Cynthia Resende Campos

Bilateral extensive cerebral infarction and mesenteric ischemia associated with segmental arterial mediolysis in two young women

Segmental arterial mediolysis (SAM) is a rare non‐atherosclerotic non‐inflammatory vascular disease that affects mainly muscular arteries of the splanchnic and cerebral territories. Reported herein are two cases of SAM in young women with fatal outcome. One of the patients had an atypical form of the disease, which primarily affected small intestinal submucosal and subserosal arteries, and resulted in acute mesenteric ischemia. The other had bilateral brain infarction with SAM of internal carotid arteries (ICA). Pathological examination of both cases did not reveal the cause of blood flow disturbance: large mesenteric branches of the former and ICA of the latter were free of either dissection or thrombosis; in addition, small intestinal arteries of the first patient did not show signs of vasculitis. These findings suggest that unusual pathways of arterial occlusion and dissection may occur in the context of SAM.

Dissecção espontânea cervical carotídea e verbal: estudo de 48 pacientes

OBJECTIVE To report a Brazilian series of spontaneous cervical arterial dissections, risk factors, warning symptoms, clinical manifestations, diagnostic tests, treatment and prognosis. METHOD We performed the retrospective analysis of clinical and neuroradiological records (MRI, A-MRI and Angiography) of patients with this diagnosis who were evaluated in a tertiary hospital for the period of 1997-2003. RESULTS 48 patients (24 men) with median age 37.9 years: 26 patients with unilateral internal carotid dissection (ICAD), 15 with unilateral vertebral artery dissection (VAD) and 7 with multivessel dissections. All patients presented neurological deficits. Hypertension, smoking and dyslipidemia were the main risk factors. More than 80% of patients presented at least one initial symptom, most of them temporoparietal headache. 44% of patients with VAD and only 3.4% of patients with ICAD had neck pain. The median interval between the onset of symptom and the appearance of neurological deficit was 5.4 days for ICAD and 13.5 days for VAD. Five patients with ICAD presented preceding TIA. Angiography was performed in 93% of patients. In 42% of these patients, MRI and A-MRI were associated. In three patients the diagnosis was made just through cervical MRI. 75% of patients received anticoagulation. Two patients received intravenous thrombolytic therapy with no complications. Prognosis was good for all patients but two patients with bilateral ICAD died. CONCLUSION Our results are similar to the literature, except for the low frequency of neck pain in ICAD patients and predominance of temporoparietal headache in cervical artery dissection patients. Vascular risk factors were commonly found.

A case of primary spinal myoclonus: clinical presentation and possible mechanisms involved

Spinal myoclonus is a rare movement disorder characterized by myoclonic involvement of a group of muscles supplied by a few contiguous segments of the spinal cord. Structural lesions are usually the cause, but in primary spinal myoclonus the etiology remains unknown. We present the case of a 26-year-old woman with cervical spinal myoclonus in which both clinical and electromyographic findings pointed to the segment C1-C3 as the origin of the myoclonus. Laboratorial examinations were normal and no structural lesion was found in magnetic resonance imaging (MRI). Botulinum toxin type A was injected in infrahyoid muscles and cervical paraspinal musculature. The patient remained free of symptoms for almost five months. The pathophysiology of spinal myoclonus remains speculative, but there is evidence that various possible mechanisms can be involved: loss of inhibitory function of local dorsal horn interneurons, abnormal hyperactivity of local anterior horn neurons, aberrant local axons re-excitations and loss of inhibition from suprasegmentar descending pathways.

Espasmo hemifacial bilateral: relato de caso

Bilateral hemifacial spasm (BHS) is a rare focal movement disorder often associated with vascular compression of both facial nerves. The contractions are usually asymmetric and asynchronous. Typically, one side is affected first and there is a long but variable interval for the symptoms on the other side to occur. BHS must be differentiated from other conditions including blefarospasm, facial myokymia, facial tics, oromandibular dystonia, and hemimasticatory spasm. The most successful and non-invasive symtomatic treatment is botulinum toxin injections but microvascular decompression surgery is another therapeutic option. We report the case of a 70 years old man with bilateral hemifacial spasms and present a brief review of the literature.

