Postintravenous immunoglobulin stroke in a toddler with Down syndrome: a diagnostic challenge

2020 ◽  
Vol 13 (5) ◽  
pp. e233149 ◽  
Author(s):  
Teck-Hock Toh ◽  
Everlyn Coxin Siew ◽  
Chae-Hee Chieng ◽  
Hussain Imam Mohd Ismail
Keyword(s):  
Down Syndrome ◽  
Cerebral Arteries ◽  
Severe Pneumonia ◽  
Cerebral Vessels ◽  
Partial Recovery ◽  
Diagnostic Challenge ◽  
Middle Cerebral Arteries ◽  
Collateral Vessels ◽  
Carotid Doppler

Children with Down syndrome have a higher risk of stroke. Similarly, intravenous immunoglobulin (IV Ig) is also known to cause a stroke. We reported a 3-year-old boy with Down syndrome who presented with severe pneumonia and received IV Ig. He developed right hemiparesis 60 hours after the infusion. Blood investigations, echocardiography and carotid Doppler did not suggest vasculitis, thrombophilia or extracranial dissection. Brain computerised tomography (CT) showed acute left frontal and parietal infarcts. Initial magnetic resonance angiography (MRA) of cerebral vessels showed short segment attenuations of both proximal middle cerebral arteries and reduction in the calibre of bilateral supraclinoid internal carotid arteries. The boy was treated with enoxaparin and aspirin. He only had partial recovery of the hemiparesis on follow-up. A repeat MRA 13 months later showed parenchymal collateral vessels suggestive of moyamoya disease. We recommend imaging the cerebral vessels in children with a high risk of moyamoya before giving IV Ig.

scholarly journals A case of Young Stroke due to Moyamoya Disease

2020 ◽  
Vol 15 (1) ◽  
pp. 110-113
Author(s):  
Md Abdur Razzak ◽  
Ghulam Kawnayn ◽  
Fateha Naznin ◽  
Quazi Audry Arafat Rahman
Keyword(s):  
Moyamoya Disease ◽  
Past History ◽  
Cerebral Arteries ◽  
Armed Forces ◽  
Cerebral Angiogram ◽  
Medical College ◽  
Middle Cerebral Arteries ◽  
History Of ◽  
Collateral Vessels ◽  
The Right

Moyamoya disease is a disease in which certain arteries in the brain are constricted. Blood flow is blocked by the constriction, and also by blood clots (thrombosis). A collateral circulation develops around the blocked vessels to compensate for the blockage, but the collateral vessels are small, weak, and prone to bleeding, aneurysm and thrombosis which may result in TIA, recurrent ischemic or hemorrhagic stroke or seizure. The disease may manifest in pediatric age or young adults. In May 2019 we have diagnosed a young lady with Moyamoya disease who presented with right sided hemiplegia, motor aphasia and dysphagia. She was labeled as hypertensive 6 months prior to this event and used to take anti-hypertensive irregularly and gave past history of occasional headache. Her CT scan and MRI of brain revealed left sided ischemic infarct involving frontotemporoparietal region and cerebral angiogram revealed narrowing of left MCA and non-visualization of distal part. There is extensive fine collaterals (Moyamoya vessels) giving the appearance of puffed smoke. The right ACA and MCA were also narrowed with appearance of early collateral vessels. She was treated with aspirin, PPI, NG feeding, antihypertensive medication, physiotherapy, rehabilitation therapy and other supportive care. His condition gradually improved and discharged on 2.7.19. He was referred to Department of Neurosurgery for cerebral revascularization by STA-MCA (superficial temporal and middle cerebral arteries) bypass surgery after stabilization and MR perfusion study. Journal of Armed Forces Medical College Bangladesh Vol.15 (1) 2019: 110-113

Moyamoya Disease

2019 ◽  
pp. 149-156
Author(s):  
Edward Smith
Keyword(s):  
Moyamoya Disease ◽  
Cerebral Arteries ◽  
Pressure Control ◽  
Vascular Supply ◽  
External Carotid Artery ◽  
External Carotid ◽  
Middle Cerebral Arteries ◽  
Detailed Assessment ◽  
Collateral Vessels ◽  
Carotid Circulation

