Spinal Cord Infarction in Congenital Afibrinogenemia: A Case Report and Review of the Literature

Baş, Demet Funda; Oğuz, Kader Karlı; Yavuz, Kivilcim; Topçuoğlu, Mehmet Akif

doi:10.1016/j.jstrokecerebrovasdis.2008.11.005

Cited by 6 publications

(2 citation statements)

References 37 publications

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“…Only seven cases of sulcal artery syndromes have been reported with similar characteristics as in our patient [6][7][8][9][10][11][12] and prognosis was indeed good in most of them, although a delayed painful spinothalamic syndrome remains possible.…”

Section: Discussionsupporting

confidence: 71%

Vertebral artery dissection as cause of sulcal artery syndrome

Duprez

Sindic

2014

Acta Neurol Belg

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Vertebral artery dissection (VAD) is a rare cause of ischaemic injury to the cervical spinal cord. We report the case of a young man presenting an extended right VAD leading to a stroke within both the posterior inferior cerebellar artery (PICA) area and within that of a right sulcal branch of the anterior spinal artery responsible for a Brown-Sequard-like syndrome, but with sparing of the lateral part of the medulla oblongata. Case reportIn September 2013, a 33-year-old man presented with sudden onset of vertigo and dizziness increased by postural changes and accompanied by vomiting. He had no tinnitus, diplopia, hearing or visual complaints. Medical history consisted of psoriasis and smoking. He practiced regularly kick-boxing. He was initially admitted to the ENT Department for suspicion of vestibular neuronitis and was treated with tapering doses of methylprednisolone for 6 days without improvement.One week later, he complained with right nuchal pain, slight right ptosis, numbness in the left hemibody under a Th3 level, and paraesthesia in the right hand and forearm.The neurological examination at that time revealed a horizontal and rotatory nystagmus in the right gaze and a right Claude-Bernard-Horner syndrome. Perception of pain and temperature was disturbed only under the left Th3 dermatoma. Right-sided deep tendon reflexes were increased. No other neurological abnormalities were detectable.Biological investigations including CSF analysis were normal, except for dyslipidemia.Brain MRI (Fig. 1) demonstrated a subacute ischaemic stroke of the right cerebellum within the PICA territory, and a tiny right vertebral artery. There was no lesion in the lateral part of the medulla oblongata. MR angiography showed a dissection of the right vertebral artery with a mural thrombus in its V2 segment, and a complete arterial occlusion from its ostium. Spinal cord MRI revealed a right lesion of 3 mm in diameter at the level of C3, matching perfectly with the neurological examination.The patient was treated with atorvastatin and acetylsalicylic acid. In July 2014, he had recovered from balance disturbances, but not from left thermoanalgesia. A control MR angiography showed no recanalization of the right vertebral artery. DiscussionSpinal cord infarction (SCI) is rarely the consequence of a VAD. In a series of 111 patients with such a dissection, only two presented with spinal manifestations [1]. More recently, Hsu et al.[2] made a systematic review of the literature and found 17 cases (nine men and eight women) with VAD-associated SCI. The mean age was 40 years. The main symptoms were headache or neck pain (88 %), presence of a sensory level (76 %), and a Brown-Sequard

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Section: Discussionsupporting

confidence: 71%

Vertebral artery dissection as cause of sulcal artery syndrome

Duprez

Sindic

2014

Acta Neurol Belg

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“…Although spinal cord infarction is rare, 20) a small number of cases of spinal cord infarction and vertebral artery dissection with afibrinogenemia have been reported. 1,5,9) Spontaneous thrombotic complications have also been reported in patients with afibrinogenemia. 8) Fibrin down-regulates thrombin generation by reducing prothrombin activation, and also binds and sequesters thrombin.…”

Section: Discussionmentioning

confidence: 99%

Chronic Subdural Hematoma in a Patient With Congenital Afibrinogenemia Successfully Treated With Fibrinogen Replacement -Case Report-

Sakai

Akamine

Tokuyama

et al. 2011

Neurol. Med. Chir.(Tokyo)

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A 37-year-old woman with congenital afibrinogenemia presented with chronic subdural hematoma (CSDH) manifesting as severe headache, nausea, and somnolence after a minor head trauma. Brain computed tomography scans showed a right subdural hematoma associated with midline shift. Laboratory studies showed prolongation of prothrombin time, activated partial thromboplastin time, and undetectably low level of fibrinogen. Until the present episode, she had received plasma-derived fibrinogen concentrate around menstruation and pregnancy. She had also suffered from spinal cord infarction due to vertebral artery occlusion. Burr-hole evacuation and drainage of CSDH was successfully performed using fibrinogen concentrate. The development of CSDH with afibrinogenemia is very rare. Although the past repeated administrations of fibrinogen concentrate were suspected to generate CSDH, paradoxical thrombotic complications caused by upregulation of prothrombin activation, thrombin generation, and growth factors released from platelets might be related to the development of CSDH with congenital afibrinogenemia.

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Sulcal artery syndrome: A Three-patient series and review of literature

Tan

Lai

et al. 2021

Journal of Clinical Neuroscience

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Spinal Cord Infarction in Congenital Afibrinogenemia: A Case Report and Review of the Literature

Cited by 6 publications

References 37 publications

Vertebral artery dissection as cause of sulcal artery syndrome

Vertebral artery dissection as cause of sulcal artery syndrome

Chronic Subdural Hematoma in a Patient With Congenital Afibrinogenemia Successfully Treated With Fibrinogen Replacement -Case Report-

Sulcal artery syndrome: A Three-patient series and review of literature

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