Developmental Medicine & Child Neurology. Symptoms are thought to arise from transient abnormalities in the blood vessels of the brain. Recurrent attacks are rare and all paediatric cases to date have shown complete resolution of symptoms. (2011) Archives of neurology. Miller, R. Shivashankar, M. Mossa-Basha, D. Gandhi. endstream Thunderclap headache is the usual primary symptom, often occipital but also potentially diffuse. High-resolution vessel wall MRI is an emerging tech-nique for characterizing intracranial arterial disease.3,4 Large artery CNS vasculitis is associated with arterial wall thickening and enhancement.4,5 RCVS is a disorder of (d) The apparent diffusion map demonstrates restricted diffusion in the right frontoparietal lobe (shown in b) and confirms that the infarcts are acute. 2). 11 0 obj These terms include 1,2,4,17: Importantly RCVS should not be confused with posterior reversible encephalopathy syndrome (PRES). Transcranial Doppler ultrasonography may have a role in the serial monitoring of cases and demonstrates that arterial abnormalities often persist well beyond resolution of clinical symptoms.5, 6, 8 The diagnosis is confirmed by demonstrations of reversal of the vascular abnormalities 1 to 3 months after initial presentation. endobj <>stream Precipitants (including immersion in cold water) are reported in 20% to 30% of cases.7 Other associations include vascular lesions (dissection/aneurysms), catecholamine‐secreting tumours, trauma, and neurosurgical procedures. They are usually fairly short in duration (a few hours) and usually, headaches will periodically recur over days or weeks 1,2,17. (2012) The Lancet. -H�z3��
����=�aK�Jݛ��{��?�i�x� Spontaneous resolution usually occurs, with improvement in angiographic findings within three months. of RCVS is currently confirmed only in retrospect, when arterial narrowing resolves. Numerous and varied terms have been used to describe RCVS, sometimes entirely synonymously and at other times one or other subset. Reversible Cerebral Vasoconstriction Syndrome, Part 1: Epidemiology, Pathogenesis, and Clinical Course. A prospective series of 67 patients. Postpartum cerebral angiopathy: reversible vasoconstriction assessed by transcranial Doppler ultrasounds. Ann. The term RCVS was coined later in 2007 by Calebrese 1. Central nervous system (CNS) vasculitis is an important differential diagnosis as, although RCVS by definition reverses spontaneously, vasculitis has a progressive or recurrent course that necessitates immunosuppression and long‐term follow‐up. Other calcium channel antagonists, such as nicardipine or verapamil, have also been used. 3. Where antecedent drug ingestion has been reported previously, the interval between drug exposure and symptoms has been variable. The patient was discharged on amitriptyline and aspirin. (b) Compted tomography angiogram (CTA) shows a focal narrowing of the M1 segment of the right middle cerebral artery (arrow) and minimal irregularity of the M1 and A1 segments of the left middle cerebral artery and anterior cerebral artery respectively (between arrowheads). x�S�*�*T0T0 BCK L�UЏ�4Rp�W� 4. 1), all within the territory of the posterior circulation. 8. Management of Stroke in Neonates and Children: A Scientific Statement From the American Heart Association/American Stroke Association. endstream Headache free >3 months, Paralysis of lower extremities, gait disturbance, CT (day 1) normal. A disturbance in the control of the smooth muscle tone inside of the brain’s blood vessel walls is thought to cause RCVS. Confusingly, an association of PRES with RCVS has been described particularly in the setting of clinical settings that both conditions have in common (e.g. 79 (6): 882-94. Lee MJ, Cha J, Choi HA, Woo SY, Kim S, Wang SJ, Chung CS. 9 0 obj The findings on imaging consist of either direct visualization of the vascular narrowings and/or complication(s) related to vascular narrowings such as: Transcranial Doppler may show increased arterial velocities suggesting vasospasm and decreased luminal caliber of ICA, MCA and ACA 8. MRI normal (day 5), Smooth narrowing of multiple bilateral proximal vessels (day 6), Normal angiography 16 days after original headache. Reversible Cerebral Vasoconstriction Syndrome in Pediatrics: A Case Series and Review. Focal neurological signs are suggestive of secondary complications including arterial ischaemic stroke or intracranial haemorrhage. As previously noted, CT-scan can be entirely normal with RCVS. 2016;37 (9): 1594-8. Cardiovascular, neurological, and systemic examination was normal. endobj ���#� �`�H