Moyamoya disease is defined by stenosis of the distal intracranial internal carotid arteries up to and including the bifurcation, with segments of the proximal anterior and middle cerebral arteries, dilated basal collateral vessels, and bilateral findings. Detailed assessment with digital subtraction angiography will define the severity of disease (Suzuki stage) and presence of spontaneous transdural collateral vessels from external carotid artery branches. These collateral vessels must be protected during surgical intervention. The surgical goal is to establish a new vascular supply to the areas of the brain at risk for stroke, utilizing vessels from the external carotid circulation. Blood pressure control and avoidance of hyperventilation are key to minimize perioperative stroke risk. Preoperative hydration, ongoing use of aspirin, and good pain control will also minimize surgical complications.

Cerebral arterial dolichoectasia with seizure

1976 ◽  
Vol 44 (4) ◽  
pp. 509-512 ◽  
Author(s):  
Joseph R. Thompson ◽  
Philip R. Weinstein ◽  
Charles R. Simmons
Keyword(s):  
Cerebral Angiography ◽  
Cerebral Arteries ◽  
Hemiplegic Migraine ◽  
Content Type ◽  
Middle Cerebral Arteries ◽  
Brain Scan ◽  
History Of ◽  
Angiographic Demonstration ◽  
Fine Print

✓ Cerebral angiography, performed after a seizure in a patient with a life-long history of typical hemiplegic migraine, disclosed markedly dolichoectatic anterior and middle cerebral arteries. No abnormality of the adjacent capillary or venous structures was present. A positive brain scan was attributed to ischemia induced by vasospasm rather than to the corresponding large tortuous anterior and middle cerebral arteries. There were no permanent sequelae and the patient has been free of seizures on Dilantin and phenobarbital over a 3-year follow-up period. Angiographic demonstration or description of a similar ectatic set of anterior and middle cerebral arteries could not be found in the literature. The concurrence of seizures and hemiplegic migraine adds to the peculiarity of this case.

Primary Stenting of Embolic Occlusions in Iliac Arteries

2003 ◽  
Vol 10 (3) ◽  
pp. 629-635 ◽  
Author(s):  
Saim Yilmaz ◽  
Timur Sindel ◽  
Ersin Lüleci
Keyword(s):  
Cerebral Arteries ◽  
Balloon Dilation ◽  
Related Complication ◽  
Iliac Arteries ◽  
Middle Cerebral Arteries ◽  
Primary Stenting ◽  
The Common ◽  
The Mean ◽  
Primary Implantation

Purpose: To present our experience with the use of primary stenting to treat embolic iliac artery occlusions. Methods and Results: Six patients (5 men; mean age 50.6±14.1 years range 37–72) underwent primary stenting for embolic occlusions lodged in the common iliac and/or external iliac arteries and were retrospectively evaluated. The probable reason for the embolism was atrial fibrillation in 4 patients and acute myocardial infarction in 2. In 5 patients, an additional embolus was demonstrated in the renal, mesenteric, popliteal, and middle cerebral arteries. Iliac emboli were treated with primary implantation of self-expanding stents followed by very low-pressure balloon dilation. In all cases, primary stenting was technically successful and provided immediate recanalization with elimination or reduction of symptoms. There was no procedure-related complication. During the mean 11-month follow-up (range 1–18), all stented iliac arteries remained patient. One patient died due to cerebral embolism at 21 months. Conclusions: Although this experience is limited, excellent midterm results suggest that primary stenting may be a valuable alternative in the treatment of embolic occlusions of the iliac arteries in selected cases.

scholarly journals Effect of NO on EDHF response in rat middle cerebral arteries

2002 ◽  
Vol 282 (2) ◽  
pp. H734-H738 ◽  
Author(s):  
Lisa A. Schildmeyer ◽  
Robert M. Bryan
Keyword(s):  
Nitric Oxide ◽  
Smooth Muscle ◽  
Methyl Ester ◽  
Guanylate Cyclase ◽  
Cerebral Arteries ◽  
No Synthase ◽  
Cerebral Vessels ◽  
No Donor ◽  
Physiological Conditions ◽  
Middle Cerebral Arteries