Isolated oculomotor nerve palsy in spontaneous internal carotid artery dissection: case report.

A paralisia oculosimpatica parcial (sindrome de Horner) seguida por manifestacoes isquemicas cerebrais ou retinianas sao os principais sintomas da disseccao da arteria carotida interna (ACI) extracraniana. O acometimento de nervos cranianos e incomum. Apenas raramente a paralisia isolada do nervo oculomotor pode ser encontrada. CASO: homem de 50 anos, sem diabetes, apresentou cefaleia occipital de inicio subito irradiada a seguir para a regiao retro-orbitaria direita. Apos 5 dias comecou a notar diplopia e visao embassada no olho direito, quando procurou o hospital. O exame neurologico evidenciou apenas prejuizo da aducao e do olhar vertical para cima no olho direito, com discreta dilatacao pupilar ipsilateral, sem ptose. RNM foi normal. A angiografia digital revelou disseccao da ACI direita com oclusao a montante ate a base do crânio. A anticoagulacao foi iniciada. A cefaleia e a movimentacao ocular melhoraram em 3 semanas. DISCUSSAO: A paralisia isolada do nervo oculomotor dificilmente e reconhecida como um possivel sinal clinico de disseccao da ACI. Se o estudo angiografico for incompleto, sem a avaliacao cuidadosa das arterias extracranianas, esse diagnostico deixa de ser feito, impedindo assim o inicio precoce da anticoagulacao para a prevencao dos eventos tromboembolicos. Por isso, reforcamos a importância do estudo das arterias cervicais nos pacientes com paralisia do nervo oculomotor. A compressao mecânica ou o estiramento do nervo sao mecanismos possiveis, mas o prejuizo direto do suprimento sanguineo arterial para o nervo e a explicacao mais plausivel.UNLABELLED Partial oculosympathetic palsy followed by ischemic manifestations in brain or retina are the main symptoms of extracranial internal carotid artery (ICA) dissection. Unusually, cranial nerves may be affected. Isolated oculomotor nerve palsy is found only rarely. CASE We present a 50-year-old nondiabetic man who experienced acute onset of right occipital headache which spread to the right retro-orbital region. Five days later he noticed diplopia and right blurred vision sensation. Neurologic examination disclosed only impaired adduction and upward gaze of right eye, slight ipsilateral pupillary dilatation, without ptosis. Brain MRI was normal. Angiography showed right internal carotid artery dissection with forward occlusion to the base of the skull. Intravenous heparin followed by warfarin was prescribed. The headache and the oculomotor nerve deficit gradually resolved in the next three weeks. DISCUSSION Isolated oculomotor nerve palsy is underrecognized as a clinical presentation of extracranial ICA dissection. If the angiographic evaluation is incomplete without careful study of extracranial arteries, misdiagnosis may lead to failure to initiate early treatment to prevent thromboembolic complications. For this reason we draw attention to the need for careful evaluation of cervical arteries in patients with oculomotor nerve palsy. Mechanical compression or stretching of the third nerve are possible mechanisms, but the direct impairment of the blood supply to the third nerve seems to be the most plausible explanation.

Treatment of subclavian steal syndrome with percutaneous transluminal angioplasty and stenting: case report.

Subclavian steal syndrome refers to the association of neurological symptoms related to vertebrobasilar insufficiency and the phenomenon of subclavian steal. We report the case of a 63 year-old male patient that presented subclavian steal syndrome and severe proximal (80%) stenosis of the left subclavian artery. The patient was submitted to percutaneous transluminal angioplasty and stenting on the left SA. The procedure was well tolerated and immediately afterwards, there was complete remission of the symptoms and of the phenomenon of subclavian steal evaluated by angiography and transcranial doppler. We propose that percutaneous transluminal angioplasty with stenting placement is a good therapeutic option for subclavian steal syndrome.