J0�l�8a�`��7w��apOޫ���d3����8����|�(nIn��4���@�S`��r>n�y6ԇ��B����/09�p,��3��K>��X,�p,���Q�� �f~�G6߂���#�>�c�� zv�����;I���F.�r�*�m�h��f�^�g��3��|��p`�j�X���&�8z�?�aϸ��端㌔MV�E
4;�pW���������Bg�:PW[ω�@3�_H��,��Z�e@�, �v@�M��ՙ3�`�!����hFXA���t�v��N�7w��]w�zW��ۙ|$�(f��-w !B�*�k�. endobj 29 (2): 102-5. Short course glucocorticoid therapy has also been advocated. (2017) Annals of neurology. We highlight the typical clinical features of RCVS in this case and suggest that the diagnosis should be considered in children with thunderclap headaches or stroke syndromes where headache is a prominent feature, especially if cerebrovascular imaging studies appear to be evolving or discrepant. 79 (6): 882-94. posterior reversible encephalopathy syndrome (PRES), primary angiitis of the central nervous system (PACNS), Vasoconstriction following subarachnoid hemorrhage, thunderclap headache associated vasospasm. (2016) Annals of neurology. Cortical SAH over right occipital, left frontal, and bilateral parietal lobes on MRI (day 5), Multifocal narrowing of cerebral arteries (day 5), MRI and MRA findings resolved 3 months later Headache free >3 months, CT (day 2) parenchymal haemorrhage between occipital and parietal lobes. Catheter cerebral angiography was undertaken on day 18 in an attempt to make a definitive diagnosis. A 13‐year‐old male developed a sudden severe headache, without other neurological deficits, after swimming in cold seawater. <>stream Around 10% of cases are associated with imaging changes compatible with posterior reversible encephalopathy syndrome. <>>>/BBox[0 0 585 783]/Length 116>>stream Check for errors and try again. These terms include 1,2,4,17: 1. acute benign cerebral angiopathy 2. benign angiopathy of the CNS 3. Five weeks after initial presentation, repeat intracranial and cervical MRA showed resolution of the internal carotid arteries changes previously observed on catheter angiography. 6. No new lesions on MRI. x�%̱�0����5Qz�Q�#QF�{���О)���Ÿ|�W �ڢ�t��[X0�P��#$�檟4�oC�.kD?�ϋ1����r��Th~�Y� w}�wڭ���+��&�m7 81 (3): 454-466. American journal of neuroradiology. Singhal AB, Hajj-Ali RA, Topcuoglu MA, Fok J, Bena J, Yang D, Calabrese LH. endstream endobj Migraine was diagnosed and he was given sumatriptan. Headache free >18 months, Nasal spray (fluticasone); bath related; hypertension, Hyperintense lesion of left occipital region and hyperintensity of sulci on MRI FLAIR images (day 12). What is the cause of reversible cerebral vasoconstriction syndrome (RCVS)? (a) Axial T2‐weighted, (b) sagittal, and (c) axial fluid‐attenuated inversion recovery images showing infarcts (arrows) of the right occipital lobe, right medial parietal lobe, and left cerebellar hemisphere, respectively. Here we report a paediatric case of RCVS to highlight this as a potential differential diagnosis for both acute childhood headache and stroke. T.R. The evolving pathology may lead to discrepancies in serial imaging. Ducros A, Boukobza M, Porcher R et-al. <>>>/BBox[0 0 585 783]/Length 116>>stream The presence of posterior circulation infarcts in a child necessitates consideration of arterial dissection. 12. 2). We report a case of RCVS in a 13‐year‐old male with severe thunderclap headaches and no focal neurological signs. Three days later he presented with an unprovoked severe diffuse headache, only partially controlled with intravenous opiates. Catheter cerebral angiography. Chen SP, Fuh JL, Chang FC, Lirng JF, Shia BC, Wang SJ. 7 0 obj Request PDF | On Dec 1, 2010, M Krämer and others published [Reversible cerebral vasoconstriction syndrome vs cerebral vasculitis? Treatment of RCVS includes pain relief and removal of potential triggers. AJNR Am J Neuroradiol. 12 0 obj 1 0 obj x��UM��0��WX����IU��բU�� 09;2012: 303152. The differential on clinical presentation is essentially that of a thunderclap headache, with the main concern being aneurysmal subarachnoid hemorrhage. x�+� � | The full text of this article hosted at iucr.org is unavailable due to technical difficulties. However, the sudden, severe, and recurrent nature of the headache in RCVS is distinctive and reliably reported in 94% of patients.13 Associated features are nausea, vomiting, photophobia, confusion, and blurred vision.