Whereas the actual identity of endothelium-derived hyperpolarizing factor (EDHF) is still not certain, it involves a process requiring the endothelium and eliciting hyperpolarization and relaxation of smooth muscle. It is neither nitric oxide (NO) nor prostacyclin, and its presence has been demonstrated in a variety of vessels. Recent studies in peripheral vessels report that EDHF-mediated dilations were either attenuated or blocked by NO. Studies presented here demonstrate that NO does not block EDHF-mediated dilations in cerebral vessels. Rat middle cerebral arteries were cannulated, pressurized, and luminally perfused. EDHF-mediated dilations were elicited by the luminal application of ATP in the presence of N G-nitro-l-arginine methyl ester (l-NAME) and indomethacin (inhibitors of NO synthase and cyclooxygenase, respectively). These dilations persisted when S-nitroso- N-acetylpenicillamine, an NO donor, was added exogenously in the presence of l-NAME, or when endogenous NO was present but its cGMP actions were blocked by 1H-[1,2,4]oxadiazolo[4,3-a]quinoxalin-1-one, an inhibitor of guanylate cyclase. These findings demonstrate that the EDHF response is not suppressed by NO in cerebral vessels and suggests a role for EDHF during normal physiological conditions.

scholarly journals Reversible Cerebral Vasoconstriction Syndrome Associated with Levonorgestrel-Releasing Intrauterine System

2021 ◽  
Vol 11 (5) ◽  
pp. 601
Author(s):  
Sangwon Choi ◽  
Ju-Young Lee ◽  
Jong Seok Bae ◽  
Hong-Ki Song ◽  
Ju-Hun Lee ◽  
...  
Keyword(s):  
Low Density Lipoprotein ◽  
Cerebral Arteries ◽  
Clinical Manifestations ◽  
Density Lipoprotein ◽  
Low Density ◽  
Mean Flow ◽  
Cerebral Vasoconstriction ◽  
Middle Cerebral Arteries ◽  
The Mean

Reversible cerebral vasoconstriction syndromes (RCVS) is a rare disease that is characterized by reversible multifocal stenosis of the cerebral arteries with various clinical manifestations. Though the pathomechanism of RCVS was unclear, we reported RCVS related to the levonorgestrel-releasing intrauterine system (IUS). A previous healthy 36-year-old woman had thunderclap headache after implanting the levonorgestrel-releasing IUS a year ago. In the serial angiography, we initially found left vertebra artery (VA), and then additionally new stenosis of both anterior cerebral arteries and middle cerebral arteries (MCA). Bilateral MCA stenosis improved but developed stenosis of right VA after a week. The mean flow velocities of both MCA increased in the first transcranial doppler (TCD), but normalized in the follow up TCD. Levonorgestrel might act as the vasoconstrictitve factor that increased the level of endothelin-1, diminished the release of NO and raised oxidative low-density lipoprotein (LDL). Although the exact pathological mechanisms for RCVS were not yet elucidated, this case might help clinicians understand the mechanisms of RCVS.

Autoimmune Pancreatitis – Diagnosis, Management and Longterm Follow-up

2014 ◽  
Vol 23 (2) ◽  
pp. 179-185 ◽  
Author(s):  
Suvadip Chatterjee ◽  
Kofi W. Oppong ◽  
John S. Scott ◽  
Dave E. Jones ◽  
Richard M. Charnley ◽  
...  
Keyword(s):  
Autoimmune Pancreatitis ◽  
Rare Condition ◽  
Team Approach ◽  
Diagnostic Challenge ◽  
Multisystem Disorder ◽  
North East ◽  
The North ◽  
Work Up ◽  
Igg4 Level