Movement disorders induced by peripheral trauma

Movement disorders induced by central nervous system trauma are well recognized. However, over the last few years, attention has been drawn to the role of peripherally induced movement disorders. We describe three patients presenting respectively dystonia, tremor and choreoathetosis associated with tremor and dystonia of the body parts previously exposed to traumatic injuries. Pathophysiological mechanisms underlying these phenomena are not entirely known, but functional changes in afferent neuronal input to the spinal cord and secondary affection of higher brain stem and subcortical centers are probably involved.

Oligosymptomatic and giant basilar artery dolichoectasia discovered after a stroke: case report

The most frequently diagnosed complication of vertebrobasilar dolichoectasia (VBD) is the compression of structures adjacent to the vertebral and basilar arteries. A giant VBD with only slight compressive symptoms is unusual. In this setting, the diagnosis of VBD may be casually revealed after the occurrence of a posterior circulation stroke, another potential complication. We report a 48-year-old woman who presented a two-month history of continuous buzz and a slight right-sided hearing loss that was followed by a cerebellar ischemic stroke. Brain CT and MRI revealed a marked compression of the brainstem due to an ectatic, tortuous and partially thrombosed basilar artery (BA). The largest cross-sectional diameter of BA was 18 mm. The patient had a good functional recovery within the two-month follow-up after stroke with modified Rankin scale score (mRSS)=2. At the one-year follow-up, patient still kept the complaints of continuous buzz, slight right-sided hearing loss and the mRSS was the same. We call attention for an unusual giant VBD that caused an impressive brainstem compression with displacement of important structures in an oligosymptomatic patient. Diagnosis was made only after the occurrence of a stroke. Despite of the good functional recovery after stroke, the presence of significant atherosclerotic changes and the large BA diameter may indicate a poor outcome. However, after one year, she remains oligosymptomatic.

INTERNAL CAROTID ARTERY DISSECTION IN A PATIENT WITH RECENT RESPIRATORY INFECTION Case report of a possible link

The pathogenesis of spontaneous cervical artery dissection remains unknown. Infection-mediated damage of the arterial wall may be an important triggering mechanism. We describe a 21 year-old man with respiratory infection (bronchial pneumonia) which was diagnosed and treated with antibiotic few days prior to the right internal carotid artery dissection. The patient presented ischemic retinal and cerebral strokes. Based on literature review, we discuss the possibility of a causal link between infection and arterial dissection.

Pure Alexia and Hemianopia Related to Dissection of the Internal Carotid Artery

Carotid artery dissection is an important cause of stroke and transient ischemic attacks in young and middle-aged patients [1–4]. For hemodynamic and anatomical reasons, it is usually associated with ischemic strokes along the anterior and middle cerebral artery (MCA) territories and in the retinal artery, with most of the patients usually presenting focal deficits related to the involvement of these territories [4]. Posterior cerebral artery (PCA) territory is rarely affected alone in a patient with carotid disease. When it occurs, it is usually associated with the fetal pattern of the circle of Willis which permits access to embolization from the carotid artery causing occipital infarction [5–7]. Few reports of PCA infarctions due to internal carotid artery dissection have been published and the visual field deficit was the main symptom [5, 6]. Differing from previous reports, we describe a patient who presented with pure alexia and mild anomia as the main symptoms of left carotid artery dissection. She is a 49-year-old woman, righthanded, Brazilian, with reading fluency in French and English, having completed university studies and developed commercial activities related to oral and written language. She came to the emergency department because she had noticed difficulty and slowness in reading words for the past 8 days. She had to analyze letter by letter to grasp the meaning. She also developed difficulty in naming objects and people, but she did not complain of any modification in her visual field. On further questioning, the patient referred to severe headaches 2 months earlier, without neck pain, but she had not noticed any modification in her reading skills until 8 days prior to the consultation. She denied transient monocular blindness or hemispheric ischemic attacks. Neurological examination concluded right homonymous hemianopia and a light aphasia with naming disturbances detected in tests with different modalities of stimulus presentation, but no color naming defect. The written language was normal. The patient was only capable of reading by using the grapheme-phoneme conversion, and had difficulty in reading foreign and irregular words which requested global reading and depended on the knowl-