apex, and complete or near-complete occlusion of the left internal carotid artery. Digital subtraction angiography confirmed complete occlusion of the left internal carotid artery with cortical, vertebrobasilar, and leptomeningeal collateral development. The decision was made to proceed with a left-sided transcavernous approach with possible petrous apex drilling. During surgery, minimal petrous apex drilling was necessary due to autopetrosectomy by the tumor. Endoscopy was used to assist achieving gross total resection (Fig. 2). Surgery and postoperative course were uneventful. MRI confirmed gross total resection of the tumor. The histopathology was a grade-II chondrosarcoma. The patient received proton therapy and continues to do well without recurrence at 4-year follow-up. This video demonstrates steps of the combined microsurgical skull base approaches for resection of these challenging tumors.The link to the video can be found at: https://youtu.be/WlmCP_-i57s.


2020 ◽  
Vol 12 (Suppl. 1) ◽  
pp. 9-14
Author(s):  
Trung Quoc Nguyen ◽  
Hoang Thi Phan ◽  
Tinh Quang Dang ◽  
Vu Thanh Tran ◽  
Thang Huy Nguyen

The efficacy of intravenous thrombolysis and endovascular therapy and their favorable treatment outcomes have been established in clinical trials irrespective of age. Current guidelines do not recommend an age limit in selecting eligible patients for reperfusion treatment as long as other criteria are satisfied. A 103-year-old woman was admitted at our hospital within 1 h of stroke onset secondary to a left internal carotid artery terminus occlusion. On admission, her National Institutes of Health Stroke Scale (NIHSS) score was 30, with a small left thalamic diffusion restriction lesion on MRI. Her medical history included paroxysmal atrial fibrillation, prior myocardial infarction, hypertension, chronic kidney disease, and diabetes mellitus. Her pre-stroke modified Rankin Scale score was 0, and she was fully independent before stroke. Once intravenous thrombolysis was started, the patient successfully underwent mechanical thrombectomy, and thrombolysis in cerebral infarction-3 recanalization was achieved 225 min after symptom onset. She showed dramatic recovery (NIHSS score of 5 after 48 h) and was discharged on day 7 with a modified Rankin Score of 1. To our knowledge, our patient is the second oldest documented patient who successfully underwent bridging therapy for stroke.


2006 ◽  
Vol 25 (12) ◽  
pp. 1547-1552 ◽  
Author(s):  
Masahiro Yasaka ◽  
Toshiyasu Ogata ◽  
Kotaro Yasumori ◽  
Tooru Inoue ◽  
Yasushi Okada

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