Background & Aims: Autoimmune pancreatitis (AIP) is a fibroinflammatory condition affecting the pancreas and could present as a multisystem disorder. Diagnosis and management can pose a diagnostic challenge in certain groups of patients. We report our experience of managing this condition in a tertiary pancreaticobiliary centre in the North East of England.Methods: Patients were identified from a prospectively maintained database of patients diagnosed with AIP between 2005 and 2013. Diagnosis of definite/probable AIP was based on the revised HISORt criteria. When indicated, patients were treated with steroids and relapses were treated with azathioprine. All patients have been followed up to date.Results: Twenty-two patients were diagnosed with AIP during this period. All patients had pancreatic protocol CT performed while some patients had either MR or EUS as part of the work up. Fourteen out of 22 (64%) had an elevated IgG4 level (mean: 10.9 g/L; range 3.4 - 31 g/L). Four (18%) patients underwent surgery. Extrapancreatic involvement was seen in 15 (68%) patients, with biliary involvement being the commonest. Nineteen (86%) were treated with steroids and five (23%) required further immunosuppression for treatment of relapses. The mean follow up period was 36.94 months (range 7 - 94).Conclusion: Autoimmune pancreatitis is being increasingly recognized in the British population. Extrapancreatic involvement, particularly extrahepatic biliary involvement seems to be a frequent feature.Diagnosis should be based on accepted criteria as this significantly reduces the chances of overlooking malignancy. Awareness of this relatively rare condition and a multi-disciplinary team approach will help us to diagnose and treat this condition more efiectively thereby reducing unnecessary interventions.

DIFFICULTIES IN DIAGNOSING MOYA-MOYA DISEASE (CLINICAL CASE)

2020 ◽  
pp. 48-53
Author(s):  
Novikova I.N. ◽  
Popova T.F. ◽  
Gribacheva I.A. ◽  
Petrova E.V. ◽  
Marushchak A.A. ◽  
...  
Keyword(s):  
Cerebral Angiography ◽  
Neurological Disorders ◽  
Clinical Case ◽  
Carotid Arteries ◽  
Internal Carotid ◽  
Cerebral Arteries ◽  
Moya Moya Disease ◽  
Middle Cerebral Arteries ◽  
Internal Carotid Arteries ◽  
Made In

Moya-Moya disease is a rare progressive chronic cer-ebrovascular disease characterized by a narrowing of the lumen of the intracranial segments of the internal carotid arteries, as well as the initial segments of the anterior and middle cerebral arteries with the devel-opment of a network of small vascular anastomoses. Violations of blood supply due to occlusion lead to the development of ischemic strokes in the correspond-ing pools, and ruptures of vascular anastomoses - to the development of hemorrhagic strokes, causing a variety of neurological disorders. The article presents a clinical case of Moya-Moya disease in a 31-year-old patient. The disease was manifested by acute disorders of cerebral circulation in ischemic and hemorrhagic types. The diagnosis was made in accordance with the diagnostic criteria of the disease based on the data of endovascular cerebral angiography.

Modulation of cerebral arterial tone by endothelium-derived relaxing factor

1993 ◽  
Vol 264 (4) ◽  
pp. H1245-H1250 ◽  
Author(s):  
J. E. Brian ◽  
R. H. Kennedy
Keyword(s):  
Nitric Oxide ◽  
Muscle Tone ◽  
Cerebral Arteries ◽  
Maximum Tension ◽  
Relaxing Factor ◽  
Middle Cerebral Arteries ◽  
Endothelium Derived Relaxing Factor ◽  
Vascular Smooth Muscle Tone ◽  
Dose Dependent ◽  
Arterial Tone

This study was designed to further elucidate the role of the endothelium in regulation of cerebral vascular smooth muscle tone. Dose-dependent vasoconstrictive effects of serotonin (5-HT) were examined in endothelium-intact and endothelium-denuded ring segments prepared from canine basilar and middle cerebral arteries. Some preparations were pretreated with 10(-5) M N omega-nitro-L-arginine (L-NNA), an agent that inhibits the production of L-arginine-derived nitric oxide, one of the compounds proposed to be endothelium-derived relaxing factor. L-NNA alone elicited marked dose-dependent increases in tension in endothelium-intact preparations; a significantly smaller response was seen in endothelium-denuded preparations. The effects of L-NNA on endothelium-intact preparations were partially reversed by washing and treatment with L-arginine. The maximum tension induced by 5-HT was approximately doubled by removal of the endothelium as well as by L-NNA treatment of endothelium-intact preparations; a slight increase in maximum tension occurred in endothelium-denuded preparations treated with L-NNA. The concentration of 5-HT producing half-maximal contraction (ED50) was not affected by L-NNA. These data suggest that L-arginine-derived nitric oxide modulates canine cerebral arterial tone in both the resting state and during contraction with 5-HT.

Sign in / Sign up

Export Citation Format

Share Document